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---
title: "Absent/Thin Infundibular Stalk"
docid: "28acc91f-2d29-40b9-8a91-b02f16fe1284"
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value: "Bernadette L. Koch, MD"
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value: "Chang Yueh Ho, MD"
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name: "Absent/Thin Infundibular Stalk"
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lastUpdated: "02/01/23"
pageDescription: "Absent/Thin Infundibular Stalk"
pageKeywords: "Brain, Differential Diagnosis, Sella/Juxtasellar, Pineal Region, Anatomically Based Differentials, Absent/Thin Infundibular Stalk"
pageTitle: "Absent/Thin Infundibular Stalk | STATdx"
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---
# ESSENTIAL INFORMATION
- ## Key Differential Diagnosis Issues
- Congenital or acquired
- History of trauma or sellar/suprasellar surgery
- ## Helpful Clues for Common Diagnoses
- **Pituitary Stalk Transection, Posttraumatic**
- Usually occurs following motor vehicle accident
- Can occur with closed head injury
- ± accompanying basilar skull fracture
- **Pituitary Stalk Transection,****Postsurgical**
- Children: Commonly after craniopharyngioma resection
- Adults: Commonly after macroadenoma resection
- ## Helpful Clues for Less Common Diagnoses
- **Septo-Optic Dysplasia**
- Optic nerve hypoplasia, often asymmetric
- ± absent septum pellucidum, sometimes partial
- ± ectopic posterior pituitary
- Pituitary dysfunction in most patients, even if normal-appearing pituitary
- ± polymicrogyria &/or schizencephaly
- **Holoprosencephaly**
- Failure of prosencephalon cleavage
- 70% with diabetes insipidus from hypothalamic fusion
- **Pituitary Stalk Anomalies**
- **Ectopic posterior pituitary**
- Posterior pituitary T1 "bright spot" in hypothalamus
- Rarely partial ectopia: 1 bright spot in normal location & 1 ectopic
- Stalk thin or absent, shallow sella, small pituitary
- Associated with septo-optic dysplasia
- **Duplicated stalk**
- 2 separate, thinned stalks seen on coronal view
- Associated with 2 pituitary glands and basilar arteries
- Tuber cinereum thickened, often fused with mammary bodies
- Craniofacial anomalies
- Theorized from splitting of rostral notochord during blastogenesis
- **Persistent embryonic infundibular recess**
- Proposed from failure of closure of embryonal infundibular recess
- Tubular cyst of stalk connecting 3rd ventricle and sella
- Best seen on sagittal views
- **Pituitary Stalk Interruption Syndrome**
- Pickardt-Fahlbusch syndrome
- Most likely multigenic origin and inheritance pattern
- Whole exome sequencing potential genes related to the Wnt, Notch, & sonic hedgehog signaling pathways that regulate pituitary embryogenesis
- Thin, interrupted, attenuated or absent pituitary stalk
- Aplasia or hypoplasia of anterior pituitary
- Absent or ectopic posterior pituitary
- Hormonal deficiencies except for hyperprolactinemia
- Most patients referred later in childhood for growth retardation
- Viewed as mild form of holoprosencephaly spectrum
- ## Helpful Clues for Rare Diagnoses
- **Meningitis**
- Children with group B streptococcus
- Diencephalic infarction
- Secondary atrophy of optic chiasm, pituitary stalk
- **Neurocysticercosis**
- Racemose neurocysticercosis cysts stretch infundibular stalk
## References
# Selected References
1. [Huang J et al: Noncontrast MRI protocol for selected pediatric pituitary endocrinopathies: a procedure with high diagnostic yield and potential to reduce anesthesia and gadolinium-based contrast exposure. AJNR Am J Neuroradiol. 42(10):1884-90, 2021](http://www.ncbi.nlm.nih.gov/pubmed/?term=34475192%5Bpmid%5D)
1. [Voutetakis A: Pituitary stalk interruption syndrome. Handb Clin Neurol. 181:9-27, 2021](http://www.ncbi.nlm.nih.gov/pubmed/?term=34238482%5Bpmid%5D)
1. [Brauner R et al: Pituitary stalk interruption syndrome is characterized by genetic heterogeneity. PLoS One. 15(12):e0242358, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=33270637%5Bpmid%5D)
1. [Ybarra M et al: A new imaging entity consistent with partial ectopic posterior pituitary gland: report of six cases. Pediatr Radiol. 50(1):107-15, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=31468085%5Bpmid%5D)
1. [Sen D et al: Duplication of the pituitary gland - plus syndrome. Indian J Radiol Imaging. 26(1):126-30, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=27081236%5Bpmid%5D)
1. [Voutetakis A et al: Pituitary stalk interruption syndrome: cause, clinical manifestations, diagnosis, and management. Curr Opin Pediatr. 28(4):545-50, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=27386973%5Bpmid%5D)
1. [Glastonbury CM et al: Masses and malformations of the third ventricle: normal anatomic relationships and differential diagnoses. Radiographics. 31(7):1889-905, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=22084178%5Bpmid%5D)
1. [Reynaud R et al: Pituitary stalk interruption syndrome in 83 patients: novel HESX1 mutation and severe hormonal prognosis in malformative forms. Eur J Endocrinol. 164(4):457-65, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=21270112%5Bpmid%5D)
1. [Steno A et al: Persisting embryonal infundibular recess. J Neurosurg 110: 359-62, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=18950267%5Bpmid%5D)
## Images
### Selected Images
![Sagittal T1 C+ MR shows surgical absence of the pituitary stalk and hypothalamus <img src='img/arrows/WS.png'/> from a transsphenoidal approach. Postsurgical changes are seen in the sphenoid sinus and nasal cavity <img src='img/arrows/WO.png'/>.](images/app.statdx.com_image_thumbnail_6d76d6a2-d15c-46fd-a2cd-5d203ab5445f_annotated_true_size_900_quality_90_b19a5633.jpg)
**Pituitary Stalk Transection, Posttraumatic**
*Sagittal T1 C+ MR shows surgical absence of the pituitary stalk and hypothalamus <img src='img/arrows/WS.png'/> from a transsphenoidal approach. Postsurgical changes are seen in the sphenoid sinus and nasal cavity <img src='img/arrows/WO.png'/>.*
![Sagittal T1 C+ MR shows surgical absence of the pituitary stalk and hypothalamus <img src='img/arrows/WS.png'/> from a transsphenoidal approach. Postsurgical changes are seen in the sphenoid sinus and nasal cavity <img src='img/arrows/WO.png'/>.](images/app.statdx.com_image_thumbnail_6d76d6a2-d15c-46fd-a2cd-5d203ab5445f_size_174_quality_85_22152398.jpg)
**Pituitary Stalk Transection, Posttraumatic**
*Sagittal T1 C+ MR shows surgical absence of the pituitary stalk and hypothalamus <img src='img/arrows/WS.png'/> from a transsphenoidal approach. Postsurgical changes are seen in the sphenoid sinus and nasal cavity <img src='img/arrows/WO.png'/>.*
![Coronal T2 MR shows absence of the septum pellucidum <img src='img/arrows/WS.png'/> and thinning of the pituitary infundibulum <img src='img/arrows/WO.png'/>. Other midline defects of septo-optic dysplasia not shown here include optic nerve hypoplasia and ectopic posterior pituitary.](images/app.statdx.com_image_thumbnail_0b4c395a-c4b4-4670-8656-e48c068ee456_annotated_true_size_900_quality_90_8c38db86.jpg)
**Septo-Optic Dysplasia**
*Coronal T2 MR shows absence of the septum pellucidum <img src='img/arrows/WS.png'/> and thinning of the pituitary infundibulum <img src='img/arrows/WO.png'/>. Other midline defects of septo-optic dysplasia not shown here include optic nerve hypoplasia and ectopic posterior pituitary.*
![Coronal T1 MR shows absence of the septum pellucidum <img src='img/arrows/WS.png'/> and nodular T1 hyperintensity in the hypothalamus <img src='img/arrows/WO.png'/>, consistent with ectopic posterior pituitary. The infundibulum is not visualized.](images/app.statdx.com_image_thumbnail_e6b8e283-8d3c-42b9-ad8f-11e869dcfca8_annotated_true_size_900_quality_90_5f5c920b.jpg)
**Septo-Optic Dysplasia**
*Coronal T1 MR shows absence of the septum pellucidum <img src='img/arrows/WS.png'/> and nodular T1 hyperintensity in the hypothalamus <img src='img/arrows/WO.png'/>, consistent with ectopic posterior pituitary. The infundibulum is not visualized.*
![Sagittal T1 MR shows ectopic posterior pituitary <img src='img/arrows/WS.png'/>, absent infundibulum, and shallow sella with hypoplastic pituitary gland <img src='img/arrows/WO.png'/>. Additionally, there is dysplasia of the callosal genu <img src='img/arrows/WC.png'/> with thickening around the anterior commissure from fusion of the caudate nuclei <img src='img/arrows/BS.png'/> in this case of semilobar holoprosencephaly.](images/app.statdx.com_image_thumbnail_346e2b4b-e78f-4ffb-9895-8fd6de44e10d_annotated_true_size_900_quality_90_6409ccec.jpg)
**Holoprosencephaly**
*Sagittal T1 MR shows ectopic posterior pituitary <img src='img/arrows/WS.png'/>, absent infundibulum, and shallow sella with hypoplastic pituitary gland <img src='img/arrows/WO.png'/>. Additionally, there is dysplasia of the callosal genu <img src='img/arrows/WC.png'/> with thickening around the anterior commissure from fusion of the caudate nuclei <img src='img/arrows/BS.png'/> in this case of semilobar holoprosencephaly.*
![Sagittal T1 MR in a child with short stature and growth hormone deficiency shows a lack of normal posterior pituitary bright spot in the dorsal aspect of the sella. Instead, there is an ectopic posterior pituitary bright spot <img src='img/arrows/WS.png'/> and absent pituitary stalk <img src='img/arrows/WO.png'/>.](images/app.statdx.com_image_thumbnail_078395ee-cc72-41f1-8e60-01e8e2850c9e_annotated_true_size_900_quality_90_0d978bc9.jpg)
**Pituitary Stalk Anomalies**
*Sagittal T1 MR in a child with short stature and growth hormone deficiency shows a lack of normal posterior pituitary bright spot in the dorsal aspect of the sella. Instead, there is an ectopic posterior pituitary bright spot <img src='img/arrows/WS.png'/> and absent pituitary stalk <img src='img/arrows/WO.png'/>.*
![T1 C+ MR shows a T1-hyperintense mass of the hypothalamus <img src='img/arrows/WS.png'/>, which was hyperintense prior to contrast. There is a lack of normal enhancing infundibulum. Ectopic posterior pituitary and absent stalk is a potential radiographic finding for stalk interruption syndrome.](images/app.statdx.com_image_thumbnail_30e80358-27e8-4cc4-8860-2675e05c97d1_annotated_true_size_900_quality_90_102b5c11.jpg)
**Pituitary Stalk Interruption Syndrome**
*T1 C+ MR shows a T1-hyperintense mass of the hypothalamus <img src='img/arrows/WS.png'/>, which was hyperintense prior to contrast. There is a lack of normal enhancing infundibulum. Ectopic posterior pituitary and absent stalk is a potential radiographic finding for stalk interruption syndrome.*
![Coronal T1 C+ FS MR in an 8-year-old child with multiple congenital anomalies demonstrates duplicated infundibular stalks <img src='img/arrows/WS.png'/> and adenohypophyses <img src='img/arrows/WO.png'/> and thickening of the hypothalamus <img src='img/arrows/WC.png'/>.](images/app.statdx.com_image_thumbnail_5f111ecd-64b5-496a-85e3-9990c167c926_annotated_true_size_900_quality_90_a516ae5e.jpg)
**Pituitary Stalk Anomalies**
*Coronal T1 C+ FS MR in an 8-year-old child with multiple congenital anomalies demonstrates duplicated infundibular stalks <img src='img/arrows/WS.png'/> and adenohypophyses <img src='img/arrows/WO.png'/> and thickening of the hypothalamus <img src='img/arrows/WC.png'/>.*
![Axial T2 MR shows a persistent infundibular recess <img src='img/arrows/WS.png'/>. There is additional cleft lip <img src='img/arrows/WO.png'/> and hydrocephalus with medial atrial diverticulum <img src='img/arrows/WC.png'/>.](images/app.statdx.com_image_thumbnail_e09bc4ee-6678-4578-9101-f801f4c9c981_annotated_true_size_900_quality_90_7c5969e4.jpg)
**Pituitary Stalk Anomalies**
*Axial T2 MR shows a persistent infundibular recess <img src='img/arrows/WS.png'/>. There is additional cleft lip <img src='img/arrows/WO.png'/> and hydrocephalus with medial atrial diverticulum <img src='img/arrows/WC.png'/>.*
### Additional Images
![Sagittal T1 MR shows enlarged CSF space communicating from a displaced 3rd ventricle into a persistent infundibular recess <img src='img/arrows/WS.png'/>. Note fat within a persistent craniopharyngeal canal <img src='img/arrows/WO.png'/>, cleft palate <img src='img/arrows/WC.png'/>, and downward herniation of the cerebellum <img src='img/arrows/BS.png'/> due to hydrocephalus from aqueductal stenosis.](images/app.statdx.com_image_thumbnail_705f6326-0234-4b0e-b422-84c774b37299_annotated_true_size_900_quality_90_f70b4793.jpg)
**Pituitary Stalk Anomalies**
*Sagittal T1 MR shows enlarged CSF space communicating from a displaced 3rd ventricle into a persistent infundibular recess <img src='img/arrows/WS.png'/>. Note fat within a persistent craniopharyngeal canal <img src='img/arrows/WO.png'/>, cleft palate <img src='img/arrows/WC.png'/>, and downward herniation of the cerebellum <img src='img/arrows/BS.png'/> due to hydrocephalus from aqueductal stenosis.*
![.Coronal T1 MR shows thinned, duplicated infundibular stalks <img src='img/arrows/WS.png'/> and thickening of the tuber cinereum <img src='img/arrows/WO.png'/>.](images/app.statdx.com_image_thumbnail_0db8504b-e574-4415-8c05-82f98921b91f_annotated_true_size_900_quality_90_0fe9295c.jpg)
**Pituitary Stalk Anomalies**
*.Coronal T1 MR shows thinned, duplicated infundibular stalks <img src='img/arrows/WS.png'/> and thickening of the tuber cinereum <img src='img/arrows/WO.png'/>.*
![Sagittal T1 MR in a patient with hypopituitarism following transsphenoidal hypophysectomy <img src='img/arrows/WS.png'/> shows an empty sella <img src='img/arrows/WO.png'/> and a very thin, attenuated infundibular stalk <img src='img/arrows/WC.png'/>.](images/app.statdx.com_image_thumbnail_6060e2fa-39ff-48a1-b79d-4646292f23ed_annotated_true_size_900_quality_90_1fb5c9ed.jpg)
**Pituitary Stalk Transection, Posttraumatic**
*Sagittal T1 MR in a patient with hypopituitarism following transsphenoidal hypophysectomy <img src='img/arrows/WS.png'/> shows an empty sella <img src='img/arrows/WO.png'/> and a very thin, attenuated infundibular stalk <img src='img/arrows/WC.png'/>.*
![Sagittal T1 C+ FS MR following transsphenoidal hypophysectomy shows that the thinned stalk <img src='img/arrows/WS.png'/> is stretched and retracted inferiorly into the partially empty sella <img src='img/arrows/BO.png'/>.](images/app.statdx.com_image_thumbnail_ebcdb319-d866-4c4f-af85-3b69e9c9a462_annotated_true_size_900_quality_90_4f962155.jpg)
**Pituitary Stalk Transection, Postsurgical**
*Sagittal T1 C+ FS MR following transsphenoidal hypophysectomy shows that the thinned stalk <img src='img/arrows/WS.png'/> is stretched and retracted inferiorly into the partially empty sella <img src='img/arrows/BO.png'/>.*
![Coronal T1 MR in a child with remote head trauma who subsequently developed pituitary insufficiency shows traumatic cephalocele <img src='img/arrows/WS.png'/> and absent/inapparent pituitary stalk <img src='img/arrows/WO.png'/>.](images/app.statdx.com_image_thumbnail_1dae8f96-c492-4c30-a71a-f511810347ba_annotated_true_size_900_quality_90_e310a940.jpg)
**Pituitary Stalk Transection, Posttraumatic**
*Coronal T1 MR in a child with remote head trauma who subsequently developed pituitary insufficiency shows traumatic cephalocele <img src='img/arrows/WS.png'/> and absent/inapparent pituitary stalk <img src='img/arrows/WO.png'/>.*
![Sagittal T1WI MR after transsphenoidal hypophysectomy shows a very thin infundibular stalk <img src='img/arrows/WS.png'/> along with secondary empty sella <img src='img/arrows/WO.png'/>.](images/app.statdx.com_image_thumbnail_d8f6d968-c411-4d65-b799-83c1b7c3d13a_annotated_true_size_900_quality_90_5221c36f.jpg)
**Pituitary Stalk Transection, Postsurgical**
*Sagittal T1WI MR after transsphenoidal hypophysectomy shows a very thin infundibular stalk <img src='img/arrows/WS.png'/> along with secondary empty sella <img src='img/arrows/WO.png'/>.*
![Sagittal T1 MR in a 2-year-old child with short stature and abnormally low bone age shows an absent infundibular stalk <img src='img/arrows/WS.png'/>, ectopic posterior pituitary bright spot <img src='img/arrows/WO.png'/>, and small pituitary gland <img src='img/arrows/WC.png'/>.](images/app.statdx.com_image_thumbnail_883db80b-f6fe-4bb0-a220-004404087434_annotated_true_size_900_quality_90_7d85cfc1.jpg)
**Pituitary Stalk Anomalies**
*Sagittal T1 MR in a 2-year-old child with short stature and abnormally low bone age shows an absent infundibular stalk <img src='img/arrows/WS.png'/>, ectopic posterior pituitary bright spot <img src='img/arrows/WO.png'/>, and small pituitary gland <img src='img/arrows/WC.png'/>.*
![Coronal T1 MR shows absent stalk <img src='img/arrows/WS.png'/> and ectopic posterior pituitary bright spot in the hypothalamus <img src='img/arrows/WO.png'/>.](images/app.statdx.com_image_thumbnail_5d976ecd-e6a5-4f04-9a6f-564c7f3b2518_annotated_true_size_900_quality_90_4e71ea09.jpg)
**Pituitary Stalk Anomalies**
*Coronal T1 MR shows absent stalk <img src='img/arrows/WS.png'/> and ectopic posterior pituitary bright spot in the hypothalamus <img src='img/arrows/WO.png'/>.*
![Sagittal T1 MR shows 2 small-sized pituitary stalks <img src='img/arrows/WS.png'/> in this case of duplicated pituitary stalks. Note abnormal configuration of posterior pituitary <img src='img/arrows/WO.png'/>.](images/app.statdx.com_image_thumbnail_7718cf06-2ba5-4c3b-8388-85f04e2baab7_annotated_true_size_900_quality_90_30f75811.jpg)
**Pituitary Stalk Anomalies**
*Sagittal T1 MR shows 2 small-sized pituitary stalks <img src='img/arrows/WS.png'/> in this case of duplicated pituitary stalks. Note abnormal configuration of posterior pituitary <img src='img/arrows/WO.png'/>.*
![Coronal T2 MR shows classic SOD with thin stalk <img src='img/arrows/BC.png'/>, inferiorly &quot;pointed&quot; frontal horns <img src='img/arrows/WS.png'/> , and absent septum pellucidum with squared-off appearance of lateral ventricles <img src='img/arrows/WO.png'/>.](images/app.statdx.com_image_thumbnail_9bcb2dfa-f9ec-45d0-9ecb-06ff59eacd45_annotated_true_size_900_quality_90_254a70d3.jpg)
**Septo-Optic Dysplasia**
*Coronal T2 MR shows classic SOD with thin stalk <img src='img/arrows/BC.png'/>, inferiorly &quot;pointed&quot; frontal horns <img src='img/arrows/WS.png'/> , and absent septum pellucidum with squared-off appearance of lateral ventricles <img src='img/arrows/WO.png'/>.*
![Sagittal T1 C+ MR shows racemose neurocysticercosis cysts <img src='img/arrows/WS.png'/> surrounding and stretching the infundibular stalk <img src='img/arrows/WO.png'/>, extending into the sella and flattening the pituitary gland against the floor <img src='img/arrows/WC.png'/>.](images/app.statdx.com_image_thumbnail_8f2dbe9c-e5f6-40a5-b3e1-2e3a96ffa6a2_annotated_true_size_900_quality_90_5e32418c.jpg)
**Neurocysticercosis**
*Sagittal T1 C+ MR shows racemose neurocysticercosis cysts <img src='img/arrows/WS.png'/> surrounding and stretching the infundibular stalk <img src='img/arrows/WO.png'/>, extending into the sella and flattening the pituitary gland against the floor <img src='img/arrows/WC.png'/>.*
docs_md/articles/adrenal-adenoma_e2916d86-5f9f-4dd3-9576-1a7b89d8dda0.md
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---
title: "Adrenal Adenoma"
docid: "e2916d86-5f9f-4dd3-9576-1a7b89d8dda0"
authors:
- key: "c3463c5c-31d3-4489-bbfe-6b895abdb86d"
value: "Mitchell Tublin, MD"
- key: "b1738976-d5a8-48bc-a435-ed1434cd451a"
value: "Mark D. Sugi, MD"
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name: "Genitourinary"
slug: "genitourinary"
treeNodeId: "bd0eb4fe-d465-4faa-a3b7-526e8f01802d"
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name: "Diagnosis"
slug: "diagnosis"
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name: "Adrenal"
slug: "adrenal"
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slug: "benign-neoplasms"
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name: "Adrenal Adenoma"
slug: "adrenal-adenoma"
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category: "Genitourinary"
documentVersionId: "8ad724c2-555b-4bbf-8716-75f7b7b7c611"
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lastUpdated: "10/04/21"
pageDescription: "Adrenal Adenoma"
pageKeywords: "Genitourinary, Diagnosis, Adrenal, Benign Neoplasms, Adrenal Adenoma"
pageTitle: "Adrenal Adenoma | STATdx"
enhancedTitle: "Adrenal Adenoma"
type: "DX"
references: true
tables: 1
breadcrumbs:
- "Genitourinary"
- "Diagnosis"
- "Adrenal"
- "Benign Neoplasms"
- "Adrenal Adenoma"
---
# KEY FACTS
- ## Imaging
- Well-circumscribed, uniform, low-attenuation, small adrenal mass
- Low attenuation due to abundant intracytoplasmic lipid
- Imaging features of typical lipid-rich adenomas
- NECT: < 10 HU (71% sensitivity, 98% specificity)
- MR: Significant decrease in signal on out-of-phase T1WI due to intravoxel lipid and water
- May show focal areas of heterogeneous attenuation or absence of signal loss due to degeneration, hemorrhage, and fibrin deposition
- Clinical context key to differentiate from collision tumor: Unlikely in absence of extraadrenal malignancy
- Lipid-poor adenomas (10-40% cases): Utilize relative or absolute CT contrast washout kinetics for diagnosis
- Accounts for vast majority of adrenal "incidentalomas"
- Imaging intensive algorithm suggested for incidental adrenal lesions, though overwhelming majority are benign and hormonally inactive
- Primary hyperaldosteronism (Conn syndrome): 80% due to unilateral, typically small (< 2 cm) adenoma
- Cushing syndrome: 80-85% due to adrenal hyperplasia
- Typically shows FDG uptake < that of liver on PET/CT
- ## Top Differential Diagnoses
- Adrenal metastases and lymphoma
- Adrenal (macronodular) hyperplasia
- Pheochromocytoma
- Adrenal carcinoma
- Adrenal myelolipoma
- Gastric diverticulum
- Adrenal cyst
- ## Diagnostic Checklist
- Asymptomatic mass: Usually nonfunctioning adenoma, even in patients with known cancer
- NECT and MR are equally accurate for diagnosis of lipid-rich adenoma
- Utilize dedicated CECT adrenal protocol with 15-minute delayed imaging for diagnosis of potential lipid-poor adenomas
# TERMINOLOGY
- ## Definitions
- Benign adrenal cortical tumor
# IMAGING
- ## General Features
- ### Best diagnostic clue
- Imaging strategies target typical adenoma histology: Abundant intracytoplasmic lipid
- Low attenuation (< 10 HU) on NECT
- Significant loss of signal on out-of-phase T1WI MR (intravoxel fat and water)
- ### Size
- Cushing syndrome adenoma: 2-5 cm
- Conn syndrome adenoma: Classically < 2 cm (20% < 1 cm)
- Vast majority of incidental, hormonally inactive adrenal adenomas are small (< 2 cm)
- ### Morphology
- Usually round to oval suprarenal mass
- Key concepts
- Most common adrenal cortex tumor (10% bilateral)
- Accounts for > 90% of all "incidentalomas"
- May occur in up to 9% of general population, diagnosed on 5% of CT exams with various indications
- Lipid-rich adrenal adenoma: 60-90% of adenomas
- Lipid-poor adrenal adenoma: 10-40% of adenomas
- Increased incidence in patients with diabetes and hypertension
- NECT (or chemical shift MR): Study of choice to diagnose incidental adrenal masses
- Classified into 2 types based on function
- Nonhyperfunctioning: Normal hormone levels
- Hyperfunctioning: Primary hyperaldosteronism, Cushing syndrome, hyperandrogenism
- **Cushing syndrome**
- 15-25% of cases are due to autonomous adrenal adenoma
- 80-85% of cases are due to**adrenal hyperplasia**
- Adenomas usually > 2 cm
- **Primary hyperaldosteronism (Conn syndrome)**
- 80% of cases are due to****adrenal adenoma
- 20% of cases are due to adrenal hyperplasia
- Adenomas are often small (< 2 cm)
- ## CT Findings
- ### NECT
- Smooth, well defined, round or oval in shape
- Homogeneous soft tissue mass of 0-20 HU
- **Lipid-rich adrenal adenoma** (60-90% of cases)
- Uniform low attenuation
- Metaanalysis of < 10 HU threshold: 71% sensitivity, 98% specificity
- Sensitivity may increase to almost 90% with histogram analysis (identify negative pixels), though variable results and scanner dependent
- **Lipid-poor adrenal adenoma** (10-40% of cases)
- Attenuation varies from 10-30 HU
- Difficult to differentiate from metastases on NECT
- Cushing syndrome due to adrenal adenoma
- Remainder of ipsilateral gland and contralateral adrenal gland may be atrophic due to ↓ ACTH levels
- ↑ cortisol: Feedback inhibition on pituitary ACTH
- ACTH-independent macronodular hyperplasia: Multiple, bilateral, functioning adrenal adenomas
- Conn syndrome due to adrenal adenoma
- Remainder of ipsilateral gland and contralateral adrenal gland appear normal
- Large adenomas
- More heterogeneous than small adenomas
- ± hemorrhage, cystic degeneration, calcification
- Growth should raise suspicion for malignancy
- ### CECT
- Enhancing adrenal mass that deenhances rapidly
- Dedicated adrenal CT exam incorporates initial dynamic enhanced phase (~ 70-second delay) and 15-minute delay
- Relative percentage washout = dynamic enhanced (HU) - delayed (HU) / dynamic enhanced HU
- Relative percentage washout > 40%: 96% sensitivity, 100% specificity
- Absolute percentage washout (if NECT available) = dynamic enhanced (HU) - delayed (HU) / dynamic enhanced (HU) - unenhanced (HU)
- Absolute percentage washout > 60%: 86-88% sensitivity, 92-96% specificity
- 10-minute delay utilized by some centers, but shorter delay may decrease sensitivity
- Adrenal washout calculators readily available online
- Utilize technique for indeterminate, potentially lipid-poor adenomas
- Clinical context critical: Rapid washout can be seen with pheochromocytomas, renal cell, hepatocellular carcinoma, and hypervascular metastases
- Dual-energy CT and iodine subtraction techniques can generate virtual noncontrast (VNC) images
- May identify lipid-rich adenomas and obviate need for additional imaging
- Iodine:VNC ratio ≥ 6.7 has sensitivity and specificity of 95% for adenoma (higher ratios in adenoma compared to metastasis)
- ## MR Findings
- T1WI and T2WI
- Low to intermediate signal
- Chemical shift (in- and out-of-phase) imaging
- Mainstay of MR diagnosis
- Sensitivity and specificity equivalent to NECT
- Signal loss on out-of-phase T1WI due to intravoxel water and fat protons
- Inverse relationship between percentage of lipid-rich cells and relative ↓ signal on out-of-phase imaging
- May not identify lipid-poor adenomas
- Visual inspection of signal in phase (SIP) and out of phase (SOP), though quantitative analysis may be helpful
- Adrenal to spleen chemical shift imaging (CSI) ratio: Lesion:spleen SOP/adrenal/spleen SIP
- < .71 = adenoma
- Adrenal signal intensity index: 100 x (SIP - SOP) / SIP
- > 16.5% = adenoma
- Beware technical pitfalls
- Sampling of 1st echo pair at 3T is challenging
- India ink artifact mimics signal loss, particularly in small adrenal lesions
- Other primary or secondary adrenal lesions may contain lipid
- Adenomas and metastases may coexist in same gland (collision tumor)
- T1 C+ MR
- Rapid, uniform enhancement and deenhancement
- Ancillary MR techniques
- Diffusion MR: Not specific (ADC overlap between adenomas and metastases)
- MR spectroscopy: Choline:creatinine and choline:lipid ratio discriminatory threshold ratios may aid in adrenal lesion characterization, though larger studies needed
- ## Ultrasonographic Findings
- ### Grayscale ultrasound
- Nonspecific, solid suprarenal mass
- Right suprarenal mass seen more clearly left due to acoustic window provided by liver
- ## Angiographic Findings
- Conventional
- Adrenal arteriography
- Catheterization of renal or inferior adrenal arteries shows vascular supply of adrenal tumors
- Adenomas are usually hypo- to moderately vascular
- No arterial encasement or venous laking or puddling, which are malignant vascular features
- Adrenal venography
- Most commonly to obtain adrenal vein samples
- Advocated for patients with primary hyperaldosteronism triaged to adrenalectomy
- Technically difficult study but may confirm laterality of small, aldosterone-secreting adenoma
- Technical approach and criteria for positive study varies; ACTH stimulation may increase accuracy
- Adrenal adenoma is seen as filling defect within adrenal gland displacing adjacent vessels
- Circumferential vein frequently seen around adrenal adenoma
- ## Nuclear Medicine Findings
- PET/CT
- Utilized as part of malignancy staging
- Markedly increased F-18 FDG uptake characteristic of metastases
- Adenomas may also accumulate F-18 FDG, typically less intense than liver
- Potential false-negatives: Metastases from primary carcinomas that are non-FDG avid (e.g., neuroendocrine tumors)
- SUV thresholds published but adenomas typically less intense than liver
- Adrenocortical scintigraphy by using NP-59
- NP-59 is cholesterol analog that binds to low-density lipoprotein receptors of adrenal cortex
- NP-59 used and dexamethasone: Accentuate uptake in non-ACTH-dependent adrenal tissues (adenoma)
- Normal NP-59: When both adrenal glands are seen 5 days after injection or thereafter
- Adrenal adenoma: Unilateral early adrenal visualization before day 5 after NP-59 injection
- Adrenal hyperplasia: Bilateral early adrenal visualization before day 5 after NP-59 injection
- ## Imaging Recommendations
- NECT is initial study of choice to confirm diagnosis of lipid-rich adrenal adenoma
- ROI should encompass lesion: Attenuation < 10 HU is diagnostic
- In- and out-of-phase MR equivalent to NECT for lipid-rich lesions
- Signal dropout on out-of-phase T1WI MR: Qualitative assessment typically suffices
- CECT, including 15-minute delayed phase, used for potential lipid-poor adenomas: Calculate either relative or absolute washout
# DIFFERENTIAL DIAGNOSIS
- [Adrenal Metastases and Lymphoma](/document/adrenal-lymphoma/44639c90-bd04-4e2a-a470-2c28a0e2ff78)
- Adrenal metastases
- Unilateral or bilateral masses ± central necrosis, hemorrhage
- Usually known to have malignancy elsewhere
- NECT: Metastases mimic lipid-poor adenoma
- CECT: Hypo- or hypervascular and prolonged washout pattern
- Adrenal lymphoma
- Usually spread to adrenal gland from retroperitoneal tumor
- Unilateral or bilateral masses
- Unilateral primary lymphoma (non-Hodgkin) can mimic adenoma
- Hypovascular; moderate enhancement with contrast
- [Adrenal Myelolipoma](/document/adrenal-myelolipoma/5813a554-06a4-4696-af71-7ce50693039d)
- Small or large, asymptomatic adrenal mass
- Intramural macroscopic fatty elements on imaging
- [Adrenal Hyperplasia](/document/adrenal-hyperplasia/90d09395-41d4-49b4-bb1d-4cb00b8bc272)
- Adrenal glands are often symmetrically enlarged
- Width of adrenal gland limbs > 10 mm (diagnostic)
- No discrete mass or nodule seen as rule
- Dominant macronodule of macronodular hyperplasia mimics small adrenal adenomas
- Cortisol-secreting adenoma: Remainder of ipsilateral and contralateral glands, atrophic (↓ ACTH)
- Macronodular hyperplasia: Both glands are enlarged (due to elevated ACTH levels)
- No obvious enhancement and washout pattern seen
- [Pheochromocytoma](/document/pheochromocytoma/7d3c4062-643c-4030-8783-f85184ad8132)
- Tumor > 3 cm in most cases; classically T2 hyperintense
- Highly vascular tumor prone to hemorrhage, necrosis
- Bilateral adrenal tumors in multiple endocrine neoplasia (MEN) syndromes
- [Unilateral Adrenal Hemorrhage](/document/adrenal-hemorrhage/5812e5c4-ca8a-4af5-884b-f75795bcde0f)
- Chronic hematoma: Well-defined, round, low-density, mass-like lesion simulating adenoma
- [Adrenal Carcinoma](/document/adrenal-cortical-carcinoma/bdc7a08b-a64f-4bd2-9dfc-24331728e85e)
- Rare, unilateral, invasive and enhancing mass
- > 6 cm when initially diagnosed
- [Gastric Diverticulum](/document/gastric-diverticulum/eeb101f0-8bdf-4771-b44a-fe6e73b3a463)
- Abnormal, rounded soft tissue lesion in left suprarenal area; mimics adrenal mass
- Diverticular contents do not enhance, whereas adenomas do
- Distend stomach with gas and fluid; scan in prone position to distend diverticulum
- [Ganglioneuroma](/document/pheochromocytoma/7d3c4062-643c-4030-8783-f85184ad8132)
- Younger patients; mean age: 27 years
- Larger mass; average tumor size: 8 cm
- ## Adrenal Cyst
- Attenuation similar to lipid-rich adenoma
- Lack of enhancement, rim calcification may suggest diagnosis
# PATHOLOGY
- ## General Features
- ### Etiology
- Unknown
- ### Associated abnormalities
- MEN syndromes
- Most adrenals with adenoma have normal function
- Occasionally adenoma causes adrenal hyperfunction
- Normal adrenocortical secretory hormones
- Cortisol, aldosterone, androgens
- ## Gross Pathologic & Surgical Features
- Well-delineated, tan-yellow, ovoid mass
- 3 microscopic patterns
- Pure (fasciculata- or reticularis-type cells), mixed, or hybrid
- May have focal areas of degeneration, hemorrhage, and fibrin deposition
- ## Microscopic Features
- 70% of adenomas: High % of intracytoplasmic lipid
- 30% of adenomas: Low % of intracytoplasmic lipid
# CLINICAL ISSUES
- ## Presentation
- ### Most common signs/symptoms
- Asymptomatic incidental CT finding
- Conn syndrome: Hypertension and weakness
- Cushing syndrome: Moon facies, truncal obesity, purple striae, and buffalo hump
- Virilization in women
- Lab data: ↑ aldosterone, cortisol, &/or androgens
- Diagnosis: Clinical, biochemical, imaging, histology
- ## Demographics
- ### Age
- Prevalence of adenoma increases with age
- Peak at 60-69 years, decreasing thereafter
- ### Epidemiology
- Most common adrenal tumor of all incidentalomas
- ↑ incidence in patients with diabetes or hypertension
- Occurs in up to 9% of population (postmortem data)
- ## Natural History & Prognosis
- Prognosis: Excellent when incidental and nonhyperfunctioning
- ## Treatment
- No treatment when asymptomatic incidental finding
- Laparoscopic removal of gland if hyperfunctioning
# DIAGNOSTIC CHECKLIST
- ## Consider
- Asymptomatic mass: Usually nonhyperfunctioning adenoma, even in patient with known cancer
- ## Image Interpretation Pearls
- Well-defined, low-density (< 10 HU) suprarenal mass
- Enhances with washout pattern > 50% within 15 minutes
- Out-of-phase T1WI MR: Signal dropout, lipid-rich mass
00eaacf1-f275-4da9-8c9b-128eb7c97fee
## References
# Selected References
1. [Nagayama Y et al: Adrenal adenomas versus metastases: diagnostic performance of dual-energy spectral CT virtual noncontrast imaging and iodine maps. Radiology. 296(2):324-32, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=32452733%5Bpmid%5D)
1. [Corwin MT et al: Differences in growth rate on CT of adrenal adenomas and malignant adrenal nodules. AJR Am J Roentgenol. 213(3):632-6, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31039016%5Bpmid%5D)
1. [Elbanan MG et al: Adrenal cortical adenoma: current update, imaging features, atypical findings, and mimics. Abdom Radiol (NY). 45(4):905-16, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=31529204%5Bpmid%5D)
1. [Hekimsoy İ et al: Characterization of adrenal lesions on chemical shift MRI: comparison of 1.5 T and 3 T MRI. Abdom Radiol (NY). 44(10):3359-69, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31129784%5Bpmid%5D)
1. [Liu T et al: Distinguishing adrenal adenomas from non-adenomas with multidetector CT: evaluation of percentage washout values at a short time delay triphasic enhanced CT. Br J Radiol. 92(1094):20180429, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=30433826%5Bpmid%5D)
1. [Platzek I et al: Chemical shift imaging for evaluation of adrenal masses: a systematic review and meta-analysis. Eur Radiol. 29(2):806-17, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=30014203%5Bpmid%5D)
1. [Humbert AL et al: The computed tomography adrenal wash-out analysis properly classifies cortisol secreting adrenocortical adenomas. Endocrine. 59(3):529-37, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=29332161%5Bpmid%5D)
1. [Mohammed MF et al: Pheochromocytomas versus adenoma: role of venous phase CT enhancement. AJR Am J Roentgenol. 210(5):1073-8, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=29570377%5Bpmid%5D)
1. [Rocha TO et al: Histogram analysis of adrenal lesions with a single measurement for 10th percentile: feasibility and incremental value for diagnosing adenomas. AJR Am J Roentgenol. 211(6):1227-33, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=30299995%5Bpmid%5D)
1. [Thomas AJ et al: Interobserver agreement in distinguishing large adrenal adenomas and adrenocortical carcinomas on computed tomography. Abdom Radiol (NY). 43(11):3101-8, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=29671009%5Bpmid%5D)
1. [Tu W et al: Can adrenal adenomas be differentiated from adrenal metastases at single-phase contrast-enhanced CT? AJR Am J Roentgenol. 211(5):1044-50, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=30207794%5Bpmid%5D)
1. [Woo S et al: Pheochromocytoma as a frequent false-positive in adrenal washout CT: a systematic review and meta-analysis. Eur Radiol. 28(3):1027-36, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=29026974%5Bpmid%5D)
1. [Mosconi C et al: Can imaging predict subclinical cortisol secretion in patients with adrenal adenomas? A CT predictive score. AJR Am J Roentgenol. 209(1):122-9, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=28402131%5Bpmid%5D)
1. [Schieda N et al: Update on CT and MRI of adrenal nodules. AJR Am J Roentgenol. 1-12, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=28225653%5Bpmid%5D)
1. [Wagner-Bartak NA et al: Cushing syndrome: diagnostic workup and imaging features, with clinical and pathologic correlation. AJR Am J Roentgenol. 209(1):19-32, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=28639924%5Bpmid%5D)
1. [Diolombi ML et al: Diagnostic dilemmas in enlarged and diffusely hemorrhagic adrenal glands. Hum Pathol. 53:63-72, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=27001431%5Bpmid%5D)
1. [Warda MH et al: Chemical-shift MRI versus washout CT for characterizing adrenal incidentalomas. Clin Imaging. 40(4):780-7, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=27317224%5Bpmid%5D)
1. [Mileto A et al: Dual-energy multidetector CT for the characterization of incidental adrenal nodules: diagnostic performance of contrast-enhanced material density analysis. Radiology. 274(2):445-54, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25207467%5Bpmid%5D)
1. [Koo HJ et al: The value of 15-minute delayed contrast-enhanced CT to differentiate hyperattenuating adrenal masses compared with chemical shift MR imaging. Eur Radiol. 24(6):1410-20, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=24647823%5Bpmid%5D)
1. [Lattin GE Jr et al: From the radiologic pathology archives: adrenal tumors and tumor-like conditions in the adult: radiologic-pathologic correlation. Radiographics. 34(3):805-29, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=24819798%5Bpmid%5D)
1. [Sebro R et al: Low yield of chemical shift MRI for characterization of adrenal lesions with high attenuation density on unenhanced CT. Abdom Imaging. 40(2):318-26 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=25095753%5Bpmid%5D)
1. [Seo JM et al: Characterization of lipid-poor adrenal adenoma: chemical-shift MRI and washout CT. AJR Am J Roentgenol. 202(5):1043-50, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=24758658%5Bpmid%5D)
1. [Kim YK et al: Adenoma characterization: adrenal protocol with dual-energy CT. Radiology. 267(1):155-63, 2013](http://www.ncbi.nlm.nih.gov/pubmed/?term=23329655%5Bpmid%5D)
1. [Northcutt BG et al: MDCT of adrenal masses: can dual-phase enhancement patterns be used to differentiate adenoma and pheochromocytoma? AJR Am J Roentgenol. 201(4):834-9, 2013](http://www.ncbi.nlm.nih.gov/pubmed/?term=24059372%5Bpmid%5D)
1. [Patel J et al: Can established CT attenuation and washout criteria for adrenal adenoma accurately exclude pheochromocytoma? AJR Am J Roentgenol. 201(1):122-7, 2013](http://www.ncbi.nlm.nih.gov/pubmed/?term=23789665%5Bpmid%5D)
1. [Nakamura S et al: Characterization of adrenal lesions using chemical shift MRI: comparison between 1.5 Tesla and two echo time pair selection at 3.0 Tesla MRI. J Magn Reson Imaging. 35(1):95-102, 2012](http://www.ncbi.nlm.nih.gov/pubmed/?term=22002867%5Bpmid%5D)
1. [Blake MA et al: Adrenal imaging. AJR Am J Roentgenol. 194(6):1450-60, 2010](http://www.ncbi.nlm.nih.gov/pubmed/?term=20489083%5Bpmid%5D)
## Tables
# Washout Criteria/Enhancement Kinetics of Adrenal Adenoma
| Absolute Washout | Relative Washout | Exceptions |
| --- | --- | --- |
| [(Post HU - delayed HU) / (post HU - pre HU)] x 100 | [(Post HU - delayed HU) / post HU] x 100 | Significant minority of pheochromocytomas meet washout criteria for adenoma (25-33%) but rarely contain intracytoplasmic lipid |
| ≥ 60% highly suggestive of adenoma | ≥ 40% highly suggestive of adenoma | Noncontrast attenuation (HU) ≥ 43 in adrenal mass without hemorrhage or calcification should raise suspicion for malignancy |
## Images
### Selected Images
![Axial in-phase T1 MR in a 77-year-old man with urothelial cancer shows a relatively homogeneous, 4.2-cm right adrenal mass <img src='img/arrows/CS.png'/>.](images/app.statdx.com_image_thumbnail_cc88fcf7-b7d7-4e76-9448-bfc9eab25df6_size_168_quality_85_84ffe5b5_20251017T212538Z.jpg)
*Axial in-phase T1 MR in a 77-year-old man with urothelial cancer shows a relatively homogeneous, 4.2-cm right adrenal mass <img src='img/arrows/CS.png'/>.*
![Axial in-phase T1 MR in a 77-year-old man with urothelial cancer shows a relatively homogeneous, 4.2-cm right adrenal mass <img src='img/arrows/CS.png'/>.](images/app.statdx.com_image_thumbnail_cc88fcf7-b7d7-4e76-9448-bfc9eab25df6_size_174_quality_85_2266401c_20251017T212328Z.jpg)
*Axial in-phase T1 MR in a 77-year-old man with urothelial cancer shows a relatively homogeneous, 4.2-cm right adrenal mass <img src='img/arrows/CS.png'/>.*
![Axial out-of-phase T1 MR in the same patient shows marked signal loss in the adrenal mass <img src='img/arrows/CS.png'/>, indicating intracytoplasmic lipid and consistent with adrenal adenoma.](images/app.statdx.com_image_thumbnail_aed4ec1f-abf5-4f30-9bf5-9786977ba5d0_size_168_quality_85_76523309_20251017T212538Z.jpg)
*Axial out-of-phase T1 MR in the same patient shows marked signal loss in the adrenal mass <img src='img/arrows/CS.png'/>, indicating intracytoplasmic lipid and consistent with adrenal adenoma.*
![Axial CECT in the same patient shows heterogeneous enhancement of the right adrenal mass <img src='img/arrows/CS.png'/> (75 HU), indeterminate on single-phase CT.](images/app.statdx.com_image_thumbnail_43b6b5c5-3dfc-435a-be9d-07c4290fa1e4_size_168_quality_85_4ca65639_20251017T212538Z.jpg)
*Axial CECT in the same patient shows heterogeneous enhancement of the right adrenal mass <img src='img/arrows/CS.png'/> (75 HU), indeterminate on single-phase CT.*
![Axial PET/CT in the same patient shows mild uptake in the right adrenal mass <img src='img/arrows/WS.png'/>, less than that of the adjacent hepatic parenchyma <img src='img/arrows/WC.png'/>, further supporting the diagnosis of adenoma made on MR.](images/app.statdx.com_image_thumbnail_151e9ee7-2187-47e3-bc18-4f1a45b78f56_size_168_quality_85_cd5e6e27_20251017T212538Z.jpg)
*Axial PET/CT in the same patient shows mild uptake in the right adrenal mass <img src='img/arrows/WS.png'/>, less than that of the adjacent hepatic parenchyma <img src='img/arrows/WC.png'/>, further supporting the diagnosis of adenoma made on MR.*
![Axial in-phase T1 MR shows a mildly heterogeneous right adrenal mass <img src='img/arrows/WS.png'/> in a 62-year-old woman with hirsutism.](images/app.statdx.com_image_thumbnail_9ab845cf-c2e7-48fe-a07c-2829383f1a42_size_168_quality_85_4c07710d_20251017T212538Z.jpg)
*Axial in-phase T1 MR shows a mildly heterogeneous right adrenal mass <img src='img/arrows/WS.png'/> in a 62-year-old woman with hirsutism.*
![Axial out-of-phase T1 MR shows signal dropout at the periphery of the mass <img src='img/arrows/WS.png'/> and in the liver <img src='img/arrows/WO.png'/>, indicating intracellular lipid. The mass was resected based on indeterminate imaging features and shown to be an adenoma with central degeneration, which can be seen with adenomas and can be confused with a collision tumor.](images/app.statdx.com_image_thumbnail_e746ad21-5007-4b26-8ff2-e2e8f77a498a_size_168_quality_85_57bc56b5_20251017T212538Z.jpg)
*Axial out-of-phase T1 MR shows signal dropout at the periphery of the mass <img src='img/arrows/WS.png'/> and in the liver <img src='img/arrows/WO.png'/>, indicating intracellular lipid. The mass was resected based on indeterminate imaging features and shown to be an adenoma with central degeneration, which can be seen with adenomas and can be confused with a collision tumor.*
![Coronal T1 MR in a 34-year-old woman with hypertension and hypokalemia shows an intermediate-signal left adrenal lesion <img src='img/arrows/WS.png'/>. Note the incidental high-signal right renal cyst <img src='img/arrows/WC.png'/>.](images/app.statdx.com_image_thumbnail_17f22719-d29b-40ec-9adf-d5e8729231af_size_168_quality_85_9c978eab_20251017T212538Z.jpg)
*Coronal T1 MR in a 34-year-old woman with hypertension and hypokalemia shows an intermediate-signal left adrenal lesion <img src='img/arrows/WS.png'/>. Note the incidental high-signal right renal cyst <img src='img/arrows/WC.png'/>.*
![Coronal out-of-phase T1 MR shows marked signal suppression of the left adrenal lesion, confirming a clinically functional, imaging apparent, lipid-rich adenoma <img src='img/arrows/WS.png'/>. Signal suppression is due to intravoxel cytoplasmic lipid and water. Aldosterone-secreting adenomas are typically small (&lt; 2 cm).](images/app.statdx.com_image_thumbnail_8ff5311e-0620-4b7a-bc80-28cc133002b7_size_168_quality_85_7ff1b80b_20251017T212538Z.jpg)
*Coronal out-of-phase T1 MR shows marked signal suppression of the left adrenal lesion, confirming a clinically functional, imaging apparent, lipid-rich adenoma <img src='img/arrows/WS.png'/>. Signal suppression is due to intravoxel cytoplasmic lipid and water. Aldosterone-secreting adenomas are typically small (&lt; 2 cm).*
![Axial NECT in the same patient shows a 1.5-cm, low-attenuation (5 HU), lipid-rich left adrenal adenoma <img src='img/arrows/WS.png'/>.](images/app.statdx.com_image_thumbnail_3437dea9-5783-4bda-bd20-df29713c8454_size_168_quality_85_29690691_20251017T212538Z.jpg)
*Axial NECT in the same patient shows a 1.5-cm, low-attenuation (5 HU), lipid-rich left adrenal adenoma <img src='img/arrows/WS.png'/>.*
![Adrenal vein sampling was subsequently performed, which confirmed an aldosterone-secreting left adrenal lesion. Gross photo of the laparoscopically resected left adrenal gland shows an exophytic, well-circumscribed adrenocortical adenoma <img src='img/arrows/WO.png'/>.](images/app.statdx.com_image_thumbnail_c0878b5f-a5dc-4a28-89f2-f61e43eb8df0_size_168_quality_85_403a72bd_20251017T212538Z.jpg)
*Adrenal vein sampling was subsequently performed, which confirmed an aldosterone-secreting left adrenal lesion. Gross photo of the laparoscopically resected left adrenal gland shows an exophytic, well-circumscribed adrenocortical adenoma <img src='img/arrows/WO.png'/>.*
![Coronal T1 MR of the lumbar spine in a 67-year-old woman shows an incidental, low-signal left adrenal mass <img src='img/arrows/CS.png'/>.](images/app.statdx.com_image_thumbnail_41972d66-e1c8-455d-af7a-ddfcaecf38cc_size_168_quality_85_1d1bbf03_20251017T212538Z.jpg)
*Coronal T1 MR of the lumbar spine in a 67-year-old woman shows an incidental, low-signal left adrenal mass <img src='img/arrows/CS.png'/>.*
![Coronal NECT in the same patient shows low attenuation of the left adrenal mass <img src='img/arrows/WS.png'/> (HU -7). Noncontrast attenuation of HU &lt; 10 is consistent with adrenal adenoma.](images/app.statdx.com_image_thumbnail_008868dd-2895-4222-acad-15705cd04f8c_size_168_quality_85_286b02e6_20251017T212538Z.jpg)
*Coronal NECT in the same patient shows low attenuation of the left adrenal mass <img src='img/arrows/WS.png'/> (HU -7). Noncontrast attenuation of HU &lt; 10 is consistent with adrenal adenoma.*
![Coronal portal venous-phase CT in the same patient shows mild enhancement of the left adrenal mass <img src='img/arrows/WS.png'/> (HU 73).](images/app.statdx.com_image_thumbnail_1088d3c3-36e9-4a3a-9ecd-f522ac655d75_size_168_quality_85_a6cc2165_20251017T212538Z.jpg)
*Coronal portal venous-phase CT in the same patient shows mild enhancement of the left adrenal mass <img src='img/arrows/WS.png'/> (HU 73).*
![Coronal 15-minute delayed-phase CECT shows washout of the left adrenal mass (HU 36) with relative washout of 51%, highly suggestive of an adrenal adenoma <img src='img/arrows/WS.png'/>. Biochemical work-up, including metanephrines, performed due to the size of the mass was normal.](images/app.statdx.com_image_thumbnail_9c2b49f1-a9ff-4261-b463-375f1b4970d2_size_168_quality_85_24ea5016_20251017T212538Z.jpg)
*Coronal 15-minute delayed-phase CECT shows washout of the left adrenal mass (HU 36) with relative washout of 51%, highly suggestive of an adrenal adenoma <img src='img/arrows/WS.png'/>. Biochemical work-up, including metanephrines, performed due to the size of the mass was normal.*
![Axial in-phase T1 MR in a 68-year-old woman with colon cancer shows a mildly heterogeneous left adrenal mass <img src='img/arrows/CS.png'/>.](images/app.statdx.com_image_thumbnail_96758bf2-b2a1-46a3-91cc-b55ebc519129_size_168_quality_85_8ea8e237_20251017T212538Z.jpg)
*Axial in-phase T1 MR in a 68-year-old woman with colon cancer shows a mildly heterogeneous left adrenal mass <img src='img/arrows/CS.png'/>.*
![Axial out-of-phase T1 MR in the same patient shows diffuse signal loss in the left adrenal mass <img src='img/arrows/CS.png'/>, consistent with intracytoplasmic lipid in an adrenal adenoma. The mass remained stable over 4 years since incidental detection on initial surveillance imaging.](images/app.statdx.com_image_thumbnail_0fd0291c-899f-4560-aa92-66586e4cf9a3_size_168_quality_85_2557180b_20251017T212538Z.jpg)
*Axial out-of-phase T1 MR in the same patient shows diffuse signal loss in the left adrenal mass <img src='img/arrows/CS.png'/>, consistent with intracytoplasmic lipid in an adrenal adenoma. The mass remained stable over 4 years since incidental detection on initial surveillance imaging.*
### Additional Images
![Axial CECT in a 68-year-old woman with colon cancer shows an indeterminate-signal, heterogeneous left adrenal mass <img src='img/arrows/CS.png'/> (HU 65) with relatively low central attenuation.](images/app.statdx.com_image_thumbnail_4c6def56-8f5e-49cd-883d-26e3a5e732e4_size_168_quality_85_b7bec102_20251017T212538Z.jpg)
*Axial CECT in a 68-year-old woman with colon cancer shows an indeterminate-signal, heterogeneous left adrenal mass <img src='img/arrows/CS.png'/> (HU 65) with relatively low central attenuation.*
![Axial T2 FS MR in the same patient shows low signal at the periphery of the mass <img src='img/arrows/CS.png'/> and increased signal centrally. Central degeneration, hemorrhage, and fibrosis are known features of some adrenal adenomas. The mass demonstrated diffuse signal loss on out-of-phase MR, consistent with intracellular lipid and adenoma.](images/app.statdx.com_image_thumbnail_90961f06-12bd-4b95-8d7f-bf97060a4b8e_size_168_quality_85_45fbd1c0_20251017T212538Z.jpg)
*Axial T2 FS MR in the same patient shows low signal at the periphery of the mass <img src='img/arrows/CS.png'/> and increased signal centrally. Central degeneration, hemorrhage, and fibrosis are known features of some adrenal adenomas. The mass demonstrated diffuse signal loss on out-of-phase MR, consistent with intracellular lipid and adenoma.*
![Axial NECT in a 34-year-old woman with elevated 24-hour urine cortisol and Cushing syndrome shows a low-attenuation (HU -6) right adrenal mass <img src='img/arrows/WO.png'/>, confirmed to be an adrenal adenoma at surgical pathology.](images/app.statdx.com_image_thumbnail_f06dd193-ade6-4a9b-bb8d-020b2467dc7e_size_168_quality_85_7f6a0cf4_20251017T212538Z.jpg)
*Axial NECT in a 34-year-old woman with elevated 24-hour urine cortisol and Cushing syndrome shows a low-attenuation (HU -6) right adrenal mass <img src='img/arrows/WO.png'/>, confirmed to be an adrenal adenoma at surgical pathology.*
![Axial in-phase T1 MR shows small, bilateral adrenal masses <img src='img/arrows/WS.png'/>.](images/app.statdx.com_image_thumbnail_5d809428-2a84-43f9-8372-8e07ae9255b2_size_168_quality_85_ddeb8d16_20251017T212538Z.jpg)
*Axial in-phase T1 MR shows small, bilateral adrenal masses <img src='img/arrows/WS.png'/>.*
![Axial opposed-phase T1 MR shows a loss of signal in both adenomas, confirming the presence of lipid.](images/app.statdx.com_image_thumbnail_3fa36d61-b2a6-4f01-b925-6748b3f97a09_size_168_quality_85_30396d2b_20251017T212538Z.jpg)
*Axial opposed-phase T1 MR shows a loss of signal in both adenomas, confirming the presence of lipid.*
![Axial NECT shows a homogeneous, low-density right adrenal adenoma <img src='img/arrows/CS.png'/> and a normal left adrenal gland.](images/app.statdx.com_image_thumbnail_0a30b0d0-1b84-40cc-a393-ba24ceb03b0e_size_168_quality_85_8f4b1b7b_20251017T212538Z.jpg)
*Axial NECT shows a homogeneous, low-density right adrenal adenoma <img src='img/arrows/CS.png'/> and a normal left adrenal gland.*
![Axial NECT shows a homogeneous, low-density adenoma that is larger than typical, which had grown slowly over 10 years.](images/app.statdx.com_image_thumbnail_e12fc310-cc29-4bd4-9486-98cea5f3bce7_size_168_quality_85_b4e06cf5_20251017T212538Z.jpg)
*Axial NECT shows a homogeneous, low-density adenoma that is larger than typical, which had grown slowly over 10 years.*
docs_md/articles/adrenal-cyst_c5d717a3-3d6e-4e86-9efe-1ad0ec14740f.md
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---
title: "Adrenal Cyst"
docid: "c5d717a3-3d6e-4e86-9efe-1ad0ec14740f"
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pageKeywords: "Genitourinary, Diagnosis, Adrenal, Benign Neoplasms, Adrenal Cyst"
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---
# KEY FACTS
- ## Imaging
- "Adrenal cyst" is descriptive term, not pathological diagnosis
- True adrenal cysts
- Majority are endothelial cysts (lymphangiomas)
- Epithelial cysts exceedingly rare
- Simple, or minimally complex, adrenal cyst, thin rim calcification, no enhancement
- Pseudocysts
- Prior hemorrhage inferred
- Nonenhancing but complex contents and wall calcification
- Relevant history (extraadrenal malignancy, rapid growth), biochemical evaluation (cortisol, metanephrines): Consider underlying adrenal neoplasm
- Enhancing soft tissue components may suggest adrenal mass hemorrhage and pseudocyst formation
- Parasitic (echinococcal) cyst
- Rare outside endemic areas
- Typically in setting of generalized echinococcus
- ## Top Differential Diagnoses
- Adrenal adenoma
- CECT: Enhancing mass without visible wall or peripheral calcifications
- Gastric diverticulum
- Air-, fluid-, or contrast-filled mass with no enhancement of contents
- Adrenal myelolipoma
- Macroscopic fat
- Necrotic adrenal tumor
- Complex wall with heterogeneous contents
- Retroperitoneal bronchogenic cyst
- ## Clinical Issues
- No treatment required usually
- Imaging surveillance performed, although intensity and length of follow-up not defined
- Biochemical evaluation (cortisol, metanephrines) routinely performed to exclude underlying adrenal neoplasm
- Surgical resection for complex cyst with enhancing components, or symptomatic cyst
- ## Diagnostic Checklist
- Complicated cyst has high attenuation, thick enhancing wall, &/or septations
# TERMINOLOGY
- ## Definitions
- "Adrenal cyst" is descriptive term, not pathological diagnosis
- Can mean true cyst, pseudocyst, or cystic mass
# IMAGING
- ## General Features
- ### Best diagnostic clue
- Well-defined, nonenhancing, water-density adrenal mass ± calcifications
- ### Location
- Suprarenal
- Unilateral > bilateral (8-10% of cases)
- ### Size
- < 5 cm (50%), up to 20 cm
- ## CT Findings
- ### NECT
- Unilocular or multilocular mass
- Well-defined, round to oval, homogeneous mass usually with water (0 HU) or near-water density
- Higher- or mixed-attenuation mass (hemorrhage, intracystic debris, crystals)
- Wall usually very thin
- ↑ wall thickness, up to 3 mm for complex cysts
- Calcifications
- Rim-like or nodular (51-69%)
- Centrally in intracystic septation (19%)
- Punctate within intracystic hemorrhage (5%)
- ### CECT
- No central enhancement ± wall enhancement
- Coronal reformats helpful to determine organ of origin if large cyst
- ## MR Findings
- ### T1WI
- Homogeneous, hypointense mass
- Hyperintense mass (hemorrhage)
- ### T2WI
- Hyperintense mass
- ## Ultrasonographic Findings
- Simple or septated suprarenal cyst
- Shadowing from calcification
- Real-time examination helpful to differentiate adrenal cyst from adjacent (renal, pancreatic) cyst
- ## Imaging Recommendations
- ### Best imaging tool
- CECT or MR; US for confirmation
# DIFFERENTIAL DIAGNOSIS
- [Adrenal Adenoma](/document/adrenal-adenoma/e2916d86-5f9f-4dd3-9576-1a7b89d8dda0)
- NECT: Lipid-rich adenoma (< 10 HU) mimics adrenal cyst
- Peripheral or septal calcification favors adrenal cyst
- CECT: **Enhancing mass** without visible wall or peripheral calcifications
- Assess washout kinetics to diagnose lipid-poor adenoma
- MR: Signal suppression at out-of-phase, chemical-shift imaging
- US: Solid adrenal lesion
- [Gastric Diverticulum](/document/gastric-diverticulum/eeb101f0-8bdf-4771-b44a-fe6e73b3a463)
- May simulate left adrenal cyst
- Air-, fluid-, or contrast-filled suprarenal mass
- No enhancement
- Normal adjacent adrenal gland
- [Adrenal Myelolipoma](/document/adrenal-myelolipoma/5813a554-06a4-4696-af71-7ce50693039d)
- Fat (not fluid) attenuation mass
- ## Necrotic Adrenal Tumor
- Primary (pheochromocytoma or carcinoma) or metastatic
- Clinical history, biochemical evaluation, lesion complexity suggest correct diagnosis
- Enhancing soft tissue components
- ## Retroperitoneal Bronchogenic Cyst
- Rare, benign, suprarenal fluid or soft tissue attenuation lesion
- Adjacent to but separate from adrenal gland
- ## Renal Cyst
- Coronal MR/CT or US useful to determine organ of origin of large, retroperitoneal cystic lesions
# PATHOLOGY
- ## General Features
- ### Etiology
- Congenital (endothelial, epithelial) cysts
- Acquired (post hemorrhagic, inflammatory) pseudocysts
- Cystic, hemorrhagic degeneration of underlying adrenal neoplasm
- ## Staging, Grading, & Classification
- Accepted classification scheme
- Pseudocyst
- Most common type of cystic adrenal lesion in surgical series
- No epithelial or endothelial lining: Fibrous cyst wall
- Potentially as complication of prior trauma or hemorrhage though history of such often not elicited
- May be associated with underlying adrenal neoplasm (pheochromocytoma, adrenal carcinoma, myelolipoma)
- Attenuation and complexity at imaging varies depending upon hemorrhagic component
- Wall and septal calcification common
- Endothelial cyst
- Subtypes: Lymphangiomatous and hemangiomatous
- True cyst: Endothelial lining
- Originate from preexisting vascular malformation or obstructed, ectatic lymphatic channels
- Thin rim calcification typical
- Epithelial cyst
- Extremely rare: No acinar structures within normal adrenal gland
- Mesothelial origin suggested (mesothelial cells potentially incorporated within adrenal gland during embryogenesis)
- Parasitic (hydatid) cyst
# CLINICAL ISSUES
- ## Presentation
- ### Most common signs/symptoms
- Typically asymptomatic
- Larger cysts may be symptomatic
- Abdominal pain
- Hemorrhage
- Clinical history (malignancy, hypertension) elicited
- May indicate cystic degeneration of underlying adrenal neoplasm (e.g., metastasis, pheochromocytoma)
- Diagnosis
- Usually incidental finding at imaging
- Endocrine-biochemical evaluation performed to exclude underlying functional adrenal tumor
- ## Demographics
- ### Age
- Any, though patients 20-50 years of age most common
- ### Sex
- M:F = 1:3
- ### Epidemiology
- Uncommon entity: Autopsy incidence 0.064-0.18%
- Accounts for 1% of incidental adrenal lesions in large imaging series
- ## Natural History & Prognosis
- Complications
- Hypertension, infection, rupture, hemorrhage
- Excellent prognosis for vast majority of incidental, benign adrenal cysts
- Prognosis for pseudocysts secondary to adrenal neoplasm depends upon tumor histology
- ## Treatment
- No treatment required usually
- Imaging follow-up typically performed
- Intensity and length of surveillance not defined
- Cysts may enlarge over time
- Endocrine evaluation (cortisol, metanephrine, etc.) performed
- Surgical resection if symptomatic, underlying adrenal neoplasm
- Laparoscopic resection preferred
# DIAGNOSTIC CHECKLIST
- ## Consider
- Complicated cyst may suggest underlying adrenal neoplasm
- Clinical history, biochemical evaluation, and prior imaging helpful
- ## Image Interpretation Pearls
- Simple adrenal cyst: Scant septation, no enhancement, thin rim calcification
- Likely benign endothelial cyst or pseudocyst
- Coronal imaging helpful to determine organ of origin (and exclude exophytic renal or pancreatic cyst)
- Complicated cyst: High attenuation, thick enhancing wall, &/or septations
- Complexity may suggest underlying adrenal neoplasm and secondary pseudocyst
2a5b237c-1238-48d3-9b6c-f76846cba1cb
## References
# Selected References
1. [Wang F et al: CT and MRI of adrenal gland pathologies. Quant Imaging Med Surg. 8(8):853-75, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=30306064%5Bpmid%5D)
1. [Lattin GE Jr et al: From the radiologic pathology archives: adrenal tumors and tumor-like conditions in the adult: radiologic-pathologic correlation. Radiographics. 34(3):805-29, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=24819798%5Bpmid%5D)
1. [Kyoda Y et al: Adrenal hemorrhagic pseudocyst as the differential diagnosis of pheochromocytoma--a review of the clinical features in cases with radiographically diagnosed pheochromocytoma. J Endocrinol Invest. 36(9):707-11, 2013](http://www.ncbi.nlm.nih.gov/pubmed/?term=23563219%5Bpmid%5D)
1. [Ricci Z et al: Adrenal cysts: natural history by long-term imaging follow-up. AJR Am J Roentgenol. 201(5):1009-16, 2013](http://www.ncbi.nlm.nih.gov/pubmed/?term=24147471%5Bpmid%5D)
1. [Saadai P et al: The pathological features of surgically managed adrenal cysts: a 15-year retrospective review. Am Surg. 79(11):1159-62, 2013](http://www.ncbi.nlm.nih.gov/pubmed/?term=24165250%5Bpmid%5D)
1. [Sebastiano C et al: Cystic lesions of the adrenal gland: our experience over the last 20 years. Hum Pathol. 44(9):1797-803, 2013](http://www.ncbi.nlm.nih.gov/pubmed/?term=23618356%5Bpmid%5D)
1. [El-Hefnawy AS et al: Surgical management of adrenal cysts: single-institution experience. BJU Int. 104(6):847-50, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=19389014%5Bpmid%5D)
1. [Chien HP et al: Adrenal cystic lesions: a clinicopathological analysis of 25 cases with proposed histogenesis and review of the literature. Endocr Pathol. 19(4):274-81, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18972224%5Bpmid%5D)
1. [Song JH et al: The incidental adrenal mass on CT: prevalence of adrenal disease in 1,049 consecutive adrenal masses in patients with no known malignancy. AJR Am J Roentgenol. 190(5):1163-8, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18430826%5Bpmid%5D)
1. Elsayes KM et al: Adrenal masses: MR imaging features with pathologic correlation. Radiographics. 24 Suppl 1:S73-86, 2004
1. [Guo YK et al: Uncommon adrenal masses: CT and MRI features with histopathologic correlation. Eur J Radiol. 62(3):359-70, 2007](http://www.ncbi.nlm.nih.gov/pubmed/?term=17532488%5Bpmid%5D)
1. [Sanal HT et al: Imaging features of benign adrenal cysts. Eur J Radiol. 60(3):465-9, 2006](http://www.ncbi.nlm.nih.gov/pubmed/?term=16962278%5Bpmid%5D)
1. [Akçay MN et al: Hydatid cysts of the adrenal gland: review of nine patients. World J Surg. 28(1):97-9, 2004](http://www.ncbi.nlm.nih.gov/pubmed/?term=14639487%5Bpmid%5D)
1. [Elsayes KM et al: Adrenal masses: MR findings with pathologic correlation. RadioGraphics 24: S73-86; 2004](http://www.ncbi.nlm.nih.gov/pubmed/?term=15486251%5Bpmid%5D)
1. [Kawashima A et al: Imaging of nontraumatic hemorrhage of the adrenal gland. Radiographics. 19(4):949-63, 1999](http://www.ncbi.nlm.nih.gov/pubmed/?term=10464802%5Bpmid%5D)
1. [Neri LM et al: Management of adrenal cysts. Am Surg. 65(2):151-63, 1999](http://www.ncbi.nlm.nih.gov/pubmed/?term=9926751%5Bpmid%5D)
1. [Otal P et al: Imaging features of uncommon adrenal masses with histopathologic correlation. Radiographics. 19(3):569-81, 1999](http://www.ncbi.nlm.nih.gov/pubmed/?term=10336189%5Bpmid%5D)
1. [Kawashima A et al: Spectrum of CT findings in nonmalignant disease of the adrenal gland. Radiographics. 18(2):393-412, 1998](http://www.ncbi.nlm.nih.gov/pubmed/?term=9536486%5Bpmid%5D)
1. [Tung GA et al: Adrenal cysts: imaging and percutaneous aspiration. Radiology. 173(1):107-10, 1989](http://www.ncbi.nlm.nih.gov/pubmed/?term=2675177%5Bpmid%5D)
## Images
### Selected Images
![Axial CECT in a 28-year-old woman with abdominal pain shows an incidental left adrenal cystic lesion <img src='img/arrows/WS.png'/>. Note thin cyst septation <img src='img/arrows/WC.png'/>, a finding characteristic of endothelial adrenal cyst.](images/app.statdx.com_image_thumbnail_066f9db3-8b3b-4db5-8b74-5ea109b74746_size_168_quality_85_ff218a4f_20251017T212542Z.jpg)
*Axial CECT in a 28-year-old woman with abdominal pain shows an incidental left adrenal cystic lesion <img src='img/arrows/WS.png'/>. Note thin cyst septation <img src='img/arrows/WC.png'/>, a finding characteristic of endothelial adrenal cyst.*
![Axial CECT in a 28-year-old woman with abdominal pain shows an incidental left adrenal cystic lesion <img src='img/arrows/WS.png'/>. Note thin cyst septation <img src='img/arrows/WC.png'/>, a finding characteristic of endothelial adrenal cyst.](images/app.statdx.com_image_thumbnail_066f9db3-8b3b-4db5-8b74-5ea109b74746_size_174_quality_85_981271a8_20251017T212328Z.jpg)
*Axial CECT in a 28-year-old woman with abdominal pain shows an incidental left adrenal cystic lesion <img src='img/arrows/WS.png'/>. Note thin cyst septation <img src='img/arrows/WC.png'/>, a finding characteristic of endothelial adrenal cyst.*
![Longitudinal US in the same patient confirms an anechoic left suprarenal-adrenal cyst <img src='img/arrows/BS.png'/>. The simple appearance of the cyst and the lack of additional relevant clinical history (malignancy, HTN, etc.) prompted surveillance rather than resection for this incidental, benign endothelial cyst.](images/app.statdx.com_image_263c08e3-8cc2-46ee-bbab-eedca9354007_c4146213_20251017T212611Z.jpg)
*Longitudinal US in the same patient confirms an anechoic left suprarenal-adrenal cyst <img src='img/arrows/BS.png'/>. The simple appearance of the cyst and the lack of additional relevant clinical history (malignancy, HTN, etc.) prompted surveillance rather than resection for this incidental, benign endothelial cyst.*
![Longitudinal US in the same patient confirms an anechoic left suprarenal-adrenal cyst <img src='img/arrows/BS.png'/>. The simple appearance of the cyst and the lack of additional relevant clinical history (malignancy, HTN, etc.) prompted surveillance rather than resection for this incidental, benign endothelial cyst.](images/app.statdx.com_image_thumbnail_263c08e3-8cc2-46ee-bbab-eedca9354007_size_168_quality_85_27446616_20251017T212542Z.jpg)
*Longitudinal US in the same patient confirms an anechoic left suprarenal-adrenal cyst <img src='img/arrows/BS.png'/>. The simple appearance of the cyst and the lack of additional relevant clinical history (malignancy, HTN, etc.) prompted surveillance rather than resection for this incidental, benign endothelial cyst.*
![Axial NECT in a 71-year-old woman shows a 4-cm, complex cystic right adrenal mass <img src='img/arrows/WC.png'/> containing coarse calcifications. Adrenalectomy (performed given lesion complexity and size) confirmed hemorrhagic pseudocyst.](images/app.statdx.com_image_9aa3e817-e7c5-4275-bf17-4469de11dd6b_39392a9f_20251017T212613Z.jpg)
*Axial NECT in a 71-year-old woman shows a 4-cm, complex cystic right adrenal mass <img src='img/arrows/WC.png'/> containing coarse calcifications. Adrenalectomy (performed given lesion complexity and size) confirmed hemorrhagic pseudocyst.*
![Axial NECT in a 71-year-old woman shows a 4-cm, complex cystic right adrenal mass <img src='img/arrows/WC.png'/> containing coarse calcifications. Adrenalectomy (performed given lesion complexity and size) confirmed hemorrhagic pseudocyst.](images/app.statdx.com_image_thumbnail_9aa3e817-e7c5-4275-bf17-4469de11dd6b_size_168_quality_85_d008bbea_20251017T212542Z.jpg)
*Axial NECT in a 71-year-old woman shows a 4-cm, complex cystic right adrenal mass <img src='img/arrows/WC.png'/> containing coarse calcifications. Adrenalectomy (performed given lesion complexity and size) confirmed hemorrhagic pseudocyst.*
![Axial T2 FS MR in a 54-year-old woman with left flank pain shows a 7-cm, complex cystic right adrenal mass with a low-signal hemosiderin ring <img src='img/arrows/WS.png'/>. A pseudocyst was resected. Signal intensity of pseudocysts varies depending on the age of hemorrhage.](images/app.statdx.com_image_ca112000-a7cc-472f-a359-99cb9885ef64_47c14519_20251017T212616Z.jpg)
*Axial T2 FS MR in a 54-year-old woman with left flank pain shows a 7-cm, complex cystic right adrenal mass with a low-signal hemosiderin ring <img src='img/arrows/WS.png'/>. A pseudocyst was resected. Signal intensity of pseudocysts varies depending on the age of hemorrhage.*
![Axial T2 FS MR in a 54-year-old woman with left flank pain shows a 7-cm, complex cystic right adrenal mass with a low-signal hemosiderin ring <img src='img/arrows/WS.png'/>. A pseudocyst was resected. Signal intensity of pseudocysts varies depending on the age of hemorrhage.](images/app.statdx.com_image_thumbnail_ca112000-a7cc-472f-a359-99cb9885ef64_size_168_quality_85_bd248aeb_20251017T212542Z.jpg)
*Axial T2 FS MR in a 54-year-old woman with left flank pain shows a 7-cm, complex cystic right adrenal mass with a low-signal hemosiderin ring <img src='img/arrows/WS.png'/>. A pseudocyst was resected. Signal intensity of pseudocysts varies depending on the age of hemorrhage.*
![Sagittal US in a 35-year-old woman shows a &gt; 10-cm right suprarenal cyst <img src='img/arrows/WS.png'/>. Real-time examination and a follow-up MR (not shown) confirmed simple extrarenal-adrenal cyst that was subsequently resected. An epithelial cyst (a rare subtype of adrenal cysts) was shown at histology.](images/app.statdx.com_image_thumbnail_9944fce3-53d2-4f85-bfe0-17b055896441_size_168_quality_85_ab5b7c5a_20251017T212542Z.jpg)
*Sagittal US in a 35-year-old woman shows a &gt; 10-cm right suprarenal cyst <img src='img/arrows/WS.png'/>. Real-time examination and a follow-up MR (not shown) confirmed simple extrarenal-adrenal cyst that was subsequently resected. An epithelial cyst (a rare subtype of adrenal cysts) was shown at histology.*
![Axial CECT in a 51-year-old woman shows an incidental, peripherally calcified left adrenal cyst <img src='img/arrows/WS.png'/>. Coarse calcification suggests a pseudocyst. Pseudocysts may be due to prior trauma/hemorrhage, but this history is often absent.](images/app.statdx.com_image_thumbnail_5b76760f-1003-479a-9a36-a01d823f7dde_size_168_quality_85_123979bc_20251017T212542Z.jpg)
*Axial CECT in a 51-year-old woman shows an incidental, peripherally calcified left adrenal cyst <img src='img/arrows/WS.png'/>. Coarse calcification suggests a pseudocyst. Pseudocysts may be due to prior trauma/hemorrhage, but this history is often absent.*
![Axial CECT in a hypertensive 36-year-old woman shows a peripherally enhancing, septated, 7-cm left adrenal pheochromocytoma <img src='img/arrows/WS.png'/>. Enhancing soft tissue should prompt testing for an underlying adrenal neoplasm.](images/app.statdx.com_image_thumbnail_2a658894-5559-4767-b100-b5864693b626_size_168_quality_85_fd031a4d_20251017T212542Z.jpg)
*Axial CECT in a hypertensive 36-year-old woman shows a peripherally enhancing, septated, 7-cm left adrenal pheochromocytoma <img src='img/arrows/WS.png'/>. Enhancing soft tissue should prompt testing for an underlying adrenal neoplasm.*
![Axial CECT in a 72-year-old man with pancreatitis shows peripancreatic infiltration <img src='img/arrows/WS.png'/> and a 3-cm adrenal pseudocyst <img src='img/arrows/WC.png'/>. The pseudocyst resolved on follow-up CT. Imaging surveillance is advocated for asymptomatic, probable pseudocysts.](images/app.statdx.com_image_thumbnail_376c9985-4425-4707-b26d-013d558547c9_size_168_quality_85_4be684be_20251017T212542Z.jpg)
*Axial CECT in a 72-year-old man with pancreatitis shows peripancreatic infiltration <img src='img/arrows/WS.png'/> and a 3-cm adrenal pseudocyst <img src='img/arrows/WC.png'/>. The pseudocyst resolved on follow-up CT. Imaging surveillance is advocated for asymptomatic, probable pseudocysts.*
![Staging CECT in a 66-year-old man with metastatic lung carcinoma shows large, bilateral, necrotic adrenal metastases <img src='img/arrows/WS.png'/>. The clinical history and an enhancing rind <img src='img/arrows/WC.png'/> prevent an erroneous diagnosis of benign adrenal cysts.](images/app.statdx.com_image_thumbnail_3c48f420-dc4c-43b6-8287-73b05b992919_size_168_quality_85_0c5e33c4_20251017T212542Z.jpg)
*Staging CECT in a 66-year-old man with metastatic lung carcinoma shows large, bilateral, necrotic adrenal metastases <img src='img/arrows/WS.png'/>. The clinical history and an enhancing rind <img src='img/arrows/WC.png'/> prevent an erroneous diagnosis of benign adrenal cysts.*
![Axial CECT of a 62-year-old man shows a 2-cm, incidental left suprarenal lesion <img src='img/arrows/WC.png'/>. Location adjacent to the left crus <img src='img/arrows/WO.png'/> and separate from the left adrenal <img src='img/arrows/WS.png'/> indicates a retroperitoneal bronchogenic cyst, a mimic of adrenal cyst.](images/app.statdx.com_image_thumbnail_fc2010de-959a-4384-8188-96d011832bf5_size_168_quality_85_473f29cf_20251017T212542Z.jpg)
*Axial CECT of a 62-year-old man shows a 2-cm, incidental left suprarenal lesion <img src='img/arrows/WC.png'/>. Location adjacent to the left crus <img src='img/arrows/WO.png'/> and separate from the left adrenal <img src='img/arrows/WS.png'/> indicates a retroperitoneal bronchogenic cyst, a mimic of adrenal cyst.*
### Additional Images
![Sagittal US shows a sonolucent mass above the right kidney that proved to be an adrenal cyst.](images/app.statdx.com_image_thumbnail_c3a7d603-130d-459c-8d09-3e170be8f6d2_size_168_quality_85_e1bbb811_20251017T212542Z.jpg)
*Sagittal US shows a sonolucent mass above the right kidney that proved to be an adrenal cyst.*
![Axial T2 MR shows a high-signal left adrenal cyst <img src='img/arrows/WS.png'/>.](images/app.statdx.com_image_thumbnail_b4cc578e-2427-443c-bb1e-f17f9ce33bf9_size_168_quality_85_8a3b70fd_20251017T212542Z.jpg)
*Axial T2 MR shows a high-signal left adrenal cyst <img src='img/arrows/WS.png'/>.*
![Axial CECT shows a nonenhancing water density right adrenal cyst.](images/app.statdx.com_image_thumbnail_ba105b20-95e3-4793-80d5-9a93ab7802bf_size_168_quality_85_de0ef4d9_20251017T212542Z.jpg)
*Axial CECT shows a nonenhancing water density right adrenal cyst.*
![Sagittal reformation of CECT shows a nonenhancing right adrenal cyst <img src='img/arrows/WS.png'/>.](images/app.statdx.com_image_thumbnail_b11ccb76-67fa-48bb-bc8a-63cb1022021f_size_168_quality_85_0c9d2825_20251017T212542Z.jpg)
*Sagittal reformation of CECT shows a nonenhancing right adrenal cyst <img src='img/arrows/WS.png'/>.*
docs_md/articles/adrenal-myelolipoma_5813a554-06a4-4696-af71-7ce50693039d.md
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---
title: "Adrenal Myelolipoma"
docid: "5813a554-06a4-4696-af71-7ce50693039d"
authors:
- key: "c3463c5c-31d3-4489-bbfe-6b895abdb86d"
value: "Mitchell Tublin, MD"
- key: "3d84d682-9451-4b02-99b2-e34970a5b440"
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pageKeywords: "Genitourinary, Diagnosis, Adrenal, Benign Neoplasms, Adrenal Myelolipoma"
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---
# KEY FACTS
- ## Terminology
- Uncommon benign tumor composed of mature fat tissue and hematopoietic elements (myeloid and erythroid cells)
- ## Imaging
- Benign, nonfunctioning adrenal tumor
- Accounts for 7-15% of incidental adrenal masses, usually in older population
- Typically unilateral and very rarely bilateral
- Large tumors can mimic retroperitoneal lipomas, liposarcomas
- Asymptomatic, though larger tumors may hemorrhage
- CT
- Lesion containing fat attenuation (-30 to -90 HU)
- Usually well-defined mass with recognizable pseudocapsule (remaining adrenal)
- Punctate calcifications seen in 24% of cases
- Coronal reconstruction helpful to differentiate from exophytic renal angiomyolipoma
- MR
- Tumor with major fat component
- T1WI in phase: Typically hyperintense
- FS sequences: Loss of signal
- ## Top Differential Diagnoses
- Adrenal adenoma
- Intracellular lipid vs. macroscopic fat
- Adrenal metastases and lymphoma
- Retroperitoneal liposarcoma
- Involving perirenal space, may simulate adrenal (or renal) fatty tumor
- Pheochromocytoma
- Highly vascular, prone to hemorrhage and necrosis
- Adrenal carcinoma
- Renal angiomyelolipoma
- Coronal CT reconstruction or MR useful to determine organ of origin
# TERMINOLOGY
- ## Definitions
- Uncommon benign tumor composed of mature adipose tissue and hematopoietic elements
# IMAGING
- ## General Features
- ### Best diagnostic clue
- Suprarenal mass containing fat
- ### Location
- Suprarenal
- Rare extraadrenal myelolipomas (presacral, retroperitoneal)
- ### Size
- Usually 2-10 cm, rarely 10-20 cm
- Key concepts
- Benign neoplasm of adrenal gland
- Autopsy prevalence rate of 0.2-0.4%
- Accounts for 7-15% of adrenal "incidentalomas"
- Usually unilateral incidental finding in older patient
- Larger tumors can bleed spontaneously
- Most are nonfunctioning (do not secrete hormones)
- Large myelolipomas can mimic retroperitoneal lipoma or liposarcoma
- ## CT Findings
- CT appearance depends on histologic composition
- Most tumors are heterogeneous adrenal masses composed of varying percentages of fat
- Low-attenuation suprarenal lesion containing fat density (-30 to -90 HU)
- Average NECT attenuation value of tumor: -74 HU in one series
- Interspersed soft tissue attenuation components: Myeloid elements, hemorrhage
- Presence of macroscopic fat within tumor is diagnostic
- Punctate calcifications seen in 24% of cases
- Usually well-defined mass with recognizable pseudocapsule (remnant adrenal)
- Coronal reconstructions may help determine organ of origin: Adrenal myelolipoma vs. exophytic renal angiomyelolipoma
- ## MR Findings
- MR appearance depends on histologic composition
- Tumor with major fat component
- T1WI in phase: Typically hyperintense
- T1WI out phase: Persistent hyperintensity of macroscopic fat
- T1WI FS: Confirmatory suppression of signal
- Bone marrow elements (myeloid and erythroid cells)
- Low signal on T1WI, moderate signal on T2WI
- Hemorrhage: Varying T1, T2 signal depending on age of blood
- ## Ultrasonographic Findings
- ### Grayscale ultrasound
- Well-defined, echogenic mass (↑ fat tissue)
- Often overlooked: Lack of mass effect and isoechogenicity relative to retroperitoneal fat
- Heterogeneous mass (↑ myeloid cells)
- ## Angiographic Findings
- Conventional
- Differentiate myelolipoma from retroperitoneal liposarcoma by determining origin of blood supply and vascularity of tumors
- ## Nuclear Medicine Findings
- Typically not metabolically active, though uptake reported at FDG PET
- ## Imaging Recommendations
- Helical NECT or MR with FS sequence
# DIFFERENTIAL DIAGNOSIS
- [Adrenal Adenoma](/document/adrenal-adenoma/e2916d86-5f9f-4dd3-9576-1a7b89d8dda0)
- Lipid-rich adenoma: ↓ attenuation (< 10 HU) at NECT
- Can contain small amounts of macroscopic fat due to lipomatous metaplasia
- CECT: Washout 15 minutes post injection: > 50%
- Relative washout: > 40%
- Absolute washout: > 60%
- [Metastases and Lymphoma, Adrenal](/document/adrenal-lymphoma/44639c90-bd04-4e2a-a470-2c28a0e2ff78)
- Bilateral lesions: Clinical history paramount
- Metastases: Soft tissue attenuation (signal)
- Lymphoma: May maintain adreniform shape, adjacent retroperitoneal adenopathy
- [Liposarcoma, Retroperitoneal](/document/retroperitoneal-sarcoma/c1466b30-b730-41c4-a065-2c2de018a5f7)
- Retroperitoneal primary sarcoma involving perirenal space may simulate adrenal (or renal) fatty tumor
- [Pheochromocytoma](/document/pheochromocytoma/7d3c4062-643c-4030-8783-f85184ad8132)
- Highly vascular, prone to hemorrhage and necrosis
- Hyperintense on T2WI, bilateral in multiple endocrine neoplasia syndromes (MEN) syndromes
- Clinical history (labile hypertension) and urinary catecholamines
- [Adrenal Carcinoma](/document/adrenal-cortical-carcinoma/bdc7a08b-a64f-4bd2-9dfc-24331728e85e)
- Rare, unilateral, invasive, enhancing mass
- Venous invasion, distant metastases
- May contain fat: Engulfed retroperitoneal fat vs. lipomatous metaplasia
- ## Renal Angiomyelolipoma
- Exophytic upper pole angiomyolipoma may mimic
- Coronal reconstruction/MR helpful to determine organ of origin
# PATHOLOGY
- ## General Features
- ### Etiology
- Unknown
- Best hypothesis: Reticuloendothelial cell metaplasia of capillaries in adrenal (stress/infection/necrosis)
- Secondary hypothesis: Myelolipoma represents site of extramedullary hematopoiesis
- ### Associated abnormalities
- Adrenal collision tumors (coexistent myelolipoma and adenoma typical)
- Large, bilateral myelolipomas reported with longstanding, poorly treated congenital adrenal hyperplasia
- ## Gross Pathologic & Surgical Features
- Cut section: Fat, soft tissue components
- ## Microscopic Features
- Mature fat cells and megakaryocytes; no malignant cells
- Calcification
- Hemorrhage within larger lesions
# CLINICAL ISSUES
- ## Presentation
- ### Most common signs/symptoms
- Asymptomatic
- Usually incidental finding on CT, MR
- Typically biochemically nonfunctioning
- Symptomatic
- Acute abdomen: Flank pain due to rupture and hemorrhage
- Case reports of hormonally active tumors: Cushing, Conn syndromes, virilization
- Diagnosis: Pathognomonic MR/CT features
- Biopsy reserved for larger, atypical lesions, though prone to sampling error
- ## Demographics
- ### Age
- Usually older patients (50-70 years old)
- ### Epidemiology
- Autopsy incidence: 0.2-0.4%
- ## Natural History & Prognosis
- Complication: Rupture with hemorrhage (rare)
- Prognosis: Excellent
- ## Treatment
- When diagnosis is certain, surgery not needed for lesions < 5-7 cm
- Surgery reserved for larger, symptomatic, or atypical lesions
- Surgical series have confirmed utility of laparoscopic resection
# DIAGNOSTIC CHECKLIST
- ## Consider
- Differentiate from other tumors (lipid-rich adenoma)
- Key is presence of imaging-apparent adipose tissue; avoid further work-up for incidental mass
- ## Image Interpretation Pearls
- Well-defined, heterogeneous, fat-attenuation tumor on CT
- T1 hyperintense, signal loss with fat suppression
81ce3ad3-c446-4b08-8b87-df9511f95360
## References
# Selected References
1. [Alshahrani MA et al: Bilateral adrenal abnormalities: imaging review of different entities. Abdom Radiol (NY). 44(1):154-79, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=29938331%5Bpmid%5D)
1. [Schieda N et al: Renal and adrenal masses containing fat at MRI: proposed nomenclature by the society of abdominal radiology disease-focused panel on renal cell carcinoma. J Magn Reson Imaging. 49(4):917-26, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=30693607%5Bpmid%5D)
1. [Decmann Á et al: Adrenal myelolipoma: a comprehensive review. Endocrine. 59(1):7-15, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=29164520%5Bpmid%5D)
1. [Campbell MJ et al: The radiographically diagnosed adrenal myelolipoma: what do we really know? Endocrine. 58(2):289-94, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=28866749%5Bpmid%5D)
1. [Farrugia FA et al: Radiology of the adrenal incidentalomas. Review of the literature. Endocr Regul. 51(1):35-51, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=28222025%5Bpmid%5D)
1. [Littrell LA et al: Extra-adrenal myelolipoma and extramedullary hematopoiesis: imaging features of two similar benign fat-containing presacral masses that may mimic liposarcoma. Eur J Radiol. 93:185-94, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=28668414%5Bpmid%5D)
1. [Mendiratta-Lala M et al: Adrenal imaging. Endocrinol Metab Clin North Am. 46(3):741-59, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=28760236%5Bpmid%5D)
1. [Tanner J et al: Case 243: extramedullary hematopoiesis in an adrenal myelolipoma. Radiology. 284(1):292-6, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=28628416%5Bpmid%5D)
1. [Shaaban AM et al: Fat-containing retroperitoneal lesions: imaging characteristics, localization, and differential diagnosis. Radiographics. 36(3):710-34, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=27163589%5Bpmid%5D)
1. [Yin L et al: A 10-year single-center experience with surgical management of adrenal myelolipoma. J Endourol. 28(2):252-5, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=24044410%5Bpmid%5D)
1. [Schieda N et al: Pitfalls of adrenal imaging with chemical shift MRI. Clin Radiol. 69(11):1186-97, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=25062926%5Bpmid%5D)
1. [Katabathina VS et al: Adrenal collision tumors and their mimics: multimodality imaging findings. Cancer Imaging. 13(4):602-10, 2013](http://www.ncbi.nlm.nih.gov/pubmed/?term=24434021%5Bpmid%5D)
1. [Castinetti F et al: Adrenal myelolipoma: an unusual cause of bilateral highly 18F-FDG-avid adrenal masses. J Clin Endocrinol Metab. 97(8):2577-8, 2012](http://www.ncbi.nlm.nih.gov/pubmed/?term=22622025%5Bpmid%5D)
1. [Giacinto J et al: Nonoperative management of adrenal myelolipoma hemorrhage resulting from trauma. Am Surg. 78(11):E463-4, 2012](http://www.ncbi.nlm.nih.gov/pubmed/?term=23089419%5Bpmid%5D)
1. [Su HC et al: Adrenal myelolipoma associated with hyperandrogenemia. Int J Urol. 19(11):1026-8, 2012](http://www.ncbi.nlm.nih.gov/pubmed/?term=22788680%5Bpmid%5D)
1. [German-Mena E et al: Adrenal myelolipomas in patients with congenital adrenal hyperplasia: review of the literature and a case report. Endocr Pract. 7(3):441-7, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=21324823%5Bpmid%5D)
1. [Daneshmand S et al: Adrenal myelolipoma: diagnosis and management. Urol J. 3(2):71-4, 2006](http://www.ncbi.nlm.nih.gov/pubmed/?term=17590837%5Bpmid%5D)
1. [Kenney PJ et al: Myelolipoma: CT and pathologic features. Radiology. 208(1):87-95, 1998](http://www.ncbi.nlm.nih.gov/pubmed/?term=9646797%5Bpmid%5D)
1. [Rao P et al: Imaging and pathologic features of myelolipoma. Radiographics. 17(6):1373-85, 1997](http://www.ncbi.nlm.nih.gov/pubmed/?term=9397452%5Bpmid%5D)
1. [Cyran KM et al: Adrenal myelolipoma. AJR Am J Roentgenol. 166(2):395-400, 1996](http://www.ncbi.nlm.nih.gov/pubmed/?term=8553954%5Bpmid%5D)
## Images
### Selected Images
![Sagittal US in a 52-year-old woman with right upper quadrant pain shows a subtle, echogenic right suprarenal mass <img src='img/arrows/WS.png'/>. Even large myelolipomas may not be perceived on US given their isoechogenicity relative to retroperitoneal fat.](images/app.statdx.com_image_thumbnail_442ee7e1-12f4-4447-8770-c20bc2232943_size_168_quality_85_799db510_20251017T212346Z.jpg)
*Sagittal US in a 52-year-old woman with right upper quadrant pain shows a subtle, echogenic right suprarenal mass <img src='img/arrows/WS.png'/>. Even large myelolipomas may not be perceived on US given their isoechogenicity relative to retroperitoneal fat.*
![Sagittal US in a 52-year-old woman with right upper quadrant pain shows a subtle, echogenic right suprarenal mass <img src='img/arrows/WS.png'/>. Even large myelolipomas may not be perceived on US given their isoechogenicity relative to retroperitoneal fat.](images/app.statdx.com_image_thumbnail_442ee7e1-12f4-4447-8770-c20bc2232943_size_174_quality_85_1ecbaf2e_20251017T212328Z.jpg)
*Sagittal US in a 52-year-old woman with right upper quadrant pain shows a subtle, echogenic right suprarenal mass <img src='img/arrows/WS.png'/>. Even large myelolipomas may not be perceived on US given their isoechogenicity relative to retroperitoneal fat.*
![Axial CECT in the same patient shows a fat-attenuation right adrenal lesion <img src='img/arrows/WC.png'/>. Macroscopic fat is the hallmark of myelolipomas, though the fat may be interspersed with soft tissue myeloid elements and hemorrhage.](images/app.statdx.com_image_thumbnail_7c5086cf-283b-4e1e-8a7c-72ba7a53567c_size_168_quality_85_6f2bf25a_20251017T212346Z.jpg)
*Axial CECT in the same patient shows a fat-attenuation right adrenal lesion <img src='img/arrows/WC.png'/>. Macroscopic fat is the hallmark of myelolipomas, though the fat may be interspersed with soft tissue myeloid elements and hemorrhage.*
![Axial T1 MR for follow-up in the same patient shows a relatively intense right adrenal lesion <img src='img/arrows/WS.png'/>. No significant signal loss was shown on out-phase imaging (not shown).](images/app.statdx.com_image_thumbnail_932b5b83-270c-4e51-9a39-7be67dfaa259_size_168_quality_85_3c8f5da0_20251017T212346Z.jpg)
*Axial T1 MR for follow-up in the same patient shows a relatively intense right adrenal lesion <img src='img/arrows/WS.png'/>. No significant signal loss was shown on out-phase imaging (not shown).*
![Axial T1 FS MR in the same patient confirms that the right adrenal lesion <img src='img/arrows/CC.png'/> is composed of fat, a finding pathognomonic of a myelolipoma. Signal suppression with fat saturation and persistent signal on out-of-phase imaging indicate macroscopic fat, rather than the intracellular lipid characteristic of adrenal adenomas.](images/app.statdx.com_image_thumbnail_ae78b57e-1386-42d6-a184-15212a61ccc5_size_168_quality_85_c4020638_20251017T212346Z.jpg)
*Axial T1 FS MR in the same patient confirms that the right adrenal lesion <img src='img/arrows/CC.png'/> is composed of fat, a finding pathognomonic of a myelolipoma. Signal suppression with fat saturation and persistent signal on out-of-phase imaging indicate macroscopic fat, rather than the intracellular lipid characteristic of adrenal adenomas.*
![Axial CECT in a 55-year-old man with left flank pain shows a large, fat-attenuation mass <img src='img/arrows/WS.png'/>, soft tissue attenuation rim, and perinephric infiltration.](images/app.statdx.com_image_thumbnail_cfdb3ea5-adaf-4871-a66c-ec6711bb1d6c_size_168_quality_85_9aa7088d_20251017T212346Z.jpg)
*Axial CECT in a 55-year-old man with left flank pain shows a large, fat-attenuation mass <img src='img/arrows/WS.png'/>, soft tissue attenuation rim, and perinephric infiltration.*
![Axial out-of-phase MR in the same patient shows a low-signal rim <img src='img/arrows/WC.png'/> (due to hemosiderin) and perinephric infiltration. Most myelolipomas are asymptomatic, but larger tumors may hemorrhage, as in this case. Adrenalectomy was performed given the large size of the tumor and evidence of repeated hemorrhage.](images/app.statdx.com_image_thumbnail_095ce718-61ae-4220-8052-0570c1f59922_size_168_quality_85_5a68c963_20251017T212346Z.jpg)
*Axial out-of-phase MR in the same patient shows a low-signal rim <img src='img/arrows/WC.png'/> (due to hemosiderin) and perinephric infiltration. Most myelolipomas are asymptomatic, but larger tumors may hemorrhage, as in this case. Adrenalectomy was performed given the large size of the tumor and evidence of repeated hemorrhage.*
![Axial CECT in a 53-year-old man shows an incidental myelolipoma <img src='img/arrows/WS.png'/> containing calcifications <img src='img/arrows/WC.png'/>.](images/app.statdx.com_image_thumbnail_4721c379-1e84-4445-9347-27a9560e5a06_size_168_quality_85_29bbb9d6_20251017T212346Z.jpg)
*Axial CECT in a 53-year-old man shows an incidental myelolipoma <img src='img/arrows/WS.png'/> containing calcifications <img src='img/arrows/WC.png'/>.*
![Coronal NECT in a 71-year-old man with left flank pain shows an incidental collision tumor composed of a myelolipoma <img src='img/arrows/WS.png'/> and an adenoma <img src='img/arrows/WC.png'/>.](images/app.statdx.com_image_thumbnail_d75a5410-204a-4e59-9a2d-e0581d24e1f3_size_168_quality_85_0203f833_20251017T212346Z.jpg)
*Coronal NECT in a 71-year-old man with left flank pain shows an incidental collision tumor composed of a myelolipoma <img src='img/arrows/WS.png'/> and an adenoma <img src='img/arrows/WC.png'/>.*
![Sagittal US in a 44-year-old woman with abdominal pain shows a large, echogenic suprarenal mass <img src='img/arrows/WS.png'/> that displaces the kidney <img src='img/arrows/WC.png'/>. CT was recommended given the size of the lesion.](images/app.statdx.com_image_thumbnail_4ad1da80-3b93-454f-8625-cd9d33ac3be8_size_168_quality_85_a27cc341_20251017T212346Z.jpg)
*Sagittal US in a 44-year-old woman with abdominal pain shows a large, echogenic suprarenal mass <img src='img/arrows/WS.png'/> that displaces the kidney <img src='img/arrows/WC.png'/>. CT was recommended given the size of the lesion.*
![Axial CECT in the same patient confirms a fat-attenuation suprarenal mass <img src='img/arrows/WS.png'/>. Extrarenal angiomyolipoma was ultimately resected. The differential diagnosis of large myelolipomas includes retroperitoneal liposarcomas, angiomyolipomas, and adrenal carcinomas with lipomatous metaplasia.](images/app.statdx.com_image_thumbnail_9278e376-e505-4f09-94a9-239eefd8f9e8_size_168_quality_85_60a777d6_20251017T212346Z.jpg)
*Axial CECT in the same patient confirms a fat-attenuation suprarenal mass <img src='img/arrows/WS.png'/>. Extrarenal angiomyolipoma was ultimately resected. The differential diagnosis of large myelolipomas includes retroperitoneal liposarcomas, angiomyolipomas, and adrenal carcinomas with lipomatous metaplasia.*
### Additional Images
![Axial CECT shows a heterogeneous, predominantly fatty right adrenal mass with calcified foci.](images/app.statdx.com_image_thumbnail_8e63e932-8b9d-4c9c-a66c-72408d20f720_size_168_quality_85_dc991339_20251017T212346Z.jpg)
*Axial CECT shows a heterogeneous, predominantly fatty right adrenal mass with calcified foci.*
![Axial CECT shows a predominantly fatty left adrenal mass.](images/app.statdx.com_image_thumbnail_b015b1c8-f5ed-4866-9d4e-bc2844f3cfbc_size_168_quality_85_34e2ce9f_20251017T212346Z.jpg)
*Axial CECT shows a predominantly fatty left adrenal mass.*
![Axial chemical shift (opposed-phase) MR shows a heterogeneous right adrenal mass with signal loss at fat/soft tissue interfaces.](images/app.statdx.com_image_thumbnail_b6e00fdd-d8a9-4e74-bf3c-c5842e7542df_size_168_quality_85_5506f0d1_20251017T212346Z.jpg)
*Axial chemical shift (opposed-phase) MR shows a heterogeneous right adrenal mass with signal loss at fat/soft tissue interfaces.*
![Axial CECT shows a large, heterogeneous fatty myelolipoma in the right adrenal gland and an adenoma in the left adrenal gland.](images/app.statdx.com_image_thumbnail_4109be83-ec4e-4d8b-9970-a696f5fe1db8_size_168_quality_85_a62180b0_20251017T212346Z.jpg)
*Axial CECT shows a large, heterogeneous fatty myelolipoma in the right adrenal gland and an adenoma in the left adrenal gland.*
docs_md/articles/adrenal_082ca43c-db5c-4770-aeed-0c6ea317e8fc.md
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---
title: "Adrenal"
docid: "082ca43c-db5c-4770-aeed-0c6ea317e8fc"
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---
# TERMINOLOGY
- ## Abbreviations
- Adrenal corticotrophic hormone (ACTH)
# GROSS ANATOMY
- ## Overview
- Adrenal (**suprarenal**) glands are part of endocrine and neurological systems
- Essentially different organs within same structure, composed of thick outer cortex and thin inner medulla
- Lie within**perirenal space**bilaterally, bounded by**renal** (**perirenal**)**fascia**, above/medial to kidneys
- Composed of "body" and 2 limbs (medial and lateral)
- ## Anatomic Relationships
- Right adrenal is usually more apical in location
- Lies anterolateral to right crus of diaphragm, medial to liver, and posterior to inferior vena cava (IVC)
- Often pyramidal in shape with inverted V shape on transverse section
- Left adrenal is usually more caudal and lies medial to upper pole of left kidney, lateral to left crus of diaphragm, and posterior to splenic vein and pancreas
- Often crescentic in shape with λ or triangular shape on transverse section
- ## Divisions
- **Adrenal cortex**
- Embryologically derived from mesoderm
- Divided into 3 distinct zones (zona glomerulosa, zona fasciculata, and zona reticularis)
- Secretes **mineralocorticoids**(aldosterone) from zona glomerulosa, **glucocorticoids**(cortisol) from zona fasciculata, and **androgens**from zona reticularis
- **Adrenal medulla**
- Embryologically derived from neural crest
- Part of sympathetic nervous system
- **Chromaffin cells** secrete **catecholamines** (mostly epinephrine) into bloodstream
- **Vessels**,**nerves**, and **lymphatics**
- Arteries
- **Superior adrenal arteries**: Typically 6-8; from inferior phrenic arteries
- **Middle adrenal artery**: 1; from abdominal aorta
- **Inferior adrenal artery**: 1; from renal arteries
- Veins
- **Right adrenal vein** drains into IVC
- **Left adrenal vein** drains into left renal vein (usually after joining left inferior phrenic vein)
- Nerves
- Extensive sympathetic connection to adrenal medulla
- Presynaptic sympathetic fibers from paravertebral ganglia end directly on secretory cells of medulla
- Lymphatics
- Drain to **lumbar** (**aortic** and **caval**) **nodes**
# ANATOMY IMAGING ISSUES
- ## Multimodality Imaging Appearance
- No consensus on "normal" size or thickness of adrenals but average thickness of ~ 3 mm for medial/lateral limbs
- While not based on any strong evidence, > 10-mm thickness can be used as threshold for hyperplasia
- MR: Generally isointense to liver on T1 MR and isointense to slightly hyperintense to liver on T2 MR
- Ultrasound: Easiest to visualize in newborns (as result of physiologic enlargement) and become progressively more difficult to visualize with age
- Right adrenal gland easier to visualize than left (due to lack of liver as acoustic window and overlying bowel gas)
- Adrenal glands in adults usually hypoechoic (juxtaposed against hyperechoic periadrenal fat), although medulla can rarely be discretely seen and appears hyperechoic
- ## Key Concepts
- **Adrenal** (**cortical**) **adenomas**
- Very common (at least 2% of general population) but usually cause no symptoms
- Mostly "nonfunctioning" but identical to "functional" adenomas that cause Cushing/Conn syndrome
- Most adenomas contain abundant lipid (precursor to steroid hormones), allowing definitive diagnosis using CT/MR sequences that highlight lipid
- Lipid is intracellular/intercellular (not macroscopic deposits of fat)
- Best CT technique: Nonenhanced CT with nodule measuring < 10 HU; or multiphase-enhanced CT with nodule demonstrating "washout" kinetics
- Best MR technique: Chemical-shift MR with signal dropout within nodule on opposed-phase images
- Standard imaging features for diagnosis of adenoma should be used for nodules measuring < 4 cm, while lesions > 4 cm should raise concern for malignancy
- **Pheochromocytoma** (tumor of adrenal medulla)
- Signs: Headache, palpitations, excessive perspiration
- 90% arise in adrenal, 90% unilateral, 90% benign
- Similar tumor arising in other chromaffin cells of sympathetic ganglia is called **paraganglioma**
- More common with multiple endocrine neoplasia, neurofibromatosis, and von Hippel-Lindau
- Often markedly hypervascular in arterial phase
- **Adrenal myelolipoma**
- Uncommon benign tumor (usually incidental finding) composed of mature adipose and hematopoietic tissue
- Characterized by presence of **macroscopic fat**
- May have internal soft tissue component or calcification
- **Adrenocortical carcinoma**
- Highly aggressive malignancy with poor prognosis
- Large, heterogeneous mass (often with necrosis, hemorrhage, or calcification) with frequent local invasion, vascular invasion, and distant metastases
- **Cushing syndrome** (excess cortisol)
- Signs: Truncal obesity, hirsutism, hypertension
- Causes: Pituitary tumors (→ adrenal corticotrophic hormone), exogenous (medications) > adrenal adenoma > carcinoma
- **Conn syndrome** (excess aldosterone)
- Signs: Hypertension, hypokalemic alkalosis
- Causes: Adrenal adenomas > hyperplasia > carcinoma
- **Addison syndrome**(adrenal insufficiency)
- Signs: Hypotension, weight loss, altered pigmentation
- Causes: Autoimmune disease > adrenal metastases > adrenal hemorrhage > adrenal infection
# CLINICAL IMPLICATIONS
- ## Clinical Importance
- Rich adrenal blood supply due to endocrine function
- Results in adrenal glands being common site for hematologic **metastases** (lung, breast, melanoma, etc.)
- Adrenal glands respond to stress (trauma, sepsis, surgery, etc.) by secreting ↑ cortisol and epinephrine
- Overwhelming stress may result in **adrenal hemorrhage**or acute adrenal insufficiency (addisonian crisis)
d7703d36-250d-428e-bf99-6439a7cdc980
## References
# Selected References
1. [Corwin MT et al: CT of hemorrhagic adrenal adenomas: radiologic-pathologic correlation. Abdom Radiol (NY). 48(2):680-7, 2023](http://www.ncbi.nlm.nih.gov/pubmed/?term=36380211%5Bpmid%5D)
1. [McCarthy CJ et al: Adrenal imaging: magnetic resonance imaging and computed tomography. Front Horm Res. 45:55-69, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=27003562%5Bpmid%5D)
1. [Park JJ et al: Adrenal imaging for adenoma characterization: imaging features, diagnostic accuracies and differential diagnoses. Br J Radiol. 89(1062):20151018, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=26867466%5Bpmid%5D)
1. [Taner AT et al: Pitfalls in adrenal imaging. Semin Roentgenol. 50(4):260-72, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=26542427%5Bpmid%5D)
1. [Kim KW et al: Sonography of the adrenal glands in the adult. J Clin Ultrasound. 40(6):357-63, 2012](http://www.ncbi.nlm.nih.gov/pubmed/?term=22585678%5Bpmid%5D)
1. [Ma G et al: Sectional anatomy of the adrenal gland in the coronal plane. Surg Radiol Anat. 30(3):271-80, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18246295%5Bpmid%5D)
1. [Matsuura T et al: Radiologic anatomy of the right adrenal vein: preliminary experience with MDCT. AJR Am J Roentgenol. 191(2):402-8, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18647909%5Bpmid%5D)
1. [Mitty HA: Embryology, anatomy, and anomalies of the adrenal gland. Semin Roentgenol. 23(4):271-9, 1988](http://www.ncbi.nlm.nih.gov/pubmed/?term=3055310%5Bpmid%5D)
## Images
### Adrenal Vessels and Relations
![The adrenal glands rest atop the kidneys with an interposed layer of fat. Reflecting their critical role in maintaining homeostasis and responding to stress, the adrenal glands have a very rich vascular supply. The superior adrenal arteries are short branches of the inferior phrenic arteries bilaterally. The middle adrenal arteries are short vessels arising from the aorta. The inferior adrenal arteries are branches of the renal arteries. The left adrenal vein drains into the left renal vein, while the right adrenal vein drains directly into the inferior vena cava (IVC). Note the size of the adrenal glands is somewhat exaggerated in this graphic to facilitate demonstration of the vascular anatomy.](images/app.statdx.com_image_thumbnail_ed9d5037-0cba-4ec5-9dc3-a518d0f5572a_size_168_quality_85_17fbbd57_20251017T212319Z.jpg)
*The adrenal glands rest atop the kidneys with an interposed layer of fat. Reflecting their critical role in maintaining homeostasis and responding to stress, the adrenal glands have a very rich vascular supply. The superior adrenal arteries are short branches of the inferior phrenic arteries bilaterally. The middle adrenal arteries are short vessels arising from the aorta. The inferior adrenal arteries are branches of the renal arteries. The left adrenal vein drains into the left renal vein, while the right adrenal vein drains directly into the inferior vena cava (IVC). Note the size of the adrenal glands is somewhat exaggerated in this graphic to facilitate demonstration of the vascular anatomy.*
![The adrenal glands rest atop the kidneys with an interposed layer of fat. Reflecting their critical role in maintaining homeostasis and responding to stress, the adrenal glands have a very rich vascular supply. The superior adrenal arteries are short branches of the inferior phrenic arteries bilaterally. The middle adrenal arteries are short vessels arising from the aorta. The inferior adrenal arteries are branches of the renal arteries. The left adrenal vein drains into the left renal vein, while the right adrenal vein drains directly into the inferior vena cava (IVC). Note the size of the adrenal glands is somewhat exaggerated in this graphic to facilitate demonstration of the vascular anatomy.](images/app.statdx.com_image_thumbnail_ed9d5037-0cba-4ec5-9dc3-a518d0f5572a_size_174_quality_85_b0310a83_20251017T212318Z.jpg)
*The adrenal glands rest atop the kidneys with an interposed layer of fat. Reflecting their critical role in maintaining homeostasis and responding to stress, the adrenal glands have a very rich vascular supply. The superior adrenal arteries are short branches of the inferior phrenic arteries bilaterally. The middle adrenal arteries are short vessels arising from the aorta. The inferior adrenal arteries are branches of the renal arteries. The left adrenal vein drains into the left renal vein, while the right adrenal vein drains directly into the inferior vena cava (IVC). Note the size of the adrenal glands is somewhat exaggerated in this graphic to facilitate demonstration of the vascular anatomy.*
### Adrenal Axial Anatomy and Relations
![The right adrenal is often more cephalic in location and lies above the right kidney, while the left adrenal lies partly in front of the upper pole of the left kidney. The left adrenal lies directly posterior to the splenic vein and body of pancreas and lateral to the left crus of the diaphragm. The right adrenal lies lateral to the crus, medial to the liver, and directly behind the IVC.](images/app.statdx.com_image_thumbnail_9db3619a-bfcf-487e-9446-565db3a3d64e_size_168_quality_85_3a56299a_20251017T212320Z.jpg)
*The right adrenal is often more cephalic in location and lies above the right kidney, while the left adrenal lies partly in front of the upper pole of the left kidney. The left adrenal lies directly posterior to the splenic vein and body of pancreas and lateral to the left crus of the diaphragm. The right adrenal lies lateral to the crus, medial to the liver, and directly behind the IVC.*
![The adrenal gland is essentially 2 organs in a single structure. The cortex is an endocrine gland, secreting primarily cortisol, aldosterone, and androgenic steroids. All of these hormones are derived from cholesterol, which imparts the characteristic lipid-rich appearance and imaging characteristics of the gland. The adrenal medulla is part of the autonomic nervous system and secretes epinephrine and norepinephrine.](images/app.statdx.com_image_thumbnail_31c96e4b-51c4-4162-abc4-9ac89fc26fe5_size_168_quality_85_18978cd7_20251017T212319Z.jpg)
*The adrenal gland is essentially 2 organs in a single structure. The cortex is an endocrine gland, secreting primarily cortisol, aldosterone, and androgenic steroids. All of these hormones are derived from cholesterol, which imparts the characteristic lipid-rich appearance and imaging characteristics of the gland. The adrenal medulla is part of the autonomic nervous system and secretes epinephrine and norepinephrine.*
### Axial CT, Normal Adrenal Anatomy
![First of 3 axial CECT images shows normal adrenal glands bilaterally. The right adrenal is usually suprarenal, touches the back of the IVC, and lies lateral to the right crus and medial to the liver. The left adrenal usually lies ventral to the upper pole of the left kidney and behind the splenic vein. The left adrenal often appears as an inverted Y shape, while the right is more like an inverted V.](images/app.statdx.com_image_thumbnail_b6765488-b561-4b6d-9bfa-ce7cc38bf48a_size_168_quality_85_71faea19_20251017T212319Z.jpg)
*First of 3 axial CECT images shows normal adrenal glands bilaterally. The right adrenal is usually suprarenal, touches the back of the IVC, and lies lateral to the right crus and medial to the liver. The left adrenal usually lies ventral to the upper pole of the left kidney and behind the splenic vein. The left adrenal often appears as an inverted Y shape, while the right is more like an inverted V.*
![Both limbs of the right adrenal are seen on this section.](images/app.statdx.com_image_thumbnail_55ccfc6f-9d04-456e-b4ee-443001b9aef5_size_168_quality_85_80973548_20251017T212319Z.jpg)
*Both limbs of the right adrenal are seen on this section.*
![The lowest portions of the adrenals are seen on this section.](images/app.statdx.com_image_thumbnail_58a2a23b-e970-4cdf-b5f0-e644d7823c23_size_168_quality_85_d3bf9617_20251017T212320Z.jpg)
*The lowest portions of the adrenals are seen on this section.*
### Coronal CT, Normal Adrenal Anatomy
![First of 2 coronal CECT images shows the adrenal glands in their suprarenal location, which accounts for their alternate name of &quot;suprarenal glands.&quot;](images/app.statdx.com_image_thumbnail_b08d6424-f10b-42ef-a9cf-d3200ef6f314_size_168_quality_85_7f263b86_20251017T212320Z.jpg)
*First of 2 coronal CECT images shows the adrenal glands in their suprarenal location, which accounts for their alternate name of &quot;suprarenal glands.&quot;*
![More anterior coronal CT shows the adrenal glands and their relation to adjacent structures, such as the crura of the diaphragm.](images/app.statdx.com_image_thumbnail_23172264-20e7-41eb-80fc-07e9cc7a84e8_size_168_quality_85_2e9c8cfb_20251017T212319Z.jpg)
*More anterior coronal CT shows the adrenal glands and their relation to adjacent structures, such as the crura of the diaphragm.*
### Axial and Coronal, Normal Adrenal Anatomy
![First of 3 CT images of a subject with normal adrenal glands shows conventional anatomy.](images/app.statdx.com_image_thumbnail_b2d11f2c-32c0-4410-9a56-171b7bdd8e66_size_168_quality_85_04c95f59_20251017T212319Z.jpg)
*First of 3 CT images of a subject with normal adrenal glands shows conventional anatomy.*
![More caudal section shows both adrenal glands with an inverted Y appearance. There is mild thickening of the left adrenal gland at the confluence of the medial and lateral limbs, a normal finding.](images/app.statdx.com_image_thumbnail_c04fdb54-8b1a-4f02-9e9b-37438386fc9e_size_168_quality_85_66861549_20251017T212319Z.jpg)
*More caudal section shows both adrenal glands with an inverted Y appearance. There is mild thickening of the left adrenal gland at the confluence of the medial and lateral limbs, a normal finding.*
![Coronal view demonstrates the relationship between the adrenals and adjacent organs.](images/app.statdx.com_image_thumbnail_54894842-d8ca-4a7a-9a0b-91a5396049c6_size_168_quality_85_08a0cd8c_20251017T212319Z.jpg)
*Coronal view demonstrates the relationship between the adrenals and adjacent organs.*
### Axial MR, Normal Adrenal Anatomy
![First of 2 axial T1 C+ MR images demonstrates the bilateral adrenal glands. At this level, the 2 limbs of each adrenal gland are not yet discretely visible.](images/app.statdx.com_image_thumbnail_33a4702f-edd8-4e76-b935-7b384e720870_size_168_quality_85_7c214e87_20251017T212320Z.jpg)
*First of 2 axial T1 C+ MR images demonstrates the bilateral adrenal glands. At this level, the 2 limbs of each adrenal gland are not yet discretely visible.*
![Axial T1 C+ MR at a slightly more caudal level now brings into view the twin limbs of each of the 2 adrenal glands. Each limb is thin and regular (without thickening or a nodule). Note that the right adrenal gland contacts the posterior margin of the IVC and is medial to the right diaphragmatic crus. The left adrenal gland is located slightly ventral and above the left kidney.](images/app.statdx.com_image_thumbnail_97e57ad6-d88b-42eb-9e92-d3c444327987_size_168_quality_85_f20aff08_20251017T212320Z.jpg)
*Axial T1 C+ MR at a slightly more caudal level now brings into view the twin limbs of each of the 2 adrenal glands. Each limb is thin and regular (without thickening or a nodule). Note that the right adrenal gland contacts the posterior margin of the IVC and is medial to the right diaphragmatic crus. The left adrenal gland is located slightly ventral and above the left kidney.*
![Axial T2 HASTE MR demonstrates a normal appearance of the adrenal glands on these images acquired without fat saturation. The adrenal glands in this image appear relatively isointense to the liver (although the relative signal can vary depending on application of fat saturation).](images/app.statdx.com_image_thumbnail_aa4ded60-a587-43fb-8694-9c0f9b374f41_size_168_quality_85_65a6b8ca_20251017T212319Z.jpg)
*Axial T2 HASTE MR demonstrates a normal appearance of the adrenal glands on these images acquired without fat saturation. The adrenal glands in this image appear relatively isointense to the liver (although the relative signal can vary depending on application of fat saturation).*
### Adrenal Venogram
![First of 2 images shows selective catheterization of the adrenal veins in a young woman with hyperaldosteronism, but no definite mass is seen on CT. Selective adrenal vein sampling was requested to assess unilateral excess aldosterone secretion. A catheter has been inserted through the right femoral vein, and the tip was advanced into the opening of the right adrenal vein. The adrenal veins are very fragile and could be easily ruptured by a forceful injection of contrast medium. The angiographer must know the vascular anatomy and gently probe the venous orifice, confirming the location with a small bolus injection of contrast medium, as shown here.](images/app.statdx.com_image_thumbnail_80ddfce4-5a49-4e91-97eb-ba46b792dd18_size_168_quality_85_68c71ba0_20251017T212319Z.jpg)
*First of 2 images shows selective catheterization of the adrenal veins in a young woman with hyperaldosteronism, but no definite mass is seen on CT. Selective adrenal vein sampling was requested to assess unilateral excess aldosterone secretion. A catheter has been inserted through the right femoral vein, and the tip was advanced into the opening of the right adrenal vein. The adrenal veins are very fragile and could be easily ruptured by a forceful injection of contrast medium. The angiographer must know the vascular anatomy and gently probe the venous orifice, confirming the location with a small bolus injection of contrast medium, as shown here.*
![A subsequent image shows the catheter repositioned. The tip has been advanced through the left renal vein to enter the left adrenal vein. No attempt is made to opacify the smaller venous tributaries.](images/app.statdx.com_image_thumbnail_cdba0f53-d87e-49e6-89f6-11c6dc9bbce2_size_168_quality_85_97305338_20251017T212320Z.jpg)
*A subsequent image shows the catheter repositioned. The tip has been advanced through the left renal vein to enter the left adrenal vein. No attempt is made to opacify the smaller venous tributaries.*
### Fetal Adrenal and Kidney
![Graphic shows the appearance of the adrenal and kidney in the fetus and neonate. The adrenal is much larger, relative to the kidney, than in an adult. The kidney has a lobulated appearance reflecting the ongoing fusion of the individual renal lobes, each composed of 1 renal pyramid and its associated renal cortex.](85f188a5-8cdf-4255-8898-e6736a510487)
*Graphic shows the appearance of the adrenal and kidney in the fetus and neonate. The adrenal is much larger, relative to the kidney, than in an adult. The kidney has a lobulated appearance reflecting the ongoing fusion of the individual renal lobes, each composed of 1 renal pyramid and its associated renal cortex.*
### Neonatal Adrenals and Kidney
![First of 3 ultrasounds of a neonate shows the characteristic prominence of the adrenal gland and the lobation of the renal surface in early infancy. This sagittal image shows the large adrenal gland adjacent to the upper pole of the kidney.](7bac6487-396d-4eda-bb7a-a5c6138dc1a4)
*First of 3 ultrasounds of a neonate shows the characteristic prominence of the adrenal gland and the lobation of the renal surface in early infancy. This sagittal image shows the large adrenal gland adjacent to the upper pole of the kidney.*
![Sagittal ultrasound shows the prominent limbs of the right adrenal gland.](3636b60f-e3ef-4805-b642-371afda96291)
*Sagittal ultrasound shows the prominent limbs of the right adrenal gland.*
![Sagittal ultrasound of the kidney shows its lobulated contour, a normal finding in the fetus and neonate.](c5b7c794-98c4-4f1e-a504-54e5df6a8756)
*Sagittal ultrasound of the kidney shows its lobulated contour, a normal finding in the fetus and neonate.*
### Adrenal Adenoma CT
![Axial NECT demonstrates a low-density nodule within the left adrenal gland with a Hounsfield attenuation of 14 HU, compatible with an adrenal adenoma. Patients with adenomas may be symptomatic (e.g., signs of excess cortisol or aldosterone) or asymptomatic. Most subjects have the adrenal lesion discovered incidentally on a CT performed for some other reason and have no clinical symptoms or signs. In this setting, the adenoma is said to be nonfunctional.](9e3a4364-8716-4a59-8fc2-1330b42874ab)
*Axial NECT demonstrates a low-density nodule within the left adrenal gland with a Hounsfield attenuation of 14 HU, compatible with an adrenal adenoma. Patients with adenomas may be symptomatic (e.g., signs of excess cortisol or aldosterone) or asymptomatic. Most subjects have the adrenal lesion discovered incidentally on a CT performed for some other reason and have no clinical symptoms or signs. In this setting, the adenoma is said to be nonfunctional.*
![Axial CECT demonstrates a low-density left adrenal nodule that demonstrated a noncontrast attenuation of 3, an absolute washout of 77.3%, and a relative washout percentage of 74.4%, features compatible with a benign adrenal adenoma (calculated based on a formal multiphase adrenal protocol CT).](72fcb0bc-fd16-4d86-8aef-d4a51bce37fd)
*Axial CECT demonstrates a low-density left adrenal nodule that demonstrated a noncontrast attenuation of 3, an absolute washout of 77.3%, and a relative washout percentage of 74.4%, features compatible with a benign adrenal adenoma (calculated based on a formal multiphase adrenal protocol CT).*
![Axial NECT demonstrates a low-density, lipid-rich adenoma with Hounsfield attenuation of 6-8 HU, characteristic of a lipid-rich adenoma. Adenomas can be either lipid rich (common between 70-90%) or lipid poor. Lipid-rich adenomas, due to their high lipid content, are of low density on NECT.](4ee3efab-0f3a-4e82-b466-3707e9bb1ebf)
*Axial NECT demonstrates a low-density, lipid-rich adenoma with Hounsfield attenuation of 6-8 HU, characteristic of a lipid-rich adenoma. Adenomas can be either lipid rich (common between 70-90%) or lipid poor. Lipid-rich adenomas, due to their high lipid content, are of low density on NECT.*
### Adrenal Adenoma MR
![Axial GRE in-phase MR demonstrates a homogeneous rounded left suprarenal mass.](b4e3263d-48a4-4606-bf3e-f20585ab6473)
*Axial GRE in-phase MR demonstrates a homogeneous rounded left suprarenal mass.*
![Axial GRE opposed-phase MR through the same level shows marked loss of signal within the adrenal mass, confirming the presence of intravoxel/intracellular lipid. This finding is diagnostic of an adrenal adenoma.](e9b4ed4b-977a-4bbf-a502-02d59d19e9c1)
*Axial GRE opposed-phase MR through the same level shows marked loss of signal within the adrenal mass, confirming the presence of intravoxel/intracellular lipid. This finding is diagnostic of an adrenal adenoma.*
![Axial T2 FS MR demonstrates that the adrenal adenoma is relatively low in signal, similar in signal to the normal-appearing left adrenal gland. Note that this is unlike most malignant adrenal masses, which tend to be T2 hyperintense.](d333db0b-aca2-4603-bac5-0fa2ad200b0f)
*Axial T2 FS MR demonstrates that the adrenal adenoma is relatively low in signal, similar in signal to the normal-appearing left adrenal gland. Note that this is unlike most malignant adrenal masses, which tend to be T2 hyperintense.*
### Adrenal Hyperplasia
![First of 3 axial CECT images of a 40-year-old woman with congenital adrenal hyperplasia shows diffuse enlargement of both adrenal glands but preservation of their normal shape.](fb262467-fbc6-489d-8b69-82b24cfcf30f)
*First of 3 axial CECT images of a 40-year-old woman with congenital adrenal hyperplasia shows diffuse enlargement of both adrenal glands but preservation of their normal shape.*
![Each limb of the adrenal is in excess of 1 cm in diameter, one criterion used to diagnose or suggest adrenal hyperplasia. Most patients with adrenal hyperplasia have less markedly enlarged glands due to pituitary (or ectopic) production of excess adrenal corticotrophic hormone. In many cases the adrenal glands may appear normal by imaging.](c3fbb3f0-cde5-42e9-813c-d0b2c4afb046)
*Each limb of the adrenal is in excess of 1 cm in diameter, one criterion used to diagnose or suggest adrenal hyperplasia. Most patients with adrenal hyperplasia have less markedly enlarged glands due to pituitary (or ectopic) production of excess adrenal corticotrophic hormone. In many cases the adrenal glands may appear normal by imaging.*
![The striking enlargement of the adrenals is again evident on this image.](b4698b52-4e1d-4365-99c3-831bb2b8bf9d)
*The striking enlargement of the adrenals is again evident on this image.*
### Adrenal Myelolipoma
![Axial NECT demonstrates a fat-density mass arising from the left adrenal gland. The presence of macroscopic fat within an adrenal mass is virtually diagnostic of an adrenal myelolipoma.](96f97a27-4cb0-45bb-95e9-23f18f9b960d)
*Axial NECT demonstrates a fat-density mass arising from the left adrenal gland. The presence of macroscopic fat within an adrenal mass is virtually diagnostic of an adrenal myelolipoma.*
![Transverse ultrasound demonstrates a left adrenal mass, which is markedly echogenic and without internal color flow vascularity, representing a myelolipoma.](8249072f-54ac-43f9-b58d-9793a1661f37)
*Transverse ultrasound demonstrates a left adrenal mass, which is markedly echogenic and without internal color flow vascularity, representing a myelolipoma.*
![Axial CECT demonstrates a left adrenal mass with internal solid enhancing components, but with clear evidence of macroscopic fat, compatible with a myelolipoma. Myelolipomas can demonstrate enhancing components and calcification, but the presence of macroscopic fat is essentially diagnostic of this entity.](68a9a82e-ec6f-4c85-81f0-93112da7afe7)
*Axial CECT demonstrates a left adrenal mass with internal solid enhancing components, but with clear evidence of macroscopic fat, compatible with a myelolipoma. Myelolipomas can demonstrate enhancing components and calcification, but the presence of macroscopic fat is essentially diagnostic of this entity.*
### Pheochromocytoma
![Coronal T2 FS MR demonstrates a T2-hyperintense mass arising from the left adrenal gland in a patient with symptoms of malignant hypertension. Pheochromocytomas are classically said to be light bulb bright on T2 MR. While not true in every case, the majority of pheochromocytomas are T2 hyperintense.](a0c2419d-b33f-4540-864d-0bc8bd757d96)
*Coronal T2 FS MR demonstrates a T2-hyperintense mass arising from the left adrenal gland in a patient with symptoms of malignant hypertension. Pheochromocytomas are classically said to be light bulb bright on T2 MR. While not true in every case, the majority of pheochromocytomas are T2 hyperintense.*
![Coronal arterial-phase CECT demonstrates a markedly hypervascular right adrenal mass, an appearance that is very suggestive of a pheochromocytoma. These lesions tend to show marked enhancement on arterial-phase imaging, making multiphase imaging (either CT or MR) critical in their imaging diagnosis.](9d93735e-ce1b-411f-8cbe-ffca0896d640)
*Coronal arterial-phase CECT demonstrates a markedly hypervascular right adrenal mass, an appearance that is very suggestive of a pheochromocytoma. These lesions tend to show marked enhancement on arterial-phase imaging, making multiphase imaging (either CT or MR) critical in their imaging diagnosis.*
![Axial CECT demonstrates an avidly enhancing mass with subtle central necrosis arising from the right adrenal gland, compatible with a pheochromocytoma, in this patient with malignant hypertension and palpitations.](f37ae353-6fc8-49f9-9c91-a6483160cb7b)
*Axial CECT demonstrates an avidly enhancing mass with subtle central necrosis arising from the right adrenal gland, compatible with a pheochromocytoma, in this patient with malignant hypertension and palpitations.*
![Axial CECT in the arterial phase demonstrates a right adrenal mass, which is markedly hypervascular around its margins and centrally necrotic. The presence of markedly hypervascular soft tissue in an adrenal mass should strongly suggest the diagnosis of pheochromocytoma. This mass was surgically resected and confirmed to represent a pheochromocytoma.](aff50f9c-5a44-46d0-83f0-addbde7b339d)
*Axial CECT in the arterial phase demonstrates a right adrenal mass, which is markedly hypervascular around its margins and centrally necrotic. The presence of markedly hypervascular soft tissue in an adrenal mass should strongly suggest the diagnosis of pheochromocytoma. This mass was surgically resected and confirmed to represent a pheochromocytoma.*
![Axial T1 C+ MR demonstrates a large right adrenal mass with a sizable central necrotic component and an avidly enhancing peripheral solid component, found to represent a pheochromocytoma upon surgical resection.](3d3908d1-ce66-4a35-be9e-a837ac2b81a5)
*Axial T1 C+ MR demonstrates a large right adrenal mass with a sizable central necrotic component and an avidly enhancing peripheral solid component, found to represent a pheochromocytoma upon surgical resection.*
![Axial arterial-phase CECT demonstrates a small nodule in the right adrenal gland, which is markedly hypervascular with a Hounsfield attenuation of 150 HU. Adrenal nodules with attenuation values &gt; 110 HU in the arterial phase are highly likely to represent pheochromocytomas, confirmed in this case at surgery.](e9b6aefe-a0c0-41fb-944a-37092250ef45)
*Axial arterial-phase CECT demonstrates a small nodule in the right adrenal gland, which is markedly hypervascular with a Hounsfield attenuation of 150 HU. Adrenal nodules with attenuation values &gt; 110 HU in the arterial phase are highly likely to represent pheochromocytomas, confirmed in this case at surgery.*
### Adrenal Metastases
![Axial CECT demonstrates large, vascular bilateral adrenal masses, compatible with metastases from the patient's primary hepatocellular carcinoma (a portion of which is visible in the liver on this image). As in this case, metastases to the adrenal gland often demonstrate very similar enhancement and texture to the primary tumor site.](dcdfb960-b854-4cd9-b512-a0beacda0043)
*Axial CECT demonstrates large, vascular bilateral adrenal masses, compatible with metastases from the patient's primary hepatocellular carcinoma (a portion of which is visible in the liver on this image). As in this case, metastases to the adrenal gland often demonstrate very similar enhancement and texture to the primary tumor site.*
![Axial CECT demonstrates a large, hypodense, heterogeneous mass arising from the right adrenal gland, representing a metastasis from the patient's primary lung cancer. Lung, breast, and renal cell carcinoma, along with malignant melanoma, frequently metastasize to the adrenal glands due to the rich blood supply of the adrenals.](2e2589bb-20da-4bae-910a-4ef88e6c5d3e)
*Axial CECT demonstrates a large, hypodense, heterogeneous mass arising from the right adrenal gland, representing a metastasis from the patient's primary lung cancer. Lung, breast, and renal cell carcinoma, along with malignant melanoma, frequently metastasize to the adrenal glands due to the rich blood supply of the adrenals.*
![Axial T C+ MR demonstrates a small hypervascular mass in the right adrenal gland, compatible with a metastasis from the patient's primary clear cell renal cell carcinoma.](8258c4bd-10f3-4b30-a66d-f1f187bac301)
*Axial T C+ MR demonstrates a small hypervascular mass in the right adrenal gland, compatible with a metastasis from the patient's primary clear cell renal cell carcinoma.*
### Adrenocortical Carcinoma
![Axial T2 FS MR demonstrates a large, infiltrative, aggressive-appearing mass arising in the right suprarenal fossa. Any mass with this size and appearance, in the absence of a history of primary malignancy elsewhere, should raise strong suspicion for adrenocortical carcinoma.](12bc3e8c-c9cf-42a3-a25c-edade88d2fea)
*Axial T2 FS MR demonstrates a large, infiltrative, aggressive-appearing mass arising in the right suprarenal fossa. Any mass with this size and appearance, in the absence of a history of primary malignancy elsewhere, should raise strong suspicion for adrenocortical carcinoma.*
![Axial CECT demonstrates a large, heterogeneous mass with central necrosis arising in the left upper quadrant. As with many cases of adrenocortical carcinoma, these masses can be quite large, and the site of origin for the mass may be difficult to determine.](69990356-84d7-4776-9865-9d3799f4396c)
*Axial CECT demonstrates a large, heterogeneous mass with central necrosis arising in the left upper quadrant. As with many cases of adrenocortical carcinoma, these masses can be quite large, and the site of origin for the mass may be difficult to determine.*
![Coronal CECT demonstrates a large mass, which is clearly separate from the left kidney and arising from the left suprarenal fossa (ostensibly from the left adrenal gland). Any mass of this size arising in this location is strongly suspicious for adrenocortical carcinoma, a diagnosis that was confirmed at surgery.](a91dc631-b70b-4087-bba7-697c6c3fefa2)
*Coronal CECT demonstrates a large mass, which is clearly separate from the left kidney and arising from the left suprarenal fossa (ostensibly from the left adrenal gland). Any mass of this size arising in this location is strongly suspicious for adrenocortical carcinoma, a diagnosis that was confirmed at surgery.*
![Coronal CECT demonstrates a large, heterogeneous, enhancing mass arising from the right suprarenal fossa with tumor directly extending into (and distending) the IVC. Adrenocortical carcinoma commonly invades surrounding venous structures, including the renal vein or IVC.](7c43ec4b-aeed-407e-92a9-2b7dd9b59e30)
*Coronal CECT demonstrates a large, heterogeneous, enhancing mass arising from the right suprarenal fossa with tumor directly extending into (and distending) the IVC. Adrenocortical carcinoma commonly invades surrounding venous structures, including the renal vein or IVC.*
![Axial CECT demonstrates a large right suprarenal mass with direct extension to involve the left kidney as well as extensive tumor thrombus involving the left renal vein. This was ultimately confirmed to represent an adrenocortical carcinoma at resection.](2869a648-d5f2-4a20-8547-72e9f1c4f789)
*Axial CECT demonstrates a large right suprarenal mass with direct extension to involve the left kidney as well as extensive tumor thrombus involving the left renal vein. This was ultimately confirmed to represent an adrenocortical carcinoma at resection.*
![Coronal CECT demonstrates a right-sided adrenocortical carcinoma that directly invades the right hepatic lobe. Note the presence of extensive calcification within the mass, a common feature with these tumors.](b50efe6d-4eef-438f-86e6-fc922ecd4081)
*Coronal CECT demonstrates a right-sided adrenocortical carcinoma that directly invades the right hepatic lobe. Note the presence of extensive calcification within the mass, a common feature with these tumors.*
### Adrenal Hemorrhage
![Coronal NECT in a patient who was hypotensive due to blunt abdominal trauma demonstrates enlargement of the left adrenal gland with heterogeneous, high-density material, characteristic of acute hemorrhage. This &quot;nodule&quot; was not present on a prior CT from a few months earlier, aiding in the diagnosis.](4254ed57-ab54-4057-bbc7-1aa2635d250b)
*Coronal NECT in a patient who was hypotensive due to blunt abdominal trauma demonstrates enlargement of the left adrenal gland with heterogeneous, high-density material, characteristic of acute hemorrhage. This &quot;nodule&quot; was not present on a prior CT from a few months earlier, aiding in the diagnosis.*
![Axial NECT in a septic, hypotensive patient in the ICU setting demonstrates a large left adrenal hemorrhage. Notably, a study performed a few days prior had shown a completely normal left adrenal gland.](c27efb42-05c0-4a55-8542-4c35b6aba979)
*Axial NECT in a septic, hypotensive patient in the ICU setting demonstrates a large left adrenal hemorrhage. Notably, a study performed a few days prior had shown a completely normal left adrenal gland.*
![Axial CECT in a patient who had recently undergone surgery for ovarian cancer demonstrates a new intermediate-density nodule arising from the right adrenal gland. This nodule was new since a recent prior study, compatible with a small adrenal hemorrhage.](696d929d-4347-4487-82df-90af3794d96e)
*Axial CECT in a patient who had recently undergone surgery for ovarian cancer demonstrates a new intermediate-density nodule arising from the right adrenal gland. This nodule was new since a recent prior study, compatible with a small adrenal hemorrhage.*
### Gastric Diverticulum Simulating Adrenal Mass
![Axial CECT shows a cystic-appearing &quot;lesion&quot; in the left suprarenal region, simulating an adrenal mass. The mass has the same density as the water-filled stomach, but note the presence of internal gas.](cae53618-b8b1-408d-a27f-a6623ced872c)
*Axial CECT shows a cystic-appearing &quot;lesion&quot; in the left suprarenal region, simulating an adrenal mass. The mass has the same density as the water-filled stomach, but note the presence of internal gas.*
![Sagittal CECT in the same patient nicely shows that this &quot;lesion&quot; is actually a gastric diverticulum directly communicating with the adjacent stomach. Gastric diverticula, which often arise in this location, are not infrequently mistaken for adrenal nodules.](f7440a33-4731-4c4b-a70d-36c4263b23e2)
*Sagittal CECT in the same patient nicely shows that this &quot;lesion&quot; is actually a gastric diverticulum directly communicating with the adjacent stomach. Gastric diverticula, which often arise in this location, are not infrequently mistaken for adrenal nodules.*
![An oblique film from a barium upper GI series shows a barium-filled gastric diverticulum projecting posteriorly from the gastric cardia.](b9935717-2009-4783-a564-7fa148d72619)
*An oblique film from a barium upper GI series shows a barium-filled gastric diverticulum projecting posteriorly from the gastric cardia.*
### Adrenal Insufficiency
![First of 3 axial CT sections in a patient with adrenal insufficiency (addisonian syndrome) due to autoimmune disease is shown. The adrenal glands are extremely small and difficult to visualize.](89e3fc49-9668-43d1-a94d-7898afb5950f)
*First of 3 axial CT sections in a patient with adrenal insufficiency (addisonian syndrome) due to autoimmune disease is shown. The adrenal glands are extremely small and difficult to visualize.*
![The small adrenals are again evident. If adrenal insufficiency were due to adrenal tumors, bleeding, or infection, the glands would be enlarged.](98e3bc50-58d4-4eb9-a1c4-b93cd420d826)
*The small adrenals are again evident. If adrenal insufficiency were due to adrenal tumors, bleeding, or infection, the glands would be enlarged.*
![The adrenal glands have a normal shape and morphology but are extremely small.](deabf3fa-96b5-4dfb-afb9-0fa0a18252bd)
*The adrenal glands have a normal shape and morphology but are extremely small.*
docs_md/articles/alzheimer-disease_2aad3ac4-44fd-43e5-8e50-a86987483af3.md
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---
title: "Alzheimer Disease"
docid: "2aad3ac4-44fd-43e5-8e50-a86987483af3"
authors:
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value: "Akiva Mintz, MD, PhD, MHA, CFA"
- key: "1f262abe-db83-4f18-99af-00bd3045cd4d"
value: "Marc Benayoun, MD, PhD"
- key: "bbc899b6-2885-44bb-a5b0-24eec7314d33"
value: "Bryan J. Neth, BS"
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name: "Nuclear Medicine"
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slug: "central-nervous-system"
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name: "Neurodegeneration"
slug: "neurodegeneration"
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name: "Alzheimer Disease"
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pageDescription: "Alzheimer Disease"
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---
# KEY FACTS
- ## Terminology
- Alzheimer disease (AD)
- Progressive neurodegenerative brain disease related to build up of (Aβ) neuritic plaques and subsequent tau neurofibrillary tangles (NFTs)
- ## Imaging
- Amyloid PET
- Cortical amyloid deposition on PET is early biomarker in AD and appears prior to clinical symptoms
- Absence of amyloid plaque rules out AD in patients with dementia
- ↓ gray-white matter differentiation in at least 2 regions or single area of focally ↑ gray matter uptake are signs of positive florbetapir study
- F-18 FDG PET
- Glucose hypometabolism in parietotemporal, posterior cingulate, and precuneus regions; usually symmetric
- Glucose hypometabolism continues to worsen with disease progression
- Atypical AD variants can show hypometabolism in occipital lobes, frontal lobes, or with marked bilateral asymmetry
- SPECT
- 2nd-line study if PET is not available/reimbursed
- Tau PET
- Cortical deposition of tau NFTs in posterolateral temporal lobes, parietal lobes, occipital lobes, and cingulate gyrus can stage disease severity
- Negative to early disease stage by tau PET can predict better response to amyloid targeting therapies (ATTs) with 50% of patients showing improved memory function at 3 years of treatment
# TERMINOLOGY
- ## Definitions
- Alzheimer disease (AD)
- Progressive neurodegenerative brain disease generally characterized by impairments in episodic memory and other cognitive domains
- Likely related to β-amyloid (Aβ) neuritic plaques and tau neurofibrillary tangles (NFTs) leading to synaptic dysfunction, neuronal/glial cell death
- ATN(C) classification defines patient status according to amyloid (A), tau (T), neurodegeneration (N), and clinical (C) status
- Amyloid determined by PET &/or certain cerebrospinal fluid (CSF) biomarkers with plasma biomarkers on horizon
- Tau determined by PET &/or CSF markers with plasma biomarkers on horizon
- Neurodegeneration typical for AD via MR, FDG PET, or CSF biomarkers
- Clinical status by formal neurocognitive evaluation/screening with MMSE or MoCA
- Role of imaging
- Early detection of AD neuropathologic changes (ADNCs) prior to symptom onset
- Diagnosis of AD with clinical presentation and other biomarkers
- Differential diagnosis between AD and other causes of dementia
- Preclinical AD or asymptomatic ADNC
- Stage of disease process where pathologic Aβ plaque or tau NFT deposition has occurred but prior to onset of significant clinically detectable symptoms
- Amyloid PET positive; positive CSF Aβ42 or p-tau181/217
- Mild cognitive impairment (MCI)
- Clinical symptoms of memory &/or other cognitive problems greater than normal for age and education
- ↑ risk of conversion to AD but not all progress to full dementia
- Annual conversion rate from MCI to dementia ~ 5-10%
# IMAGING
- ## General Features
- ### Best diagnostic clue
- ADNC (A+T+/-N+/-C-)
- Amyloid PET positive (or CSF biomarker positive)
- May or may not show changes on tau PET or FDG PET/MR
- ↑ risk of developing AD symptoms
- MCI and early AD (A+T+/-N+/-C+) clinically meeting criteria for MCI
- Amyloid PET positive
- F-18 FDG PET, tau PET, and MR abnormalities become more evident and can help stage disease severity, risk for symptomatic conversion of ADNC, and help assess likelihood that symptoms are related to AD
- Mild clinical symptoms
- Late AD (A+T+/-N+/-C+) clinically meeting criteria for dementia
- Amyloid and F-18 FDG PET grossly positive
- FDG PET hypometabolism and cortical tau PET accumulation can become more extensive or involve atypical areas
- More extensive atrophy present (CT/MR)
- ## Nuclear Medicine Findings
- Amyloid PET imaging
- F-18 florbetapir, flutemetamol, and florbetaben tracers are FDA approved
- ↑ brain amyloid in gray matter on PET, normal off-target white matter binding to myelin proteins
- Earliest imaging biomarker in AD
- Absence of amyloid plaque rules out AD in patients with dementia
- Interpretation of amyloid PET images
- View in black-on-white background at high contrast levels (flutemetamol can be read in color)
- View axial images 1st
- Coronal and sagittal views for confirmation
- Cerebellum gray-white differentiation is baseline for discerning normal gray matter from physiologic tracer retained in white matter
- Positive scan (moderate or frequent Aβ deposition in cerebral cortex)
- Blurring of gray/white matter junction due to radiotracer uptake in gray matter
- ↑ gray matter uptake in temporal, parietal, and frontal cortices
- Uptake in posterior cingulate gyrus and precuneus (may be early deposition sign)
- F-18 florbetapir: ↓ gray-white matter differentiation in at least 2 regions or single area of focally ↑ gray matter uptake ≥ adjacent white matter = positive
- Extent of amyloid deposition does not correlate with severity of AD
- Negative scan (no evidence of significant Aβ deposition)
- Symptoms unlikely due to AD
- Does not exclude non-AD dementia
- Artifacts and pitfalls
- Severely diminished gray matter volume in AD patients may make abnormal exam appear normal due to contoured cortex
- View fused PET/CT or PET/MR images as supplement to evaluate uptake relative to white and gray matter
- Highest transaxial images above orbits often have diminished gray-white matter differentiation, even in normal patients
- F-18 FDG PET
- General F-18 FDG uptake patterns
- MCI: Medial temporal lobe hypometabolism
- Early AD
- Relative reduction in activity in parietal, temporal lobes and posterior cingulate gyri and precuneus, usually symmetric
- Advanced AD
- Progression of findings present in early AD
- Usually symmetric
- Frontal lobe hypometabolism can develop later in disease course
- Moderate to severe atrophy
- Atypical AD patterns
- Posterior cortical atrophy: Similar hypometabolism to typical AD + asymmetric occipital hypometabolism
- Logopenic variant primary progressive aphasia (lvPPA): Similar to typical AD though with strong left-sided predominance (language dominant hemisphere)
- Behavioral or dysexecutive AD: 50% similar to typical AD + extensive frontal hypometabolism; 50% indistinguishable from frontotemporal dementia (FTD) without parietal involvement
- F-18 FDG PET most accurate when read in conjunction with quantitative software that compares F-18 FDG uptake to age-matched normal database
- Surface projections also ↑ sensitivity for AD detection
- SPECT perfusion with Tc-99m HMPAO or Tc-99m ethyl cystine dimer (ECD) has similar appearance as PET but ↓ resolution and sensitivity
- Tau PET
- Flortaucipir: Only FDA-approved tau PET tracer
- Compare uptake to reference region [1.65 x mean standardized uptake value ratio (SUV) cerebellum], which acts as internal negative control during interpretation
- Negative if cortical binding is limited to mesial and anteromedial temporal lobes or isolated frontal lobes, off-target binding to choroid plexus
- Mild/early AD may be indistinguishable from normal study
- Moderate AD if cortical binding extends to posterolateral temporal lobes and temporooccipital regions
- Advanced AD if cortical binding extends beyond to parietal, occipital, and cingulate regions
- Negative to early disease stage by tau PET can predict better response to amyloid targeting therapies (ATTs) with 50% of patients showing improved memory function at 3 years of treatment
- ## MR Findings
- T1WI, T2WI
- Atrophy of medial temporal lobe structures (entorhinal cortex, hippocampus); visible as early as MCI and posterior structures (precuneus and parietal convexities)
- Rates of whole-brain and hippocampal atrophy may be used to monitor progression of neurodegeneration and help stage AD severity
- Can use volumetrix to quantitatively assess volume loss
- ## Imaging Recommendations
- ### Best imaging tool
- Amyloid PET best for ruling out AD
- Appropriate Use Criteria (AUC) for amyloid PET (SNMMI and AA joint task force)
- Objectively confirmed persistent or progressive unexplained cognitive impairment
- May satisfy core clinical criteria for AD but unclear presentation, such as atypical or mixed
- Progressive dementia and atypically early age of onset (< 65 years)
- Candidacy for ATTs
- If considering amyloid PET, knowledge of Aβ pathology should ↑ diagnostic certainty &/or patient management
- Amyloid and F-18 FDG PET can help to differentiate between AD and other causes of dementia (FTD, Lewy body dementia)
- PET may be used in early diagnosis of ADNC
- Correlate imaging results with clinical picture and other biomarkers of AD
- ### Protocol advice
- Amyloid PET
- Patient preparation
- Patient needs to lie still for 20-30 min; thus, immobilization techniques may be necessary
- Radiopharmaceutical, dose, and time of scan post injection
- F-18 florbetapir (Amyvid), 10 mCi, 30-50 min
- F-18 flutemetamol (Vizamyl), 5 mCi, 90 min
- F-18 florbetaben (Neuraceq), 8 mCi, 45-130 min
- Dosimetry: Gallbladder wall receives highest dose, followed by intestines
- Image acquisition
- Depends largely on available PET scanner
- CT typically used for attenuation correction; older PET scanners may use separate source (e.g., Ge-68/68-Ga) for transmission scan
- Imaging begins 30-130 min after injection
- 20-min acquisition
- Matrix: Transaxial 128 x 128 or 256 x 256
- Pixel size: 2-3 mm
- Slice thickness: 2-4 mm
- Filtered back projection or iterative reconstruction
- F-18 FDG PET
- Patient preparation
- Patient should fast, stop IV fluids containing dextrose, and stop parenteral feeding for 4-6 hours
- Blood sugar should be < 150-200 mg/dL
- Patient should be placed in quiet, dimly lit room prior to and after injection for 30 min
- Radiopharmaceutical: F-18 FDG
- Dose: 5-20 mCi (185-740 MBq)
- Dosimetry: Urinary bladder receives largest dose
- Image acquisition: 30-60 min after injection
- Perfusion SPECT
- 2nd-line study if PET is not available
- Patient preparation
- Place patient in quiet, dimly lit room prior to and after injection for 30 min
- Radiopharmaceutical
- Tc-99m exametazime (HMPAO)
- Tc-99m bicisate (ECD)
- Dose: 15-30 mCi (555 MBq to 1.1 GBq)
- Dosimetry
- Tc-99m HMPAO: Kidneys receive highest dose
- Tc-99m ECD: Bladder wall receives highest dose
- Image acquisition
- Optimal imaging time for Tc-99m HMPAO: 90 min post injection
- Optimal imaging time for Tc-99m ECD: 45 min post injection
- Tau PET
- Dose: 10 mCi flortaucipir
- Image acquisition: ~ 80 min after injection, 20-min acquisition
- Dosimetry: Intestines and liver are critical organs
# DIFFERENTIAL DIAGNOSIS
- ## Vascular Dementia (Multiinfarct Dementia)
- Impaired blood supply to brain regions
- 2nd most common cause of dementia
- Global atrophy with diffuse white matter lesions/infarcts that generally correlate with cognitive symptoms
- ## Alzheimer Disease Mixed Dementia
- AD and other dementia
- ## Dementia With Lewy Bodies
- Commonly presents with hallucinations, sleep disturbances, and parkinsonian motor features
- F-18 FDG PET hypometabolism in occipital cortex distinguishes from typical amnestic AD
- Consider DaT SPECT or FDOPA PET to help exclude posterior cortical atrophy if clinically uncertain
- Can consider cardiac MIBG scan (reduced cardiac sympathetic activity in dementia with Lewy bodies, see on MIBG)
- Skin biopsy for α-synuclein
- ## Frontotemporal Dementia
- Commonly presents with personality and behavioral changes, which can also be seen with behavioral/dysexecutive AD variants
- Atrophy of frontal and anterior temporal lobes
- Language variant FTDs (semantic and nonfluent agrammatic PPA) can mimic lvPPA
- F-18 FDG PET hypometabolism primarily in frontal and anterior temporal lobes
- Negative amyloid PET; helps exclude AD in favor of FTD
- ## Creutzfeldt-Jakob Disease
- Rapidly fatal, prion-related disease with impairments in cognition and behavioral changes
- MR DWI: Hyperintensity in striatum, cingulum, neocortex
- ## Causes of Reversible Dementia
- [Normal-pressure hydrocephalus](/document/normal-pressure-hydrocephalus/834ccc3e-2116-4295-8408-0ac9a06bd2ff)
- Hypothyroidism
- Infections: Neurosyphilis, HIV
- Trauma (e.g., chronic subdural hematoma)
- Tumor, other mass lesions
- Depression
- Vitamin B12 deficiency
- ## Other Neurodegenerative Disease
- Parkinson disease
- Huntington disease
- ## Cerebral Amyloid Angiopathy
- Abnormal accumulation of amyloid in leptomeningeal and cortical vessels, leading to intracranial hemorrhage
- Can present with cognitive decline concerning for AD
- Amyloid positive scan
- MR is most helpful for finding lobar predominant microhemorrhages and superficial siderosis
- Can have inflammatory variants and even amyloidomas (mimicking intracranial mass)
- ## Primary Age-Related Tauopathy (A-T+N+/-C+)
- Tau-related NFTs limited to temporal lobes without amyloid deposition on PET
- Patients are typically much older than AD, and cognitive decline rate is typically slower
- ## Limbic-Predominant Age-Related TDP-43 Encephalopathy
- Amyloid negative (unless associated with AD copathology)
- Typically in older patients (> 80 years)
- Extensive temporal lobe FDG hypometabolism with less involvement of parietal structures
- MR may show marked hippocampal sclerosis
# PATHOLOGY
- ## General Features
- ### Etiology
- Most likely combination of genetic, lifestyle, and environmental factors
- Pathologic deposition of Aβ neuritic plaques and subsequent tau NFTs in pathogenesis
- Accumulation of extracellular amyloid plaques contribute to disrupted synaptic communication and neuronal death
- Accumulation of intracellular tau NFTs contribute to disruption of nutrient and molecular transfer and neuronal death
- ### Genetics
- Early-onset, familial AD
- Single-gene mutation
- βA precursor protein (*APP*) gene on chromosome 21
- Presenilin 1 (*PSEN1*) gene on chromosome 14
- Presenilin 2 (*PSEN2*) gene on chromosome 1
- Results in formation of abnormal proteins involved in APP
- May contribute to production of harmful forms of βA and βA-related pathology
- Late-onset, sporadic AD
- Significant risk is related to apolipoprotein E (*APOE*) gene on chromosome 19
- ApoE plays role in cholesterol transport and βA maintenance
- ApoE exists as 3 alleles (e2, e3, e4) with each individual carrying 2 copies
- ApoE-e4 allele is present in 20-30% of USA population and confers ↑ risk for AD development
- 40-65% of individuals with AD carry at least 1 copy of e4 allele
# CLINICAL ISSUES
- ## Presentation
- ### Most common signs/symptoms
- Significant impairment in memory and cognition with typical AD
- Mood and personality changes with behavioral/dysexecutive AD variant
- Language impairment, including sentence repetition, preserved single-word meaning with lvPPA
- Visuospatial and visuoperceptual impairment with posterior cortical atrophy
- ### Clinical profile
- Preclinical AD
- Amyloid PET turns positive during this period
- No noticeable symptoms of AD with early AD brain changes (up to 20 years before symptoms)
- MCI
- Change in cognition within ≥ 1 cognitive domains with functional independence intact
- Possible AD
- Significant cognitive/behavioral symptoms
- Must represent change from prior status
- Must interfere with functional ability
- In presence of sudden onset &/or another disorder that could cause similar symptoms, e.g., cerebrovascular disease
- Probable AD
- Insidious change of cognitive/behavioral symptoms that interferes with functional ability
- Not in presence of another disorder that could cause similar symptoms
- ## Demographics
- ### Epidemiology
- ~ 5.2 million in USA affected by AD
- Most common cause of dementia (60-80% of cases)
- Prevalence
- ~ 11% of adults ≥ 65 years
- ~ 32% of adults ≥ 85 years
- ## Treatment
- Novel amyloid-targeting antibody therapies slow decline and may improve mental status if started early
- Requires initial baseline MR to exclude cerebral amyloid and preexisting risk for hemorrhage
- Requires surveillance for development of amyloid-related imaging abnormalities (ARIA)
- Cholinesterase inhibitors may delay worsening of cognitive symptoms for 6-12 months
- NMDA inhibitors may temporarily delay worsening of symptoms
# DIAGNOSTIC CHECKLIST
- ## Key Imaging Findings
- AD neuropathic change
- Amyloid PET positive or positive key CSF biomarkers
- AD
- FDG hypometabolism develops in parietal lobes, precuneus, posterior cingulate gyrus, posterior temporal lobes, and frontal lobes in advanced cases
- Tau PET shows NFTs in posterolateral temporal neocortex, temporooccipital regions, and, eventually, parietal and cingulate regions
47897414-6c3f-419d-8061-50a189ef4e1f
## References
# Selected References
1. [Rabinovici GD et al: Updated Appropriate Use Criteria for amyloid and tau PET: a report from the Alzheimer's Association and Society for Nuclear Medicine and Molecular Imaging workgroup. J Nucl Med. ePub, 2025](http://www.ncbi.nlm.nih.gov/pubmed/?term=39778970%5Bpmid%5D)
1. [Jack CR Jr et al: Revised criteria for diagnosis and staging of Alzheimer's disease: Alzheimer's Association workgroup. Alzheimers Dement. 20(8):5143-69, 2024](http://www.ncbi.nlm.nih.gov/pubmed/?term=38934362%5Bpmid%5D)
1. [Malpetti M et al: From clinical trials to memory clinics, Tau-PET visual reads can help diagnosis and patient stratification. Neurology. 101(19):813-4, 2023](http://www.ncbi.nlm.nih.gov/pubmed/?term=37748880%5Bpmid%5D)
1. [Polsinelli AJ et al: Atypical Alzheimer disease variants. Continuum (Minneap Minn). 28(3):676-701, 2022](http://www.ncbi.nlm.nih.gov/pubmed/?term=35678398%5Bpmid%5D)
1. [Jagust W: Imaging the evolution and pathophysiology of Alzheimer disease. Nat Rev Neurosci. 19(11):687-700, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=30266970%5Bpmid%5D)
1. [ScienceDirect: Alzheimer's Association report 2018 Alzheimer's disease facts and figures. Published May 2018. Accessed April 2025. https://www.sciencedirect.com/science/article/pii/S1552526018300414?via%3Dihub](https://www.sciencedirect.com/science/article/pii/S1552526018300414?via%3Dihub)
1. [Rice L et al: The diagnostic value of FDG and amyloid PET in Alzheimer's disease-a systematic review. Eur J Radiol. 94:16-24, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=28941755%5Bpmid%5D)
1. [Weidman DA et al: Added value and limitations of amyloid-PET imaging: review and analysis of selected cases of mild cognitive impairment and dementia. Neurocase. 23(1):41-51, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=28376695%5Bpmid%5D)
1. [Minoshima S et al: SNMMI procedure standard/EANM practice guideline for amyloid PET imaging of the brain 1.0. J Nucl Med. 57(8):1316-22, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=27481605%5Bpmid%5D)
1. [Johnson KA et al: Appropriate use criteria for amyloid PET: a report of the Amyloid Imaging Task Force, the Society of Nuclear Medicine and Molecular Imaging, and the Alzheimer's Association. Alzheimers Dement. 9(1):e-1-16, 2013](http://www.ncbi.nlm.nih.gov/pubmed/?term=23360977%5Bpmid%5D)
1. [Hyman BT et al: National Institute on Aging-Alzheimer's Association guidelines for the neuropathologic assessment of Alzheimer's disease. Alzheimers Dement. 8(1):1-13, 2012](http://www.ncbi.nlm.nih.gov/pubmed/?term=-1%5Bpmid%5D)
1. [Herholz K et al: Clinical amyloid imaging in Alzheimer's disease. Lancet Neurol. 10(7):667-70, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=21683932%5Bpmid%5D)
1. [McKhann GM et al: The diagnosis of dementia due to Alzheimer's disease: recommendations from the National Institute on Aging-Alzheimer's Association workgroups on diagnostic guidelines for Alzheimer's disease. Alzheimers Dement. 7(3):263-9, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=-1%5Bpmid%5D)
## Images
### Selected Images
![Axial F-18 florbetapir PET in a patient with mild cognitive impairment (MCI) demonstrates homogeneous uptake throughout the brain without clear distinction between gray <img src='img/arrows/BS.png'/> and white <img src='img/arrows/WS.png'/> matter, consistent with heavy β-amyloid deposition in the gray matter, which is seen in Alzheimer disease (AD).](images/app.statdx.com_image_thumbnail_0e50cc9f-f820-4fce-b604-5fb93f8881a9_size_168_quality_85_ebda1bb6_20251014T193414Z.jpg)
*Axial F-18 florbetapir PET in a patient with mild cognitive impairment (MCI) demonstrates homogeneous uptake throughout the brain without clear distinction between gray <img src='img/arrows/BS.png'/> and white <img src='img/arrows/WS.png'/> matter, consistent with heavy β-amyloid deposition in the gray matter, which is seen in Alzheimer disease (AD).*
![Axial F-18 florbetapir PET in a patient with mild cognitive impairment (MCI) demonstrates homogeneous uptake throughout the brain without clear distinction between gray <img src='img/arrows/BS.png'/> and white <img src='img/arrows/WS.png'/> matter, consistent with heavy β-amyloid deposition in the gray matter, which is seen in Alzheimer disease (AD).](images/app.statdx.com_image_thumbnail_0e50cc9f-f820-4fce-b604-5fb93f8881a9_size_174_quality_85_07ba70ac_20251014T185333Z.jpg)
*Axial F-18 florbetapir PET in a patient with mild cognitive impairment (MCI) demonstrates homogeneous uptake throughout the brain without clear distinction between gray <img src='img/arrows/BS.png'/> and white <img src='img/arrows/WS.png'/> matter, consistent with heavy β-amyloid deposition in the gray matter, which is seen in Alzheimer disease (AD).*
![Axial F-18 florbetapir PET in a patient with mild cognitive impairment (MCI) demonstrates homogeneous uptake throughout the brain without clear distinction between gray <img src='img/arrows/BS.png'/> and white <img src='img/arrows/WS.png'/> matter, consistent with heavy β-amyloid deposition in the gray matter, which is seen in Alzheimer disease (AD).](images/app.statdx.com_image_thumbnail_0e50cc9f-f820-4fce-b604-5fb93f8881a9_size_174_quality_85_cb5ecae8_20251014T193347Z.jpg)
*Axial F-18 florbetapir PET in a patient with mild cognitive impairment (MCI) demonstrates homogeneous uptake throughout the brain without clear distinction between gray <img src='img/arrows/BS.png'/> and white <img src='img/arrows/WS.png'/> matter, consistent with heavy β-amyloid deposition in the gray matter, which is seen in Alzheimer disease (AD).*
![Axial F-18 florbetapir PET in a patient with mild cognitive impairment (MCI) demonstrates homogeneous uptake throughout the brain without clear distinction between gray <img src='img/arrows/BS.png'/> and white <img src='img/arrows/WS.png'/> matter, consistent with heavy β-amyloid deposition in the gray matter, which is seen in Alzheimer disease (AD).](images/app.statdx.com_image_thumbnail_0e50cc9f-f820-4fce-b604-5fb93f8881a9_size_174_quality_85_ce82be9e_20251014T190917Z.jpg)
*Axial F-18 florbetapir PET in a patient with mild cognitive impairment (MCI) demonstrates homogeneous uptake throughout the brain without clear distinction between gray <img src='img/arrows/BS.png'/> and white <img src='img/arrows/WS.png'/> matter, consistent with heavy β-amyloid deposition in the gray matter, which is seen in Alzheimer disease (AD).*
![Axial F-18 florbetapir PET in a patient with mild cognitive impairment (MCI) demonstrates homogeneous uptake throughout the brain without clear distinction between gray <img src='img/arrows/BS.png'/> and white <img src='img/arrows/WS.png'/> matter, consistent with heavy β-amyloid deposition in the gray matter, which is seen in Alzheimer disease (AD).](images/app.statdx.com_image_thumbnail_0e50cc9f-f820-4fce-b604-5fb93f8881a9_size_174_quality_85_ff145669_20251014T204456Z.jpg)
*Axial F-18 florbetapir PET in a patient with mild cognitive impairment (MCI) demonstrates homogeneous uptake throughout the brain without clear distinction between gray <img src='img/arrows/BS.png'/> and white <img src='img/arrows/WS.png'/> matter, consistent with heavy β-amyloid deposition in the gray matter, which is seen in Alzheimer disease (AD).*
![Axial F-18 florbetapir PET demonstrates characteristic physiologic uptake in the white matter <img src='img/arrows/WS.png'/> but no significant uptake in the gray matter <img src='img/arrows/BS.png'/>. This clear distinction indicates no detectable amyloid deposition.](images/app.statdx.com_image_thumbnail_d4f2e003-777d-4bf6-ba10-bf95e784caa1_size_168_quality_85_fec4e8e0_20251014T193414Z.jpg)
*Axial F-18 florbetapir PET demonstrates characteristic physiologic uptake in the white matter <img src='img/arrows/WS.png'/> but no significant uptake in the gray matter <img src='img/arrows/BS.png'/>. This clear distinction indicates no detectable amyloid deposition.*
![Left lateral F-18 FDG PET quantitative analysis in a patient with advanced AD shows hypometabolism in the parietal <img src='img/arrows/CS.png'/> and posterior temporal <img src='img/arrows/CO.png'/> lobes. The frontal lobe hypometabolism <img src='img/arrows/BS.png'/> occurs in later stages of the disease.](images/app.statdx.com_image_thumbnail_c61cc6c2-b138-4e0c-b4a0-f640be1fdbd2_size_168_quality_85_192696e7_20251014T193414Z.jpg)
*Left lateral F-18 FDG PET quantitative analysis in a patient with advanced AD shows hypometabolism in the parietal <img src='img/arrows/CS.png'/> and posterior temporal <img src='img/arrows/CO.png'/> lobes. The frontal lobe hypometabolism <img src='img/arrows/BS.png'/> occurs in later stages of the disease.*
![Left sagittal F-18 FDG PET quantitative analysis in the same patient shows hypometabolism in the precuneus <img src='img/arrows/CS.png'/> and posterior cingulate gyrus <img src='img/arrows/CO.png'/>. Note the pattern of AD is posterior-predominate hypometabolism.](images/app.statdx.com_image_thumbnail_93abe5c8-560b-4489-a870-c810f63ca955_size_168_quality_85_276ad772_20251014T193414Z.jpg)
*Left sagittal F-18 FDG PET quantitative analysis in the same patient shows hypometabolism in the precuneus <img src='img/arrows/CS.png'/> and posterior cingulate gyrus <img src='img/arrows/CO.png'/>. Note the pattern of AD is posterior-predominate hypometabolism.*
![Axial F-18 florbetapir PET in a patient with MCI demonstrates homogeneous uptake throughout the brain without clear distinction between gray <img src='img/arrows/BS.png'/> and white matter <img src='img/arrows/WS.png'/>, consistent with heavy β-amyloid (Aβ) deposition in the gray matter, which is seen in AD.](images/app.statdx.com_image_thumbnail_7d9a384e-6c82-4c9a-a70a-718ecaf5c602_size_168_quality_85_7c77f9d7_20251014T193415Z.jpg)
*Axial F-18 florbetapir PET in a patient with MCI demonstrates homogeneous uptake throughout the brain without clear distinction between gray <img src='img/arrows/BS.png'/> and white matter <img src='img/arrows/WS.png'/>, consistent with heavy β-amyloid (Aβ) deposition in the gray matter, which is seen in AD.*
![Axial F-18 florbetapir PET shows uptake in the white matter <img src='img/arrows/WS.png'/> but no significant uptake in the gray matter <img src='img/arrows/BS.png'/>, especially in the precuneus <img src='img/arrows/BC.png'/>, indicating no detectable amyloid deposition, representing a normal scan.](images/app.statdx.com_image_thumbnail_95bfc9b6-69f3-48b1-8009-6f5f690faaa6_size_168_quality_85_eb17a2bd_20251014T193415Z.jpg)
*Axial F-18 florbetapir PET shows uptake in the white matter <img src='img/arrows/WS.png'/> but no significant uptake in the gray matter <img src='img/arrows/BS.png'/>, especially in the precuneus <img src='img/arrows/BC.png'/>, indicating no detectable amyloid deposition, representing a normal scan.*
![More inferior axial F-18 florbetapir PET in the same patient shows homogeneous uptake throughout the brain without clear distinction between gray <img src='img/arrows/BS.png'/> and white <img src='img/arrows/WS.png'/> matter in the temporal lobe, consistent with heavy Aβ deposition in the gray matter, which is seen in AD.](images/app.statdx.com_image_thumbnail_14a9fc62-dd8a-4fb0-b742-15fabfc80ae9_size_168_quality_85_03e02cfe_20251014T193415Z.jpg)
*More inferior axial F-18 florbetapir PET in the same patient shows homogeneous uptake throughout the brain without clear distinction between gray <img src='img/arrows/BS.png'/> and white <img src='img/arrows/WS.png'/> matter in the temporal lobe, consistent with heavy Aβ deposition in the gray matter, which is seen in AD.*
![More inferior axial F-18 florbetapir PET in a normal exam shows characteristic uptake in the white matter <img src='img/arrows/WS.png'/> but no significant uptake extending in the gray matter <img src='img/arrows/BS.png'/>, indicating no detectable amyloid deposition.](images/app.statdx.com_image_thumbnail_c2dfe0b6-1032-4348-a2c5-a06ad0d949d4_size_168_quality_85_5b708bfd_20251014T193415Z.jpg)
*More inferior axial F-18 florbetapir PET in a normal exam shows characteristic uptake in the white matter <img src='img/arrows/WS.png'/> but no significant uptake extending in the gray matter <img src='img/arrows/BS.png'/>, indicating no detectable amyloid deposition.*
![More inferior axial F-18 florbetapir PET in the same patient shows homogeneous uptake in the temporal lobe gray matter <img src='img/arrows/BO.png'/>. The cerebellum maintains a clear distinction between gray <img src='img/arrows/BS.png'/> and white <img src='img/arrows/WS.png'/> matter, which is expected even in patients with significant Aβ deposition. Therefore, the cerebellum is used as a reference for gray-white distinction in all patients.](images/app.statdx.com_image_thumbnail_9d024d40-acff-4341-b692-8d6a2d350927_size_168_quality_85_7d1adf5f_20251014T193415Z.jpg)
*More inferior axial F-18 florbetapir PET in the same patient shows homogeneous uptake in the temporal lobe gray matter <img src='img/arrows/BO.png'/>. The cerebellum maintains a clear distinction between gray <img src='img/arrows/BS.png'/> and white <img src='img/arrows/WS.png'/> matter, which is expected even in patients with significant Aβ deposition. Therefore, the cerebellum is used as a reference for gray-white distinction in all patients.*
![More inferior axial F-18 florbetapir PET in a normal exam also demonstrates clear cerebellar gray <img src='img/arrows/BS.png'/> and white <img src='img/arrows/BO.png'/> differentiation.](images/app.statdx.com_image_thumbnail_a64ae948-4562-4c63-9234-3a67203da3a7_size_168_quality_85_ea9130f0_20251014T193415Z.jpg)
*More inferior axial F-18 florbetapir PET in a normal exam also demonstrates clear cerebellar gray <img src='img/arrows/BS.png'/> and white <img src='img/arrows/BO.png'/> differentiation.*
![Coronal F-18 florbetapir PET shows areas of uptake that resemble only white matter uptake <img src='img/arrows/WS.png'/> at 1st glance without activity reaching the gray matter <img src='img/arrows/BS.png'/>.](images/app.statdx.com_image_thumbnail_499aea61-93e7-4a7c-bf01-b1fc73d2f4c4_size_168_quality_85_c4cc4e32_20251014T193415Z.jpg)
*Coronal F-18 florbetapir PET shows areas of uptake that resemble only white matter uptake <img src='img/arrows/WS.png'/> at 1st glance without activity reaching the gray matter <img src='img/arrows/BS.png'/>.*
![Coronal CT in the same patient shows significant areas of brain atrophy <img src='img/arrows/BS.png'/>, accounting for areas that may appear to be negative for gray matter Aβ deposition but in reality represent areas of significant neurodegeneration. This underlines the importance of looking at the correlative imaging in addition to the qualitative or quantitative PET imaging.](images/app.statdx.com_image_thumbnail_f3311f1f-ed29-4b0a-80a6-e39141bd574d_size_168_quality_85_c1762375_20251014T193415Z.jpg)
*Coronal CT in the same patient shows significant areas of brain atrophy <img src='img/arrows/BS.png'/>, accounting for areas that may appear to be negative for gray matter Aβ deposition but in reality represent areas of significant neurodegeneration. This underlines the importance of looking at the correlative imaging in addition to the qualitative or quantitative PET imaging.*
![Axial F-18 florbetapir PET in the same patient demonstrates decreased gray-white differentiation indicating gray matter uptake <img src='img/arrows/BS.png'/> in some areas, concerning for Aβ deposition.](images/app.statdx.com_image_thumbnail_f509e713-0312-43ce-9f79-d6b53a7d1295_size_168_quality_85_f8078ecc_20251014T193415Z.jpg)
*Axial F-18 florbetapir PET in the same patient demonstrates decreased gray-white differentiation indicating gray matter uptake <img src='img/arrows/BS.png'/> in some areas, concerning for Aβ deposition.*
![Axial CT in the same patient shows significant areas of brain atrophy <img src='img/arrows/WS.png'/> in the parietal lobe.](images/app.statdx.com_image_thumbnail_2247714f-5804-41f0-b74b-b7602126e910_size_168_quality_85_d87f9550_20251014T193415Z.jpg)
*Axial CT in the same patient shows significant areas of brain atrophy <img src='img/arrows/WS.png'/> in the parietal lobe.*
![Coronal fusion F-18 florbetapir PET/CT in the same patient shows that activity correlates to remaining brain tissue <img src='img/arrows/WS.png'/>, which should not be misinterpreted as gray matter sparing.](images/app.statdx.com_image_thumbnail_41c6f654-a9f4-4c5a-a30b-f27d0c02bf31_size_168_quality_85_ef24e9e2_20251014T193415Z.jpg)
*Coronal fusion F-18 florbetapir PET/CT in the same patient shows that activity correlates to remaining brain tissue <img src='img/arrows/WS.png'/>, which should not be misinterpreted as gray matter sparing.*
![Axial fusion F-18 florbetapir PET/CT in the same patient confirms activity correlates to gray matter <img src='img/arrows/WS.png'/>, consistent with Aβ deposition.](images/app.statdx.com_image_thumbnail_eab292b9-5d29-401e-be68-842e0719b3ed_size_168_quality_85_ea2bbef2_20251014T193415Z.jpg)
*Axial fusion F-18 florbetapir PET/CT in the same patient confirms activity correlates to gray matter <img src='img/arrows/WS.png'/>, consistent with Aβ deposition.*
![Axial F-18 FDG PET in an older adult with MCI shows AD findings of reduced metabolism in the posterior parietal <img src='img/arrows/WO.png'/> and frontal <img src='img/arrows/CO.png'/> cortices. Importantly, there is sparing of the sensorimotor region <img src='img/arrows/WC.png'/>.](images/app.statdx.com_image_thumbnail_f51f89a5-ed4f-42ac-8f8d-a06193c867de_size_168_quality_85_41ebd74a_20251014T193415Z.jpg)
*Axial F-18 FDG PET in an older adult with MCI shows AD findings of reduced metabolism in the posterior parietal <img src='img/arrows/WO.png'/> and frontal <img src='img/arrows/CO.png'/> cortices. Importantly, there is sparing of the sensorimotor region <img src='img/arrows/WC.png'/>.*
![Axial F-18 FDG PET in a patient with late-stage AD demonstrates severely reduced metabolism in the posterior parietal <img src='img/arrows/WO.png'/> and frontal <img src='img/arrows/CO.png'/> cortices. Importantly, there is characteristic sparing of the sensorimotor region <img src='img/arrows/WC.png'/>.](images/app.statdx.com_image_thumbnail_5881d761-0146-4c8d-a411-617566c4d3df_size_168_quality_85_1a991d75_20251014T193415Z.jpg)
*Axial F-18 FDG PET in a patient with late-stage AD demonstrates severely reduced metabolism in the posterior parietal <img src='img/arrows/WO.png'/> and frontal <img src='img/arrows/CO.png'/> cortices. Importantly, there is characteristic sparing of the sensorimotor region <img src='img/arrows/WC.png'/>.*
![More inferior axial F-18 FDG PET in the patient with MCI shows further evidence of hypometabolism of the posterior parietal <img src='img/arrows/WO.png'/> and frontal <img src='img/arrows/CO.png'/> cortices. Importantly, there is characteristic sparing of the sensorimotor region <img src='img/arrows/WC.png'/> and visual cortex <img src='img/arrows/WS.png'/>.](images/app.statdx.com_image_thumbnail_c11d1b6e-82a9-4c18-92ef-e1f0f8f3a0df_size_168_quality_85_0975f6d4_20251014T193415Z.jpg)
*More inferior axial F-18 FDG PET in the patient with MCI shows further evidence of hypometabolism of the posterior parietal <img src='img/arrows/WO.png'/> and frontal <img src='img/arrows/CO.png'/> cortices. Importantly, there is characteristic sparing of the sensorimotor region <img src='img/arrows/WC.png'/> and visual cortex <img src='img/arrows/WS.png'/>.*
![More inferior axial F-18 FDG PET in the patient with late-stage AD shows severe hypometabolism of the posterior parietal <img src='img/arrows/WO.png'/> and frontal <img src='img/arrows/CO.png'/> cortices. Importantly, there is sparing of the sensorimotor region <img src='img/arrows/WC.png'/> and visual cortex <img src='img/arrows/WS.png'/>.](images/app.statdx.com_image_thumbnail_93fd22a3-610c-4d45-a57f-7df48db67039_size_168_quality_85_7e84a10b_20251014T193415Z.jpg)
*More inferior axial F-18 FDG PET in the patient with late-stage AD shows severe hypometabolism of the posterior parietal <img src='img/arrows/WO.png'/> and frontal <img src='img/arrows/CO.png'/> cortices. Importantly, there is sparing of the sensorimotor region <img src='img/arrows/WC.png'/> and visual cortex <img src='img/arrows/WS.png'/>.*
![More inferior axial F-18 FDG PET in the patient with MCI shows hypometabolism of the temporal <img src='img/arrows/CC.png'/> and frontal <img src='img/arrows/WO.png'/> cortices. Importantly, there is sparing of the basal ganglia <img src='img/arrows/WC.png'/> and visual cortex <img src='img/arrows/WS.png'/>.](images/app.statdx.com_image_thumbnail_6848eac9-5edf-4eaf-9dcd-1872b8307594_size_168_quality_85_4441fff2_20251014T193415Z.jpg)
*More inferior axial F-18 FDG PET in the patient with MCI shows hypometabolism of the temporal <img src='img/arrows/CC.png'/> and frontal <img src='img/arrows/WO.png'/> cortices. Importantly, there is sparing of the basal ganglia <img src='img/arrows/WC.png'/> and visual cortex <img src='img/arrows/WS.png'/>.*
![More inferior axial F-18 FDG PET in the patient with late-stage AD shows severe hypometabolism of the temporal <img src='img/arrows/CC.png'/> and frontal <img src='img/arrows/WO.png'/> cortices. Importantly, there is characteristic sparing of the basal ganglia <img src='img/arrows/WC.png'/> and visual cortex <img src='img/arrows/WS.png'/>.](images/app.statdx.com_image_thumbnail_71c51fd7-855b-49fa-accb-f0f971faa97f_size_168_quality_85_ad02b233_20251014T193415Z.jpg)
*More inferior axial F-18 FDG PET in the patient with late-stage AD shows severe hypometabolism of the temporal <img src='img/arrows/CC.png'/> and frontal <img src='img/arrows/WO.png'/> cortices. Importantly, there is characteristic sparing of the basal ganglia <img src='img/arrows/WC.png'/> and visual cortex <img src='img/arrows/WS.png'/>.*
![Axial fused tau PET/MR shows parietal <img src='img/arrows/CS.png'/> and precuneus <img src='img/arrows/WS.png'/> cortical binding to neurofibrillary tangles in this patient with late AD.](images/app.statdx.com_image_thumbnail_5dc9d89d-ca47-48e2-a863-ce9512af6051_size_168_quality_85_2379c4df_20251014T193415Z.jpg)
*Axial fused tau PET/MR shows parietal <img src='img/arrows/CS.png'/> and precuneus <img src='img/arrows/WS.png'/> cortical binding to neurofibrillary tangles in this patient with late AD.*
![Coronal fused tau PET/MR in the same patient shows additional cortical binding in the lateral temporal lobes <img src='img/arrows/CS.png'/> and cingulate gyrus <img src='img/arrows/WS.png'/>.](images/app.statdx.com_image_thumbnail_03520e67-ef25-4ef6-9eb3-a002630aef1a_size_168_quality_85_e9fe537a_20251014T193415Z.jpg)
*Coronal fused tau PET/MR in the same patient shows additional cortical binding in the lateral temporal lobes <img src='img/arrows/CS.png'/> and cingulate gyrus <img src='img/arrows/WS.png'/>.*
![Axial FDG PET/MR shows R &gt; L occipital hypometabolism <img src='img/arrows/CS.png'/> with additional involvement of the R &gt; L posterior cingulate gyrus <img src='img/arrows/WS.png'/> in a patient with near-cortical blindness.](images/app.statdx.com_image_thumbnail_22a41467-8d75-4673-9fee-22a8fc364015_size_168_quality_85_37282795_20251014T193415Z.jpg)
*Axial FDG PET/MR shows R &gt; L occipital hypometabolism <img src='img/arrows/CS.png'/> with additional involvement of the R &gt; L posterior cingulate gyrus <img src='img/arrows/WS.png'/> in a patient with near-cortical blindness.*
![Surface projection in same patient shows additional hypometabolism in the R &gt; L precuneus <img src='img/arrows/CS.png'/>, parietal <img src='img/arrows/WS.png'/>, and posterior temporal lobes <img src='img/arrows/CC.png'/>. Findings are consistent with posterior cortical atrophy. Aβ and tau pathology was confirmed by cerebrospinal fluid (CSF) biomarkers.](images/app.statdx.com_image_thumbnail_12c551af-89f0-4041-9122-44598cf56ada_size_168_quality_85_a3bd6172_20251014T193415Z.jpg)
*Surface projection in same patient shows additional hypometabolism in the R &gt; L precuneus <img src='img/arrows/CS.png'/>, parietal <img src='img/arrows/WS.png'/>, and posterior temporal lobes <img src='img/arrows/CC.png'/>. Findings are consistent with posterior cortical atrophy. Aβ and tau pathology was confirmed by cerebrospinal fluid (CSF) biomarkers.*
![Surface projections show anterior and middle cingulate hypometabolism <img src='img/arrows/CS.png'/> with subtle hypometabolism of the precuneus <img src='img/arrows/WS.png'/> in this patient with suspected frontotemporal dementia. Note relative sparing of the parietal and posterior temporal lobes. Amyloid CSF confirmed dysexecutive variant AD.](images/app.statdx.com_image_thumbnail_e8669f67-a84d-45fc-8b76-225f34e3d7a8_size_168_quality_85_783700ef_20251014T193415Z.jpg)
*Surface projections show anterior and middle cingulate hypometabolism <img src='img/arrows/CS.png'/> with subtle hypometabolism of the precuneus <img src='img/arrows/WS.png'/> in this patient with suspected frontotemporal dementia. Note relative sparing of the parietal and posterior temporal lobes. Amyloid CSF confirmed dysexecutive variant AD.*
![Stereotactic surface projections show marked asymmetric left-sided parietal <img src='img/arrows/WS.png'/> and posterior temporal <img src='img/arrows/CS.png'/> hypometabolism in this patient with clinical lvPPA.](images/app.statdx.com_image_thumbnail_54f46849-1187-421e-8e80-2dbc0a7751ca_size_168_quality_85_aad45407_20251014T193415Z.jpg)
*Stereotactic surface projections show marked asymmetric left-sided parietal <img src='img/arrows/WS.png'/> and posterior temporal <img src='img/arrows/CS.png'/> hypometabolism in this patient with clinical lvPPA.*
### Additional Images
![Axial Tc-99m ECD SPECT perfusion study in a patient with mild dementia shows findings of reduced perfusion in the posterior parietal <img src='img/arrows/WO.png'/> and frontal <img src='img/arrows/CO.png'/> cortices. Importantly, there is sparing of the sensorimotor region <img src='img/arrows/WC.png'/>.](383609db-d7d1-404d-bf85-f57f6940a8ed)
*Axial Tc-99m ECD SPECT perfusion study in a patient with mild dementia shows findings of reduced perfusion in the posterior parietal <img src='img/arrows/WO.png'/> and frontal <img src='img/arrows/CO.png'/> cortices. Importantly, there is sparing of the sensorimotor region <img src='img/arrows/WC.png'/>.*
![More inferior axial Tc-99m ECD perfusion SPECT in the same patient shows mildly decreased perfusion of the posterior parietal cortex <img src='img/arrows/WO.png'/>. Importantly, there is characteristic sparing of the visual cortex <img src='img/arrows/WS.png'/>.](fe076f39-4cbb-49c3-8041-495c97c3ac69)
*More inferior axial Tc-99m ECD perfusion SPECT in the same patient shows mildly decreased perfusion of the posterior parietal cortex <img src='img/arrows/WO.png'/>. Importantly, there is characteristic sparing of the visual cortex <img src='img/arrows/WS.png'/>.*
![More inferior axial Tc-99m ECD perfusion SPECT in the same patient shows decreased perfusion of the posterior temporal cortex <img src='img/arrows/CC.png'/> with sparing of the basal ganglia <img src='img/arrows/WC.png'/> and visual cortex <img src='img/arrows/WS.png'/>.](972c60e7-ab0e-4e79-97fb-6bd34e2b8615)
*More inferior axial Tc-99m ECD perfusion SPECT in the same patient shows decreased perfusion of the posterior temporal cortex <img src='img/arrows/CC.png'/> with sparing of the basal ganglia <img src='img/arrows/WC.png'/> and visual cortex <img src='img/arrows/WS.png'/>.*
![More inferior axial Tc-99m ECD perfusion SPECT in the same patient shows relatively similar perfusion of the posterior temporal <img src='img/arrows/CC.png'/>, frontal <img src='img/arrows/WO.png'/>, and visual cortex <img src='img/arrows/WS.png'/>.](e27cbfbb-69fc-4917-acc3-c3038362bd2d)
*More inferior axial Tc-99m ECD perfusion SPECT in the same patient shows relatively similar perfusion of the posterior temporal <img src='img/arrows/CC.png'/>, frontal <img src='img/arrows/WO.png'/>, and visual cortex <img src='img/arrows/WS.png'/>.*
docs_md/articles/alzheimer-disease_f71f5cf5-b1af-4c6d-b145-b4c10eec7b58.md
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---
title: "Alzheimer Disease"
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value: "Santhosh Gaddikeri, MD"
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pageTitle: "Alzheimer Disease | STATdx"
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---
# KEY FACTS
- ## Terminology
- Alzheimer disease (AD)
- Slowly progressive neurodegenerative disease
- ## Imaging
- Current role of imaging in AD
- Exclude other causes of dementia
- Identify region-specific patterns of brain volume loss
- Identify imaging markers of coexistent disease, such as amyloid angiopathy
- Identify early AD for possible innovative therapy
- Best imaging = volumetric MR, F-18 FDG PET
- Thinned gyri, widened sulci, and enlarged ventricles
- Medial temporal lobe particularly hippocampus and entorhinal cortex disproportionately affected
- F-18 FDG PET
- Early-stage AD: ↓ metabolism in parietotemporal association cortices, posterior cingulate, and precuneus regions
- Moderate to severe AD: Additional frontal lobe involvement
- Amyloid PET imaging: High sensitivity in detecting amyloid plaques and vascular amyloid in vivo
- ## Top Differential Diagnoses
- Normal aging
- Vascular dementia
- Normal-pressure hydrocephalus
- Frontotemporal lobar degeneration
- Dementia with Lewy bodies
- ## Clinical Issues
- Most common cause of dementia > age 65
- Age is biggest risk factor
- 1-2% prevalence at age 65
- Incidence doubles every 5 years after age of 60
- ## Diagnostic Checklist
- Look for reversible causes of dementia
# TERMINOLOGY
- ## Abbreviations
- Alzheimer disease (AD)
- ## Synonyms
- Senile/presenile dementia of Alzheimer type
- ## Definitions
- AD dementia is progressive neurodegenerative condition characterized by progressive cognitive decline, memory impairment, and adverse impact on activities of daily living
- National Institute on Aging and Alzheimer's Association (NIA-AA) 2011 workgroup recommendations
- Phases of AD pathophysiological processes
- Preclinical AD
- Mild cognitive impairment (MCI) in AD
- AD dementia
- AD is pathologic process reflected in specific postmortem histopathologic criteria, which is frequently but not necessarily associated with characteristic dementia syndrome
- Probable AD dementia: Clinical syndrome meeting core clinical criteria specified in NIA-AA workgroup report
- Possible AD dementia: Clinical syndrome meeting core clinical criteria for AD dementia in terms of nature of cognitive deficits for AD dementia, but either 1) has sudden onset of impairment or demonstrates insufficient historical detail or objective documentation of progression, or 2) has mixed etiological presentation due to evidence of vascular or Lewy body pathology
- MCI: Clinical syndrome meeting published core clinical criteria for MCI; generally agreed core features include 1) concern for change in cognition, 2) impairment in one or more cognitive domains, and 3) preservation of independence in functional activities, but 4) not demented
# IMAGING
- ## General Features
- ### Best diagnostic clue
- MR: Temporal/parietal cortical atrophy
- Disproportionate hippocampal volume loss
- FDG PET: Regional ↓ glucose metabolism
- Temporoparietal lobes, posterior cingulum
- **Current role of imaging in AD**
- Exclude other structural abnormalities
- Evaluate degree and location of atrophic changes
- Evaluate for metabolic abnormalities
- When structural abnormalities absent/uncharacteristic (i.e., early in disease course)
- Identify preclinical and MCI in AD for possible innovative therapy
- ## CT Findings
- ### NECT
- Screening to exclude potentially reversible or treatable causes of dementia
- Medial temporal lobe atrophy in early disease and generalized atrophy in late stages
- ## MR Findings
- Current role of MR
- Exclude other causes of dementia
- Identify region-specific patterns of brain volume loss
- Identify imaging markers of coexistent disease, such as amyloid angiopathy
- T1 to assess medial temporal atrophy score and atrophy patterns
- High resolution (MPRAGE/SPGR) for volumetric analysis
- Thinned gyri, widened sulci, and enlarged ventricles
- Medial temporal lobe disproportionately affected
- May help distinguish patients with MCI from normal elderly
- Average hippocampal volume reduction 20-25% in AD and 10-15% in MCI
- T2* GRE/SWI for microhemorrhages, amyloid angiopathy
- MRS
- ↓ NAA and ↑ mI in AD, even in early stage
- NAA:mI ratio relatively sensitive and highly specific in differentiating AD from normal elderly
- NAA:Cr ratio in posterior cingulate gyri and left occipital cortex predicts conversion of MCI to probable AD
- DTI: ↓ FA in multiple regions, especially superior longitudinal fasciculus and splenium
- Perfusion MR: ↓ rCBV in temporal, parietal regions
- ## Nuclear Medicine Findings
- F-18 FDG PET
- Early-stage AD
- ↓ metabolism in parietotemporal association cortices, posterior cingulate, and precuneus regions
- Most reliable early changes in posterior cingulate
- Moderate to severe AD
- Additional frontal lobe involvement
- MCI in AD
- Same pattern of ↓ metabolism as AD
- Higher accuracy than MR for diagnosing early AD
- Amyloid (Aβ) PET imaging
- Specifically bind to Aβ plaques and retention of tracer is specific for Aβ neuritic plaque pathology
- F-18 florbetapir, F-18 florbetaben, and F-18 flutemetamol FDA approved for clinical use
- Positive scan shows loss of gray/white matter distinction due to tracer uptake in neocortex
- Negative scan retains gray/white matter distinction
- Criteria for appropriate use (AUC) of amyloid PET
- Persistent/progressive unexplained MCI
- Possible AD with unclear presentation
- Atypical early-onset progressive dementia
- In patients with MCI fulfilling clinical AUC, Aβ-PET is associated with
- Significant improvement in diagnostic confidence
- High impact on therapeutic management
- Tau PET imaging
- Currently under development
- Signal matches anatomic distribution of neurofibrillary tangles
- Earliest detection in entorhinal cortex and hippocampus, later inferior and lateral temporal, followed by parietal and occipital, and finally frontal cortices
- ## Imaging Recommendations
- ### Best imaging tool
- Volumetric MR (MPRAGE/SPGR sequences)
- F-18 FDG PET
- Aβ PET for patients who meet AUC
- ### Protocol advice
- MPRAGE or SPGR for volumetric measurement
# DIFFERENTIAL DIAGNOSIS
- ## Causes of Reversible Dementia
- [Thiamine deficiency, vitamin B12 deficiency, hypothyroidism](/document/alcoholic-encephalopathy/88021852-b73d-4cdf-a719-dd4ae3231e45)
- Depression ("pseudodementia")
- [Normal-pressure hydrocephalus](/document/normal-pressure-hydrocephalus/ba3f857d-58de-4f21-8463-1631b4cb9972)
- [Mass lesions (chronic subdural hematoma, tumor, etc.)](/document/chronic-subdural-hematoma/cc7b52b4-c6a0-4b4e-ae8c-f05bfc5c5cb2)
- [Vascular Dementia](/document/vascular-dementia/f59dab57-c511-4369-8fcc-592421a4b8d1)
- 2nd most common dementia (15-30%)
- Parenchymal hyperintensities, focal atrophy (infarcts)
- [Frontotemporal Lobar Degeneration](/document/frontotemporal-lobar-degeneration/49510d0e-acf7-45cb-9eb1-53f8193b0b6d)
- Frontal &/or anterior temporal atrophy
- [Dementia With Lewy Bodies](/document/dementia-with-lewy-bodies/e8e46d1d-46d2-4e5a-880f-f025a84c5871)
- Hypometabolism of entire brain
- [Corticobasal Degeneration](/document/corticobasal-degeneration/23f97d4e-8724-4229-b9f8-08f63906ebd8)
- Prominent extrapyramidal, cortical symptoms
- Asymmetric severe frontoparietal atrophy
- [Creutzfeldt-Jakob Disease](/document/creutzfeldt-jakob-disease-cjd/e1b27954-6591-4bb0-a659-b13790492620)
- Dementia with myoclonus, EEG abnormalities
- Hyperintensity in anterior basal ganglia, cortex
- [Cerebral Amyloid Angiopathy](/document/cerebral-amyloid-disease/48e9458e-102f-40bd-8eec-bc0bc97101c6)
- Often coexists with AD
- Microhemorrhages on T2* GRE/SWI
# PATHOLOGY
- ## General Features
- ### Etiology
- Extracellular β-amyloid plaques
- Located in cerebral cortex
- Intracellular accumulation of neurofibrillary tangles (NTs)
- Initially around hippocampus, later spread to other cortical areas
- ### Genetics
- Most cases late-onset sporadic AD
- Deterministic genetic mutation not found
- Apolipoprotein E (*ApoE*) ε4 allele is major genetic risk factor
- Rare early-onset AD
- Mutations in 1 of 3 genes
- Amyloid precursor protein gene on chromosome 21
- Presenilin-1 (*PSEN1*) gene on chromosome 14
- Presenilin-2 (*PSEN2*) gene on chromosome 1
- ## Staging, Grading, & Classification
- Consortium to Establish a Registry for Alzheimer Disease (CERAD)
- Semiquantitative approach counting plaques/tangles
- Frequent, moderate, or infrequent
- Braak and Braak (B&B)
- 6 levels of staging
- Transentorhinal stage (1-2): NTs develop in parahippocampal gyrus (clinically asymptomatic)
- Limbic stage (3-4): NTs dramatically increase in parahippocampal gyrus, begin to develop in hippocampus (mild cognitive impairment)
- Neocortical stage (5-6): NTs develop in temporal and parietal cortex, eventually spread to entire neocortex (severe dementia)
- NIA-Reagan
- Likelihood high
- CERAD frequent, B&B 5/6
- Likelihood intermediate
- CERAD moderate, B&B 3/4
- Likelihood low
- CERAD infrequent, B&B 1/2
- ## Gross Pathologic & Surgical Features
- Shrunken gyri, widened sulci
- ## Microscopic Features
- 2 abnormal protein aggregates characterize AD pathologically
- Neurofibrillary tangles
- Intracellular aggregates in neurons due to hyperphosphorylation of tau protein
- Begins in entorhinal cortex, progresses to hippocampus, paralimbic system, and adjacent medial-basal temporal lobe
- Aβ deposition
- Hallmark of Aβ peptide deposit in AD is neuritic plaque
- Dense Aβ core with inflammatory cells and dystrophic neurites in its periphery
- Neurodegeneration: Synapse and neuron loss
# CLINICAL ISSUES
- ## Presentation
- ### Most common signs/symptoms
- Slowly progressive neurodegenerative disease
- Initially affects episodic memory
- Then, at least 1 other area of cognition
- ### Clinical profile
- Clinical subtypes
- MCI: Early, mild memory impairment; no deficits in cognitive domains other than memory, not impairing daily function
- Possible AD: Dementia features in presence of 2nd disease that could cause memory deficit but is not likely cause
- Probable AD: Memory deficits on neuropsychological testing, progressive worsening of memory and ≥ 2 cognitive functions
- Definite AD: Pathologic diagnosis
- 5 major biomarkers for AD
- Amyloid accumulation: CSF Aβ 42, Aβ-PET imaging
- Neurogeneration or neuronal injury: CSF tau (total and phosphorylated), structural MR, and FDG PET
- ## Demographics
- ### Age
- Biggest risk factor
- 1-2% prevalence at age 65
- Incidence doubles every 5 years after age of 60
- ### Sex
- Women more commonly affected
- ### Epidemiology
- AD most common neurodegenerative dementia
- 5-7 million new AD dementia cases every year
- Currently ~ 5.3 million in USA
- 13% of individuals > 65 years and > 50% of individuals > 85 years
- Other risk factors
- Family history (20%)
- Head trauma, metabolic syndrome
- ## Natural History & Prognosis
- Chronic, progressive
- Patients live average 8-10 years after diagnosis
- ## Treatment
- No established treatments
- May transiently improve cognitive function
- Cholinesterase inhibitors, NMDA receptor antagonists
- Many current disease-modifying drugs to reduce Aβ
# DIAGNOSTIC CHECKLIST
- ## Consider
- Look for
- Reversible causes of dementia
- Ventricular enlargement, sulcal widening proportionate
- ↑ temporal horns of lateral ventricle
- Hippocampal, entorhinal cortex volume loss
- ## Image Interpretation Pearls
- MR volumetric analysis helps distinguish MCI in AD from normal elderly subjects, measure change hippocampus/parahippocampal gyri over time
- F-18 FDG PET
- Helps distinguish AD from frontotemporal dementia
- May identify early AD when MR normal
5dea6eed-cbf2-4d32-8c2b-43f74913fca6
## References
# Selected References
1. [Chandra A et al: Applications of amyloid, tau, and neuroinflammation PET imaging to Alzheimer's disease and mild cognitive impairment. Hum Brain Mapp. ePub, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31520513%5Bpmid%5D)
1. [Huang Q et al: Three-dimensional pseudocontinuous arterial spin labeling and susceptibility-weighted imaging associated with clinical progression in amnestic mild cognitive impairment and Alzheimer's disease. Medicine (Baltimore). 98(23):e15972, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31169728%5Bpmid%5D)
1. [Matsuda H et al: Neuroimaging of Alzheimer's disease: focus on amyloid and tau PET. Jpn J Radiol. ePub, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31493197%5Bpmid%5D)
1. [Rabinovici GD et al: Association of Amyloid Positron Emission Tomography With Subsequent Change in Clinical Management Among Medicare Beneficiaries With Mild Cognitive Impairment or Dementia. JAMA. 321(13):1286-1294, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=30938796%5Bpmid%5D)
1. [Triviño-Ibáñez EM et al: Impact of amyloid-PET in daily clinical management of patients with cognitive impairment fulfilling appropriate use criteria. Medicine (Baltimore). 98(29):e16509, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31335725%5Bpmid%5D)
1. [Veitch DP et al: Understanding disease progression and improving Alzheimer's disease clinical trials: Recent highlights from the Alzheimer's Disease Neuroimaging Initiative. Alzheimers Dement. 15(1):106-152, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=30321505%5Bpmid%5D)
1. [Femminella GD et al: Imaging and Molecular Mechanisms of Alzheimer's Disease: A Review. Int J Mol Sci. 19(12), 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=30469491%5Bpmid%5D)
1. [Morley JE et al: Alzheimer Disease. Clin Geriatr Med. 34(4):591-601, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=30336989%5Bpmid%5D)
1. [Xia C et al: Multimodal PET Imaging of Amyloid and Tau Pathology in Alzheimer Disease and Non-Alzheimer Disease Dementias. PET Clin. 12(3):351-359, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=28576172%5Bpmid%5D)
1. [Brown RK et al: Brain PET in suspected dementia: patterns of altered FDG metabolism. Radiographics. 34(3):684-701, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=24819789%5Bpmid%5D)
1. [Nasrallah IM et al: Multimodality imaging of Alzheimer disease and other neurodegenerative dementias. J Nucl Med. 55(12):2003-11, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=25413136%5Bpmid%5D)
1. [Ishii K: PET Approaches for Diagnosis of Dementia. AJNR Am J Neuroradiol. Epub ahead of print, 2013](http://www.ncbi.nlm.nih.gov/pubmed/?term=23945233%5Bpmid%5D)
1. [Petrella JR: Neuroimaging and the search for a cure for Alzheimer disease. Radiology. 269(3):671-91, 2013](http://www.ncbi.nlm.nih.gov/pubmed/?term=24261497%5Bpmid%5D)
1. [Croisile B et al: [The new 2011 recommendations of the National Institute on Aging and the Alzheimer's Association on diagnostic guidelines for Alzheimer's disease: Preclinal stages, mild cognitive impairment, and dementia.] Rev Neurol (Paris). 168(6-7):471-82, 2012](http://www.ncbi.nlm.nih.gov/pubmed/?term=22579080%5Bpmid%5D)
1. [Jack CR Jr: Alzheimer disease: new concepts on its neurobiology and the clinical role imaging will play. Radiology. 263(2):344-61, 2012](http://www.ncbi.nlm.nih.gov/pubmed/?term=22517954%5Bpmid%5D)
1. [Albert MS et al: The diagnosis of mild cognitive impairment due to Alzheimer's disease: recommendations from the National Institute on Aging-Alzheimer's Association workgroups on diagnostic guidelines for Alzheimer's disease. Alzheimers Dement. 7(3):270-9, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=21514249%5Bpmid%5D)
1. [McKhann GM et al: The diagnosis of dementia due to Alzheimer's disease: recommendations from the National Institute on Aging-Alzheimer's Association workgroups on diagnostic guidelines for Alzheimer's disease. Alzheimers Dement. 7(3):263-9, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=21514250%5Bpmid%5D)
1. [Shen Q et al: Volumetric and visual rating of magnetic resonance imaging scans in the diagnosis of amnestic mild cognitive impairment and Alzheimer's disease. Alzheimers Dement. 7(4):e101-8, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=21784342%5Bpmid%5D)
1. [Sperling RA et al: Toward defining the preclinical stages of Alzheimer's disease: recommendations from the National Institute on Aging-Alzheimer's Association workgroups on diagnostic guidelines for Alzheimer's disease. Alzheimers Dement. 7(3):280-92, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=21514248%5Bpmid%5D)
1. [Dai W et al: Mild cognitive impairment and alzheimer disease: patterns of altered cerebral blood flow at MR imaging. Radiology. 250(3):856-66, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=19164119%5Bpmid%5D)
1. [McEvoy LK et al: Alzheimer disease: quantitative structural neuroimaging for detection and prediction of clinical and structural changes in mild cognitive impairment. Radiology. 251(1):195-205, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=19201945%5Bpmid%5D)
1. [Duara R et al: Medial temporal lobe atrophy on MRI scans and the diagnosis of Alzheimer disease. Neurology. 71(24):1986-92, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=19064880%5Bpmid%5D)
1. [Petrella JR et al: Cortical deactivation in mild cognitive impairment: high-field-strength functional MR imaging. Radiology. 2007 Oct;245(1):224-35. Erratum in: Radiology. 246(1):338, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=17885190%5Bpmid%5D)
1. [Norfray JF et al: Alzheimer's disease: neuropathologic findings and recent advances in imaging. AJR Am J Roentgenol. 182(1):3-13, 2004](http://www.ncbi.nlm.nih.gov/pubmed/?term=14684506%5Bpmid%5D)
## Images
### Selected Images
![Clinical examination of a 72-year-old woman with AD shows typical findings for long history of memory deficit. FDG PET raw data and 3D SSP show preservation of the primary sensorimotor cortex <img src='img/arrows/WC.png'/>. There is hypometabolism involving the bilateral parietotemporal association cortices <img src='img/arrows/WO.png'/> and mild hypometabolism within the right greater than left posterior cingulate regions <img src='img/arrows/WS.png'/> and, to a lesser extent, the precuneus regions <img src='img/arrows/CS.png'/>. There is no hypometabolism in the primary visual cortices or frontal lobes. (Courtesy M. Matesan MD.)](images/app.statdx.com_image_thumbnail_c1584b3b-5358-41b3-92ea-8416c9f03019_size_168_quality_85_15a74876_20251014T193423Z.jpg)
*Clinical examination of a 72-year-old woman with AD shows typical findings for long history of memory deficit. FDG PET raw data and 3D SSP show preservation of the primary sensorimotor cortex <img src='img/arrows/WC.png'/>. There is hypometabolism involving the bilateral parietotemporal association cortices <img src='img/arrows/WO.png'/> and mild hypometabolism within the right greater than left posterior cingulate regions <img src='img/arrows/WS.png'/> and, to a lesser extent, the precuneus regions <img src='img/arrows/CS.png'/>. There is no hypometabolism in the primary visual cortices or frontal lobes. (Courtesy M. Matesan MD.)*
![Clinical examination of a 72-year-old woman with AD shows typical findings for long history of memory deficit. FDG PET raw data and 3D SSP show preservation of the primary sensorimotor cortex <img src='img/arrows/WC.png'/>. There is hypometabolism involving the bilateral parietotemporal association cortices <img src='img/arrows/WO.png'/> and mild hypometabolism within the right greater than left posterior cingulate regions <img src='img/arrows/WS.png'/> and, to a lesser extent, the precuneus regions <img src='img/arrows/CS.png'/>. There is no hypometabolism in the primary visual cortices or frontal lobes. (Courtesy M. Matesan MD.)](images/app.statdx.com_image_thumbnail_c1584b3b-5358-41b3-92ea-8416c9f03019_size_174_quality_85_07154800_20251014T190917Z.jpg)
*Clinical examination of a 72-year-old woman with AD shows typical findings for long history of memory deficit. FDG PET raw data and 3D SSP show preservation of the primary sensorimotor cortex <img src='img/arrows/WC.png'/>. There is hypometabolism involving the bilateral parietotemporal association cortices <img src='img/arrows/WO.png'/> and mild hypometabolism within the right greater than left posterior cingulate regions <img src='img/arrows/WS.png'/> and, to a lesser extent, the precuneus regions <img src='img/arrows/CS.png'/>. There is no hypometabolism in the primary visual cortices or frontal lobes. (Courtesy M. Matesan MD.)*
![Clinical examination of a 72-year-old woman with AD shows typical findings for long history of memory deficit. FDG PET raw data and 3D SSP show preservation of the primary sensorimotor cortex <img src='img/arrows/WC.png'/>. There is hypometabolism involving the bilateral parietotemporal association cortices <img src='img/arrows/WO.png'/> and mild hypometabolism within the right greater than left posterior cingulate regions <img src='img/arrows/WS.png'/> and, to a lesser extent, the precuneus regions <img src='img/arrows/CS.png'/>. There is no hypometabolism in the primary visual cortices or frontal lobes. (Courtesy M. Matesan MD.)](images/app.statdx.com_image_thumbnail_c1584b3b-5358-41b3-92ea-8416c9f03019_size_174_quality_85_16a43726_20251014T185333Z.jpg)
*Clinical examination of a 72-year-old woman with AD shows typical findings for long history of memory deficit. FDG PET raw data and 3D SSP show preservation of the primary sensorimotor cortex <img src='img/arrows/WC.png'/>. There is hypometabolism involving the bilateral parietotemporal association cortices <img src='img/arrows/WO.png'/> and mild hypometabolism within the right greater than left posterior cingulate regions <img src='img/arrows/WS.png'/> and, to a lesser extent, the precuneus regions <img src='img/arrows/CS.png'/>. There is no hypometabolism in the primary visual cortices or frontal lobes. (Courtesy M. Matesan MD.)*
![Clinical examination of a 72-year-old woman with AD shows typical findings for long history of memory deficit. FDG PET raw data and 3D SSP show preservation of the primary sensorimotor cortex <img src='img/arrows/WC.png'/>. There is hypometabolism involving the bilateral parietotemporal association cortices <img src='img/arrows/WO.png'/> and mild hypometabolism within the right greater than left posterior cingulate regions <img src='img/arrows/WS.png'/> and, to a lesser extent, the precuneus regions <img src='img/arrows/CS.png'/>. There is no hypometabolism in the primary visual cortices or frontal lobes. (Courtesy M. Matesan MD.)](images/app.statdx.com_image_thumbnail_c1584b3b-5358-41b3-92ea-8416c9f03019_size_174_quality_85_327b9188_20251014T204456Z.jpg)
*Clinical examination of a 72-year-old woman with AD shows typical findings for long history of memory deficit. FDG PET raw data and 3D SSP show preservation of the primary sensorimotor cortex <img src='img/arrows/WC.png'/>. There is hypometabolism involving the bilateral parietotemporal association cortices <img src='img/arrows/WO.png'/> and mild hypometabolism within the right greater than left posterior cingulate regions <img src='img/arrows/WS.png'/> and, to a lesser extent, the precuneus regions <img src='img/arrows/CS.png'/>. There is no hypometabolism in the primary visual cortices or frontal lobes. (Courtesy M. Matesan MD.)*
![Clinical examination of a 72-year-old woman with AD shows typical findings for long history of memory deficit. FDG PET raw data and 3D SSP show preservation of the primary sensorimotor cortex <img src='img/arrows/WC.png'/>. There is hypometabolism involving the bilateral parietotemporal association cortices <img src='img/arrows/WO.png'/> and mild hypometabolism within the right greater than left posterior cingulate regions <img src='img/arrows/WS.png'/> and, to a lesser extent, the precuneus regions <img src='img/arrows/CS.png'/>. There is no hypometabolism in the primary visual cortices or frontal lobes. (Courtesy M. Matesan MD.)](images/app.statdx.com_image_thumbnail_c1584b3b-5358-41b3-92ea-8416c9f03019_size_174_quality_85_ea21372e_20251014T193347Z.jpg)
*Clinical examination of a 72-year-old woman with AD shows typical findings for long history of memory deficit. FDG PET raw data and 3D SSP show preservation of the primary sensorimotor cortex <img src='img/arrows/WC.png'/>. There is hypometabolism involving the bilateral parietotemporal association cortices <img src='img/arrows/WO.png'/> and mild hypometabolism within the right greater than left posterior cingulate regions <img src='img/arrows/WS.png'/> and, to a lesser extent, the precuneus regions <img src='img/arrows/CS.png'/>. There is no hypometabolism in the primary visual cortices or frontal lobes. (Courtesy M. Matesan MD.)*
![Volumetric MR in a patient FDG PET positive for hypometabolic areas in bilateral temporoparietal lobes, posterior cingulate, and precuneus regions is shown. Bilateral hippocampi and lateral ventricle volumes are in normal range, indicating that FDG PET is more specific than quantitative MR in early AD. (Courtesy M. Matesan, MD.)](images/app.statdx.com_image_thumbnail_0d48bff9-d0c5-439b-834b-ecddfb1196ec_size_168_quality_85_1ee6bff4_20251014T193423Z.jpg)
*Volumetric MR in a patient FDG PET positive for hypometabolic areas in bilateral temporoparietal lobes, posterior cingulate, and precuneus regions is shown. Bilateral hippocampi and lateral ventricle volumes are in normal range, indicating that FDG PET is more specific than quantitative MR in early AD. (Courtesy M. Matesan, MD.)*
![Axial T2 MR in a patient with AD shows enlarged temporal horns <img src='img/arrows/CC.png'/> and disproportionate volume loss in the temporal lobes <img src='img/arrows/CS.png'/> as compared to the normal-appearing occipital lobes <img src='img/arrows/CO.png'/>.](images/app.statdx.com_image_thumbnail_93f64ebd-db31-4bcc-aa65-a661bde91ddd_size_168_quality_85_1cc0997e_20251014T193423Z.jpg)
*Axial T2 MR in a patient with AD shows enlarged temporal horns <img src='img/arrows/CC.png'/> and disproportionate volume loss in the temporal lobes <img src='img/arrows/CS.png'/> as compared to the normal-appearing occipital lobes <img src='img/arrows/CO.png'/>.*
![F-18 AV-45 (florbetapir) PET in a healthy control (left) shows nonspecific white matter uptake <img src='img/arrows/WS.png'/> and preserved gray matter-white matter differentiation. In a patient with AD (right), there is marked cerebral gray matter uptake <img src='img/arrows/WC.png'/> (Aβ deposition) with loss of gray matter-white matter differentiation. (Courtesy A. Ali, MD.)](images/app.statdx.com_image_thumbnail_8254ff44-ef28-4dfd-84ee-d44a781c6e05_size_168_quality_85_af27cd62_20251014T193423Z.jpg)
*F-18 AV-45 (florbetapir) PET in a healthy control (left) shows nonspecific white matter uptake <img src='img/arrows/WS.png'/> and preserved gray matter-white matter differentiation. In a patient with AD (right), there is marked cerebral gray matter uptake <img src='img/arrows/WC.png'/> (Aβ deposition) with loss of gray matter-white matter differentiation. (Courtesy A. Ali, MD.)*
![Tc-99m HMPAO SPECT in a 67-year-old woman with suspected AD shows decreased perfusion in bilateral parietal lobes <img src='img/arrows/WS.png'/>. (Courtesy J. Singh, MD.)](images/app.statdx.com_image_thumbnail_976f167f-70b4-400a-bf55-6fba2cf83920_size_168_quality_85_e80e3ef2_20251014T193423Z.jpg)
*Tc-99m HMPAO SPECT in a 67-year-old woman with suspected AD shows decreased perfusion in bilateral parietal lobes <img src='img/arrows/WS.png'/>. (Courtesy J. Singh, MD.)*
![Coronal FDG PET in a 45 year old with slowly progressive dementia shows decreased metabolic activity in bilateral medial temporal lobes <img src='img/arrows/WS.png'/>, slightly greater on the right.](images/app.statdx.com_image_thumbnail_bad4934b-e24b-4178-9341-ac8e012dedf2_size_168_quality_85_7ba2e461_20251014T193423Z.jpg)
*Coronal FDG PET in a 45 year old with slowly progressive dementia shows decreased metabolic activity in bilateral medial temporal lobes <img src='img/arrows/WS.png'/>, slightly greater on the right.*
![Axial FDG PET in the same patient shows decreased metabolic activity in the left parietal lobe <img src='img/arrows/WS.png'/>. Also note mild decreased metabolic activity in bilateral frontal lobes <img src='img/arrows/WO.png'/>, indicating possible advanced disease. (Courtesy J. Singh, MD.)](images/app.statdx.com_image_thumbnail_a41dd265-0d11-4022-830b-517a4b6489bb_size_168_quality_85_ac4e4eb3_20251014T193423Z.jpg)
*Axial FDG PET in the same patient shows decreased metabolic activity in the left parietal lobe <img src='img/arrows/WS.png'/>. Also note mild decreased metabolic activity in bilateral frontal lobes <img src='img/arrows/WO.png'/>, indicating possible advanced disease. (Courtesy J. Singh, MD.)*
### Additional Images
![Axial T2WI MR through the lateral ventricles depicts atrophy of the superior aspect of the temporal lobes and parietal lobe atrophy. (Courtesy J. Norfray, MD.)](images/app.statdx.com_image_thumbnail_c25bdb1c-d362-46f8-b57c-a272df71bbac_size_168_quality_85_4c9da12f_20251014T193423Z.jpg)
*Axial T2WI MR through the lateral ventricles depicts atrophy of the superior aspect of the temporal lobes and parietal lobe atrophy. (Courtesy J. Norfray, MD.)*
![Coronal T2WI MR through the temporal lobes depicts marked atrophy of the hippocampi. (Courtesy J. Norfray, MD.)](images/app.statdx.com_image_thumbnail_1d3fc0c2-926b-4dd6-b99d-e019cb4d650b_size_168_quality_85_fcf97ef6_20251014T193424Z.jpg)
*Coronal T2WI MR through the temporal lobes depicts marked atrophy of the hippocampi. (Courtesy J. Norfray, MD.)*
![Axial T1WI MR through the inferior temporal lobes shows marked atrophy of the temporal lobes and enlargement of the lateral ventricles.](images/app.statdx.com_image_thumbnail_85582fa2-45dd-4266-9c48-6b9a0aaf6543_size_168_quality_85_93348f32_20251014T193424Z.jpg)
*Axial T1WI MR through the inferior temporal lobes shows marked atrophy of the temporal lobes and enlargement of the lateral ventricles.*
![Axial FLAIR MR shows marked atrophy of the temporal lobes and enlargement of the sylvian fissures.](images/app.statdx.com_image_thumbnail_e0ceba9d-43b7-473b-a23a-b52f5097221c_size_168_quality_85_adde319c_20251014T193424Z.jpg)
*Axial FLAIR MR shows marked atrophy of the temporal lobes and enlargement of the sylvian fissures.*
![Axial T2WI MR through the inferior temporal lobes shows marked atrophy of the temporal lobes and enlargement of the parahippocampal fissures. (Courtesy J. Norfray, MD.)](images/app.statdx.com_image_thumbnail_9e9a07d3-235d-44c8-a6e8-5a7f3aaa6d7a_size_168_quality_85_43c72e02_20251014T193424Z.jpg)
*Axial T2WI MR through the inferior temporal lobes shows marked atrophy of the temporal lobes and enlargement of the parahippocampal fissures. (Courtesy J. Norfray, MD.)*
![MRS spectrum in the parietal lobe of a patient with probable AD shows decreased N-acetyl aspartate level (neuronal loss) and elevated myoinositol level (gliosis). (Courtesy J. Norfray, MD.)](images/app.statdx.com_image_thumbnail_fbe76f72-f2de-42c9-9785-85b8a391630d_size_168_quality_85_98215ff8_20251014T193424Z.jpg)
*MRS spectrum in the parietal lobe of a patient with probable AD shows decreased N-acetyl aspartate level (neuronal loss) and elevated myoinositol level (gliosis). (Courtesy J. Norfray, MD.)*
![FDG PET in a patient with dementia depicts hypometabolism (green and blue regions in cortex) in both parietal lobes, typical of AD. (Courtesy N. Foster, MD.)](images/app.statdx.com_image_thumbnail_4be7259e-3105-4d36-91e6-dff9bd8ef247_size_168_quality_85_cb931494_20251014T193424Z.jpg)
*FDG PET in a patient with dementia depicts hypometabolism (green and blue regions in cortex) in both parietal lobes, typical of AD. (Courtesy N. Foster, MD.)*
![FDG PET in the same patient shows a decreased rate of glucose metabolism in the posterior aspects of both temporal lobes, typical of AD. (Courtesy N. Foster, MD.)](images/app.statdx.com_image_thumbnail_d730dbd4-f56c-4a03-b65f-dfd63f3f9ead_size_168_quality_85_7f726d7f_20251014T193424Z.jpg)
*FDG PET in the same patient shows a decreased rate of glucose metabolism in the posterior aspects of both temporal lobes, typical of AD. (Courtesy N. Foster, MD.)*
![Stereotaxic surface projection in AD shows mild hypometabolism in the temporal lobes and especially diminished in the parietal lobes <img src='img/arrows/WS.png'/>. The Z-score map demonstrates the hypometabolism <img src='img/arrows/WO.png'/> very well. (Courtesy N. Foster, MD.)](images/app.statdx.com_image_thumbnail_d701f9c5-1815-4dcb-bb62-9cacdad3c821_size_168_quality_85_821d7380_20251014T193424Z.jpg)
*Stereotaxic surface projection in AD shows mild hypometabolism in the temporal lobes and especially diminished in the parietal lobes <img src='img/arrows/WS.png'/>. The Z-score map demonstrates the hypometabolism <img src='img/arrows/WO.png'/> very well. (Courtesy N. Foster, MD.)*
![Axial NECT in a 59 year old with AD shows hippocampal atrophy is present, as evidenced by temporal horn enlargement <img src='img/arrows/CS.png'/>. Both sylvian fissures are also very prominent. There was no evidence for intracranial mass lesion or ischemia/infarction.](images/app.statdx.com_image_thumbnail_6ad441d4-70ca-45d2-9db9-1a321d982610_size_168_quality_85_c6983cc2_20251014T193424Z.jpg)
*Axial NECT in a 59 year old with AD shows hippocampal atrophy is present, as evidenced by temporal horn enlargement <img src='img/arrows/CS.png'/>. Both sylvian fissures are also very prominent. There was no evidence for intracranial mass lesion or ischemia/infarction.*
![Axial amyloid β PET using florbetapir in a 63-year-old man with slowly progressive dementia shows moderate amyloid neuritic plaque burden <img src='img/arrows/CS.png'/> indicating AD.](images/app.statdx.com_image_thumbnail_d0383595-2945-45ac-95d1-bc5458fe2bd7_size_168_quality_85_2b9ac007_20251014T193424Z.jpg)
*Axial amyloid β PET using florbetapir in a 63-year-old man with slowly progressive dementia shows moderate amyloid neuritic plaque burden <img src='img/arrows/CS.png'/> indicating AD.*
![Sagittal PET in early AD shows a classic pattern of hypometabolism in the posterior cingulate gyrus and precuneus <img src='img/arrows/CC.png'/>. (Courtesy S. Nayak, MD.)](images/app.statdx.com_image_thumbnail_9d70b838-9e8e-4c0b-b7d5-7bad4a5389d0_size_168_quality_85_e6eec88e_20251014T193424Z.jpg)
*Sagittal PET in early AD shows a classic pattern of hypometabolism in the posterior cingulate gyrus and precuneus <img src='img/arrows/CC.png'/>. (Courtesy S. Nayak, MD.)*
![Axial graphic illustrates the presence of amyloid plaques in the cerebral gray matter <img src='img/arrows/WC.png'/>.](images/app.statdx.com_image_thumbnail_6ee0ce31-8e3f-4179-a8b3-9f799267ec43_size_168_quality_85_28cf246a_20251014T193424Z.jpg)
*Axial graphic illustrates the presence of amyloid plaques in the cerebral gray matter <img src='img/arrows/WC.png'/>.*
![Axial FDG PET in a patient with AD shows decreased glucose metabolism in both medial temporal lobes <img src='img/arrows/WO.png'/>. Note the preserved glucose metabolism in the cerebellum and frontal lobes.](images/app.statdx.com_image_thumbnail_3c1c9f70-05c8-4064-897b-da80f9f86de1_size_168_quality_85_c9456adc_20251014T193424Z.jpg)
*Axial FDG PET in a patient with AD shows decreased glucose metabolism in both medial temporal lobes <img src='img/arrows/WO.png'/>. Note the preserved glucose metabolism in the cerebellum and frontal lobes.*
![Axial FDG PET in the same patient shows decreased metabolism in both parietal lobes <img src='img/arrows/WO.png'/>. Hypometabolism within both the temporal and parietal cortices is typical of AD.](images/app.statdx.com_image_thumbnail_0ce9ea7c-25be-4872-ae62-06673e0bb501_size_168_quality_85_cee4d1ab_20251014T193424Z.jpg)
*Axial FDG PET in the same patient shows decreased metabolism in both parietal lobes <img src='img/arrows/WO.png'/>. Hypometabolism within both the temporal and parietal cortices is typical of AD.*
![Sagittal T1WI MR in a 72-year-old patient with suspected AD shows marked enlargement of the sylvian fissure <img src='img/arrows/CS.png'/> compared to the other subarachnoid spaces. Cortical atrophy of structures around the sylvian fissures can be striking, as in this case.](images/app.statdx.com_image_thumbnail_c01a9642-4155-48c0-b728-3b4d5ce322fd_size_168_quality_85_e3b07b79_20251014T193424Z.jpg)
*Sagittal T1WI MR in a 72-year-old patient with suspected AD shows marked enlargement of the sylvian fissure <img src='img/arrows/CS.png'/> compared to the other subarachnoid spaces. Cortical atrophy of structures around the sylvian fissures can be striking, as in this case.*
![Axial T1WI MR in the same patient 3 years later shows more prominent medial temporal lobe atrophy as well as enlargement of parahippocampal fissures. In addition, diffuse cerebral atrophy has progressed further. The patient was diagnosed as probable AD.](images/app.statdx.com_image_thumbnail_0a242846-161d-4f12-9d33-928ba2ec661a_size_168_quality_85_7b4a24e7_20251014T193424Z.jpg)
*Axial T1WI MR in the same patient 3 years later shows more prominent medial temporal lobe atrophy as well as enlargement of parahippocampal fissures. In addition, diffuse cerebral atrophy has progressed further. The patient was diagnosed as probable AD.*
docs_md/articles/amyotrophic-lateral-sclerosis-als_23de52b7-d9bd-441c-a18c-95c8afccb470.md
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---
title: "Amyotrophic Lateral Sclerosis (ALS)"
docid: "23de52b7-d9bd-441c-a18c-95c8afccb470"
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value: "Santhosh Gaddikeri, MD"
- key: "a25c450b-3d34-4f64-bba3-cc0834813df6"
value: "Miral D. Jhaveri, MD, MBA"
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name: "Amyotrophic Lateral Sclerosis (ALS)"
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pageDescription: "Amyotrophic Lateral Sclerosis (ALS)"
pageKeywords: "Brain, Diagnosis, Pathology-Based Diagnoses, Acquired Toxic/Metabolic/Degenerative Disorders, Dementias and Degenerative Disorders, Amyotrophic Lateral Sclerosis (ALS)"
pageTitle: "Amyotrophic Lateral Sclerosis (ALS) | STATdx"
enhancedTitle: "Amyotrophic Lateral Sclerosis (ALS)"
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breadcrumbs:
- "Brain"
- "Diagnosis"
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- "Amyotrophic Lateral Sclerosis (ALS)"
---
# KEY FACTS
- ## Terminology
- Amyotrophic lateral sclerosis (ALS)
- Selective degeneration of somatic motor neurons of brainstem/spinal cord & large pyramidal neurons of motor cortex
- Eventual loss of corticospinal tract (CST) fibers
- ## Imaging
- Small percentage demonstrate CST hyperintensity
- As CST is normally slightly hyperintense, especially at 3.0 T, this finding lacks sensitivity & specificity
- T2-hyperintense CST may be specific for ALS when seen on corresponding PD images
- Consider FLAIR & PD in suspected ALS
- DWI hyperintensity (↓ diffusivity) in CST
- Hypointense gray matter in precentral gyrus (motor cortex)
- ## Top Differential Diagnoses
- Primary lateral sclerosis
- Wallerian degeneration
- Hypertrophic olivary degeneration
- Metabolic diseases involving bilateral CSTs
- Demyelinating & inflammatory diseases
- Neoplasms: Brainstem glioma, malignant lymphoma
- CST can appear hyperintense on 3T MR normally
- ## Pathology
- Majority of ALS cases are sALS
- 5-10% are familial (fALS)
- ## Clinical Issues
- UMN signs: Babinski sign, spasticity, hyperreflexia
- LMN signs: Asymmetric muscle weakness, atrophy, fasciculations, hyporeflexia
- Bulbar signs: Slurred speech, dysphagia
- Classic ALS: Both UMN & LMN affected
- Peaks in 6th-8th decades of life
- Complete disability & death within 1 decade
- Some patients with familial, juvenile-onset ALS survive for longer periods (2-3 decades)
# TERMINOLOGY
- ## Abbreviations
- Amyotrophic lateral sclerosis (ALS)
- ## Synonyms
- Lou Gehrig disease, motor neuron disease (MND)
- ## Definitions
- Selective degeneration of somatic motor neurons of brainstem/spinal cord {lower motor neurons (LMN) & large pyramidal neurons of motor cortex [upper motor neurons (UMN)]}
- Eventual loss of corticospinal tract (CST) fibers
# IMAGING
- ## General Features
- ### Best diagnostic clue
- Bilateral hyperintensities along CST extending from corona radiata to brainstem on T2WI/PD/FLAIR
- ### Location
- Hallmark is CST & LMN degeneration
- LMN in anterior horn of spinal cord & brainstem
- Corticospinal UMN in precentral gyrus (motor cortex)
- White matter (WM) & gray matter (GM)
- Frequently, prefrontal motor neurons involved in planning or orchestrating work of UMN & LMN
- ### Size
- Atrophy of motor system, particularly pyramidal tract, in advanced stages of ALS
- ### Morphology
- Oval or thin, curvilinear hyperintensities conforming to CST
- ## CT Findings
- ### NECT
- Serial CT exams may show progressive atrophy
- Frontal, anterior temporal lobes → precentral gyrus → postcentral gyrus, anterior cingulate gyrus, corpus callosum, tegmentum
- ## MR Findings
- ### T1WI
- Different T1 appearances of CST
- Isointensity (most common) may reflect ↑ content of free radicals
- Hypointense or mild hyperintense signal
- CST differs between ALS patients & normal subjects only at internal capsule
- T1 hyperintensity in anterolateral cervical cord is associated with younger patients & rapid disease progression
- ### T2WI
- Hyperintense CST
- Hyperintensity can occur anywhere from subcortical WM of precentral gyrus to posterior limb internal capsules, cerebral peduncles, & pons
- As CST is normally slightly hyperintense especially at 3.0 T, this finding lacks sensitivity & specificity
- T2 hyperintense CST may be specific for ALS when seen on corresponding PD images
- Hypointense GM in precentral gyrus (motor cortex)
- Nonspecific; may be due to iron & heavy metals accumulating in cortex of aged patients
- ### PD/intermediate
- Hyperintense CST
- ### FLAIR
- More sensitive & less specific than T2 FSE for detecting hypointensity in precentral gyrus
- Hyperintense CST
- More frequently seen on FLAIR than on T2/T1/PD
- ### T2* GRE
- Hypointensity in precentral gyri
- ### DWI
- Hyperintensity in CST
- May be seen in absence of T2 hyperintensity
- Diffusion tensor imaging (DTI)
- ROI-based approaches & tractography demonstrates significant changes in diffusion parameters along CST
- Most common finding: ↓ fractional anisotropy (FA) in CST due to UMN degeneration
- ↓ FA in CST; most significant in posterior limb internal capsule & correlates with disease severity
- ↑ mean diffusivity (MD) along CST which correlates with disease duration
- ↑ MD in corpus callosum, frontal & temporal WM
- ↓ FA & ↑ MD in cervical cord correlate with disease severity & duration respectively
- ¹H-MRS useful for assessing UMN involvement
- ↓ NAA, ↓ NAA/Cr, ↓ NAA/Cho, & ↓ NAA/(Cr + Cho) in motor cortex
- NAA present primarily in neurons; these metabolic changes reflect loss or dysfunction of motor neurons
- ↓ NAA/Cr:NAA/Cho ratio along CST; most pronounced in precentral gyrus & corona radiata
- ↓ NAA in pons & upper medulla in patients with prominent UMN or bulbar signs
- ↑ Cho in posterior limb internal capsule
- ↑ myo-inositol (mI) in motor cortex
- ↓ NAA:mI ratio has better sensitivity & specificity in detecting ALS than any other metabolites
- Magnetization-transfer ratio (MTR) measurements
- ↓ MTR in CST
- CST hyperintensity on T1 MT contrast-enhanced images: 80% sensitivity, 100% specificity
- May detect CST degeneration of ALS at early stage
- Voxel-based morphometry (VBM)
- Regional GM loss in motor cortex, frontal, temporal, parietal, & limbic regions
- Frontal severe atrophy in ALS & frontotemporal dementia
- WM loss in corpus callosum, cerebellum, frontotemporal, & occipital regions
- ↓ Brain parenchymal fraction (BPF) & very mild global brain atrophy
- Functional MR
- Pattern of cortical reorganization
- ↑ activation of contralateral sensorimotor cortex, supplementary motor area, basal ganglia, & cerebellum during motor tasks
- ## Nuclear Medicine Findings
- PET, Tc-99m HMPAO SPECT
- ↓ regional cerebral metabolism/perfusion throughout brain with marked changes in sensorimotor cortex & putamen
- ↑ ALS severity correlated with ↓ GM perfusion
- ## Imaging Recommendations
- Best imaging tool: MR with T2, PD, FLAIR, DTI
# DIFFERENTIAL DIAGNOSIS
- ## Primary Lateral Sclerosis
- Neurodegeneration restricted to UMN
- T2WI shows changes in motor pathways
- *ALS2* mutations reported
- Autosomal recessive disease with juvenile onset
- Infantile-onset hereditary spastic paraplegia
- Spastic paralysis with ascending progression, only UMN involvement
- Mutations described in alsin & spastin genes
- [Wallerian Degeneration](/document/wallerian-degeneration/e4bb682d-6534-4176-9d39-34c1a42f3771)
- Dynamic signal intensities change along CST in patients with various cortical/subcortical lesions
- [Hypertrophic Olivary Degeneration](/document/hypertrophic-olivary-degeneration/78257543-6d52-4879-84b1-445f3611d996)
- Secondary degeneration of inferior olivary nucleus (ION), usually caused by primary lesions in dento-rubro-olivary pathway
- ## Conditions With T2-Hyperintense Lesions Along CST
- Metabolic diseases may involve CST bilaterally
- [X-linked adrenoleukodystrophy, Wilson disease](/document/wilson-disease/3d4d4876-4ce4-4af0-9e75-1a419bdd813c)
- Hypoglycemic coma: Reversible CST changes
- [Demyelinating & inflammatory diseases](/document/adem/a3fafeb7-5861-4364-beb8-c0e30220564e)
- [Multiple sclerosis, ADEM, Behçet disease, AIDS, cervical myelopathy](/document/behet-disease/4e447bb6-0f14-40e1-929a-4c1465feec0a)
- Neoplasms: Brainstem glioma, malignant lymphoma
- [Intoxication: Heroin inhalation](/document/drug-abuse/e4502a67-4b96-4d98-a167-6e90f6b65faf)
- ## Normal Individuals
- CST can appear hyperintense on 3T MR (normal fully myelinated brain at any age) & mimic ALS
# PATHOLOGY
- ## General Features
- ### Etiology
- Sporadic ALS (sALS) etiology is largely unknown
- Proposed potential mechanisms include
- Abnormal RNA processing, SOD1-mediated toxicity, excitotoxicity, cytoskeletal derangements, mitochondrial dysfunction
- Viral infections, apoptosis, growth factor abnormalities, & inflammatory responses
- Pathological hallmarks include loss of MNs with intraneuronal ubiquitin-immunoreactive inclusions in UMN & TDP-43 immunoreactive inclusions in degenerating LMN
- ↑ expression of cyclooxygenase-2 in spinal cord, frontal cortex, & hippocampus
- Apoptosis, free radical-mediated oxidative stress, excessive glutamate-mediated excitotoxicity
- Dopamine deficiency probably has important role
- Biochemical studies have shown ↓ glutamate levels in CNS tissue & ↑ levels in CSF
- Mutations in single gene can lead to selective degeneration of motor neurons
- ### Genetics
- 90-95% of cases are sALS
- 5-10% of cases are familial (fALS)
- Most common ALS-related gene mutation occurs in *C9ORF72*, *SOD1*, *TARDBP*, & *FUS*
- In European population: Most common is *C9ORF72*repeats
- In Asian population: Most common is *SOD1* mutation
- Rare autosomal recessive juvenile-onset ALS
- *ALS2* gene on chromosome 2q encodes alsin
- ### Associated abnormalities
- ALS-plus syndrome: MND with other symptoms or signs outside of voluntary motor system
- 2–3% of cases, ALS is accompanied by frontotemporal dementia
- ~ 50% of cases show cognitive impairment
- Can be associated with frontotemporal dementia (FTD), autonomic insufficiency, parkinsonism, supranuclear gaze paresis, &/or sensory loss
- ALS-like MND can occur as paraneoplastic syndrome
- ## Gross Pathologic & Surgical Features
- Focal atrophy of motor cortex & along course of CST
- Atrophy of anterior & lateral portions of spinal cord
- ## Microscopic Features
- Loss of cortical motor neurons (pyramidal & Betz cells) & astrocytosis
- Retrograde axonal loss & gliosis in CST
- "Senescent changes" with lipofuscin pigment atrophy
- Various cytoplasmic inclusions with chromatolysis
- Proximal & distal axonopathy with axonal spheroids
- Surviving motor neurons are smaller & abnormal
- Frequently undetected CST pathology in progressive muscular atrophy variant of ALS
- Bunina bodies (eosinophilic aggregates are positive for cystatin C) are unique for ALS
- Other intracellular inclusions: Neurofilament inclusions, ubiquitinated inclusions, TDP-43 inclusions, & immunoreactive inclusions to*FUS*
# CLINICAL ISSUES
- ## Presentation
- ### Most common signs/symptoms
- UMN signs: Babinski sign, spasticity, hyperreflexia
- LMN signs: Asymmetric muscle weakness, atrophy, fasciculations, hyporeflexia
- Split-hand syndrome: Frequent pattern of weakness & atrophy in ALS involving thenar muscles more than hypothenar muscles
- Bulbar signs: Slurred speech, dysphagia
- Difficulty walking, unexplained weight loss
- Hypoxia, cardiac arrhythmia
- ### Other signs/symptoms
- El Escorial criteria diagnosis of ALS: Evidence of UMN findings, LMN findings, & progression
- 4 regions or levels: Bulbar, cervical, thoracic, lumbosacral
- ### Clinical profile
- Classic ALS: Both UMN & LMN affected
- UMN-dominant ALS can be difficult to distinguish from primary lateral sclerosis
- Predominantly bulbar form usually leads to more rapid deterioration & death
- fALS associated with *SOD1* abnormality has mean age at 42 years limb onset, slow evolution
- ## Demographics
- ### Age
- Peaks in 6th to 8th decades; can occur in young adults
- ### Sex
- M:F = 1.5:1.0
- ### Ethnicity
- Slightly higher in Non-Hispanics white population
- ### Epidemiology
- Incidence: 1-3 cases/100,000 people
- Prevalence: 2.7-7.4 cases/100,000 people
- ## Natural History & Prognosis
- Progressive (distal to proximal)
- Median survival from diagnosis to death: 3-4 years
- 10% of patients survive > 10 years
- Some patients with familial, juvenile-onset ALS survive for longer periods (2-3 decades)
- ## Treatment
- Riluzole (glutamate release inhibitor & insulin-like growth factor) may prolong survival
- ↑ NAA/Cr in precentral gyrus & supplementary motor area after riluzole therapy
- This suggests population of sublethally injured, metabolically compromised neurons that are amenable to therapeutic rescue
- No improvement in perirolandic neuronal integrity (no change in NAA:Cr ratio) was detected after gabapentin treatment, which agrees with equivocal clinical effectiveness
- Baclofen, dantrolene, or diazepam for spasticity
# DIAGNOSTIC CHECKLIST
- ## Consider
- FLAIR & PD MR in all patients with suspected ALS
- ## Image Interpretation Pearls
- High T2 signal intensity in posterior limb of IC is suggestive for ALS when also visible on PD MR
- T1- & PD-weighted images differentiate real degeneration from normal areas
- DTI can assess CST lesions before pyramidal symptoms
c693ffa3-6052-4eb1-8840-2ce1ab8bebf8
## References
# Selected References
1. [Braun N et al: The revised El Escorial criteria "clinically probable laboratory supported ALS"-once a promising now a superfluous category? Amyotroph Lateral Scler Frontotemporal Degener. 1-5, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31561715%5Bpmid%5D)
1. [Lai JD et al: C9ORF72 protein function and immune dysregulation in amyotrophic lateral sclerosis. Neurosci Lett. 134523, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31568865%5Bpmid%5D)
1. [Barthel H et al: PET/MR in dementia and other neurodegenerative diseases. Semin Nucl Med. 45(3):224-33, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25841277%5Bpmid%5D)
1. [Goveas J et al: Diffusion-MRI in neurodegenerative disorders. Magn Reson Imaging. 33(7):853-76, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25917917%5Bpmid%5D)
1. [Verstraete E et al: Neuroimaging as a new diagnostic modality in amyotrophic lateral sclerosis. Neurotherapeutics. 12(2):403-16, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25791072%5Bpmid%5D)
1. [Chiò A et al: Neuroimaging in amyotrophic lateral sclerosis: insights into structural and functional changes. Lancet Neurol. 13(12):1228-40, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=25453462%5Bpmid%5D)
1. [Vucic S et al: Advances in treating amyotrophic lateral sclerosis: insights from pathophysiological studies. Trends Neurosci. 37(8):433-42, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=24927875%5Bpmid%5D)
1. [Wang S et al: Neuroimaging in amyotrophic lateral sclerosis. Neurotherapeutics. 8(1):63-71, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=21274686%5Bpmid%5D)
1. [Filippi M et al: EFNS guidelines on the use of neuroimaging in the management of motor neuron diseases. Eur J Neurol. 17(4):526-e20, 2010](http://www.ncbi.nlm.nih.gov/pubmed/?term=20136647%5Bpmid%5D)
1. [Wijesekera LC et al: Amyotrophic lateral sclerosis. Orphanet J Rare Dis. 4:3, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=19192301%5Bpmid%5D)
1. [Sage CA et al: Quantitative diffusion tensor imaging in amyotrophic lateral sclerosis. Neuroimage. 34(2):486-99, 2007](http://www.ncbi.nlm.nih.gov/pubmed/?term=17097892%5Bpmid%5D)
1. [Unrath A et al: Brain metabolites in definite amyotrophic lateral sclerosis. A longitudinal proton magnetic resonance spectroscopy study. J Neurol. 254(8):1099-106, 2007](http://www.ncbi.nlm.nih.gov/pubmed/?term=17431700%5Bpmid%5D)
1. [Kalra S et al: Rapid improvement in cortical neuronal integrity in amyotrophic lateral sclerosis detected by proton magnetic resonance spectroscopic imaging. J Neurol. 253(8):1060-3, 2006](http://www.ncbi.nlm.nih.gov/pubmed/?term=16609809%5Bpmid%5D)
1. [Cosottini M et al: Diffusion-tensor MR imaging of corticospinal tract in amyotrophic lateral sclerosis and progressive muscular atrophy. Radiology. 237(1):258-64, 2005](http://www.ncbi.nlm.nih.gov/pubmed/?term=16183935%5Bpmid%5D)
1. [Sach M et al: Diffusion tensor MRI of early upper motor neuron involvement in amyotrophic lateral sclerosis. Brain. 127(Pt 2):340-50, 2004](http://www.ncbi.nlm.nih.gov/pubmed/?term=14607785%5Bpmid%5D)
1. [Kalra S et al: Gabapentin therapy for amyotrophic lateral sclerosis: lack of improvement in neuronal integrity shown by MR spectroscopy. AJNR Am J Neuroradiol. 24(3):476-80, 2003](http://www.ncbi.nlm.nih.gov/pubmed/?term=12637300%5Bpmid%5D)
1. [Kalra S et al: Neuroimaging in amyotrophic lateral sclerosis. Amyotroph Lateral Scler Other Motor Neuron Disord. 4(4):243-8, 2003](http://www.ncbi.nlm.nih.gov/pubmed/?term=14753658%5Bpmid%5D)
1. [Strong M et al: Amyotrophic lateral sclerosis: a review of current concepts. Amyotroph Lateral Scler Other Motor Neuron Disord. 4(3):136-43, 2003](http://www.ncbi.nlm.nih.gov/pubmed/?term=13129799%5Bpmid%5D)
1. [Heath PR et al: Update on the glutamatergic neurotransmitter system and the role of excitotoxicity in amyotrophic lateral sclerosis. Muscle Nerve. 26(4):438-58, 2002](http://www.ncbi.nlm.nih.gov/pubmed/?term=12362409%5Bpmid%5D)
1. [Bowen BC et al: MR imaging and localized proton spectroscopy of the precentral gyrus in amyotrophic lateral sclerosis. AJNR Am J Neuroradiol. 21(4):647-58, 2000](http://www.ncbi.nlm.nih.gov/pubmed/?term=10782773%5Bpmid%5D)
1. [Chan S et al: Motor neuron diseases: comparison of single-voxel proton MR spectroscopy of the motor cortex with MR imaging of the brain. Radiology. 212(3):763-9, 1999](http://www.ncbi.nlm.nih.gov/pubmed/?term=10478245%5Bpmid%5D)
1. [Ellis CM et al: Diffusion tensor MRI assesses corticospinal tract damage in ALS. Neurology. 53(5):1051-8, 1999](http://www.ncbi.nlm.nih.gov/pubmed/?term=10496265%5Bpmid%5D)
1. [Kato Y et al: Detection of pyramidal tract lesions in amyotrophic lateral sclerosis with magnetization-transfer measurements. AJNR Am J Neuroradiol. 18(8):1541-7, 1997](http://www.ncbi.nlm.nih.gov/pubmed/?term=9296197%5Bpmid%5D)
1. [Mascalchi M et al: Corticospinal tract degeneration in motor neuron disease. AJNR Am J Neuroradiol. 16(4 Suppl):878-80, 1995](http://www.ncbi.nlm.nih.gov/pubmed/?term=7611062%5Bpmid%5D)
## Tables
# European Federation of Neurological Societies Guidelines on Use of Neuroimaging in Management of Motor Neuron Disease
| Role of MR in Diagnosis of Amyotrophic Lateral Sclerosis |
| --- |
| Conventional MR is restricted to exclude other causes of signs and symptoms of motor neuron pathology |
| Corticospinal tract T2 hyperintensities and precentral gyrus T2-hypointense rim can support preexisting suspicion of motor neuron disease; specific search of these abnormalities for purpose of making firm diagnosis of motor neuron disease is not recommended |
| Currently advanced neuroimaging techniques (including DTI and MRS) have no role in diagnosis/routine monitoring of motor neuron diseases |
| Advanced neuroimaging techniques are strongly recommended to be incorporated into new clinical trials |
## Images
### Selected Images
![A 56-year-old man with a clinical diagnosis of amyotrophic lateral sclerosis (ALS) presents with progressive bilateral lower extremity weakness (he has been wheelchair bound for 6 months of symptom onset). Axial DWI MR shows hyperintense signal in posterior limb of bilateral internal capsules <img src='img/arrows/CS.png'/>.](images/app.statdx.com_image_thumbnail_7980f8a8-d23c-4ddc-8b7a-7f8bd73f560f_size_168_quality_85_99fc77ee_20251014T204658Z.jpg)
*A 56-year-old man with a clinical diagnosis of amyotrophic lateral sclerosis (ALS) presents with progressive bilateral lower extremity weakness (he has been wheelchair bound for 6 months of symptom onset). Axial DWI MR shows hyperintense signal in posterior limb of bilateral internal capsules <img src='img/arrows/CS.png'/>.*
![Coronal T2 MR in the same patient shows hyperintense signal along the bilateral corticospinal tract (CST) <img src='img/arrows/CC.png'/>, more prominent in the internal capsule and cerebral peduncles.](images/app.statdx.com_image_thumbnail_342bc7c1-519b-444b-9225-2964d67fb255_size_168_quality_85_64f0b844_20251014T204658Z.jpg)
*Coronal T2 MR in the same patient shows hyperintense signal along the bilateral corticospinal tract (CST) <img src='img/arrows/CC.png'/>, more prominent in the internal capsule and cerebral peduncles.*
![Axial FLAIR MR in the same patient shows hyperintense signal in the posterior limbs of bilateral internal capsule along the CSTs <img src='img/arrows/CO.png'/>.](images/app.statdx.com_image_thumbnail_afd212f0-0cc3-439c-bd0a-c55341884298_size_168_quality_85_5a172d7b_20251014T204658Z.jpg)
*Axial FLAIR MR in the same patient shows hyperintense signal in the posterior limbs of bilateral internal capsule along the CSTs <img src='img/arrows/CO.png'/>.*
![Axial 3D SWI in the same patient at the level of vertex shows rim of low signal in bilateral precentral gyri (motor cortex) <img src='img/arrows/CS.png'/>. This finding is nonspecific and may be due to iron and heavy metals accumulating in the cortex of older patients.](images/app.statdx.com_image_thumbnail_17df99b6-8f6c-46bb-bcdc-957e077d4007_size_168_quality_85_e9fa73f8_20251014T204658Z.jpg)
*Axial 3D SWI in the same patient at the level of vertex shows rim of low signal in bilateral precentral gyri (motor cortex) <img src='img/arrows/CS.png'/>. This finding is nonspecific and may be due to iron and heavy metals accumulating in the cortex of older patients.*
![A 52-year-old male patient with ALS presenting with progressive bilateral lower extremity weakness followed by upper extremity weakness is shown. Axial T2WI (top) <img src='img/arrows/CS.png'/> and FLAIR (bottom) <img src='img/arrows/CO.png'/> show hyperintense signal involving the internal capsules posterior limb.](images/app.statdx.com_image_thumbnail_2964e1a8-2c96-4018-ba7d-3934e81dfc4b_size_168_quality_85_7f2a2de8_20251014T204658Z.jpg)
*A 52-year-old male patient with ALS presenting with progressive bilateral lower extremity weakness followed by upper extremity weakness is shown. Axial T2WI (top) <img src='img/arrows/CS.png'/> and FLAIR (bottom) <img src='img/arrows/CO.png'/> show hyperintense signal involving the internal capsules posterior limb.*
![Axial SWI in same patient shows linear low signal along bilateral motor cortex <img src='img/arrows/CC.png'/>. EMG shows degeneration and chronic reinnervation in a multisegmental distribution, consistent with motor neuron disease.](images/app.statdx.com_image_thumbnail_3bfe9e9a-75ff-4398-a317-34958eca4d5b_size_168_quality_85_862b9570_20251014T204658Z.jpg)
*Axial SWI in same patient shows linear low signal along bilateral motor cortex <img src='img/arrows/CC.png'/>. EMG shows degeneration and chronic reinnervation in a multisegmental distribution, consistent with motor neuron disease.*
![Axial DWI MR shows oval hyperintensities corresponding to CSTs in the pons <img src='img/arrows/CC.png'/>. DWI/DTI can help differentiate progressive muscular atrophy [no change in fractional anisotropy (FA) or mean diffusivity (MD)] from ALS (↑ MD, ↓ FA), which can be clinically difficult.](images/app.statdx.com_image_thumbnail_70e8e81a-caec-4607-b991-d2b49a721ab3_size_168_quality_85_922e0f82_20251014T204658Z.jpg)
*Axial DWI MR shows oval hyperintensities corresponding to CSTs in the pons <img src='img/arrows/CC.png'/>. DWI/DTI can help differentiate progressive muscular atrophy [no change in fractional anisotropy (FA) or mean diffusivity (MD)] from ALS (↑ MD, ↓ FA), which can be clinically difficult.*
![Sagittal T1WI MR shows atrophy of the posterior corpus callosum body <img src='img/arrows/CS.png'/>. DTI showed ↓ FA in the corpus callosum. Voxel-based morphometry has high sensitivity in detecting local tissue atrophy in the motor cortex and along the CSTs.](images/app.statdx.com_image_thumbnail_98b3a079-a5fa-4558-86b5-5424e15cdd55_size_168_quality_85_75c69711_20251014T204658Z.jpg)
*Sagittal T1WI MR shows atrophy of the posterior corpus callosum body <img src='img/arrows/CS.png'/>. DTI showed ↓ FA in the corpus callosum. Voxel-based morphometry has high sensitivity in detecting local tissue atrophy in the motor cortex and along the CSTs.*
![Axial T2WI FS MR shows central cortical hypointense signal intensity <img src='img/arrows/CC.png'/> in the precentral gyri due to iron deposition. While this is common in ALS patients, it is nonspecific and may be seen in older patients due to iron and heavy metals accumulation.](images/app.statdx.com_image_thumbnail_a65d37b8-a592-4b8c-83f3-c01526c12ffa_size_168_quality_85_b161e67e_20251014T204658Z.jpg)
*Axial T2WI FS MR shows central cortical hypointense signal intensity <img src='img/arrows/CC.png'/> in the precentral gyri due to iron deposition. While this is common in ALS patients, it is nonspecific and may be seen in older patients due to iron and heavy metals accumulation.*
![Axial T2* SWI MR demonstrates curvilinear hypointensity along the cortical gray matter of bilateral precentral gyri <img src='img/arrows/WS.png'/>. The T2* SWI technique accentuates the T2 hypointensity seen in the precentral gyrus gray matter of ALS patients.](images/app.statdx.com_image_thumbnail_1789caa5-6fce-4bad-ae4f-7b8a27caf563_size_168_quality_85_58633dff_20251014T204658Z.jpg)
*Axial T2* SWI MR demonstrates curvilinear hypointensity along the cortical gray matter of bilateral precentral gyri <img src='img/arrows/WS.png'/>. The T2* SWI technique accentuates the T2 hypointensity seen in the precentral gyrus gray matter of ALS patients.*
### Additional Images
![Axial ADC map in the same ALS patient appears normal.](images/app.statdx.com_image_thumbnail_469c82bc-9232-42ec-92c2-95f572815a41_size_168_quality_85_84649b2f_20251014T204658Z.jpg)
*Axial ADC map in the same ALS patient appears normal.*
![Axial DWI MR shows increased signal involving subcortical white matter of both precentral (motor) gyri, extending caudally into CSTs (not shown). These findings are typical for ALS.](images/app.statdx.com_image_thumbnail_8a1ca269-27bb-49df-9399-09947e5ca981_size_168_quality_85_0f5d1073_20251014T204658Z.jpg)
*Axial DWI MR shows increased signal involving subcortical white matter of both precentral (motor) gyri, extending caudally into CSTs (not shown). These findings are typical for ALS.*
![Axial T2WI MR in the same patient shows hyperintense CSTs <img src='img/arrows/WS.png'/> at the level of the cerebral peduncle. (Courtesy O.Q. Castro, MD.)](images/app.statdx.com_image_thumbnail_7cf95e98-eb1f-4898-8af2-348f7b2a6bd8_size_168_quality_85_238ee8b6_20251014T204658Z.jpg)
*Axial T2WI MR in the same patient shows hyperintense CSTs <img src='img/arrows/WS.png'/> at the level of the cerebral peduncle. (Courtesy O.Q. Castro, MD.)*
![Coronal T2WI MR shows hyperintense CSTs <img src='img/arrows/WS.png'/> in a patient with ALS. (Courtesy O.Q. Castro, MD.)](images/app.statdx.com_image_thumbnail_885cac62-e866-414c-849d-2c218554acda_size_168_quality_85_042df8b6_20251014T204658Z.jpg)
*Coronal T2WI MR shows hyperintense CSTs <img src='img/arrows/WS.png'/> in a patient with ALS. (Courtesy O.Q. Castro, MD.)*
![Axial T2WI MR in the same patient shows symmetrical high signal intensity in corona radiata fibers corresponding to CSTs.](images/app.statdx.com_image_thumbnail_09ee7032-3c39-43af-b367-276605d1babc_size_168_quality_85_1d377957_20251014T204658Z.jpg)
*Axial T2WI MR in the same patient shows symmetrical high signal intensity in corona radiata fibers corresponding to CSTs.*
![Axial T2WI MR in the same patient demonstrates bilateral low signal intensity in the precentral (motor) cortex <img src='img/arrows/WS.png'/>.](images/app.statdx.com_image_thumbnail_8039d26c-24d0-4cb6-a4ab-292123888bd5_size_168_quality_85_59b633b7_20251014T204658Z.jpg)
*Axial T2WI MR in the same patient demonstrates bilateral low signal intensity in the precentral (motor) cortex <img src='img/arrows/WS.png'/>.*
![Axial T2WI MR in the same patient with ALS shows symmetric hyperintense CSTs <img src='img/arrows/WS.png'/> at the level of the cerebral peduncle.](images/app.statdx.com_image_thumbnail_76efcb70-a53c-4052-b210-82805555ae02_size_168_quality_85_588016de_20251014T204658Z.jpg)
*Axial T2WI MR in the same patient with ALS shows symmetric hyperintense CSTs <img src='img/arrows/WS.png'/> at the level of the cerebral peduncle.*
![Axial T2WI MR in a young man with ALS shows symmetric hyperintense CSTs <img src='img/arrows/WS.png'/> at the level of the internal capsule.](images/app.statdx.com_image_thumbnail_5426980d-0fa8-4966-8002-a344cec9344a_size_168_quality_85_101cc33b_20251014T204658Z.jpg)
*Axial T2WI MR in a young man with ALS shows symmetric hyperintense CSTs <img src='img/arrows/WS.png'/> at the level of the internal capsule.*
![Axial DWI MR shows small foci of hyperintensity in the posterior limbs of bilateral internal capsules <img src='img/arrows/WO.png'/> in this ALS patient.](images/app.statdx.com_image_thumbnail_44774761-1a70-44c5-bd5f-d5aa88f76212_size_168_quality_85_3a5f14c0_20251014T204658Z.jpg)
*Axial DWI MR shows small foci of hyperintensity in the posterior limbs of bilateral internal capsules <img src='img/arrows/WO.png'/> in this ALS patient.*
![Axial T2 MR in a patient with ALS shows hyperintensity along the course of the CST <img src='img/arrows/CC.png'/> bilaterally. Important to note that CST is typically slightly hyperintense on T2 especially at 3.0 T.](images/app.statdx.com_image_thumbnail_5807b62a-1167-4816-be1f-17929c835887_size_168_quality_85_ec6211e3_20251014T204658Z.jpg)
*Axial T2 MR in a patient with ALS shows hyperintensity along the course of the CST <img src='img/arrows/CC.png'/> bilaterally. Important to note that CST is typically slightly hyperintense on T2 especially at 3.0 T.*
![Axial DTI trace image shows symmetric hyperintensity in the internal capsule posterior limbs <img src='img/arrows/WO.png'/>. FA correlates with measures of disease severity and UMN involvement, whereas the MD correlates with disease duration.](images/app.statdx.com_image_thumbnail_078a8831-7cec-4943-8af3-031cb2a01dd9_size_168_quality_85_b0b15385_20251014T204658Z.jpg)
*Axial DTI trace image shows symmetric hyperintensity in the internal capsule posterior limbs <img src='img/arrows/WO.png'/>. FA correlates with measures of disease severity and UMN involvement, whereas the MD correlates with disease duration.*
![Axial T2WI FS MR demonstrates ovoid hyperintensity along the CSTs bilaterally <img src='img/arrows/WS.png'/>. Atrophy and hyperintense foci is due to myelin loss and gliosis. There is frequently involvement of prefrontal motor neurons that play a role in planning or orchestrating the work of the upper and lower motor neurons.](images/app.statdx.com_image_thumbnail_9dd78675-0881-4658-b307-5f0411d631f9_size_168_quality_85_bfad1dcb_20251014T204658Z.jpg)
*Axial T2WI FS MR demonstrates ovoid hyperintensity along the CSTs bilaterally <img src='img/arrows/WS.png'/>. Atrophy and hyperintense foci is due to myelin loss and gliosis. There is frequently involvement of prefrontal motor neurons that play a role in planning or orchestrating the work of the upper and lower motor neurons.*
![Coronal FLAIR MR shows linear hyperintensity <img src='img/arrows/WS.png'/> along the CST from the precentral gyrus to the cerebri crus. Right CST signal abnormality is out of this imaging slice. Hyperintensity of the precentral gyrus subcortical white matter on FLAIR is a potentially useful and specific sign of ALS that is not seen in healthy, asymptomatic patients.](images/app.statdx.com_image_thumbnail_b5a25f0d-214b-4443-80cd-252a401e7e0c_size_168_quality_85_c91b1f09_20251014T204658Z.jpg)
*Coronal FLAIR MR shows linear hyperintensity <img src='img/arrows/WS.png'/> along the CST from the precentral gyrus to the cerebri crus. Right CST signal abnormality is out of this imaging slice. Hyperintensity of the precentral gyrus subcortical white matter on FLAIR is a potentially useful and specific sign of ALS that is not seen in healthy, asymptomatic patients.*
docs_md/articles/attention-control-network_a1bedda5-6478-40b2-98e7-6c5f5363b06f.md
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---
title: "Attention Control Network"
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---
# IMAGING ANATOMY
- ## Overview
- Attention control network is a constellation of distributed brain networks processing attention to external stimuli and symbols
- Many aliases: Task-positive network, frontoparietal network, executive control network, and central executive network, each referring to subsets of the attention control network
- Terminology is not standard in the literature, and different authors use many of these terms interchangeably or to refer to different subsets of a broader attentional network
- ## Dorsal Attention Network
- Function: Voluntary control of attentional focus and goal-directed behavior
- Regions: Intraparietal sulcus, frontal eye fields, middle temporal, dorsolateral prefrontal cortex
- Intraparietal sulcus
- Weighting of sensory inputs: Direct control of relative "value" or "attention" to sensory stimuli
- Topographically organized by stimulus modality and spatial location
- Frontal eye fields and supplementary eye fields
- Control of direction of gaze to attentionally relevant stimuli
- Middle temporal (MT)
- Motion perception, dynamic features of attention
- Dorsolateral prefrontal cortex (DLPFC)
- Shared with ventral attention network, representations of objects and symbols, working memory
- ## Ventral Attention Network
- Function: Control of reorienting to relevant stimuli, working memory
- Aliases: Frontoparietal control network, executive control network
- Regions: Supramarginal and angular gyri, inferior frontal gyrus, dorsolateral prefrontal cortex, ventral anterior cingulate cortex
- Inferior parietal lobule (supramarginal and angular gyri)
- Inferior frontal gyrus
- DLPFC
- Ventral anterior cingulate cortex
- ## Salience Network
- Function: Detection of novel or salient stimuli
- Aliases: Novelty detection network, cingulo-opercular network
- Regions: Frontoinsula, dorsal anterior cingulate cortex
- Frontoinsula
- "Sensory" arm of salience network
- Mid superior insula: Detection of stimulus salience
- Mid inferior insula: Detection of emotive salience
- Anterior insula: Control of attention
- Dorsal anterior cingulate cortex
- "Motor" arm of salience network
- More anterior (pregenual) cingulate more associated with emotive salience, merges with ventral attention network
# ANATOMY IMAGING ISSUES
- ## Imaging Recommendations
- Specific subnetworks may be activated by specific tasks (e.g., oddball task for salience network, n-back task for dorsal attention network)
- ## Imaging Pitfalls
- Many fMRI tasks have differential stimulus attention between active and control conditions
- Activation in attentional regions may not be task specific, but a general consequence of differential attention between conditions
- ## Network Relationships
- Attention control network is anticorrelated to default mode network: When one is active, the other tends to be less active
- Allows focus of attention to shift between external and internal stimuli
- Gradients of anticorrelation across the attention control network are seen with specific subregions of each network hub showing greatest anticorrelation
- Attentional regions typically located in association cortex in regions spatially "equidistant" from primary sensory areas
- Flow of information from primary sensory to unimodal sensory association cortex to polymodal association cortex
# CLINICAL IMPLICATIONS
- ## Clinical Importance
- Right-dominant network for attention in most individuals
- Lesions of right ventral attention network may produce hemispatial neglect
6e3d29ca-3af0-4a42-b125-7ec5fad53f3f
## References
# Selected References
1. [Petersen SE et al: The attention system of the human brain: 20 years after. Annu Rev Neurosci. 35:73-89, 2012](http://www.ncbi.nlm.nih.gov/pubmed/?term=22524787%5Bpmid%5D)
1. [Anderson JS et al: Connectivity gradients between the default mode and attention control networks. Brain Connect. 1(2):147-57, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=22076305%5Bpmid%5D)
1. [Anderson JS et al: Topographic maps of multisensory attention. Proc Natl Acad Sci U S A. 107(46):20110-4, 2010](http://www.ncbi.nlm.nih.gov/pubmed/?term=21041658%5Bpmid%5D)
1. [Dosenbach NU et al: A dual-networks architecture of top-down control. Trends Cogn Sci. 12(3):99-105, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18262825%5Bpmid%5D)
1. [Seeley WW et al: Dissociable intrinsic connectivity networks for salience processing and executive control. J Neurosci. 27(9):2349-56, 2007](http://www.ncbi.nlm.nih.gov/pubmed/?term=17329432%5Bpmid%5D)
1. [Fox MD et al: Spontaneous neuronal activity distinguishes human dorsal and ventral attention systems. Proc Natl Acad Sci U S A. 103(26):10046-51, 2006](http://www.ncbi.nlm.nih.gov/pubmed/?term=16788060%5Bpmid%5D)
1. [Fox MD et al: The human brain is intrinsically organized into dynamic, anticorrelated functional networks. Proc Natl Acad Sci U S A. 102(27):9673-8, 2005](http://www.ncbi.nlm.nih.gov/pubmed/?term=15976020%5Bpmid%5D)
1. [Corbetta M et al: Control of goal-directed and stimulus-driven attention in the brain. Nat Rev Neurosci. 3(3):201-15, 2002](http://www.ncbi.nlm.nih.gov/pubmed/?term=11994752%5Bpmid%5D)
## Images
### Attention Control Network
![Subnetworks of the attention control network are shown. The mask of attention control network regions was obtained from brain voxels correlated with r &gt; 0.15 to 4 seeds in the bilateral intraparietal sulcus and bilateral superior insula in 1,353 subjects. Functional connectivity was calculated for each voxel within this mask to 7,266 gray matter regions, and voxels were parcellated into 3 clusters using a k-means algorithm based on similar connectivity to the rest of the brain. The voxels in blue show characteristic distribution of the dorsal attention network, including frontal eye fields, intraparietal sulcus, and middle temporal areas. The voxels in green show characteristic distribution of the salience network, including insula and dorsal anterior cingulate areas. The voxels in red show a distribution most consistent with the ventral attention network, including dorsolateral prefrontal, inferior frontal, ventral anterior cingulate, and supramarginal gyrus areas.](images/app.statdx.com_image_b49bad74-6fc8-4185-881e-cec0fb281984_65483bd0_20251014T185159Z.jpg)
*Subnetworks of the attention control network are shown. The mask of attention control network regions was obtained from brain voxels correlated with r &gt; 0.15 to 4 seeds in the bilateral intraparietal sulcus and bilateral superior insula in 1,353 subjects. Functional connectivity was calculated for each voxel within this mask to 7,266 gray matter regions, and voxels were parcellated into 3 clusters using a k-means algorithm based on similar connectivity to the rest of the brain. The voxels in blue show characteristic distribution of the dorsal attention network, including frontal eye fields, intraparietal sulcus, and middle temporal areas. The voxels in green show characteristic distribution of the salience network, including insula and dorsal anterior cingulate areas. The voxels in red show a distribution most consistent with the ventral attention network, including dorsolateral prefrontal, inferior frontal, ventral anterior cingulate, and supramarginal gyrus areas.*
![Subnetworks of the attention control network are shown. The mask of attention control network regions was obtained from brain voxels correlated with r &gt; 0.15 to 4 seeds in the bilateral intraparietal sulcus and bilateral superior insula in 1,353 subjects. Functional connectivity was calculated for each voxel within this mask to 7,266 gray matter regions, and voxels were parcellated into 3 clusters using a k-means algorithm based on similar connectivity to the rest of the brain. The voxels in blue show characteristic distribution of the dorsal attention network, including frontal eye fields, intraparietal sulcus, and middle temporal areas. The voxels in green show characteristic distribution of the salience network, including insula and dorsal anterior cingulate areas. The voxels in red show a distribution most consistent with the ventral attention network, including dorsolateral prefrontal, inferior frontal, ventral anterior cingulate, and supramarginal gyrus areas.](images/app.statdx.com_image_thumbnail_b49bad74-6fc8-4185-881e-cec0fb281984_size_168_quality_85_80971dd9_20251014T185127Z.jpg)
*Subnetworks of the attention control network are shown. The mask of attention control network regions was obtained from brain voxels correlated with r &gt; 0.15 to 4 seeds in the bilateral intraparietal sulcus and bilateral superior insula in 1,353 subjects. Functional connectivity was calculated for each voxel within this mask to 7,266 gray matter regions, and voxels were parcellated into 3 clusters using a k-means algorithm based on similar connectivity to the rest of the brain. The voxels in blue show characteristic distribution of the dorsal attention network, including frontal eye fields, intraparietal sulcus, and middle temporal areas. The voxels in green show characteristic distribution of the salience network, including insula and dorsal anterior cingulate areas. The voxels in red show a distribution most consistent with the ventral attention network, including dorsolateral prefrontal, inferior frontal, ventral anterior cingulate, and supramarginal gyrus areas.*
![Subnetworks of the attention control network are shown. The mask of attention control network regions was obtained from brain voxels correlated with r &gt; 0.15 to 4 seeds in the bilateral intraparietal sulcus and bilateral superior insula in 1,353 subjects. Functional connectivity was calculated for each voxel within this mask to 7,266 gray matter regions, and voxels were parcellated into 3 clusters using a k-means algorithm based on similar connectivity to the rest of the brain. The voxels in blue show characteristic distribution of the dorsal attention network, including frontal eye fields, intraparietal sulcus, and middle temporal areas. The voxels in green show characteristic distribution of the salience network, including insula and dorsal anterior cingulate areas. The voxels in red show a distribution most consistent with the ventral attention network, including dorsolateral prefrontal, inferior frontal, ventral anterior cingulate, and supramarginal gyrus areas.](images/app.statdx.com_image_thumbnail_b49bad74-6fc8-4185-881e-cec0fb281984_size_174_quality_85_8d0b7afb_20251014T185126Z.jpg)
*Subnetworks of the attention control network are shown. The mask of attention control network regions was obtained from brain voxels correlated with r &gt; 0.15 to 4 seeds in the bilateral intraparietal sulcus and bilateral superior insula in 1,353 subjects. Functional connectivity was calculated for each voxel within this mask to 7,266 gray matter regions, and voxels were parcellated into 3 clusters using a k-means algorithm based on similar connectivity to the rest of the brain. The voxels in blue show characteristic distribution of the dorsal attention network, including frontal eye fields, intraparietal sulcus, and middle temporal areas. The voxels in green show characteristic distribution of the salience network, including insula and dorsal anterior cingulate areas. The voxels in red show a distribution most consistent with the ventral attention network, including dorsolateral prefrontal, inferior frontal, ventral anterior cingulate, and supramarginal gyrus areas.*
docs_md/articles/brain-tumor-in-child-1-year_7d64f5fb-c62c-4861-8ff4-654a12074605.md
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title: "Brain Tumor in Child > 1 Year"
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---
# ESSENTIAL INFORMATION
- ## Key Differential Diagnosis Issues
- General rule: Decreased diffusion = higher grade
- ## Helpful Clues for Common Diagnoses
- **Posterior Fossa** (most common location)
- **Pilocytic astrocytoma**
- Low-density NECT
- Solid, enhancing nodule and cyst: Classic appearance
- Solid nodule has increased diffusion = good prognosis
- Off midline compared to classic 4th ventricular tumors but may pedunculate into ventricle
- Characterized by *KIAA1549*::*BRAF* fusion
- **Medulloblastoma**
- Hyperdense posterior fossa mass on NECT
- Decreased diffusion
- Molecular subgroups predicts outcome
- WNT-activated: Up to 90% overall survival, 4th ventricle and cerebellopontine angle (CPA) location, rarest subgroup
- SHH-activated: Poor to good prognosis, cerebellar hemispheric location, + *TP53* mutation = poor prognosis
- Group 3: Worst prognosis with frequent metastases, midline 4th ventricle
- Group 4: Intermediate prognosis with occasional metastases, midline 4th ventricle, minimal to no enhancement, most common subgroup
- **Ependymoma**
- Less common than pilocytic astrocytoma and medulloblastoma
- 60% posterior fossa
- "Plastic" tumor in 4th ventricle, extrudes through foramina
- Can have mixed calcification and cysts
- Molecular subgroups predict outcome
- Posterior fossa type A: Younger children, lateral location, characterized by H3 K27 alteration, poor outcome
- Posterior fossa type B: Adolescents, midline location, good outcome, uncommon
- **Diffuse midline glioma, H3 K27-altered**
- **Diffuse intrinsic pontine glioma**
- T2-hyperintense expansion of pons, little to no enhancement
- Can also involve cerebellum and spinal cord
- Poor prognosis
- **Supratentorial**(more common in infants and adolescents)
- **Craniopharyngioma**
- Nearly 1/2 of pediatric suprasellar masses, typically adamantinomatous
- 90% calcification/cystic/enhance
- Squamous cell from Rathke cleft
- **Pilocytic astrocytoma**
- Commonly involves optic pathway or around 3rd ventricle, tectal plate
- Optic "gliomas" are commonly associated with *NF1*
- Heterogeneous appearance and enhancement
- Pilomyxoid astrocytoma variant is more locally aggressive and more likely to present with leptomeningeal metastases
- Similar genetic and molecular characteristics; was removed as separate grading designation in WHO 2016
- Supratentorial likely to have *BRAF*V600E mutation
- **Diffuse low-grade glioma, pediatric**
- Hemispheres, thalami (can be bithalamic), tectum
- 50% of brainstem "gliomas" are low-grade diffusely infiltrating astrocytomas
- Can be poorly marginated or focal
- Hypointense on T1WI, hyperintense on T2WI, little to no enhancement
- WHO 2021 has 4 tumor types in this family
- **Diffuse astrocytoma, MYB- or MYBL1-altered**
- **Diffuse low-grade glioma, MAPK pathway-altered**
- **Polymorphous low-grade neuroepithelial tumor of the young** (PLNTY): Commonly cortical with heterogeneous T2 signal from calcification
- **Angiocentric glioma**
- **Subependymal giant cell astrocytoma**
- Seen in tuberous sclerosis
- Location at foramina of Monro is typical
- Look for cortical/subcortical tubers and subependymal nodules
- Heterogenous calcification, marked enhancement
- ## Helpful Clues for Less Common Diagnoses
- **Intraaxial: Peripheral and Cortical**
- **Dysembryoplastic neuroepithelial tumor**
- Bubbly-appearing, cortically based mass
- Bright ring sign on FLAIR MR
- Almost all in patients < 20 years old, chronic epilepsy
- **Pleomorphic xanthoastrocytoma**
- Cortically based tumor (temporal lobe most common)
- Enhancing mass + cyst, dural reaction (tail) common
- Majority demonstrate *BRAF*V600E mutation
- **Ganglioglioma**
- Temporal lobe predilection with seizure presentation
- Solid or solid with cyst, ± enhancement
- **Oligodendroglioma**
- Cortically based, frontal lobe predominance
- Calcification common
- Predominantly T2 hyperintense ± enhancement
- Characterized by 1p/19q codeletion; if 1p/19q intact, represents astrocytoma with poorer outcome
- **Intraaxial: Deep and Hemispheric**
- **Diffuse high-grade glioma, pediatric**
- Diffusely infiltrating, heterogeneous, ± enhancement
- WHO 2021 has 4 tumor types in this family (all have poor outcome)
- **Diffuse midline glioma, H3 K27-altered**: Central location, involves thalami and brainstem
- **Diffuse hemispheric glioma, H3 G34-mutant**
- **Diffuse pediatric-type high-grade glioma, H3-wildtype and IDH-wildtype**
- **Infant-type hemispheric glioma**
- **CNS embryonal tumor**
- Primitive neuroepithelial tumor terminology removed from 2016 WHO CNS tumors
- Infant with large, bulky, complex hemispheric mass
- Calcification, hemorrhage, necrosis common
- Peritumoral edema sparse/absent, less than expected for size
- **Supratentorial ependymoma**
- Diffuse infiltrating, heterogeneously enhancing tumor
- Origin is periventricular from ependymal rests
- Molecular subgroups predict outcome
- *ZFTA* fusion-positive, younger children, poor outcome
- *YAP1* fusion-positive, older children, good outcome, rare
- **Extraaxial**
- **Choroid plexus tumor**
- Intraventricular: Lateral > 4th > 3rd ventricles
- Densely enhancing, frond-like
- Although **choroid plexus carcinoma** may show parenchymal invasion, it is not reliably distinguished from **papilloma** on imaging
- **Neurofibromatosis type 2**
- Vestibular **schwannomas**
- If multiple schwannomas, think neurofibromatosis type 2
- Look for "hidden" dural-based**meningiomas**
- **Midline Pineal and Suprasellar Location**
- **Germ cell tumor**
- Organized by cell lineage and maturity
- Germinomatous germ cell tumor
- **Germinoma**: Common in older children and adolescents, excellent prognosis
- Homogeneous with enhancement and decreased diffusion in pure germinomas
- Nongerminomatous germ cell tumors
- **Teratoma**: Fetal life to adolescence; mature teratomas may have fat and calcification; smaller teratomas that are easily resected have good prognosis; **immature teratomas** have poor prognosis
- **Embryonal carcinoma****,** **yolk sac tumor****, and** **choriocarcinoma**: Rare; older children and adolescents, poor prognosis
- Heterogeneous with enhancement
- **Mixed germ cell tumor**: Includes germinomatous and nongerminomatous components
- Worse prognosis than pure germinomas due to nongerminomatous components
- Suprasellar + pineal lobular, enhancing masses together best clue
- Engulfs pineal calcification on CT
- **Pineoblastoma**
- Decreased diffusion
- Look for CSF spread (ventricles, ependyma)
- Difficult to distinguish from germinoma
- "Exploding" pineal calcification on CT
- Pineoblastoma more common than lower grade varieties, such as **pineocytoma** and **pineal parenchymal tumor of intermediate differentiation**
- ## Helpful Clues for Rare Diagnoses
- **Atypical Teratoid-Rhabdoid Tumor**
- Heterogeneous intracranial mass in infants and children
- 50% infratentorial; early CSF spread
- Typically decreased diffusion, greater amount of cysts than medulloblastoma
- **Astroblastoma,****MN1****-Altered**
- Typically peripheral, well circumscribed
- Solid and cystic heterogeneous enhancement
- **Central Neurocytoma**
- "Bubbly," lobulated mass in body of lateral ventricle
- Often along septum pellucidum
- **Dysplastic Cerebellar Gangliocytoma**
- Not associated with PTEN hamartoma syndrome in children
- T2-hyperintense, striated cerebellum with enlarged folia
## References
# Selected References
1. [Louis DN et al: The 2021 WHO Classification of Tumors of the Central Nervous System: a summary. Neuro Oncol. 23(8):1231-51, 2021](http://www.ncbi.nlm.nih.gov/pubmed/?term=34185076%5Bpmid%5D)
1. [Bayliss J et al: Lowered H3K27me3 and DNA hypomethylation define poorly prognostic pediatric posterior fossa ependymomas. Sci Transl Med. 8(366):366ra161, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=27881822%5Bpmid%5D)
1. [Louis DN et al: The 2016 World Health Organization Classification of Tumors of the Central Nervous System: a summary. Acta Neuropathol. 131(6):803-20, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=27157931%5Bpmid%5D)
1. [Smits M: Imaging of oligodendroglioma. Br J Radiol. 89(1060):20150857, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=26849038%5Bpmid%5D)
1. [DeSouza RM et al: Pediatric medulloblastoma - update on molecular classification driving targeted therapies. Front Oncol. 4:176, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=25101241%5Bpmid%5D)
1. [Perreault S et al: MRI surrogates for molecular subgroups of medulloblastoma. AJNR Am J Neuroradiol. 35(7):1263-9, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=24831600%5Bpmid%5D)
## Images
### Selected Images
![Axial T2 FS MR shows a solid tumor <img src='img/arrows/CS.png'/> with a cyst <img src='img/arrows/CC.png'/> in the right cerebellar hemisphere effacing the 4th ventricle <img src='img/arrows/CO.png'/>. The solid component has relative increased T2 signal, suggesting a low-grade tumor.](images/app.statdx.com_image_thumbnail_2482280b-2ada-4804-8b16-2d9853dc0f02_size_168_quality_85_44c0fe04_20251014T202430Z.jpg)
**Pilocytic Astrocytoma**
*Axial T2 FS MR shows a solid tumor <img src='img/arrows/CS.png'/> with a cyst <img src='img/arrows/CC.png'/> in the right cerebellar hemisphere effacing the 4th ventricle <img src='img/arrows/CO.png'/>. The solid component has relative increased T2 signal, suggesting a low-grade tumor.*
![Axial T2 FS MR shows a solid tumor <img src='img/arrows/CS.png'/> with a cyst <img src='img/arrows/CC.png'/> in the right cerebellar hemisphere effacing the 4th ventricle <img src='img/arrows/CO.png'/>. The solid component has relative increased T2 signal, suggesting a low-grade tumor.](images/app.statdx.com_image_thumbnail_2482280b-2ada-4804-8b16-2d9853dc0f02_size_168_quality_85_7c33c721_20251014T200552Z.jpg)
**Pilocytic Astrocytoma**
*Axial T2 FS MR shows a solid tumor <img src='img/arrows/CS.png'/> with a cyst <img src='img/arrows/CC.png'/> in the right cerebellar hemisphere effacing the 4th ventricle <img src='img/arrows/CO.png'/>. The solid component has relative increased T2 signal, suggesting a low-grade tumor.*
![Axial T2 FS MR shows a solid tumor <img src='img/arrows/CS.png'/> with a cyst <img src='img/arrows/CC.png'/> in the right cerebellar hemisphere effacing the 4th ventricle <img src='img/arrows/CO.png'/>. The solid component has relative increased T2 signal, suggesting a low-grade tumor.](images/app.statdx.com_image_thumbnail_2482280b-2ada-4804-8b16-2d9853dc0f02_size_174_quality_85_d8a1268a.jpg)
**Pilocytic Astrocytoma**
*Axial T2 FS MR shows a solid tumor <img src='img/arrows/CS.png'/> with a cyst <img src='img/arrows/CC.png'/> in the right cerebellar hemisphere effacing the 4th ventricle <img src='img/arrows/CO.png'/>. The solid component has relative increased T2 signal, suggesting a low-grade tumor.*
![Axial T1 C+ MR shows homogeneous enhancement of the solid component. A low-grade glial tumor, such as a pilocytic astrocytoma, has fluffy-appearing enhancement <img src='img/arrows/CS.png'/> as much of the enhancement is from vessel leakage.](b0da333a-a4ac-45f2-ba11-a1f6dfbf5a39)
**Pilocytic Astrocytoma**
*Axial T1 C+ MR shows homogeneous enhancement of the solid component. A low-grade glial tumor, such as a pilocytic astrocytoma, has fluffy-appearing enhancement <img src='img/arrows/CS.png'/> as much of the enhancement is from vessel leakage.*
![Axial b=1000 DWI MR shows the solid component has similar signal to the brain <img src='img/arrows/CS.png'/>, not suggesting decreased diffusion, which is compatible with a low-grade neoplasm.](91a6d162-919a-4a2e-a81e-2480fe565edf)
**Pilocytic Astrocytoma**
*Axial b=1000 DWI MR shows the solid component has similar signal to the brain <img src='img/arrows/CS.png'/>, not suggesting decreased diffusion, which is compatible with a low-grade neoplasm.*
![Axial T1 C+ MR shows a heterogeneously enhancing mass in the 4th ventricle <img src='img/arrows/CS.png'/>. Adjacent nodules of enhancement are seen in the cerebellar hemisphere from leptomeningeal metastases <img src='img/arrows/CO.png'/>. WNT medulloblastomas have a &gt; 90% survival rate despite metastatic disease.](images/app.statdx.com_image_thumbnail_bf847f62-c4d0-4af8-ba82-8ae5001d9f04_size_168_quality_85_2178d5fe_20251014T202430Z.jpg)
**Medulloblastoma**
*Axial T1 C+ MR shows a heterogeneously enhancing mass in the 4th ventricle <img src='img/arrows/CS.png'/>. Adjacent nodules of enhancement are seen in the cerebellar hemisphere from leptomeningeal metastases <img src='img/arrows/CO.png'/>. WNT medulloblastomas have a &gt; 90% survival rate despite metastatic disease.*
![Axial T1 C+ MR shows a heterogeneously enhancing mass in the 4th ventricle <img src='img/arrows/CS.png'/>. Adjacent nodules of enhancement are seen in the cerebellar hemisphere from leptomeningeal metastases <img src='img/arrows/CO.png'/>. WNT medulloblastomas have a &gt; 90% survival rate despite metastatic disease.](images/app.statdx.com_image_thumbnail_bf847f62-c4d0-4af8-ba82-8ae5001d9f04_size_168_quality_85_dc58e7fa_20251014T200553Z.jpg)
**Medulloblastoma**
*Axial T1 C+ MR shows a heterogeneously enhancing mass in the 4th ventricle <img src='img/arrows/CS.png'/>. Adjacent nodules of enhancement are seen in the cerebellar hemisphere from leptomeningeal metastases <img src='img/arrows/CO.png'/>. WNT medulloblastomas have a &gt; 90% survival rate despite metastatic disease.*
![Axial T1 C+ MR shows a heterogeneous mass with central necrosis in the left cerebellar hemisphere <img src='img/arrows/CS.png'/>. This was an SHH medulloblastoma, which typically presents in the cerebellar hemispheres.](905fd38c-44d2-4e24-9168-dcfdbce89838)
**Medulloblastoma**
*Axial T1 C+ MR shows a heterogeneous mass with central necrosis in the left cerebellar hemisphere <img src='img/arrows/CS.png'/>. This was an SHH medulloblastoma, which typically presents in the cerebellar hemispheres.*
![Axial b=1000 DWI MR shows the cerebellar hemisphere mass has decreased diffusion consistent with a high-grade neoplasm <img src='img/arrows/CS.png'/>. This SHH medulloblastoma is TP53-wildtype, which has a better prognosis than SHH medulloblastoma with TP53 mutation.](4348ad90-6940-4491-8cde-827730dde7d7)
**Medulloblastoma**
*Axial b=1000 DWI MR shows the cerebellar hemisphere mass has decreased diffusion consistent with a high-grade neoplasm <img src='img/arrows/CS.png'/>. This SHH medulloblastoma is TP53-wildtype, which has a better prognosis than SHH medulloblastoma with TP53 mutation.*
![Axial T1 C+ MR demonstrates a small, 4th ventricle, enhancing mass <img src='img/arrows/CS.png'/> with diffuse leptomeningeal enhancement <img src='img/arrows/CC.png'/> from metastatic disease in this group 3 medulloblastoma. With the exception of WNT, metastasis increases the risk for any subtype.](8d964d5d-8cbe-40c9-a261-f147393eb27a)
**Medulloblastoma**
*Axial T1 C+ MR demonstrates a small, 4th ventricle, enhancing mass <img src='img/arrows/CS.png'/> with diffuse leptomeningeal enhancement <img src='img/arrows/CC.png'/> from metastatic disease in this group 3 medulloblastoma. With the exception of WNT, metastasis increases the risk for any subtype.*
![Axial T1 C+ MR shows a heterogeneously enhancing mass extending through right foramen of Luschka <img src='img/arrows/CS.png'/> in this posterior fossa type A (PFA) ependymoma, which are more laterally located and have a worse prognosis compared to posterior fossa type B.](images/app.statdx.com_image_thumbnail_159fe31c-3aa2-4639-8abe-bb4236778fc7_size_168_quality_85_33e1f510_20251014T200552Z.jpg)
**Ependymoma**
*Axial T1 C+ MR shows a heterogeneously enhancing mass extending through right foramen of Luschka <img src='img/arrows/CS.png'/> in this posterior fossa type A (PFA) ependymoma, which are more laterally located and have a worse prognosis compared to posterior fossa type B.*
![Axial T1 C+ MR shows a heterogeneously enhancing mass extending through right foramen of Luschka <img src='img/arrows/CS.png'/> in this posterior fossa type A (PFA) ependymoma, which are more laterally located and have a worse prognosis compared to posterior fossa type B.](images/app.statdx.com_image_thumbnail_159fe31c-3aa2-4639-8abe-bb4236778fc7_size_168_quality_85_883638d2_20251014T202430Z.jpg)
**Ependymoma**
*Axial T1 C+ MR shows a heterogeneously enhancing mass extending through right foramen of Luschka <img src='img/arrows/CS.png'/> in this posterior fossa type A (PFA) ependymoma, which are more laterally located and have a worse prognosis compared to posterior fossa type B.*
![Axial T1 C+ MR shows a heterogeneous enhancing mass in the left thalamus <img src='img/arrows/CS.png'/>. This was an H3 K27-mutant diffuse midline glioma. These have poor prognosis and are located in the thalami, brainstem, and spinal cord.](images/app.statdx.com_image_thumbnail_fb0f23ac-9a77-4dd2-90a5-483eeec8213d_size_168_quality_85_63d8177d_20251014T202430Z.jpg)
**Diffuse Midline Glioma, H3 K27-Altered**
*Axial T1 C+ MR shows a heterogeneous enhancing mass in the left thalamus <img src='img/arrows/CS.png'/>. This was an H3 K27-mutant diffuse midline glioma. These have poor prognosis and are located in the thalami, brainstem, and spinal cord.*
![Axial T1 C+ MR shows a heterogeneous enhancing mass in the left thalamus <img src='img/arrows/CS.png'/>. This was an H3 K27-mutant diffuse midline glioma. These have poor prognosis and are located in the thalami, brainstem, and spinal cord.](images/app.statdx.com_image_thumbnail_fb0f23ac-9a77-4dd2-90a5-483eeec8213d_size_168_quality_85_7ab682ca_20251014T200552Z.jpg)
**Diffuse Midline Glioma, H3 K27-Altered**
*Axial T1 C+ MR shows a heterogeneous enhancing mass in the left thalamus <img src='img/arrows/CS.png'/>. This was an H3 K27-mutant diffuse midline glioma. These have poor prognosis and are located in the thalami, brainstem, and spinal cord.*
![Axial b=1000 DWI MR shows the right thalamic tumor has some areas of decreased diffusion <img src='img/arrows/CS.png'/>, suggesting a high-grade neoplasm.](70bb4e5e-cb6a-4175-9099-7166cdff00cd)
**Diffuse Midline Glioma, H3 K27-Altered**
*Axial b=1000 DWI MR shows the right thalamic tumor has some areas of decreased diffusion <img src='img/arrows/CS.png'/>, suggesting a high-grade neoplasm.*
![Sagittal NECT shows a mass in the suprasellar cistern with a hyperdense cyst <img src='img/arrows/CS.png'/> and calcification <img src='img/arrows/CO.png'/>, consistent with craniopharyngioma. This is the most common suprasellar tumor in children.](images/app.statdx.com_image_thumbnail_6797ca31-40a3-49d5-a6bb-ce93cc7eebfc_size_168_quality_85_79d255bc_20251014T200552Z.jpg)
**Craniopharyngioma**
*Sagittal NECT shows a mass in the suprasellar cistern with a hyperdense cyst <img src='img/arrows/CS.png'/> and calcification <img src='img/arrows/CO.png'/>, consistent with craniopharyngioma. This is the most common suprasellar tumor in children.*
![Sagittal NECT shows a mass in the suprasellar cistern with a hyperdense cyst <img src='img/arrows/CS.png'/> and calcification <img src='img/arrows/CO.png'/>, consistent with craniopharyngioma. This is the most common suprasellar tumor in children.](images/app.statdx.com_image_thumbnail_6797ca31-40a3-49d5-a6bb-ce93cc7eebfc_size_168_quality_85_eaa4643c_20251014T202430Z.jpg)
**Craniopharyngioma**
*Sagittal NECT shows a mass in the suprasellar cistern with a hyperdense cyst <img src='img/arrows/CS.png'/> and calcification <img src='img/arrows/CO.png'/>, consistent with craniopharyngioma. This is the most common suprasellar tumor in children.*
![Coronal T1 C+ MR shows a solid and cystic mass in the right thalamus with &quot;fluffy&quot; enhancement <img src='img/arrows/CS.png'/>. This child has neurofibromatosis type 1 (NF1), and the tumor was a pilocytic astrocytoma.](dabe68c8-af52-4142-ae4c-1e1a29c4519f)
**Pilocytic Astrocytoma**
*Coronal T1 C+ MR shows a solid and cystic mass in the right thalamus with &quot;fluffy&quot; enhancement <img src='img/arrows/CS.png'/>. This child has neurofibromatosis type 1 (NF1), and the tumor was a pilocytic astrocytoma.*
![Axial b=1000 DWI MR of the tumor in the right thalamus in the same patient demonstrates low signal <img src='img/arrows/CS.png'/> or increased diffusion, consistent with a low-grade pilocytic astrocytoma.](3bda6756-961d-4c71-9356-538f929e2929)
**Pilocytic Astrocytoma**
*Axial b=1000 DWI MR of the tumor in the right thalamus in the same patient demonstrates low signal <img src='img/arrows/CS.png'/> or increased diffusion, consistent with a low-grade pilocytic astrocytoma.*
![Sagittal T2 MR shows a T2-hyperintense mass <img src='img/arrows/CS.png'/> in the tectal plate obstructing the cerebral aqueduct. Despite the lack of biopsy, this is most commonly a low-grade diffuse glioma or tectal glioma. The course is generally indolent and commonly requires ventricular shunting.](images/app.statdx.com_image_thumbnail_99f09d3c-13f7-489c-bc35-8aa589393955_size_168_quality_85_3957ad37_20251014T200553Z.jpg)
**Diffuse Low-Grade Glioma, Pediatric**
*Sagittal T2 MR shows a T2-hyperintense mass <img src='img/arrows/CS.png'/> in the tectal plate obstructing the cerebral aqueduct. Despite the lack of biopsy, this is most commonly a low-grade diffuse glioma or tectal glioma. The course is generally indolent and commonly requires ventricular shunting.*
![Sagittal T2 MR shows a T2-hyperintense mass <img src='img/arrows/CS.png'/> in the tectal plate obstructing the cerebral aqueduct. Despite the lack of biopsy, this is most commonly a low-grade diffuse glioma or tectal glioma. The course is generally indolent and commonly requires ventricular shunting.](images/app.statdx.com_image_thumbnail_99f09d3c-13f7-489c-bc35-8aa589393955_size_168_quality_85_5297b5b2_20251014T202430Z.jpg)
**Diffuse Low-Grade Glioma, Pediatric**
*Sagittal T2 MR shows a T2-hyperintense mass <img src='img/arrows/CS.png'/> in the tectal plate obstructing the cerebral aqueduct. Despite the lack of biopsy, this is most commonly a low-grade diffuse glioma or tectal glioma. The course is generally indolent and commonly requires ventricular shunting.*
![Axial T1 C+ MR shows a lobular, enhancing mass near the foramen of Monro <img src='img/arrows/CS.png'/>. Subependymal giant cell astrocytomas are invariably located at this location. Other subependymal nodules and cortical tubers should be sought to confirm tuberous sclerosis.](images/app.statdx.com_image_thumbnail_46362eeb-7e38-449e-a9dc-df295c4979e7_size_168_quality_85_52ca1269_20251014T200553Z.jpg)
**Subependymal Giant Cell Astrocytoma**
*Axial T1 C+ MR shows a lobular, enhancing mass near the foramen of Monro <img src='img/arrows/CS.png'/>. Subependymal giant cell astrocytomas are invariably located at this location. Other subependymal nodules and cortical tubers should be sought to confirm tuberous sclerosis.*
![Axial T1 C+ MR shows a lobular, enhancing mass near the foramen of Monro <img src='img/arrows/CS.png'/>. Subependymal giant cell astrocytomas are invariably located at this location. Other subependymal nodules and cortical tubers should be sought to confirm tuberous sclerosis.](images/app.statdx.com_image_thumbnail_46362eeb-7e38-449e-a9dc-df295c4979e7_size_168_quality_85_ea61d22b_20251014T202430Z.jpg)
**Subependymal Giant Cell Astrocytoma**
*Axial T1 C+ MR shows a lobular, enhancing mass near the foramen of Monro <img src='img/arrows/CS.png'/>. Subependymal giant cell astrocytomas are invariably located at this location. Other subependymal nodules and cortical tubers should be sought to confirm tuberous sclerosis.*
![Coronal FLAIR MR shows a cortically based, triangular mass that is well circumscribed <img src='img/arrows/CS.png'/>. There is no contrast enhancement (not shown). Dysembryoplastic neuroepithelial tumors are typically cortically based, low-grade tumors that can have a cystic appearance.](images/app.statdx.com_image_thumbnail_ef7ddc56-e0e1-4317-aad1-5d46bb01b497_size_168_quality_85_0a37f96f_20251014T200553Z.jpg)
**Dysembryoplastic Neuroepithelial Tumor**
*Coronal FLAIR MR shows a cortically based, triangular mass that is well circumscribed <img src='img/arrows/CS.png'/>. There is no contrast enhancement (not shown). Dysembryoplastic neuroepithelial tumors are typically cortically based, low-grade tumors that can have a cystic appearance.*
![Coronal FLAIR MR shows a cortically based, triangular mass that is well circumscribed <img src='img/arrows/CS.png'/>. There is no contrast enhancement (not shown). Dysembryoplastic neuroepithelial tumors are typically cortically based, low-grade tumors that can have a cystic appearance.](images/app.statdx.com_image_thumbnail_ef7ddc56-e0e1-4317-aad1-5d46bb01b497_size_168_quality_85_30688876_20251014T202430Z.jpg)
**Dysembryoplastic Neuroepithelial Tumor**
*Coronal FLAIR MR shows a cortically based, triangular mass that is well circumscribed <img src='img/arrows/CS.png'/>. There is no contrast enhancement (not shown). Dysembryoplastic neuroepithelial tumors are typically cortically based, low-grade tumors that can have a cystic appearance.*
![Coronal T1 C+ MR shows a cyst and a solid enhancing, cortically based mass with a broad dural base <img src='img/arrows/CS.png'/>. The broad dural base of this cortically based intraaxial tumor is a typical appearance for pleomorphic xanthoastrocytoma.](images/app.statdx.com_image_thumbnail_b9c0d89e-7726-49c7-ba9d-8e0a6f37dcec_size_168_quality_85_885704ac_20251014T204343Z.jpg)
**Pleomorphic Xanthoastrocytoma**
*Coronal T1 C+ MR shows a cyst and a solid enhancing, cortically based mass with a broad dural base <img src='img/arrows/CS.png'/>. The broad dural base of this cortically based intraaxial tumor is a typical appearance for pleomorphic xanthoastrocytoma.*
![Coronal T1 C+ MR shows a heterogeneous, large, enhancing mass in the temporal lobe <img src='img/arrows/CS.png'/>. This was a ganglioglioma at resection. The temporal lobe is a common location for this tumor when there is seizure presentation.](images/app.statdx.com_image_thumbnail_8d2855f9-820a-47ec-a35f-7c0d913d570a_size_168_quality_85_0936ec6a_20251014T204343Z.jpg)
**Ganglioglioma**
*Coronal T1 C+ MR shows a heterogeneous, large, enhancing mass in the temporal lobe <img src='img/arrows/CS.png'/>. This was a ganglioglioma at resection. The temporal lobe is a common location for this tumor when there is seizure presentation.*
![Axial T1 C+ MR shows an enhancing mass in the posterior temporal lobe <img src='img/arrows/CS.png'/> with a satellite nodule <img src='img/arrows/CO.png'/>. This was a diffuse, high-grade glioma with H3 K27 mutation at resection.](images/app.statdx.com_image_thumbnail_d2f0160e-2e45-46b9-8fbf-dca0386c913f_size_168_quality_85_a14097d5_20251014T204343Z.jpg)
**Diffuse High-Grade Glioma, Pediatric**
*Axial T1 C+ MR shows an enhancing mass in the posterior temporal lobe <img src='img/arrows/CS.png'/> with a satellite nodule <img src='img/arrows/CO.png'/>. This was a diffuse, high-grade glioma with H3 K27 mutation at resection.*
![Axial T1 C+ MR shows a large right frontal mass with central cystic change <img src='img/arrows/CS.png'/>. Supratentorial ependymomas are usually periventricular and are thought to arise from ependymal rests in the periventricular white matter.](images/app.statdx.com_image_thumbnail_92e53050-1be0-4dc1-b777-318c90a9c486_size_168_quality_85_d1ad2b0f_20251014T204343Z.jpg)
**Supratentorial Ependymoma**
*Axial T1 C+ MR shows a large right frontal mass with central cystic change <img src='img/arrows/CS.png'/>. Supratentorial ependymomas are usually periventricular and are thought to arise from ependymal rests in the periventricular white matter.*
![Axial T1 C+ MR shows a frond-like mass within the left temporal horn, consistent with a choroid plexus papilloma <img src='img/arrows/CS.png'/>. Choroid plexus tumor locations follow larger volumes of choroid: Lateral ventricle &gt; 4th &gt; 3rd.](images/app.statdx.com_image_thumbnail_db36a0da-f032-43e5-9554-f8fb4031eab7_size_168_quality_85_9670e677_20251014T204343Z.jpg)
**Choroid Plexus Tumor**
*Axial T1 C+ MR shows a frond-like mass within the left temporal horn, consistent with a choroid plexus papilloma <img src='img/arrows/CS.png'/>. Choroid plexus tumor locations follow larger volumes of choroid: Lateral ventricle &gt; 4th &gt; 3rd.*
![Axial T1 C+ MR shows bilateral enhancing tumors in the internal auditory canals compressing the brainstem <img src='img/arrows/CS.png'/>, consistent with bilateral vestibular schwannomas, pathognomonic for neurofibromatosis type 2. Search for additional cranial nerve schwannomas and dural margin meningiomas.](images/app.statdx.com_image_thumbnail_74f3e9d2-12a3-4330-917a-4dfc34343f24_size_168_quality_85_115b4c52_20251014T204343Z.jpg)
**Neurofibromatosis Type 2**
*Axial T1 C+ MR shows bilateral enhancing tumors in the internal auditory canals compressing the brainstem <img src='img/arrows/CS.png'/>, consistent with bilateral vestibular schwannomas, pathognomonic for neurofibromatosis type 2. Search for additional cranial nerve schwannomas and dural margin meningiomas.*
![Sagittal NECT shows a heterogeneous mass with fat <img src='img/arrows/CS.png'/> and calcification <img src='img/arrows/CO.png'/> in the pineal region extending to the 3rd ventricle, consistent with mature teratoma. There is a small amount of intraventricular air <img src='img/arrows/CC.png'/> from recent biopsy.](images/app.statdx.com_image_thumbnail_d82b2455-5b7f-454c-a3dd-b2ef4f6508f4_size_168_quality_85_cf6ccbdb_20251014T204343Z.jpg)
**Germ Cell Tumor**
*Sagittal NECT shows a heterogeneous mass with fat <img src='img/arrows/CS.png'/> and calcification <img src='img/arrows/CO.png'/> in the pineal region extending to the 3rd ventricle, consistent with mature teratoma. There is a small amount of intraventricular air <img src='img/arrows/CC.png'/> from recent biopsy.*
![Sagittal T1 C+ MR shows a large, enhancing mass in the pineal region <img src='img/arrows/CS.png'/>. This was a pineoblastoma at resection. High-grade pineoblastomas are the most common pineal parenchymal tumor.](images/app.statdx.com_image_thumbnail_1499f3f7-61ea-4f44-a9ae-010562214e7f_size_168_quality_85_a26f29e2_20251014T204343Z.jpg)
**Pineoblastoma**
*Sagittal T1 C+ MR shows a large, enhancing mass in the pineal region <img src='img/arrows/CS.png'/>. This was a pineoblastoma at resection. High-grade pineoblastomas are the most common pineal parenchymal tumor.*
![Axial T1 C+ MR shows a heterogeneous mass in the inferior left frontal lobe <img src='img/arrows/CS.png'/>. This was an atypical teratoid-rhabdoid tumor at resection. DWI images demonstrate decreased diffusion (not shown), consistent with a high-grade neoplasm.](images/app.statdx.com_image_thumbnail_1a24c7ba-aa5a-46e3-870f-71a06c05110d_size_168_quality_85_ff31278b_20251014T204343Z.jpg)
**Atypical Teratoid-Rhabdoid Tumor**
*Axial T1 C+ MR shows a heterogeneous mass in the inferior left frontal lobe <img src='img/arrows/CS.png'/>. This was an atypical teratoid-rhabdoid tumor at resection. DWI images demonstrate decreased diffusion (not shown), consistent with a high-grade neoplasm.*
![Axial T1 C+ MR shows a large, heterogeneous mass <img src='img/arrows/CS.png'/> with a solid periphery involving the cortex and central cyst. There is resulting subfalcine herniation. Although rare, &quot;bubbly,&quot; solid peripheral tumors with a cyst are the typical appearance for astroblastomas.](images/app.statdx.com_image_thumbnail_f03d0fb7-86af-4613-9da6-fe433d4e3bc0_size_168_quality_85_b7c987f9_20251014T204343Z.jpg)
**Astroblastoma, MN1-Altered**
*Axial T1 C+ MR shows a large, heterogeneous mass <img src='img/arrows/CS.png'/> with a solid periphery involving the cortex and central cyst. There is resulting subfalcine herniation. Although rare, &quot;bubbly,&quot; solid peripheral tumors with a cyst are the typical appearance for astroblastomas.*
### Additional Images
![Axial NECT shows a typical midline cystic tumor with a large, low-density mural nodule <img src='img/arrows/WS.png'/>. There is hydrocephalus with interstitial edema. Low density on CT is typical of low-grade tumors.](6db0d994-a8ca-4088-8354-9f1030b7189e)
**Pilocytic Astrocytoma**
*Axial NECT shows a typical midline cystic tumor with a large, low-density mural nodule <img src='img/arrows/WS.png'/>. There is hydrocephalus with interstitial edema. Low density on CT is typical of low-grade tumors.*
![Axial T2 MR shows the nodule to be high signal intensity, a clue to the low nuclear:cytoplasmic ratio in cerebellar juvenile pilocytic astrocytoma tumors.](2de47a6c-a815-48d1-9def-b79b2e3bf6a4)
**Pilocytic Astrocytoma**
*Axial T2 MR shows the nodule to be high signal intensity, a clue to the low nuclear:cytoplasmic ratio in cerebellar juvenile pilocytic astrocytoma tumors.*
![Axial T2 MR shows a large, hyperintense, well-circumscribed mass. It arises from the hypothalamic region and demonstrates no edema of adjacent structures. Pilomyxoid astrocytomas often arise from similar locations as pilocytic astrocytomas but are more locally aggressive with higher likelihood of leptomeningeal enhancement.](3b309892-a462-4829-8d1d-00f6c8654dfd)
**Pilocytic Astrocytoma**
*Axial T2 MR shows a large, hyperintense, well-circumscribed mass. It arises from the hypothalamic region and demonstrates no edema of adjacent structures. Pilomyxoid astrocytomas often arise from similar locations as pilocytic astrocytomas but are more locally aggressive with higher likelihood of leptomeningeal enhancement.*
![Axial T1 C+ MR in a child with metastatic medulloblastoma shows enhancement of the interfoliate sulci <img src='img/arrows/WO.png'/> and circummesencephalic cistern <img src='img/arrows/WS.png'/>.](083fb0ab-8fd3-40b8-a93d-2f14d844a131)
**Medulloblastoma**
*Axial T1 C+ MR in a child with metastatic medulloblastoma shows enhancement of the interfoliate sulci <img src='img/arrows/WO.png'/> and circummesencephalic cistern <img src='img/arrows/WS.png'/>.*
![Axial T2 MR shows a low-signal midline tumor. There is an associated cyst <img src='img/arrows/WS.png'/>. Low T2 signal of the solid component of tumors typically correlates with higher grade malignancy.](22ed3293-8259-4521-b363-3c1a25db859f)
**Medulloblastoma**
*Axial T2 MR shows a low-signal midline tumor. There is an associated cyst <img src='img/arrows/WS.png'/>. Low T2 signal of the solid component of tumors typically correlates with higher grade malignancy.*
![Axial DWI MR shows diffusion restriction within the tumor nodule, an excellent clue to the high-grade nature of the lesion.](fe926a7d-9054-4b86-8c7f-1402c7b4b0be)
**Medulloblastoma**
*Axial DWI MR shows diffusion restriction within the tumor nodule, an excellent clue to the high-grade nature of the lesion.*
![Axial CECT in metastatic medulloblastoma shows comb-like enhancement of the interfoliate sulci <img src='img/arrows/BO.png'/>. Note moderately enlarged lateral ventricles <img src='img/arrows/WS.png'/> caused by extraventricular obstructive hydrocephalus from diffuse cisternal metastases.](5728e334-2250-4038-863a-49e3ea92455c)
**Medulloblastoma**
*Axial CECT in metastatic medulloblastoma shows comb-like enhancement of the interfoliate sulci <img src='img/arrows/BO.png'/>. Note moderately enlarged lateral ventricles <img src='img/arrows/WS.png'/> caused by extraventricular obstructive hydrocephalus from diffuse cisternal metastases.*
![Sagittal T2 MR shows a large, heterogeneous, low-signal mass that widens the tegmenta-cerebellar angle and extends through the inferior recesses of the 4th ventricle. There is extension into the upper cervical spinal canal <img src='img/arrows/WS.png'/>.](4fd86ae6-8cb7-4533-ab4b-b047aed83248)
**Ependymoma**
*Sagittal T2 MR shows a large, heterogeneous, low-signal mass that widens the tegmenta-cerebellar angle and extends through the inferior recesses of the 4th ventricle. There is extension into the upper cervical spinal canal <img src='img/arrows/WS.png'/>.*
![Sagittal T2 MR shows diffuse expansion of the pons and medulla due to an infiltrating glioma.](4ac016a6-71b7-4a22-aec3-f3885c1c4124)
**Diffuse Midline Glioma, H3 K27-Altered**
*Sagittal T2 MR shows diffuse expansion of the pons and medulla due to an infiltrating glioma.*
![Axial FLAIR MR shows brainstem expansion and signal increase. The tumor expands to engulf the basilar artery <img src='img/arrows/WC.png'/>.](339caceb-bde3-4143-b280-80a589361ee7)
**Diffuse Midline Glioma, H3 K27-Altered**
*Axial FLAIR MR shows brainstem expansion and signal increase. The tumor expands to engulf the basilar artery <img src='img/arrows/WC.png'/>.*
![Coronal T2 MR shows an ill-defined, T2-hyperintense infiltrating mass involving the basal ganglia, temporal lobe, and frontal lobes <img src='img/arrows/WS.png'/>. This is typical of diffuse astrocytomas and was WHO grade 2 at biopsy. The term gliomatosis cerebri, which described a diffusely infiltrating astrocytoma involving at least 3 lobes of the brain, has been removed for the 2016 WHO classification of CNS tumors.](9fe5d1e3-39b3-4d15-8c23-08bd70f62752)
**Diffuse Low-Grade Glioma, Pediatric**
*Coronal T2 MR shows an ill-defined, T2-hyperintense infiltrating mass involving the basal ganglia, temporal lobe, and frontal lobes <img src='img/arrows/WS.png'/>. This is typical of diffuse astrocytomas and was WHO grade 2 at biopsy. The term gliomatosis cerebri, which described a diffusely infiltrating astrocytoma involving at least 3 lobes of the brain, has been removed for the 2016 WHO classification of CNS tumors.*
![Sagittal T1 MR shows a suprasellar collection of cysts of many signal intensities. One <img src='img/arrows/WC.png'/> is very high signal intensity, likely due to protein; another extends behind the clivus <img src='img/arrows/WO.png'/>; and the remainder herniate into the 3rd ventricle. Calcification <img src='img/arrows/WS.png'/> is noted in the solid component above the dorsum sella.](91bacdd7-bde0-46b3-889a-7c28bd302ef8)
**Craniopharyngioma**
*Sagittal T1 MR shows a suprasellar collection of cysts of many signal intensities. One <img src='img/arrows/WC.png'/> is very high signal intensity, likely due to protein; another extends behind the clivus <img src='img/arrows/WO.png'/>; and the remainder herniate into the 3rd ventricle. Calcification <img src='img/arrows/WS.png'/> is noted in the solid component above the dorsum sella.*
![Coronal T2 MR shows a mixed solid <img src='img/arrows/WS.png'/>, cystic <img src='img/arrows/BO.png'/>, and calcified <img src='img/arrows/BC.png'/> suprasellar tumor, which herniates into the 3rd ventricle and causes obstructive hydrocephalus.](fb763f2b-5760-48cf-86c3-fdae3317e12c)
**Craniopharyngioma**
*Coronal T2 MR shows a mixed solid <img src='img/arrows/WS.png'/>, cystic <img src='img/arrows/BO.png'/>, and calcified <img src='img/arrows/BC.png'/> suprasellar tumor, which herniates into the 3rd ventricle and causes obstructive hydrocephalus.*
![Coronal T1 C+ MR shows bilateral, asymmetric enhancing lesions at the foramina of Monro. The location is characteristic for subependymal giant cell astrocytoma. The child also had skin and other brain lesions typical of tuberous sclerosis.](52eed33a-4d03-419b-b69d-af32aeac9836)
**Subependymal Giant Cell Astrocytoma**
*Coronal T1 C+ MR shows bilateral, asymmetric enhancing lesions at the foramina of Monro. The location is characteristic for subependymal giant cell astrocytoma. The child also had skin and other brain lesions typical of tuberous sclerosis.*
![Sagittal T1 C+ MR shows a medium-sized pineal mass with central necrosis <img src='img/arrows/WS.png'/>. There is a very small, enhancing mass in the infundibular recess <img src='img/arrows/WC.png'/>.](67337622-c771-498d-aeb5-0c326eeaa377)
**Germ Cell Tumor**
*Sagittal T1 C+ MR shows a medium-sized pineal mass with central necrosis <img src='img/arrows/WS.png'/>. There is a very small, enhancing mass in the infundibular recess <img src='img/arrows/WC.png'/>.*
![Axial NECT shows a dense lesion, correlating with the increased cellularity known to be present in pineoblastoma.](cde43e1d-8f00-4024-86ed-afc5c6ca96d8)
**Pineoblastoma**
*Axial NECT shows a dense lesion, correlating with the increased cellularity known to be present in pineoblastoma.*
![Sagittal T2 MR shows a low-signal pineal mass that obstructs the aqueduct. This lesion was dense on NECT and restricted on DWI.](b764169f-9ee7-4fef-8663-e94cc3945233)
**Pineoblastoma**
*Sagittal T2 MR shows a low-signal pineal mass that obstructs the aqueduct. This lesion was dense on NECT and restricted on DWI.*
![Coronal FLAIR MR in a child with seizures shows an insular-based lesion with a partial bright ring <img src='img/arrows/WS.png'/>, the dysembryoplastic neuroepithelial tumor FLAIR ring sign.](65f01b7f-7872-4821-a565-3bedba72fab6)
**Dysembryoplastic Neuroepithelial Tumor**
*Coronal FLAIR MR in a child with seizures shows an insular-based lesion with a partial bright ring <img src='img/arrows/WS.png'/>, the dysembryoplastic neuroepithelial tumor FLAIR ring sign.*
![Axial T1 C+ MR shows a very low-density, nonenhancing dysembryoplastic neuroepithelial tumor <img src='img/arrows/WS.png'/>.](24712378-3b82-4c23-80ba-8532c5a060a3)
**Dysembryoplastic Neuroepithelial Tumor**
*Axial T1 C+ MR shows a very low-density, nonenhancing dysembryoplastic neuroepithelial tumor <img src='img/arrows/WS.png'/>.*
![Coronal T1 C+ MR shows a cortically based temporal lobe tumor. It is ill defined, invades adjacent brain tissue, enhances, and contains a rim-enhancing cyst <img src='img/arrows/WC.png'/>.](46b0b43a-53d2-4730-87eb-2cc9755f721d)
**Pleomorphic Xanthoastrocytoma**
*Coronal T1 C+ MR shows a cortically based temporal lobe tumor. It is ill defined, invades adjacent brain tissue, enhances, and contains a rim-enhancing cyst <img src='img/arrows/WC.png'/>.*
![Axial T1 C+ MR shows a cystic and solid thalamic mass. This lesion was heavily calcified on NECT (not shown).](54600ddb-8d57-4340-bfec-0c9eb76ebd84)
**Ganglioglioma**
*Axial T1 C+ MR shows a cystic and solid thalamic mass. This lesion was heavily calcified on NECT (not shown).*
![Axial DWI MR in the same case shows a lack of diffusion restriction, indicating a low-grade tumor.](070711f7-07ae-4b11-aba6-6df080d0a6c7)
**Ganglioglioma**
*Axial DWI MR in the same case shows a lack of diffusion restriction, indicating a low-grade tumor.*
![Axial T2 MR shows a cystic and solid thalamic ganglioglioma. The lesion was heavily calcified on NECT, but only a few calcifications <img src='img/arrows/WO.png'/> are identified on T2 MR imaging.](6b53f2ea-2b1b-4e2b-b5c2-e09dce1148c8)
**Ganglioglioma**
*Axial T2 MR shows a cystic and solid thalamic ganglioglioma. The lesion was heavily calcified on NECT, but only a few calcifications <img src='img/arrows/WO.png'/> are identified on T2 MR imaging.*
![Coronal T1WI MR shows marked expansion of the left temporal lobe by a hemorrhagic mass <img src='img/arrows/WO.png'/>.](images/app.statdx.com_image_thumbnail_7b3881d8-57ec-4ae8-b77e-c1610a39917b_size_168_quality_85_731cbae2_20251014T204343Z.jpg)
**CNS Embryonal Tumor**
*Coronal T1WI MR shows marked expansion of the left temporal lobe by a hemorrhagic mass <img src='img/arrows/WO.png'/>.*
![Axial T2 MR shows bithalamic involvement by homogeneous tumor, which did not enhance on T1 C+ MR (not shown).](00679681-f4d8-40d6-91e7-46f7c9509e45)
**Supratentorial Ependymoma**
*Axial T2 MR shows bithalamic involvement by homogeneous tumor, which did not enhance on T1 C+ MR (not shown).*
![Coronal T1 C+ MR shows a large, enhancing mass within the right lateral ventricle. The surface is frond-like, and there is no brain invasion. The appearance is typical for a choroid plexus papilloma.](583dfe2c-1780-45f0-be4a-f51d5c3db829)
**Choroid Plexus Tumor**
*Coronal T1 C+ MR shows a large, enhancing mass within the right lateral ventricle. The surface is frond-like, and there is no brain invasion. The appearance is typical for a choroid plexus papilloma.*
![Coronal T2 MR shows a large intraventricular mass with central necrosis <img src='img/arrows/BO.png'/> and speckled calcification <img src='img/arrows/BS.png'/>.](69f9525a-5030-4055-bf45-457bcc9e705f)
**Choroid Plexus Tumor**
*Coronal T2 MR shows a large intraventricular mass with central necrosis <img src='img/arrows/BO.png'/> and speckled calcification <img src='img/arrows/BS.png'/>.*
![Axial T1 C+ MR shows a large, heterogeneously enhancing trigonal mass with brain invasion and ependymal spread <img src='img/arrows/WO.png'/>.](7d57bb8f-652e-46f9-a19a-e2591ecd70c3)
**Choroid Plexus Tumor**
*Axial T1 C+ MR shows a large, heterogeneously enhancing trigonal mass with brain invasion and ependymal spread <img src='img/arrows/WO.png'/>.*
![Axial ADC map shows diffusion restriction in the same lesion.](cecf7dc5-a018-4803-a094-e7c4f381e1f6)
**Choroid Plexus Tumor**
*Axial ADC map shows diffusion restriction in the same lesion.*
![Coronal TI MR shows multiple dural-based meningiomas <img src='img/arrows/WO.png'/> at the vertex. There are also bilateral, asymmetric, vestibular schwannomas <img src='img/arrows/WC.png'/> in this teen with neurofibromatosis type 2.](fd777184-1a21-4d95-8050-7034ee2aab32)
**Neurofibromatosis Type 2**
*Coronal TI MR shows multiple dural-based meningiomas <img src='img/arrows/WO.png'/> at the vertex. There are also bilateral, asymmetric, vestibular schwannomas <img src='img/arrows/WC.png'/> in this teen with neurofibromatosis type 2.*
![Axial T2 MR shows a mixed-signal mass obstructing both the right <img src='img/arrows/WO.png'/> and left foramina of Monro.](26778d27-cf20-43ab-841c-4d6c6e80b0e3)
**Atypical Teratoid-Rhabdoid Tumor**
*Axial T2 MR shows a mixed-signal mass obstructing both the right <img src='img/arrows/WO.png'/> and left foramina of Monro.*
![Axial DWI MR in the same patient shows extensive diffusion restriction in the left frontal atypical teratoid-rhabdoid tumor.](26215a01-3d7b-470f-a1e9-1971b2d7fb0a)
**Atypical Teratoid-Rhabdoid Tumor**
*Axial DWI MR in the same patient shows extensive diffusion restriction in the left frontal atypical teratoid-rhabdoid tumor.*
![Axial T1 C+ MR shows a large, heterogeneously enhancing caudate mass.](f00ee573-8e84-4b2f-a381-bad0a62fffca)
**Atypical Teratoid-Rhabdoid Tumor**
*Axial T1 C+ MR shows a large, heterogeneously enhancing caudate mass.*
![Axial T1 MR shows a striated appearance of extremely low-signal dysplastic gangliocytoma, a.k.a. Lhermitte-Duclos disease. The gyri <img src='img/arrows/WO.png'/> are in disarray.](images/app.statdx.com_image_thumbnail_f3e0fb63-32d1-4231-a1eb-3df4dd255d2b_size_168_quality_85_55dc24fa_20251014T204343Z.jpg)
**Dysplastic Cerebellar Gangliocytoma**
*Axial T1 MR shows a striated appearance of extremely low-signal dysplastic gangliocytoma, a.k.a. Lhermitte-Duclos disease. The gyri <img src='img/arrows/WO.png'/> are in disarray.*
![Coronal FLAIR MR shows a T2-hyperintense mass <img src='img/arrows/CS.png'/> located in the right fusiform gyrus in this child presenting with intractable temporal lobe seizures. Gangliogliomas are a common cause of tumor-related temporal lobe seizures.](f8b61491-0316-4b62-842d-dba0222061ab)
**Ganglioglioma**
*Coronal FLAIR MR shows a T2-hyperintense mass <img src='img/arrows/CS.png'/> located in the right fusiform gyrus in this child presenting with intractable temporal lobe seizures. Gangliogliomas are a common cause of tumor-related temporal lobe seizures.*
![Axial FLAIR MR shows a well-circumscribed mass of the left cerebellar hemisphere. There is persistent faint visualization of cerebellar folia within the tumor <img src='img/arrows/CS.png'/>, consistent with dysplastic cerebellar gangliocytoma.](1fa91ac9-eb0e-49a1-b6ce-9c939bb2f357)
**Dysplastic Cerebellar Gangliocytoma**
*Axial FLAIR MR shows a well-circumscribed mass of the left cerebellar hemisphere. There is persistent faint visualization of cerebellar folia within the tumor <img src='img/arrows/CS.png'/>, consistent with dysplastic cerebellar gangliocytoma.*
![Axial T2 MR shows a heterogeneous, partially cystic mass along the left septum pellucidum <img src='img/arrows/CS.png'/>. This is a classic location of a central neurocytoma with a bubbly, cystic appearance.](images/app.statdx.com_image_thumbnail_0179d5bb-f659-46fd-9643-20411def45ef_size_168_quality_85_fa59c45e_20251014T204343Z.jpg)
**Central Neurocytoma**
*Axial T2 MR shows a heterogeneous, partially cystic mass along the left septum pellucidum <img src='img/arrows/CS.png'/>. This is a classic location of a central neurocytoma with a bubbly, cystic appearance.*
![Coronal T1 C+ MR shows a heterogeneously enhancing, centrally necrotic mass in the right thalamus <img src='img/arrows/CS.png'/>. This was an atypical teratoid-rhabdoid tumor at resection. These rare tumors will typically have decreased diffusion, consistent with high-grade malignancy.](a2791c89-ab9a-4597-b61f-6f5a3ee10571)
**Atypical Teratoid-Rhabdoid Tumor**
*Coronal T1 C+ MR shows a heterogeneously enhancing, centrally necrotic mass in the right thalamus <img src='img/arrows/CS.png'/>. This was an atypical teratoid-rhabdoid tumor at resection. These rare tumors will typically have decreased diffusion, consistent with high-grade malignancy.*
![Axial CT shows a calcified mass in the anterior 3rd ventricle <img src='img/arrows/CS.png'/> with associated hydrocephalus <img src='img/arrows/CO.png'/>. Although 3rd ventricular choroid plexus tumors are uncommon, dense calcification of an intraventricular tumor is suggestive of this entity.](b4167359-c5a9-4a47-85d6-69586bd734a6)
**Choroid Plexus Tumor**
*Axial CT shows a calcified mass in the anterior 3rd ventricle <img src='img/arrows/CS.png'/> with associated hydrocephalus <img src='img/arrows/CO.png'/>. Although 3rd ventricular choroid plexus tumors are uncommon, dense calcification of an intraventricular tumor is suggestive of this entity.*
![Coronal T1 C+ MR shows a dural-based, homogeneous enhancing, extraaxial mass, consistent with a meningioma <img src='img/arrows/CS.png'/>. Given the bilateral enhancing schwannomas in the internal auditory canals <img src='img/arrows/CO.png'/>, this is diagnostic of neurofibromatosis type 2.](e81db840-0051-49d6-b907-7fc276a0367f)
**Neurofibromatosis Type 2**
*Coronal T1 C+ MR shows a dural-based, homogeneous enhancing, extraaxial mass, consistent with a meningioma <img src='img/arrows/CS.png'/>. Given the bilateral enhancing schwannomas in the internal auditory canals <img src='img/arrows/CO.png'/>, this is diagnostic of neurofibromatosis type 2.*
![Axial T1 C+ MR shows a heterogeneously enhancing mass in the right parietal and occipital lobes, which invades the right lateral ventricle. By the 2016 WHO CNS tumor classification, PNETs are now CNS embryonal tumors. In young children, they tend to present as a large, lobar tumor.](4f9fdd70-dbda-49c1-8253-b3a8aa1bdb58)
**CNS Embryonal Tumor**
*Axial T1 C+ MR shows a heterogeneously enhancing mass in the right parietal and occipital lobes, which invades the right lateral ventricle. By the 2016 WHO CNS tumor classification, PNETs are now CNS embryonal tumors. In young children, they tend to present as a large, lobar tumor.*
![Axial T1 C+ MR shows a heterogeneously enhancing mass in the left insula, basal ganglia, and temporal lobe. There is central necrosis <img src='img/arrows/CS.png'/>. WHO 2016 CNS tumor classification has removed &quot;multiforme&quot; from glioblastoma.](d6602d98-c9f7-41cf-99e2-2be7e6bcada7)
**Supratentorial Ependymoma**
*Axial T1 C+ MR shows a heterogeneously enhancing mass in the left insula, basal ganglia, and temporal lobe. There is central necrosis <img src='img/arrows/CS.png'/>. WHO 2016 CNS tumor classification has removed &quot;multiforme&quot; from glioblastoma.*
![An rCBV map from DSC perfusion shows increased perfusion of the mass, indicating a high-grade tumor consistent with glioblastoma. Note the area of increased perfusion <img src='img/arrows/WS.png'/> that did not enhance on a conventional T1 C+ sequence, indicating nonenhancing tumor.](a1b5c69d-a2b9-4b57-bcf1-a2c613403231)
**Supratentorial Ependymoma**
*An rCBV map from DSC perfusion shows increased perfusion of the mass, indicating a high-grade tumor consistent with glioblastoma. Note the area of increased perfusion <img src='img/arrows/WS.png'/> that did not enhance on a conventional T1 C+ sequence, indicating nonenhancing tumor.*
![Axial T2 MR shows a well-circumscribed, peripheral, homogeneously T2-hyperintense mass <img src='img/arrows/CS.png'/> in the right frontal lobe involving both the cortex and subcortical white matter. The frontal lobe is the most common location for oligodendrogliomas.](images/app.statdx.com_image_thumbnail_05fd6861-db97-4ac5-bfc7-213d38504f6f_size_168_quality_85_6f68e586_20251014T204343Z.jpg)
**Oligodendroglioma**
*Axial T2 MR shows a well-circumscribed, peripheral, homogeneously T2-hyperintense mass <img src='img/arrows/CS.png'/> in the right frontal lobe involving both the cortex and subcortical white matter. The frontal lobe is the most common location for oligodendrogliomas.*
![Coronal T1 C+ MR shows a heterogeneous enhancing mass at the periphery of the left parietal lobe. Note enhancement of the overlying dura <img src='img/arrows/CS.png'/>, which is a characteristic finding of pleomorphic xanthoastrocytoma.](b5d40dcf-54d7-44a1-b476-69c9b08e6107)
**Pleomorphic Xanthoastrocytoma**
*Coronal T1 C+ MR shows a heterogeneous enhancing mass at the periphery of the left parietal lobe. Note enhancement of the overlying dura <img src='img/arrows/CS.png'/>, which is a characteristic finding of pleomorphic xanthoastrocytoma.*
![Axial T2 MR shows a &quot;bubbly&quot; mass <img src='img/arrows/CS.png'/> centered in the left temporal lobe, particularly involving the hippocampus and medial cortex. There is minimal to no adjacent T2 hyperintensity. This bubbly appearance is a typical appearance for dysembryoplastic neuroepithelial tumors.](8d2cb0a9-a5e9-45aa-9543-b80327ff26ac)
**Dysembryoplastic Neuroepithelial Tumor**
*Axial T2 MR shows a &quot;bubbly&quot; mass <img src='img/arrows/CS.png'/> centered in the left temporal lobe, particularly involving the hippocampus and medial cortex. There is minimal to no adjacent T2 hyperintensity. This bubbly appearance is a typical appearance for dysembryoplastic neuroepithelial tumors.*
![Coronal CT shows an iso- to hyperdense mass in the pineal region <img src='img/arrows/CS.png'/>. Note the peripheral &quot;exploded&quot; calcification <img src='img/arrows/CO.png'/>, consistent with a pineal parenchymal tumor, such as pineoblastoma in this case.](7d8b1d05-fa12-4208-b0c5-1cbb0552eac0)
**Pineoblastoma**
*Coronal CT shows an iso- to hyperdense mass in the pineal region <img src='img/arrows/CS.png'/>. Note the peripheral &quot;exploded&quot; calcification <img src='img/arrows/CO.png'/>, consistent with a pineal parenchymal tumor, such as pineoblastoma in this case.*
![Sagittal T1 C+ MR shows lobular enhancing masses in both the pineal region and suprasellar cistern <img src='img/arrows/CS.png'/>. Note the central dark structure <img src='img/arrows/CO.png'/>, consistent with engulfed pineal calcification within a germ cell tumor, specifically germinoma.](fb5be943-fff9-4c01-8d37-68f61f3c6a6f)
**Germ Cell Tumor**
*Sagittal T1 C+ MR shows lobular enhancing masses in both the pineal region and suprasellar cistern <img src='img/arrows/CS.png'/>. Note the central dark structure <img src='img/arrows/CO.png'/>, consistent with engulfed pineal calcification within a germ cell tumor, specifically germinoma.*
![Sagittal CT shows an isodense mass in the suprasellar cistern with coarse calcification <img src='img/arrows/CS.png'/>. Given the calcification and suggestion of cystic mass in the suprasellar cistern, craniopharyngioma is suspected.](b719c23f-1223-4021-8e36-b80d21853f39)
**Craniopharyngioma**
*Sagittal CT shows an isodense mass in the suprasellar cistern with coarse calcification <img src='img/arrows/CS.png'/>. Given the calcification and suggestion of cystic mass in the suprasellar cistern, craniopharyngioma is suspected.*
![Sagittal T1 C+ MR shows thin enhancement of the cyst wall. Thicker, solid enhancement is seen <img src='img/arrows/CS.png'/> above the flattened pituitary gland <img src='img/arrows/CO.png'/>, consistent with a unilocular craniopharyngioma.](daba1c00-6288-4200-9a8a-27a2849c6e93)
**Craniopharyngioma**
*Sagittal T1 C+ MR shows thin enhancement of the cyst wall. Thicker, solid enhancement is seen <img src='img/arrows/CS.png'/> above the flattened pituitary gland <img src='img/arrows/CO.png'/>, consistent with a unilocular craniopharyngioma.*
![Axial T1 C+ MR shows a heterogeneous enhancing mass at the right foramen of Monro <img src='img/arrows/CS.png'/>. Given the involvement of the septum pellucidum, a central neurocytoma can be considered. In a child with tuberous sclerosis, this is a subependymal giant cell astrocytoma.](04defc3a-8c19-402c-8ece-8a5163e9a8d1)
**Subependymal Giant Cell Astrocytoma**
*Axial T1 C+ MR shows a heterogeneous enhancing mass at the right foramen of Monro <img src='img/arrows/CS.png'/>. Given the involvement of the septum pellucidum, a central neurocytoma can be considered. In a child with tuberous sclerosis, this is a subependymal giant cell astrocytoma.*
![Coronal FLAIR MR shows a focal lesion with ill-defined borders in the left deep periventricular white matter <img src='img/arrows/CS.png'/>. This is a biopsy-proven low-grade astrocytoma.](e581c4ab-4ff2-4cbe-bffc-aad3e2c4bf84)
**Diffuse Low-Grade Glioma, Pediatric**
*Coronal FLAIR MR shows a focal lesion with ill-defined borders in the left deep periventricular white matter <img src='img/arrows/CS.png'/>. This is a biopsy-proven low-grade astrocytoma.*
![Axial FLAIR MR shows an expansile mass of the pons with diffuse involvement and expansion. Note the encasement of the basilar artery <img src='img/arrows/CS.png'/>. This appearance is typical of diffuse intrinsic pontine glioma, which typically has poor outcome with a median survival &lt; 1 year.](1ace3f07-6930-4ba2-a9a1-85b104eab66c)
**Diffuse Midline Glioma, H3 K27-Altered**
*Axial FLAIR MR shows an expansile mass of the pons with diffuse involvement and expansion. Note the encasement of the basilar artery <img src='img/arrows/CS.png'/>. This appearance is typical of diffuse intrinsic pontine glioma, which typically has poor outcome with a median survival &lt; 1 year.*
![Axial T2 MR shows a heterogeneous, hyperintense mass <img src='img/arrows/CS.png'/> expanding the right dorsal medulla and inferior cerebellar peduncle. Pedunculated focal brainstem gliomas are typically low grade with favorable outcome.](f99e2606-b7ed-415f-a5e8-1a13f2edf29e)
**Diffuse Midline Glioma, H3 K27-Altered**
*Axial T2 MR shows a heterogeneous, hyperintense mass <img src='img/arrows/CS.png'/> expanding the right dorsal medulla and inferior cerebellar peduncle. Pedunculated focal brainstem gliomas are typically low grade with favorable outcome.*
![Axial T2 MR shows a heterogeneous mass asymmetrically located in the inferior 4th ventricle and extending out of the right foramen of Luschka <img src='img/arrows/CS.png'/> with mass effect on the dorsal medulla. Extension of ependymoma through the 4th ventricle foramina is a typical finding.](4b983db8-2fc3-43ff-81eb-5e371b7f1d68)
**Ependymoma**
*Axial T2 MR shows a heterogeneous mass asymmetrically located in the inferior 4th ventricle and extending out of the right foramen of Luschka <img src='img/arrows/CS.png'/> with mass effect on the dorsal medulla. Extension of ependymoma through the 4th ventricle foramina is a typical finding.*
![Axial T2 MR shows a heterogeneous mass <img src='img/arrows/CS.png'/> within and expanding the 4th ventricle. The solid component is T2 iso- to hypointense to the brain parenchyma, which is typical of high-grade neoplasms like medulloblastoma.](b4ba467b-7703-410b-8dd6-9c9d0df9dac4)
**Medulloblastoma**
*Axial T2 MR shows a heterogeneous mass <img src='img/arrows/CS.png'/> within and expanding the 4th ventricle. The solid component is T2 iso- to hypointense to the brain parenchyma, which is typical of high-grade neoplasms like medulloblastoma.*
![Axial DWI MR shows hyperintensity <img src='img/arrows/CS.png'/> compared to surrounding cerebellum consistent with decreased diffusion (ADC map not shown). Medulloblastomas, like other high-grade tumors, have decreased diffusion.](4ccedcae-8bd9-44b1-9b0f-05ffd30fd3bc)
**Medulloblastoma**
*Axial DWI MR shows hyperintensity <img src='img/arrows/CS.png'/> compared to surrounding cerebellum consistent with decreased diffusion (ADC map not shown). Medulloblastomas, like other high-grade tumors, have decreased diffusion.*
![Sagittal 3D T1 C+ SPGR MR shows an enhancing mass <img src='img/arrows/CS.png'/> with a large cyst <img src='img/arrows/CO.png'/>, consistent with a classic cyst and mural nodule appearance. Note the effacement of the 4th ventricle <img src='img/arrows/CC.png'/>, indicating a cerebellar origin.](6e6be99b-9c07-4250-b1e3-1dbf00192b49)
**Pilocytic Astrocytoma**
*Sagittal 3D T1 C+ SPGR MR shows an enhancing mass <img src='img/arrows/CS.png'/> with a large cyst <img src='img/arrows/CO.png'/>, consistent with a classic cyst and mural nodule appearance. Note the effacement of the 4th ventricle <img src='img/arrows/CC.png'/>, indicating a cerebellar origin.*
docs_md/articles/brain-tumor-in-newborn-infant_12b32579-c99b-41c0-95fd-f2ad1fc4a4fd.md
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---
title: "Brain Tumor in Newborn/Infant"
docid: "12b32579-c99b-41c0-95fd-f2ad1fc4a4fd"
authors:
- key: "f184750a-90b4-47a7-907b-23b05d70357a"
value: "Chang Yueh Ho, MD"
breadcrumbs:
-
name: "Brain"
slug: "brain"
treeNodeId: "6d8829f1-14d7-45af-8675-255189aa526a"
-
name: "Differential Diagnosis"
slug: "differential-diagnosis"
treeNodeId: "a7fdd139-664e-4bb8-8d18-400e4733ff60"
-
name: "Brain Parenchyma, General"
slug: "brain-parenchyma-general"
treeNodeId: "e79be97b-28c0-4023-be87-334c0579d35d"
-
name: "Clinically Based Differentials"
slug: "clinically-based-differentials"
treeNodeId: "108519f7-93d7-4662-85dd-2239f2422821"
-
name: "Brain Tumor in Newborn/Infant"
slug: "brain-tumor-in-newborninfant"
treeNodeId: null
category: "Brain"
documentVersionId: "6d309907-eb33-42ec-abac-50970b8269ba"
imageCount: 71
lastUpdated: "01/25/23"
pageDescription: "Brain Tumor in Newborn/Infant"
pageKeywords: "Brain, Differential Diagnosis, Brain Parenchyma, General, Clinically Based Differentials, Brain Tumor in Newborn/Infant"
pageTitle: "Brain Tumor in Newborn/Infant | STATdx"
enhancedTitle: "Brain Tumor in Newborn/Infant"
type: "DDX"
references: true
breadcrumbs:
- "Brain"
- "Differential Diagnosis"
- "Brain Parenchyma, General"
- "Clinically Based Differentials"
- "Brain Tumor in Newborn/Infant"
---
# ESSENTIAL INFORMATION
- ## Key Differential Diagnosis Issues
- Newborn/infant brain tumors
- Typically large, bulky, inhomogeneous
- 60-70% supratentorial
- Infratentorial more common in older children
- Immature, high-grade tumors more common
- ## Helpful Clues for Common Diagnoses
- **Teratoma**
- Most common fetal and congenital brain tumor
- Midline, supratentorial
- Small, lobular or holocranial
- Contents
- Ca⁺⁺, cysts
- Fat in mature teratoma, less commonly in immature teratoma; enhancing soft tissue
- Look for associated congenital brain anomalies
- **Infant-Type Hemispheric Glioma**
- Large, heterogeneous, hemispheric
- High-grade cellular astrocytoma
- Better outcome than other histone-associated pediatric high-grade gliomas
- **Medulloblastoma**
- SHH-activated and non-WNT/non-SHH (group 3) more common in infants
- Posterior fossa mass with hydrocephalus
- Restricts on DWI (best MR clue)
- Enhancement usual (may be late/slow)
- Sparse Ca⁺⁺: ~ 20%; hemorrhage rare
- Hypercellularity reflected on imaging
- Hyperdense (NECT), hypointense (T2)
- SHH-activated
- Cerebellar hemisphere, not centered in 4th ventricle
- Intense enhancement
- Additional *TP53* mutation carries poor prognosis
- Group 3
- Classic 4th ventricular location
- Enhancement common, group 4 has less enhancement
- Poor prognosis when presenting with dissemination
- **Ependymoma,****Posterior Fossa Type A**
- Posterior fossa A ependymomas characterized by ↓ H3 K27 expression
- Younger children, poor outcome
- Lateral in 4th ventricle, extends through foramina of Luschka
- Heterogeneous enhancement
- Ca⁺⁺ ± hemorrhage
- **Supratentorial Ependymoma**
- Periventricular/extraventricular > intraventricular
- Derived from periventricular ependymal rests
- Large, bulky; Ca⁺⁺: ~ 50%
- Variable necrosis, hemorrhage
- *ZFTA* fusion-positive: Seen in infants and older children, poor prognosis
- *YAP1* fusion-positive: Usually seen in infants, good prognosis
- **Choroid Plexus Papilloma**
- Choroid plexus papilloma (CPP): Lobulated intraventricular mass
- Lateral > 4th > 3rd
- NECT: Isointense to dense
- Isointense to slightly hyperintense on T2WI
- Vividly enhancing
- Hydrocephalus common
- ## Helpful Clues for Less Common Diagnoses
- **Pilocytic Astrocytoma, Pilomyxoid Variant**
- Younger age presentation than typical pilocytic astrocytoma (PA)
- Often presents as large, enhancing, infiltrative mass involving optic pathway
- Despite low-grade tumor, it can have leptomeningeal seeding
- **Atypical Teratoid-Rhabdoid Tumor**
- Medulloblastoma-like, +
- Metastases at diagnosis more common
- Cysts, hemorrhage more common
- Variable contrast enhancement
- Cerebellopontine angle cistern location more common
- Seeding via CSF pathway common
- **CNS Embryonal Tumor**
- Previously primitive neuroectodermal tumor (PNET)
- Large, complex mass
- Restricts on DWI
- Heterogeneous signal enhancement
- Ca⁺⁺ more common than in posterior fossa PNETs
- Hemorrhage, necrosis common
- Hemispheric
- Mean diameter: 5 cm
- Especially newborn/infants
- Minimal peritumoral edema
- Suprasellar: Early neuroendocrine, visual disturbances
- **Desmoplastic Infantile Ganglioglioma/Astrocytoma**
- Desmoplastic infantile gangliogliomas(DIGs)/astrocytoma often have large cyst
- Cortically based, enhancing tumor nodule
- Enhancing adjacent pia and dura; low grade
- Good outcome with complete surgical resection
- ## Helpful Clues for Rare Diagnoses
- **Choroid Plexus Carcinoma**
- Similar to CPP, +
- Brain invasion; Ca⁺⁺, cysts, bleed
- Ependymal, subarachnoid space seeding (can be seen with both CPP, choroid plexus carcinoma)
- **Embryonal Tumor With Multilayered Rosettes**
- Rare malignant embryonal brain tumor
- Young children (< 5 years)
- Histologic differentiation varies
- Neuronal, astrocytic, ependymal, melanotic, etc.
- Imaging appearance reflects variable differentiation
- Medulloepithelioma, ependymoblastoma, and embryonal tumor with abundant neuropil and true rosettes (ETANTR) all have similar molecular features and are grouped as embryonal tumor with multilayered rosettes (ETMR)
- **Neurocutaneous Melanosis (Melanoma/Melanocytoma)**
- Giant or multiple cutaneous melanocytic nevi, +
- Melanosis: Bright T1 lesions in amygdala, cerebellum without fat saturation
- T2 hypointense to isointense, no enhancement
- Melanoma: Melanosis + diffuse leptomeningeal enhancement
- Degeneration into malignant melanoma common
## References
# Selected References
1. [Louis DN et al: The 2021 WHO Classification of Tumors of the Central Nervous System: a summary. Neuro Oncol. 23(8):1231-51, 2021](http://www.ncbi.nlm.nih.gov/pubmed/?term=34185076%5Bpmid%5D)
1. [Clarke M et al: Infant high-grade gliomas comprise multiple subgroups characterized by novel targetable gene fusions and favorable outcomes. Cancer Discov. 10(7):942-63, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=32238360%5Bpmid%5D)
1. [Lambo S et al: ETMR: a tumor entity in its infancy. Acta Neuropathol. 140(3):249-66, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=32601913%5Bpmid%5D)
1. [Gessi M et al: Medulloblastoma with extensive nodularity: a tumor exclusively of infancy? Neuropathol Appl Neurobiol. 43(3):267-70, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=26990710%5Bpmid%5D)
1. [Shekdar KV et al: Brain tumors in the neonate. Neuroimaging Clin N Am. 27(1):69-83, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=27889024%5Bpmid%5D)
1. [Munjal S et al: Infant brain tumours: a tale of two cities. Childs Nerv Syst. 32(9):1633-40, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=27299432%5Bpmid%5D)
1. [Kralik SF et al: Diffusion imaging for tumor grading of supratentorial brain tumors in the first year of life. AJNR Am J Neuroradiol. 35(4):815-23, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=24200900%5Bpmid%5D)
## Images
### Selected Images
![Axial T1 C+ MR shows a large, enhancing mass in a temporal off-midline location <img src='img/arrows/CS.png'/> in a neonate with maximal hydrocephalus and layering blood products <img src='img/arrows/CO.png'/> as well as cellular debris <img src='img/arrows/CC.png'/>.](images/app.statdx.com_image_thumbnail_e9986857-8a55-4f23-bb55-fe7c4110e35f_size_168_quality_85_cf9d7859_20251014T202907Z.jpg)
**Teratoma**
*Axial T1 C+ MR shows a large, enhancing mass in a temporal off-midline location <img src='img/arrows/CS.png'/> in a neonate with maximal hydrocephalus and layering blood products <img src='img/arrows/CO.png'/> as well as cellular debris <img src='img/arrows/CC.png'/>.*
![Axial T1 C+ MR shows a large, enhancing mass in a temporal off-midline location <img src='img/arrows/CS.png'/> in a neonate with maximal hydrocephalus and layering blood products <img src='img/arrows/CO.png'/> as well as cellular debris <img src='img/arrows/CC.png'/>.](images/app.statdx.com_image_thumbnail_e9986857-8a55-4f23-bb55-fe7c4110e35f_size_174_quality_85_089cba09_20251014T200550Z.jpg)
**Teratoma**
*Axial T1 C+ MR shows a large, enhancing mass in a temporal off-midline location <img src='img/arrows/CS.png'/> in a neonate with maximal hydrocephalus and layering blood products <img src='img/arrows/CO.png'/> as well as cellular debris <img src='img/arrows/CC.png'/>.*
![Axial T1 C+ MR shows a large, enhancing mass in a temporal off-midline location <img src='img/arrows/CS.png'/> in a neonate with maximal hydrocephalus and layering blood products <img src='img/arrows/CO.png'/> as well as cellular debris <img src='img/arrows/CC.png'/>.](images/app.statdx.com_image_thumbnail_e9986857-8a55-4f23-bb55-fe7c4110e35f_size_174_quality_85_575cce2d_20251014T202417Z.jpg)
**Teratoma**
*Axial T1 C+ MR shows a large, enhancing mass in a temporal off-midline location <img src='img/arrows/CS.png'/> in a neonate with maximal hydrocephalus and layering blood products <img src='img/arrows/CO.png'/> as well as cellular debris <img src='img/arrows/CC.png'/>.*
![Axial T1 C+ MR shows a large, enhancing mass in a temporal off-midline location <img src='img/arrows/CS.png'/> in a neonate with maximal hydrocephalus and layering blood products <img src='img/arrows/CO.png'/> as well as cellular debris <img src='img/arrows/CC.png'/>.](images/app.statdx.com_image_thumbnail_e9986857-8a55-4f23-bb55-fe7c4110e35f_size_174_quality_85_a4053efc_20251014T195930Z.jpg)
**Teratoma**
*Axial T1 C+ MR shows a large, enhancing mass in a temporal off-midline location <img src='img/arrows/CS.png'/> in a neonate with maximal hydrocephalus and layering blood products <img src='img/arrows/CO.png'/> as well as cellular debris <img src='img/arrows/CC.png'/>.*
![Coronal T2 MR shows the heterogeneous mass in the left middle cranial fossa <img src='img/arrows/CS.png'/> with severe hydrocephalus and a thinned brain mantle <img src='img/arrows/CO.png'/>. Fetal teratomas often have poor outcome due to compression of the brain.](4b32679c-86b1-4829-9d90-38d1f4202cf8)
**Teratoma**
*Coronal T2 MR shows the heterogeneous mass in the left middle cranial fossa <img src='img/arrows/CS.png'/> with severe hydrocephalus and a thinned brain mantle <img src='img/arrows/CO.png'/>. Fetal teratomas often have poor outcome due to compression of the brain.*
![Axial T2 MR shows a large, heterogeneous mass with central necrosis and blood products <img src='img/arrows/CS.png'/> causing midline shift <img src='img/arrows/CO.png'/> and hydrocephalus <img src='img/arrows/CC.png'/>.](images/app.statdx.com_image_thumbnail_e090103e-8471-4879-b60a-415bddbd4638_size_168_quality_85_37f2b9a3_20251014T202907Z.jpg)
**Infant-Type Hemispheric Glioma**
*Axial T2 MR shows a large, heterogeneous mass with central necrosis and blood products <img src='img/arrows/CS.png'/> causing midline shift <img src='img/arrows/CO.png'/> and hydrocephalus <img src='img/arrows/CC.png'/>.*
![Axial T1 C+ MR shows heterogeneous enhancement of the large left frontal lobe. In an infant, this is consistent with an infant-type hemispheric glioma, a high-grade tumor characterized by NTRK, ROS1, ALK, or MET alteration of tyrosine kinases.](5110257b-1b64-4cbb-a8c5-6813d1d3b3b1)
**Infant-Type Hemispheric Glioma**
*Axial T1 C+ MR shows heterogeneous enhancement of the large left frontal lobe. In an infant, this is consistent with an infant-type hemispheric glioma, a high-grade tumor characterized by NTRK, ROS1, ALK, or MET alteration of tyrosine kinases.*
![Axial T1 C+ MR shows multiple nodules in the right middle cerebellar peduncle <img src='img/arrows/CS.png'/> and vermis <img src='img/arrows/CO.png'/> in an infant. This was an SHH desmoplastic medulloblastoma. Desmoplastic histology is associated with SHH pathway alteration and can be nodular in appearance.](images/app.statdx.com_image_thumbnail_cf310618-3177-4075-9c07-96d4c1f7317d_size_168_quality_85_77e6b6de_20251014T202907Z.jpg)
**Medulloblastoma**
*Axial T1 C+ MR shows multiple nodules in the right middle cerebellar peduncle <img src='img/arrows/CS.png'/> and vermis <img src='img/arrows/CO.png'/> in an infant. This was an SHH desmoplastic medulloblastoma. Desmoplastic histology is associated with SHH pathway alteration and can be nodular in appearance.*
![Axial b=1000 DWI MR shows the nodular masses <img src='img/arrows/CS.png'/> have decreased diffusion compatible with high-grade neoplasm. SHH medulloblastomas have variable outcomes with a TP53 mutation associated with poor outcome.](d81fe497-e613-4fbe-8af4-508133d77869)
**Medulloblastoma**
*Axial b=1000 DWI MR shows the nodular masses <img src='img/arrows/CS.png'/> have decreased diffusion compatible with high-grade neoplasm. SHH medulloblastomas have variable outcomes with a TP53 mutation associated with poor outcome.*
![Axial T2 MR shows a heterogeneous mass in the 4th ventricle extending laterally through the left foramen of Luschka <img src='img/arrows/CS.png'/>. This was a posterior fossa type A (PFA) ependymoma at resection. Ependymomas have a &quot;toothpaste&quot; propensity to extend through the 4th ventricular foramina.](images/app.statdx.com_image_thumbnail_f71a23c5-abf3-4195-8876-6015ca3322fa_size_168_quality_85_afd816a6_20251014T202907Z.jpg)
**Ependymoma, Posterior Fossa Type A**
*Axial T2 MR shows a heterogeneous mass in the 4th ventricle extending laterally through the left foramen of Luschka <img src='img/arrows/CS.png'/>. This was a posterior fossa type A (PFA) ependymoma at resection. Ependymomas have a &quot;toothpaste&quot; propensity to extend through the 4th ventricular foramina.*
![Axial T1 C+ MR shows the heterogeneous enhancement of the PFA ependymoma. PFA ependymomas tend to occur in young children and carry a worse prognosis than posterior fossa type B (PFB) ependymomas.](f24657ee-4998-4e18-9cce-f55969620d5d)
**Ependymoma, Posterior Fossa Type A**
*Axial T1 C+ MR shows the heterogeneous enhancement of the PFA ependymoma. PFA ependymomas tend to occur in young children and carry a worse prognosis than posterior fossa type B (PFB) ependymomas.*
![Axial T2 TSE FS MR shows a heterogeneous mass in a periventricular location <img src='img/arrows/CS.png'/>. Supratentorial ependymomas are thought to arise from periventricular ependymal rests.](images/app.statdx.com_image_thumbnail_a223d4e2-2022-4565-86a2-57646d7cd016_size_168_quality_85_eb0c48e6_20251014T202907Z.jpg)
**Supratentorial Ependymoma**
*Axial T2 TSE FS MR shows a heterogeneous mass in a periventricular location <img src='img/arrows/CS.png'/>. Supratentorial ependymomas are thought to arise from periventricular ependymal rests.*
![Axial T1 C+ MR shows cystic and heterogeneous enhancement in this ZFTA fusion supratentorial ependymoma. This subtype has a worse prognosis than the more rare YAP1 subgroup, which tends to present in younger children.](e5181b76-8c5a-4d12-9160-9a8c90d4883a)
**Supratentorial Ependymoma**
*Axial T1 C+ MR shows cystic and heterogeneous enhancement in this ZFTA fusion supratentorial ependymoma. This subtype has a worse prognosis than the more rare YAP1 subgroup, which tends to present in younger children.*
![Axial T2 MR shows a large intraventricular mass coinciding with the left lateral choroid plexus glomus <img src='img/arrows/CS.png'/>. The mass has decreased T2 signal, which may represent Ca⁺⁺ or blood products. There is obstructive hydrocephalus, which is common in choroid plexus tumors.](images/app.statdx.com_image_thumbnail_3c1c0f6d-ca80-4067-9cbd-066eab94aae8_size_168_quality_85_86e83f36_20251014T202907Z.jpg)
**Choroid Plexus Papilloma**
*Axial T2 MR shows a large intraventricular mass coinciding with the left lateral choroid plexus glomus <img src='img/arrows/CS.png'/>. The mass has decreased T2 signal, which may represent Ca⁺⁺ or blood products. There is obstructive hydrocephalus, which is common in choroid plexus tumors.*
![Coronal T1 C+ MR shows a heterogeneous, lobular, enhancing mass in the left lateral ventricle causing obstructive hydrocephalus. At resection, there was an atypical choroid plexus papilloma (CPP), WHO grade 2.](742d993a-4aae-497d-bb99-c91bd6700c12)
**Choroid Plexus Papilloma**
*Coronal T1 C+ MR shows a heterogeneous, lobular, enhancing mass in the left lateral ventricle causing obstructive hydrocephalus. At resection, there was an atypical choroid plexus papilloma (CPP), WHO grade 2.*
![Coronal T1 C+ MR shows a large left hemispheric mass with solid enhancement centrally <img src='img/arrows/CS.png'/> and cystic component <img src='img/arrows/CO.png'/> peripherally. Pilomyxoid variant of pilocytic astrocytomas may present in younger patients, involve the optic pathway, and are more locally aggressive.](images/app.statdx.com_image_thumbnail_b514ee9c-d337-4a23-8b6b-16de14182cb1_size_168_quality_85_8c1075d4_20251014T202907Z.jpg)
**Pilocytic Astrocytoma, Pilomyxoid Variant**
*Coronal T1 C+ MR shows a large left hemispheric mass with solid enhancement centrally <img src='img/arrows/CS.png'/> and cystic component <img src='img/arrows/CO.png'/> peripherally. Pilomyxoid variant of pilocytic astrocytomas may present in younger patients, involve the optic pathway, and are more locally aggressive.*
![Axial T2 MR shows the mass centered in the central gray nuclei with midline shift <img src='img/arrows/CS.png'/>. The solid component has relative increased T2 content <img src='img/arrows/CO.png'/>, suggesting a low-grade neoplasm.](07e8e8ea-ea3b-46b5-87dc-dbba2194be69)
**Pilocytic Astrocytoma, Pilomyxoid Variant**
*Axial T2 MR shows the mass centered in the central gray nuclei with midline shift <img src='img/arrows/CS.png'/>. The solid component has relative increased T2 content <img src='img/arrows/CO.png'/>, suggesting a low-grade neoplasm.*
![Axial b=1000 DWI MR shows increased diffusion of the solid tumor portions <img src='img/arrows/CS.png'/>, consistent with the low-grade nature of pilomyxoid astrocytoma. In infants, DWI is helpful in differentiating high- vs. low-grade tumors that tend to be large, heterogeneous, and hemispheric.](66fd89ba-1824-4a9f-b524-9d1e81cec200)
**Pilocytic Astrocytoma, Pilomyxoid Variant**
*Axial b=1000 DWI MR shows increased diffusion of the solid tumor portions <img src='img/arrows/CS.png'/>, consistent with the low-grade nature of pilomyxoid astrocytoma. In infants, DWI is helpful in differentiating high- vs. low-grade tumors that tend to be large, heterogeneous, and hemispheric.*
![Sagittal T1 C+ MR shows 3 nodular masses in the posterior fossa within the cisterna magna <img src='img/arrows/CS.png'/>, 4th ventricle <img src='img/arrows/CO.png'/>, and premedullary cistern <img src='img/arrows/CC.png'/>.](images/app.statdx.com_image_thumbnail_e555470b-4ff7-486a-8bfb-32737eea3187_size_168_quality_85_573fded7_20251014T202907Z.jpg)
**Atypical Teratoid-Rhabdoid Tumor**
*Sagittal T1 C+ MR shows 3 nodular masses in the posterior fossa within the cisterna magna <img src='img/arrows/CS.png'/>, 4th ventricle <img src='img/arrows/CO.png'/>, and premedullary cistern <img src='img/arrows/CC.png'/>.*
![Axial b=1000 DWI shows the multiple posterior fossa masses <img src='img/arrows/CS.png'/> have decreased diffusion compatible with high-grade neoplasm. Consider atypical teratoid-rhabdoid tumor in infants.](2a9a62d8-52db-48a6-be5f-125569f31a09)
**Atypical Teratoid-Rhabdoid Tumor**
*Axial b=1000 DWI shows the multiple posterior fossa masses <img src='img/arrows/CS.png'/> have decreased diffusion compatible with high-grade neoplasm. Consider atypical teratoid-rhabdoid tumor in infants.*
![Axial b=1000 DWI shows the peripheral solid component has decreased diffusion consistent with a hypercellular high-grade tumor <img src='img/arrows/CS.png'/>. CNS embryonal tumors were previously termed primitive neuroectodermal tumors (PNETs).](4163ab55-dc4d-487a-a1fc-73048a1c161e)
**CNS Embryonal Tumor**
*Axial b=1000 DWI shows the peripheral solid component has decreased diffusion consistent with a hypercellular high-grade tumor <img src='img/arrows/CS.png'/>. CNS embryonal tumors were previously termed primitive neuroectodermal tumors (PNETs).*
![Axial T1 C+ MR shows a large left hemispheric mass with a large central proteinaceous cyst with intrinsic T1 shortening <img src='img/arrows/CS.png'/>. The solid peripheral component has minimal enhancement <img src='img/arrows/CO.png'/>.](2f437f13-2cf9-43d5-9b23-bb21e22b3b10)
**CNS Embryonal Tumor**
*Axial T1 C+ MR shows a large left hemispheric mass with a large central proteinaceous cyst with intrinsic T1 shortening <img src='img/arrows/CS.png'/>. The solid peripheral component has minimal enhancement <img src='img/arrows/CO.png'/>.*
![Axial T1 C+ MR shows a heterogeneous mass with solid enhancement peripherally with a broad dural base <img src='img/arrows/CS.png'/> and cysts <img src='img/arrows/CO.png'/> medially in an infant. Desmoplastic infantile tumors have a broad dural base as a characteristic appearance.](5362633c-c9fd-48c1-8c21-8e347924d551)
**Desmoplastic Infantile Ganglioglioma/Astrocytoma**
*Axial T1 C+ MR shows a heterogeneous mass with solid enhancement peripherally with a broad dural base <img src='img/arrows/CS.png'/> and cysts <img src='img/arrows/CO.png'/> medially in an infant. Desmoplastic infantile tumors have a broad dural base as a characteristic appearance.*
![Axial T2 TSE MR shows the solid component has a hypointense appearance <img src='img/arrows/CS.png'/>.](22fe201b-42bd-4991-92c4-82f627ec62f2)
**Desmoplastic Infantile Ganglioglioma/Astrocytoma**
*Axial T2 TSE MR shows the solid component has a hypointense appearance <img src='img/arrows/CS.png'/>.*
![Axial b=1000 DWI MR shows the solid components do not have decreased diffusion <img src='img/arrows/CS.png'/>, correlating with the low-grade nature of this tumor. Infants that have a complete resection have a good outcome.](8c2d5147-cb14-46aa-b00c-5f8d0ee16f78)
**Desmoplastic Infantile Ganglioglioma/Astrocytoma**
*Axial b=1000 DWI MR shows the solid components do not have decreased diffusion <img src='img/arrows/CS.png'/>, correlating with the low-grade nature of this tumor. Infants that have a complete resection have a good outcome.*
![Axial T1 C+ MR shows a large, heterogeneous mass centered in the left atria of the lateral ventricle <img src='img/arrows/CS.png'/>, consistent with a choroid plexus tumor. This was a carcinoma at resection.](d58780ed-00e9-44da-b6e3-34b2a91d9e73)
**Choroid Plexus Carcinoma**
*Axial T1 C+ MR shows a large, heterogeneous mass centered in the left atria of the lateral ventricle <img src='img/arrows/CS.png'/>, consistent with a choroid plexus tumor. This was a carcinoma at resection.*
![Axial T2 TSE MR shows the large, isointense lateral ventricular mass. Radiographic size, invasion, and dissemination are not helpful to distinguish between a low-grade papilloma and a high-grade carcinoma. Carcinomas have a propensity to present in younger children.](b806a537-6243-4b8f-90eb-6da1f7e19a6e)
**Choroid Plexus Carcinoma**
*Axial T2 TSE MR shows the large, isointense lateral ventricular mass. Radiographic size, invasion, and dissemination are not helpful to distinguish between a low-grade papilloma and a high-grade carcinoma. Carcinomas have a propensity to present in younger children.*
![Axial T2 MR demonstrates a homogeneous cortical mass in a young child presenting with seizures. A dysembryoplastic neuroepithelial tumor (DNET) was the favored differential, but this was an embryonal tumor with multilayered rosettes (ETMR) at resection.](2d5116a4-7a26-429b-9a7f-eedd1e099f86)
**Embryonal Tumor With Multilayered Rosettes**
*Axial T2 MR demonstrates a homogeneous cortical mass in a young child presenting with seizures. A dysembryoplastic neuroepithelial tumor (DNET) was the favored differential, but this was an embryonal tumor with multilayered rosettes (ETMR) at resection.*
![Axial ADC map shows an area of decreased diffusion <img src='img/arrows/CS.png'/> within the cortically based tumor, consistent with a high-grade neoplasm. ETMR is typically an aggressive and large high-grade tumor, with this tumor having an atypical early presentation.](b90db7b6-0b81-4943-ac8b-adb465eaa2bd)
**Embryonal Tumor With Multilayered Rosettes**
*Axial ADC map shows an area of decreased diffusion <img src='img/arrows/CS.png'/> within the cortically based tumor, consistent with a high-grade neoplasm. ETMR is typically an aggressive and large high-grade tumor, with this tumor having an atypical early presentation.*
![Coronal T1 MR in this neonate with large segmental cutaneous nevus shows an area of intrinsic T1 shortening in the left cerebellum <img src='img/arrows/CS.png'/>, consistent with neurocutaneous melanosis.](64155791-aa39-4e18-8da7-49fc660207bf)
**Neurocutaneous Melanosis (Melanoma/Melanocytoma)**
*Coronal T1 MR in this neonate with large segmental cutaneous nevus shows an area of intrinsic T1 shortening in the left cerebellum <img src='img/arrows/CS.png'/>, consistent with neurocutaneous melanosis.*
![Coronal T1 C+ MR shows 2 masses in the amygdala with intrinsic T1 shortening <img src='img/arrows/CS.png'/> compared to precontrast T1 (not shown). Amygdala involvement is a common location for neurocutaneous melanosis.](f94afdc2-932f-4027-b99b-7e281ab90715)
**Neurocutaneous Melanosis (Melanoma/Melanocytoma)**
*Coronal T1 C+ MR shows 2 masses in the amygdala with intrinsic T1 shortening <img src='img/arrows/CS.png'/> compared to precontrast T1 (not shown). Amygdala involvement is a common location for neurocutaneous melanosis.*
### Additional Images
![Axial T1 MR in this 7-day-old infant shows T1-bright signal from fat <img src='img/arrows/WS.png'/> scattered throughout the lesion.](80c8cb35-b67f-44a8-8914-e9501e1ff3c3)
**Teratoma**
*Axial T1 MR in this 7-day-old infant shows T1-bright signal from fat <img src='img/arrows/WS.png'/> scattered throughout the lesion.*
![Axial NECT in the same child at 15 months old shows a complicated pineal region mass consisting of fat <img src='img/arrows/WO.png'/>, solid tissue <img src='img/arrows/WS.png'/>, and calcification <img src='img/arrows/WC.png'/>.](f8ea3a8c-397a-4c6c-b1e7-46d4392d1af9)
**Teratoma**
*Axial NECT in the same child at 15 months old shows a complicated pineal region mass consisting of fat <img src='img/arrows/WO.png'/>, solid tissue <img src='img/arrows/WS.png'/>, and calcification <img src='img/arrows/WC.png'/>.*
![Axial CECT in a 1-year-old infant with macrocrania and vomiting shows a giant enhancing posterior fossa mass <img src='img/arrows/BS.png'/> that fills the posterior fossa. Note peripheral myxoid or cystic tumor components <img src='img/arrows/WC.png'/>.](cbf02da7-2d98-4d58-a930-d740148699f1)
**Medulloblastoma**
*Axial CECT in a 1-year-old infant with macrocrania and vomiting shows a giant enhancing posterior fossa mass <img src='img/arrows/BS.png'/> that fills the posterior fossa. Note peripheral myxoid or cystic tumor components <img src='img/arrows/WC.png'/>.*
![Axial T2 MR in a 10-month-old shows a giant isointense posterior fossa mass with distinct, thick, nodular morphology <img src='img/arrows/BS.png'/>. PNET-medulloblastoma with extensive nodularity, a subtype of medulloblastoma, has a somewhat better prognosis.](16b1e4fc-ee86-4429-a0b3-57928fe168ca)
**Medulloblastoma**
*Axial T2 MR in a 10-month-old shows a giant isointense posterior fossa mass with distinct, thick, nodular morphology <img src='img/arrows/BS.png'/>. PNET-medulloblastoma with extensive nodularity, a subtype of medulloblastoma, has a somewhat better prognosis.*
![Axial T2 MR in a 4-month-old shows an intermediate- to low-signal mass that splays and encases posterior communicating <img src='img/arrows/WO.png'/> and superior cerebellar <img src='img/arrows/WS.png'/> arteries.](1e5c0c8d-f1e7-4c6d-b5e4-15c3d2825cf3)
**Medulloblastoma**
*Axial T2 MR in a 4-month-old shows an intermediate- to low-signal mass that splays and encases posterior communicating <img src='img/arrows/WO.png'/> and superior cerebellar <img src='img/arrows/WS.png'/> arteries.*
![Axial DWI MR shows diffusion restriction in this tumor <img src='img/arrows/WC.png'/> involving mesencephalon, circummesencephalic cistern, and mesial temporal lobe.](4c7ae380-65b0-461e-a2c3-fe3c68f6793d)
**Medulloblastoma**
*Axial DWI MR shows diffusion restriction in this tumor <img src='img/arrows/WC.png'/> involving mesencephalon, circummesencephalic cistern, and mesial temporal lobe.*
![Coronal T1 C+ MR in this 10-month-old shows grape-like nodular enhancement <img src='img/arrows/WS.png'/>. Medulloblastoma with extensive nodularity is a PNET-medulloblastoma variant that has somewhat better prognosis.](4b4c1d0f-87fc-447e-bfa8-f389782bbe9e)
**Medulloblastoma**
*Coronal T1 C+ MR in this 10-month-old shows grape-like nodular enhancement <img src='img/arrows/WS.png'/>. Medulloblastoma with extensive nodularity is a PNET-medulloblastoma variant that has somewhat better prognosis.*
![Axial T2 MR in a 12-week-old infant shows a mixed heterogeneity left temporal lobe mass.](7578a08e-efc0-4573-90bf-f7a10f037872)
**Supratentorial Ependymoma**
*Axial T2 MR in a 12-week-old infant shows a mixed heterogeneity left temporal lobe mass.*
![Axial T2* GRE MR shows multifocal hemosiderin and calcific foci <img src='img/arrows/WO.png'/>.](c205e3f7-7477-4614-9d63-f39db38fb07f)
**Supratentorial Ependymoma**
*Axial T2* GRE MR shows multifocal hemosiderin and calcific foci <img src='img/arrows/WO.png'/>.*
![Coronal T1 C+ MR shows bilateral choroid plexus papillomas. The left <img src='img/arrows/WC.png'/> is bulky and frond-like, while the right <img src='img/arrows/WO.png'/> is stretched by the associated cyst.](9ba1ab66-e34b-487b-816b-305081192b05)
**Choroid Plexus Papilloma**
*Coronal T1 C+ MR shows bilateral choroid plexus papillomas. The left <img src='img/arrows/WC.png'/> is bulky and frond-like, while the right <img src='img/arrows/WO.png'/> is stretched by the associated cyst.*
![Axial T2 MR shows a large cyst <img src='img/arrows/BC.png'/>, coloboma <img src='img/arrows/WO.png'/>, and temporal lobe subependymal heterotopia <img src='img/arrows/WS.png'/> in a 4-day-old girl with Aicardi syndrome.](790089eb-201f-4ff7-924d-7cd43d00d488)
**Choroid Plexus Papilloma**
*Axial T2 MR shows a large cyst <img src='img/arrows/BC.png'/>, coloboma <img src='img/arrows/WO.png'/>, and temporal lobe subependymal heterotopia <img src='img/arrows/WS.png'/> in a 4-day-old girl with Aicardi syndrome.*
![Sagittal ultrasound shows a bulky subependymal giant cell astrocytoma <img src='img/arrows/WO.png'/> at the foramen of Monro in this newborn with cardiac rhabdomyoma and tuberous sclerosis. There are multiple additional tubers <img src='img/arrows/WC.png'/> on the same image.](75cd48a5-dd64-4570-a60c-db6ccb6d881a)
**Supratentorial Ependymoma**
*Sagittal ultrasound shows a bulky subependymal giant cell astrocytoma <img src='img/arrows/WO.png'/> at the foramen of Monro in this newborn with cardiac rhabdomyoma and tuberous sclerosis. There are multiple additional tubers <img src='img/arrows/WC.png'/> on the same image.*
![Coronal T1 C+ MR in a 7-month-old infant shows a massive right frontal cystic tumor with a solid enhancing component that involves the medial frontal cortex <img src='img/arrows/WS.png'/> and falx.](219657af-4af5-49fd-b180-6fab98886b6c)
**Desmoplastic Infantile Ganglioglioma/Astrocytoma**
*Coronal T1 C+ MR in a 7-month-old infant shows a massive right frontal cystic tumor with a solid enhancing component that involves the medial frontal cortex <img src='img/arrows/WS.png'/> and falx.*
![Axial DWI MR shows a lack of diffusion restriction in the medial cortical tumor rind <img src='img/arrows/WO.png'/>, which enhanced following contrast administration in this infant (not shown).](b228bad8-711b-4d38-ada4-81cc1aadd42b)
**Desmoplastic Infantile Ganglioglioma/Astrocytoma**
*Axial DWI MR shows a lack of diffusion restriction in the medial cortical tumor rind <img src='img/arrows/WO.png'/>, which enhanced following contrast administration in this infant (not shown).*
![Axial T1 C+ MR in this 9-month-old infant shows a large, bulky, avidly enhancing left intraventricular tumor <img src='img/arrows/BC.png'/> with invasion of the overlying brain <img src='img/arrows/WO.png'/>. There are multiple intraventricular metastases <img src='img/arrows/BO.png'/>.](a7f4ea18-34f8-4e08-8ceb-695f627b70b1)
**Choroid Plexus Carcinoma**
*Axial T1 C+ MR in this 9-month-old infant shows a large, bulky, avidly enhancing left intraventricular tumor <img src='img/arrows/BC.png'/> with invasion of the overlying brain <img src='img/arrows/WO.png'/>. There are multiple intraventricular metastases <img src='img/arrows/BO.png'/>.*
![AP angiography performed as a part of preoperative embolization shows hypervascularity <img src='img/arrows/BS.png'/> and multiple areas of contrast puddling <img src='img/arrows/BC.png'/>.](512ffe61-a093-463b-b9e8-e60cd825192f)
**Choroid Plexus Carcinoma**
*AP angiography performed as a part of preoperative embolization shows hypervascularity <img src='img/arrows/BS.png'/> and multiple areas of contrast puddling <img src='img/arrows/BC.png'/>.*
![Sagittal T2 MR in this 7-month-old infant shows hydrocephalus and a complicated solid and cystic tumor filling the 4th ventricle, supravermian cistern, and extending through the tentorial incisura <img src='img/arrows/WO.png'/>.](1f33654a-6bb9-4b8d-bb94-003bc3da7447)
**Atypical Teratoid-Rhabdoid Tumor**
*Sagittal T2 MR in this 7-month-old infant shows hydrocephalus and a complicated solid and cystic tumor filling the 4th ventricle, supravermian cistern, and extending through the tentorial incisura <img src='img/arrows/WO.png'/>.*
![Coronal T1 C+ MR in the same 7-month-old shows a right frontal metastatic deposit <img src='img/arrows/WO.png'/>.](9d846115-1bf6-4630-9c2e-29d818a32592)
**Atypical Teratoid-Rhabdoid Tumor**
*Coronal T1 C+ MR in the same 7-month-old shows a right frontal metastatic deposit <img src='img/arrows/WO.png'/>.*
![Sagittal T1 MR shows increased signal intensity of the hippocampus <img src='img/arrows/WO.png'/> in this 10-month-old with a large cutaneous nevus. Pachymeningeal thickening <img src='img/arrows/WS.png'/> is present prior to contrast administration.](0f3f6ce8-5e57-44bd-9df6-f1dbf08c37e7)
**Neurocutaneous Melanosis (Melanoma/Melanocytoma)**
*Sagittal T1 MR shows increased signal intensity of the hippocampus <img src='img/arrows/WO.png'/> in this 10-month-old with a large cutaneous nevus. Pachymeningeal thickening <img src='img/arrows/WS.png'/> is present prior to contrast administration.*
![Sagittal T1 MR in a 5-day-old infant shows a massive hemorrhagic tumor replacing and expanding the upper cervical spinal cord, the brainstem, and the cerebellum. The tumor protrudes through the incisura and displaces the straight sinus <img src='img/arrows/WO.png'/>.](0051be29-a76d-4aea-ac93-3d0e659d523c)
**Embryonal Tumor With Multilayered Rosettes**
*Sagittal T1 MR in a 5-day-old infant shows a massive hemorrhagic tumor replacing and expanding the upper cervical spinal cord, the brainstem, and the cerebellum. The tumor protrudes through the incisura and displaces the straight sinus <img src='img/arrows/WO.png'/>.*
![Axial T2 MR shows a well-circumscribed, heterogeneous mass asymmetrically expanding the pons and the right middle cerebellar peduncle.](ebd20221-808d-41ff-b16b-b53cf270e416)
**Embryonal Tumor With Multilayered Rosettes**
*Axial T2 MR shows a well-circumscribed, heterogeneous mass asymmetrically expanding the pons and the right middle cerebellar peduncle.*
![Axial DWI of the well-circumscribed pontine tumor shows intense increased signal from decreased diffusion, suggesting this is a high-grade neoplasm. Medulloepithelioma is a highly primitive and aggressive tumor with a dismal prognosis.](a9989e3e-bebc-44e1-8ab8-118833c6129d)
**Embryonal Tumor With Multilayered Rosettes**
*Axial DWI of the well-circumscribed pontine tumor shows intense increased signal from decreased diffusion, suggesting this is a high-grade neoplasm. Medulloepithelioma is a highly primitive and aggressive tumor with a dismal prognosis.*
![Axial T1 C+ MR shows heterogeneous enhancement of the cerebellopontine angle tumor. Posterior fossa atypical teratoid-rhabdoid tumors can be difficult to distinguish from medulloblastomas, and both high-grade tumors have decreased diffusion.](14ca664f-76bb-4dd1-b711-02a3e5c4f71f)
**Atypical Teratoid-Rhabdoid Tumor**
*Axial T1 C+ MR shows heterogeneous enhancement of the cerebellopontine angle tumor. Posterior fossa atypical teratoid-rhabdoid tumors can be difficult to distinguish from medulloblastomas, and both high-grade tumors have decreased diffusion.*
![Axial T2 MR shows a heterogeneous mass with cystic change and low T2 signal in the left cerebellopontine angle with invasion into the left internal auditory canal <img src='img/arrows/CS.png'/>.](fbc4bf0b-c5ad-472a-b557-b973979c10bf)
**Atypical Teratoid-Rhabdoid Tumor**
*Axial T2 MR shows a heterogeneous mass with cystic change and low T2 signal in the left cerebellopontine angle with invasion into the left internal auditory canal <img src='img/arrows/CS.png'/>.*
![Axial T1 C+ MR shows heterogeneous enhancement of the large intraventricular mass. While hemorrhage and parenchymal invasion are more likely with choroid plexus carcinoma, no particular imaging sign is able to specifically differentiate a papilloma from a carcinoma.](306e9bf8-5434-4ea5-9726-4d4fb3f08f37)
**Choroid Plexus Carcinoma**
*Axial T1 C+ MR shows heterogeneous enhancement of the large intraventricular mass. While hemorrhage and parenchymal invasion are more likely with choroid plexus carcinoma, no particular imaging sign is able to specifically differentiate a papilloma from a carcinoma.*
![Axial T2 MR shows a large lobular, heterogeneous mass centered in the left lateral ventricle. There are fluid-fluid levels <img src='img/arrows/CS.png'/> from layering blood products.](f2510809-adab-45ba-9154-bab75fbf1d6e)
**Choroid Plexus Carcinoma**
*Axial T2 MR shows a large lobular, heterogeneous mass centered in the left lateral ventricle. There are fluid-fluid levels <img src='img/arrows/CS.png'/> from layering blood products.*
![Axial T2 MR shows a mass expanding the chiasm and optic tracts <img src='img/arrows/CS.png'/> with mass effect on the medial temporal lobes and midbrain. The T2 hyperintensity suggests a low-grade neoplasm.](d9ec1c3c-83cc-4806-a804-92b60bd54943)
**Pilocytic Astrocytoma, Pilomyxoid Variant**
*Axial T2 MR shows a mass expanding the chiasm and optic tracts <img src='img/arrows/CS.png'/> with mass effect on the medial temporal lobes and midbrain. The T2 hyperintensity suggests a low-grade neoplasm.*
![Sagittal T1 C+ MR shows intense enhancement with central hypointensity <img src='img/arrows/CS.png'/> from infiltration of the optic chiasm and hypothalamus. In very young children, pilomyxoid astrocytomas often present very large in the optic pathway.](79f1d7c2-2cc0-4963-9785-bd810f131212)
**Pilocytic Astrocytoma, Pilomyxoid Variant**
*Sagittal T1 C+ MR shows intense enhancement with central hypointensity <img src='img/arrows/CS.png'/> from infiltration of the optic chiasm and hypothalamus. In very young children, pilomyxoid astrocytomas often present very large in the optic pathway.*
![Axial T2 MR shows a heterogeneous mass with central cysts and a hypointense peripheral solid component with broad dural attachment <img src='img/arrows/CS.png'/>. There is adjacent left frontal lobe edema and midline shift.](f34deca5-a63e-4b97-b093-6a9a0b714109)
**Desmoplastic Infantile Ganglioglioma/Astrocytoma**
*Axial T2 MR shows a heterogeneous mass with central cysts and a hypointense peripheral solid component with broad dural attachment <img src='img/arrows/CS.png'/>. There is adjacent left frontal lobe edema and midline shift.*
![Coronal T1 C+ MR shows intense enhancement of the peripheral solid component of the left frontal heterogeneous mass. This peripheral, broad dural attachment of a solid, enhancing nodule <img src='img/arrows/CS.png'/> is a specific sign of a desmoplastic infantile ganglioglioma or astrocytoma in the appropriate age range.](4d3e7589-21f3-4af2-b42b-6331c19de265)
**Desmoplastic Infantile Ganglioglioma/Astrocytoma**
*Coronal T1 C+ MR shows intense enhancement of the peripheral solid component of the left frontal heterogeneous mass. This peripheral, broad dural attachment of a solid, enhancing nodule <img src='img/arrows/CS.png'/> is a specific sign of a desmoplastic infantile ganglioglioma or astrocytoma in the appropriate age range.*
![Axial CT shows an isodense, lobular, frond-like mass <img src='img/arrows/CS.png'/> centered in the right atria of the lateral ventricle. There are layering blood products <img src='img/arrows/CO.png'/> within enlarged lateral ventricles, indicating hydrocephalus.](9a45ad8a-fa48-4a07-9c71-9d607b10d804)
**Choroid Plexus Papilloma**
*Axial CT shows an isodense, lobular, frond-like mass <img src='img/arrows/CS.png'/> centered in the right atria of the lateral ventricle. There are layering blood products <img src='img/arrows/CO.png'/> within enlarged lateral ventricles, indicating hydrocephalus.*
![Coronal T1 C+ MR shows an intensely enhancing lobular mass <img src='img/arrows/CS.png'/> centered in the right choroid plexus glomus consistent with a choroid plexus tumor. There is associated hydrocephalus.](ba2bf35b-d08f-4866-88b6-b9783538d010)
**Choroid Plexus Papilloma**
*Coronal T1 C+ MR shows an intensely enhancing lobular mass <img src='img/arrows/CS.png'/> centered in the right choroid plexus glomus consistent with a choroid plexus tumor. There is associated hydrocephalus.*
![Axial T1 C+ MR shows heterogeneous peripheral enhancement <img src='img/arrows/CS.png'/> of mass surrounding central cystic necrosis. Supratentorial ependymomas arise most commonly in a periventricular location from ependymal rest cells.](0ee16839-0830-4be6-8555-f881941c495f)
**Supratentorial Ependymoma**
*Axial T1 C+ MR shows heterogeneous peripheral enhancement <img src='img/arrows/CS.png'/> of mass surrounding central cystic necrosis. Supratentorial ependymomas arise most commonly in a periventricular location from ependymal rest cells.*
![Axial T2 MR shows a heterogeneous mass with central necrosis in a periventricular location in this infant's frontal lobe. There is a peripheral hypointense T2 component indicating blood products <img src='img/arrows/CS.png'/>. There is ventricular enlargement from hydrocephalus.](d3179092-ff5f-4c73-a5d7-077ef663c19b)
**Supratentorial Ependymoma**
*Axial T2 MR shows a heterogeneous mass with central necrosis in a periventricular location in this infant's frontal lobe. There is a peripheral hypointense T2 component indicating blood products <img src='img/arrows/CS.png'/>. There is ventricular enlargement from hydrocephalus.*
![Axial T2 MR shows a nodular and invasive mass within the superior 4th ventricle <img src='img/arrows/CS.png'/> and cerebellar vermis <img src='img/arrows/CO.png'/>. There is severe hydrocephalus and transependymal interstitial edema. Note susceptibility artifact from a ventriculostomy reservoir <img src='img/arrows/CC.png'/>.](e1c93f97-36ec-4b82-8695-dfa16c0d5f05)
**Medulloblastoma**
*Axial T2 MR shows a nodular and invasive mass within the superior 4th ventricle <img src='img/arrows/CS.png'/> and cerebellar vermis <img src='img/arrows/CO.png'/>. There is severe hydrocephalus and transependymal interstitial edema. Note susceptibility artifact from a ventriculostomy reservoir <img src='img/arrows/CC.png'/>.*
![Sagittal T1 C+ MR shows nodular, grape-like enhancement of the posterior fossa mass filling the superior 4th ventricle <img src='img/arrows/CS.png'/> and invading the vermis <img src='img/arrows/CO.png'/>. This appearance is suggestive of medulloblastoma with extensive nodularity, which has a favorable prognosis.](b08ed82a-a53f-46d1-a805-f9b4d2a92251)
**Medulloblastoma**
*Sagittal T1 C+ MR shows nodular, grape-like enhancement of the posterior fossa mass filling the superior 4th ventricle <img src='img/arrows/CS.png'/> and invading the vermis <img src='img/arrows/CO.png'/>. This appearance is suggestive of medulloblastoma with extensive nodularity, which has a favorable prognosis.*
![Axial ADC map shows decreased diffusion <img src='img/arrows/CS.png'/> surrounding the central necrosis. This was a WHO grade 4 CNS embryonal tumor, not otherwise specified. This was previously called PNET. The 2016 WHO CNS tumor classification has removed the term PNET.](7335f8cb-db37-4c61-9168-c7c48c257f8d)
**CNS Embryonal Tumor**
*Axial ADC map shows decreased diffusion <img src='img/arrows/CS.png'/> surrounding the central necrosis. This was a WHO grade 4 CNS embryonal tumor, not otherwise specified. This was previously called PNET. The 2016 WHO CNS tumor classification has removed the term PNET.*
![Axial T1 C+ MR shows a heterogeneous mass in the right cerebral hemisphere, which causes midline shift and ipsilateral ventricular effacement. There is central necrosis with surrounding ring enhancement <img src='img/arrows/CS.png'/>.](c665a739-63cb-4441-a816-a394109785b2)
**CNS Embryonal Tumor**
*Axial T1 C+ MR shows a heterogeneous mass in the right cerebral hemisphere, which causes midline shift and ipsilateral ventricular effacement. There is central necrosis with surrounding ring enhancement <img src='img/arrows/CS.png'/>.*
![Axial DWI MR shows increased signal from decreased diffusion of the 4th ventricular tumor, consistent with a high-grade, hypercellular tumor, such as medulloblastoma. Decreased DWI can be seen in many high-grade neoplasms.](f2e319e5-2b4c-4b6c-af46-c201835bfc76)
**Medulloblastoma**
*Axial DWI MR shows increased signal from decreased diffusion of the 4th ventricular tumor, consistent with a high-grade, hypercellular tumor, such as medulloblastoma. Decreased DWI can be seen in many high-grade neoplasms.*
![Axial T2 MR shows a heterogeneous tumor within the 4th ventricle consistent with a classic medulloblastoma. Note the CSF cleft <img src='img/arrows/CS.png'/> with the dorsal pons (floor) suggesting attachment to the roof of the 4th ventricle (vermis) <img src='img/arrows/CO.png'/>.](5a1d5947-cd1d-45e4-9147-1c541cd1929f)
**Medulloblastoma**
*Axial T2 MR shows a heterogeneous tumor within the 4th ventricle consistent with a classic medulloblastoma. Note the CSF cleft <img src='img/arrows/CS.png'/> with the dorsal pons (floor) suggesting attachment to the roof of the 4th ventricle (vermis) <img src='img/arrows/CO.png'/>.*
![Axial CT shows a midline lobular mass with solid and cystic components with focal coarse calcifications <img src='img/arrows/CS.png'/>. There is extreme hydrocephalus, leaving only a small rind of brain parenchyma <img src='img/arrows/CO.png'/>.](edb897a9-e612-44f3-8581-7c0b93fbfbb3)
**Teratoma**
*Axial CT shows a midline lobular mass with solid and cystic components with focal coarse calcifications <img src='img/arrows/CS.png'/>. There is extreme hydrocephalus, leaving only a small rind of brain parenchyma <img src='img/arrows/CO.png'/>.*
![Sagittal T1 MR shows a heterogeneous, lobular midline mass with an area of T1 hyperintensity consistent with areas of fat <img src='img/arrows/CS.png'/>. There is extreme hydrocephalus with macrocrania. Although mature teratomas are low grade, they present very large with poor prognosis due to massive hydrocephalus.](307492bc-8b03-4013-ad99-7d8c45e5a192)
**Teratoma**
*Sagittal T1 MR shows a heterogeneous, lobular midline mass with an area of T1 hyperintensity consistent with areas of fat <img src='img/arrows/CS.png'/>. There is extreme hydrocephalus with macrocrania. Although mature teratomas are low grade, they present very large with poor prognosis due to massive hydrocephalus.*
![Coronal T1 C+ MR in a child with a large congenital nevi shows diffuse patchy and leptomeningeal enhancement from metastatic melanoma. The leptomeningeal form of neurocutaneous melanosis portends a poor prognosis.](1c419999-e6bf-4b8d-a805-299a6fba9573)
**Neurocutaneous Melanosis (Melanoma/Melanocytoma)**
*Coronal T1 C+ MR in a child with a large congenital nevi shows diffuse patchy and leptomeningeal enhancement from metastatic melanoma. The leptomeningeal form of neurocutaneous melanosis portends a poor prognosis.*
docs_md/articles/calcified-suprasellar-mass_b61b0b70-de36-45f9-88a2-162a9758b729.md
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title: "Calcified Suprasellar Mass"
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---
# ESSENTIAL INFORMATION
- ## Key Differential Diagnosis Issues
- Is Ca⁺⁺ curvilinear, punctate, globular, etc.?
- Does lesion enhance?
- ## Helpful Clues for Common Diagnoses
- **Atherosclerosis****, Intracranial**
- Curvilinear Ca⁺⁺ along vessel walls
- Usually bilateral, often multifocal
- Older patients
- **Craniopharyngioma**
- Globular, punctate, &/or ring Ca⁺⁺
- **Adamantinomatous**: Bimodal age distribution (peaks 5-15 years and 45-60 years)
- **Papillary**: Older adult tumors more often solid, Ca⁺⁺ less frequent
- **Meningioma**
- Psammomatous (sand-like) Ca⁺⁺
- Solid > rim enhancement
- Middle-aged, older patients (unless NF2)
- **Aneurysm**
- **Saccular****a****neurysm**
- Calcification less common than with fusiform aneurysm, ASVD
- Curvilinear (peripheral arcs, rings) pattern
- **Fusiform****a****neurysm, atherosclerotic**
- Linear ± rim Ca⁺⁺
- Ca⁺⁺ often present in other vessels
- ## Helpful Clues for Less Common Diagnoses
- **Neurocysticercosis**
- Nodular calcified stage
- Usually parenchymal > > cisternal Ca⁺⁺
- **Pilocytic Astrocytoma**
- Common in children, young adults
- Ca⁺⁺ uncommon in hypothalamic PA
- **Dermoid Cyst**
- 20% have capsular Ca⁺⁺
- Contain lipid
- Look for evidence of rupture (fatty droplets in subarachnoid spaces, cisterns)
- No enhancement unless chemical meningitis
- ## Helpful Clues for Rare Diagnoses
- **Pituitary Macroadenoma**
- Only 1-2% of macroadenomas calcify
- Adenomas usually isodense with gray matter
- Majority result in smooth enlargement of sella turcica
- **Rathke Cleft Cyst**
- Vast majority have no calcification (> 85%)
- May rarely see thin, curvilinear rim calcification (10-15%)
- Typically hypodense to brain parenchyma
- **Tuberculosis**
- TB, healing/healed granulomatous infections cause parenchymal > > cisternal Ca⁺⁺
- **Chondroid Tumor**
- Chondromas, enchondromas arise from central skull base
- Chondroid matrix on CT
- May extend to suprasellar region
## References
# Selected References
1. [Alali AA et al: Do we need gadolinium-based contrast agents for routine MRI surveillance of unoperated pituitary macroadenoma? AJNR Am J Neuroradiol. 43(7):1024-8, 2022](http://www.ncbi.nlm.nih.gov/pubmed/?term=35738673%5Bpmid%5D)
1. [Perret CM et al: A giant internal carotid bifurcation aneurysm as a rare and dangerous differential diagnosis of a craniopharyngioma. Cureus. 14(1):e21588, 2022](http://www.ncbi.nlm.nih.gov/pubmed/?term=35228946%5Bpmid%5D)
1. [Jipa A et al: Imaging of the sellar and parasellar regions. Clin Imaging. 77:254-75, 2021](http://www.ncbi.nlm.nih.gov/pubmed/?term=34153590%5Bpmid%5D)
1. [Chapman PR et al: Neuroimaging of the pituitary gland: practical anatomy and pathology. Radiol Clin North Am. 58(6):1115-33, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=33040852%5Bpmid%5D)
1. [Snyder MH et al: Neurocysticercosis presenting as an isolated suprasellar lesion. World Neurosurg. 141:352-6, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=32522639%5Bpmid%5D)
1. [Xin WQ et al: Patient with epilepsy caused by the spontaneous rupture of an intracerebral dermoid cyst. World Neurosurg. 136:140-5, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=31954900%5Bpmid%5D)
1. [Jacków J et al: Ruptured intracranial dermoid cysts: a pictorial review. Pol J Radiol. 83:e465-70, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=30655926%5Bpmid%5D)
1. [Seeburg DP et al: Imaging of the sella and parasellar region in the pediatric population. Neuroimaging Clin N Am. 27(1):99-121, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=27889026%5Bpmid%5D)
1. [Hoffmann A et al: Fusiform dilatation of the internal carotid artery in childhood-onset craniopharyngioma: multicenter study on incidence and long-term outcome. Pituitary. 19(4):422-8, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=27125511%5Bpmid%5D)
1. [Pan J et al: Growth patterns of craniopharyngiomas: clinical analysis of 226 patients. J Neurosurg Pediatr. 17(4):418-33, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=26636252%5Bpmid%5D)
1. [Sekiguchi K et al: Osteochondroma presenting as a calcified mass in the sellar region and review of the literature. J Neurol Surg A Cent Eur Neurosurg. 78(4):380-5, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=27903019%5Bpmid%5D)
1. [Yildiz AE et al: Suprasellar masses in children: characteristic MR imaging features. J Neuroradiol. 43(4):246-59, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=27131616%5Bpmid%5D)
1. [Ben Nsir A et al: Calcified suprasellar xanthogranuloma presenting with primary amenorrhea in a 17-year-old girl: case report and literature review. World Neurosurg. 84(3):866.e11-4, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25916181%5Bpmid%5D)
## Images
### Selected Images
![Axial NECT shows a fusiform, partially calcified mass in the suprasellar cistern <img src='img/arrows/CS.png'/> that represents an ectatic, supraclinoid, internal carotid artery (ICA) with calcified atherosclerotic plaque. Coronal and sagittal reformatted images make this diagnosis straightforward.](images/app.statdx.com_image_thumbnail_eecea3f1-ae69-42be-a896-9b64a38071b4_annotated_true_size_900_quality_90_b311b11e.jpg)
**Atherosclerosis, Intracranial**
*Axial NECT shows a fusiform, partially calcified mass in the suprasellar cistern <img src='img/arrows/CS.png'/> that represents an ectatic, supraclinoid, internal carotid artery (ICA) with calcified atherosclerotic plaque. Coronal and sagittal reformatted images make this diagnosis straightforward.*
![Axial NECT shows a fusiform, partially calcified mass in the suprasellar cistern <img src='img/arrows/CS.png'/> that represents an ectatic, supraclinoid, internal carotid artery (ICA) with calcified atherosclerotic plaque. Coronal and sagittal reformatted images make this diagnosis straightforward.](images/app.statdx.com_image_thumbnail_eecea3f1-ae69-42be-a896-9b64a38071b4_size_174_quality_85_f64bfc5f.jpg)
**Atherosclerosis, Intracranial**
*Axial NECT shows a fusiform, partially calcified mass in the suprasellar cistern <img src='img/arrows/CS.png'/> that represents an ectatic, supraclinoid, internal carotid artery (ICA) with calcified atherosclerotic plaque. Coronal and sagittal reformatted images make this diagnosis straightforward.*
![Axial NECT shows a cystic suprasellar mass with globular calcifications <img src='img/arrows/CS.png'/> typical of an adamantinomatous craniopharyngioma. These tumors are WHO grade 1 and are typically treated with surgical resection.](images/app.statdx.com_image_thumbnail_056d0e22-497b-47d2-92c0-632887d9c322_annotated_true_size_900_quality_90_dafeab3f.jpg)
**Craniopharyngioma**
*Axial NECT shows a cystic suprasellar mass with globular calcifications <img src='img/arrows/CS.png'/> typical of an adamantinomatous craniopharyngioma. These tumors are WHO grade 1 and are typically treated with surgical resection.*
![Sagittal NECT shows a partially calcified extraaxial mass projecting from the tuberculum sella <img src='img/arrows/CS.png'/>, related to a meningioma. This is separate from the adjacent pituitary gland <img src='img/arrows/CO.png'/> within a normal-sized sella turcica. Meningiomas in this location often involve the planum sphenoidale and have a dural tail on contrast-enhanced imaging.](images/app.statdx.com_image_thumbnail_ae28f3d5-00a6-4764-9a86-47904bee32be_annotated_true_size_900_quality_90_173c54ee.jpg)
**Meningioma**
*Sagittal NECT shows a partially calcified extraaxial mass projecting from the tuberculum sella <img src='img/arrows/CS.png'/>, related to a meningioma. This is separate from the adjacent pituitary gland <img src='img/arrows/CO.png'/> within a normal-sized sella turcica. Meningiomas in this location often involve the planum sphenoidale and have a dural tail on contrast-enhanced imaging.*
![Coronal arterial-phase CTA shows a saccular aneurysm projecting medially from the right cavernous ICA with internal arterial enhancement <img src='img/arrows/CO.png'/> and rim calcifications <img src='img/arrows/CS.png'/>.](images/app.statdx.com_image_thumbnail_bd4f9717-20ae-478c-bb19-10e0a1b49b41_annotated_true_size_900_quality_90_48710b22.jpg)
**Saccular Aneurysm**
*Coronal arterial-phase CTA shows a saccular aneurysm projecting medially from the right cavernous ICA with internal arterial enhancement <img src='img/arrows/CO.png'/> and rim calcifications <img src='img/arrows/CS.png'/>.*
![Axial NECT shows a hyperdense, fusiform basilar artery aneurysm <img src='img/arrows/CO.png'/> and calcified, fusiform aneurysmal ectasias of both ICAs <img src='img/arrows/WS.png'/>. Fusiform aneurysms are often related to atherosclerotic disease, as in this patient.](images/app.statdx.com_image_thumbnail_c4bf6e23-0c1b-480d-98f2-364c5dd61993_annotated_true_size_900_quality_90_f5e9fda8.jpg)
**Fusiform Aneurysm, Atherosclerotic**
*Axial NECT shows a hyperdense, fusiform basilar artery aneurysm <img src='img/arrows/CO.png'/> and calcified, fusiform aneurysmal ectasias of both ICAs <img src='img/arrows/WS.png'/>. Fusiform aneurysms are often related to atherosclerotic disease, as in this patient.*
![Axial NECT shows a low-density, suprasellar mass with rim <img src='img/arrows/CS.png'/> and globular calcification <img src='img/arrows/CO.png'/>. Biopsy disclosed a pilomyxoid subtype of pilocytic astrocytoma. This variant often occurs in the hypothalamic region in an infant or young child. These tumors rarely calcify.](d01dec64-a534-45b7-8fdf-e179fbbe9998)
**Pilocytic Astrocytoma**
*Axial NECT shows a low-density, suprasellar mass with rim <img src='img/arrows/CS.png'/> and globular calcification <img src='img/arrows/CO.png'/>. Biopsy disclosed a pilomyxoid subtype of pilocytic astrocytoma. This variant often occurs in the hypothalamic region in an infant or young child. These tumors rarely calcify.*
![Sagittal NECT shows a fat density lesion <img src='img/arrows/CO.png'/> with rim calcification <img src='img/arrows/CC.png'/> in the suprasellar cistern. There are scattered fat droplets in the subarachnoid spaces <img src='img/arrows/CS.png'/> in this case of a ruptured dermoid cyst.](b1cf55ae-d0d7-4a2d-92f4-3cedacf35c4c)
**Dermoid Cyst**
*Sagittal NECT shows a fat density lesion <img src='img/arrows/CO.png'/> with rim calcification <img src='img/arrows/CC.png'/> in the suprasellar cistern. There are scattered fat droplets in the subarachnoid spaces <img src='img/arrows/CS.png'/> in this case of a ruptured dermoid cyst.*
![Axial NECT shows curvilinear calcification <img src='img/arrows/CO.png'/> along the anterior aspect of a cystic suprasellar mass in a young adult. This mass was T1 hyperintense and did not enhance. A Rathke cleft cyst was diagnosed at resection. These benign cysts are usually not calcified, which helps to differentiate them from craniopharyngiomas.](e9c364e9-ab7e-42cd-be55-44b8c19ef17d)
**Rathke Cleft Cyst**
*Axial NECT shows curvilinear calcification <img src='img/arrows/CO.png'/> along the anterior aspect of a cystic suprasellar mass in a young adult. This mass was T1 hyperintense and did not enhance. A Rathke cleft cyst was diagnosed at resection. These benign cysts are usually not calcified, which helps to differentiate them from craniopharyngiomas.*
### Additional Images
![Axial CECT shows a very large intra- and suprasellar enhancing mass invading the central skull base. Note floccular calcifications <img src='img/arrows/BS.png'/> within the mass, an uncommon finding in macroadenomas.](95b8c653-566b-4169-8af8-5fe85a13bdfb)
**Pituitary Macroadenoma**
*Axial CECT shows a very large intra- and suprasellar enhancing mass invading the central skull base. Note floccular calcifications <img src='img/arrows/BS.png'/> within the mass, an uncommon finding in macroadenomas.*
![Sagittal bone CT shows globular calcification in a suprasellar mass <img src='img/arrows/WS.png'/> in this typical osteochondroma of the central skull base with calcification in the cartilaginous cap.](db0f24ca-c1e8-48f9-8071-f087589fa914)
**Chondroid Tumor**
*Sagittal bone CT shows globular calcification in a suprasellar mass <img src='img/arrows/WS.png'/> in this typical osteochondroma of the central skull base with calcification in the cartilaginous cap.*
![Axial NECT shows a hyperdense suprasellar mass <img src='img/arrows/CS.png'/> with focal calcifications <img src='img/arrows/CO.png'/> in this patient with a classic meningioma arising from the central skull base. Note the associated hydrocephalus <img src='img/arrows/WS.png'/> related to ventricular obstruction.](images/app.statdx.com_image_thumbnail_2111ba3c-137d-4717-9311-8d32dbfc155c_annotated_true_size_900_quality_90_73f0f783.jpg)
**Meningioma**
*Axial NECT shows a hyperdense suprasellar mass <img src='img/arrows/CS.png'/> with focal calcifications <img src='img/arrows/CO.png'/> in this patient with a classic meningioma arising from the central skull base. Note the associated hydrocephalus <img src='img/arrows/WS.png'/> related to ventricular obstruction.*
![Axial NECT shows a huge, well-delineated, hyperdense suprasellar mass with rim calcifications <img src='img/arrows/CS.png'/>. A giant, mostly thrombosed, saccular aneurysm was diagnosed and treated surgically. MR may show a laminated appearance with pulsation artifact if the aneurysm is patent.](images/app.statdx.com_image_thumbnail_192bafea-6ad6-4a4b-b208-47af9165fc27_annotated_true_size_900_quality_90_e1a755d3.jpg)
**Saccular Aneurysm**
*Axial NECT shows a huge, well-delineated, hyperdense suprasellar mass with rim calcifications <img src='img/arrows/CS.png'/>. A giant, mostly thrombosed, saccular aneurysm was diagnosed and treated surgically. MR may show a laminated appearance with pulsation artifact if the aneurysm is patent.*
![Axial NECT shows a large, hypodense, calcified suprasellar mass <img src='img/arrows/CS.png'/>. Note fat density droplets <img src='img/arrows/WO.png'/> in the subarachnoid space related to a ruptured dermoid cyst.](aaeaa251-a331-4ca5-b854-b42d581c1782)
**Dermoid Cyst**
*Axial NECT shows a large, hypodense, calcified suprasellar mass <img src='img/arrows/CS.png'/>. Note fat density droplets <img src='img/arrows/WO.png'/> in the subarachnoid space related to a ruptured dermoid cyst.*
![Coronal NECT shows a complex cystic expansile mass in the sella and suprasellar cistern <img src='img/arrows/WO.png'/> with peripheral calcification <img src='img/arrows/WS.png'/> in this case of pituitary macroadenoma with cystic degeneration.](16317746-cdd8-4e58-a609-f831cab62bd6)
**Pituitary Macroadenoma**
*Coronal NECT shows a complex cystic expansile mass in the sella and suprasellar cistern <img src='img/arrows/WO.png'/> with peripheral calcification <img src='img/arrows/WS.png'/> in this case of pituitary macroadenoma with cystic degeneration.*
![Coronal NECT shows a destructive low- density mass <img src='img/arrows/WO.png'/> with internal chondroid calcifications <img src='img/arrows/WS.png'/> extending from the central skull base into the suprasellar cistern in this case of chondrosarcoma.](a4d5358b-ae13-4c83-b768-58c4c3c2881d)
**Chondroid Tumor**
*Coronal NECT shows a destructive low- density mass <img src='img/arrows/WO.png'/> with internal chondroid calcifications <img src='img/arrows/WS.png'/> extending from the central skull base into the suprasellar cistern in this case of chondrosarcoma.*
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title: "Craniocervical Junction Acute Injury"
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---
# ESSENTIAL INFORMATION
- ## Key Differential Diagnosis Issues
- Coronal, sagittal CT reformations essential for full evaluation of injury
- MR very useful to evaluate for ligament injuries, cord injury, disc herniations
- CT angiogram equally accurate (and faster) than MR angiogram for vertebral dissection
- Time is often of essence in these patients, who tend to have multiple injuries
- ## Helpful Clues for Common Diagnoses
- **Occipital Condyle Fracture**
- CT best method of diagnosis
- Symptoms and outcome determined by associated head injury
- Cranial nerve deficits in 30%
- **Jefferson C1 Fracture**
- If combined displacement of lateral masses > 6.9 mm, unstable
- High likelihood of other fractures: Spine, skull, pelvis, lower extremity
- **Odontoid C2 Fracture**
- Usually low-velocity injury in older adult
- Type II most frequent
- **Chronic Injury Mimics**
- **Os odontoideum**
- Chronic, nonunited odontoid fracture
- Smooth corticated margins
- **Nontraumatic Mimics**
- **Pseudosubluxation C2-C3**
- Children < 10 years old; anterolisthesis may measure up to 4 mm
- ## Helpful Clues for Less Common Diagnoses
- **Atlantoccipital Dislocation**
- High incidence of cord injury
- Formerly usually fatal; now patients often survive to hospital
- **Atlantoaxial Dislocation**
- Normal atlantoaxial joint space < 3.4 mm in adults on CT
- **Spinal Cord Injury Without Radiographic Abnormality (SCIWORA)**
- Occurs primarily in children
- MR: Injuries to cord, ligaments, intervertebral discs, cartilaginous endplates
- 2/3 of severe cervical injuries in children < 8 years are SCIWORA
## Images
### Selected Images
![Coronal NECT shows a nondisplaced occipital condyle fracture <img src='img/arrows/WS.png'/> as well as a C4 articular pillar fracture <img src='img/arrows/WO.png'/>, reflecting lateral flexion injury.](images/app.statdx.com_image_thumbnail_22402513-d562-489c-8431-9e1d973aedc7_annotated_true_size_900_quality_90_19489e91.jpg)
**Occipital Condyle Fracture**
*Coronal NECT shows a nondisplaced occipital condyle fracture <img src='img/arrows/WS.png'/> as well as a C4 articular pillar fracture <img src='img/arrows/WO.png'/>, reflecting lateral flexion injury.*
![Coronal NECT shows a nondisplaced occipital condyle fracture <img src='img/arrows/WS.png'/> as well as a C4 articular pillar fracture <img src='img/arrows/WO.png'/>, reflecting lateral flexion injury.](images/app.statdx.com_image_thumbnail_22402513-d562-489c-8431-9e1d973aedc7_size_174_quality_85_bd3c6fac.jpg)
**Occipital Condyle Fracture**
*Coronal NECT shows a nondisplaced occipital condyle fracture <img src='img/arrows/WS.png'/> as well as a C4 articular pillar fracture <img src='img/arrows/WO.png'/>, reflecting lateral flexion injury.*
![Axial CT shows a type 3 condyle fracture <img src='img/arrows/WS.png'/> rotated into the foramen magnum. Note the normal position of the right condyle <img src='img/arrows/WO.png'/>.](images/app.statdx.com_image_thumbnail_31a951d8-aba5-459c-b13e-2f69d8276bff_annotated_true_size_900_quality_90_23074150.jpg)
**Occipital Condyle Fracture**
*Axial CT shows a type 3 condyle fracture <img src='img/arrows/WS.png'/> rotated into the foramen magnum. Note the normal position of the right condyle <img src='img/arrows/WO.png'/>.*
![Axial bone CT shows multiple fractures of the C1 ring <img src='img/arrows/WS.png'/>. Lateral displacement in this patient indicates rupture of the transverse ligament of dens and resultant instability.](images/app.statdx.com_image_thumbnail_19499959-3e5b-48bd-9b30-b909cff6d8b0_annotated_true_size_900_quality_90_7d2689c1.jpg)
**Jefferson C1 Fracture**
*Axial bone CT shows multiple fractures of the C1 ring <img src='img/arrows/WS.png'/>. Lateral displacement in this patient indicates rupture of the transverse ligament of dens and resultant instability.*
![Parasagittal CT shows a condyle fracture <img src='img/arrows/WS.png'/> that has maintained its relationship with the lateral mass of C1 <img src='img/arrows/WO.png'/> despite the posterior displacement.](images/app.statdx.com_image_thumbnail_2f771c4a-f8d7-4d16-a80f-6355efe3eb0b_annotated_true_size_900_quality_90_a2de4bd8.jpg)
**Occipital Condyle Fracture**
*Parasagittal CT shows a condyle fracture <img src='img/arrows/WS.png'/> that has maintained its relationship with the lateral mass of C1 <img src='img/arrows/WO.png'/> despite the posterior displacement.*
![Sagittal NECT shows a type 2 dens fracture <img src='img/arrows/WS.png'/> in an osteoporotic patient. Soft tissue swelling is mild. These fractures are commonly subtle on radiographs and best seen on lateral (not odontoid) view. If there is any question, perform CT.](images/app.statdx.com_image_thumbnail_3dadafa9-51c9-4e1a-ab36-c6d405d30c98_annotated_true_size_900_quality_90_b8da6fbe.jpg)
**Odontoid C2 Fracture**
*Sagittal NECT shows a type 2 dens fracture <img src='img/arrows/WS.png'/> in an osteoporotic patient. Soft tissue swelling is mild. These fractures are commonly subtle on radiographs and best seen on lateral (not odontoid) view. If there is any question, perform CT.*
![Plain radiograph shows a fracture through the base of the odontoid <img src='img/arrows/WS.png'/>, which is displaced posteriorly with slight extension.](images/app.statdx.com_image_thumbnail_aa3c67e2-3714-401d-9f44-21a90698c14a_annotated_true_size_900_quality_90_8f8bc33f.jpg)
**Odontoid C2 Fracture**
*Plain radiograph shows a fracture through the base of the odontoid <img src='img/arrows/WS.png'/>, which is displaced posteriorly with slight extension.*
![Sagittal bone CT shows a comminuted C2 body fracture with characteristic retropulsion of the posterior cortex <img src='img/arrows/WS.png'/>. Additional burst fractures are commonly present elsewhere in the spine.](images/app.statdx.com_image_thumbnail_16441d90-afd1-4d99-92d6-89b22dd6b410_annotated_true_size_900_quality_90_84149ae5.jpg)
**Burst Fracture, C2**
*Sagittal bone CT shows a comminuted C2 body fracture with characteristic retropulsion of the posterior cortex <img src='img/arrows/WS.png'/>. Additional burst fractures are commonly present elsewhere in the spine.*
![Sagittal T1WI MR shows a well-defined fracture line <img src='img/arrows/WO.png'/>. MR is useful for defining soft tissue abnormalities but should not be relied upon for pure bony fracture identification.](images/app.statdx.com_image_thumbnail_6bf9d862-33fc-4cb3-9dde-9479af9438bb_annotated_true_size_900_quality_90_c1ef2150.jpg)
**Odontoid C2 Fracture**
*Sagittal T1WI MR shows a well-defined fracture line <img src='img/arrows/WO.png'/>. MR is useful for defining soft tissue abnormalities but should not be relied upon for pure bony fracture identification.*
![Sagittal oblique 3D CT shows bilateral C2 pedicle fractures <img src='img/arrows/WS.png'/> without fracture of the vertebral body. Effendi classification uses the presence of disruption of the C2-C3 disc and facet joints as a measure of the severity of injury.](images/app.statdx.com_image_thumbnail_dcf76a6a-f291-4769-9c32-5adff2b85b11_annotated_true_size_900_quality_90_f006a904.jpg)
**Hangman's C2 Fracture**
*Sagittal oblique 3D CT shows bilateral C2 pedicle fractures <img src='img/arrows/WS.png'/> without fracture of the vertebral body. Effendi classification uses the presence of disruption of the C2-C3 disc and facet joints as a measure of the severity of injury.*
![3D CT reconstruction visualizes the markedly left rotated C1 <img src='img/arrows/WS.png'/> with respect to the mildly rotated C2 <img src='img/arrows/WO.png'/>. Findings are typical of a Fielding-Hawkins type I atlantoaxial rotatory fixation.](images/app.statdx.com_image_thumbnail_389cbe27-130d-43d4-bb61-0de9b7351a60_annotated_true_size_900_quality_90_4a71dae3.jpg)
**Atlantoaxial Rotary Subluxation/Fixation**
*3D CT reconstruction visualizes the markedly left rotated C1 <img src='img/arrows/WS.png'/> with respect to the mildly rotated C2 <img src='img/arrows/WO.png'/>. Findings are typical of a Fielding-Hawkins type I atlantoaxial rotatory fixation.*
![Sagittal bone CT shows a chronic, nonunited dens fracture <img src='img/arrows/WS.png'/>, so-called os odontoideum. This may be unstable, and flexion-extension views should be performed.](images/app.statdx.com_image_thumbnail_be54fabc-1abd-4497-9a41-d0026f038bee_annotated_true_size_900_quality_90_7bd5114c.jpg)
**Os Odontoideum**
*Sagittal bone CT shows a chronic, nonunited dens fracture <img src='img/arrows/WS.png'/>, so-called os odontoideum. This may be unstable, and flexion-extension views should be performed.*
![Axial bone CT shows anterior and posterior clefts <img src='img/arrows/WO.png'/> of C1. Smooth, corticated margins are signs distinguishing this from trauma.](images/app.statdx.com_image_thumbnail_8d090e1e-c409-4243-8174-776cc1e1a3b5_annotated_true_size_900_quality_90_badad089.jpg)
**Craniovertebral Junction Variants**
*Axial bone CT shows anterior and posterior clefts <img src='img/arrows/WO.png'/> of C1. Smooth, corticated margins are signs distinguishing this from trauma.*
![Sagittal bone CT shows the anterior arch of C1 fused to the clivus <img src='img/arrows/WS.png'/> and posterior arch fused to C2 <img src='img/arrows/WO.png'/>.](images/app.statdx.com_image_thumbnail_f24c53ea-0379-4092-ace0-2ae942371b20_annotated_true_size_900_quality_90_b395b841.jpg)
**Craniovertebral Junction Variants**
*Sagittal bone CT shows the anterior arch of C1 fused to the clivus <img src='img/arrows/WS.png'/> and posterior arch fused to C2 <img src='img/arrows/WO.png'/>.*
![Parasagittal CT shows posterior displacement of the lateral mass of C1 with respect to the occipital condyle <img src='img/arrows/WO.png'/>. The normal condyle would be closely applied to the lateral mass of C1.](images/app.statdx.com_image_thumbnail_407a344a-a2cd-40a5-8118-8c26359f3560_annotated_true_size_900_quality_90_4224ccac.jpg)
**Atlantooccipital Dislocation**
*Parasagittal CT shows posterior displacement of the lateral mass of C1 with respect to the occipital condyle <img src='img/arrows/WO.png'/>. The normal condyle would be closely applied to the lateral mass of C1.*
![Sagittal CT shows widening of the basion-dental interval <img src='img/arrows/WS.png'/> and slight posterior displacement of the odontoid with respect to the foramen magnum <img src='img/arrows/WC.png'/> (i.e., abnormal Wackenheim clival line).](images/app.statdx.com_image_thumbnail_a1a12cf6-aa3c-46c9-a6a9-4d2a23636c49_annotated_true_size_900_quality_90_26665e78.jpg)
**Atlantooccipital Dislocation**
*Sagittal CT shows widening of the basion-dental interval <img src='img/arrows/WS.png'/> and slight posterior displacement of the odontoid with respect to the foramen magnum <img src='img/arrows/WC.png'/> (i.e., abnormal Wackenheim clival line).*
![Parasagittal CT shows slight widening of both C0-C1 <img src='img/arrows/WS.png'/> and C1-C2 <img src='img/arrows/WO.png'/> articulations in this patient with both atlantooccipital and atlantoaxial subluxations.](images/app.statdx.com_image_thumbnail_98037363-0472-44eb-8b5a-fc14ec05b686_annotated_true_size_900_quality_90_c0a16653.jpg)
**Atlantooccipital Dislocation**
*Parasagittal CT shows slight widening of both C0-C1 <img src='img/arrows/WS.png'/> and C1-C2 <img src='img/arrows/WO.png'/> articulations in this patient with both atlantooccipital and atlantoaxial subluxations.*
![Sagittal STIR MR shows diffuse prevertebral high signal from edema <img src='img/arrows/WS.png'/> and a widened basion-dental interval <img src='img/arrows/WO.png'/>. Note the disruption of the tectorial membrane <img src='img/arrows/WC.png'/>.](images/app.statdx.com_image_thumbnail_8f33afe7-f373-4a01-8367-1326adb50a2f_annotated_true_size_900_quality_90_48528ce6.jpg)
**Atlantooccipital Dislocation**
*Sagittal STIR MR shows diffuse prevertebral high signal from edema <img src='img/arrows/WS.png'/> and a widened basion-dental interval <img src='img/arrows/WO.png'/>. Note the disruption of the tectorial membrane <img src='img/arrows/WC.png'/>.*
![Sagittal STIR MR shows abnormal hyperintensity within the C0-C1 and C1-C2 articulations <img src='img/arrows/WC.png'/> due to simultaneous atlantooccipital and atlantoaxial subluxations. The 2-level abnormality is important to identify since the posterior fusion will be extended more inferiorly.](images/app.statdx.com_image_thumbnail_97696e35-2c9f-4005-ad52-e16beb479377_annotated_true_size_900_quality_90_d3b240bb.jpg)
**Atlantoaxial Dislocation**
*Sagittal STIR MR shows abnormal hyperintensity within the C0-C1 and C1-C2 articulations <img src='img/arrows/WC.png'/> due to simultaneous atlantooccipital and atlantoaxial subluxations. The 2-level abnormality is important to identify since the posterior fusion will be extended more inferiorly.*
![Coronal CT shows the disrupted spinal articulations at C1-C2 (atlantoaxial dissociation). There is widening of the atlantoodontoid articulation <img src='img/arrows/WS.png'/> and widening/dislocation of the lateral masses of C1 and C2 <img src='img/arrows/WC.png'/>.](images/app.statdx.com_image_thumbnail_0c22bdc8-8b24-422e-b106-286a9b4aa82a_annotated_true_size_900_quality_90_7d289946.jpg)
**Atlantoaxial Dislocation**
*Coronal CT shows the disrupted spinal articulations at C1-C2 (atlantoaxial dissociation). There is widening of the atlantoodontoid articulation <img src='img/arrows/WS.png'/> and widening/dislocation of the lateral masses of C1 and C2 <img src='img/arrows/WC.png'/>.*
![Sagittal CT shows gross widening of the atlantoodontoid articulation <img src='img/arrows/WS.png'/>. The ring of C1 has maintained position with respect to the foramen magnum <img src='img/arrows/WO.png'/>, but C2 is displaced posteriorly <img src='img/arrows/WC.png'/>.](images/app.statdx.com_image_thumbnail_28794e41-818e-4487-ad3b-e985c6c7189e_annotated_true_size_900_quality_90_edba48d6.jpg)
**Atlantoaxial Dislocation**
*Sagittal CT shows gross widening of the atlantoodontoid articulation <img src='img/arrows/WS.png'/>. The ring of C1 has maintained position with respect to the foramen magnum <img src='img/arrows/WO.png'/>, but C2 is displaced posteriorly <img src='img/arrows/WC.png'/>.*
### Additional Images
![Sagittal bone CT shows occipital condyle <img src='img/arrows/BC.png'/> widely separated from lateral mass of C1 <img src='img/arrows/WC.png'/>, indicating longitudinal atlantooccipital dislocation. Dislocation may also be anterior or posterior in direction.](images/app.statdx.com_image_thumbnail_63bcd917-55ed-466c-b898-55064cf3cd8a_annotated_true_size_900_quality_90_234d1fdf.jpg)
**Atlantooccipital Dislocation**
*Sagittal bone CT shows occipital condyle <img src='img/arrows/BC.png'/> widely separated from lateral mass of C1 <img src='img/arrows/WC.png'/>, indicating longitudinal atlantooccipital dislocation. Dislocation may also be anterior or posterior in direction.*
docs_md/articles/craniopharyngioma_00e66680-6731-4287-b5a1-3f0b3f09053b.md
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---
title: "Craniopharyngioma"
docid: "00e66680-6731-4287-b5a1-3f0b3f09053b"
authors:
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slug: "diagnosis"
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pageDescription: "Craniopharyngioma"
pageKeywords: "Brain, Diagnosis, Anatomy-Based Diagnoses, Sella and Pituitary, Neoplasms, Craniopharyngioma"
pageTitle: "Craniopharyngioma | STATdx"
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breadcrumbs:
- "Brain"
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- "Sella and Pituitary"
- "Neoplasms"
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---
# KEY FACTS
- ## Terminology
- Benign, partially cystic sellar region tumor derived from remnants of craniopharyngeal duct/Rathke pouch epithelium
- 2 types
- Adamantinomatous (cystic mass in childhood)
- Papillary (solid mass in older adults)
- ## Imaging
- General features
- Multilobulated, often large (> 5 cm)
- Occasionally giant, multicompartmental
- CT: Cystic (90%), Ca⁺⁺ (90%), enhancing (90%)
- MR: Signal varies with cyst contents
- Cysts variably hyperintense on T1WI and T2WI
- Solid portions enhance heterogeneously; cyst walls enhance strongly
- Cyst contents show broad lipid peak (0.9-1.5 ppm) on MR spectroscopy
- ## Pathology
- Most common pediatric intracranial tumor of nonglial origin
- WHO grade 1
- ## Clinical Issues
- Bimodal age distribution
- Peak 5-15 years; adults 45-60 years (commonly papillary)
- Pediatric patient with morning headache, visual defect, short stature
- Endocrine disturbances include growth hormone (GH) deficiency, luteinizing hormone (LH)/follicle-stimulating hormone (FSH) deficiency
- Others = hypothyroidism > adrenal failure > diabetes insipidus
- Surgical resection is primary therapy
- Surgery, radiation therapy, or cyst aspiration for recurrent tumors
# TERMINOLOGY
- ## Abbreviations
- Craniopharyngioma (CP)
- ## Synonyms
- Craniopharyngeal duct tumor, Rathke pouch tumor, adamantinoma
- ## Definitions
- Benign, partially cystic sellar region tumor derived from Rathke pouch epithelium
- 2 histologies: Adamantinomatous and papillary
# IMAGING
- ## General Features
- ### Best diagnostic clue
- CT: Partially Ca⁺⁺ mixed solid/cystic suprasellar mass in child
- MR: Complex signal intensity suprasellar mass
- ### Location
- Surgical division of CPs into 3 groups
- Sellar
- Prechiasmatic
- Retrochiasmatic
- Imaging locations of CPs (adamantinomatous type)
- Suprasellar (75%)
- Suprasellar + intrasellar component (21%)
- Entirely intrasellar (4%)
- Often extends into multiple cranial fossae: Anterior (30%), middle (23%), posterior, &/or retroclival (20%)
- Rare ectopic locations
- Optic chiasm, 3rd ventricle
- Other: Nasopharynx, paranasal sinuses, pineal gland, sphenoid (clivus), cerebellopontine angle
- ### Size
- Variable; often large at presentation (> 5 cm)
- Occasionally giant, multicompartmental
- ### Morphology
- Multilobulated, multicystic
- ## CT Findings
- ### NECT
- Adamantinomatous type (90% rule)
- 90% mixed solid (isodense), cystic (hypodense)
- 90% calcify
- 90% enhance (solid = nodule; rim = capsule)
- Papillary type: Often solid, isodense, rarely calcifies
- ## MR Findings
- ### T1WI
- Signal varies with cyst contents
- Short T1 due to high protein content
- Classic (adamantinomatous type)
- Hyperintense cyst + heterogeneous nodule
- Less common (papillary type)
- Isointense solid component
- ### T2WI
- Cysts are variably hyperintense
- Solid component = heterogeneous (iso-/hyperintense, Ca⁺⁺ portions hypointense)
- Hyperintense signal in brain parenchyma adjacent to tumor may indicate
- Gliosis, tumor invasion, irritation from leaking cyst fluid
- Edema from compression of optic chiasm/tracts
- Hypointense T2* = Ca⁺⁺
- ### FLAIR
- Cyst contents typically hyperintense
- ### DWI
- Variable depending upon character of cyst fluid
- ### T1WI C+
- Solid portions enhance heterogeneously; cyst walls enhance strongly
- ### MRA
- Vascular displacement &/or encasement
- ### MRS
- Cyst contents show broad lipid spectrum (0.9-1.5 ppm)
- ## Imaging Recommendations
- ### Best imaging tool
- MR with thin sagittal, coronal sequences
- ### Protocol advice
- Pre-/postcontrast T1WI, T2, FLAIR, GRE, DWI, MRS
# DIFFERENTIAL DIAGNOSIS
- [Rathke Cleft Cyst](/document/rathke-cleft-cyst/8f1561f7-92a7-485c-a0ae-2e2d5c8c1628)
- Noncalcified, less heterogeneous
- Look for intracystic nodule on T2
- Does not enhance
- Claw sign (enhancing pituitary draped around cyst)
- Small Rathke cleft cyst (RCC) may be indistinguishable from rare intrasellar CP
- RCCs express CK8 and CK20 (CPs generally do not)
- ## Suprasellar Arachnoid Cyst
- No Ca⁺⁺, enhancement
- ## Hypothalamic/Chiasmatic Astrocytoma
- Solid or with small cystic/necrotic components
- Ca⁺⁺ is rare; robust enhancement is common
- ## Pituitary Adenoma
- Rare in prepubescent children
- Isointense with brain
- Enhances strongly
- Can mimic CP when cystic and hemorrhagic
- ## Epidermoid/Dermoid Tumors
- Minimal or no enhancement
- ## Thrombosed Aneurysm
- Contains blood products; use SWI
- Look for residual patent lumen, phase artifact
- [Germinoma or Mixed Germ Cell Tumor With Cystic Component(s)](/document/germinoma/078b68a2-67de-457e-818a-63655cec95aa)
- Cerebrospinal fluid spread is common, Ca⁺⁺ is rare
# PATHOLOGY
- ## General Features
- ### Etiology
- 2 proposed theories
- CPs arise from remnants of craniopharyngeal duct and Rathke pouch epithelium
- CPs arise from squamous epithelial cells in pars tuberalis of adenohypophysis
- ### Genetics
- No known genetic susceptibility (rare reports of siblings, parent-child)
- Small subset of CPs are monoclonal tumors that arise from oncogenes at specific loci
- Adamantinomatous: *CTNNB1* mutations and aberrant nuclear expression of β-catenin in up to 95% of cases
- Papillary:*BRAF* V600E mutations in 81-95% of cases
- ## Staging, Grading, & Classification
- WHO grade 1
- MIB-1 labeling index > 7% predicts recurrence
- ## Gross Pathologic & Surgical Features
- Solid tumor with variable cysts
- Adamantinomatous cysts often contain thick "crankcase oil" fluid
- Epithelial fronds penetrate adjacent hypothalamus/chiasm
- ## Microscopic Features
- Adamantinomatous (mostly pediatric)
- Multistratified squamous epithelium with nuclear palisading
- Nodules of "wet" keratin
- Dystrophic Ca⁺⁺
- Papillary (mostly adults)
- Sheets of squamous epithelium form pseudopapillae
- Villous fibrovascular stroma
- Malignant transformation, distant metastases rare
- May occur with varied histologies, resulting in poor prognosis
# CLINICAL ISSUES
- ## Presentation
- ### Most common signs/symptoms
- Symptoms vary with location, size of tumor, age of patient
- Visual disturbances (60-85%)
- Bitemporal hemianopsia
- ### Other signs/symptoms
- Endocrine disturbances (52-87%)
- Growth hormone (GH) deficiency (75%) > luteinizing hormone (LH)/follicle-stimulating hormone (FSH) deficiency > hypothyroidism > adrenal failure > diabetes insipidus
- Headaches
- Cognitive impairment (~ 50%)
- ### Clinical profile
- Pediatric patient with morning headache, visual defect, short stature
- ## Demographics
- ### Age
- Bimodal distribution (peak 5-15 years, with smaller peak 45-60 years)
- Papillary CP: 40-55 years
- ### Sex
- M = F
- ### Ethnicity
- More common in Japanese children
- ### Epidemiology
- Most common pediatric intracranial tumor of nonglial origin
- Comprise 1.2-4.6% of all intracranial tumors across all ages
- 6-11% of all pediatric intracranial tumors
- Incidence = 0.5-2.5 new cases per 1 million per year
- ~ 54% of all pediatric sellar/chiasmatic region tumors are CPs
- ## Natural History & Prognosis
- Typically slow-growing benign neoplasm
- Prognosis based upon size, extent of tumor at presentation
- < 5 cm, recurrence rate: 20%
- > 5 cm, recurrence rate: 83%
- Overall 10-year survival: 64-96%
- ## Treatment
- Methods of primary treatment
- Radical surgery = gross total resection
- Complications = hypothalamic injury, endocrine symptoms, vasa vasorum injury, and pseudoaneurysm
- Surgery may occur via craniotomy, transnasal, transorbital, or endoscopic routes
- Less invasive surgery = subtotal resection + radiation therapy
- Biopsy, cyst drainage, and radiation therapy
- Treatment for residual or recurrent tumor
- Surgery, radiation therapy, or cyst aspiration
- Cyst instillation with intracavitary radioisotopes, bleomycin, or other sclerosing agents
# DIAGNOSTIC CHECKLIST
- ## Consider
- Preoperative ophthalmologic and endocrine evaluations
- ## Image Interpretation Pearls
- Use NECT to detect Ca⁺⁺ if MR diagnosis is in question
- Adamantinomatous CP = 90% rule (90% cystic, calcified, enhancing)
- Papillary CP is typically solid and primarily adult neoplasm
c497473c-5835-4221-bfa1-0d2be04bee73
## References
# Selected References
1. [Azuma M et al: Usefulness of contrast-enhanced 3D-FLAIR MR imaging for differentiating Rathke cleft cyst from cystic craniopharyngioma. AJNR Am J Neuroradiol. 41(1):106-10, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=31857323%5Bpmid%5D)
1. [Fouda MA et al: Sixty years single institutional experience with pediatric craniopharyngioma: between the past and the future. Childs Nerv Syst. 36(2):291-6, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=31292757%5Bpmid%5D)
1. [Goldman S et al: Phase II study of peginterferon alpha-2b for patients with unresectable or recurrent craniopharyngiomas: a Pediatric Brain Tumor Consortium report. Neuro Oncol. ePub, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=32393959%5Bpmid%5D)
1. [Prince E et al: Transcriptional analyses of adult and pediatric adamantinomatous craniopharyngioma reveals similar expression signatures regarding potential therapeutic targets. Acta Neuropathol Commun. 8(1):68, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=32404202%5Bpmid%5D)
1. [Sadashivam S et al: Adult craniopharyngioma: the role of extent of resection in tumor recurrence and long-term functional outcome. Clin Neurol Neurosurg. 192:105711, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=32036264%5Bpmid%5D)
1. [Soldozy S et al: Endoscopic endonasal surgery outcomes for pediatric craniopharyngioma: a systematic review. Neurosurg Focus. 48(1):E6, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=31896083%5Bpmid%5D)
1. [Drapeau A et al: Pediatric craniopharyngioma. Childs Nerv Syst. 35(11):2133-45, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31385085%5Bpmid%5D)
1. [Madsen PJ et al: Endoscopic endonasal resection versus open surgery for pediatric craniopharyngioma: comparison of outcomes and complications. J Neurosurg Pediatr. 1-10, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31174192%5Bpmid%5D)
1. [Marcus HJ et al: Craniopharyngioma in children: trends from a third consecutive single-center cohort study. J Neurosurg Pediatr. 1-9, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31860822%5Bpmid%5D)
1. [Whelan R et al: Interrater reliability of a method to assess hypothalamic involvement in pediatric adamantinomatous craniopharyngioma. J Neurosurg Pediatr. 1-6, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31604324%5Bpmid%5D)
1. Buslei et al: Craniopharyngioma. In Louis DN et al: WHO Classification of Tumors of the Central Nervous System. IARC. 324-8, 2016
1. [Greenfield BJ et al: Long-term disease control and toxicity outcomes following surgery and intensity modulated radiation therapy (IMRT) in pediatric craniopharyngioma. Radiother Oncol. 114(2):224-9, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25542650%5Bpmid%5D)
1. [Kim JH et al: BRAF V600E mutation is a useful marker for differentiating Rathke's cleft cyst with squamous metaplasia from papillary craniopharyngioma. J Neurooncol. 123(1):189-91, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25820214%5Bpmid%5D)
1. [Lee HJ et al: Pretreatment diagnosis of suprasellar papillary craniopharyngioma and germ cell tumors of adult patients. AJNR Am J Neuroradiol. 36(3):508-17, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25339645%5Bpmid%5D)
1. [Sterkenburg AS et al: Survival, hypothalamic obesity, and neuropsychological/psychosocial status after childhood-onset craniopharyngioma: newly reported long-term outcomes. Neuro Oncol. 17(7):1029-38, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25838139%5Bpmid%5D)
1. [Lee CC et al: Gamma Knife surgery for craniopharyngioma: report on a 20-year experience. J Neurosurg. 121 Suppl:167-78, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=25434950%5Bpmid%5D)
1. [Clark AJ et al: A systematic review of the results of surgery and radiotherapy on tumor control for pediatric craniopharyngioma. Childs Nerv Syst. 29(2):231-8, 2013](http://www.ncbi.nlm.nih.gov/pubmed/?term=23089933%5Bpmid%5D)
1. [Müller HL: Childhood craniopharyngioma. Pituitary. 16(1):56-67, 2013](http://www.ncbi.nlm.nih.gov/pubmed/?term=22678820%5Bpmid%5D)
1. [Chentli F et al: Congenital craniopharyngioma: a case report and literature review. J Pediatr Endocrinol Metab. 25(11-12):1181-3, 2012](http://www.ncbi.nlm.nih.gov/pubmed/?term=23329768%5Bpmid%5D)
1. [Clark AJ et al: Treatment-related morbidity and the management of pediatric craniopharyngioma: a systematic review. J Neurosurg Pediatr. 10(4):293-301, 2012](http://www.ncbi.nlm.nih.gov/pubmed/?term=22920295%5Bpmid%5D)
1. [İnci MF et al: A rare presentation of craniopharyngioma: delayed puberty. BMJ Case Rep. 2012, 2012](http://www.ncbi.nlm.nih.gov/pubmed/?term=23195827%5Bpmid%5D)
1. [Shi Z et al: Transient enlargement of craniopharyngioma after radiation therapy: pattern of magnetic resonance imaging response following radiation. J Neurooncol. 109(2):349-55, 2012](http://www.ncbi.nlm.nih.gov/pubmed/?term=22692563%5Bpmid%5D)
1. [Boongird A et al: Malignant craniopharyngioma; case report and review of the literature. Neuropathology. 29(5):591-6, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=19077042%5Bpmid%5D)
1. [Frangou EM et al: Metastatic craniopharyngioma: case report and literature review. Childs Nerv Syst. 25(9):1143-7, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=19517118%5Bpmid%5D)
1. [Keil MF et al: Pituitary tumors in childhood: update of diagnosis, treatment and molecular genetics. Expert Rev Neurother. 8(4):563-74, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18416659%5Bpmid%5D)
1. [Garrè ML et al: Craniopharyngioma: modern concepts in pathogenesis and treatment. Curr Opin Pediatr. 19(4):471-9, 2007](http://www.ncbi.nlm.nih.gov/pubmed/?term=17630614%5Bpmid%5D)
1. [Powers CJ et al: Cerebellopontine angle craniopharyngioma: case report and literature review. Pediatr Neurosurg. 43(2):158-63, 2007](http://www.ncbi.nlm.nih.gov/pubmed/?term=17337933%5Bpmid%5D)
1. [Rodriguez FJ et al: The spectrum of malignancy in craniopharyngioma. Am J Surg Pathol. 31(7):1020-8, 2007](http://www.ncbi.nlm.nih.gov/pubmed/?term=17592268%5Bpmid%5D)
1. [Shuman AG et al: Extracranial nasopharyngeal craniopharyngioma: case report. Neurosurgery. 60(4):E780-1; discussion E781, 2007](http://www.ncbi.nlm.nih.gov/pubmed/?term=17415187%5Bpmid%5D)
1. [Aquilina K et al: Primary cerebellopontine angle craniopharyngioma in a patient with gardner syndrome. Case report and review of the literature. J Neurosurg. 105(2):330-3, 2006](http://www.ncbi.nlm.nih.gov/pubmed/?term=17219843%5Bpmid%5D)
1. [Haupt R et al: Epidemiological aspects of craniopharyngioma. J Pediatr Endocrinol Metab. 19 Suppl 1:289-93, 2006](http://www.ncbi.nlm.nih.gov/pubmed/?term=16700303%5Bpmid%5D)
1. [Prabhu VC et al: The pathogenesis of craniopharyngiomas. Childs Nerv Syst. 21(8-9):622-7, 2005](http://www.ncbi.nlm.nih.gov/pubmed/?term=15965669%5Bpmid%5D)
1. [Wang KC et al: Origin of craniopharyngiomas: implication on the growth pattern. Childs Nerv Syst. 21(8-9):628-34, 2005](http://www.ncbi.nlm.nih.gov/pubmed/?term=16059733%5Bpmid%5D)
1. [Srinivasan S et al: Features of the metabolic syndrome after childhood craniopharyngioma. J Clin Endocrinol Metab. 89(1):81-6, 2004](http://www.ncbi.nlm.nih.gov/pubmed/?term=14715831%5Bpmid%5D)
1. [Behari S et al: Intrinsic third ventricular craniopharyngiomas: report on six cases and a review of the literature. Surg Neurol. 60(3):245-52; discussion 252-3, 2003](http://www.ncbi.nlm.nih.gov/pubmed/?term=12922045%5Bpmid%5D)
1. [Saeki N et al: MR imaging study of edema-like change along the optic tract in patients with pituitary region tumors. AJNR Am J Neuroradiol. 24(3):336-42, 2003](http://www.ncbi.nlm.nih.gov/pubmed/?term=12637278%5Bpmid%5D)
1. [Barajas MA et al: Multimodal management of craniopharyngiomas: neuroendoscopy, microsurgery, and radiosurgery. J Neurosurg. 97(5 Suppl):607-9, 2002](http://www.ncbi.nlm.nih.gov/pubmed/?term=12507105%5Bpmid%5D)
1. [Fujimoto Y et al: Craniopharyngioma involving the infrasellar region: a case report and review of the literature. Pediatr Neurosurg. 37(4):210-6, 2002](http://www.ncbi.nlm.nih.gov/pubmed/?term=12372916%5Bpmid%5D)
1. [Green AL et al: Craniopharyngioma in a mother and daughter. Acta Neurochir (Wien). 144(4):403-4, 2002](http://www.ncbi.nlm.nih.gov/pubmed/?term=12021891%5Bpmid%5D)
1. [Sekine S et al: Craniopharyngiomas of adamantinomatous type harbor beta-catenin gene mutations. Am J Pathol. 161(6):1997-2001, 2002](http://www.ncbi.nlm.nih.gov/pubmed/?term=12466115%5Bpmid%5D)
1. [Van Effenterre R et al: Craniopharyngioma in adults and children: a study of 122 surgical cases. J Neurosurg. 97(1):3-11, 2002](http://www.ncbi.nlm.nih.gov/pubmed/?term=12134929%5Bpmid%5D)
1. [Chen CJ: Suprasellar and infrasellar craniopharyngioma with a persistent craniopharyngeal canal: case report and review of the literature. Neuroradiology. 43(9):760-2, 2001](http://www.ncbi.nlm.nih.gov/pubmed/?term=11594427%5Bpmid%5D)
## Images
### Selected Images
![Sagittal graphic shows a predominantly cystic, partially solid, suprasellar mass with focal rim Ca⁺⁺. Note the small intrasellar component and fluid-fluid level. Craniopharyngiomas are the 90% tumors (90% cystic, 90% Ca⁺⁺, and 90% enhancing).](images/app.statdx.com_image_thumbnail_f94f79c3-abc9-4850-a981-c09355881668_size_168_quality_85_a65f69d1_20251014T205219Z.jpg)
*Sagittal graphic shows a predominantly cystic, partially solid, suprasellar mass with focal rim Ca⁺⁺. Note the small intrasellar component and fluid-fluid level. Craniopharyngiomas are the 90% tumors (90% cystic, 90% Ca⁺⁺, and 90% enhancing).*
![Sagittal graphic shows a predominantly cystic, partially solid, suprasellar mass with focal rim Ca⁺⁺. Note the small intrasellar component and fluid-fluid level. Craniopharyngiomas are the 90% tumors (90% cystic, 90% Ca⁺⁺, and 90% enhancing).](images/app.statdx.com_image_thumbnail_f94f79c3-abc9-4850-a981-c09355881668_size_174_quality_85_f5f4f66b_20251014T205217Z.jpg)
*Sagittal graphic shows a predominantly cystic, partially solid, suprasellar mass with focal rim Ca⁺⁺. Note the small intrasellar component and fluid-fluid level. Craniopharyngiomas are the 90% tumors (90% cystic, 90% Ca⁺⁺, and 90% enhancing).*
![Sagittal T2 MR in a 9 year old with headache and visual changes shows a heterogeneous cystic and solid sellar and suprasellar craniopharyngioma <img src='img/arrows/CO.png'/> with anterior extension into the sphenoid sinus <img src='img/arrows/WS.png'/> and superior displacement of the optic chiasm.](images/app.statdx.com_image_thumbnail_ff36ded9-e912-42b4-929c-ef63a5e8fa57_size_168_quality_85_f43afdfe_20251014T205219Z.jpg)
*Sagittal T2 MR in a 9 year old with headache and visual changes shows a heterogeneous cystic and solid sellar and suprasellar craniopharyngioma <img src='img/arrows/CO.png'/> with anterior extension into the sphenoid sinus <img src='img/arrows/WS.png'/> and superior displacement of the optic chiasm.*
![Coronal T2 MR in a 48-year-old man who presented with visual changes shows a cystic suprasellar mass <img src='img/arrows/WO.png'/> with a focal T2-hypointense nodule <img src='img/arrows/CS.png'/>. The T2 imaging mimics a Rathke cleft cyst.](images/app.statdx.com_image_thumbnail_a46de544-a40b-46b9-bd8e-dfba34730496_size_168_quality_85_27db027b_20251014T205219Z.jpg)
*Coronal T2 MR in a 48-year-old man who presented with visual changes shows a cystic suprasellar mass <img src='img/arrows/WO.png'/> with a focal T2-hypointense nodule <img src='img/arrows/CS.png'/>. The T2 imaging mimics a Rathke cleft cyst.*
![Sagittal T1 C+ MR in a 45-year-old woman shows a cystic and solid mass with an enhancing portion <img src='img/arrows/CO.png'/>, which distinguishes this cystic lesion as a craniopharyngioma, not a Rathke cleft cyst. Surgical resection is the primary therapy for this WHO grade 1 neoplasm. However, the recurrence rate at 10 years approaches 20%.](images/app.statdx.com_image_thumbnail_3db34480-5109-41cc-8a67-ce27096172de_size_168_quality_85_2a792ca4_20251014T205219Z.jpg)
*Sagittal T1 C+ MR in a 45-year-old woman shows a cystic and solid mass with an enhancing portion <img src='img/arrows/CO.png'/>, which distinguishes this cystic lesion as a craniopharyngioma, not a Rathke cleft cyst. Surgical resection is the primary therapy for this WHO grade 1 neoplasm. However, the recurrence rate at 10 years approaches 20%.*
![Gross pathologic specimen shows a typical solid and cystic composition <img src='img/arrows/BO.png'/> of an adamantinomatous craniopharyngioma. The cystic spaces contain a thick gelatinous material. (Courtesy AFIP.)](images/app.statdx.com_image_thumbnail_8fe443b2-0b89-48cc-a021-94c248ae56c4_size_168_quality_85_275ebdf6_20251014T205219Z.jpg)
*Gross pathologic specimen shows a typical solid and cystic composition <img src='img/arrows/BO.png'/> of an adamantinomatous craniopharyngioma. The cystic spaces contain a thick gelatinous material. (Courtesy AFIP.)*
![Coronal T2 MR in a 2 year old with a huge suprasellar mass shows multiple hyperintense cysts <img src='img/arrows/CS.png'/>. Adamantinomatous craniopharyngiomas typically present between 5-15 years in childhood and between 45-60 years in adults. They are the most common pediatric intracranial tumor of nonglial origin.](images/app.statdx.com_image_thumbnail_7e7095db-3e50-4824-a7f2-b2d59382d174_size_168_quality_85_dd1d82dd_20251014T205220Z.jpg)
*Coronal T2 MR in a 2 year old with a huge suprasellar mass shows multiple hyperintense cysts <img src='img/arrows/CS.png'/>. Adamantinomatous craniopharyngiomas typically present between 5-15 years in childhood and between 45-60 years in adults. They are the most common pediatric intracranial tumor of nonglial origin.*
![Axial NECT shows classic findings of an adamantinomatous craniopharyngioma. Note the large suprasellar cyst with a fluid-fluid level <img src='img/arrows/WO.png'/>, rim <img src='img/arrows/CC.png'/>, and globular <img src='img/arrows/CS.png'/> Ca⁺⁺. Enhancement was present on postcontrast images.](images/app.statdx.com_image_thumbnail_20fed2a7-ce3d-4ef8-aab8-c4cf436a651f_size_168_quality_85_f9740012_20251014T205220Z.jpg)
*Axial NECT shows classic findings of an adamantinomatous craniopharyngioma. Note the large suprasellar cyst with a fluid-fluid level <img src='img/arrows/WO.png'/>, rim <img src='img/arrows/CC.png'/>, and globular <img src='img/arrows/CS.png'/> Ca⁺⁺. Enhancement was present on postcontrast images.*
![Sagittal gross pathology shows classic adamantinomatous craniopharyngioma with mixed solid, cystic components. The classic machine or &quot;crankcase oil&quot; <img src='img/arrows/WO.png'/> is present. Note the intrasellar extension <img src='img/arrows/WC.png'/>. (Courtesy R. Hewlett, MD.)](images/app.statdx.com_image_thumbnail_5737449d-e941-4480-bb6c-7a8d8fc2f41a_size_168_quality_85_6ada1f4b_20251014T205219Z.jpg)
*Sagittal gross pathology shows classic adamantinomatous craniopharyngioma with mixed solid, cystic components. The classic machine or &quot;crankcase oil&quot; <img src='img/arrows/WO.png'/> is present. Note the intrasellar extension <img src='img/arrows/WC.png'/>. (Courtesy R. Hewlett, MD.)*
![Sagittal T1 C+ MR in a 45-year-old man with visual changes shows a solid enhancing suprasellar mass <img src='img/arrows/WS.png'/>. Papillary craniopharyngioma was diagnosed at resection. Papillary craniopharyngiomas are WHO grade 1 tumors; however, they are more commonly solid, noncalcified lesions.](images/app.statdx.com_image_thumbnail_1a1382d8-4859-4020-bd60-8f778d681863_size_168_quality_85_bb86662d_20251014T205219Z.jpg)
*Sagittal T1 C+ MR in a 45-year-old man with visual changes shows a solid enhancing suprasellar mass <img src='img/arrows/WS.png'/>. Papillary craniopharyngioma was diagnosed at resection. Papillary craniopharyngiomas are WHO grade 1 tumors; however, they are more commonly solid, noncalcified lesions.*
![Sagittal T1WI C+ MR shows a large recurrent craniopharyngioma in the central skull base and nasopharynx <img src='img/arrows/WS.png'/> with typical heterogeneous cystic and solid morphology. (Courtesy S. Blaser, MD.)](images/app.statdx.com_image_thumbnail_e2df5ace-0993-4081-9e77-4d8541d3bd6a_size_168_quality_85_c4a419f8_20251014T205219Z.jpg)
*Sagittal T1WI C+ MR shows a large recurrent craniopharyngioma in the central skull base and nasopharynx <img src='img/arrows/WS.png'/> with typical heterogeneous cystic and solid morphology. (Courtesy S. Blaser, MD.)*
### Additional Images
![Axial T1 C+ MR in a child with a huge multilobulated craniopharyngioma shows rim <img src='img/arrows/WS.png'/> and solid nodular <img src='img/arrows/WO.png'/> enhancement. Note that the cyst fluid is moderately hyperintense compared to CSF in the lateral ventricles.](images/app.statdx.com_image_thumbnail_97cdf507-1f5d-43cc-9886-65f26e0655fb_size_168_quality_85_3525f13e_20251014T205219Z.jpg)
*Axial T1 C+ MR in a child with a huge multilobulated craniopharyngioma shows rim <img src='img/arrows/WS.png'/> and solid nodular <img src='img/arrows/WO.png'/> enhancement. Note that the cyst fluid is moderately hyperintense compared to CSF in the lateral ventricles.*
![Axial DWI MR in the same patient with a large craniopharyngioma shows no restriction in the fluid-containing part of the tumor <img src='img/arrows/WS.png'/>.](images/app.statdx.com_image_thumbnail_12002415-4879-47aa-9a98-a0097e492049_size_168_quality_85_a3ee5d4f_20251014T205219Z.jpg)
*Axial DWI MR in the same patient with a large craniopharyngioma shows no restriction in the fluid-containing part of the tumor <img src='img/arrows/WS.png'/>.*
![Axial NECT shows a low-attenuation suprasellar mass with rim <img src='img/arrows/WS.png'/> and globular <img src='img/arrows/WC.png'/> Ca⁺⁺. Note the fluid-fluid level formed by intracystic keratin debris <img src='img/arrows/WO.png'/>.](images/app.statdx.com_image_thumbnail_ee1a8e06-2a41-47f1-b559-ea7a6c233786_size_168_quality_85_d549a7de_20251014T205219Z.jpg)
*Axial NECT shows a low-attenuation suprasellar mass with rim <img src='img/arrows/WS.png'/> and globular <img src='img/arrows/WC.png'/> Ca⁺⁺. Note the fluid-fluid level formed by intracystic keratin debris <img src='img/arrows/WO.png'/>.*
![Sagittal T1WI MR shows a complex predominantly cystic suprasellar mass. Note the T1 shortening within the cyst due to machine oil-like proteinaceous fluid <img src='img/arrows/WS.png'/>.](images/app.statdx.com_image_thumbnail_b5c10b79-9905-45ca-969b-3ecbb2003ac1_size_168_quality_85_39e04a96_20251014T205219Z.jpg)
*Sagittal T1WI MR shows a complex predominantly cystic suprasellar mass. Note the T1 shortening within the cyst due to machine oil-like proteinaceous fluid <img src='img/arrows/WS.png'/>.*
![Axial NECT shows a predominantly solid, minimally calcified <img src='img/arrows/WS.png'/>, suprasellar craniopharyngioma.](images/app.statdx.com_image_thumbnail_7b7888df-3246-4226-a063-e277639bdc87_size_168_quality_85_2370a8d3_20251014T205219Z.jpg)
*Axial NECT shows a predominantly solid, minimally calcified <img src='img/arrows/WS.png'/>, suprasellar craniopharyngioma.*
![Sagittal T1 C+ MR shows a principally cystic, sellar/suprasellar mass with rim enhancement <img src='img/arrows/WS.png'/>.](images/app.statdx.com_image_thumbnail_e8b015d3-9981-4dc9-ac0f-f3c80b3eda85_size_168_quality_85_d77cca56_20251014T205219Z.jpg)
*Sagittal T1 C+ MR shows a principally cystic, sellar/suprasellar mass with rim enhancement <img src='img/arrows/WS.png'/>.*
![Sagittal T1 C+ MR in a 48-year-old man with visual changes shows an enhancing nodule <img src='img/arrows/WO.png'/>, which distinguishes this cystic lesion as a craniopharyngioma, not a Rathke cleft cyst. Surgical resection is the primary therapy for this WHO grade 1 neoplasm; however, the recurrence rate at 10 years approaches 20%.](images/app.statdx.com_image_thumbnail_03c2e4a8-4ffe-4f84-975e-88dd1180d675_size_168_quality_85_c18501f8_20251014T205219Z.jpg)
*Sagittal T1 C+ MR in a 48-year-old man with visual changes shows an enhancing nodule <img src='img/arrows/WO.png'/>, which distinguishes this cystic lesion as a craniopharyngioma, not a Rathke cleft cyst. Surgical resection is the primary therapy for this WHO grade 1 neoplasm; however, the recurrence rate at 10 years approaches 20%.*
![Sagittal T1WI C+ MR shows a complex cystic suprasellar mass with an enhancing rim <img src='img/arrows/WC.png'/> and solid components <img src='img/arrows/WO.png'/>. The cysts contain fluid of different signal intensities. Note the large suprasellar, smaller intrasellar <img src='img/arrows/WS.png'/> components in this classic craniopharyngioma.](images/app.statdx.com_image_thumbnail_e7b6d109-2017-437b-9268-f9d51f5b4e9d_size_168_quality_85_26288ca2_20251014T205219Z.jpg)
*Sagittal T1WI C+ MR shows a complex cystic suprasellar mass with an enhancing rim <img src='img/arrows/WC.png'/> and solid components <img src='img/arrows/WO.png'/>. The cysts contain fluid of different signal intensities. Note the large suprasellar, smaller intrasellar <img src='img/arrows/WS.png'/> components in this classic craniopharyngioma.*
![A short TE (35) H-MRS in a patient with a solid and cystic craniopharyngioma acquired from the center of the cystic portion of the mass shows large lipid-lactate peaks <img src='img/arrows/WS.png'/>, characteristic of the cholesterol and lipid constituents found in cysts of craniopharyngiomas.](images/app.statdx.com_image_thumbnail_4d9c1527-9689-4e7b-b956-6ea22b77a13d_size_168_quality_85_d0f20fb4_20251014T205219Z.jpg)
*A short TE (35) H-MRS in a patient with a solid and cystic craniopharyngioma acquired from the center of the cystic portion of the mass shows large lipid-lactate peaks <img src='img/arrows/WS.png'/>, characteristic of the cholesterol and lipid constituents found in cysts of craniopharyngiomas.*
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---
# ESSENTIAL INFORMATION
- ## Key Differential Diagnosis Issues
- **Hint**: Differentiate trauma vs. bony congenital variant
- Soft tissue swelling usually evident in trauma
- Cortication of bone indicates nonacute trauma
- Os odontoideum thought to be nonunited fracture, not necessarily congenital variant
- **Hint**: Watch for mass adjacent to dens
- Pannus from rheumatoid arthritis (RA): Dens eroded, no calcification
- Seronegative spondyloarthropathy: Like RA, plus enthesophytes, joint fusion
- Juvenile inflammatory arthropathy: Like adult RA or seronegative spondyloarthropathy
- Usually involves multiple levels in cervical spine
- Growth disturbance characteristic
- Calcium pyrophosphate deposition disease (CPPD): Calcifications, cysts in bone
- Infection: Usually involves disc space
- Tuberculosis involves disc space later in course of infection
- Tumor: Origin in bone, meninges, or cord
- **Hint**: Watch for heterogeneous high signal in bone marrow without cortical breakthrough
- Myeloma
- Lymphoma
- Metastases
- ## Helpful Clues for Common Diagnoses
- **Bone Trauma**
- **Odontoid fracture, C2**
- Type I: Obliquely oriented through tip
- Type II: Horizontally oriented through base
- Type III: Really fracture of body; horizontally oriented, through body and below base of dens
- **Hangman's fracture, C2**
- Hyperflexion or hyperextension, usually from motor vehicle accident
- Traumatic spondylolisthesis of C2
- Fracture through C2 pedicles
- Usually see focal kyphosis and anterolisthesis at C2-C3
- Effendi type I: Traumatic spondylolisthesis isolated
- Effendi type II: Also disruption of C2-C3 disc
- Effendi type III: Also disruption of C2-C3 facet joints
- **Burst fracture, C2**
- Axial load injury
- Extends through posterior cortex of vertebral body
- **Os odontoideum**
- Chronic nonunited fracture
- **Congenital Bone and Ligament Abnormalities**
- May be multiple
- May be isolated, detected as incidental finding in adulthood
- Often cause adjacent premature degeneration
- **Trisomy 21**
- Spinal stenosis
- Instability occiput-C1 and C1-C2
- Unlike RA, no erosion of dens
- **Arthritis**
- **Osteoarthritis**
- Common at craniocervical junction
- Involves synovial articulations: Facet joints, dens/C1 articulation
- Dens and anterior arch of C1 develop osteophytes, sclerosis best seen on CT
- May have prominent soft tissues posterior to dens but no erosions
- Facet osteoarthritis at occiput-C1 or C1-C2 may develop large osteophytes, synovial cysts
- **Calcium pyrophosphate deposition disease**
- Mimics RA on MR, but subchondral bone plate not eroded
- Calcifications visible on CT, radiographs
- **Rheumatoid arthritis**
- Calcification never present
- Pannus heterogeneous signal intensity on MR
- Low signal intensity areas on T2WI mimic crystals, calcification
- Almost always see erosion of dens
- Early erosion: Loss of subchondral bone plate
- Late erosion: Pencilling of dens
- Facet erosion: Atlantoaxial impaction
- Craniocervical disease does not occur without peripheral disease (hands/feet)
## References
# Selected References
1. [Patel R et al: Surgical outcomes of posterior occipito-cervical decompression and fusion for basilar invagination: a prospective study. J Clin Orthop Trauma. 13:127-33, 2021](http://www.ncbi.nlm.nih.gov/pubmed/?term=33680811%5Bpmid%5D)
1. [Berritto D et al: Trauma imaging of the acute cervical spine. Semin Musculoskelet Radiol. 21(3):184-98, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=28571084%5Bpmid%5D)
1. [Hadley MN et al: Introduction to the uidelines for the management of acute cervical spine and spinal cord injuries. Neurosurgery. 72 Suppl 2:5-16, 2013](http://www.ncbi.nlm.nih.gov/pubmed/?term=23417174%5Bpmid%5D)
1. [Ryken TC et al: Radiographic assessment. Neurosurgery. 72 Suppl 2:54-72, 2013](http://www.ncbi.nlm.nih.gov/pubmed/?term=23417179%5Bpmid%5D)
1. [Theodore N et al: The diagnosis and management of traumatic atlanto-occipital dislocation injuries. Neurosurgery. 72 Suppl 2():114-26, 2013](http://www.ncbi.nlm.nih.gov/pubmed/?term=23417184%5Bpmid%5D)
1. [Munera F et al: Imaging evaluation of adult spinal injuries: emphasis on multidetector CT in cervical spine trauma. Radiology. 263(3):645-60, 2012](http://www.ncbi.nlm.nih.gov/pubmed/?term=22623691%5Bpmid%5D)
## Images
### Selected Images
![Axial bone CT shows small, bony density without cortical margins adjacent to the right condyle, consistent with a nondisplaced right occipital condyle avulsion fracture <img src='img/arrows/WS.png'/>.](images/app.statdx.com_image_thumbnail_42be470a-23e0-49db-93a6-05fff9707b64_annotated_true_size_900_quality_90_7311f508.jpg)
**Occipital Condyle Fracture**
*Axial bone CT shows small, bony density without cortical margins adjacent to the right condyle, consistent with a nondisplaced right occipital condyle avulsion fracture <img src='img/arrows/WS.png'/>.*
![Axial bone CT shows small, bony density without cortical margins adjacent to the right condyle, consistent with a nondisplaced right occipital condyle avulsion fracture <img src='img/arrows/WS.png'/>.](images/app.statdx.com_image_thumbnail_42be470a-23e0-49db-93a6-05fff9707b64_size_174_quality_85_8d291de2.jpg)
**Occipital Condyle Fracture**
*Axial bone CT shows small, bony density without cortical margins adjacent to the right condyle, consistent with a nondisplaced right occipital condyle avulsion fracture <img src='img/arrows/WS.png'/>.*
![Axial bone CT shows severe comminuted fractures involving both the posterior and anterior arches of C1 <img src='img/arrows/WS.png'/>. There is also an avulsion off of the medial aspect of the left C1 lateral mass at the insertion of the transverse ligament <img src='img/arrows/WO.png'/>. The atlantodental interval is normal.](images/app.statdx.com_image_thumbnail_fd74c237-f2e4-4f19-9200-33c12fa74bfe_annotated_true_size_900_quality_90_64675565.jpg)
**Jefferson C1 Fracture**
*Axial bone CT shows severe comminuted fractures involving both the posterior and anterior arches of C1 <img src='img/arrows/WS.png'/>. There is also an avulsion off of the medial aspect of the left C1 lateral mass at the insertion of the transverse ligament <img src='img/arrows/WO.png'/>. The atlantodental interval is normal.*
![Sagittal NECT shows a type II odontoid fracture <img src='img/arrows/WS.png'/>. There is slight posterior angulation and displacement of the anterior aspect of the fracture. This fracture usually occurs in older adult patients, often from a ground-level fall, and may be missed on radiographs due to osteopenia.](images/app.statdx.com_image_thumbnail_dd781c82-e5b8-48b7-9d23-e78108598646_annotated_true_size_900_quality_90_2249f82f.jpg)
**Odontoid Fracture, C2**
*Sagittal NECT shows a type II odontoid fracture <img src='img/arrows/WS.png'/>. There is slight posterior angulation and displacement of the anterior aspect of the fracture. This fracture usually occurs in older adult patients, often from a ground-level fall, and may be missed on radiographs due to osteopenia.*
![Sagittal NECT shows horizontal and vertical fractures of C2 <img src='img/arrows/WS.png'/> due to axial load injury. The posterior fragment can displace posteriorly and cause cord compression <img src='img/arrows/WO.png'/>.](images/app.statdx.com_image_thumbnail_0ce6a57a-70ee-4d09-bac3-ee3696ca5170_annotated_true_size_900_quality_90_1fa85219.jpg)
**Burst Fracture, C2**
*Sagittal NECT shows horizontal and vertical fractures of C2 <img src='img/arrows/WS.png'/> due to axial load injury. The posterior fragment can displace posteriorly and cause cord compression <img src='img/arrows/WO.png'/>.*
![Sagittal T2WI MR shows changes of Chiari 1 malformation with pronounced inferior position of the cerebellar tonsils <img src='img/arrows/WS.png'/>. There is assimilation of C1 to the occiput with abnormal position of the C1 arch <img src='img/arrows/WO.png'/>. Note the associated syrinx <img src='img/arrows/WC.png'/>.](be19bab7-01f5-4533-aa26-615cea990952)
**Chiari 1 Malformation**
*Sagittal T2WI MR shows changes of Chiari 1 malformation with pronounced inferior position of the cerebellar tonsils <img src='img/arrows/WS.png'/>. There is assimilation of C1 to the occiput with abnormal position of the C1 arch <img src='img/arrows/WO.png'/>. Note the associated syrinx <img src='img/arrows/WC.png'/>.*
![Sagittal T1WI MR shows the typical changes of Chiari 2 malformation with hindbrain herniation <img src='img/arrows/WS.png'/>, small posterior fossa, beaked tectum <img src='img/arrows/WO.png'/>, and dysmorphic clivus <img src='img/arrows/WC.png'/>. Note also the associated callosal hypoplasia <img src='img/arrows/CS.png'/>.](5d2efe84-86eb-4f40-88ea-9fb7ce864833)
**Chiari 2 Malformation**
*Sagittal T1WI MR shows the typical changes of Chiari 2 malformation with hindbrain herniation <img src='img/arrows/WS.png'/>, small posterior fossa, beaked tectum <img src='img/arrows/WO.png'/>, and dysmorphic clivus <img src='img/arrows/WC.png'/>. Note also the associated callosal hypoplasia <img src='img/arrows/CS.png'/>.*
![Lateral radiograph shows the C1 <img src='img/arrows/WS.png'/> fused to the occiput, resulting in dysmorphic C1-C2 articulations and dysmorphic C2 body. The odontoid is triangular in shape <img src='img/arrows/WC.png'/>.](c2bdac12-24ca-4601-88ec-62c86fc4ceb6)
**Craniovertebral Junction Variants**
*Lateral radiograph shows the C1 <img src='img/arrows/WS.png'/> fused to the occiput, resulting in dysmorphic C1-C2 articulations and dysmorphic C2 body. The odontoid is triangular in shape <img src='img/arrows/WC.png'/>.*
![Sagittal bone CT shows C1/C2 subluxation <img src='img/arrows/WO.png'/> without erosions. There was no history of trauma. Marked narrowing of the spinal canal was symptomatic.](6c7a2a1f-d3b1-4275-9c8f-5f0802c5b009)
**Trisomy 21**
*Sagittal bone CT shows C1/C2 subluxation <img src='img/arrows/WO.png'/> without erosions. There was no history of trauma. Marked narrowing of the spinal canal was symptomatic.*
![Coronal NECT shows marked degenerative change about the right C1-C2 joint <img src='img/arrows/WS.png'/> with joint space loss, bony sclerosis, and irregularity. Compare to the more normal left C1-C2 joint <img src='img/arrows/WO.png'/>.](2a64647d-97d4-412d-b03c-17b26bf9ce4e)
**Osteoarthritis**
*Coronal NECT shows marked degenerative change about the right C1-C2 joint <img src='img/arrows/WS.png'/> with joint space loss, bony sclerosis, and irregularity. Compare to the more normal left C1-C2 joint <img src='img/arrows/WO.png'/>.*
![Sagittal T2WI MR shows the cortical margin of the odontoid lost anteriorly due to erosions; the soft tissue mass <img src='img/arrows/WO.png'/> is due to pannus. Cranial settling is present with the odontoid at the level of clivus.](f72658a3-0656-4202-b810-e328cdf38208)
**Juvenile Idiopathic Arthritis**
*Sagittal T2WI MR shows the cortical margin of the odontoid lost anteriorly due to erosions; the soft tissue mass <img src='img/arrows/WO.png'/> is due to pannus. Cranial settling is present with the odontoid at the level of clivus.*
![Sagittal T2WI MR shows a large, predominately low signal intensity mass involving the dorsal retrodental soft tissues <img src='img/arrows/WS.png'/>. There is severe cord compression from the mass with cord signal abnormality <img src='img/arrows/WO.png'/>.](cb3b1744-c0fd-4207-8ff8-3344b2138aa8)
**Calcium Pyrophosphate Deposition Disease**
*Sagittal T2WI MR shows a large, predominately low signal intensity mass involving the dorsal retrodental soft tissues <img src='img/arrows/WS.png'/>. There is severe cord compression from the mass with cord signal abnormality <img src='img/arrows/WO.png'/>.*
![Axial NECT at the level of C2 shows a well-defined, lobulated mass with increased attenuation surrounding the lateral C1-C2 articulation. The lesion shows the appearance of milk of calcium with diffuse increased density.](cccbb711-fc39-421e-bf49-6ec2bfdd5dc5)
**Tumoral Calcinosis**
*Axial NECT at the level of C2 shows a well-defined, lobulated mass with increased attenuation surrounding the lateral C1-C2 articulation. The lesion shows the appearance of milk of calcium with diffuse increased density.*
![Sagittal T2WI MR shows a large prevertebral abscess spanning C1 to C4 <img src='img/arrows/WS.png'/> and extension posteriorly involving the interspinous region <img src='img/arrows/WO.png'/>. There is involvement of the C1-C2 articulation with widening of the atlantodental interval <img src='img/arrows/WC.png'/>.](e2a4c5db-af07-4147-b954-0054d019d151)
**Osteomyelitis, C1-C2**
*Sagittal T2WI MR shows a large prevertebral abscess spanning C1 to C4 <img src='img/arrows/WS.png'/> and extension posteriorly involving the interspinous region <img src='img/arrows/WO.png'/>. There is involvement of the C1-C2 articulation with widening of the atlantodental interval <img src='img/arrows/WC.png'/>.*
![Sagittal T2WI MR shows a mass involving the craniocervical junction with marked T2 hyperintensity and spiculated internal morphology <img src='img/arrows/WS.png'/>. Morphology would be consistent with either chordoma or chondrosarcoma. The location is much more typical for chordoma.](1a6ee850-ea76-41b5-b248-9dee2dba22ac)
**Chordoma**
*Sagittal T2WI MR shows a mass involving the craniocervical junction with marked T2 hyperintensity and spiculated internal morphology <img src='img/arrows/WS.png'/>. Morphology would be consistent with either chordoma or chondrosarcoma. The location is much more typical for chordoma.*
![Sagittal NECT shows findings typical for congenital assimilation of C1 to the occiput with a high-riding position of the C1 anterior arch parked underneath the inferior clivus <img src='img/arrows/WS.png'/>, upward translocation of the odontoid <img src='img/arrows/WO.png'/>, and lack of visible posterior C1 arch <img src='img/arrows/WC.png'/>.](b79a7ed0-70c5-4b7a-b18c-fceae7f142ed)
**Atlantooccipital Assimilation**
*Sagittal NECT shows findings typical for congenital assimilation of C1 to the occiput with a high-riding position of the C1 anterior arch parked underneath the inferior clivus <img src='img/arrows/WS.png'/>, upward translocation of the odontoid <img src='img/arrows/WO.png'/>, and lack of visible posterior C1 arch <img src='img/arrows/WC.png'/>.*
![Sagittal T1WI MR in this patient with extensive Paget disease involving the calvarium <img src='img/arrows/WS.png'/> shows basilar impression with upward translocation of the odontoid into the foramen magnum and draping deformity <img src='img/arrows/WO.png'/> of the brainstem.](750d385d-2a85-4e22-b626-b2a9d89fba00)
**Paget Disease**
*Sagittal T1WI MR in this patient with extensive Paget disease involving the calvarium <img src='img/arrows/WS.png'/> shows basilar impression with upward translocation of the odontoid into the foramen magnum and draping deformity <img src='img/arrows/WO.png'/> of the brainstem.*
### Additional Images
![Sagittal bone CT shows atrophic odontoid fracture nonunion <img src='img/arrows/WO.png'/> after open reduction and internal fixation (ORIF). This usually is due not to infection but poor vascularity.](images/app.statdx.com_image_thumbnail_d5ff872f-cb4b-48ee-8100-627a740b631d_annotated_true_size_900_quality_90_a1bffda1.jpg)
**Odontoid Fracture, C2**
*Sagittal bone CT shows atrophic odontoid fracture nonunion <img src='img/arrows/WO.png'/> after open reduction and internal fixation (ORIF). This usually is due not to infection but poor vascularity.*
![Sagittal STIR MR shows a classic example of a type 2 Hangman's fracture (Effendi classification) with bilateral pars fractures and disruption of the C2/C3 disc but intact facet joints. There is disruption of the anterior longitudinal ligament <img src='img/arrows/WS.png'/>, posterior longitudinal ligament <img src='img/arrows/WO.png'/>, and interspinous ligaments <img src='img/arrows/WC.png'/>, indicating both column disruption.](images/app.statdx.com_image_thumbnail_2be64b6f-55e2-4fc6-89b7-39d86606be5b_annotated_true_size_900_quality_90_0ea7aba2.jpg)
**Hangman's Fracture, C2**
*Sagittal STIR MR shows a classic example of a type 2 Hangman's fracture (Effendi classification) with bilateral pars fractures and disruption of the C2/C3 disc but intact facet joints. There is disruption of the anterior longitudinal ligament <img src='img/arrows/WS.png'/>, posterior longitudinal ligament <img src='img/arrows/WO.png'/>, and interspinous ligaments <img src='img/arrows/WC.png'/>, indicating both column disruption.*
![Sagittal T1WI MR shows a nonunited fracture of dens <img src='img/arrows/WS.png'/>, so-called os odontoideum. Posterior displacement of the ossicle has resulted in cord impingement.](images/app.statdx.com_image_thumbnail_9f268e08-0f26-47ca-b173-e06d7c7a731a_annotated_true_size_900_quality_90_683a5142.jpg)
**Os Odontoideum**
*Sagittal T1WI MR shows a nonunited fracture of dens <img src='img/arrows/WS.png'/>, so-called os odontoideum. Posterior displacement of the ossicle has resulted in cord impingement.*
![Sagittal T1WI MR shows atlantooccipital assimilation <img src='img/arrows/WS.png'/> and a small os odontoideum <img src='img/arrows/WO.png'/>. Vertebral anomalies are also seen in the subaxial region <img src='img/arrows/WC.png'/>.](78910ce4-8298-4435-bb4b-bf2a6cf8cc81)
**Craniovertebral Junction Variants**
*Sagittal T1WI MR shows atlantooccipital assimilation <img src='img/arrows/WS.png'/> and a small os odontoideum <img src='img/arrows/WO.png'/>. Vertebral anomalies are also seen in the subaxial region <img src='img/arrows/WC.png'/>.*
![Sagittal NECT shows ballooning of the posterior process of C2 <img src='img/arrows/WS.png'/>, typical of aneurysmal bone cyst. Tumor also involves vertebral body and has resulted in a pathologic fracture <img src='img/arrows/WO.png'/>.](55802fce-b7cd-42c7-a801-cf03a18d74dd)
**Aneurysmal Bone Cyst**
*Sagittal NECT shows ballooning of the posterior process of C2 <img src='img/arrows/WS.png'/>, typical of aneurysmal bone cyst. Tumor also involves vertebral body and has resulted in a pathologic fracture <img src='img/arrows/WO.png'/>.*
![Sagittal T2WI MR shows the typical peg-shaped appearance of cerebellar tonsils <img src='img/arrows/WC.png'/>, which descend to the level of the C1 arch. The 4th ventricle is normal. There is a small syrinx <img src='img/arrows/WS.png'/>.](541b420a-0e67-4cea-801b-946269b91010)
**Chiari 1 Malformation**
*Sagittal T2WI MR shows the typical peg-shaped appearance of cerebellar tonsils <img src='img/arrows/WC.png'/>, which descend to the level of the C1 arch. The 4th ventricle is normal. There is a small syrinx <img src='img/arrows/WS.png'/>.*
![Sagittal T2WI MR shows characteristic Chiari 2 features of a small posterior fossa and 4th ventricle, medullary kink <img src='img/arrows/WS.png'/>, and verminal ectopia through the foramen magnum <img src='img/arrows/WO.png'/>.](c488c6ad-49bd-4936-a0ee-a4fd7c9cee0f)
**Chiari 2 Malformation**
*Sagittal T2WI MR shows characteristic Chiari 2 features of a small posterior fossa and 4th ventricle, medullary kink <img src='img/arrows/WS.png'/>, and verminal ectopia through the foramen magnum <img src='img/arrows/WO.png'/>.*
![Sagittal bone CT shows calcifications and soft tissue fullness <img src='img/arrows/WS.png'/> at the craniocervical junction due to calcium pyrophosphate deposition disease (CPPD). CPPD of the craniocervical junction is not uncommon in older adult patients and may cause instability.](686f2a65-93d1-4a82-8745-0c1a2d332510)
**Calcium Pyrophosphate Deposition Disease**
*Sagittal bone CT shows calcifications and soft tissue fullness <img src='img/arrows/WS.png'/> at the craniocervical junction due to calcium pyrophosphate deposition disease (CPPD). CPPD of the craniocervical junction is not uncommon in older adult patients and may cause instability.*
![Sagittal STIR MR shows extensive erosion of the odontoid process and a large soft tissue mass <img src='img/arrows/WS.png'/> from rheumatoid arthritis (RA). RA may mimic infection or tumor.](f7ce5b5f-1a62-4e3c-b41b-1efb3bfe2e1a)
**Pannus From Rheumatoid Arthritis**
*Sagittal STIR MR shows extensive erosion of the odontoid process and a large soft tissue mass <img src='img/arrows/WS.png'/> from rheumatoid arthritis (RA). RA may mimic infection or tumor.*
![Sagittal T2WI MR shows epidural abscess <img src='img/arrows/WS.png'/> compressing the spinal cord.](7f2a3ab8-c8a6-48a7-abf1-359de4496c99)
**Abscess, Epidural, Paravertebral**
*Sagittal T2WI MR shows epidural abscess <img src='img/arrows/WS.png'/> compressing the spinal cord.*
![Sagittal bone CT shows a calcified mass <img src='img/arrows/WS.png'/> with a dural tail arising from the ventral dura at C2, providing a clue to the dural origin. There is mass effect on adjacent spinal cord.](f7883c73-7587-4ae4-a00f-067c7de8538e)
**Meningioma**
*Sagittal bone CT shows a calcified mass <img src='img/arrows/WS.png'/> with a dural tail arising from the ventral dura at C2, providing a clue to the dural origin. There is mass effect on adjacent spinal cord.*
![Sagittal T2WI FS MR shows multiple small foci <img src='img/arrows/WS.png'/> of abnormal signal intensity in the bone marrow of C-spine, clivus, and occiput. Note posterior element involvement, which is a common MR finding with myeloma.](a0faccb4-749a-4d4f-840f-5bcc2112f4b4)
**Multiple Myeloma**
*Sagittal T2WI FS MR shows multiple small foci <img src='img/arrows/WS.png'/> of abnormal signal intensity in the bone marrow of C-spine, clivus, and occiput. Note posterior element involvement, which is a common MR finding with myeloma.*
docs_md/articles/craniovertebral-junction-soft-tissue-abnormality_3a8dca6c-3452-4cbb-ab83-5e9610d3f0f0.md
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title: "Craniovertebral Junction Soft Tissue Abnormality"
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---
# ESSENTIAL INFORMATION
- ## Key Differential Diagnosis Issues
- Do intralesion calcifications represent arc-whorl intralesional calcifications (chondrosarcoma) or fragmented destroyed bone (chordoma, metastasis)?
- Does patient have known primary neoplasm (metastasis), myeloma (plasmacytoma), or nasopharyngeal mass (nasopharyngeal carcinoma)?
- ## Helpful Clues for Common Diagnoses
- **Rheumatoid Arthritis**
- Thickened and inflamed synovium called pannus
- Never involves spine without involvement of hands &/or feet
- Odontoid erosions, ligamentous laxity
- C1-C2 instability in 33% of all rheumatoid arthritis (RA) patients
- Neutral, flexion, and extension lateral radiographs performed for evaluation
- High correlation to neurologic symptoms with distance 9 mm or more between C1-C2
- **Retroodontoid Pseudotumor**
- Increased soft tissue dorsal to odontoid secondary to C1-C2 osteoarthritis
- Low-signal mass on T1 and T2 (fibrotic)
- May cause cervicomedullary junction compression
- Usually seen with altered biomechanics of lower cervical spine → surgical/congenital fusion
- Mimics appearance of RA
- Multiple other levels of degenerative disc disease
- **Calcium Pyrophosphate Dihydrate Deposition Disease**
- Pseudogout: Acute, painful episode due to calcium pyrophosphate dihydrate deposition disease (CPPD)
- Crowned dens syndrome: CPPD of atlantooccipital joint causing periodic acute cervicooccipital pain with fever, neck stiffness, and laboratory inflammatory signs
- Calcifications commonly in transverse and alar ligaments, posterior longitudinal ligament, ligamentum flavum
- **Osteomyelitis, C1-C2**
- Infection starts as septic arthritis of C1-C2
- Risk factors include diabetes, drug abuse, endocarditis, immunocompromise
- Soft tissue mass and bone destruction at C1-C2 level
- *Staphylococcus aureus* most common organism in USA
- *Mycobacterium tuberculosis* most common worldwide
- MR shows low T1 signal mass centered at C1-C2 with variable involvement of odontoid and lateral masses at C2
- May show enlarged atlantodental interval
- Epidural mass with thecal sac/cord compression
- Grisel syndrome: Inflammatory, nontraumatic subluxation of C1-C2 following peripharyngeal infection
- **Extramedullary Tumor**
- **Metastases**
- Multiple lesions, bone destruction, systemic primary
- **Lymphoma**
- Large pharyngeal mucosal space mass with associated cervical adenopathy > 50% of time
- Non-Hodgkin lymphoma (NHL) 5x as common as Hodgkin disease in head & neck
- **Nasopharyngeal carcinoma**
- Mass centered in lateral pharyngeal recess of nasopharyngeal with deep extension and cervical adenopathy
- Nodal metastases present in 90% of cases at presentation
- Multiplanar images show invasion of clivus, sphenoid bone and sinus, C1 and C2 bodies
- **Neurofibromatosis type 1**
- Plexiform neurofibroma → diffuse enlargement of major nerve trunks/branches → bulky rope-like ("bag of worms") nerve expansion with adjacent tissue distortion
- Look for kyphoscoliosis ± multiple nerve root tumors, plexiform neurofibroma, dural ectasia/lateral meningocele
- **Schwannoma**
- Hypoglossal or upper cervical roots as site of origin
- Hypoglossal neuropathy results in tongue denervation
- Dumbbell with uniform enhancement
- Larger lesions may show central cystic formation
- **Paraganglioma**
- Multiple black dots ("pepper") in tumor substance indicating high-velocity flow voids from feeding arterial branches
- Jugular foramen (JF) or vagal varieties may present with upper cervical/skull base level mass
- **Chordoma**
- Mass is hyperintense to discs on T2WI with multiple septa
- Destructive, lytic lesion
- May extend into disc, involve 2 or more adjacent vertebrae
- **Chondrosarcoma**
- Lytic mass ± chondroid matrix, cortical disruption, and extension into soft tissues
- Chondroid matrix mineralization of rings and arcs (characteristic)
- **Meningioma**
- Foramen magnum, JF, upper cervical dura locations
- Carotid space → connection to JF above with JF margins showing permeative-sclerotic or hyperostotic changes on bone CT
- Absence of high-velocity flow voids on T1 MR
- T1 C+ MR shows enhancing JF mass
- ## Helpful Clues for Less Common Diagnoses
- **Aneurysm/Vertebral Dissection**
- Multiple etiologies → dissection, posttraumatic, atherosclerotic, iatrogenic, congenital
- **Synovial Cyst**
- Round, central T2-hyperintense mass with low-signal margin
- Associated with dorsal C1-C2 articulation or degenerated facets
- ## Helpful Clues for Rare Diagnoses
- **Neurenteric Cyst**
- Intraspinal cyst + vertebral abnormalities (persistent canal of Kovalevsky, segmentation and fusion anomalies)
## References
# Selected References
1. [Wang HB et al: Cervical myelopathy due to idiopathic retro-odontoid pseudotumor. World Neurosurg.160:e256-60, 2022](http://www.ncbi.nlm.nih.gov/pubmed/?term=34999265%5Bpmid%5D)
1. [Bi WL et al: Skull base tumors: neuropathology and clinical implications. Neurosurgery. 90(3):243-61, 2021](http://www.ncbi.nlm.nih.gov/pubmed/?term=34164689%5Bpmid%5D)
1. [Parperis K et al: Management of calcium pyrophosphate crystal deposition disease: a systematic review. Semin Arthritis Rheum. 51(1):84-94, 2021](http://www.ncbi.nlm.nih.gov/pubmed/?term=33360232%5Bpmid%5D)
1. [Pascart T et al: Treatment of nongout joint deposition diseases: an update. Arthritis. 2014:375202, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=24895535%5Bpmid%5D)
1. [Chang EY et al: Frequency of atlantoaxial calcium pyrophosphate dihydrate deposition at CT. Radiology. 269(2):519-24, 2013](http://www.ncbi.nlm.nih.gov/pubmed/?term=23737539%5Bpmid%5D)
1. [Tojo S et al: Factors influencing on retro-odontoid soft-tissue thickness: analysis by magnetic resonance imaging. Spine (Phila Pa 1976). 38(5):401-6, 2013](http://www.ncbi.nlm.nih.gov/pubmed/?term=23448899%5Bpmid%5D)
1. [Di Maio S et al: Current comprehensive management of cranial base chordomas: 10-year meta-analysis of observational studies. J Neurosurg. 115(6):1094-5, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=21819197%5Bpmid%5D)
1. [Smith JS et al: Basilar invagination. Neurosurgery. 66(3 Suppl):39-47, 2010](http://www.ncbi.nlm.nih.gov/pubmed/?term=20173526%5Bpmid%5D)
## Images
### Selected Images
![Sagittal T1WI MR shows rheumatoid arthritis (RA) involving C1-C2 articulation with dens erosion and extensive pannus formation <img src='img/arrows/WS.png'/>. There is mild compression of medulla by the pannus and obscuration of fat planes.](images/app.statdx.com_image_thumbnail_1e8e97b6-3801-41f7-91f8-a8e00d56c18b_annotated_true_size_900_quality_90_d7c6a53b.jpg)
**Rheumatoid Arthritis**
*Sagittal T1WI MR shows rheumatoid arthritis (RA) involving C1-C2 articulation with dens erosion and extensive pannus formation <img src='img/arrows/WS.png'/>. There is mild compression of medulla by the pannus and obscuration of fat planes.*
![Sagittal T1WI MR shows rheumatoid arthritis (RA) involving C1-C2 articulation with dens erosion and extensive pannus formation <img src='img/arrows/WS.png'/>. There is mild compression of medulla by the pannus and obscuration of fat planes.](images/app.statdx.com_image_thumbnail_1e8e97b6-3801-41f7-91f8-a8e00d56c18b_size_174_quality_85_2e47c86c.jpg)
**Rheumatoid Arthritis**
*Sagittal T1WI MR shows rheumatoid arthritis (RA) involving C1-C2 articulation with dens erosion and extensive pannus formation <img src='img/arrows/WS.png'/>. There is mild compression of medulla by the pannus and obscuration of fat planes.*
![Sagittal T1WI MR shows C1-C2 degenerative pseudopannus <img src='img/arrows/WS.png'/> in a patient with diffuse idiopathic skeletal hyperostosis. The odontoid is not eroded, but the atlantodental interval is increased. Cord compression occurs between the degenerative pannus and posterior C1.](images/app.statdx.com_image_thumbnail_91e10d96-25be-4075-a417-75c2ce381d48_annotated_true_size_900_quality_90_b084d779.jpg)
**Retroodontoid Pseudotumor**
*Sagittal T1WI MR shows C1-C2 degenerative pseudopannus <img src='img/arrows/WS.png'/> in a patient with diffuse idiopathic skeletal hyperostosis. The odontoid is not eroded, but the atlantodental interval is increased. Cord compression occurs between the degenerative pannus and posterior C1.*
![CTA study shows multiple punctate foci of calcification within a large soft tissue mass posterior to the odontoid process <img src='img/arrows/WS.png'/> that causes cord compression <img src='img/arrows/BO.png'/>. No gross erosive changes are present within the odontoid process, as would be present in RA.](images/app.statdx.com_image_thumbnail_b9c089b6-5c25-48d2-a435-b1e13a873488_annotated_true_size_900_quality_90_95c69b21.jpg)
**Calcium Pyrophosphate Dihydrate Deposition Disease**
*CTA study shows multiple punctate foci of calcification within a large soft tissue mass posterior to the odontoid process <img src='img/arrows/WS.png'/> that causes cord compression <img src='img/arrows/BO.png'/>. No gross erosive changes are present within the odontoid process, as would be present in RA.*
![Sagittal T1WI MR shows a large mass with low T2 signal <img src='img/arrows/WS.png'/> posterior to the odontoid process with severe cord compression <img src='img/arrows/WO.png'/>.](images/app.statdx.com_image_thumbnail_3eea911f-372e-4f76-a62c-d36e4e54e961_annotated_true_size_900_quality_90_f12897b0.jpg)
**Calcium Pyrophosphate Dihydrate Deposition Disease**
*Sagittal T1WI MR shows a large mass with low T2 signal <img src='img/arrows/WS.png'/> posterior to the odontoid process with severe cord compression <img src='img/arrows/WO.png'/>.*
![Sagittal T2WI MR shows a large prevertebral abscess spanning C1 to C4 <img src='img/arrows/WS.png'/> and extension posteriorly involving the interspinous region <img src='img/arrows/WO.png'/>. These findings are typical for tuberculosis (TB).](images/app.statdx.com_image_thumbnail_d528e938-6fe7-4bce-b3aa-9a1227e29f1e_annotated_true_size_900_quality_90_20290116.jpg)
**Osteomyelitis, C1-C2**
*Sagittal T2WI MR shows a large prevertebral abscess spanning C1 to C4 <img src='img/arrows/WS.png'/> and extension posteriorly involving the interspinous region <img src='img/arrows/WO.png'/>. These findings are typical for tuberculosis (TB).*
![Axial T1 C+ FS MR shows metastatic lung cancer with extensive extracapsular nodal spread. The mass has ill-defined borders with invasion of the longus capitis muscle <img src='img/arrows/WC.png'/> and invasion to the pharyngeal mucosal space <img src='img/arrows/WO.png'/>.](images/app.statdx.com_image_thumbnail_1ead54d2-e7f9-466a-aab9-a6329250bcf1_annotated_true_size_900_quality_90_4865f802.jpg)
**Metastases**
*Axial T1 C+ FS MR shows metastatic lung cancer with extensive extracapsular nodal spread. The mass has ill-defined borders with invasion of the longus capitis muscle <img src='img/arrows/WC.png'/> and invasion to the pharyngeal mucosal space <img src='img/arrows/WO.png'/>.*
![Axial CTA shows the classic appearance of thyroid metastasis with a thin, expansile, bony margin with the predominately lytic lesion within the left facet/lamina of C3 <img src='img/arrows/BC.png'/>.](images/app.statdx.com_image_thumbnail_483f8d25-4da6-4f41-9f47-66464fb427eb_annotated_true_size_900_quality_90_9711bb56.jpg)
**Metastases**
*Axial CTA shows the classic appearance of thyroid metastasis with a thin, expansile, bony margin with the predominately lytic lesion within the left facet/lamina of C3 <img src='img/arrows/BC.png'/>.*
![Axial CECT shows a homogeneous mass in the retropharyngeal space, displacing the parapharyngeal fat anterolaterally <img src='img/arrows/WO.png'/> and encircling the right internal carotid artery <img src='img/arrows/BC.png'/>.](images/app.statdx.com_image_thumbnail_2fcb0f45-ed93-4509-992c-9133ebf7fe36_annotated_true_size_900_quality_90_e91969a8.jpg)
**Lymphoma**
*Axial CECT shows a homogeneous mass in the retropharyngeal space, displacing the parapharyngeal fat anterolaterally <img src='img/arrows/WO.png'/> and encircling the right internal carotid artery <img src='img/arrows/BC.png'/>.*
![Sagittal T1 C+ MR shows a variant MR case of an unusually large skull base plasmacytoma <img src='img/arrows/WS.png'/> engulfing the clivus and extending into the nasopharynx and abutting C1-C2.](images/app.statdx.com_image_thumbnail_f4902e38-fa3b-4f95-a156-f625a100293a_annotated_true_size_900_quality_90_cb7d91a8.jpg)
**Plasmacytoma**
*Sagittal T1 C+ MR shows a variant MR case of an unusually large skull base plasmacytoma <img src='img/arrows/WS.png'/> engulfing the clivus and extending into the nasopharynx and abutting C1-C2.*
![Sagittal T1WI MR shows a typical case of an aggressive nasopharyngeal squamous cell carcinoma with invasion of the skull base by direct extension <img src='img/arrows/WO.png'/>.](images/app.statdx.com_image_thumbnail_47ce9e93-7c2c-4ec4-b67a-86056601c0ff_annotated_true_size_900_quality_90_1c21a885.jpg)
**Nasopharyngeal Carcinoma**
*Sagittal T1WI MR shows a typical case of an aggressive nasopharyngeal squamous cell carcinoma with invasion of the skull base by direct extension <img src='img/arrows/WO.png'/>.*
![Axial T1WI C+ MR shows multiple large neurofibromas within dorsal soft tissues and paravertebral regions. Symmetrical, large intradural lesions compress the cervical cord <img src='img/arrows/WS.png'/> at the C2 level.](images/app.statdx.com_image_thumbnail_4a5d71e2-b456-4a94-b113-cae60016cfff_annotated_true_size_900_quality_90_b5c6789e.jpg)
**Neurofibromatosis Type 1**
*Axial T1WI C+ MR shows multiple large neurofibromas within dorsal soft tissues and paravertebral regions. Symmetrical, large intradural lesions compress the cervical cord <img src='img/arrows/WS.png'/> at the C2 level.*
![Sagittal T1 C+ MR demonstrates an isointense, expansile mass arising from the clivus <img src='img/arrows/WC.png'/>. Notice the posterior indentation, or &quot;thumbing,&quot; of the pons.](images/app.statdx.com_image_thumbnail_c37eb229-98bc-4eac-b791-b39f55229281_annotated_true_size_900_quality_90_d6b0bcc5.jpg)
**Chordoma**
*Sagittal T1 C+ MR demonstrates an isointense, expansile mass arising from the clivus <img src='img/arrows/WC.png'/>. Notice the posterior indentation, or &quot;thumbing,&quot; of the pons.*
![Sagittal T1WI C+ MR shows a large, heterogeneous-signal mass in the cervical epidural space involving the dorsal aspect of C2-C3 junction and extending laterally with diffuse enhancement.](f40b5322-7e53-48c4-87a0-00d5b0268a3d)
**Chordoma**
*Sagittal T1WI C+ MR shows a large, heterogeneous-signal mass in the cervical epidural space involving the dorsal aspect of C2-C3 junction and extending laterally with diffuse enhancement.*
![Coronal T1WI MR shows a typical MR case of petrooccipital fissure skull base chondrosarcoma <img src='img/arrows/WS.png'/>.](22724c28-0ea6-4685-88db-3dfd3b1fbd33)
**Chondrosarcoma**
*Coronal T1WI MR shows a typical MR case of petrooccipital fissure skull base chondrosarcoma <img src='img/arrows/WS.png'/>.*
![Sagittal T1 C+ MR shows a well-defined, homogeneously enhancing mass with a broad dural margin <img src='img/arrows/WO.png'/> at the foramen magnum, typical for meningioma. There is compression of the medulla.](588eeaa8-b7ce-44c4-9a0d-b0a9dab2e441)
**Meningioma**
*Sagittal T1 C+ MR shows a well-defined, homogeneously enhancing mass with a broad dural margin <img src='img/arrows/WO.png'/> at the foramen magnum, typical for meningioma. There is compression of the medulla.*
![Axial CTA shows the CT features of a pseudoaneurysm of the internal carotid artery <img src='img/arrows/WS.png'/> located below the skull base.](6ab883dd-2ee4-4138-b25f-f62b04c16051)
**Aneurysm/Vertebral Dissection**
*Axial CTA shows the CT features of a pseudoaneurysm of the internal carotid artery <img src='img/arrows/WS.png'/> located below the skull base.*
### Additional Images
![Axial T1 C+ FS MR shows the appearance of multiple neurofibromas in a child with neurofibromatosis type 1 with bilateral, symmetric masses involving neural foramen <img src='img/arrows/WC.png'/> in the carotid sheath and posterior cervical space, consistent with neurofibromas.](images/app.statdx.com_image_thumbnail_5013cba3-8f33-4d46-9b6a-d8e337a76a64_annotated_true_size_900_quality_90_f5cc8568.jpg)
**Neurofibromatosis Type 1**
*Axial T1 C+ FS MR shows the appearance of multiple neurofibromas in a child with neurofibromatosis type 1 with bilateral, symmetric masses involving neural foramen <img src='img/arrows/WC.png'/> in the carotid sheath and posterior cervical space, consistent with neurofibromas.*
![Axial T1 C+ FS MR shows the typical imaging appearance of a glomus tumor (vagale paraganglioma): A soft tissue mass <img src='img/arrows/WS.png'/> below the skull base with small, focal signal voids indicating the presence of high-flow vessels.](images/app.statdx.com_image_thumbnail_bcc66408-ee3e-474a-8c3c-b3fa66384044_annotated_true_size_900_quality_90_57d10efd.jpg)
**Paraganglioma**
*Axial T1 C+ FS MR shows the typical imaging appearance of a glomus tumor (vagale paraganglioma): A soft tissue mass <img src='img/arrows/WS.png'/> below the skull base with small, focal signal voids indicating the presence of high-flow vessels.*
![Axial CECT shows a variant case of carotid space meningioma from recurrent intracranial disease with an oval-shaped mass <img src='img/arrows/WS.png'/> in the right carotid space, medial to the styloid process <img src='img/arrows/WO.png'/>.](72487488-035a-4cd7-9782-1f265ab3d6b6)
**Meningioma**
*Axial CECT shows a variant case of carotid space meningioma from recurrent intracranial disease with an oval-shaped mass <img src='img/arrows/WS.png'/> in the right carotid space, medial to the styloid process <img src='img/arrows/WO.png'/>.*
![Sagittal T2WI MR shows a Chiari 1 malformation with inferiorly positioned, peg-shaped tonsils <img src='img/arrows/WS.png'/> with small, associated cervical syrinx <img src='img/arrows/WO.png'/>.](d9102dee-362d-4b33-a768-686fbdbd31b1)
**Chiari 1 Malformation**
*Sagittal T2WI MR shows a Chiari 1 malformation with inferiorly positioned, peg-shaped tonsils <img src='img/arrows/WS.png'/> with small, associated cervical syrinx <img src='img/arrows/WO.png'/>.*
![Sagittal T2WI MR shows a typical case of mild Chiari 2 malformation with a small posterior fossa and vermian ectopia to the C2/C3 level <img src='img/arrows/WS.png'/>. The 4th ventricle is small and elongated.](da716203-9bc6-429f-99ee-b0edd39d89c6)
**Chiari 2 Malformation**
*Sagittal T2WI MR shows a typical case of mild Chiari 2 malformation with a small posterior fossa and vermian ectopia to the C2/C3 level <img src='img/arrows/WS.png'/>. The 4th ventricle is small and elongated.*
![Sagittal T2WI MR shows a variant Chiari 2 with syringobulbia. There is a characteristic small posterior fossa and vermian ectopia <img src='img/arrows/WC.png'/>. There is focal cervicomedullary syrinx <img src='img/arrows/WS.png'/>.](1d668b13-67b6-4e00-90c1-943b0be43245)
**Chiari 2 Malformation**
*Sagittal T2WI MR shows a variant Chiari 2 with syringobulbia. There is a characteristic small posterior fossa and vermian ectopia <img src='img/arrows/WC.png'/>. There is focal cervicomedullary syrinx <img src='img/arrows/WS.png'/>.*
![Sagittal T2WI MR demonstrates an extensive T2-hyperintense mass expanding the medulla and cervical cord from astrocytoma <img src='img/arrows/WS.png'/>. The tumor causes a septated-appearing neoplastic syrinx in the more caudal cord <img src='img/arrows/WO.png'/>.](92a6b8af-7f28-4e01-b27f-8e4fe228d33e)
**Glioma, Brainstem**
*Sagittal T2WI MR demonstrates an extensive T2-hyperintense mass expanding the medulla and cervical cord from astrocytoma <img src='img/arrows/WS.png'/>. The tumor causes a septated-appearing neoplastic syrinx in the more caudal cord <img src='img/arrows/WO.png'/>.*
![Sagittal T1WI MR shows well-defined, complex cyst at the cervicomedullary junction due to hemangioblastoma.](ea638d8d-2c6e-4c46-a6e8-f9bbd914f31d)
**Hemangioblastoma, Spinal Cord**
*Sagittal T1WI MR shows well-defined, complex cyst at the cervicomedullary junction due to hemangioblastoma.*
![Axial T1 C+ MR shows a well-defined, homogeneously enhancing mass with a broad dural margin at the foramen magnum, typical for meningioma. The left distal vertebral artery is adjacent to the tumor <img src='img/arrows/WS.png'/>.](6e3bd0b6-5522-43a4-be28-3d1c4af74dd5)
**Meningioma**
*Axial T1 C+ MR shows a well-defined, homogeneously enhancing mass with a broad dural margin at the foramen magnum, typical for meningioma. The left distal vertebral artery is adjacent to the tumor <img src='img/arrows/WS.png'/>.*
docs_md/articles/creutzfeldt-jakob-disease-cjd_e1b27954-6591-4bb0-a659-b13790492620.md
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title: "Creutzfeldt-Jakob Disease (CJD)"
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pageDescription: "Creutzfeldt-Jakob Disease (CJD)"
pageKeywords: "Brain, Diagnosis, Pathology-Based Diagnoses, Acquired Toxic/Metabolic/Degenerative Disorders, Dementias and Degenerative Disorders, Creutzfeldt-Jakob Disease (CJD)"
pageTitle: "Creutzfeldt-Jakob Disease (CJD) | STATdx"
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- "Creutzfeldt-Jakob Disease (CJD)"
---
# KEY FACTS
- ## Terminology
- Creutzfeldt-Jakob disease (CJD): Rapidly progressing, fatal, potentially transmissible dementia caused by prion
- ## Imaging
- Best imaging clue: Progressive DWI/FLAIR hyperintensity of basal ganglia (BG), thalamus, and cerebral cortex
- Predominantly gray matter (GM): Caudate and putamen > globus pallidus (GP)
- Thalamus: Common in variant CJD (vCJD)
- Cerebral cortex: Frontal, parietal, and temporal
- Heidenhain variant: Occipital lobe
- 2 signs seen in 90% of vCJD but can also occur in sporadic CJD (sCJD)
- Pulvinar sign: Symmetric T2 hyperintensity of pulvinar of thalamus
- Hockey stick sign: Symmetric pulvinar and dorsomedial thalamic nuclear hyperintensity
- Best imaging tool: MR with DWI
- ## Top Differential Diagnoses
- Hypoxic-ischemic injury
- Osmotic demyelination syndrome
- Other causes of dementia
- Alzheimer, frontotemporal, and multiinfarct dementia; dementia in motor neuron disease
- Leigh syndrome
- Corticobasal degeneration
- ## Clinical Issues
- Definite CJD diagnosed by neuropathology
- Progressive dementia associated with myoclonic jerks and akinetic mutism; variable constellation of pyramidal, extrapyramidal, and cerebellar signs
- CSF protein biomarkers: 14-3-3 protein, total tau (t-tau), S100 and neuron-specific enolase (NSE)
- DWI MR has higher diagnostic accuracy, 97% more than any or all of these CSF biomarkers
- Incidence 1 per 1,000,000 (USA and internationally)
- sCJD (85%), familial (15%), infectious/iatrogenic (< 1%) (includes vCJD)
- Death usually ensues within months of onset
# TERMINOLOGY
- ## Abbreviations
- Creutzfeldt-Jakob disease (CJD)
- Sporadic Creutzfeldt-Jakob disease (sCJD)
- Variant Creutzfeldt-Jakob disease (vCJD)
- ## Definitions
- Rapidly progressing, fatal, neurodegenerative disorder caused by prion (proteinaceous infectious particle devoid of DNA and RNA)
- Transmissible spongiform encephalopathy
# IMAGING
- ## General Features
- ### Best diagnostic clue
- Progressive T2 hyperintensity of basal ganglia (BG), thalamus, and cerebral cortex
- ### Location
- Predominantly gray matter (GM)
- BG: Caudate and putamen > globus pallidus (GP)
- Thalamus (common in vCJD)
- Cerebral cortex (most commonly frontal, parietal, and temporal lobes)
- Cortical involvement often asymmetric
- Heidenhain variant: Occipital lobe
- Brownell-Oppenheimer: Cerebellum
- May involve only peripheral cortex
- Cortical involvement often asymmetric
- Primary sensorimotor cortex relatively spared
- White matter (WM) usually not involved
- Size: Slight decrease (atrophy)
- Morphology: Hyperintense T2 signal conforms to outline of BG and gyriform pattern in cortex
- ## CT Findings
- NECT: Usually normal
- May show rapidly progressive atrophy and ventricular dilatation on serial CT
- Serial CT illustrates atrophy progression
- ## MR Findings
- ### T1WI
- Normal
- GP hyperintensity reported in sCJD
- ### T2WI
- Hyperintense signal in BG, thalami, cortex
- Cerebral atrophy
- With time, hyperintense foci may develop in WM
- ### FLAIR
- 2 signs seen in 90% of vCJD but can also occur in sCJD
- **Pulvinar**sign: Bilateral symmetrical hyperintensity of **pulvinar** (posterior) nuclei of **thalamus**
- **Hockey stick** sign: Symmetrical **pulvinar and dorsomedial thalamic** nuclear hyperintensity
- Periaqueductal GM hyperintensity
- Cortical hyperintensity (common in sCJD)
- ### DWI
- Progressive hyperintensity in striatum and cortex
- Gyriform hyperintense areas in cerebral cortex (cortical ribbon sign*)*
- Correspond to localization of periodic sharp wave complexes on EEG
- DWI hyperintensity may disappear late in disease
- T1WI C+: No abnormal enhancement
- ## Nuclear Medicine Findings
- F-18 FDG PET: Regional glucose hypometabolism correlates with sites of neuropathologic lesions
- SPECT with N-isopropyl-p-(I-123) iodoamphetamine (DaTSCAN)
- ↓ uptake of tracer in BG reported
- Sometimes in asymmetrical pattern
- ## Imaging Recommendations
- Best imaging tool: MR with DWI and FLAIR
# DIFFERENTIAL DIAGNOSIS
- [Hypoxic-Ischemic Injury](/document/adult-hypoxic-ischemic-injury/dfb41c7a-2914-4a29-b05b-3a45c241f4a1)
- BG and parasagittal cortical areas involved
- Hyperintense BG lesions on T1WI and T2WI
- DWI + symmetric GM involvement
- [Osmotic Demyelination Syndrome](/document/osmotic-demyelination-syndrome/e061cc1f-61b4-4c4c-8b91-f53bf170180e)
- Extrapontine: T2-hyperintense putamen and caudate
- DWI positive acutely
- ## Leigh Syndrome
- Primarily seen in pediatric patients
- T2 hyperintensity in putamen and GP
- [Other Causes of Dementia](/document/alzheimer-disease/f71f5cf5-b1af-4c6d-b145-b4c10eec7b58)
- [Alzheimer disease](/document/alzheimer-disease/f71f5cf5-b1af-4c6d-b145-b4c10eec7b58)
- [Dementia in motor neuron disease](/document/amyotrophic-lateral-sclerosis-als/23de52b7-d9bd-441c-a18c-95c8afccb470)
- [Frontotemporal dementia](/document/frontotemporal-lobar-degeneration/49510d0e-acf7-45cb-9eb1-53f8193b0b6d)
- [Multiinfarct dementia](/document/vascular-dementia/f59dab57-c511-4369-8fcc-592421a4b8d1)
- [Corticobasal Degeneration](/document/corticobasal-degeneration/23f97d4e-8724-4229-b9f8-08f63906ebd8)
- Neuronal loss in substantia nigra, frontoparietal cortex, and striatum (BG atrophy may be subtle)
- MR: Symmetric/asymmetric atrophy of pre- and postcentral gyri; prominent parasagittal involvement
- Subcortical gliosis: High intensity on T2WI
- [Wilson Disease](/document/wilson-disease/3d4d4876-4ce4-4af0-9e75-1a419bdd813c)
- WM and deep GM lesions (BG, dentate nucleus, brainstem); variably T2 hyperintense
- T1-hypointense (rarely hyperintense) lesions
- [Arteriolosclerosis](/document/arteriolosclerosis/ce5a75ed-3a88-42a4-a0e8-4f339375c062)
- BG involvement: Typically asymmetric and multifocal (rather than diffuse as in CJD)
- Focal hyperintensities in deep WM
- DWI negative, unless acute
# PATHOLOGY
- ## General Features
- ### Etiology
- Prion protein is misfolded isoform (PrPSc) of normal host-encoded protein (PrPc)
- PrPSc = conformationally isomer of PrPc
- PrPSc introduced into healthy cells → initiates self-perpetuating vicious cycle: PrPc → PrPSc → neurotoxicity
- sCJD: Spontaneous PrPc → PrPSc or somatic mutation
- Familial CJD (fCJD): Mutations in *PRNP*gene
- Iatrogenic CJD: Infection from prion-containing material
- Surgical instruments, dura mater grafts, stereotactic electrodes
- Cadaveric corneal transplants, human pituitary hormones (growth hormone and gonadotropins)
- vCJD: Bovine spongiform encephalopathy in cattle is transmitted to humans through infected beef
- Primarily present in UK
- a.k.a. new variant CJD (nvCJD)
- Risk for health care workers
- Physical contact with patients is no risk for transmission
- Special precautions in handling brain tissue
- All used materials and instruments decontaminated as per established protocols
- ### Genetics
- Can be inherited, sporadic, or acquired (infectious)
- 10-15% of human prion disease cases associated with dominant mutations in autosomal prion protein (PrPc) gene (*PRNP*) on chromosome 20
- PrPc is normal host protein on surface of many cells, particularly neurons
- ### Associated abnormalities
- EEG: Periodic (high-voltage) sharp wave complexes (PSWCs) on background of low-voltage activity
- 67-95% patients with sCJD show PSWCs at some point during course of illness
- False-positive EEG findings in Alzheimer dementia and vascular dementia patients
- PSWCs helpful in differentiating sCJD from other prion disease
- ## Staging, Grading, & Classification
- Sporadic CJD
- Definite
- Characteristic neuropathology (biopsy or autopsy)
- Protease-resistant PrPSc (PrPres) by Western blot
- Probable
- Neuropsychiatric disorder with positive RT-QuIC in CSF or other tissues
- OR
- Rapidly progressive dementia and at least 2 out of 4 clinical features listed in table 1
- AND positive result on at least 1 of 3 lab tests listed in table 1
- AND without routine investigations indicating alternative diagnosis
- Possible
- Progressive dementia and at least 2 out of 4 clinical features listed in table 1
- AND absence of positive lab tests that would classify case as "probable"
- AND duration of illness < 2 years
- AND without routine investigations indicating alternative diagnosis
- Iatrogenic CJD: Progressive cerebellar syndrome in recipient of human cadaveric-derived pituitary hormone; or sporadic CJD with recognized exposure risk, e.g., antecedent neurosurgery with dura mater implantation
- Familial CJD: Definite or probable CJD **with** definite or probable CJD in 1st-degree relative; &/or neuropsychiatric disorder **with** disease-specific PrP gene mutation
- ## Gross Pathologic & Surgical Features
- Mild cortical atrophy
- Diffuse or confined to affected structures
- Ventricular enlargement
- ## Microscopic Features
- Spongiform encephalopathy: GM most affected
- Marked neuronal loss with reactive astrocytosis
- Replacement gliosis
- Neuronal vacuolation with spongiform changes
- Spongiform panencephalopathy (very rare)
- Primary extensive involvement of WM
- Loss of myelin and axons associated with generalized spongiform change in WM
- ± diffuse cerebral atrophy, loss of neurons, and proliferation of astrocytes in cerebral cortex
- 10% of patients with CJD have amyloid plaques in cerebellum or cerebral hemispheres
- Apple-green birefringence using Congo red staining when viewed under polarized light
- Variable accumulation of PrPSc in brain tissue
- PrPSc = abnormal, insoluble, protease-resistant amyloid form of PrPc
- Diffuse (common in sCJD) or discrete plaques
# CLINICAL ISSUES
- ## Presentation
- ### Most common signs/symptoms
- Rapidly progressive dementia associated with myoclonic jerks and akinetic mutism
- Variable constellation of pyramidal, extrapyramidal, and cerebellar signs
- ### Clinical profile
- **sCJD**: Cerebellar dysfunction, rapidly progressive cognitive impairment, both
- 6 molecular subtypes: MM1, MM2 (thalamic and cortical), MV1, MV2, VV1, and VV2
- Vary with respect to age at onset, disease duration, early symptoms, and neuropathology
- **vCJD**: Psychiatric and sensory symptoms
- **Heidenhain variant** of CJD
- Isolated visual signs/symptoms (initially)
- Predominantly occipital lobe degeneration
- Normal conventional T1 and T2WI of brain
- DWI/FLAIR may detect early cortical abnormalities
- **Brownell-Oppenheimer**: Cerebellar signs/symptoms
- Extrapyramidal type of CJD
- May show ↑ signal intensity in BG
- Pyramidal involvement with disease progression
- BG dysfunction
- Spinal cord involvement → muscle atrophy and fasciculations
- **CSF studies**
- CSF protein biomarkers: 14-3-3 protein, total tau (t-tau), S100, neuron-specific enolase (NSE), and thymosin β4
- 14-3-3 protein detection is adjunctive rather than diagnostic for prior disease
- t-tau > 1,150 picogram/mL has superior accuracy and specificity than 14-3-3 protein for CJD
- Significant false-positives and negatives with 14-3-3 and t-tau protein test results
- DWI MR has higher diagnostic accuracy than any or all CSF biomarkers
- Real-time quaking-induced conversion (RT-QUIC) testing of CSF to detected PrPsc
- More sensitive using olfactory epithelium (nasal brushing) than CSF
- Nasal brushing not performed in USA
- ## Demographics
- ### Age
- Younger in vCJD, older in sCJD (6th-7th decades)
- ### Sex
- No sex preponderance
- ### Ethnicity
- sCJD occurs throughout world, in all races
- In USA, CJD ↓ in Blacks, American Indians, and Alaskan natives than White population
- vCJD limited to Europe (nearly all cases in UK)
- ### Epidemiology
- Incidence 1.0-1.5 per million in USA
- sCJD (85-95%), familial (5-15%), infectious/iatrogenic (< 1%)
- ## Natural History & Prognosis
- Long incubation period but rapidly progressive once clinical symptoms begin
- Rapidly progressing dementia with death usually ensuing within months of onset
- Median survival from time of onset of symptoms to death is 4.5 months
- 90% live < 1 year
- ## Treatment
- No effective treatment
# DIAGNOSTIC CHECKLIST
- ## Consider
- Heidenhain variant of CJD in patients with visual disorders of unclear origin and dementia
- ## Image Interpretation Pearls
- Lack of BG findings does not rule out CJD
94600a52-3136-46e8-a827-05e281f28d7e
## References
# Selected References
1. [Alaoui A et al: [MRI role in Creutzfeldt-Jakob disease: about a case.] Pan Afr Med J. 32:95, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31223386%5Bpmid%5D)
1. [Baldwin KJ et al: Prion disease. Semin Neurol. 39(4):428-39, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31533183%5Bpmid%5D)
1. [Groveman BR et al: Sporadic Creutzfeldt-Jakob disease prion infection of human cerebral organoids. Acta Neuropathol Commun. 7(1):12, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31196223%5Bpmid%5D)
1. [Hayashi Y et al: Clinicopathological findings of an MM2-cortical-type sporadic Creutzfeldt-Jakob disease patient with cortical blindness during a course of glaucoma and age-related macular degeneration. Prion. 13(1):124-31, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31219399%5Bpmid%5D)
1. [Llorens F et al: Plasma total prion protein as a potential biomarker for neurodegenerative dementia: diagnostic accuracy in the spectrum of prion diseases. Neuropathol Appl Neurobiol. ePub, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31216593%5Bpmid%5D)
1. [Muniz BC et al: The Heidenhain variant of Creutzfeldt-Jakob disease. Radiol Bras. 52(3):199-200, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31210697%5Bpmid%5D)
1. [Xu Y et al: Sporadic Creutzfeldt-Jakob disease presenting as dizziness and cognitive decline: a case report. Medicine (Baltimore). 98(24):e16002, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31192942%5Bpmid%5D)
1. [Fragoso DC et al: Imaging of Creutzfeldt-Jakob disease: imaging patterns and their differential diagnosis. Radiographics. 37(1):234-57, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=28076012%5Bpmid%5D)
1. [Koeller KK et al: Viral and prion infections of the central nervous system: radiologic-pathologic correlation: from the radiologic pathology archives. Radiographics. 37(1):199-233, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=28076019%5Bpmid%5D)
1. [Caobelli F et al: The role of neuroimaging in evaluating patients affected by Creutzfeldt-Jakob disease: a systematic review of the literature. J Neuroimaging. 25(1):2-13, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=24593302%5Bpmid%5D)
1. [Kim MO et al: Clinical update of Jakob-Creutzfeldt disease. Curr Opin Neurol. 28(3):302-10, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25923128%5Bpmid%5D)
1. [Felix-Morais R et al: Creutzfeldt-Jakob disease: typical imaging findings. BMJ Case Rep. 2014, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=24682142%5Bpmid%5D)
1. [Risacher SL et al: Neuroimaging biomarkers of neurodegenerative diseases and dementia. Semin Neurol. 33(4):386-416, 2013](http://www.ncbi.nlm.nih.gov/pubmed/?term=24234359%5Bpmid%5D)
1. [Appleby BS et al: Characteristics of established and proposed sporadic Creutzfeldt-Jakob disease variants. Arch Neurol. 66(2):208-15, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=19204157%5Bpmid%5D)
1. [Iwasaki Y et al: Clinical diagnosis of Creutzfeldt-Jakob disease: accuracy based on analysis of autopsy-confirmed cases. J Neurol Sci. 277(1-2):119-23, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=19056094%5Bpmid%5D)
1. [Josephs KA et al: Rapidly progressive neurodegenerative dementias. Arch Neurol. 66(2):201-7, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=19204156%5Bpmid%5D)
1. [Manners DN et al: Pathologic correlates of diffusion MRI changes in Creutzfeldt-Jakob disease. Neurology. 72(16):1425-31, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=19380702%5Bpmid%5D)
1. [Meissner B et al: MRI lesion profiles in sporadic Creutzfeldt-Jakob disease. Neurology. 72(23):1994-2001, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=19506221%5Bpmid%5D)
1. [Clarençon F et al: MRI and FDG PET/CT findings in a case of probable Heidenhain variant Creutzfeldt-Jakob disease. J Neuroradiol. 35(4):240-3, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18466976%5Bpmid%5D)
1. [Fulbright RK et al: MR imaging of familial Creutzfeldt-Jakob disease: a blinded and controlled study. AJNR Am J Neuroradiol. 29(9):1638-43, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18635614%5Bpmid%5D)
1. [Heinemann U et al: Brain biopsy in patients with suspected Creutzfeldt-Jakob disease. J Neurosurg. 109(4):735-41, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18826363%5Bpmid%5D)
1. [Meissner B et al: Isolated cortical signal increase on MR imaging as a frequent lesion pattern in sporadic Creutzfeldt-Jakob disease. AJNR Am J Neuroradiol. 29(8):1519-24, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18599580%5Bpmid%5D)
1. [Ward HJ et al: Risk factors for sporadic Creutzfeldt-Jakob disease. Ann Neurol. 63(3):347-54, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18074392%5Bpmid%5D)
1. [Yi SH et al: Relationship between clinical course and diffusion-weighted MRI findings in sporadic Creutzfeldt-Jakob Disease. Neurol Sci. 29(4):251-5, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18810600%5Bpmid%5D)
1. [Kallenberg K et al: Creutzfeldt-Jakob disease: comparative analysis of MR imaging sequences. AJNR Am J Neuroradiol. 27(7):1459-62, 2006](http://www.ncbi.nlm.nih.gov/pubmed/?term=16908558%5Bpmid%5D)
1. [Lin YR et al: Creutzfeldt-jakob disease involvement of rolandic cortex: a quantitative apparent diffusion coefficient evaluation. AJNR Am J Neuroradiol. 27(8):1755-9, 2006](http://www.ncbi.nlm.nih.gov/pubmed/?term=16971630%5Bpmid%5D)
1. [Tschampa HJ et al: MRI in the diagnosis of sporadic Creutzfeldt-Jakob disease: a study on inter-observer agreement. Brain. 128(Pt 9):2026-33, 2005](http://www.ncbi.nlm.nih.gov/pubmed/?term=15958503%5Bpmid%5D)
1. [Young GS et al: Diffusion-weighted and fluid-attenuated inversion recovery imaging in Creutzfeldt-Jakob disease: high sensitivity and specificity for diagnosis. AJNR Am J Neuroradiol. 26(6):1551-62, 2005](http://www.ncbi.nlm.nih.gov/pubmed/?term=15956529%5Bpmid%5D)
1. [Collins SJ et al: Transmissible spongiform encephalopathies. Lancet. 363(9402):51-61, 2004](http://www.ncbi.nlm.nih.gov/pubmed/?term=14723996%5Bpmid%5D)
1. [Summers DM et al: The pulvinar sign in variant Creutzfeldt-Jakob disease. Arch Neurol. 61(3):446-7, 2004](http://www.ncbi.nlm.nih.gov/pubmed/?term=15023827%5Bpmid%5D)
## Tables
# CDC Diagnostic Criteria for Creutzfeldt-Jakob Disease 2018
| Clinical Features | Lab Tests |
| --- | --- |
| Myoclonus | Typical EEG (PSWCs) |
| Visual or cerebellar signs | 14-3-3 CSF protein positive |
| Pyramidal/extrapyramidal signs | DWI/FLAIR ↑ signal in caudate/putamen or at least 2 cortical regions (temporal, parietal, occipital) |
| Akinetic mutism | |
## Images
### Selected Images
![A 53-year-old man with rapidly progressing cognitive decline due to sporadic Creutzfeldt-Jakob disease (sCJD) is shown. Axial DWI MR demonstrates symmetric bilateral basal ganglia (BG) diffusion restriction <img src='img/arrows/CS.png'/> and asymmetric cortical restricted diffusion (cortical ribbon sign) in bilateral (right &gt; &gt; left) hemispheres <img src='img/arrows/CO.png'/>.](images/app.statdx.com_image_thumbnail_e4dd9275-5048-4513-8288-980aa8250267_size_168_quality_85_5cf734ec_20251014T204650Z.jpg)
*A 53-year-old man with rapidly progressing cognitive decline due to sporadic Creutzfeldt-Jakob disease (sCJD) is shown. Axial DWI MR demonstrates symmetric bilateral basal ganglia (BG) diffusion restriction <img src='img/arrows/CS.png'/> and asymmetric cortical restricted diffusion (cortical ribbon sign) in bilateral (right &gt; &gt; left) hemispheres <img src='img/arrows/CO.png'/>.*
![A 53-year-old man with rapidly progressing cognitive decline due to sporadic Creutzfeldt-Jakob disease (sCJD) is shown. Axial DWI MR demonstrates symmetric bilateral basal ganglia (BG) diffusion restriction <img src='img/arrows/CS.png'/> and asymmetric cortical restricted diffusion (cortical ribbon sign) in bilateral (right &gt; &gt; left) hemispheres <img src='img/arrows/CO.png'/>.](images/app.statdx.com_image_thumbnail_e4dd9275-5048-4513-8288-980aa8250267_size_168_quality_85_df7b6786_20251014T193441Z.jpg)
*A 53-year-old man with rapidly progressing cognitive decline due to sporadic Creutzfeldt-Jakob disease (sCJD) is shown. Axial DWI MR demonstrates symmetric bilateral basal ganglia (BG) diffusion restriction <img src='img/arrows/CS.png'/> and asymmetric cortical restricted diffusion (cortical ribbon sign) in bilateral (right &gt; &gt; left) hemispheres <img src='img/arrows/CO.png'/>.*
![A 53-year-old man with rapidly progressing cognitive decline due to sporadic Creutzfeldt-Jakob disease (sCJD) is shown. Axial DWI MR demonstrates symmetric bilateral basal ganglia (BG) diffusion restriction <img src='img/arrows/CS.png'/> and asymmetric cortical restricted diffusion (cortical ribbon sign) in bilateral (right &gt; &gt; left) hemispheres <img src='img/arrows/CO.png'/>.](images/app.statdx.com_image_thumbnail_e4dd9275-5048-4513-8288-980aa8250267_size_174_quality_85_1de39304_20251014T193347Z.jpg)
*A 53-year-old man with rapidly progressing cognitive decline due to sporadic Creutzfeldt-Jakob disease (sCJD) is shown. Axial DWI MR demonstrates symmetric bilateral basal ganglia (BG) diffusion restriction <img src='img/arrows/CS.png'/> and asymmetric cortical restricted diffusion (cortical ribbon sign) in bilateral (right &gt; &gt; left) hemispheres <img src='img/arrows/CO.png'/>.*
![A 53-year-old man with rapidly progressing cognitive decline due to sporadic Creutzfeldt-Jakob disease (sCJD) is shown. Axial DWI MR demonstrates symmetric bilateral basal ganglia (BG) diffusion restriction <img src='img/arrows/CS.png'/> and asymmetric cortical restricted diffusion (cortical ribbon sign) in bilateral (right &gt; &gt; left) hemispheres <img src='img/arrows/CO.png'/>.](images/app.statdx.com_image_thumbnail_e4dd9275-5048-4513-8288-980aa8250267_size_174_quality_85_5e6d48cb_20251014T204456Z.jpg)
*A 53-year-old man with rapidly progressing cognitive decline due to sporadic Creutzfeldt-Jakob disease (sCJD) is shown. Axial DWI MR demonstrates symmetric bilateral basal ganglia (BG) diffusion restriction <img src='img/arrows/CS.png'/> and asymmetric cortical restricted diffusion (cortical ribbon sign) in bilateral (right &gt; &gt; left) hemispheres <img src='img/arrows/CO.png'/>.*
![A 53-year-old man with rapidly progressing cognitive decline due to sporadic Creutzfeldt-Jakob disease (sCJD) is shown. Axial DWI MR demonstrates symmetric bilateral basal ganglia (BG) diffusion restriction <img src='img/arrows/CS.png'/> and asymmetric cortical restricted diffusion (cortical ribbon sign) in bilateral (right &gt; &gt; left) hemispheres <img src='img/arrows/CO.png'/>.](images/app.statdx.com_image_thumbnail_e4dd9275-5048-4513-8288-980aa8250267_size_174_quality_85_f31d11ff_20251014T185333Z.jpg)
*A 53-year-old man with rapidly progressing cognitive decline due to sporadic Creutzfeldt-Jakob disease (sCJD) is shown. Axial DWI MR demonstrates symmetric bilateral basal ganglia (BG) diffusion restriction <img src='img/arrows/CS.png'/> and asymmetric cortical restricted diffusion (cortical ribbon sign) in bilateral (right &gt; &gt; left) hemispheres <img src='img/arrows/CO.png'/>.*
![Axial FLAIR MR in the same patient demonstrates hyperintense signal in bilateral BG <img src='img/arrows/CS.png'/> and cortex <img src='img/arrows/CO.png'/>. CSF was positive for 14-3-3 protein indicating probable sCJD.](images/app.statdx.com_image_thumbnail_2a44ce46-87e8-4e48-9ace-510263f1d912_size_168_quality_85_6ce3f560_20251014T193441Z.jpg)
*Axial FLAIR MR in the same patient demonstrates hyperintense signal in bilateral BG <img src='img/arrows/CS.png'/> and cortex <img src='img/arrows/CO.png'/>. CSF was positive for 14-3-3 protein indicating probable sCJD.*
![Axial FLAIR MR in the same patient demonstrates hyperintense signal in bilateral BG <img src='img/arrows/CS.png'/> and cortex <img src='img/arrows/CO.png'/>. CSF was positive for 14-3-3 protein indicating probable sCJD.](images/app.statdx.com_image_thumbnail_2a44ce46-87e8-4e48-9ace-510263f1d912_size_168_quality_85_a530e880_20251014T204650Z.jpg)
*Axial FLAIR MR in the same patient demonstrates hyperintense signal in bilateral BG <img src='img/arrows/CS.png'/> and cortex <img src='img/arrows/CO.png'/>. CSF was positive for 14-3-3 protein indicating probable sCJD.*
![Axial DWI MR in a patient with variant CJD (vCJD) demonstrates diffusion restriction in bilateral posteromedial aspect of thalami <img src='img/arrows/CS.png'/> representing hockey stick sign.](images/app.statdx.com_image_thumbnail_7470503b-d147-43f8-85df-10bb7518afa1_size_168_quality_85_33100e2b_20251014T204650Z.jpg)
*Axial DWI MR in a patient with variant CJD (vCJD) demonstrates diffusion restriction in bilateral posteromedial aspect of thalami <img src='img/arrows/CS.png'/> representing hockey stick sign.*
![Axial DWI MR in a patient with variant CJD (vCJD) demonstrates diffusion restriction in bilateral posteromedial aspect of thalami <img src='img/arrows/CS.png'/> representing hockey stick sign.](images/app.statdx.com_image_thumbnail_7470503b-d147-43f8-85df-10bb7518afa1_size_168_quality_85_60d4e947_20251014T193441Z.jpg)
*Axial DWI MR in a patient with variant CJD (vCJD) demonstrates diffusion restriction in bilateral posteromedial aspect of thalami <img src='img/arrows/CS.png'/> representing hockey stick sign.*
![Axial DWI MR in a different patient with vCJD shows diffusion restriction in bilateral posterior aspect of thalami in pulvinar region indicating pulvinar sign <img src='img/arrows/CO.png'/>. These signs are more common in vCJD but can occur in sporadic cases.](images/app.statdx.com_image_thumbnail_e24185d3-ceca-4ae7-ac34-c7d96cf7ecbf_size_168_quality_85_27339c1d_20251014T204650Z.jpg)
*Axial DWI MR in a different patient with vCJD shows diffusion restriction in bilateral posterior aspect of thalami in pulvinar region indicating pulvinar sign <img src='img/arrows/CO.png'/>. These signs are more common in vCJD but can occur in sporadic cases.*
![Axial DWI MR in a different patient with vCJD shows diffusion restriction in bilateral posterior aspect of thalami in pulvinar region indicating pulvinar sign <img src='img/arrows/CO.png'/>. These signs are more common in vCJD but can occur in sporadic cases.](images/app.statdx.com_image_thumbnail_e24185d3-ceca-4ae7-ac34-c7d96cf7ecbf_size_168_quality_85_47787a9f_20251014T193441Z.jpg)
*Axial DWI MR in a different patient with vCJD shows diffusion restriction in bilateral posterior aspect of thalami in pulvinar region indicating pulvinar sign <img src='img/arrows/CO.png'/>. These signs are more common in vCJD but can occur in sporadic cases.*
![Axial DWI MR in a patient with sCJD shows typical bilateral asymmetric (right &gt; &gt; left) cortical diffusion restriction <img src='img/arrows/CS.png'/> with sparing of perirolandic cortex <img src='img/arrows/CC.png'/>.](images/app.statdx.com_image_thumbnail_ecb3a595-fee9-4143-b831-0f033d48423c_size_168_quality_85_9faba41b_20251014T193441Z.jpg)
*Axial DWI MR in a patient with sCJD shows typical bilateral asymmetric (right &gt; &gt; left) cortical diffusion restriction <img src='img/arrows/CS.png'/> with sparing of perirolandic cortex <img src='img/arrows/CC.png'/>.*
![Axial DWI MR in a patient with sCJD shows typical bilateral asymmetric (right &gt; &gt; left) cortical diffusion restriction <img src='img/arrows/CS.png'/> with sparing of perirolandic cortex <img src='img/arrows/CC.png'/>.](images/app.statdx.com_image_thumbnail_ecb3a595-fee9-4143-b831-0f033d48423c_size_168_quality_85_ff5dd56d_20251014T204650Z.jpg)
*Axial DWI MR in a patient with sCJD shows typical bilateral asymmetric (right &gt; &gt; left) cortical diffusion restriction <img src='img/arrows/CS.png'/> with sparing of perirolandic cortex <img src='img/arrows/CC.png'/>.*
![Axial DWI MR of a 59-year-old woman patient with rapidly progressive dementia and myoclonus demonstrates asymmetric patchy cortical restricted diffusion (cortical ribbon sign) <img src='img/arrows/CS.png'/> in cingulate gyri and frontal lobe. EEG showed periodic sharp wave complexes.](images/app.statdx.com_image_thumbnail_26b30c04-3753-4055-9913-7f5979df8a58_size_168_quality_85_2d741ca5_20251014T204650Z.jpg)
*Axial DWI MR of a 59-year-old woman patient with rapidly progressive dementia and myoclonus demonstrates asymmetric patchy cortical restricted diffusion (cortical ribbon sign) <img src='img/arrows/CS.png'/> in cingulate gyri and frontal lobe. EEG showed periodic sharp wave complexes.*
![Axial DWI MR of a 59-year-old woman patient with rapidly progressive dementia and myoclonus demonstrates asymmetric patchy cortical restricted diffusion (cortical ribbon sign) <img src='img/arrows/CS.png'/> in cingulate gyri and frontal lobe. EEG showed periodic sharp wave complexes.](images/app.statdx.com_image_thumbnail_26b30c04-3753-4055-9913-7f5979df8a58_size_168_quality_85_34ae1a3d_20251014T193441Z.jpg)
*Axial DWI MR of a 59-year-old woman patient with rapidly progressive dementia and myoclonus demonstrates asymmetric patchy cortical restricted diffusion (cortical ribbon sign) <img src='img/arrows/CS.png'/> in cingulate gyri and frontal lobe. EEG showed periodic sharp wave complexes.*
![Axial DWI MR in a patient with sCJD shows asymmetric diffusion restriction in right BG <img src='img/arrows/CS.png'/> and cortex <img src='img/arrows/CO.png'/>.](images/app.statdx.com_image_thumbnail_3569a76a-3b14-4b0c-a2d7-3eeacfa9ebfa_size_168_quality_85_5e7d9755_20251014T204650Z.jpg)
*Axial DWI MR in a patient with sCJD shows asymmetric diffusion restriction in right BG <img src='img/arrows/CS.png'/> and cortex <img src='img/arrows/CO.png'/>.*
![Axial DWI MR in a patient with sCJD shows asymmetric diffusion restriction in right BG <img src='img/arrows/CS.png'/> and cortex <img src='img/arrows/CO.png'/>.](images/app.statdx.com_image_thumbnail_3569a76a-3b14-4b0c-a2d7-3eeacfa9ebfa_size_168_quality_85_673ce587_20251014T193441Z.jpg)
*Axial DWI MR in a patient with sCJD shows asymmetric diffusion restriction in right BG <img src='img/arrows/CS.png'/> and cortex <img src='img/arrows/CO.png'/>.*
![Axial DWI MR of a 61-year-old woman patient with sCJD 'Heidenhain clinical phenotype presenting with visual hallucination and optical distortion is shown. Image demonstrates cortical restricted diffusion in bilateral occipital lobes <img src='img/arrows/CS.png'/>.](images/app.statdx.com_image_thumbnail_e200bdfc-69c5-47fb-9a37-514c2580d3b1_size_168_quality_85_088ba1a2_20251014T204650Z.jpg)
*Axial DWI MR of a 61-year-old woman patient with sCJD 'Heidenhain clinical phenotype presenting with visual hallucination and optical distortion is shown. Image demonstrates cortical restricted diffusion in bilateral occipital lobes <img src='img/arrows/CS.png'/>.*
![Axial DWI MR of a 61-year-old woman patient with sCJD 'Heidenhain clinical phenotype presenting with visual hallucination and optical distortion is shown. Image demonstrates cortical restricted diffusion in bilateral occipital lobes <img src='img/arrows/CS.png'/>.](images/app.statdx.com_image_thumbnail_e200bdfc-69c5-47fb-9a37-514c2580d3b1_size_168_quality_85_f215fc51_20251014T193441Z.jpg)
*Axial DWI MR of a 61-year-old woman patient with sCJD 'Heidenhain clinical phenotype presenting with visual hallucination and optical distortion is shown. Image demonstrates cortical restricted diffusion in bilateral occipital lobes <img src='img/arrows/CS.png'/>.*
![Axial DWI MR of a 58-year-old man with rapidly progressive dementia, myoclonus, and ataxia due to sCJD demonstrates diffusion restriction involving the left cerebellum <img src='img/arrows/CS.png'/>.](images/app.statdx.com_image_thumbnail_23666349-5a51-4bc8-970d-632cbc7521b4_size_168_quality_85_387a9727_20251014T204650Z.jpg)
*Axial DWI MR of a 58-year-old man with rapidly progressive dementia, myoclonus, and ataxia due to sCJD demonstrates diffusion restriction involving the left cerebellum <img src='img/arrows/CS.png'/>.*
![Axial DWI MR of a 58-year-old man with rapidly progressive dementia, myoclonus, and ataxia due to sCJD demonstrates diffusion restriction involving the left cerebellum <img src='img/arrows/CS.png'/>.](images/app.statdx.com_image_thumbnail_23666349-5a51-4bc8-970d-632cbc7521b4_size_168_quality_85_c5dfceeb_20251014T193441Z.jpg)
*Axial DWI MR of a 58-year-old man with rapidly progressive dementia, myoclonus, and ataxia due to sCJD demonstrates diffusion restriction involving the left cerebellum <img src='img/arrows/CS.png'/>.*
![Axial DWI MR through the vertex in the same patient shows involvement of perirolandic cortex on right <img src='img/arrows/CC.png'/>. Typically the perirolandic cortex, cerebellum, and white matter are relatively spared in CJD.](images/app.statdx.com_image_thumbnail_53bdbc50-3e43-4e4b-b833-564556d0bbd8_size_168_quality_85_73aa8b6b_20251014T193441Z.jpg)
*Axial DWI MR through the vertex in the same patient shows involvement of perirolandic cortex on right <img src='img/arrows/CC.png'/>. Typically the perirolandic cortex, cerebellum, and white matter are relatively spared in CJD.*
![Axial DWI MR through the vertex in the same patient shows involvement of perirolandic cortex on right <img src='img/arrows/CC.png'/>. Typically the perirolandic cortex, cerebellum, and white matter are relatively spared in CJD.](images/app.statdx.com_image_thumbnail_53bdbc50-3e43-4e4b-b833-564556d0bbd8_size_168_quality_85_aa7e435b_20251014T204650Z.jpg)
*Axial DWI MR through the vertex in the same patient shows involvement of perirolandic cortex on right <img src='img/arrows/CC.png'/>. Typically the perirolandic cortex, cerebellum, and white matter are relatively spared in CJD.*
### Additional Images
![Axial DWI MR in a different patient shows hyperintense signal consistent with restricted diffusion in right posterior temporal lobe and occipital lobe cortex.](images/app.statdx.com_image_thumbnail_e7b406a9-c0c5-4e0f-8080-bde8eaf75927_size_168_quality_85_b6ecca3e_20251014T193441Z.jpg)
*Axial DWI MR in a different patient shows hyperintense signal consistent with restricted diffusion in right posterior temporal lobe and occipital lobe cortex.*
![Axial DWI MR in a different patient shows hyperintense signal consistent with restricted diffusion in right posterior temporal lobe and occipital lobe cortex.](images/app.statdx.com_image_thumbnail_e7b406a9-c0c5-4e0f-8080-bde8eaf75927_size_168_quality_85_f2f309eb_20251014T204650Z.jpg)
*Axial DWI MR in a different patient shows hyperintense signal consistent with restricted diffusion in right posterior temporal lobe and occipital lobe cortex.*
![Axial DWI MR shows hyperintense signal consistent with restricted diffusion within both amygdalae.](images/app.statdx.com_image_thumbnail_b380d676-53f3-4d4a-93a1-9513bdf6dcb9_size_168_quality_85_a40d8098_20251014T193441Z.jpg)
*Axial DWI MR shows hyperintense signal consistent with restricted diffusion within both amygdalae.*
![Axial DWI MR shows hyperintense signal consistent with restricted diffusion within both amygdalae.](images/app.statdx.com_image_thumbnail_b380d676-53f3-4d4a-93a1-9513bdf6dcb9_size_168_quality_85_eed4a5ed_20251014T204650Z.jpg)
*Axial DWI MR shows hyperintense signal consistent with restricted diffusion within both amygdalae.*
![Axial DWI MR demonstrates bright signal of restricted diffusion in bodies of both caudate nuclei.](images/app.statdx.com_image_thumbnail_8539fe0b-d13a-4ea3-95b6-7d19f22564c1_size_168_quality_85_7969fb9b_20251014T204650Z.jpg)
*Axial DWI MR demonstrates bright signal of restricted diffusion in bodies of both caudate nuclei.*
![Axial DWI MR demonstrates bright signal of restricted diffusion in bodies of both caudate nuclei.](images/app.statdx.com_image_thumbnail_8539fe0b-d13a-4ea3-95b6-7d19f22564c1_size_168_quality_85_9df386fa_20251014T193441Z.jpg)
*Axial DWI MR demonstrates bright signal of restricted diffusion in bodies of both caudate nuclei.*
![Axial FLAIR MR shows bilateral hyperintense signal in putamina and thalami from CJD.](images/app.statdx.com_image_thumbnail_a0e09357-95c6-4dc3-a87f-de6ae33b54b9_size_168_quality_85_aa5ecfb2_20251014T204650Z.jpg)
*Axial FLAIR MR shows bilateral hyperintense signal in putamina and thalami from CJD.*
![Axial FLAIR MR shows bilateral hyperintense signal in putamina and thalami from CJD.](images/app.statdx.com_image_thumbnail_a0e09357-95c6-4dc3-a87f-de6ae33b54b9_size_168_quality_85_b05a5da5_20251014T193441Z.jpg)
*Axial FLAIR MR shows bilateral hyperintense signal in putamina and thalami from CJD.*
![Coronal FLAIR MR in the same patient with CJD demonstrates hyperintense signal in both thalami.](images/app.statdx.com_image_thumbnail_51cedbed-f543-428f-b099-7eb36adc1516_size_168_quality_85_7e58b261_20251014T193441Z.jpg)
*Coronal FLAIR MR in the same patient with CJD demonstrates hyperintense signal in both thalami.*
![Coronal FLAIR MR in the same patient with CJD demonstrates hyperintense signal in both thalami.](images/app.statdx.com_image_thumbnail_51cedbed-f543-428f-b099-7eb36adc1516_size_168_quality_85_ed09b9eb_20251014T204650Z.jpg)
*Coronal FLAIR MR in the same patient with CJD demonstrates hyperintense signal in both thalami.*
![Coronal FLAIR MR shows hyperintense signal in caudate nuclei, lentiform nuclei, and within temporal lobe cortices and hippocampi.](images/app.statdx.com_image_thumbnail_55fa3369-b475-4197-8fd9-8007acf79036_size_168_quality_85_78dfc5ef_20251014T193441Z.jpg)
*Coronal FLAIR MR shows hyperintense signal in caudate nuclei, lentiform nuclei, and within temporal lobe cortices and hippocampi.*
![Coronal FLAIR MR shows hyperintense signal in caudate nuclei, lentiform nuclei, and within temporal lobe cortices and hippocampi.](images/app.statdx.com_image_thumbnail_55fa3369-b475-4197-8fd9-8007acf79036_size_168_quality_85_f8758a07_20251014T204650Z.jpg)
*Coronal FLAIR MR shows hyperintense signal in caudate nuclei, lentiform nuclei, and within temporal lobe cortices and hippocampi.*
![Axial DWI MR shows bilateral restricted diffusion in the putamen and caudate nuclei with small foci in thalami.](images/app.statdx.com_image_thumbnail_c089c985-2ef0-466d-8b21-81e2a5680c21_size_168_quality_85_0cb17b32_20251014T204650Z.jpg)
*Axial DWI MR shows bilateral restricted diffusion in the putamen and caudate nuclei with small foci in thalami.*
![Axial DWI MR shows bilateral restricted diffusion in the putamen and caudate nuclei with small foci in thalami.](images/app.statdx.com_image_thumbnail_c089c985-2ef0-466d-8b21-81e2a5680c21_size_168_quality_85_31985812_20251014T193441Z.jpg)
*Axial DWI MR shows bilateral restricted diffusion in the putamen and caudate nuclei with small foci in thalami.*
![Axial T2WI MR shows bilateral increased signal intensity in putamen and caudate nuclei in a patient with CJD.](images/app.statdx.com_image_thumbnail_1e39af12-6d04-4f42-8485-4f3017afaee1_size_168_quality_85_ce9786af_20251014T193441Z.jpg)
*Axial T2WI MR shows bilateral increased signal intensity in putamen and caudate nuclei in a patient with CJD.*
![Axial T2WI MR shows bilateral increased signal intensity in putamen and caudate nuclei in a patient with CJD.](images/app.statdx.com_image_thumbnail_1e39af12-6d04-4f42-8485-4f3017afaee1_size_168_quality_85_e44a767a_20251014T204650Z.jpg)
*Axial T2WI MR shows bilateral increased signal intensity in putamen and caudate nuclei in a patient with CJD.*
![Axial FLAIR MR shows symmetric hyperintensity in the caudate and putamen, characteristic of sCJD. sCJD is the most common type of CJD, representing 85% of cases.](images/app.statdx.com_image_thumbnail_d2ae5a20-a66c-4811-ae20-1ed4157f169d_size_168_quality_85_10d4aa6f_20251014T204650Z.jpg)
*Axial FLAIR MR shows symmetric hyperintensity in the caudate and putamen, characteristic of sCJD. sCJD is the most common type of CJD, representing 85% of cases.*
![Axial FLAIR MR shows symmetric hyperintensity in the caudate and putamen, characteristic of sCJD. sCJD is the most common type of CJD, representing 85% of cases.](images/app.statdx.com_image_thumbnail_d2ae5a20-a66c-4811-ae20-1ed4157f169d_size_168_quality_85_9f4d36ac_20251014T193441Z.jpg)
*Axial FLAIR MR shows symmetric hyperintensity in the caudate and putamen, characteristic of sCJD. sCJD is the most common type of CJD, representing 85% of cases.*
![Axial DWI MR shows asymmetric diffusion restriction in the caudate nuclei and putamen. Involvement of the anterior more than the posterior putamen is typical of CJD. There is also asymmetric hyperintensity in the frontal and temporal lobe cortical ribbons <img src='img/arrows/WS.png'/>, typical of sCJD. (Courtesy N. Fischbein, MD.)](images/app.statdx.com_image_thumbnail_679fe5b1-5eb5-4ed4-805b-61d64cc3f0f8_size_168_quality_85_49d86b4d_20251014T193441Z.jpg)
*Axial DWI MR shows asymmetric diffusion restriction in the caudate nuclei and putamen. Involvement of the anterior more than the posterior putamen is typical of CJD. There is also asymmetric hyperintensity in the frontal and temporal lobe cortical ribbons <img src='img/arrows/WS.png'/>, typical of sCJD. (Courtesy N. Fischbein, MD.)*
![Axial DWI MR shows asymmetric diffusion restriction in the caudate nuclei and putamen. Involvement of the anterior more than the posterior putamen is typical of CJD. There is also asymmetric hyperintensity in the frontal and temporal lobe cortical ribbons <img src='img/arrows/WS.png'/>, typical of sCJD. (Courtesy N. Fischbein, MD.)](images/app.statdx.com_image_thumbnail_679fe5b1-5eb5-4ed4-805b-61d64cc3f0f8_size_168_quality_85_5b528feb_20251014T204650Z.jpg)
*Axial DWI MR shows asymmetric diffusion restriction in the caudate nuclei and putamen. Involvement of the anterior more than the posterior putamen is typical of CJD. There is also asymmetric hyperintensity in the frontal and temporal lobe cortical ribbons <img src='img/arrows/WS.png'/>, typical of sCJD. (Courtesy N. Fischbein, MD.)*
![Axial DWI MR shows classic sCJD with diffusion restriction in the caudate and putamen as well as throughout the cortex. Frontal, temporal, and parietal cortical involvement is most common. Relative sparing of the pre- and postcentral gyri is typical of CJD.](images/app.statdx.com_image_thumbnail_8ad70683-e456-410d-9465-fa3cdf52c09f_size_168_quality_85_2e8acda3_20251014T204650Z.jpg)
*Axial DWI MR shows classic sCJD with diffusion restriction in the caudate and putamen as well as throughout the cortex. Frontal, temporal, and parietal cortical involvement is most common. Relative sparing of the pre- and postcentral gyri is typical of CJD.*
![Axial DWI MR shows classic sCJD with diffusion restriction in the caudate and putamen as well as throughout the cortex. Frontal, temporal, and parietal cortical involvement is most common. Relative sparing of the pre- and postcentral gyri is typical of CJD.](images/app.statdx.com_image_thumbnail_8ad70683-e456-410d-9465-fa3cdf52c09f_size_168_quality_85_399462aa_20251014T193441Z.jpg)
*Axial DWI MR shows classic sCJD with diffusion restriction in the caudate and putamen as well as throughout the cortex. Frontal, temporal, and parietal cortical involvement is most common. Relative sparing of the pre- and postcentral gyri is typical of CJD.*
![Axial FLAIR MR shows bilateral, symmetric hyperintensities in the posterior thalami representing the &quot;pulvinar&quot; sign, which is characteristic of vCJD. Another &quot;pulvinar&quot; sign is the T1 shortening seen in Fabry disease.](images/app.statdx.com_image_thumbnail_dedcf7be-2668-4a63-ac0f-86a7a932a23c_size_168_quality_85_96790fd2_20251014T193441Z.jpg)
*Axial FLAIR MR shows bilateral, symmetric hyperintensities in the posterior thalami representing the &quot;pulvinar&quot; sign, which is characteristic of vCJD. Another &quot;pulvinar&quot; sign is the T1 shortening seen in Fabry disease.*
![Axial FLAIR MR shows bilateral, symmetric hyperintensities in the posterior thalami representing the &quot;pulvinar&quot; sign, which is characteristic of vCJD. Another &quot;pulvinar&quot; sign is the T1 shortening seen in Fabry disease.](images/app.statdx.com_image_thumbnail_dedcf7be-2668-4a63-ac0f-86a7a932a23c_size_168_quality_85_d4346279_20251014T204650Z.jpg)
*Axial FLAIR MR shows bilateral, symmetric hyperintensities in the posterior thalami representing the &quot;pulvinar&quot; sign, which is characteristic of vCJD. Another &quot;pulvinar&quot; sign is the T1 shortening seen in Fabry disease.*
![Axial DWI MR shows symmetric hyperintensity in the BG and thalami bilaterally. The thalamic involvement shows the &quot;hockey stick&quot; sign, which is symmetric pulvinar and dorsomedial thalamus hyperintensity. This sign is most commonly seen in vCJD but may also be present in sCJD, as in this case.](images/app.statdx.com_image_thumbnail_33b09555-58a3-4258-a169-d579478591d4_size_168_quality_85_760f79ce_20251014T204650Z.jpg)
*Axial DWI MR shows symmetric hyperintensity in the BG and thalami bilaterally. The thalamic involvement shows the &quot;hockey stick&quot; sign, which is symmetric pulvinar and dorsomedial thalamus hyperintensity. This sign is most commonly seen in vCJD but may also be present in sCJD, as in this case.*
![Axial DWI MR shows symmetric hyperintensity in the BG and thalami bilaterally. The thalamic involvement shows the &quot;hockey stick&quot; sign, which is symmetric pulvinar and dorsomedial thalamus hyperintensity. This sign is most commonly seen in vCJD but may also be present in sCJD, as in this case.](images/app.statdx.com_image_thumbnail_33b09555-58a3-4258-a169-d579478591d4_size_168_quality_85_c81c8d1d_20251014T193441Z.jpg)
*Axial DWI MR shows symmetric hyperintensity in the BG and thalami bilaterally. The thalamic involvement shows the &quot;hockey stick&quot; sign, which is symmetric pulvinar and dorsomedial thalamus hyperintensity. This sign is most commonly seen in vCJD but may also be present in sCJD, as in this case.*
docs_md/articles/crossed-cerebellar-diaschisis_c1e384b3-3c6e-4f67-bf79-5187bd6a1b86.md
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---
title: "Crossed Cerebellar Diaschisis"
docid: "c1e384b3-3c6e-4f67-bf79-5187bd6a1b86"
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value: "Santhosh Gaddikeri, MD"
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value: "Miral D. Jhaveri, MD, MBA"
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pageDescription: "Crossed Cerebellar Diaschisis"
pageKeywords: "Brain, Diagnosis, Pathology-Based Diagnoses, Acquired Toxic/Metabolic/Degenerative Disorders, Dementias and Degenerative Disorders, Crossed Cerebellar Diaschisis"
pageTitle: "Crossed Cerebellar Diaschisis | STATdx"
enhancedTitle: "Crossed Cerebellar Diaschisis"
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breadcrumbs:
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- "Crossed Cerebellar Diaschisis"
---
# KEY FACTS
- ## Terminology
- Diaschisis: Sudden loss of function in brain connected to (but at distance from) damaged area
- CCD: ↓ blood flow/metabolism in cerebellar hemisphere contralateral to supratentorial infarct
- ## Imaging
- Acute: CT/MR perfusion shows ↓ CBF in cerebellar hemisphere opposite acute hemispheric infarct
- ↑ TTP, ↓ CBF in cerebellum contralateral to infarct
- Add DTI as subtle cases may show ↓ FA when conventional MR normal
- F-18 FDG PET/CT shows diffusely reduced uptake in contralateral cerebellar hemisphere
- Chronic: CT or MR shows atrophic cerebellar hemisphere opposite old cerebral hemispheric infarct/insult
- ## Top Differential Diagnoses
- Superior cerebellar artery infarct
- CCD involved > just SCA territory
- Encephalomalacia
- Trauma, infection, surgery
- Cerebellitis
- Cerebellum swollen, hyperintense (not shrunken, atrophic)
- Bilateral > unilateral
- ## Pathology
- CPC tract
- Input to cerebellum via CPC tracts 40x all other afferent sources combined
- Injury at any point along CPC can result in ↓ CBF, metabolism in contralateral cerebellar hemisphere
- Most common cause: MCA infarct
- Others: Status epilepticus, neoplasm, trauma, surgery,migraine, Rasmussen encephalitis, etc.
- Occurrence & severity of CCD related to degree of low supratentorial perfusion & decrease in ADC value of infarct
# TERMINOLOGY
- ## Abbreviations
- Crossed cerebellar diaschisis (CCD)
- ## Definitions
- Diaschisis: Sudden loss of function in brain connected to (but at distance from) damaged area
- CCD: Decreased blood flow/metabolism in cerebellar hemisphere contralateral to supratentorial infarct
- Caused by interrupted afferent input through corticopontocerebellar tract (CPC)
- CCD occurs in both acute & chronic phases
- Acute CCD results from functional deafferentation
- Subacute, chronic CCD reflects transneuronal degeneration
# IMAGING
- ## General Features
- ### Best diagnostic clue
- Acute: CT/MR perfusion shows ↓ cerebral blood flow (CBF) in cerebellar hemisphere opposite acute cerebral hemispheric infarct
- Chronic: CT or MR shows atrophic cerebellar hemisphere opposite old cerebral hemispheric infarct/insult
- ### Location
- Cerebellar hemisphere opposite cerebral hemispheric infarct
- ## Imaging Recommendations
- ### Best imaging tool
- Acute: CT or MR perfusion
- PET/CT also effective but expensive; variable availability
- Chronic: MR with T2WI, FLAIR, DTI
- ### Protocol advice
- Add DTI as subtle cases may show ↓ fractional anisotropy (FA) when conventional MR normal
- ## CT Findings
- ### NECT
- Acute: Normal
- Chronic: Cerebellar atrophy contralateral to supratentorial infarct
- ### CTA
- Middle cerebral artery (MCA) occlusion
- Cerebellar vessels appear normal
- CT perfusion
- ↑ TTP, ↓ CBF in cerebellum contralateral to infarct
- ## MR Findings
- ### T1WI
- Unilateral cerebellar atrophy
- ### T2WI
- Folia shrunken, fissures enlarged
- ### FLAIR
- Except for atrophy, cerebellum usually normal
- ### MRA
- Posterior fossa vasculature normal
- DTI
- Shows ↓ FA in middle cerebellar peduncle
- Visualizes altered CPC in chronic CCD that may not be seen on conventional MR
- Arterial spin labeling (ASL) perfusion
- ↓ CBF in cerebellum contralateral to cerebral hemispheric abnormality
- ## Nuclear Medicine Findings
- ### PET/CT
- F-18 FDG PET/CT shows diffusely reduced uptake in contralateral cerebellar hemisphere
- L-(methyl-11C) methionine (MET) uptake not reduced
- ### Tc-99m sulfur colloid
- Tc-99m ECD, HMPAO SPECT can demonstrate distant areas of ↓ CBF, metabolism (diaschisis)
# DIFFERENTIAL DIAGNOSIS
- ## Superior Cerebellar Artery Infarct
- CCD involves most of cerebellum, not just superior cerebellar artery (SCA) territory
- Contralateral MCA infarct absent
- ## Encephalomalacia
- No history of trauma, contralateral MCA infarct
- [Cerebellitis](/document/cerebellitis/2a2d695e-63be-4839-9e1a-cd8813b005d6)
- Cerebellum swollen, not shrunken
- Bilateral > unilateral
# PATHOLOGY
- ## General Features
- ### Etiology
- CPC tract
- Large afferent pathway derived from very extensive areas of cortex
- Input to cerebellum via CPC tracts 40x all other afferent sources combined
- 1st-order neurons arrive in ipsilateral pons
- Synapse with 2nd-order neurons
- Then cross to opposite cerebellar hemisphere via middle cerebellar peduncle
- Injury at any point along CPC can result in ↓ CBF, metabolism in contralateral cerebellar hemisphere
- Most common cause: MCA infarct
- Others: Status epilepticus, neoplasm, trauma, surgery, migraine, Rasmussen encephalitis, etc.
- Occurrence & severity of CCD related to degree of low supratentorial perfusion & decrease in ADC value of infarct
# CLINICAL ISSUES
- ## Natural History & Prognosis
- CCD represents temporal continuum
- Early, reversible functional hypometabolism
- Cerebellum recovers (typical)
- Irreversible degeneration in up to 20%
- Cerebellar atrophy
- Can be seen decades after initial insult
d7407fea-fff9-488b-9712-71a4e559c250
## References
# Selected References
1. [Zhang M et al: Characteristics of cerebral perfusion and diffusion associated with crossed cerebellar diaschisis after acute ischemic stroke. Jpn J Radiol. 38(2):126-34, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=31720951%5Bpmid%5D)
1. [Kim JS et al: Degeneration of the inferior cerebellar peduncle after middle cerebral artery stroke: another perspective on crossed cerebellar diaschisis. Stroke. 50(10):2700-7, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31446886%5Bpmid%5D)
1. [Yokota H et al: Crossed cerebellar diaschisis in status epilepticus. Neurochirurgie. 65(6):425-6, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31505193%5Bpmid%5D)
1. [Liu X et al: Pathological factors contributing to crossed cerebellar diaschisis in cerebral gliomas: a study combining perfusion, diffusion, and structural MR imaging. Neuroradiology. 60(6):643-50, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=29666881%5Bpmid%5D)
1. [Reesink FE et al: Crossed cerebellar diaschisis in Alzheimer's disease. Curr Alzheimer Res. 15(13):1267-75, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=30210001%5Bpmid%5D)
1. [Strother MK et al: Crossed cerebellar diaschisis after stroke identified noninvasively with cerebral blood flow-weighted arterial spin labeling MRI. Eur J Radiol. 85(1):136-42, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=26724658%5Bpmid%5D)
1. [Chen S et al: Crossed cerebellar diaschisis detected by arterial spin-labeled perfusion magnetic resonance imaging in subacute ischemic stroke. J Stroke Cerebrovasc Dis. 23(9):2378-83, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=25183560%5Bpmid%5D)
1. [Zaidi SA et al: Crossed cerebellar diaschisis: a radiological finding in status epilepticus not to miss. BMJ Case Rep. 2013, 2013](http://www.ncbi.nlm.nih.gov/pubmed/?term=24234428%5Bpmid%5D)
1. [Jeon YW et al: Dynamic CT perfusion imaging for the detection of crossed cerebellar diaschisis in acute ischemic stroke. Korean J Radiol. 13(1):12-9, 2012](http://www.ncbi.nlm.nih.gov/pubmed/?term=22247631%5Bpmid%5D)
1. [Massaro AM: Teaching neuroimages: crossed cerebellar diaschisis in hemispheric status epilepticus. Neurology. 79(20):e182, 2012](http://www.ncbi.nlm.nih.gov/pubmed/?term=23150537%5Bpmid%5D)
1. [Agrawal KL et al: Crossed cerebellar diaschisis on F-18 FDG PET/CT. Indian J Nucl Med. 26(2):102-3, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=22174518%5Bpmid%5D)
1. [Garg G et al: Crossed cerebellar diaschisis demonstrated by (18)F- FDG-PET/CT. Hell J Nucl Med. 12(2):171-2, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=19675876%5Bpmid%5D)
1. [Huang YC et al: Periictal magnetic resonance imaging in status epilepticus. Epilepsy Res. 86(1):72-81, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=19541453%5Bpmid%5D)
1. [Lin DD et al: Crossed cerebellar diaschisis in acute stroke detected by dynamic susceptibility contrast MR perfusion imaging. AJNR Am J Neuroradiol. 30(4):710-5, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=19193758%5Bpmid%5D)
1. [Dodick DW et al: Crossed cerebellar diaschisis during migraine with prolonged aura: a possible mechanism for cerebellar infarctions. Cephalalgia. 28(1):83-6, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=17970765%5Bpmid%5D)
1. [Kajimoto K et al: Crossed cerebellar diaschisis: a positron emission tomography study with L-[methyl-11C]methionine and 2-deoxy-2-[18F]fluoro-D-glucose. Ann Nucl Med. 21(2):109-13, 2007](http://www.ncbi.nlm.nih.gov/pubmed/?term=17424977%5Bpmid%5D)
1. [Liu Y et al: Crossed cerebellar diaschisis in acute ischemic stroke: a study with serial SPECT and MRI. J Cereb Blood Flow Metab. 27(10):1724-32, 2007](http://www.ncbi.nlm.nih.gov/pubmed/?term=17311077%5Bpmid%5D)
1. [Kim J et al: Decreased fractional anisotropy of middle cerebellar peduncle in crossed cerebellar diaschisis: diffusion-tensor imaging-positron-emission tomography correlation study. AJNR Am J Neuroradiol. 26(9):2224-8, 2005](http://www.ncbi.nlm.nih.gov/pubmed/?term=16219826%5Bpmid%5D)
## Images
### Selected Images
![Coronal T2 TSE of 55-year-old man with past history of right middle cerebral artery (MCA) distribution infarction shows encephalomalacia in the right frontal &amp; temporal lobes <img src='img/arrows/CS.png'/> with ex vacuo dilation of right lateral ventricle <img src='img/arrows/CO.png'/> due to volume loss.](images/app.statdx.com_image_thumbnail_d35d8833-e3cc-45e4-9ab3-16c591cc8b67_size_168_quality_85_27722937_20251014T204643Z.jpg)
*Coronal T2 TSE of 55-year-old man with past history of right middle cerebral artery (MCA) distribution infarction shows encephalomalacia in the right frontal &amp; temporal lobes <img src='img/arrows/CS.png'/> with ex vacuo dilation of right lateral ventricle <img src='img/arrows/CO.png'/> due to volume loss.*
![Coronal T2 TSE through the posterior fossa in the same patient shows asymmetric atrophy with shrunken folia &amp; prominent fissures <img src='img/arrows/CC.png'/> of the left cerebellar hemisphere contralateral to supratentorial insult due to crossed cerebellar diaschisis (CCD).](images/app.statdx.com_image_thumbnail_c1a21b5a-5118-41cb-ab96-9c73e74647d5_size_168_quality_85_7c9b0842_20251014T204643Z.jpg)
*Coronal T2 TSE through the posterior fossa in the same patient shows asymmetric atrophy with shrunken folia &amp; prominent fissures <img src='img/arrows/CC.png'/> of the left cerebellar hemisphere contralateral to supratentorial insult due to crossed cerebellar diaschisis (CCD).*
![Axial CECT in 72-year-old woman with history of metastatic lung cancer &amp; remote lacunar infarction in left basal ganglia &amp; adjacent internal capsule shows a remote lacunar infarction in left gangliocapsular region <img src='img/arrows/CS.png'/>.](images/app.statdx.com_image_thumbnail_7373e8c5-1de5-4c12-9111-676f51d47fab_size_168_quality_85_e2a99f10_20251014T204643Z.jpg)
*Axial CECT in 72-year-old woman with history of metastatic lung cancer &amp; remote lacunar infarction in left basal ganglia &amp; adjacent internal capsule shows a remote lacunar infarction in left gangliocapsular region <img src='img/arrows/CS.png'/>.*
![Axial F-18 FDG PET/CT in the same patient performed for lung cancer restaging is shown. Axial fused images through the inferior aspect of posterior fossa show asymmetric ↓ metabolic activity in the right cerebellum <img src='img/arrows/CC.png'/> due to CCD. (Courtesy M. Matesan, MD.)](images/app.statdx.com_image_thumbnail_2efb45b4-cd21-4947-a44a-20fab7cffdf8_size_168_quality_85_0dd44345_20251014T204643Z.jpg)
*Axial F-18 FDG PET/CT in the same patient performed for lung cancer restaging is shown. Axial fused images through the inferior aspect of posterior fossa show asymmetric ↓ metabolic activity in the right cerebellum <img src='img/arrows/CC.png'/> due to CCD. (Courtesy M. Matesan, MD.)*
### Additional Images
![Axial T2 MR in the same patient shows ipsilateral hypertrophic olivary degeneration <img src='img/arrows/BS.png'/> (chronic, with shrunken hyperintense olive). CCD with atrophy of the contralateral cerebellar hemisphere is also present <img src='img/arrows/BO.png'/>. While CCD is usually caused by an MCA stroke in the contralateral cerebral hemisphere, interruption of the corticopontocerebellar (CPC) tract anywhere along its course can result in similar findings. In this case, midbrain surgery caused deafferentation of the contralateral cerebellar hemisphere. It is rare to find both hypertrophic olivary degeneration &amp; CCD in the same patient.](images/app.statdx.com_image_thumbnail_702f7a2b-eb34-4add-b66d-17bf7cf5d92d_size_168_quality_85_c7ade354_20251014T204643Z.jpg)
*Axial T2 MR in the same patient shows ipsilateral hypertrophic olivary degeneration <img src='img/arrows/BS.png'/> (chronic, with shrunken hyperintense olive). CCD with atrophy of the contralateral cerebellar hemisphere is also present <img src='img/arrows/BO.png'/>. While CCD is usually caused by an MCA stroke in the contralateral cerebral hemisphere, interruption of the corticopontocerebellar (CPC) tract anywhere along its course can result in similar findings. In this case, midbrain surgery caused deafferentation of the contralateral cerebellar hemisphere. It is rare to find both hypertrophic olivary degeneration &amp; CCD in the same patient.*
![Axial T2 MR shows a 29-year-old man who developed palatal myoclonus several months following midbrain surgery for cavernous malformation. Imaging was obtained 1 year after surgery. This scan through the upper midbrain shows atrophy of the left pons &amp; cerebral peduncle <img src='img/arrows/BS.png'/>.](images/app.statdx.com_image_thumbnail_2961d0a1-3fe3-47b7-acda-81d87535c507_size_168_quality_85_b8854552_20251014T204643Z.jpg)
*Axial T2 MR shows a 29-year-old man who developed palatal myoclonus several months following midbrain surgery for cavernous malformation. Imaging was obtained 1 year after surgery. This scan through the upper midbrain shows atrophy of the left pons &amp; cerebral peduncle <img src='img/arrows/BS.png'/>.*
![Axial T2 MR in a patient with a history of remote right MCA infarct shows the typical changes of volume loss &amp; hyperintensity in the cortex &amp; subcortical white matter <img src='img/arrows/WS.png'/>. The ipsilateral ventricle is enlarged.](images/app.statdx.com_image_thumbnail_ce748546-4bcd-4002-b017-68be804cb16d_size_168_quality_85_65e7e772_20251014T204643Z.jpg)
*Axial T2 MR in a patient with a history of remote right MCA infarct shows the typical changes of volume loss &amp; hyperintensity in the cortex &amp; subcortical white matter <img src='img/arrows/WS.png'/>. The ipsilateral ventricle is enlarged.*
![Axial T2 MR in the same patient shows volume loss with enlarged horizontal sulci in the contralateral cerebellar hemisphere <img src='img/arrows/WO.png'/>. These findings are consistent with chronic CCD.](images/app.statdx.com_image_thumbnail_08d57c6c-4b91-4984-9eb7-fec86d23ec10_size_168_quality_85_1cded8c8_20251014T204643Z.jpg)
*Axial T2 MR in the same patient shows volume loss with enlarged horizontal sulci in the contralateral cerebellar hemisphere <img src='img/arrows/WO.png'/>. These findings are consistent with chronic CCD.*
![Whole-brain CT perfusion study was obtained as part of an emergency stroke evaluation in a patient with acute onset of right-sided weakness. CBF shows ↓ blood flow <img src='img/arrows/WS.png'/> (blue area) in the left MCA distribution.](images/app.statdx.com_image_thumbnail_449f7451-535d-45f5-83af-2acc18d821f7_size_168_quality_85_5a429dd0_20251014T204643Z.jpg)
*Whole-brain CT perfusion study was obtained as part of an emergency stroke evaluation in a patient with acute onset of right-sided weakness. CBF shows ↓ blood flow <img src='img/arrows/WS.png'/> (blue area) in the left MCA distribution.*
![Section through the cerebellar hemispheres in the same patient shows acutely ↓ CBF in the right cerebellar hemisphere <img src='img/arrows/WO.png'/>. As afferent input to the cerebellum is reduced, regional metabolism diminishes &amp; CBF falls. This is hyperacute CCD.](images/app.statdx.com_image_thumbnail_8b62b006-7562-4020-83b2-061b9cab9054_size_168_quality_85_78539d04_20251014T204643Z.jpg)
*Section through the cerebellar hemispheres in the same patient shows acutely ↓ CBF in the right cerebellar hemisphere <img src='img/arrows/WO.png'/>. As afferent input to the cerebellum is reduced, regional metabolism diminishes &amp; CBF falls. This is hyperacute CCD.*
![CBF map in the same patient demonstrates ↓ CBF in the right cerebellar hemisphere <img src='img/arrows/WS.png'/> consistent with acute CCD. CCD is caused by an interruption of the CPC fibers. As a result of ↓ afferent input, there is a ↓ in cerebellar metabolism, coupled with a ↓ in cerebellar perfusion.](images/app.statdx.com_image_thumbnail_d74feac0-ed66-4483-9953-f76485129ee9_size_168_quality_85_9602fa53_20251014T204643Z.jpg)
*CBF map in the same patient demonstrates ↓ CBF in the right cerebellar hemisphere <img src='img/arrows/WS.png'/> consistent with acute CCD. CCD is caused by an interruption of the CPC fibers. As a result of ↓ afferent input, there is a ↓ in cerebellar metabolism, coupled with a ↓ in cerebellar perfusion.*
![MR perfusion study CBF map in a patient with acute right hemiparesis shows ↓ blood flow <img src='img/arrows/WS.png'/> (blue area) in the left MCA distribution.](images/app.statdx.com_image_thumbnail_1808e97b-244a-489c-b46c-40a4d17f171d_size_168_quality_85_d8fc3fa5_20251014T204643Z.jpg)
*MR perfusion study CBF map in a patient with acute right hemiparesis shows ↓ blood flow <img src='img/arrows/WS.png'/> (blue area) in the left MCA distribution.*
![Axial T2 MR in a patient with a remote left MCA infarct shows the typical changes of encephalomalacia &amp; volume loss <img src='img/arrows/CC.png'/>. The ipsilateral ventricle is enlarged <img src='img/arrows/CS.png'/>.](images/app.statdx.com_image_thumbnail_501513c1-4158-47b1-a12c-1d5a749ba157_size_168_quality_85_37491498_20251014T204643Z.jpg)
*Axial T2 MR in a patient with a remote left MCA infarct shows the typical changes of encephalomalacia &amp; volume loss <img src='img/arrows/CC.png'/>. The ipsilateral ventricle is enlarged <img src='img/arrows/CS.png'/>.*
![Axial T2 MR in the same patient shows volume loss with enlarged horizontal sulci in the contralateral cerebellar hemisphere <img src='img/arrows/CC.png'/>. These findings are consistent with chronic CCD.](images/app.statdx.com_image_thumbnail_3616b284-41a3-4d67-849e-f0d88b56f4aa_size_168_quality_85_81553915_20251014T204643Z.jpg)
*Axial T2 MR in the same patient shows volume loss with enlarged horizontal sulci in the contralateral cerebellar hemisphere <img src='img/arrows/CC.png'/>. These findings are consistent with chronic CCD.*
docs_md/articles/csf-like-parenchymal-lesions_24559f7a-ed5a-4ab6-90ba-769f0b5c1197.md
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---
title: "CSF-Like Parenchymal Lesion(s)"
docid: "24559f7a-ed5a-4ab6-90ba-769f0b5c1197"
authors:
- key: "4b6589b0-9b8d-4467-8a90-01a0a59742fc"
value: "Troy A. Hutchins, MD"
- key: "5cff4116-3654-4b3a-bb75-5ebe0b8c9850"
value: "Anne G. Osborn, MD, FACR"
breadcrumbs:
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name: "Brain"
slug: "brain"
treeNodeId: "6d8829f1-14d7-45af-8675-255189aa526a"
-
name: "Differential Diagnosis"
slug: "differential-diagnosis"
treeNodeId: "a7fdd139-664e-4bb8-8d18-400e4733ff60"
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name: "Brain Parenchyma, General"
slug: "brain-parenchyma-general"
treeNodeId: "e79be97b-28c0-4023-be87-334c0579d35d"
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name: "Generic Imaging Patterns"
slug: "generic-imaging-patterns"
treeNodeId: "66ab9cf6-74ad-42b7-a40a-4b6224edaa25"
-
name: "CSF-Like Parenchymal Lesion(s)"
slug: "csf-like-parenchymal-lesions"
treeNodeId: null
category: "Brain"
documentVersionId: "5368df09-e002-41e2-8b4e-fbc71286b47d"
imageCount: 51
lastUpdated: "01/18/23"
pageDescription: "CSF-Like Parenchymal Lesion(s)"
pageKeywords: "Brain, Differential Diagnosis, Brain Parenchyma, General, Generic Imaging Patterns, CSF-Like Parenchymal Lesion(s)"
pageTitle: "CSF-Like Parenchymal Lesion(s) | STATdx"
enhancedTitle: "CSF-Like Parenchymal Lesion(s)"
type: "DDX"
references: true
breadcrumbs:
- "Brain"
- "Differential Diagnosis"
- "Brain Parenchyma, General"
- "Generic Imaging Patterns"
- "CSF-Like Parenchymal Lesion(s)"
---
# ESSENTIAL INFORMATION
- ## Key Differential Diagnosis Issues
- Key imaging questions
- Does lesion follow CSF on all modalities/sequences?
- Is there any associated mass effect?
- Does lesion enhance?
- Included
- CSF-like cystic mass(es), e.g., enlarged perivascular spaces (PVS), neuroglial cysts
- Excluded
- Cystic neoplasms, abscess, resolving hematoma (rarely exactly like CSF)
- Developmental cysts that do not behave exactly like CSF (e.g., epidermoid, neurenteric cysts)
- ## Helpful Clues for Common Diagnoses
- **Enlarged Perivascular Spaces**
- PVS
- Can be seen at all ages but ↑ with age
- Filled with interstitial fluid but follow CSF on all sequences
- Most have no abnormality in surrounding parenchyma on FLAIR
- ~ 25% have thin, hyperintense rim
- Bilateral > unilateral
- Multiple > solitary
- "Clusters" of variably sized, CSF-like cysts characteristic
- Can occur anywhere but most common locations = basal ganglia (BG), hemispheric white matter (WM), midbrain, dentate nuclei
- Variant (mostly in older adults) = "état criblé" ("cribriform state") with multiple tiny cysts in BG
- Classification
- Type I: BG
- Type II: High convexity WM
- Type III: Midbrain
- Large PVS cause mass effect, assume bizarre configurations, and can mimic cystic neoplasm
- **Encephalomalacia**
- Etiology varies (trauma, infarction, etc.)
- Can be solitary, multifocal, multicystic
- CSF-like ± adjacent FLAIR hyperintensity
- **Lacunar Infarction**
- Solitary or multiple
- Typically along single long, unpaired penetrating arteries &/or vascular watershed zones
- BG, thalamus, WM common
- Multifocal BG infarcts + surrounding gliosis = "état lacunaire" or "lacunar state"
- **Neurocysticercosis**
- Most neurocysticercosis (NCC) cysts are actually in sulci
- Cysts in vesicular stage smooth, thin walled, with scolex generally visible as "dot" within cyst
- Multiple lesions in mixed stages common
- Some enhance, some do not
- Ca⁺⁺ (multiple = starry-sky pattern)
- ## Helpful Clues for Less Common Diagnoses
- **Porencephalic Cyst**
- CSF-filled parenchymal cavity
- Communicates with ventricle &/or pial surface
- Lined by reactive gliosis/astrocytic proliferation
- Does not enhance
- Etiology varies (trauma, infarction, etc.)
- **Multiple Sclerosis**
- Chronic "burned-out" lesions
- Appear as CSF foci with hyperintense rinds on FLAIR
- Look for faint T1 hyperintensity surrounding lesions ("lesion within lesion")
- Obtain sagittal FLAIR to look for other lesions along callososeptal interface
- **Hippocampal Sulcus Remnants**
- "String of beads" cysts medial to temporal horns of lateral ventricles
- Developmental variant, incidental
- Remnants of vestigial primary embryonic hippocampal sulcus
- Imaging
- Between hippocampus, dentate gyrus
- Follow CSF on all sequences
- No surrounding gliosis
- **Connatal Cysts**
- Single or multiple
- Location
- Intra- or periventricular (may actually be cysts of anterior choroid plexus)
- Small cyst adjacent to tip of frontal horn may be normal anatomic variant
- Lined with ependyma
- Present at birth
- Usually transient
- Occasionally seen in older patients
- No septations, no hemosiderin
- Generally isolated without associated abnormalities
- ## Helpful Clues for Rare Diagnoses
- **Neuroglial Cyst**
- Benign, glial-lined, nonenhancing CSF-like cyst
- No surrounding signal abnormality
- Does not communicate with ventricle
- Subcortical WM, choroidal fissure common sites
- Does not restrict on DWI
- No enhancement
- **Cryptococcosis**
- Opportunistic fungal infection
- Nonenhancing, gelatinous pseudocysts in PVS
- BG, thalamus, brainstem, cerebellum, dentate nucleus, periventricular WM
- Multifocal > > solitary lesions
- Most patients have HIV/AIDS
- **Parasites, Miscellaneous**
- Other than NCC, parasitic brain cysts uncommon
- Hydatid cyst
- Unilocular cyst, isointense to CSF
- T2-hypointense rim, no enhancement
- **Mucopolysaccharidoses**
- Group of lysosomal storage disorders
- PVS dilated by accumulated glycosaminoglycans
- Corpus callosum, peritrigonal WM
- Multiple, bilateral
- Dilated PVS in deep periventricular WM
- FLAIR-hyperintense rim surrounding dilated PVS
- **Germinolytic Cysts**
- Periventricular/subependymal cysts
- Cyst(s) along caudothalamic groove probably resulting from germinolysis
- Glial (not ependymal)-lined cysts/pseudocysts
- Distinguish from "connatal" cysts (intraventricular anterior choroid plexus cysts)
- Many etiologies, including inherited metabolic disorders (e.g., Zellweger, infantile Refsum), congenital infections (CMV)
- CSF-like; ± septations, hemosiderin; do not enhance
- Look for associated abnormalities
- Leukoencephalopathy
- Delayed myelination
- Polymicrogyria, pachygyria, heterotopias
- **Miscellaneous Congenital Malformations**
- Several have parenchymal CSF-like cysts as part of syndrome
- van der Knaap leukoencephalopathies (megaloencephalic leukoencephalopathy with subcortical cysts, anterior temporal lobe cavitations)
- Congenital muscular dystrophy (cerebellar cysts common, may represent dilated PVS)
- Dorsal interhemispheric CSF cyst
- Corpus callosal dysgenesis
- Holoprosencephaly
- ## Alternative Differential Approaches
- **Based on location**
- Deep gray nuclei
- Enlarged PVS
- Lacunar infarction
- Cryptococcosis
- Periventricular WM
- Multiple sclerosis
- Connatal cysts
- Germinolytic cysts
- Lobar
- Encephalomalacia
- NCC
- Porencephalic cyst
- Neuroglial cyst
- Hydatid cyst
## References
# Selected References
1. [Filippi M et al: Present and future of the diagnostic work-up of multiple sclerosis: the imaging perspective. J Neurol. ePub, 2022](http://www.ncbi.nlm.nih.gov/pubmed/?term=36427168%5Bpmid%5D)
1. [Gyanwali B et al: Arterial spin-labeling parameters and their associations with risk factors, cerebral small-vessel disease, and etiologic subtypes of cognitive impairment and dementia. AJNR Am J Neuroradiol. 43(10):1418-23, 2022](http://www.ncbi.nlm.nih.gov/pubmed/?term=36562454%5Bpmid%5D)
1. [Kim HG et al: MRI-visible dilated perivascular space in the brain by age: the human connectome project. Radiology. 213254, 2022](http://www.ncbi.nlm.nih.gov/pubmed/?term=36378031%5Bpmid%5D)
1. [Loh DDL et al: Tumefactive perivascular spaces causing obstructive hydrocephalus. Radiology. 221724, 2022](http://www.ncbi.nlm.nih.gov/pubmed/?term=36472539%5Bpmid%5D)
1. [Roosendaal SD et al: Imaging patterns characterizing mitochondrial leukodystrophies. AJNR Am J Neuroradiol. 42(7):1334-40, 2021](http://www.ncbi.nlm.nih.gov/pubmed/?term=34255734%5Bpmid%5D)
1. [Ajtai B et al: Imaging of intracranial cysts. Continuum (Minneap Minn). 22(5, Neuroimaging):1553-73, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=27740988%5Bpmid%5D)
1. [Taillibert S et al: Intracranial cystic lesions: a review. Curr Neurol Neurosci Rep. 14(9):481, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=25106500%5Bpmid%5D)
1. [Osborn AG, Preece MT. Intracranial cysts: radiologic-pathologic correlation and imaging approach. Radiology. 239(3):650-64, 2006](http://www.ncbi.nlm.nih.gov/pubmed/?term=16714456%5Bpmid%5D)
1. [Salzman KL et al: Giant tumefactive perivascular spaces. AJNR Am J Neuroradiol. 26:298-305, 2005](http://www.ncbi.nlm.nih.gov/pubmed/?term=15709127%5Bpmid%5D)
## Images
### Selected Images
![Axial T2 MR shows a cluster of CSF-like cysts in the inferior left basal ganglia <img src='img/arrows/CS.png'/>, a common location for enlarged perivascular spaces. They are often seen at the level of the anterior commissure.](images/app.statdx.com_image_thumbnail_444b7609-9961-4277-a969-7a56d10a6b02_size_168_quality_85_040f98ab_20251014T195936Z.jpg)
**Enlarged Perivascular Spaces**
*Axial T2 MR shows a cluster of CSF-like cysts in the inferior left basal ganglia <img src='img/arrows/CS.png'/>, a common location for enlarged perivascular spaces. They are often seen at the level of the anterior commissure.*
![Axial T2 MR shows a cluster of CSF-like cysts in the inferior left basal ganglia <img src='img/arrows/CS.png'/>, a common location for enlarged perivascular spaces. They are often seen at the level of the anterior commissure.](images/app.statdx.com_image_thumbnail_444b7609-9961-4277-a969-7a56d10a6b02_size_168_quality_85_259ac5d1_20251014T195834Z.jpg)
**Enlarged Perivascular Spaces**
*Axial T2 MR shows a cluster of CSF-like cysts in the inferior left basal ganglia <img src='img/arrows/CS.png'/>, a common location for enlarged perivascular spaces. They are often seen at the level of the anterior commissure.*
![Axial T2 MR shows a cluster of CSF-like cysts in the inferior left basal ganglia <img src='img/arrows/CS.png'/>, a common location for enlarged perivascular spaces. They are often seen at the level of the anterior commissure.](images/app.statdx.com_image_thumbnail_444b7609-9961-4277-a969-7a56d10a6b02_size_168_quality_85_56f0de2d_20251014T200033Z.jpg)
**Enlarged Perivascular Spaces**
*Axial T2 MR shows a cluster of CSF-like cysts in the inferior left basal ganglia <img src='img/arrows/CS.png'/>, a common location for enlarged perivascular spaces. They are often seen at the level of the anterior commissure.*
![Axial T2 MR shows a cluster of CSF-like cysts in the inferior left basal ganglia <img src='img/arrows/CS.png'/>, a common location for enlarged perivascular spaces. They are often seen at the level of the anterior commissure.](images/app.statdx.com_image_thumbnail_444b7609-9961-4277-a969-7a56d10a6b02_size_174_quality_85_3ff9dc13_20251014T195933Z.jpg)
**Enlarged Perivascular Spaces**
*Axial T2 MR shows a cluster of CSF-like cysts in the inferior left basal ganglia <img src='img/arrows/CS.png'/>, a common location for enlarged perivascular spaces. They are often seen at the level of the anterior commissure.*
![Axial T2 MR shows a cluster of CSF-like cysts in the inferior left basal ganglia <img src='img/arrows/CS.png'/>, a common location for enlarged perivascular spaces. They are often seen at the level of the anterior commissure.](images/app.statdx.com_image_thumbnail_444b7609-9961-4277-a969-7a56d10a6b02_size_174_quality_85_db0250ac_20251014T204438Z.jpg)
**Enlarged Perivascular Spaces**
*Axial T2 MR shows a cluster of CSF-like cysts in the inferior left basal ganglia <img src='img/arrows/CS.png'/>, a common location for enlarged perivascular spaces. They are often seen at the level of the anterior commissure.*
![Axial T2 MR shows a cluster of CSF-like cysts in the inferior left basal ganglia <img src='img/arrows/CS.png'/>, a common location for enlarged perivascular spaces. They are often seen at the level of the anterior commissure.](images/app.statdx.com_image_thumbnail_444b7609-9961-4277-a969-7a56d10a6b02_size_174_quality_85_ef696965_20251014T195831Z.jpg)
**Enlarged Perivascular Spaces**
*Axial T2 MR shows a cluster of CSF-like cysts in the inferior left basal ganglia <img src='img/arrows/CS.png'/>, a common location for enlarged perivascular spaces. They are often seen at the level of the anterior commissure.*
![Axial FLAIR MR in the same patient demonstrates complete suppression of central T2 signal <img src='img/arrows/CS.png'/>. While most enlarged perivascular spaces have normal signal in the adjacent parenchyma, 25% have thin, FLAIR-hyperintense rim. These lesions are typically incidental but may be enlarged in certain conditions.](4035a43b-aedf-407e-8689-75592c26d201)
**Enlarged Perivascular Spaces**
*Axial FLAIR MR in the same patient demonstrates complete suppression of central T2 signal <img src='img/arrows/CS.png'/>. While most enlarged perivascular spaces have normal signal in the adjacent parenchyma, 25% have thin, FLAIR-hyperintense rim. These lesions are typically incidental but may be enlarged in certain conditions.*
![Coronal T1 C+ MR shows a nonenhancing, multiloculated cystic structure <img src='img/arrows/CO.png'/> in left midbrain, thalamus, and medial temporal lobe, which followed CSF signal on all pulse sequences, consistent with giant tumefactive perivascular space. Mass effect results in hydrocephalus <img src='img/arrows/CC.png'/>.](31a75cc2-ed3d-4c9c-a86b-19eca22208b8)
**Enlarged Perivascular Spaces**
*Coronal T1 C+ MR shows a nonenhancing, multiloculated cystic structure <img src='img/arrows/CO.png'/> in left midbrain, thalamus, and medial temporal lobe, which followed CSF signal on all pulse sequences, consistent with giant tumefactive perivascular space. Mass effect results in hydrocephalus <img src='img/arrows/CC.png'/>.*
![Axial FLAIR MR in a newborn shows large, fairly symmetric cystic areas of encephalomalacia <img src='img/arrows/CC.png'/> in both cerebral hemispheres with associated volume loss. Cystic encephalomalacia was a result of hypoxic-ischemic encephalopathy at birth.](images/app.statdx.com_image_thumbnail_58aaae80-61aa-4932-ac20-bd8f2108ee46_size_168_quality_85_9a852b18_20251014T200033Z.jpg)
**Encephalomalacia**
*Axial FLAIR MR in a newborn shows large, fairly symmetric cystic areas of encephalomalacia <img src='img/arrows/CC.png'/> in both cerebral hemispheres with associated volume loss. Cystic encephalomalacia was a result of hypoxic-ischemic encephalopathy at birth.*
![Axial FLAIR MR in a newborn shows large, fairly symmetric cystic areas of encephalomalacia <img src='img/arrows/CC.png'/> in both cerebral hemispheres with associated volume loss. Cystic encephalomalacia was a result of hypoxic-ischemic encephalopathy at birth.](images/app.statdx.com_image_thumbnail_58aaae80-61aa-4932-ac20-bd8f2108ee46_size_168_quality_85_a074fb03_20251014T195936Z.jpg)
**Encephalomalacia**
*Axial FLAIR MR in a newborn shows large, fairly symmetric cystic areas of encephalomalacia <img src='img/arrows/CC.png'/> in both cerebral hemispheres with associated volume loss. Cystic encephalomalacia was a result of hypoxic-ischemic encephalopathy at birth.*
![Axial FLAIR MR in a newborn shows large, fairly symmetric cystic areas of encephalomalacia <img src='img/arrows/CC.png'/> in both cerebral hemispheres with associated volume loss. Cystic encephalomalacia was a result of hypoxic-ischemic encephalopathy at birth.](images/app.statdx.com_image_thumbnail_58aaae80-61aa-4932-ac20-bd8f2108ee46_size_168_quality_85_c51ba8f8_20251014T195834Z.jpg)
**Encephalomalacia**
*Axial FLAIR MR in a newborn shows large, fairly symmetric cystic areas of encephalomalacia <img src='img/arrows/CC.png'/> in both cerebral hemispheres with associated volume loss. Cystic encephalomalacia was a result of hypoxic-ischemic encephalopathy at birth.*
![Axial T2 3D SPACE MR demonstrates subtotal right middle cerebral artery <img src='img/arrows/CO.png'/> and left posterior cerebral artery <img src='img/arrows/CC.png'/> encephalomalacia from chronic central embolic infarcts in this patient with atrial fibrillation.](e27e3d45-b8ea-421a-aa79-87e024c8ec0d)
**Encephalomalacia**
*Axial T2 3D SPACE MR demonstrates subtotal right middle cerebral artery <img src='img/arrows/CO.png'/> and left posterior cerebral artery <img src='img/arrows/CC.png'/> encephalomalacia from chronic central embolic infarcts in this patient with atrial fibrillation.*
![Axial T2 MR shows multiple T2-hyperintense lesions in the periventricular white matter <img src='img/arrows/CC.png'/> and thalami <img src='img/arrows/CS.png'/> due to remote lacunar infarcts. Lacunar infarcts in the deep gray nuclei are classically seen in patients with vascular dementia.](images/app.statdx.com_image_thumbnail_34b7f1a5-1f62-45b4-8284-a89ca851e3bb_size_168_quality_85_020a8d49_20251014T195936Z.jpg)
**Lacunar Infarction**
*Axial T2 MR shows multiple T2-hyperintense lesions in the periventricular white matter <img src='img/arrows/CC.png'/> and thalami <img src='img/arrows/CS.png'/> due to remote lacunar infarcts. Lacunar infarcts in the deep gray nuclei are classically seen in patients with vascular dementia.*
![Axial T2 MR shows multiple T2-hyperintense lesions in the periventricular white matter <img src='img/arrows/CC.png'/> and thalami <img src='img/arrows/CS.png'/> due to remote lacunar infarcts. Lacunar infarcts in the deep gray nuclei are classically seen in patients with vascular dementia.](images/app.statdx.com_image_thumbnail_34b7f1a5-1f62-45b4-8284-a89ca851e3bb_size_168_quality_85_43666b75_20251014T195834Z.jpg)
**Lacunar Infarction**
*Axial T2 MR shows multiple T2-hyperintense lesions in the periventricular white matter <img src='img/arrows/CC.png'/> and thalami <img src='img/arrows/CS.png'/> due to remote lacunar infarcts. Lacunar infarcts in the deep gray nuclei are classically seen in patients with vascular dementia.*
![Axial T2 MR shows multiple T2-hyperintense lesions in the periventricular white matter <img src='img/arrows/CC.png'/> and thalami <img src='img/arrows/CS.png'/> due to remote lacunar infarcts. Lacunar infarcts in the deep gray nuclei are classically seen in patients with vascular dementia.](images/app.statdx.com_image_thumbnail_34b7f1a5-1f62-45b4-8284-a89ca851e3bb_size_168_quality_85_8961ae18_20251014T200033Z.jpg)
**Lacunar Infarction**
*Axial T2 MR shows multiple T2-hyperintense lesions in the periventricular white matter <img src='img/arrows/CC.png'/> and thalami <img src='img/arrows/CS.png'/> due to remote lacunar infarcts. Lacunar infarcts in the deep gray nuclei are classically seen in patients with vascular dementia.*
![Axial FLAIR MR shows cystic lesions <img src='img/arrows/CS.png'/> in the right frontal and left parietal lobes. An eccentric nodule (scolex) <img src='img/arrows/CC.png'/> seen in the right frontal lesion on this image helps make the diagnosis of neurocysticercosis.](images/app.statdx.com_image_thumbnail_cdedca4f-6709-49bb-842e-39f7b17cc70c_size_168_quality_85_5cfcb5cf_20251014T195834Z.jpg)
**Neurocysticercosis**
*Axial FLAIR MR shows cystic lesions <img src='img/arrows/CS.png'/> in the right frontal and left parietal lobes. An eccentric nodule (scolex) <img src='img/arrows/CC.png'/> seen in the right frontal lesion on this image helps make the diagnosis of neurocysticercosis.*
![Axial FLAIR MR shows cystic lesions <img src='img/arrows/CS.png'/> in the right frontal and left parietal lobes. An eccentric nodule (scolex) <img src='img/arrows/CC.png'/> seen in the right frontal lesion on this image helps make the diagnosis of neurocysticercosis.](images/app.statdx.com_image_thumbnail_cdedca4f-6709-49bb-842e-39f7b17cc70c_size_168_quality_85_98287874_20251014T195936Z.jpg)
**Neurocysticercosis**
*Axial FLAIR MR shows cystic lesions <img src='img/arrows/CS.png'/> in the right frontal and left parietal lobes. An eccentric nodule (scolex) <img src='img/arrows/CC.png'/> seen in the right frontal lesion on this image helps make the diagnosis of neurocysticercosis.*
![Axial FLAIR MR shows cystic lesions <img src='img/arrows/CS.png'/> in the right frontal and left parietal lobes. An eccentric nodule (scolex) <img src='img/arrows/CC.png'/> seen in the right frontal lesion on this image helps make the diagnosis of neurocysticercosis.](images/app.statdx.com_image_thumbnail_cdedca4f-6709-49bb-842e-39f7b17cc70c_size_168_quality_85_b49650d9_20251014T200034Z.jpg)
**Neurocysticercosis**
*Axial FLAIR MR shows cystic lesions <img src='img/arrows/CS.png'/> in the right frontal and left parietal lobes. An eccentric nodule (scolex) <img src='img/arrows/CC.png'/> seen in the right frontal lesion on this image helps make the diagnosis of neurocysticercosis.*
![Axial T2 MR reveals multiple cystic lesions in the right inferior frontal region <img src='img/arrows/CC.png'/> and the left sylvian fissure <img src='img/arrows/CS.png'/> due to racemose neurocysticercosis.](c10125d5-0d7d-4bbd-97ce-4e9e01b72968)
**Neurocysticercosis**
*Axial T2 MR reveals multiple cystic lesions in the right inferior frontal region <img src='img/arrows/CC.png'/> and the left sylvian fissure <img src='img/arrows/CS.png'/> due to racemose neurocysticercosis.*
![Axial FLAIR MR shows a porencephalic cyst <img src='img/arrows/CO.png'/> in the right frontal lobe communicating with the right frontal horn.](images/app.statdx.com_image_thumbnail_eaf43efd-5c3e-42c9-aeac-d05e105720bf_size_168_quality_85_10e3512f_20251014T195834Z.jpg)
**Porencephalic Cyst**
*Axial FLAIR MR shows a porencephalic cyst <img src='img/arrows/CO.png'/> in the right frontal lobe communicating with the right frontal horn.*
![Axial FLAIR MR shows a porencephalic cyst <img src='img/arrows/CO.png'/> in the right frontal lobe communicating with the right frontal horn.](images/app.statdx.com_image_thumbnail_eaf43efd-5c3e-42c9-aeac-d05e105720bf_size_168_quality_85_11ef66b8_20251014T200034Z.jpg)
**Porencephalic Cyst**
*Axial FLAIR MR shows a porencephalic cyst <img src='img/arrows/CO.png'/> in the right frontal lobe communicating with the right frontal horn.*
![Axial FLAIR MR shows a porencephalic cyst <img src='img/arrows/CO.png'/> in the right frontal lobe communicating with the right frontal horn.](images/app.statdx.com_image_thumbnail_eaf43efd-5c3e-42c9-aeac-d05e105720bf_size_168_quality_85_4c78aaac_20251014T195936Z.jpg)
**Porencephalic Cyst**
*Axial FLAIR MR shows a porencephalic cyst <img src='img/arrows/CO.png'/> in the right frontal lobe communicating with the right frontal horn.*
![Axial T2 MR demonstrates a classic porencephalic cyst <img src='img/arrows/CC.png'/> in the right parietal lobe communicating with the posterior horn of the right lateral ventricle. Porencephalic cysts have CSF signal on all sequences and communicate with the ventricle &amp;/or the pial surface.](e7b930fa-4236-4455-ad4d-b1a35fe62c99)
**Porencephalic Cyst**
*Axial T2 MR demonstrates a classic porencephalic cyst <img src='img/arrows/CC.png'/> in the right parietal lobe communicating with the posterior horn of the right lateral ventricle. Porencephalic cysts have CSF signal on all sequences and communicate with the ventricle &amp;/or the pial surface.*
![Axial T1 MR in a 34-year-old patient with longstanding, severe multiple sclerosis reveals multiple hypointense foci that are low signal and almost, but not quite, CSF-like. Note the faint, hyperintense rims <img src='img/arrows/CS.png'/> that surround some of the MS plaques. The T1 hypointensity correlates with axonal destruction.](images/app.statdx.com_image_thumbnail_7d109edc-d979-4ee4-8ad1-af10032aa141_size_168_quality_85_670b2787_20251014T200034Z.jpg)
**Multiple Sclerosis**
*Axial T1 MR in a 34-year-old patient with longstanding, severe multiple sclerosis reveals multiple hypointense foci that are low signal and almost, but not quite, CSF-like. Note the faint, hyperintense rims <img src='img/arrows/CS.png'/> that surround some of the MS plaques. The T1 hypointensity correlates with axonal destruction.*
![Axial T1 MR in a 34-year-old patient with longstanding, severe multiple sclerosis reveals multiple hypointense foci that are low signal and almost, but not quite, CSF-like. Note the faint, hyperintense rims <img src='img/arrows/CS.png'/> that surround some of the MS plaques. The T1 hypointensity correlates with axonal destruction.](images/app.statdx.com_image_thumbnail_7d109edc-d979-4ee4-8ad1-af10032aa141_size_168_quality_85_8418b0a7_20251014T195834Z.jpg)
**Multiple Sclerosis**
*Axial T1 MR in a 34-year-old patient with longstanding, severe multiple sclerosis reveals multiple hypointense foci that are low signal and almost, but not quite, CSF-like. Note the faint, hyperintense rims <img src='img/arrows/CS.png'/> that surround some of the MS plaques. The T1 hypointensity correlates with axonal destruction.*
![Axial T1 MR in a 34-year-old patient with longstanding, severe multiple sclerosis reveals multiple hypointense foci that are low signal and almost, but not quite, CSF-like. Note the faint, hyperintense rims <img src='img/arrows/CS.png'/> that surround some of the MS plaques. The T1 hypointensity correlates with axonal destruction.](images/app.statdx.com_image_thumbnail_7d109edc-d979-4ee4-8ad1-af10032aa141_size_168_quality_85_c262f651_20251014T195936Z.jpg)
**Multiple Sclerosis**
*Axial T1 MR in a 34-year-old patient with longstanding, severe multiple sclerosis reveals multiple hypointense foci that are low signal and almost, but not quite, CSF-like. Note the faint, hyperintense rims <img src='img/arrows/CS.png'/> that surround some of the MS plaques. The T1 hypointensity correlates with axonal destruction.*
![Axial T2 MR in the same patient shows that some lesions have CSF signal intensity <img src='img/arrows/CO.png'/>. Several others are hyperintense to brain parenchyma <img src='img/arrows/CC.png'/> but clearly do not resemble CSF in the lateral ventricles.](ddc176fa-fdac-46db-b8bc-33016ab41eae)
**Multiple Sclerosis**
*Axial T2 MR in the same patient shows that some lesions have CSF signal intensity <img src='img/arrows/CO.png'/>. Several others are hyperintense to brain parenchyma <img src='img/arrows/CC.png'/> but clearly do not resemble CSF in the lateral ventricles.*
![Axial T2 MR shows multiple CSF-like cysts in both hippocampi <img src='img/arrows/CS.png'/> just medial to the temporal horns of the lateral ventricles.](images/app.statdx.com_image_thumbnail_aa5214e9-ccca-4dbe-9603-0074716f12e8_size_168_quality_85_12d02f22_20251014T195936Z.jpg)
**Hippocampal Sulcus Remnants**
*Axial T2 MR shows multiple CSF-like cysts in both hippocampi <img src='img/arrows/CS.png'/> just medial to the temporal horns of the lateral ventricles.*
![Axial T2 MR shows multiple CSF-like cysts in both hippocampi <img src='img/arrows/CS.png'/> just medial to the temporal horns of the lateral ventricles.](images/app.statdx.com_image_thumbnail_aa5214e9-ccca-4dbe-9603-0074716f12e8_size_168_quality_85_deda105a_20251014T195834Z.jpg)
**Hippocampal Sulcus Remnants**
*Axial T2 MR shows multiple CSF-like cysts in both hippocampi <img src='img/arrows/CS.png'/> just medial to the temporal horns of the lateral ventricles.*
![Axial T2 MR shows multiple CSF-like cysts in both hippocampi <img src='img/arrows/CS.png'/> just medial to the temporal horns of the lateral ventricles.](images/app.statdx.com_image_thumbnail_aa5214e9-ccca-4dbe-9603-0074716f12e8_size_168_quality_85_eea7ec04_20251014T200034Z.jpg)
**Hippocampal Sulcus Remnants**
*Axial T2 MR shows multiple CSF-like cysts in both hippocampi <img src='img/arrows/CS.png'/> just medial to the temporal horns of the lateral ventricles.*
![Coronal T2 MR reveals tiny cysts <img src='img/arrows/CC.png'/> in the hippocampi bilaterally, classic for hippocampal sulcus remnant cysts, which are incidental findings.](703be719-a896-4c5f-b763-ae0d82638cb5)
**Hippocampal Sulcus Remnants**
*Coronal T2 MR reveals tiny cysts <img src='img/arrows/CC.png'/> in the hippocampi bilaterally, classic for hippocampal sulcus remnant cysts, which are incidental findings.*
![Coronal ultrasound in a 1-day-old premature infant shows a CSF-like intra- or periventricular cyst <img src='img/arrows/CS.png'/> with a tiny strand of tissue <img src='img/arrows/CO.png'/> that connects the walls of anterior horn.](images/app.statdx.com_image_thumbnail_00fbe173-03e1-4a59-9d6f-380bb1eb7dc7_size_168_quality_85_004f1471_20251014T195834Z.jpg)
**Connatal Cysts**
*Coronal ultrasound in a 1-day-old premature infant shows a CSF-like intra- or periventricular cyst <img src='img/arrows/CS.png'/> with a tiny strand of tissue <img src='img/arrows/CO.png'/> that connects the walls of anterior horn.*
![Coronal ultrasound in a 1-day-old premature infant shows a CSF-like intra- or periventricular cyst <img src='img/arrows/CS.png'/> with a tiny strand of tissue <img src='img/arrows/CO.png'/> that connects the walls of anterior horn.](images/app.statdx.com_image_thumbnail_00fbe173-03e1-4a59-9d6f-380bb1eb7dc7_size_168_quality_85_2354f160_20251014T195936Z.jpg)
**Connatal Cysts**
*Coronal ultrasound in a 1-day-old premature infant shows a CSF-like intra- or periventricular cyst <img src='img/arrows/CS.png'/> with a tiny strand of tissue <img src='img/arrows/CO.png'/> that connects the walls of anterior horn.*
![Coronal ultrasound in a 1-day-old premature infant shows a CSF-like intra- or periventricular cyst <img src='img/arrows/CS.png'/> with a tiny strand of tissue <img src='img/arrows/CO.png'/> that connects the walls of anterior horn.](images/app.statdx.com_image_thumbnail_00fbe173-03e1-4a59-9d6f-380bb1eb7dc7_size_168_quality_85_3966b6bc_20251014T200034Z.jpg)
**Connatal Cysts**
*Coronal ultrasound in a 1-day-old premature infant shows a CSF-like intra- or periventricular cyst <img src='img/arrows/CS.png'/> with a tiny strand of tissue <img src='img/arrows/CO.png'/> that connects the walls of anterior horn.*
![Axial T2 MR demonstrates connatal cysts surrounded by mild hyperintensity <img src='img/arrows/CO.png'/>. Whether these are connatal/germinolytic cysts persisting into adulthood or neuroglial cyst is uncertain. Regardless of etiology, such asymptomatic cysts are benign and typically nonprogressive.](e4dab000-aa65-453c-bf64-7ac7d37751a4)
**Connatal Cysts**
*Axial T2 MR demonstrates connatal cysts surrounded by mild hyperintensity <img src='img/arrows/CO.png'/>. Whether these are connatal/germinolytic cysts persisting into adulthood or neuroglial cyst is uncertain. Regardless of etiology, such asymptomatic cysts are benign and typically nonprogressive.*
![Axial T2 MR shows a large, well-defined cyst <img src='img/arrows/CO.png'/> in the left posterior frontal region. The cyst showed CSF signal on all sequences, which is typical for a neuroglial cyst.](images/app.statdx.com_image_thumbnail_d45ef797-9590-40f6-8954-f778da37830d_size_168_quality_85_bafd32b3_20251014T195837Z.jpg)
**Neuroglial Cyst**
*Axial T2 MR shows a large, well-defined cyst <img src='img/arrows/CO.png'/> in the left posterior frontal region. The cyst showed CSF signal on all sequences, which is typical for a neuroglial cyst.*
![Axial T2 MR shows a large, well-defined cyst <img src='img/arrows/CO.png'/> in the left posterior frontal region. The cyst showed CSF signal on all sequences, which is typical for a neuroglial cyst.](images/app.statdx.com_image_thumbnail_d45ef797-9590-40f6-8954-f778da37830d_size_168_quality_85_de916308_20251014T200034Z.jpg)
**Neuroglial Cyst**
*Axial T2 MR shows a large, well-defined cyst <img src='img/arrows/CO.png'/> in the left posterior frontal region. The cyst showed CSF signal on all sequences, which is typical for a neuroglial cyst.*
![Axial T1 MR demonstrates a well-defined cyst <img src='img/arrows/CC.png'/> in the left choroidal fissure, a typical location for a neuroglial cyst/choroidal fissure cyst.](d630fc96-c277-4826-8f5a-371cde0b20d8)
**Neuroglial Cyst**
*Axial T1 MR demonstrates a well-defined cyst <img src='img/arrows/CC.png'/> in the left choroidal fissure, a typical location for a neuroglial cyst/choroidal fissure cyst.*
![Axial T2 MR in a patient with HIV/AIDS shows several hyperintense cystic areas representing dilated perivascular spaces <img src='img/arrows/CO.png'/> from cryptococcosis. Fungi and gelatinous material collect within the spaces. There is typically little to no enhancement following contrast administration.](images/app.statdx.com_image_thumbnail_352b32f0-b55a-4ae4-94ed-f5af511da5b2_size_168_quality_85_69937a6b_20251014T195838Z.jpg)
**Cryptococcosis**
*Axial T2 MR in a patient with HIV/AIDS shows several hyperintense cystic areas representing dilated perivascular spaces <img src='img/arrows/CO.png'/> from cryptococcosis. Fungi and gelatinous material collect within the spaces. There is typically little to no enhancement following contrast administration.*
![Axial T2 MR in a patient with HIV/AIDS shows several hyperintense cystic areas representing dilated perivascular spaces <img src='img/arrows/CO.png'/> from cryptococcosis. Fungi and gelatinous material collect within the spaces. There is typically little to no enhancement following contrast administration.](images/app.statdx.com_image_thumbnail_352b32f0-b55a-4ae4-94ed-f5af511da5b2_size_168_quality_85_a08524b5_20251014T200034Z.jpg)
**Cryptococcosis**
*Axial T2 MR in a patient with HIV/AIDS shows several hyperintense cystic areas representing dilated perivascular spaces <img src='img/arrows/CO.png'/> from cryptococcosis. Fungi and gelatinous material collect within the spaces. There is typically little to no enhancement following contrast administration.*
![Axial CECT reveals a unilocular cyst <img src='img/arrows/CS.png'/> in the right cerebral hemisphere with no surrounding edema or enhancement, typical of echinococcus (hydatid disease).](images/app.statdx.com_image_thumbnail_f75d16d5-7b0f-40c8-b459-b8c1cd475479_size_168_quality_85_b2f1b82d_20251014T200034Z.jpg)
**Parasites, Miscellaneous**
*Axial CECT reveals a unilocular cyst <img src='img/arrows/CS.png'/> in the right cerebral hemisphere with no surrounding edema or enhancement, typical of echinococcus (hydatid disease).*
![Axial CECT reveals a unilocular cyst <img src='img/arrows/CS.png'/> in the right cerebral hemisphere with no surrounding edema or enhancement, typical of echinococcus (hydatid disease).](images/app.statdx.com_image_thumbnail_f75d16d5-7b0f-40c8-b459-b8c1cd475479_size_168_quality_85_ef03b0c0_20251014T195838Z.jpg)
**Parasites, Miscellaneous**
*Axial CECT reveals a unilocular cyst <img src='img/arrows/CS.png'/> in the right cerebral hemisphere with no surrounding edema or enhancement, typical of echinococcus (hydatid disease).*
![Axial T1 MR shows multiple enlarged perivascular spaces <img src='img/arrows/CC.png'/> in this young child with mucopolysaccharidoses 1H and minimal neurological symptoms. Note severe peritrigonal, callosal involvement.](images/app.statdx.com_image_thumbnail_7ac27a57-27b3-4a9c-aa53-8d875d1d3260_size_168_quality_85_b35745d1_20251014T200034Z.jpg)
**Mucopolysaccharidoses**
*Axial T1 MR shows multiple enlarged perivascular spaces <img src='img/arrows/CC.png'/> in this young child with mucopolysaccharidoses 1H and minimal neurological symptoms. Note severe peritrigonal, callosal involvement.*
![Axial FLAIR MR demonstrates 3 findings typical of mucopolysaccharidosis: CSF-like dilated perivascular spaces filled with mucopolysaccharides <img src='img/arrows/WS.png'/>, hyperintense white matter, and global atrophy.](0c8008ea-97fe-44dc-8990-3294c067fab7)
**Mucopolysaccharidoses**
*Axial FLAIR MR demonstrates 3 findings typical of mucopolysaccharidosis: CSF-like dilated perivascular spaces filled with mucopolysaccharides <img src='img/arrows/WS.png'/>, hyperintense white matter, and global atrophy.*
![Axial T2 MR in an infant with congenital CMV infection shows multiple periventricular germinolytic cysts <img src='img/arrows/CS.png'/>.](images/app.statdx.com_image_thumbnail_334c5586-4ee5-42fa-b1ff-6cbc0d1f80a6_size_168_quality_85_e9742b15_20251014T200034Z.jpg)
**Germinolytic Cysts**
*Axial T2 MR in an infant with congenital CMV infection shows multiple periventricular germinolytic cysts <img src='img/arrows/CS.png'/>.*
![Coronal FLAIR MR in a patient with infantile Refsum disease reveals bilateral periventricular germinolytic cysts <img src='img/arrows/CC.png'/> mimicking Zellweger syndrome. Germinolytic cysts are subependymal along a caudothalamic groove, probably resulting from germinolysis.](d9eb2759-098c-4e40-8130-dee3e3e3bf64)
**Germinolytic Cysts**
*Coronal FLAIR MR in a patient with infantile Refsum disease reveals bilateral periventricular germinolytic cysts <img src='img/arrows/CC.png'/> mimicking Zellweger syndrome. Germinolytic cysts are subependymal along a caudothalamic groove, probably resulting from germinolysis.*
![Axial T2 MR in a child with congenital muscular dystrophy shows multiple small, cystic lesions in the dysplastic cerebellum <img src='img/arrows/CS.png'/>. The pons is hypoplastic with dorsal clefting <img src='img/arrows/CC.png'/>. Hypomyelination of the temporal lobes <img src='img/arrows/CO.png'/> is present.](images/app.statdx.com_image_thumbnail_799426bc-8252-4b9e-8c24-0dbcbe949243_size_168_quality_85_7a9b10e1_20251014T200034Z.jpg)
**Miscellaneous Congenital Malformations**
*Axial T2 MR in a child with congenital muscular dystrophy shows multiple small, cystic lesions in the dysplastic cerebellum <img src='img/arrows/CS.png'/>. The pons is hypoplastic with dorsal clefting <img src='img/arrows/CC.png'/>. Hypomyelination of the temporal lobes <img src='img/arrows/CO.png'/> is present.*
![Coronal FLAIR MR in an 18-month-old infant with van der Knaap leukoencephalopathy shows cystic changes in both temporal lobes <img src='img/arrows/CS.png'/>, characteristic of this condition.](fb9374a7-63c1-4bb7-be68-25535b905357)
**Miscellaneous Congenital Malformations**
*Coronal FLAIR MR in an 18-month-old infant with van der Knaap leukoencephalopathy shows cystic changes in both temporal lobes <img src='img/arrows/CS.png'/>, characteristic of this condition.*
### Additional Images
![Axial T2 MR shows a cluster of CSF-like cysts <img src='img/arrows/CC.png'/> in the right frontal subcortical white matter.](0dc961c9-f48e-4c42-9a44-0cab01cd16bb)
**Enlarged Perivascular Spaces**
*Axial T2 MR shows a cluster of CSF-like cysts <img src='img/arrows/CC.png'/> in the right frontal subcortical white matter.*
![Axial FLAIR MR in the same patient shows suppression of the signal <img src='img/arrows/CS.png'/> within the cysts. Note subtle FLAIR hyperintensity <img src='img/arrows/CO.png'/> surrounding the cysts. Large perivascular spaces can assume a bizarre configuration and mimic a cystic neoplasm.](4550d328-4568-43c7-9d3e-9e59c88f3415)
**Enlarged Perivascular Spaces**
*Axial FLAIR MR in the same patient shows suppression of the signal <img src='img/arrows/CS.png'/> within the cysts. Note subtle FLAIR hyperintensity <img src='img/arrows/CO.png'/> surrounding the cysts. Large perivascular spaces can assume a bizarre configuration and mimic a cystic neoplasm.*
![Coronal T2 MR shows a cluster of variable-sized CSF-like cysts in left parietal subcortical white matter <img src='img/arrows/WS.png'/>. Lesions did not enhance. Follow-up scan 5 years later showed no change.](90393401-fd46-48ba-a782-35381da20868)
**Enlarged Perivascular Spaces**
*Coronal T2 MR shows a cluster of variable-sized CSF-like cysts in left parietal subcortical white matter <img src='img/arrows/WS.png'/>. Lesions did not enhance. Follow-up scan 5 years later showed no change.*
![Axial T1 MR in a patient with old left internal artery occlusion shows multicystic encephalomalacia. FLAIR, T2-weighted scans showed extensive hyperintensity in the residual parenchyma secondary to gliosis and spongiosis.](bc5ca49f-14fb-4cc6-9c76-e75f4004f911)
**Encephalomalacia**
*Axial T1 MR in a patient with old left internal artery occlusion shows multicystic encephalomalacia. FLAIR, T2-weighted scans showed extensive hyperintensity in the residual parenchyma secondary to gliosis and spongiosis.*
![Axial FLAIR MR demonstrates cystic encephalomalacia <img src='img/arrows/CC.png'/> in the right frontal lobe due to an old infarct. Note associated volume loss with prominence of the adjacent sulcal spaces.](aad6fcc3-75cf-4de6-8da5-b18424cbdf1a)
**Encephalomalacia**
*Axial FLAIR MR demonstrates cystic encephalomalacia <img src='img/arrows/CC.png'/> in the right frontal lobe due to an old infarct. Note associated volume loss with prominence of the adjacent sulcal spaces.*
![Axial T2 MR shows a cystic lesion <img src='img/arrows/CO.png'/> in the left caudate nucleus due to an old lacunar infarct. Lacunar infarcts are small, deep cerebral infarcts located in basal ganglia and thalamus, pons, or cerebral white matter, which are ≤ 15 mm in size.](f7b1b8ec-2e9f-4c37-bcc8-9c3c3aa92435)
**Lacunar Infarction**
*Axial T2 MR shows a cystic lesion <img src='img/arrows/CO.png'/> in the left caudate nucleus due to an old lacunar infarct. Lacunar infarcts are small, deep cerebral infarcts located in basal ganglia and thalamus, pons, or cerebral white matter, which are ≤ 15 mm in size.*
![Coronal T1 MR in an older adult patient with bilateral subacute subdural hematomas <img src='img/arrows/BO.png'/> shows multiple lacunar infarcts <img src='img/arrows/BS.png'/> in white matter and basal ganglia.](cf377dbd-f002-4724-98da-90ef3c44b619)
**Lacunar Infarction**
*Coronal T1 MR in an older adult patient with bilateral subacute subdural hematomas <img src='img/arrows/BO.png'/> shows multiple lacunar infarcts <img src='img/arrows/BS.png'/> in white matter and basal ganglia.*
![Axial T1 C+ MR shows several nonenhancing CSF-like cysts <img src='img/arrows/BS.png'/> of variable sizes in a patient with neurocysticercosis. Several may be cisternal, invaginating into brain. (Courtesy E. Bravo, MD.)](df7a7e09-253d-4091-837e-e5fc5c2a3a2d)
**Neurocysticercosis**
*Axial T1 C+ MR shows several nonenhancing CSF-like cysts <img src='img/arrows/BS.png'/> of variable sizes in a patient with neurocysticercosis. Several may be cisternal, invaginating into brain. (Courtesy E. Bravo, MD.)*
![Axial FLAIR MR shows a cystic lesion <img src='img/arrows/CC.png'/> in the left parietal region with an eccentric nodule <img src='img/arrows/CS.png'/> and mild perilesional edema <img src='img/arrows/CO.png'/> typical of the colloid vesicular stage of neurocysticercosis.](1d1075e8-e520-48be-b3d8-4179156e3bdc)
**Neurocysticercosis**
*Axial FLAIR MR shows a cystic lesion <img src='img/arrows/CC.png'/> in the left parietal region with an eccentric nodule <img src='img/arrows/CS.png'/> and mild perilesional edema <img src='img/arrows/CO.png'/> typical of the colloid vesicular stage of neurocysticercosis.*
![Sagittal T1 MR shows a variant case of neurocysticercosis with multiple large, well-delineated parenchymal cysts.](2da39730-0dae-4eee-8d0f-15559b978256)
**Neurocysticercosis**
*Sagittal T1 MR shows a variant case of neurocysticercosis with multiple large, well-delineated parenchymal cysts.*
![Coronal FLAIR MR shows tiny cysts <img src='img/arrows/CC.png'/> in the hippocampi bilaterally, classic for hippocampal sulcus remnant cysts.](f5a4167f-4bdc-499e-9ed2-8de5b233865b)
**Hippocampal Sulcus Remnants**
*Coronal FLAIR MR shows tiny cysts <img src='img/arrows/CC.png'/> in the hippocampi bilaterally, classic for hippocampal sulcus remnant cysts.*
![Axial FLAIR MR shows a classic porencephalic cyst <img src='img/arrows/WS.png'/> that suppresses completely on FLAIR. Some gliosis is present, seen here as a faint area of increased signal intensity <img src='img/arrows/WO.png'/>.](9837846f-dcd2-48d6-8ece-1ce3bd8f4d28)
**Porencephalic Cyst**
*Axial FLAIR MR shows a classic porencephalic cyst <img src='img/arrows/WS.png'/> that suppresses completely on FLAIR. Some gliosis is present, seen here as a faint area of increased signal intensity <img src='img/arrows/WO.png'/>.*
![Axial T1 MR shows fluid replacing a portion of the anteromedial left temporal lobe <img src='img/arrows/WS.png'/>. The cystic space communicates with the lateral ventricle <img src='img/arrows/BO.png'/> and the pial surface of the brain <img src='img/arrows/WO.png'/>.](34130468-6c39-435c-9c27-10050e8b7ff4)
**Porencephalic Cyst**
*Axial T1 MR shows fluid replacing a portion of the anteromedial left temporal lobe <img src='img/arrows/WS.png'/>. The cystic space communicates with the lateral ventricle <img src='img/arrows/BO.png'/> and the pial surface of the brain <img src='img/arrows/WO.png'/>.*
![Axial T2 MR shows an array of several tiny round and ovoid CSF-like cysts in both hippocampi <img src='img/arrows/BS.png'/>, just medial to the temporal horns of lateral ventricles. FLAIR scan (not shown) demonstrated that the cysts suppressed completely.](d002b49e-e169-4cf3-b413-d10e0d4e69e3)
**Hippocampal Sulcus Remnants**
*Axial T2 MR shows an array of several tiny round and ovoid CSF-like cysts in both hippocampi <img src='img/arrows/BS.png'/>, just medial to the temporal horns of lateral ventricles. FLAIR scan (not shown) demonstrated that the cysts suppressed completely.*
![Coronal T2 MR shows a well-defined cyst <img src='img/arrows/CC.png'/> in the right choroidal fissure, a typical location for a neuroglial cyst.](2e4e6b94-97bb-48e5-a66f-db99fd481fbc)
**Neuroglial Cyst**
*Coronal T2 MR shows a well-defined cyst <img src='img/arrows/CC.png'/> in the right choroidal fissure, a typical location for a neuroglial cyst.*
![Axial T1 MR in an asymptomatic patient shows a CSF-like cyst <img src='img/arrows/CS.png'/> adjacent to, but separated from, the left frontal horn. A smaller cyst present posteriorly <img src='img/arrows/CO.png'/>.](e491d8d1-494f-49f2-8da6-ea550ecbd784)
**Connatal Cysts**
*Axial T1 MR in an asymptomatic patient shows a CSF-like cyst <img src='img/arrows/CS.png'/> adjacent to, but separated from, the left frontal horn. A smaller cyst present posteriorly <img src='img/arrows/CO.png'/>.*
![Sagittal T2 MR in the same infant shows that the cyst <img src='img/arrows/CC.png'/> is definitely CSF-like.](ef286faf-c223-48be-9cb7-271d7de3d95d)
**Connatal Cysts**
*Sagittal T2 MR in the same infant shows that the cyst <img src='img/arrows/CC.png'/> is definitely CSF-like.*
![Sagittal T2 MR shows a variant case of neuroglial cyst <img src='img/arrows/BS.png'/> that appears to arise from the tectum, which appears stretched <img src='img/arrows/BO.png'/> around the cyst.](9bd26c99-5d0c-43af-9828-e830bef18759)
**Neuroglial Cyst**
*Sagittal T2 MR shows a variant case of neuroglial cyst <img src='img/arrows/BS.png'/> that appears to arise from the tectum, which appears stretched <img src='img/arrows/BO.png'/> around the cyst.*
![Axial FLAIR MR shows a large, cystic mass <img src='img/arrows/WS.png'/> that suppresses completely but neither enhanced nor restricted. At surgery, the cyst wall was composed of benign glial cells.](f508c815-70ab-46e7-81d4-bf6f6e2c28a1)
**Neuroglial Cyst**
*Axial FLAIR MR shows a large, cystic mass <img src='img/arrows/WS.png'/> that suppresses completely but neither enhanced nor restricted. At surgery, the cyst wall was composed of benign glial cells.*
![Axial FLAIR MR shows a small, subcortical cyst <img src='img/arrows/BS.png'/> in the left frontal lobe. The cyst suppresses completely and shows no surrounding gliosis.](c1782365-f16d-4081-80de-5da244f11c53)
**Neuroglial Cyst**
*Axial FLAIR MR shows a small, subcortical cyst <img src='img/arrows/BS.png'/> in the left frontal lobe. The cyst suppresses completely and shows no surrounding gliosis.*
![Coronal T2 MR in a child with infantile Refsum disease shows patchy hyperintensity in cerebellar <img src='img/arrows/WC.png'/> and periventricular white matter <img src='img/arrows/WO.png'/> as well as the dentate nuclei <img src='img/arrows/CS.png'/>.](98143a7c-3bdb-426d-8e59-95d0c5d9e61d)
**Germinolytic Cysts**
*Coronal T2 MR in a child with infantile Refsum disease shows patchy hyperintensity in cerebellar <img src='img/arrows/WC.png'/> and periventricular white matter <img src='img/arrows/WO.png'/> as well as the dentate nuclei <img src='img/arrows/CS.png'/>.*
![Coronal T2 MR in an infant with Zellweger syndrome shows hypomyelination, multiple small germinolytic cysts <img src='img/arrows/WS.png'/>, and polymicrogyria <img src='img/arrows/WO.png'/>.](47993f19-1912-4932-a08c-80372a61f578)
**Germinolytic Cysts**
*Coronal T2 MR in an infant with Zellweger syndrome shows hypomyelination, multiple small germinolytic cysts <img src='img/arrows/WS.png'/>, and polymicrogyria <img src='img/arrows/WO.png'/>.*
![Axial T2 MR in an infant with congenital CMV shows hyperintense germinolytic cysts <img src='img/arrows/BO.png'/> and extensive perisylvian cortical dysplasia <img src='img/arrows/WC.png'/>. Unexplained periventricular T2 hyperintensity <img src='img/arrows/BS.png'/>, periventricular cysts, and neuronal migration and organization abnormalities should suggest congenital CMV infection.](bb852ecb-4fb9-42a9-b341-bb4d9e167f8c)
**Germinolytic Cysts**
*Axial T2 MR in an infant with congenital CMV shows hyperintense germinolytic cysts <img src='img/arrows/BO.png'/> and extensive perisylvian cortical dysplasia <img src='img/arrows/WC.png'/>. Unexplained periventricular T2 hyperintensity <img src='img/arrows/BS.png'/>, periventricular cysts, and neuronal migration and organization abnormalities should suggest congenital CMV infection.*
![Axial T2 MR in the same patient shows that some lesions show CSF signal intensity <img src='img/arrows/CO.png'/>. Several others are &quot;bright&quot; <img src='img/arrows/CC.png'/> but clearly do not resemble the other lesions or CSF in the lateral ventricles.](ad4ef5d6-daf5-472a-89bf-282684e25f24)
**Multiple Sclerosis**
*Axial T2 MR in the same patient shows that some lesions show CSF signal intensity <img src='img/arrows/CO.png'/>. Several others are &quot;bright&quot; <img src='img/arrows/CC.png'/> but clearly do not resemble the other lesions or CSF in the lateral ventricles.*
![Axial T1 MR in a patient with longstanding multiple sclerosis reveals multiple hypointense foci that are almost (but not quite) CSF-like. Note the faint hyperintense rims <img src='img/arrows/CS.png'/> that surround plaques.](86d6e4f3-3179-4a80-8fea-879ff15db1fc)
**Multiple Sclerosis**
*Axial T1 MR in a patient with longstanding multiple sclerosis reveals multiple hypointense foci that are almost (but not quite) CSF-like. Note the faint hyperintense rims <img src='img/arrows/CS.png'/> that surround plaques.*
docs_md/articles/cyst-with-nodule_6cb71737-f574-4121-a8fb-02eeada9f9f7.md
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---
title: "Cyst With Nodule"
docid: "6cb71737-f574-4121-a8fb-02eeada9f9f7"
authors:
- key: "4b6589b0-9b8d-4467-8a90-01a0a59742fc"
value: "Troy A. Hutchins, MD"
- key: "8d5254e9-8dda-478b-8f08-bdee97a32c79"
value: "Karen L. Salzman, MD, FACR"
breadcrumbs:
-
name: "Brain"
slug: "brain"
treeNodeId: "6d8829f1-14d7-45af-8675-255189aa526a"
-
name: "Differential Diagnosis"
slug: "differential-diagnosis"
treeNodeId: "a7fdd139-664e-4bb8-8d18-400e4733ff60"
-
name: "Brain Parenchyma, General"
slug: "brain-parenchyma-general"
treeNodeId: "e79be97b-28c0-4023-be87-334c0579d35d"
-
name: "Generic Imaging Patterns"
slug: "generic-imaging-patterns"
treeNodeId: "66ab9cf6-74ad-42b7-a40a-4b6224edaa25"
-
name: "Cyst With Nodule"
slug: "cyst-with-nodule"
treeNodeId: null
category: "Brain"
documentVersionId: "a8a6b610-83a2-461f-bc34-b34af327c00c"
imageCount: 35
lastUpdated: "03/14/23"
pageDescription: "Cyst With Nodule"
pageKeywords: "Brain, Differential Diagnosis, Brain Parenchyma, General, Generic Imaging Patterns, Cyst With Nodule"
pageTitle: "Cyst With Nodule | STATdx"
enhancedTitle: "Cyst With Nodule"
type: "DDX"
references: true
breadcrumbs:
- "Brain"
- "Differential Diagnosis"
- "Brain Parenchyma, General"
- "Generic Imaging Patterns"
- "Cyst With Nodule"
---
# ESSENTIAL INFORMATION
- ## Key Differential Diagnosis Issues
- Cystic lesions with solid nodular components can be divided into 2 categories
- Lesions that typically demonstrate cyst with nodule morphology
- Neurocysticercosis (NCC), pilocytic astrocytoma, ganglioglioma, hemangioblastoma, pleomorphic xanthoastrocytoma (PXA), desmoplastic infantile ganglioglioma (DIG), intraparenchymal schwannoma
- Lesions that may demonstrate cyst with nodule morphology
- Metastases, glioblastoma (GBM), abscess, toxoplasmosis, parasites, dysplastic neuroepithelial tumor (DNET), thrombosed arteriovenous malformation (AVM), supratentorial ependymoma
- Although metastases, abscesses, & GBMs do not classically present as "cysts with nodules," they are included because of their overall prevalence
- Statistically, atypical form of these common diseases may be more likely than some of other "classic" cysts with nodule lesions
- ## Helpful Clues for Common Diagnoses
- **Neurocysticercosis**
- Intracranial parasitic infection caused by pork tapeworm *Taenia solium*
- Cyst with "dot" inside representing scolex
- Imaging appearance varies with stage; increased enhancement & edema when organism dies (inflammatory host response)
- Location: Convexity subarachnoid space > > cisterns > parenchyma > ventricles
- Lesions may be at different stages in same patient
- **Pilocytic Astrocytoma**
- Cerebellar cystic mass with mural nodule in child; rarely supratentorial
- T1 C+ MR: Nodule shows intense but heterogeneous enhancement
- Cyst wall may show enhancement
- T1 & T2 MR: Cyst content iso- to hyperintense to CSF
- Most common brain tumor in children
- **Ganglioglioma**
- Cortically based, slow-growing, enhancing mass in older child or young adult
- Circumscribed cyst with mural nodule most common
- May be solid and appear well circumscribed
- Often expands cortex; calcification common
- Most common tumor to cause temporal lobe epilepsy
- Cortical dysplasia is commonly associated
- **Hemangioblastoma**
- Vascular neoplasm of uncertain etiology
- Parenchymal posterior fossa cyst with nodule mass in adult
- T1 C+ MR: Nodule abuts pial surface & shows intense, homogeneous enhancement
- Prominent flow voids may be seen
- Multiple in von Hippel-Lindau syndrome (VHL) (25-40% of hemangioblastomas)
- ## Helpful Clues for Less Common Diagnoses
- **Metastases, Parenchymal**
- Discrete, gray-white interface mass(es) with adjacent vasogenic edema
- Multiplicity, history of primary malignancy helpful if present
- Solitary metastasis may mimic GBM
- Often known history of primary neoplasm
- **Glioblastoma, IDH-Wildtype**
- Malignant white matter mass with central necrosis
- Predilection to spread across midline along corpus callosum; "butterfly glioma"
- T1 C+ MR: Thick, irregular, nodular, enhancing margins
- T2/FLAIR MR: Surrounding hyperintensity & mass effect reflect edema + infiltrative tumor
- **Pleomorphic Xanthoastrocytoma**
- Cortically based cyst + nodule ± involvement of adjacent meninges
- T1 C+ MR
- Enhancing nodule
- Thickening, enhancement of adjacent meninges
- 70% have dural tail
- Temporal lobe predominance; young adult
- Maybe associated with cortical dysplasia
- **Abscess**
- T2 MR: Hypointense rim with surrounding edema classic
- T1 C+ MR: Enhancing capsule thinnest at ventricular side
- DWI MR: Cystic component bright (diffusion restriction)
- SWI MR: Dual rim sign (hypointense outside, hyperintense inside )
- **Opportunistic Infection, AIDS, Toxoplasmosis**
- Caused by parasite *Toxoplasma gondii*
- Toxoplasmosis: Ring-enhancing lesion containing eccentric nodule = eccentric target sign specific but not sensitive
- Location: Basal ganglia > hemispheres
- Clinical: Immunocompromised patient
- **Parasites, Miscellaneous**
- Multiple enhancing lesions typical
- May mimic brain tumor
- Travel history critical
- **Dysplastic Neuroepithelial Tumor**
- Bubbly, wedge-shaped, cortically based mass "points" toward lateral ventricle
- T2 MR: Very hyperintense; nodular, septate; no surrounding edema
- FLAIR MR: Hyperintense ring sign
- Thin rim of well-defined peritumoral hyperintensity separating it from surrounding normal brain
- T1 C+ MR: No to minimal enhancement; may be nodular
- Temporal lobe predominance
- ## Helpful Clues for Rare Diagnoses
- **Desmoplastic Infantile Ganglioglioma**
- Supratentorial cystic/nodular mass with dominance of cyst
- Cortically based nodule with intense enhancement & dural tail
- May be massive
- Peak age: 3-6 months
- **Schwannoma, Intraparenchymal**
- Only 1-2% of schwannomas are parenchymal
- Cyst with strongly enhancing nodule
- **Arteriovenous Malformation**
- When hemorrhagic with partial or complete thrombosis, may present as cyst with nodule
- Blood breakdown products of various ages; fluid-fluid levels
- **Ependymoma, Supratentorial**
- 40% of supratentorial ependymomas are extraventricular
- Large, complex, mixed solid/cystic mass
- Calcification, intratumoral hemorrhage common
- Moderate but inhomogeneous enhancement
- **Meningioma (Cystic)**
- Meningioma with intraparenchymal cyst may mimic cyst + nodule mass
- **Rosette-Forming Glioneuronal Tumor**
- Rare, slowly growing benign tumor of young adults
- 4th ventricle most common site > cerebellum
- Mixed solid-cystic appearance, variable Ca⁺⁺, hemorrhage
- May show cyst with nodule configuration
- **Papillary Glioneuronal Tumor**
- Temporal lobe predilection
- Parenchymal mass with solid, cystic, or cyst/mural nodule architecture
- May show calcification
- Imaging may be indistinguishable from ganglioglioma
- ## Alternative Differential Approaches
- By location
- Posterior fossa: Pilocytic astrocytoma, hemangioblastoma, metastasis, Rosette-forming glioneuronal tumor
- Temporal lobe: Ganglioglioma, PXA, DNET, papillary glioneuronal tumor
- Gray-white junction: Metastases, abscess
- Hemispheric: NCC, metastases, GBM, infections, DIG, AVM, supratentorial ependymoma
- Patient age
- Child & young adult: Pilocytic astrocytoma, ganglioglioma, PXA, DNET
- Adult: Hemangioblastoma, GBM, metastases
- Any age: NCC, abscess, other infections
- Multiple lesions
- Metastases (50-55%), NCC (50-70%), hemangioblastoma (VHL), abscesses (septic emboli), toxoplasmosis, parasites
## References
# Selected References
1. [Sotoudeh H et al: Radiomics for differentiation of the posterior fossa pilocytic astrocytoma versus hemangioblastomas in adults. A pilot study. Clin Imaging. 93:26-30, 2022](http://www.ncbi.nlm.nih.gov/pubmed/?term=36370592%5Bpmid%5D)
1. [Medhi G et al: Imaging features of rosette-forming glioneuronal tumours (RGNTs): a series of seven cases. Eur Radiol. 26(1):262-70, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=26017735%5Bpmid%5D)
1. [Carangelo B et al: Papillary glioneuronal tumor: case report and review of literature. G Chir. 36(2):63-9, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=26017104%5Bpmid%5D)
1. [Raz E et al: Cyst with a mural nodule tumor of the brain. Cancer Imaging. 12:237-44, 2012](http://www.ncbi.nlm.nih.gov/pubmed/?term=22935908%5Bpmid%5D)
1. [Kumar GG et al: Eccentric target sign in cerebral toxoplasmosis: neuropathological correlate to the imaging feature. J Magn Reson Imaging. 31(6):1469-72, 2010](http://www.ncbi.nlm.nih.gov/pubmed/?term=20512900%5Bpmid%5D)
1. [Smirniotopoulos JG et al: Patterns of contrast enhancement in the brain and meninges. Radiographics. Mar-Apr;27(2):525-51, 2007](http://www.ncbi.nlm.nih.gov/pubmed/?term=17374867%5Bpmid%5D)
## Images
### Selected Images
![Axial FLAIR MR demonstrates a neurocysticercosis cyst <img src='img/arrows/WC.png'/> with an eccentric nodule (scolex) <img src='img/arrows/WS.png'/>. Additional lesions have surrounding edema <img src='img/arrows/WO.png'/>. Edema and enhancement vary with the stage of neurocysticercosis and host response.](images/app.statdx.com_image_thumbnail_e25e1c8a-0345-4b38-89ff-714d2a60ffcc_size_168_quality_85_5bce5347_20251014T195847Z.jpg)
**Neurocysticercosis**
*Axial FLAIR MR demonstrates a neurocysticercosis cyst <img src='img/arrows/WC.png'/> with an eccentric nodule (scolex) <img src='img/arrows/WS.png'/>. Additional lesions have surrounding edema <img src='img/arrows/WO.png'/>. Edema and enhancement vary with the stage of neurocysticercosis and host response.*
![Axial FLAIR MR demonstrates a neurocysticercosis cyst <img src='img/arrows/WC.png'/> with an eccentric nodule (scolex) <img src='img/arrows/WS.png'/>. Additional lesions have surrounding edema <img src='img/arrows/WO.png'/>. Edema and enhancement vary with the stage of neurocysticercosis and host response.](images/app.statdx.com_image_thumbnail_e25e1c8a-0345-4b38-89ff-714d2a60ffcc_size_174_quality_85_e6f7bdfc.jpg)
**Neurocysticercosis**
*Axial FLAIR MR demonstrates a neurocysticercosis cyst <img src='img/arrows/WC.png'/> with an eccentric nodule (scolex) <img src='img/arrows/WS.png'/>. Additional lesions have surrounding edema <img src='img/arrows/WO.png'/>. Edema and enhancement vary with the stage of neurocysticercosis and host response.*
![Axial T1 C+ SPGR MR shows a pilocytic astrocytoma with enhancing, eccentric nodule <img src='img/arrows/WO.png'/>. There is cyst wall enhancement <img src='img/arrows/WC.png'/> (present in ~ 50% of cases). Note obstructive hydrocephalus with dilation of superior 4th ventricle <img src='img/arrows/WS.png'/> secondary to mass effect.](images/app.statdx.com_image_thumbnail_669436e9-c162-487b-9b93-b73e1d741507_size_168_quality_85_9fb4d56a_20251014T195847Z.jpg)
**Pilocytic Astrocytoma**
*Axial T1 C+ SPGR MR shows a pilocytic astrocytoma with enhancing, eccentric nodule <img src='img/arrows/WO.png'/>. There is cyst wall enhancement <img src='img/arrows/WC.png'/> (present in ~ 50% of cases). Note obstructive hydrocephalus with dilation of superior 4th ventricle <img src='img/arrows/WS.png'/> secondary to mass effect.*
![Coronal T1 C+ MR in a young adult patient with epilepsy shows a cystic and solid mass in the temporal lobe with intense enhancement of the solid mural nodule <img src='img/arrows/WS.png'/>. Gangliogliomas are the most common tumor to cause temporal lobe epilepsy.](images/app.statdx.com_image_thumbnail_9e7dea7d-06a5-4b81-b111-1353d1de1493_size_168_quality_85_62bcf559_20251014T195847Z.jpg)
**Ganglioglioma**
*Coronal T1 C+ MR in a young adult patient with epilepsy shows a cystic and solid mass in the temporal lobe with intense enhancement of the solid mural nodule <img src='img/arrows/WS.png'/>. Gangliogliomas are the most common tumor to cause temporal lobe epilepsy.*
![Coronal T1 C+ MR in an adult patient reveals a cystic mass with an intensely enhancing mural nodule <img src='img/arrows/WC.png'/> in the posterior fossa. Additional enhancing nodules <img src='img/arrows/WS.png'/> are noted in this patient with von Hippel-Lindau.](images/app.statdx.com_image_thumbnail_13712527-4c9d-4a54-8943-4f94b1e06cdf_size_168_quality_85_d9fd02f4_20251014T195847Z.jpg)
**Hemangioblastoma**
*Coronal T1 C+ MR in an adult patient reveals a cystic mass with an intensely enhancing mural nodule <img src='img/arrows/WC.png'/> in the posterior fossa. Additional enhancing nodules <img src='img/arrows/WS.png'/> are noted in this patient with von Hippel-Lindau.*
![Coronal T1 C+ MR in a patient with primary malignancy demonstrates a cystic and solid, enhancing, nodular mass <img src='img/arrows/WO.png'/> with the surrounding vasogenic edema <img src='img/arrows/WC.png'/>. Multiple additional lesions <img src='img/arrows/WS.png'/> and clinical history can help with diagnosis.](images/app.statdx.com_image_thumbnail_19b7cc9a-b952-43fe-8744-cd40c3191251_size_168_quality_85_d36899b7_20251014T195847Z.jpg)
**Metastases, Parenchymal**
*Coronal T1 C+ MR in a patient with primary malignancy demonstrates a cystic and solid, enhancing, nodular mass <img src='img/arrows/WO.png'/> with the surrounding vasogenic edema <img src='img/arrows/WC.png'/>. Multiple additional lesions <img src='img/arrows/WS.png'/> and clinical history can help with diagnosis.*
![Axial T1 C+ FS MR shows an infiltrative left frontal lobe glioblastoma with cystic <img src='img/arrows/WS.png'/> and heterogeneously enhancing, solid, nodular <img src='img/arrows/WC.png'/> components. Glioblastomas have a predilection for crossing midline through the corpus callosum.](images/app.statdx.com_image_thumbnail_d55a5ddf-3c7b-4226-b932-f7b983c18125_size_168_quality_85_a50dc4a2_20251014T195847Z.jpg)
**Glioblastoma, IDH-Wildtype**
*Axial T1 C+ FS MR shows an infiltrative left frontal lobe glioblastoma with cystic <img src='img/arrows/WS.png'/> and heterogeneously enhancing, solid, nodular <img src='img/arrows/WC.png'/> components. Glioblastomas have a predilection for crossing midline through the corpus callosum.*
![Coronal T1 C+ MR shows a cortically based left temporal lobe cystic mass <img src='img/arrows/CO.png'/> with an enhancing nodule <img src='img/arrows/CC.png'/> in a young adult. Enhancement &amp; thickening of the adjacent dura <img src='img/arrows/CS.png'/> help diagnose PXA &amp; differentiate from a ganglioglioma.](images/app.statdx.com_image_thumbnail_5191f79e-6206-4dc0-a3a9-1eadd8da6b52_size_168_quality_85_34d41e2d_20251014T195847Z.jpg)
**Pleomorphic Xanthoastrocytoma**
*Coronal T1 C+ MR shows a cortically based left temporal lobe cystic mass <img src='img/arrows/CO.png'/> with an enhancing nodule <img src='img/arrows/CC.png'/> in a young adult. Enhancement &amp; thickening of the adjacent dura <img src='img/arrows/CS.png'/> help diagnose PXA &amp; differentiate from a ganglioglioma.*
![Sagittal T1 C+ MR in a patient treated for sinusitis who presented with headache, fever, and seizures shows a large, ring-enhancing abscess <img src='img/arrows/CC.png'/> with an eccentric nodule <img src='img/arrows/CO.png'/>. DWI (not shown) showed characteristic diffusion restriction in the central nonenhancing component.](images/app.statdx.com_image_thumbnail_eb1f83f2-3e99-4889-9ac6-13610e0bfbd2_size_168_quality_85_8ed8dc00_20251014T195847Z.jpg)
**Abscess**
*Sagittal T1 C+ MR in a patient treated for sinusitis who presented with headache, fever, and seizures shows a large, ring-enhancing abscess <img src='img/arrows/CC.png'/> with an eccentric nodule <img src='img/arrows/CO.png'/>. DWI (not shown) showed characteristic diffusion restriction in the central nonenhancing component.*
![Axial T1 C+ FS MR in an immunocompromised patient shows a ring-enhancing lesion with peripheral enhancing nodule <img src='img/arrows/WC.png'/>. This eccentric target sign is specific but not sensitive for toxoplasmosis. Note the numerous additional nodular and ring-enhancing lesions <img src='img/arrows/WS.png'/> and surrounding edema <img src='img/arrows/WO.png'/>.](f201bfca-6b9a-4699-8490-0175f1d16393)
**Opportunistic Infection, AIDS, Toxoplasmosis**
*Axial T1 C+ FS MR in an immunocompromised patient shows a ring-enhancing lesion with peripheral enhancing nodule <img src='img/arrows/WC.png'/>. This eccentric target sign is specific but not sensitive for toxoplasmosis. Note the numerous additional nodular and ring-enhancing lesions <img src='img/arrows/WS.png'/> and surrounding edema <img src='img/arrows/WO.png'/>.*
![Axial CECT demonstrates a ring-enhancing lesion with an associated nodule <img src='img/arrows/CC.png'/> and surrounding vasogenic edema. Multiple punctate lesions <img src='img/arrows/CS.png'/> are also apparent in this patient with amebic encephalitis.](69bce238-10aa-4cf8-ac21-9a3cd75ce283)
**Parasites, Miscellaneous**
*Axial CECT demonstrates a ring-enhancing lesion with an associated nodule <img src='img/arrows/CC.png'/> and surrounding vasogenic edema. Multiple punctate lesions <img src='img/arrows/CS.png'/> are also apparent in this patient with amebic encephalitis.*
![Axial T1 C+ MR in a patient with epilepsy shows a cortically based, bubbly, cystic mass <img src='img/arrows/CC.png'/> with an eccentric, enhancing nodule <img src='img/arrows/CO.png'/>. Faint, nodular enhancement can be seen in 20% of DNETs. FLAIR images often show a well-defined, hyperintense ring surrounding the mass.](4ef2a135-3f21-4e63-9edb-64c36d4127cf)
**Dysplastic Neuroepithelial Tumor**
*Axial T1 C+ MR in a patient with epilepsy shows a cortically based, bubbly, cystic mass <img src='img/arrows/CC.png'/> with an eccentric, enhancing nodule <img src='img/arrows/CO.png'/>. Faint, nodular enhancement can be seen in 20% of DNETs. FLAIR images often show a well-defined, hyperintense ring surrounding the mass.*
![Coronal T1 C+ MR shows a large cyst <img src='img/arrows/CS.png'/> with a cortically based, intensely enhancing mural nodule <img src='img/arrows/WS.png'/> in an infant. Note the adjacent dural thickening &amp; enhancement <img src='img/arrows/CC.png'/>, typical of desmoplastic infantile ganglioglioma.](87758bde-f6d0-4dbb-8378-ae81b5efe383)
**Desmoplastic Infantile Ganglioglioma**
*Coronal T1 C+ MR shows a large cyst <img src='img/arrows/CS.png'/> with a cortically based, intensely enhancing mural nodule <img src='img/arrows/WS.png'/> in an infant. Note the adjacent dural thickening &amp; enhancement <img src='img/arrows/CC.png'/>, typical of desmoplastic infantile ganglioglioma.*
![Axial T1 C+ MR shows a cystic parenchymal mass <img src='img/arrows/CS.png'/> with an intensely enhancing mural nodule <img src='img/arrows/CO.png'/> in the right occipital lobe. Although ganglioglioma was the preoperative diagnosis, schwannoma was found on pathology.](8973cba5-cddb-4983-8b42-46e7c37e1911)
**Schwannoma, Intraparenchymal**
*Axial T1 C+ MR shows a cystic parenchymal mass <img src='img/arrows/CS.png'/> with an intensely enhancing mural nodule <img src='img/arrows/CO.png'/> in the right occipital lobe. Although ganglioglioma was the preoperative diagnosis, schwannoma was found on pathology.*
![Axial T2 MR shows a recurrent supratentorial ependymoma with a cyst <img src='img/arrows/WC.png'/> and nodule <img src='img/arrows/WS.png'/> architecture. 40% of supratentorial ependymomas are extraventricular. They are commonly associated with calcification and hemorrhage.](f4bdd4ff-e03b-49b6-88f5-f5af14854ae7)
**Ependymoma, Supratentorial**
*Axial T2 MR shows a recurrent supratentorial ependymoma with a cyst <img src='img/arrows/WC.png'/> and nodule <img src='img/arrows/WS.png'/> architecture. 40% of supratentorial ependymomas are extraventricular. They are commonly associated with calcification and hemorrhage.*
### Additional Images
![Axial FLAIR MR demonstrates a classic neurocysticercosis cyst <img src='img/arrows/CC.png'/> with an eccentric nodule (scolex) <img src='img/arrows/CS.png'/> in the right parietal lobe with surrounding vasogenic edema. The enhancement and edema varies with the stage of neurocysticercosis and host response.](46b5b236-6457-428a-8c36-68efbc201d83)
**Neurocysticercosis**
*Axial FLAIR MR demonstrates a classic neurocysticercosis cyst <img src='img/arrows/CC.png'/> with an eccentric nodule (scolex) <img src='img/arrows/CS.png'/> in the right parietal lobe with surrounding vasogenic edema. The enhancement and edema varies with the stage of neurocysticercosis and host response.*
![Axial T1WI MR shows a frontal <img src='img/arrows/BC.png'/> &amp; left lateral ventricular <img src='img/arrows/WO.png'/> &quot;cyst with dot.&quot; The &quot;dot&quot; or scolex may be T1 hyperintense <img src='img/arrows/WC.png'/>. Edema &amp; enhancement vary with stage &amp; host response.](3d10372e-9f99-4e2a-b41f-3be239a24680)
**Neurocysticercosis**
*Axial T1WI MR shows a frontal <img src='img/arrows/BC.png'/> &amp; left lateral ventricular <img src='img/arrows/WO.png'/> &quot;cyst with dot.&quot; The &quot;dot&quot; or scolex may be T1 hyperintense <img src='img/arrows/WC.png'/>. Edema &amp; enhancement vary with stage &amp; host response.*
![Axial T1 C+ MR shows a classic cerebellar pilocytic astrocytoma. There is well-defined enhancement of the cyst wall <img src='img/arrows/CS.png'/> with an enhancing eccentric nodule <img src='img/arrows/CO.png'/>. Note the mass effect on the 4th ventricle with obstructive hydrocephalus.](6e3dff21-f046-4e89-8cf6-39bb2dea1b66)
**Pilocytic Astrocytoma**
*Axial T1 C+ MR shows a classic cerebellar pilocytic astrocytoma. There is well-defined enhancement of the cyst wall <img src='img/arrows/CS.png'/> with an enhancing eccentric nodule <img src='img/arrows/CO.png'/>. Note the mass effect on the 4th ventricle with obstructive hydrocephalus.*
![Axial T1 C+ MR shows a cystic mass <img src='img/arrows/WC.png'/> with an intense, heterogeneously enhancing mural nodule <img src='img/arrows/WO.png'/> in the posterior fossa of a child. Note associated temporal horn dilatation related to the tumor.](85458799-95cd-401d-acab-f40f92a97955)
**Pilocytic Astrocytoma**
*Axial T1 C+ MR shows a cystic mass <img src='img/arrows/WC.png'/> with an intense, heterogeneously enhancing mural nodule <img src='img/arrows/WO.png'/> in the posterior fossa of a child. Note associated temporal horn dilatation related to the tumor.*
![Coronal T1 C+ MR in a child with epilepsy demonstrates a cystic and solid mass <img src='img/arrows/CO.png'/> in the temporal lobe with intense enhancement of the solid mural nodule <img src='img/arrows/CC.png'/>. Gangliogliomas commonly cause temporal lobe epilepsy.](da049feb-4df7-4fc7-a7b8-f30b39b82cd0)
**Ganglioglioma**
*Coronal T1 C+ MR in a child with epilepsy demonstrates a cystic and solid mass <img src='img/arrows/CO.png'/> in the temporal lobe with intense enhancement of the solid mural nodule <img src='img/arrows/CC.png'/>. Gangliogliomas commonly cause temporal lobe epilepsy.*
![Coronal T1 C+ MR shows a circumscribed, cystic and solid mass in the temporal lobe with intense enhancement of the solid mural nodule <img src='img/arrows/WC.png'/>. Note cortical location and lack of significant mass effect and edema. Gangliogliomas commonly cause temporal lobe epilepsy.](4b3aec05-d631-4891-8893-43c49e5ca3ff)
**Ganglioglioma**
*Coronal T1 C+ MR shows a circumscribed, cystic and solid mass in the temporal lobe with intense enhancement of the solid mural nodule <img src='img/arrows/WC.png'/>. Note cortical location and lack of significant mass effect and edema. Gangliogliomas commonly cause temporal lobe epilepsy.*
![Axial CECT in a middle-aged woman shows a cystic mass <img src='img/arrows/CC.png'/> with an intensely enhancing mural nodule <img src='img/arrows/CS.png'/> in the posterior fossa. Note extensive perilesional edema with mass effect and obstructive hydrocephalus. Histopathology revealed a hemangioblastoma.](256c1c61-164f-4320-9a15-a02b7de53932)
**Hemangioblastoma**
*Axial CECT in a middle-aged woman shows a cystic mass <img src='img/arrows/CC.png'/> with an intensely enhancing mural nodule <img src='img/arrows/CS.png'/> in the posterior fossa. Note extensive perilesional edema with mass effect and obstructive hydrocephalus. Histopathology revealed a hemangioblastoma.*
![Sagittal T1 C+ MR shows a cystic mass <img src='img/arrows/BC.png'/> with an intensely and homogeneously enhancing mural nodule <img src='img/arrows/BO.png'/> in the posterior fossa of an adult. The nodule typically abuts the pial surface.](442c4f93-fd1d-448e-8a5e-d892fb5d31f6)
**Hemangioblastoma**
*Sagittal T1 C+ MR shows a cystic mass <img src='img/arrows/BC.png'/> with an intensely and homogeneously enhancing mural nodule <img src='img/arrows/BO.png'/> in the posterior fossa of an adult. The nodule typically abuts the pial surface.*
![Axial T1 C+ MR in a patient with breast carcinoma shows multiple metastatic lesions <img src='img/arrows/CC.png'/> in the posterior fossa with a few showing a cyst and nodule morphology.](e387cccb-608b-48ca-b121-5a42079a2a4e)
**Metastases, Parenchymal**
*Axial T1 C+ MR in a patient with breast carcinoma shows multiple metastatic lesions <img src='img/arrows/CC.png'/> in the posterior fossa with a few showing a cyst and nodule morphology.*
![Coronal T1 C+ MR shows a cystic mass with a large, enhancing nodule in the cerebellar hemisphere with rim enhancement <img src='img/arrows/WS.png'/>. This is an atypical appearance for a metastasis. History of primary malignancy &amp; presence of other lesions are helpful for diagnosis.](840d7bb1-004c-46ff-9a48-3a9e7ac07e17)
**Metastases, Parenchymal**
*Coronal T1 C+ MR shows a cystic mass with a large, enhancing nodule in the cerebellar hemisphere with rim enhancement <img src='img/arrows/WS.png'/>. This is an atypical appearance for a metastasis. History of primary malignancy &amp; presence of other lesions are helpful for diagnosis.*
![Axial T1 C+ FS MR shows a &quot;cystic&quot; mass with nodular enhancement <img src='img/arrows/WC.png'/> in this patient with colon cancer. Multiple lesions and clinical history are helpful for diagnosis.](0836d29c-5066-4d06-8809-479de0b92523)
**Metastases, Parenchymal**
*Axial T1 C+ FS MR shows a &quot;cystic&quot; mass with nodular enhancement <img src='img/arrows/WC.png'/> in this patient with colon cancer. Multiple lesions and clinical history are helpful for diagnosis.*
![Coronal T1 C+ MR shows an irregularly enhancing perisylvian cystic &amp; solid mass <img src='img/arrows/WO.png'/> in this patient with multiple lesions related to metastatic disease. Note the surrounding vasogenic edema in the frontal lobe.](946b35ab-4c14-4535-89ba-5ca57aa9468e)
**Metastases, Parenchymal**
*Coronal T1 C+ MR shows an irregularly enhancing perisylvian cystic &amp; solid mass <img src='img/arrows/WO.png'/> in this patient with multiple lesions related to metastatic disease. Note the surrounding vasogenic edema in the frontal lobe.*
![Coronal T1 C+ MR shows a large, cystic mass with rim enhancement <img src='img/arrows/CS.png'/> and an intensely enhancing, eccentric nodule <img src='img/arrows/CC.png'/>. Note the mass effect with mild subfalcine herniation. Glioblastoma was the final pathology in this case.](92e72d0f-eebb-4e82-950c-0c487e416ea1)
**Glioblastoma, IDH-Wildtype**
*Coronal T1 C+ MR shows a large, cystic mass with rim enhancement <img src='img/arrows/CS.png'/> and an intensely enhancing, eccentric nodule <img src='img/arrows/CC.png'/>. Note the mass effect with mild subfalcine herniation. Glioblastoma was the final pathology in this case.*
![Axial CECT shows a heterogeneous mass <img src='img/arrows/WS.png'/> with irregular peripheral enhancement containing a nodular component <img src='img/arrows/WC.png'/>. Aggressive features help diagnose this malignant tumor.](f8fbfd56-3921-4ac9-9ac6-4224a3c0a786)
**Glioblastoma, IDH-Wildtype**
*Axial CECT shows a heterogeneous mass <img src='img/arrows/WS.png'/> with irregular peripheral enhancement containing a nodular component <img src='img/arrows/WC.png'/>. Aggressive features help diagnose this malignant tumor.*
![Axial T1 C+ FS MR demonstrates a ring-enhancing lesion with a small, enhancing mural nodule <img src='img/arrows/WC.png'/>. DWI MR (not shown) showed characteristic diffusion restriction in the central nonenhancing component.](5b57f4aa-20e9-40c9-a996-80bcf4c554cb)
**Abscess**
*Axial T1 C+ FS MR demonstrates a ring-enhancing lesion with a small, enhancing mural nodule <img src='img/arrows/WC.png'/>. DWI MR (not shown) showed characteristic diffusion restriction in the central nonenhancing component.*
![Sagittal T1 C+ MR in an HIV-positive patient shows a ring-enhancing lesion <img src='img/arrows/CC.png'/> in the parietal lobe with marked perilesional edema. Note the target appearance with central nodule <img src='img/arrows/CS.png'/>, typical of toxoplasmosis.](aed7a5cd-8d38-4b26-9bca-c2720a115338)
**Opportunistic Infection, AIDS, Toxoplasmosis**
*Sagittal T1 C+ MR in an HIV-positive patient shows a ring-enhancing lesion <img src='img/arrows/CC.png'/> in the parietal lobe with marked perilesional edema. Note the target appearance with central nodule <img src='img/arrows/CS.png'/>, typical of toxoplasmosis.*
![Coronal T1 C+ MR shows basal ganglia, thalamic, &amp; parenchymal ring-enhancing lesions <img src='img/arrows/WS.png'/> in an immunocompromised patient. Note the target appearance with a central nodule <img src='img/arrows/BC.png'/> in the right temporal lobe lesion.](201f7074-c6f4-491c-ae3c-f8d00fe27c65)
**Opportunistic Infection, AIDS, Toxoplasmosis**
*Coronal T1 C+ MR shows basal ganglia, thalamic, &amp; parenchymal ring-enhancing lesions <img src='img/arrows/WS.png'/> in an immunocompromised patient. Note the target appearance with a central nodule <img src='img/arrows/BC.png'/> in the right temporal lobe lesion.*
![Axial T2WI MR shows a wedge-shaped, extremely hyperintense, cortically based, bubbly mass <img src='img/arrows/WC.png'/> that &quot;points&quot; toward the lateral ventricle. Faint nodular enhancement can be seen in 20% of cases.](4157b6c4-d945-4092-bcf4-14636c7fdea3)
**Dysplastic Neuroepithelial Tumor**
*Axial T2WI MR shows a wedge-shaped, extremely hyperintense, cortically based, bubbly mass <img src='img/arrows/WC.png'/> that &quot;points&quot; toward the lateral ventricle. Faint nodular enhancement can be seen in 20% of cases.*
![Axial T1 C+ MR shows a left temporal lobe mass with a small focus of mild enhancement <img src='img/arrows/WS.png'/> within the bubbly, cystic mass. Faint nodular enhancement can be seen in 20% of DNETs. Lesions are typically T2 hyperintense &amp; may erode the adjacent calvarium, as in this case.](92d1827c-6180-4a6d-a500-54829dc4c5c4)
**Dysplastic Neuroepithelial Tumor**
*Axial T1 C+ MR shows a left temporal lobe mass with a small focus of mild enhancement <img src='img/arrows/WS.png'/> within the bubbly, cystic mass. Faint nodular enhancement can be seen in 20% of DNETs. Lesions are typically T2 hyperintense &amp; may erode the adjacent calvarium, as in this case.*
![Axial T2 MR shows a recurrent supratentorial ependymoma with a cyst <img src='img/arrows/CC.png'/> and nodule <img src='img/arrows/CO.png'/> architecture. NECT showed calcifications with the solid component. Note the old parietal craniotomy.](f14959a7-4f71-4081-b01d-6cdb636f1f50)
**Ependymoma, Supratentorial**
*Axial T2 MR shows a recurrent supratentorial ependymoma with a cyst <img src='img/arrows/CC.png'/> and nodule <img src='img/arrows/CO.png'/> architecture. NECT showed calcifications with the solid component. Note the old parietal craniotomy.*
![Axial CECT shows a mixed-density, cystic and solid lesion with rim enhancement <img src='img/arrows/WS.png'/>. There is a fluid-fluid level within 1 of the cysts <img src='img/arrows/WO.png'/>, representing hemorrhage in this partially thrombosed arteriovenous malformation.](bebcee77-61cd-409f-afc0-7c63f7671d0d)
**Arteriovenous Malformation**
*Axial CECT shows a mixed-density, cystic and solid lesion with rim enhancement <img src='img/arrows/WS.png'/>. There is a fluid-fluid level within 1 of the cysts <img src='img/arrows/WO.png'/>, representing hemorrhage in this partially thrombosed arteriovenous malformation.*
docs_md/articles/cystic-intrasellar-mass_e5a24511-dd27-495c-b20a-c56534351a06.md
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---
title: "Cystic Intrasellar Mass"
docid: "e5a24511-dd27-495c-b20a-c56534351a06"
authors:
- key: "8d5254e9-8dda-478b-8f08-bdee97a32c79"
value: "Karen L. Salzman, MD, FACR"
- key: "5cff4116-3654-4b3a-bb75-5ebe0b8c9850"
value: "Anne G. Osborn, MD, FACR"
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name: "Brain"
slug: "brain"
treeNodeId: "6d8829f1-14d7-45af-8675-255189aa526a"
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name: "Differential Diagnosis"
slug: "differential-diagnosis"
treeNodeId: "a7fdd139-664e-4bb8-8d18-400e4733ff60"
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name: "Sella/Juxtasellar, Pineal Region"
slug: "sellajuxtasellar-pineal-region"
treeNodeId: "5e38b9c1-3137-47e3-aa83-1fc82cb4099a"
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name: "Anatomically Based Differentials"
slug: "anatomically-based-differentials"
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name: "Cystic Intrasellar Mass"
slug: "cystic-intrasellar-mass"
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category: "Brain"
documentVersionId: "853dca59-915a-4a45-8c80-d20bbbfd9733"
imageCount: 23
lastUpdated: "01/24/23"
pageDescription: "Cystic Intrasellar Mass"
pageKeywords: "Brain, Differential Diagnosis, Sella/Juxtasellar, Pineal Region, Anatomically Based Differentials, Cystic Intrasellar Mass"
pageTitle: "Cystic Intrasellar Mass | STATdx"
enhancedTitle: "Cystic Intrasellar Mass"
type: "DDX"
references: true
breadcrumbs:
- "Brain"
- "Differential Diagnosis"
- "Sella/Juxtasellar, Pineal Region"
- "Anatomically Based Differentials"
- "Cystic Intrasellar Mass"
---
# ESSENTIAL INFORMATION
- ## Key Differential Diagnosis Issues
- Cystic mass originating **within** sella turcica vs. intrasellar extension from suprasellar lesion
- Intrasellar extension of suprasellar lesion > intrasellar mass
- ## Helpful Clues for Common Diagnoses
- **Empty Sella**
- Small crescent of compressed pituitary gland lines bottom of sella turcica
- Primary empty sella considered normal variant
- Secondary = surgery, pituitary infarction
- **Intracranial****H****ypertension, Idiopathic**
- Pseudotumor cerebri: F > > M
- Empty sella ± dilated optic nerve sheaths, small ventricles, transverse sinus/sigmoid sinus junction stenosis
- Obese female with headaches
- **Cystic Pituitary Adenoma**
- May be functional, often prolactinoma, or null cell
- Small (≤ 10 mm), enhances < gland
- ## Helpful Clues for Less Common Diagnoses
- **Rathke Cleft Cyst**
- Usually < 1 cm; may be giant, erode sella
- 40-75% have intracystic nodule
- ± claw sign (enhancing rim of pituitary around nonenhancing cyst)
- **Craniopharyngioma**
- Truly intrasellar craniopharyngioma rare
- If no Ca⁺⁺, difficult to distinguish from Rathke cleft cyst
- 90% rule (adamantinomatous): 90% calcify, 90% cystic, 90% enhance
- ## Helpful Clues for Rare Diagnoses
- **Pituitary****A****poplexy**
- Can be life threatening (secondary to pituitary insufficiency)
- Acutely may present as necrotic, rim-enhancing mass
- Typically sellar and suprasellar
- **Arachnoid Cyst**
- Truly intrasellar arachnoid cyst (AC) rare
- Usually extension from suprasellar AC
- **Neurocysticercosis Cyst**
- Suprasellar cysts → intrasellar
- May have racemose cysts in basal cisterns
- **Epidermoid Cyst**
- Suprasellar < off-midline location
- T2 hyperintense, DWI hyperintense
- **Saccular Aneurysm****(Thrombosed)**
- Medially projecting from cavernous ICA
- If thrombosed, may appear low signal intensity on T1 C+ scans
- **Par Intermedia Cyst**
- Benign cyst in between adenohypophysis and neurohypophysis
- **Obstructive Hydrocephalus (Mimic)**
- Anterior recesses of 3rd ventricle enlarge
- Herniate inferiorly into sella
- If chronic may expand, erode bony sella
## References
# Selected References
1. [Kuzan BN et al: Accuracy and reliability of magnetic resonance imaging in the diagnosis of idiopathic intracranial hypertension. Eur J Radiol. 155:110491, 2022](http://www.ncbi.nlm.nih.gov/pubmed/?term=36007323%5Bpmid%5D)
1. [Azuma M et al: Added value of contrast-enhanced 3D-FLAIR MR imaging for differentiating cystic pituitary adenoma from Rathke's cleft cyst. Magn Reson Med Sci. 20(4):404-9, 2021](http://www.ncbi.nlm.nih.gov/pubmed/?term=33487606%5Bpmid%5D)
1. [Belachew NF et al: Evolution of MRI findings in patients with idiopathic intracranial hypertension after venous sinus stenting. AJNR Am J Neuroradiol. 42(11):1993-2000, 2021](http://www.ncbi.nlm.nih.gov/pubmed/?term=34620591%5Bpmid%5D)
1. [Azuma M et al: Usefulness of contrast-enhanced 3D-FLAIR MR imaging for differentiating Rathke cleft cyst from cystic craniopharyngioma. AJNR Am J Neuroradiol. 41(1):106-10, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=31857323%5Bpmid%5D)
1. [Kuan EC et al: Treatment outcomes of Rathke's cleft cysts managed with marsupialization. J Neurol Surg B Skull Base. 78(2):112-5, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=28321372%5Bpmid%5D)
1. [Morris PP et al: Transverse sinus stenosis is the most sensitive MR imaging correlate of idiopathic intracranial hypertension. AJNR Am J Neuroradiol. 38(3):471-7, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=28104635%5Bpmid%5D)
1. [Sasagawa Y et al: Clinical characteristics of acromegalic patients with empty sella and their outcomes following transsphenoidal surgery. Pituitary. 20(4):403-8, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=28233140%5Bpmid%5D)
1. [Sivaraju L et al: Giant multi-compartmental suprasellar Rathke's cleft cyst with restriction on diffusion weighted images. Neuroradiol J. 1971400916682512, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=28059626%5Bpmid%5D)
1. [Zhang X et al: Sellar cysticercosis and septum pellucidum cyst: a case report and review of the literature. Southeast Asian J Trop Med Public Health. 45(3):584-7, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=24974642%5Bpmid%5D)
1. [Saindane AM et al: Factors determining the clinical significance of an "empty" sella turcica. AJR Am J Roentgenol. 200(5):1125-31, 2013](http://www.ncbi.nlm.nih.gov/pubmed/?term=23617499%5Bpmid%5D)
1. [Arriada-Mendicoa N et al: Imaging features of sellar cysticercosis. AJNR Am J Neuroradiol. 24(7):1386-9, 2003](http://www.ncbi.nlm.nih.gov/pubmed/?term=12917134%5Bpmid%5D)
## Images
### Selected Images
![Sagittal T1WI MR shows empty sella with herniation of CSF through the diaphragma sellae <img src='img/arrows/WS.png'/>, flattening the pituitary gland inferiorly against the sellar floor <img src='img/arrows/CO.png'/>. Empty sella may be asymptomatic or may be associated with idiopathic intracranial hypertension.](images/app.statdx.com_image_thumbnail_1d61ad1c-498f-4e58-a36f-beb4931ad4d7_annotated_true_size_900_quality_90_4bf267ba.jpg)
**Empty Sella**
*Sagittal T1WI MR shows empty sella with herniation of CSF through the diaphragma sellae <img src='img/arrows/WS.png'/>, flattening the pituitary gland inferiorly against the sellar floor <img src='img/arrows/CO.png'/>. Empty sella may be asymptomatic or may be associated with idiopathic intracranial hypertension.*
![Sagittal T1WI MR shows empty sella with herniation of CSF through the diaphragma sellae <img src='img/arrows/WS.png'/>, flattening the pituitary gland inferiorly against the sellar floor <img src='img/arrows/CO.png'/>. Empty sella may be asymptomatic or may be associated with idiopathic intracranial hypertension.](images/app.statdx.com_image_thumbnail_1d61ad1c-498f-4e58-a36f-beb4931ad4d7_size_174_quality_85_b8dbce79.jpg)
**Empty Sella**
*Sagittal T1WI MR shows empty sella with herniation of CSF through the diaphragma sellae <img src='img/arrows/WS.png'/>, flattening the pituitary gland inferiorly against the sellar floor <img src='img/arrows/CO.png'/>. Empty sella may be asymptomatic or may be associated with idiopathic intracranial hypertension.*
![Axial T2WI FS MR in a young female with headaches shows classic features of idiopathic intracranial hypertension with an empty sella <img src='img/arrows/BS.png'/>, dilatation of the optic nerve sheaths <img src='img/arrows/WO.png'/>, and prominent optic nerve heads <img src='img/arrows/CS.png'/>, related to papilledema.](images/app.statdx.com_image_thumbnail_924aa312-2d82-4ade-89db-dae8bf0287af_annotated_true_size_900_quality_90_adb22e0b.jpg)
**Intracranial Hypertension, Idiopathic**
*Axial T2WI FS MR in a young female with headaches shows classic features of idiopathic intracranial hypertension with an empty sella <img src='img/arrows/BS.png'/>, dilatation of the optic nerve sheaths <img src='img/arrows/WO.png'/>, and prominent optic nerve heads <img src='img/arrows/CS.png'/>, related to papilledema.*
![Coronal T2WI MR shows a cystic microadenoma <img src='img/arrows/CO.png'/> in the right pituitary gland. Microadenomas may be asymptomatic or may present with abnormal endocrine values, commonly elevated prolactin. When cystic, microadenomas mimic benign cysts, such as Rathke cleft cysts.](images/app.statdx.com_image_thumbnail_3b38465b-23d0-4f45-91c7-d43187cc58ae_annotated_true_size_900_quality_90_e6559b79.jpg)
**Cystic Pituitary Adenoma**
*Coronal T2WI MR shows a cystic microadenoma <img src='img/arrows/CO.png'/> in the right pituitary gland. Microadenomas may be asymptomatic or may present with abnormal endocrine values, commonly elevated prolactin. When cystic, microadenomas mimic benign cysts, such as Rathke cleft cysts.*
![Sagittal T1WI C+ MR shows an intrasellar Rathke cleft cyst <img src='img/arrows/CO.png'/> as a CSF-like mass that displaces the normal pituitary gland anteriorly <img src='img/arrows/WS.png'/>, forming a claw sign. Often, a nonenhancing, intracystic nodule may be seen.](images/app.statdx.com_image_thumbnail_e51ed243-84ae-4fe7-9e7e-154d7a07074f_annotated_true_size_900_quality_90_e73aecf9.jpg)
**Rathke Cleft Cyst**
*Sagittal T1WI C+ MR shows an intrasellar Rathke cleft cyst <img src='img/arrows/CO.png'/> as a CSF-like mass that displaces the normal pituitary gland anteriorly <img src='img/arrows/WS.png'/>, forming a claw sign. Often, a nonenhancing, intracystic nodule may be seen.*
![Sagittal T2WI MR shows a cystic sellar and suprasellar mass <img src='img/arrows/CO.png'/>.Calcification and enhancement help differentiate craniopharyngioma from Rathke cleft cyst. Only 5% of craniopharyngiomas are purely intrasellar.](images/app.statdx.com_image_thumbnail_d326a5b9-1981-4eb3-b8f9-3368600d0f06_annotated_true_size_900_quality_90_45e31d15.jpg)
**Craniopharyngioma**
*Sagittal T2WI MR shows a cystic sellar and suprasellar mass <img src='img/arrows/CO.png'/>.Calcification and enhancement help differentiate craniopharyngioma from Rathke cleft cyst. Only 5% of craniopharyngiomas are purely intrasellar.*
![Coronal T2WI MR shows a cystic sellar and suprasellar mass <img src='img/arrows/CS.png'/> in a 40-year-old male patient with acute onset of headache and cranial neuropathy. Pituitary apoplexy may result from hemorrhage or infarction of a macroadenoma, as in this patient.](1769c427-7f0d-487c-8671-6c12ae3c3996)
**Pituitary Apoplexy**
*Coronal T2WI MR shows a cystic sellar and suprasellar mass <img src='img/arrows/CS.png'/> in a 40-year-old male patient with acute onset of headache and cranial neuropathy. Pituitary apoplexy may result from hemorrhage or infarction of a macroadenoma, as in this patient.*
![Coronal T1WI C+ MR shows a sellar and suprasellar arachnoid cyst <img src='img/arrows/CO.png'/> with leftward displacement of the pituitary infundibulum <img src='img/arrows/WS.png'/>. Note the pituitary gland <img src='img/arrows/WC.png'/> is flattened beneath the cyst. Arachnoid cysts follow CSF signal intensity on all sequences.](35254557-7c7b-41e8-8bc7-486d1dce3a0d)
**Arachnoid Cyst**
*Coronal T1WI C+ MR shows a sellar and suprasellar arachnoid cyst <img src='img/arrows/CO.png'/> with leftward displacement of the pituitary infundibulum <img src='img/arrows/WS.png'/>. Note the pituitary gland <img src='img/arrows/WC.png'/> is flattened beneath the cyst. Arachnoid cysts follow CSF signal intensity on all sequences.*
![Sagittal T2WI SPACE MR shows obstructive hydrocephalus related to a cystic lesion at the anterior cerebral aqueduct <img src='img/arrows/WS.png'/>. Note mild enlargement of bony sella <img src='img/arrows/BS.png'/> and suprasellar herniation of the anterior 3rd ventricle <img src='img/arrows/CC.png'/>.](929a6a4f-53f2-4a56-9411-ac7aa2080464)
**Obstructive Hydrocephalus (Mimic)**
*Sagittal T2WI SPACE MR shows obstructive hydrocephalus related to a cystic lesion at the anterior cerebral aqueduct <img src='img/arrows/WS.png'/>. Note mild enlargement of bony sella <img src='img/arrows/BS.png'/> and suprasellar herniation of the anterior 3rd ventricle <img src='img/arrows/CC.png'/>.*
### Additional Images
![Sagittal T1WI MR shows a sellar and suprasellar arachnoid cyst <img src='img/arrows/CO.png'/>. Note the pituitary gland <img src='img/arrows/WS.png'/> is stretched anteriorly around the cyst. Arachnoid cysts follow CSF signal intensity on all sequences.](d70d7b6d-3e2f-4e2d-a4de-24e4d96fa195)
**Arachnoid Cyst**
*Sagittal T1WI MR shows a sellar and suprasellar arachnoid cyst <img src='img/arrows/CO.png'/>. Note the pituitary gland <img src='img/arrows/WS.png'/> is stretched anteriorly around the cyst. Arachnoid cysts follow CSF signal intensity on all sequences.*
![Axial T2WI MR shows a large epidermoid cyst extending into the sella and suprasellar subarachnoid space <img src='img/arrows/CC.png'/>. Epidermoids are congenital lesions that classically show DWI hyperintensity. They are most commonly found in the cerebellopontine angle.](ae1c6c9e-6a64-4633-93e9-c1920810ff00)
**Epidermoid Cyst**
*Axial T2WI MR shows a large epidermoid cyst extending into the sella and suprasellar subarachnoid space <img src='img/arrows/CC.png'/>. Epidermoids are congenital lesions that classically show DWI hyperintensity. They are most commonly found in the cerebellopontine angle.*
![Coronal T2WI MR shows a CSF-like intrasellar mass <img src='img/arrows/CO.png'/>. Surgery disclosed intrasellar craniopharyngioma with only a small suprasellar component. Calcification and enhancement help differentiate craniopharyngioma from a Rathke cleft cyst. Only 5% of craniopharyngiomas are purely intrasellar.](fe482745-21a3-4177-88ca-7025d0f9cd9e)
**Craniopharyngioma**
*Coronal T2WI MR shows a CSF-like intrasellar mass <img src='img/arrows/CO.png'/>. Surgery disclosed intrasellar craniopharyngioma with only a small suprasellar component. Calcification and enhancement help differentiate craniopharyngioma from a Rathke cleft cyst. Only 5% of craniopharyngiomas are purely intrasellar.*
![Sagittal T2WI MR shows aqueductal stenosis <img src='img/arrows/WC.png'/> with severe obstructive hydrocephalus. Note marked enlargement of bony sella <img src='img/arrows/WS.png'/> and intrasellar herniation of the inferior 3rd ventricle <img src='img/arrows/BC.png'/>.](ece04c2f-9249-435e-a453-344761f40129)
**Obstructive Hydrocephalus (Mimic)**
*Sagittal T2WI MR shows aqueductal stenosis <img src='img/arrows/WC.png'/> with severe obstructive hydrocephalus. Note marked enlargement of bony sella <img src='img/arrows/WS.png'/> and intrasellar herniation of the inferior 3rd ventricle <img src='img/arrows/BC.png'/>.*
![Coronal T1WI C+ MR shows rim <img src='img/arrows/WS.png'/> and nodular <img src='img/arrows/WO.png'/> enhancement around necrotic macroadenoma in a patient with sudden onset of visual problems and pituitary insufficiency.](b26125bb-9423-402e-bea6-b806dbb4956d)
**Pituitary Apoplexy**
*Coronal T1WI C+ MR shows rim <img src='img/arrows/WS.png'/> and nodular <img src='img/arrows/WO.png'/> enhancement around necrotic macroadenoma in a patient with sudden onset of visual problems and pituitary insufficiency.*
![Sagittal T1WI MR in a patient with a remote history of pituitary apoplexy caused by Sheehan syndrome shows secondary empty sella <img src='img/arrows/WS.png'/>.](9728fb7e-a492-4205-be63-50d45a67d3dd)
**Pituitary Apoplexy**
*Sagittal T1WI MR in a patient with a remote history of pituitary apoplexy caused by Sheehan syndrome shows secondary empty sella <img src='img/arrows/WS.png'/>.*
![Axial T1WI C+ FS MR shows pituitary apoplexy mimic. A nonenhancing, cystic-appearing intrasellar mass <img src='img/arrows/WS.png'/> was diagnosed as pituitary apoplexy. Thrombosed ICA aneurysm was found at surgery.](b0ae48ee-ab27-4361-8a9b-b9d2381a34a5)
**Saccular Aneurysm (Thrombosed)**
*Axial T1WI C+ FS MR shows pituitary apoplexy mimic. A nonenhancing, cystic-appearing intrasellar mass <img src='img/arrows/WS.png'/> was diagnosed as pituitary apoplexy. Thrombosed ICA aneurysm was found at surgery.*
![Sagittal T1WI MR shows empty sella with herniation of CSF through the diaphragma sellae <img src='img/arrows/WS.png'/>, flattening the pituitary gland inferiorly against the sellar floor <img src='img/arrows/WO.png'/>.](images/app.statdx.com_image_thumbnail_b86b973e-19cb-4dc6-937d-7dd41dea8158_annotated_true_size_900_quality_90_ab481f0a.jpg)
**Empty Sella**
*Sagittal T1WI MR shows empty sella with herniation of CSF through the diaphragma sellae <img src='img/arrows/WS.png'/>, flattening the pituitary gland inferiorly against the sellar floor <img src='img/arrows/WO.png'/>.*
![Axial T2WI MR shows idiopathic intracranial hypertension (pseudotumor cerebri) with empty sella <img src='img/arrows/WS.png'/> and dilated optic nerve sheaths <img src='img/arrows/WC.png'/>.](images/app.statdx.com_image_thumbnail_a38d1097-de4f-4954-bc32-57c587fa4e08_annotated_true_size_900_quality_90_e4fa172e.jpg)
**Intracranial Hypertension, Idiopathic**
*Axial T2WI MR shows idiopathic intracranial hypertension (pseudotumor cerebri) with empty sella <img src='img/arrows/WS.png'/> and dilated optic nerve sheaths <img src='img/arrows/WC.png'/>.*
![Coronal T1WI MR shows an intrasellar Rathke cleft cyst <img src='img/arrows/WS.png'/>, seen here as a CSF-like mass that displaces the pituitary gland inferiorly and laterally around it.](images/app.statdx.com_image_thumbnail_e2eae37c-df12-456c-86ed-a057c32b9fee_annotated_true_size_900_quality_90_641dcbcb.jpg)
**Rathke Cleft Cyst**
*Coronal T1WI MR shows an intrasellar Rathke cleft cyst <img src='img/arrows/WS.png'/>, seen here as a CSF-like mass that displaces the pituitary gland inferiorly and laterally around it.*
![Sagittal T1WI C+ MR shows suprasellar racemose neurocysticercosis (NCC) cysts <img src='img/arrows/WC.png'/> extending into sella turcica <img src='img/arrows/WO.png'/>, flattening pituitary gland against sellar floor <img src='img/arrows/BS.png'/>.](98d760f8-f112-48c6-811b-fcc685f9d32c)
**Neurocysticercosis Cyst**
*Sagittal T1WI C+ MR shows suprasellar racemose neurocysticercosis (NCC) cysts <img src='img/arrows/WC.png'/> extending into sella turcica <img src='img/arrows/WO.png'/>, flattening pituitary gland against sellar floor <img src='img/arrows/BS.png'/>.*
![Axial T2WI MR shows racemose (multilobulated, grape-like) NCC cysts extending into the suprasellar cistern <img src='img/arrows/CO.png'/> and the cerebellopontine angles <img src='img/arrows/CS.png'/>. Racemose NCC typically involves the basal cisterns and lacks a typical scolex.](32c0889d-3371-4d39-a634-312a095dc10f)
**Neurocysticercosis Cyst**
*Axial T2WI MR shows racemose (multilobulated, grape-like) NCC cysts extending into the suprasellar cistern <img src='img/arrows/CO.png'/> and the cerebellopontine angles <img src='img/arrows/CS.png'/>. Racemose NCC typically involves the basal cisterns and lacks a typical scolex.*
![Coronal T2WI MR shows a CSF-like intrasellar mass <img src='img/arrows/BO.png'/>. Surgery disclosed intrasellar craniopharyngioma with only a small suprasellar component. The vast majority of craniopharyngiomas are sellar and suprasellar. Only 5% of craniopharyngiomas are intrasellar.](f3ef7e14-61fa-4490-aa72-47b36f854d4f)
**Craniopharyngioma**
*Coronal T2WI MR shows a CSF-like intrasellar mass <img src='img/arrows/BO.png'/>. Surgery disclosed intrasellar craniopharyngioma with only a small suprasellar component. The vast majority of craniopharyngiomas are sellar and suprasellar. Only 5% of craniopharyngiomas are intrasellar.*
![Sagittal T1WI MR shows an intra- and suprasellar arachnoid cyst <img src='img/arrows/WS.png'/>. The lesion did not restrict on DWI, differentiating it from epidermoid cyst.](bbf79056-04dd-4fd2-af2e-6ed04580adef)
**Arachnoid Cyst**
*Sagittal T1WI MR shows an intra- and suprasellar arachnoid cyst <img src='img/arrows/WS.png'/>. The lesion did not restrict on DWI, differentiating it from epidermoid cyst.*
![Axial T2WI MR shows a large epidermoid cyst extending into the sella and suprasellar subarachnoid space <img src='img/arrows/WC.png'/> from the quadrigeminal and ambient cisterns <img src='img/arrows/WS.png'/>.](18570c6f-bb45-48ea-b937-fb7698fd8327)
**Epidermoid Cyst**
*Axial T2WI MR shows a large epidermoid cyst extending into the sella and suprasellar subarachnoid space <img src='img/arrows/WC.png'/> from the quadrigeminal and ambient cisterns <img src='img/arrows/WS.png'/>.*
docs_md/articles/dementia-with-lewy-bodies_e8e46d1d-46d2-4e5a-880f-f025a84c5871.md
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---
title: "Dementia With Lewy Bodies"
docid: "e8e46d1d-46d2-4e5a-880f-f025a84c5871"
authors:
- key: "1fa14dfd-71ea-4960-908e-e720313bc63a"
value: "Santhosh Gaddikeri, MD"
- key: "a25c450b-3d34-4f64-bba3-cc0834813df6"
value: "Miral D. Jhaveri, MD, MBA"
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name: "Brain"
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slug: "diagnosis"
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slug: "pathology-based-diagnoses"
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name: "Dementia With Lewy Bodies"
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lastUpdated: "08/10/20"
pageDescription: "Dementia With Lewy Bodies"
pageKeywords: "Brain, Diagnosis, Pathology-Based Diagnoses, Acquired Toxic/Metabolic/Degenerative Disorders, Dementias and Degenerative Disorders, Dementia With Lewy Bodies"
pageTitle: "Dementia With Lewy Bodies | STATdx"
enhancedTitle: "Dementia With Lewy Bodies"
type: "DX"
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breadcrumbs:
- "Brain"
- "Diagnosis"
- "Pathology-Based Diagnoses"
- "Acquired Toxic/Metabolic/Degenerative Disorders"
- "Dementias and Degenerative Disorders"
- "Dementia With Lewy Bodies"
---
# KEY FACTS
- ## Terminology
- Progressive neurodegenerative dementia
- Parkinsonism, visual hallucinations prominent
- Caused by abnormal accumulation of α-synuclein protein
- ## Imaging
- MR may differentiate Alzheimer disease (AD) from dementia with Lewy bodies (DLB)
- PET, SPECT most useful for DLB diagnosis
- Voxel-based morphometry
- Relatively preserved hippocampal/medial temporal lobe volume in DLB vs. AD
- ↓ volume of hypothalamus, substantia innominata, & putamen in DLB vs. AD
- FDG PET
- ↓ in glucose metabolism in occipital cortex, especially primary visual cortex
- F-18 fluorodopa-PET: ↓ striatal dopamine uptake in DLB vs. AD
- SPECT: Occipital lobe hypoperfusion, especially visual cortex
- 123 FP-CIT SPECT: ↓ uptake in striatum in DLB vs. AD
- ## Top Differential Diagnoses
- Parkinson disease-associated dementia (PDD)
- Similar clinical, pathological, imaging features with DLB
- AD
- Frontotemporal lobar degeneration (FTLD)
- Vascular dementia
- ## Pathology
- Pathologic aggregation of α-synuclein protein in neurites (LB)
- ## Diagnostic Checklist
- Unlike AD, medial temporal lobe atrophy not prominent feature
# TERMINOLOGY
- ## Abbreviations
- Dementia with Lewy bodies (DLB)
- ## Definitions
- Neurodegenerative dementia characterized by cognitive fluctuations, visual hallucinations, & motor parkinsonism
- Caused by pathologic aggregation of α-synuclein protein in neurites (LB)
# IMAGING
- ## General Features
- ### Best diagnostic clue
- MR may differentiate Alzheimer disease (AD) from DLB
- PET, SPECT most useful for DLB diagnosis
- ## Imaging Recommendations
- ### Best imaging tool
- PET or SPECT
- ## MR Findings
- ### T1WI
- Mild generalized atrophy
- ### T2WI
- Nonspecific white matter (WM) hyperintensities
- ### MRS
- ↓ WM NAA/Cr in DLB vs. healthy controls (HC)
- ↑ Cho/Cr ratios in DLB vs. HC
- Normal levels of NAA/Cr & myoinositol in DLB vs. AD
- Voxel-based morphometry
- Relatively preserved hippocampal/medial temporal lobe volume in DLB vs. AD
- ↓ volume of hypothalamus, substantia innominata, & putamen in DLB vs. AD
- ↓ gray matter in temporal, parietal, & occipital regions vs. HC
- DTI
- ↑ mean diffusivity in amygdala
- ↓ fractional anisotropy in pons & left thalamus vs. AD
- ↓ fractional anisotropy in inferior longitudinal fasciculus & inferior occipitofrontal fasciculi vs. HC
- ## Nuclear Medicine Findings
- ### PET
- FDG PET: ↓ glucose metabolism in occipital cortex & visual association cortex with relative preservation of posterior cingulate
- F-18 fluorodopa-PET: ↓ striatal dopamine uptake in DLB vs. AD
- ### MIBG scintigraphy
- ↓ myocardial uptake in DLB due to ↓ postganglionic sympathetic cardiac innervation
- SPECT
- Occipital lobe hypoperfusion, especially visual cortex
- 123 FP-CIT SPECT: Visualize DAT (dopamine transporter) loss
- ↓ uptake in striatum in DLB
# DIFFERENTIAL DIAGNOSIS
- [Parkinson Disease-Associated Dementia](/document/parkinson-disease/0bc3188a-935b-416d-b1a0-25b2d52c6399)
- Dementia typically develops at least 12 months after onset of initial parkinsonian symptoms
- Similar clinical, pathologic, imaging features to DLB
- Less pronounced atrophy in temporal, occipital, & parietal lobes vs. DLB
- [Alzheimer Disease](/document/alzheimer-disease/f71f5cf5-b1af-4c6d-b145-b4c10eec7b58)
- Parietal/temporal cortical atrophy
- Disproportionate hippocampal volume loss
- Amyloid uptake of cerebral cortex in PiB-PET
- More severe, faster rate of progression than DLB
- [Frontotemporal Lobar Degeneration](/document/frontotemporal-lobar-degeneration/49510d0e-acf7-45cb-9eb1-53f8193b0b6d)
- Asymmetric frontal, anterior temporal lobar atrophy
- Behavioral variant: Both frontal lobes atrophic
- Semantic variant: Asymmetric anterior temporal lobe atrophy
- [Vascular Dementia](/document/vascular-dementia/f59dab57-c511-4369-8fcc-592421a4b8d1)
- 2nd most common dementia (15-30%)
- WM & deep gray lacunae
- Infarcts of different ages
- Hyperintense lesions on T2WI, hypodense areas on CT, & focal atrophy suggestive of chronic infarcts
# PATHOLOGY
- ## General Features
- ### Etiology
- Accumulation of α-synuclein protein (LB)
- LB, neuronal loss in substantia nigra → dopamine depletion
- Loss of cholinergic neurons in nucleus basalis of Meynert
- → cognitive impairment, visual hallucinations
- ### Genetics
- Majority of DLB is sporadic; some are familial
- α-synuclein gene mutation on chromosome 4 (*A53T*, *E46K* mutation)
- Similar inheritance, similar genetic risk for PD
- Other genes associated with DLB include *SNCA*, *APP*, *PSEN1*/*PSEN2*, *MAPT*, *GBA*, & *APOE*
- ## Staging, Grading, & Classification
- 3 major forms: Brainstem dominant, limbic/transitional, diffuse neocortical
- ## Gross Pathologic & Surgical Features
- Nonspecific & overlap with other neurodegenerative dementias
- Cortical atrophy is less than AD
- Atrophy affects frontal, temporal, & parietal lobes, relative sparing of occipital lobes
- Amygdala & cingulate gyri can show severe atrophy
- ## Microscopic Features
- LB in substantia nigra, neocortex, limbic system
- α-synuclein protein aggregates: Pale eosinophilic inclusions
- α-synuclein
- Physiologic function: Synaptic transmission, neuroprotective effect
- Predominantly expressed in neurons
- Lewy neurites in hippocampus, amygdala, brainstem nuclei
- Neuronal loss in substantia nigra, locus ceruleus, nucleus basalis of Meynert, dorsal raphe nuclei
- Relative preservation of cortical neurons
- Superficial microvacuolation of cerebral cortex, especially temporal cortex in severe cases
- 80% have associated AD-like pathology
- Neuritic/diffuse plaques or neurofibrillary tangles
# CLINICAL ISSUES
- ## Presentation
- ### Most common signs/symptoms
- Cognitive fluctuations, visual hallucinations, parkinsonism
- Dysautonomia & sleep disorders
- Clinical criteria for DLB diagnosis
- Core clinical features
- Fluctuating cognition with pronounced variations in attention & alertness
- Recurrent visual hallucinations (typically well formed & detailed)
- REM sleep behavior disorder (May precede cognitive decline)
- 1 or more spontaneous cardinal features of parkinsonism (bradykinesia, rest tremor, rigidity)
- Supportive clinical features
- Severe sensitivity to antipsychotic agents
- Postural instability, repeated falls
- Syncope or other transient episodes of unresponsiveness
- Severe autonomic dysfunction (constipation, orthostatic hypotension, urinary incontinence)
- Hypersomnia, hyposmia, hallucinations in other modalities, systematized delusions
- Apathy, anxiety, & depression
- Indicative biomarkers
- ↓ dopamine transporter uptake in basal ganglia by SPECT or PET
- Abnormal (low-uptake) I-123-MIBG myocardial scintigraphy
- Polysomnographic confirmation of REM sleep without atonia
- Supportive biomarkers
- Relative preservation of medial temporal lobe structures on CT/MR
- Generalized low uptake on SPECT/PET perfusion/metabolism scan with ↓ occipital activity ± cingulate island sign on FDG PET imaging
- Prominent posterior slow-wave activity on EEG with periodic fluctuations in pre-alpha/theta range
- Probable DLB
- ≥ 2 core clinical features of DLB with or without indicative biomarkers; **or**
- Only 1 core clinical feature but with ≥ 1 indicative biomarkers
- Probable DLB should not be diagnosed on basis of biomarkers alone
- Possible DLB
- Only 1 core clinical feature of DLB with no indicative biomarker evidence; **or**
- ≥ 1 indicative biomarkers but no core clinical features
- DLB is less likely
- Presence of any other physical illness or brain disorder, including cerebrovascular disease, sufficient to account in part or in total for clinical picture
- If parkinsonian features are only core clinical feature & appear for 1st time at stage of severe dementia
- ## Demographics
- ### Age
- 55-85 years; age is only risk factor
- Average at presentation is 75 years
- ### Ethnicity
- LB formation more common in African Americans than Caucasians, but clinical diagnosis of DLB is not significantly different
- ### Sex
- M:F = 4:1
- ### Epidemiology
- 5% of general population & 30% of dementia cases
- 2nd most common neurodegenerative dementia (after AD)
- Incidence rate of 0.1% per year in general population & 3.2% for new dementia cases
- ## Natural History & Prognosis
- Average survival after diagnosis < 8 years
- ## Treatment
- ### Options, risks, complications
- No disease-modifying treatments for DLB
- Symptomatic, targeted toward specific disease manifestations
- Cholinesterase inhibitor for cognitive features
- DLB responds better to cholinesterase inhibitor than AD
- Treatment against hallucination should be conservative due to neuroleptic hypersensitivity of DLB
# DIAGNOSTIC CHECKLIST
- ## Image Interpretation Pearls
- No characteristic features on standard MR
- Clinical dementia + no/relatively mild medial temporal lobe atrophy
- Unlike AD, medial temporal lobe atrophy is not prominent
f336b865-74c1-456e-9130-6db7efb9e7b3
## References
# Selected References
1. [Colloby SJ et al: Cortical thinning in dementia with Lewy bodies and Parkinson disease dementia. Aust N Z J Psychiatry. 4867419885165, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31696728%5Bpmid%5D)
1. [Gupta V et al: Metabolic imaging patterns in posterior cortical atrophy and Lewy body dementia. Nucl Med Commun. 40(12):1275-82, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31633646%5Bpmid%5D)
1. [Yamada M et al: Diagnostic criteria for dementia with lewy bodies: updates and future directions. J Mov Disord. ePub, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31694357%5Bpmid%5D)
1. [Orimo S: [Differential diagnosis of dementia with lewy bodies.] Brain Nerve. 67(4):413-25, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25846590%5Bpmid%5D)
1. [Bertelson JA et al: Neuroimaging of dementia. Neurol Clin. 32(1):59-93, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=24287385%5Bpmid%5D)
1. [Broski SM et al: Structural and functional imaging in parkinsonian syndromes. Radiographics. 34(5):1273-92, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=25208280%5Bpmid%5D)
1. [Mak E et al: Neuroimaging characteristics of dementia with Lewy bodies. Alzheimers Res Ther. 6(2):18, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=25031634%5Bpmid%5D)
1. [Peraza LR et al: fMRI resting state networks and their association with cognitive fluctuations in dementia with Lewy bodies. Neuroimage Clin. 4:558-65, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=24818081%5Bpmid%5D)
1. [Bhogal P et al: The common dementias: a pictorial review. Eur Radiol. 23(12):3405-17, 2013](http://www.ncbi.nlm.nih.gov/pubmed/?term=24081643%5Bpmid%5D)
1. [Mortimer AM et al: Neuroimaging in dementia: a practical guide. Pract Neurol. 13(2):92-103, 2013](http://www.ncbi.nlm.nih.gov/pubmed/?term=23468560%5Bpmid%5D)
1. [Burton EJ et al: Medial temporal lobe atrophy on MRI differentiates Alzheimer's disease from dementia with Lewy bodies and vascular cognitive impairment: a prospective study with pathological verification of diagnosis. Brain. 132(Pt 1):195-203, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=19022858%5Bpmid%5D)
1. [Ota M et al: Degeneration of dementia with Lewy bodies measured by diffusion tensor imaging. NMR Biomed. 22(3):280-4, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=19009555%5Bpmid%5D)
1. [Watson R et al: Magnetic resonance imaging in lewy body dementias. Dement Geriatr Cogn Disord. 28(6):493-506, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=19996594%5Bpmid%5D)
1. [Edison P et al: Amyloid load in Parkinson's disease dementia and Lewy body dementia measured with [11C]PIB positron emission tomography. J Neurol Neurosurg Psychiatry. 79(12):1331-8, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18653550%5Bpmid%5D)
1. [Perneczky R et al: Cerebral metabolic dysfunction in patients with dementia with Lewy bodies and visual hallucinations. Dement Geriatr Cogn Disord. 25(6):531-8, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18477846%5Bpmid%5D)
1. [Schmidt SL et al: Value of combining activated brain FDG-PET and cardiac MIBG for the differential diagnosis of dementia: differentiation of dementia with Lewy bodies and Alzheimer disease when the diagnoses based on clinical and neuroimaging criteria are difficult. Clin Nucl Med. 33(6):398-401, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18496445%5Bpmid%5D)
1. [McKeith I et al: Sensitivity and specificity of dopamine transporter imaging with 123I-FP-CIT SPECT in dementia with Lewy bodies: a phase III, multicentre study. Lancet Neurol. 6(4):305-13, 2007](http://www.ncbi.nlm.nih.gov/pubmed/?term=17362834%5Bpmid%5D)
1. [Seppi K et al: Dementia with Lewy bodies and Parkinson disease with dementia: can MRI make the difference? Neurology. 69(8):717-8, 2007](http://www.ncbi.nlm.nih.gov/pubmed/?term=17709701%5Bpmid%5D)
1. [Whitwell JL et al: Focal atrophy in dementia with Lewy bodies on MRI: a distinct pattern from Alzheimer's disease. Brain. 130(Pt 3):708-19, 2007](http://www.ncbi.nlm.nih.gov/pubmed/?term=17267521%5Bpmid%5D)
1. [Burton EJ et al: Progression of white matter hyperintensities in Alzheimer disease, dementia with lewy bodies, and Parkinson disease dementia: a comparison with normal aging. Am J Geriatr Psychiatry. 14(10):842-9, 2006](http://www.ncbi.nlm.nih.gov/pubmed/?term=17001024%5Bpmid%5D)
## Images
### Selected Images
![Right &amp; left medial sagittal FDG PET source &amp; 3DSSP images of a 74-year-old man with dementia with Lewy bodies (DLB) presenting with visual hallucinations show severe ↓ metabolic activity in left visual cortex <img src='img/arrows/CO.png'/> &amp; precuneus <img src='img/arrows/WS.png'/>. Note moderate ↓ metabolic activity in right occipital lobe <img src='img/arrows/CS.png'/> &amp; precuneus <img src='img/arrows/WC.png'/> &amp; relative sparing of bilateral posterior cingulate gyri <img src='img/arrows/CC.png'/>.](images/app.statdx.com_image_thumbnail_d2ff700c-f618-401b-94df-595198c45827_size_168_quality_85_36e94475_20251014T193405Z.jpg)
*Right &amp; left medial sagittal FDG PET source &amp; 3DSSP images of a 74-year-old man with dementia with Lewy bodies (DLB) presenting with visual hallucinations show severe ↓ metabolic activity in left visual cortex <img src='img/arrows/CO.png'/> &amp; precuneus <img src='img/arrows/WS.png'/>. Note moderate ↓ metabolic activity in right occipital lobe <img src='img/arrows/CS.png'/> &amp; precuneus <img src='img/arrows/WC.png'/> &amp; relative sparing of bilateral posterior cingulate gyri <img src='img/arrows/CC.png'/>.*
![Right &amp; left medial sagittal FDG PET source &amp; 3DSSP images of a 74-year-old man with dementia with Lewy bodies (DLB) presenting with visual hallucinations show severe ↓ metabolic activity in left visual cortex <img src='img/arrows/CO.png'/> &amp; precuneus <img src='img/arrows/WS.png'/>. Note moderate ↓ metabolic activity in right occipital lobe <img src='img/arrows/CS.png'/> &amp; precuneus <img src='img/arrows/WC.png'/> &amp; relative sparing of bilateral posterior cingulate gyri <img src='img/arrows/CC.png'/>.](images/app.statdx.com_image_thumbnail_d2ff700c-f618-401b-94df-595198c45827_size_168_quality_85_7b4870ec_20251014T190919Z.jpg)
*Right &amp; left medial sagittal FDG PET source &amp; 3DSSP images of a 74-year-old man with dementia with Lewy bodies (DLB) presenting with visual hallucinations show severe ↓ metabolic activity in left visual cortex <img src='img/arrows/CO.png'/> &amp; precuneus <img src='img/arrows/WS.png'/>. Note moderate ↓ metabolic activity in right occipital lobe <img src='img/arrows/CS.png'/> &amp; precuneus <img src='img/arrows/WC.png'/> &amp; relative sparing of bilateral posterior cingulate gyri <img src='img/arrows/CC.png'/>.*
![Right &amp; left medial sagittal FDG PET source &amp; 3DSSP images of a 74-year-old man with dementia with Lewy bodies (DLB) presenting with visual hallucinations show severe ↓ metabolic activity in left visual cortex <img src='img/arrows/CO.png'/> &amp; precuneus <img src='img/arrows/WS.png'/>. Note moderate ↓ metabolic activity in right occipital lobe <img src='img/arrows/CS.png'/> &amp; precuneus <img src='img/arrows/WC.png'/> &amp; relative sparing of bilateral posterior cingulate gyri <img src='img/arrows/CC.png'/>.](images/app.statdx.com_image_thumbnail_d2ff700c-f618-401b-94df-595198c45827_size_174_quality_85_1fb4bccd_20251014T204456Z.jpg)
*Right &amp; left medial sagittal FDG PET source &amp; 3DSSP images of a 74-year-old man with dementia with Lewy bodies (DLB) presenting with visual hallucinations show severe ↓ metabolic activity in left visual cortex <img src='img/arrows/CO.png'/> &amp; precuneus <img src='img/arrows/WS.png'/>. Note moderate ↓ metabolic activity in right occipital lobe <img src='img/arrows/CS.png'/> &amp; precuneus <img src='img/arrows/WC.png'/> &amp; relative sparing of bilateral posterior cingulate gyri <img src='img/arrows/CC.png'/>.*
![Right &amp; left medial sagittal FDG PET source &amp; 3DSSP images of a 74-year-old man with dementia with Lewy bodies (DLB) presenting with visual hallucinations show severe ↓ metabolic activity in left visual cortex <img src='img/arrows/CO.png'/> &amp; precuneus <img src='img/arrows/WS.png'/>. Note moderate ↓ metabolic activity in right occipital lobe <img src='img/arrows/CS.png'/> &amp; precuneus <img src='img/arrows/WC.png'/> &amp; relative sparing of bilateral posterior cingulate gyri <img src='img/arrows/CC.png'/>.](images/app.statdx.com_image_thumbnail_d2ff700c-f618-401b-94df-595198c45827_size_174_quality_85_29ed5b27_20251014T185333Z.jpg)
*Right &amp; left medial sagittal FDG PET source &amp; 3DSSP images of a 74-year-old man with dementia with Lewy bodies (DLB) presenting with visual hallucinations show severe ↓ metabolic activity in left visual cortex <img src='img/arrows/CO.png'/> &amp; precuneus <img src='img/arrows/WS.png'/>. Note moderate ↓ metabolic activity in right occipital lobe <img src='img/arrows/CS.png'/> &amp; precuneus <img src='img/arrows/WC.png'/> &amp; relative sparing of bilateral posterior cingulate gyri <img src='img/arrows/CC.png'/>.*
![Right &amp; left medial sagittal FDG PET source &amp; 3DSSP images of a 74-year-old man with dementia with Lewy bodies (DLB) presenting with visual hallucinations show severe ↓ metabolic activity in left visual cortex <img src='img/arrows/CO.png'/> &amp; precuneus <img src='img/arrows/WS.png'/>. Note moderate ↓ metabolic activity in right occipital lobe <img src='img/arrows/CS.png'/> &amp; precuneus <img src='img/arrows/WC.png'/> &amp; relative sparing of bilateral posterior cingulate gyri <img src='img/arrows/CC.png'/>.](images/app.statdx.com_image_thumbnail_d2ff700c-f618-401b-94df-595198c45827_size_174_quality_85_2ff5fd3e_20251014T193347Z.jpg)
*Right &amp; left medial sagittal FDG PET source &amp; 3DSSP images of a 74-year-old man with dementia with Lewy bodies (DLB) presenting with visual hallucinations show severe ↓ metabolic activity in left visual cortex <img src='img/arrows/CO.png'/> &amp; precuneus <img src='img/arrows/WS.png'/>. Note moderate ↓ metabolic activity in right occipital lobe <img src='img/arrows/CS.png'/> &amp; precuneus <img src='img/arrows/WC.png'/> &amp; relative sparing of bilateral posterior cingulate gyri <img src='img/arrows/CC.png'/>.*
![Right &amp; left medial sagittal FDG PET source &amp; 3DSSP images of a 74-year-old man with dementia with Lewy bodies (DLB) presenting with visual hallucinations show severe ↓ metabolic activity in left visual cortex <img src='img/arrows/CO.png'/> &amp; precuneus <img src='img/arrows/WS.png'/>. Note moderate ↓ metabolic activity in right occipital lobe <img src='img/arrows/CS.png'/> &amp; precuneus <img src='img/arrows/WC.png'/> &amp; relative sparing of bilateral posterior cingulate gyri <img src='img/arrows/CC.png'/>.](images/app.statdx.com_image_thumbnail_d2ff700c-f618-401b-94df-595198c45827_size_174_quality_85_7be15c01_20251014T190917Z.jpg)
*Right &amp; left medial sagittal FDG PET source &amp; 3DSSP images of a 74-year-old man with dementia with Lewy bodies (DLB) presenting with visual hallucinations show severe ↓ metabolic activity in left visual cortex <img src='img/arrows/CO.png'/> &amp; precuneus <img src='img/arrows/WS.png'/>. Note moderate ↓ metabolic activity in right occipital lobe <img src='img/arrows/CS.png'/> &amp; precuneus <img src='img/arrows/WC.png'/> &amp; relative sparing of bilateral posterior cingulate gyri <img src='img/arrows/CC.png'/>.*
![Axial PET of the same patient shows preserved metabolism in frontal <img src='img/arrows/CS.png'/> &amp; temporal <img src='img/arrows/CO.png'/> lobes. (Courtesy S Behnia, MD.)](images/app.statdx.com_image_thumbnail_141c724e-0817-4b70-98b1-eca06cdbef62_size_168_quality_85_0a602e19_20251014T190919Z.jpg)
*Axial PET of the same patient shows preserved metabolism in frontal <img src='img/arrows/CS.png'/> &amp; temporal <img src='img/arrows/CO.png'/> lobes. (Courtesy S Behnia, MD.)*
![Axial PET of the same patient shows preserved metabolism in frontal <img src='img/arrows/CS.png'/> &amp; temporal <img src='img/arrows/CO.png'/> lobes. (Courtesy S Behnia, MD.)](images/app.statdx.com_image_thumbnail_141c724e-0817-4b70-98b1-eca06cdbef62_size_168_quality_85_85a134e4_20251014T193405Z.jpg)
*Axial PET of the same patient shows preserved metabolism in frontal <img src='img/arrows/CS.png'/> &amp; temporal <img src='img/arrows/CO.png'/> lobes. (Courtesy S Behnia, MD.)*
![Axial T2WI MR in a patient with DLB shows nonspecific diffuse cortical atrophy. Conventional MR findings are frequently nonspecific in DLB.](images/app.statdx.com_image_thumbnail_784ee22f-8cfd-4f22-bddf-44c1616eebfb_size_168_quality_85_5c3c8b25_20251014T190919Z.jpg)
*Axial T2WI MR in a patient with DLB shows nonspecific diffuse cortical atrophy. Conventional MR findings are frequently nonspecific in DLB.*
![Axial T2WI MR in a patient with DLB shows nonspecific diffuse cortical atrophy. Conventional MR findings are frequently nonspecific in DLB.](images/app.statdx.com_image_thumbnail_784ee22f-8cfd-4f22-bddf-44c1616eebfb_size_168_quality_85_9f5d1b8b_20251014T193405Z.jpg)
*Axial T2WI MR in a patient with DLB shows nonspecific diffuse cortical atrophy. Conventional MR findings are frequently nonspecific in DLB.*
![123FP-CIT-SPECT, DAT imaging (dopamine transporter) shows normal symmetric uptake in the striatum <img src='img/arrows/WS.png'/> of a healthy control (HC). In DLB, there is marked ↓ update in the putamen <img src='img/arrows/WC.png'/> &amp; mild in the caudate nuclei <img src='img/arrows/WO.png'/>. Using DAT imaging, it is not possible to distinguish DLB from atypical parkinsonian syndromes like MSA, PSP, &amp; CBD.](images/app.statdx.com_image_thumbnail_d5cf7988-1211-4bec-888b-b72a8bdf8139_size_168_quality_85_22e550ce_20251014T193405Z.jpg)
*123FP-CIT-SPECT, DAT imaging (dopamine transporter) shows normal symmetric uptake in the striatum <img src='img/arrows/WS.png'/> of a healthy control (HC). In DLB, there is marked ↓ update in the putamen <img src='img/arrows/WC.png'/> &amp; mild in the caudate nuclei <img src='img/arrows/WO.png'/>. Using DAT imaging, it is not possible to distinguish DLB from atypical parkinsonian syndromes like MSA, PSP, &amp; CBD.*
![123FP-CIT-SPECT, DAT imaging (dopamine transporter) shows normal symmetric uptake in the striatum <img src='img/arrows/WS.png'/> of a healthy control (HC). In DLB, there is marked ↓ update in the putamen <img src='img/arrows/WC.png'/> &amp; mild in the caudate nuclei <img src='img/arrows/WO.png'/>. Using DAT imaging, it is not possible to distinguish DLB from atypical parkinsonian syndromes like MSA, PSP, &amp; CBD.](images/app.statdx.com_image_thumbnail_d5cf7988-1211-4bec-888b-b72a8bdf8139_size_168_quality_85_73efa92a_20251014T190920Z.jpg)
*123FP-CIT-SPECT, DAT imaging (dopamine transporter) shows normal symmetric uptake in the striatum <img src='img/arrows/WS.png'/> of a healthy control (HC). In DLB, there is marked ↓ update in the putamen <img src='img/arrows/WC.png'/> &amp; mild in the caudate nuclei <img src='img/arrows/WO.png'/>. Using DAT imaging, it is not possible to distinguish DLB from atypical parkinsonian syndromes like MSA, PSP, &amp; CBD.*
### Additional Images
![Axial T2WI MR in a patient with cognitive decline, visual hallucination, &amp; parkinsonism shows diffuse cortical atrophy consistent with DLB.](images/app.statdx.com_image_thumbnail_08a51897-3ce0-46a6-bab2-3e44cae2a189_size_168_quality_85_378e2105_20251014T193405Z.jpg)
*Axial T2WI MR in a patient with cognitive decline, visual hallucination, &amp; parkinsonism shows diffuse cortical atrophy consistent with DLB.*
![Axial T2WI MR in a patient with cognitive decline, visual hallucination, &amp; parkinsonism shows diffuse cortical atrophy consistent with DLB.](images/app.statdx.com_image_thumbnail_08a51897-3ce0-46a6-bab2-3e44cae2a189_size_168_quality_85_be8af0ff_20251014T190919Z.jpg)
*Axial T2WI MR in a patient with cognitive decline, visual hallucination, &amp; parkinsonism shows diffuse cortical atrophy consistent with DLB.*
![Axial T2WI MR in the same patient exhibits mild atrophy of the medial temporal lobes.](images/app.statdx.com_image_thumbnail_d231658a-07ca-4c4f-bcee-284d2e169ba7_size_168_quality_85_0805c87e_20251014T193405Z.jpg)
*Axial T2WI MR in the same patient exhibits mild atrophy of the medial temporal lobes.*
![Axial T2WI MR in the same patient exhibits mild atrophy of the medial temporal lobes.](images/app.statdx.com_image_thumbnail_d231658a-07ca-4c4f-bcee-284d2e169ba7_size_168_quality_85_51e1ee0e_20251014T190920Z.jpg)
*Axial T2WI MR in the same patient exhibits mild atrophy of the medial temporal lobes.*
![Coronal T1WI MR in a patient with DLB shows prominent frontal lobe volume loss with relative sparing of hippocampal volume. (Courtesy M.J. Firbank, MD &amp; J.T. O'Brien, MD.)](images/app.statdx.com_image_thumbnail_348b2550-0f89-42b7-bd6d-167244be21c0_size_168_quality_85_7a473fce_20251014T193405Z.jpg)
*Coronal T1WI MR in a patient with DLB shows prominent frontal lobe volume loss with relative sparing of hippocampal volume. (Courtesy M.J. Firbank, MD &amp; J.T. O'Brien, MD.)*
![Coronal T1WI MR in a patient with DLB shows prominent frontal lobe volume loss with relative sparing of hippocampal volume. (Courtesy M.J. Firbank, MD &amp; J.T. O'Brien, MD.)](images/app.statdx.com_image_thumbnail_348b2550-0f89-42b7-bd6d-167244be21c0_size_168_quality_85_fd060e13_20251014T190919Z.jpg)
*Coronal T1WI MR in a patient with DLB shows prominent frontal lobe volume loss with relative sparing of hippocampal volume. (Courtesy M.J. Firbank, MD &amp; J.T. O'Brien, MD.)*
![Coronal T1WI MR in a patient with Alzheimer disease (AD) shows marked hippocampal volume loss <img src='img/arrows/CC.png'/> &amp; relative sparing of frontal lobes. (Courtesy M. J. Firbank, MD &amp; J. T. O'Brien, MD.)](images/app.statdx.com_image_thumbnail_a6f64f25-ad7f-4840-a6db-76d3b759a94b_size_168_quality_85_abd6ed9b_20251014T193405Z.jpg)
*Coronal T1WI MR in a patient with Alzheimer disease (AD) shows marked hippocampal volume loss <img src='img/arrows/CC.png'/> &amp; relative sparing of frontal lobes. (Courtesy M. J. Firbank, MD &amp; J. T. O'Brien, MD.)*
![Coronal T1WI MR in a patient with Alzheimer disease (AD) shows marked hippocampal volume loss <img src='img/arrows/CC.png'/> &amp; relative sparing of frontal lobes. (Courtesy M. J. Firbank, MD &amp; J. T. O'Brien, MD.)](images/app.statdx.com_image_thumbnail_a6f64f25-ad7f-4840-a6db-76d3b759a94b_size_168_quality_85_f88af611_20251014T190919Z.jpg)
*Coronal T1WI MR in a patient with Alzheimer disease (AD) shows marked hippocampal volume loss <img src='img/arrows/CC.png'/> &amp; relative sparing of frontal lobes. (Courtesy M. J. Firbank, MD &amp; J. T. O'Brien, MD.)*
docs_md/articles/empty-sella_39a0d2d1-1439-4558-8f5d-86a2a6d93e3a.md
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---
title: "Empty Sella"
docid: "39a0d2d1-1439-4558-8f5d-86a2a6d93e3a"
authors:
- key: "5cff4116-3654-4b3a-bb75-5ebe0b8c9850"
value: "Anne G. Osborn, MD, FACR"
breadcrumbs:
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name: "Brain"
slug: "brain"
treeNodeId: "6d8829f1-14d7-45af-8675-255189aa526a"
-
name: "Diagnosis"
slug: "diagnosis"
treeNodeId: "51c00394-446e-4a38-94af-d3b1d14d34e8"
-
name: "Anatomy-Based Diagnoses"
slug: "anatomy-based-diagnoses"
treeNodeId: "529d3e33-f508-498c-bc70-cf962e81e629"
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name: "Sella and Pituitary"
slug: "sella-and-pituitary"
treeNodeId: "9afaeeb6-661c-49be-b55f-5bdc1c98a53e"
-
name: "Miscellaneous"
slug: "miscellaneous"
treeNodeId: "7941c33d-0063-41a2-b035-39440c09b829"
-
name: "Empty Sella"
slug: "empty-sella"
treeNodeId: null
category: "Brain"
documentVersionId: "91612570-e8d2-417b-8345-8226e2028fbf"
imageCount: 18
lastUpdated: "08/10/20"
pageDescription: "Empty Sella"
pageKeywords: "Brain, Diagnosis, Anatomy-Based Diagnoses, Sella and Pituitary, Miscellaneous, Empty Sella"
pageTitle: "Empty Sella | STATdx"
enhancedTitle: "Empty Sella"
type: "DX"
references: true
breadcrumbs:
- "Brain"
- "Diagnosis"
- "Anatomy-Based Diagnoses"
- "Sella and Pituitary"
- "Miscellaneous"
- "Empty Sella"
---
# KEY FACTS
- ## Terminology
- Sella partially filled with arachnoid-lined CSF collection
- Primary empty sella
- Common normal variant (15% of brain MRs), incidental finding
- Normal or increased CSF pressure
- Near-normal volume of compressed pituitary tissue
- Secondary empty sella
- Prior pituitary surgery, radiation, or injury
- ## Imaging
- Intrasellar CSF, pituitary flattened against sellar floor
- Bony sella may be normal or moderately enlarged (secondary to pulsatile CSF)
- Bony margins intact, not eroded/demineralized
- Infundibular stalk, pituitary gland enhance normally
- Fluid exactly like CSF
- Suppresses completely on FLAIR
- Does not restrict on DWI
- ## Top Differential Diagnoses
- Idiopathic intracranial hypertension
- Secondary intracranial hypertension
- Arachnoid cyst
- Pituitary apoplexy
- Pituitary anomalies
- ## Pathology
- "Deficient" diaphragma sellae
- Dural covering of sella is incomplete (widened)
- Leaves large opening for infundibular stalk
- Allows intrasellar herniation of arachnoid with CSF from suprasellar subarachnoid cistern above
- ## Clinical Issues
- Mostly incidental, asymptomatic (adults)
- F:M = 5:1
- Headache, visual disturbances if related to intracranial hypertension
- Frequent endocrine abnormalities in children
# TERMINOLOGY
- ## Abbreviations
- Empty sella (ES)
- ## Definitions
- Herniation of suprasellar arachnoid and cerebrospinal fluid (CSF) through wide diaphragma sellae into bony sella turcica
- Sella turcica is partially filled with CSF
- Rarely completely empty
- Pituitary gland
- Almost never completely absent
- Thin, flattened rim of residual pituitary tissue
- Generally at posteroinferior sellar floor
- Primary or secondary
- Primary empty sella
- Common normal variant (15% of brain MRs), incidental finding
- Normal or increased CSF pressure
- Near-normal volume of compressed pituitary tissue
- No history of trauma, surgery, radiation
- Patients typically endocrinologically normal
- Secondary empty sella
- Many etiologies
- Surgery
- Radiation
- Bromocriptine therapy
- Trauma
- Sheehan syndrome (postpartum pituitary necrosis)
- Pituitary apoplexy
- Pituitary abscess
# IMAGING
- ## General Features
- ### Best diagnostic clue
- Intrasellar CSF with pituitary gland flattened against sellar floor
- Bony sella may be normal or large
- ### Location
- Intrasellar CSF
- ### Size
- Variable
- ## Imaging Recommendations
- ### Best imaging tool
- Sagittal T1WI
- Coronal T2WI
- ## CT Findings
- ### NECT
- CSF-like herniation of CSF into bony sella
- Bony sella typically appears normal
- May also be moderately enlarged (secondary to pulsatile CSF)
- Bony margins intact, not eroded/demineralized
- ### CECT
- Infundibular stalk and pituitary gland enhance normally
- Occasionally intrasellar CSF collection may be asymmetric
- Stalk may appear tilted to one side
- ## MR Findings
- ### T1WI
- Primary empty sella
- Fluid looks exactly like CSF
- Stalk usually midline
- Gland + stalk = anchor sign on coronal imaging
- Stalk may be tilted to one side if intrasellar CSF herniation is asymmetric
- 3rd ventricle, hypothalamus usually normal
- Rare: Herniation of optic chiasm, anterior 3rd ventricle into sella
- Secondary empty sella
- Look for changes of transsphenoidal hypophysectomy
- Defect in sellar floor
- Fat packing
- May cause distortion of stalk, chiasm
- Stalk and pituitary remnant(s) may be scarred/adhesed to side or bottom of sella turcica
- ### T2WI
- Fluid exactly like CSF
- ### FLAIR
- Intrasellar fluid suppresses completely on FLAIR
- ### DWI
- No restriction
- ### T1WI C+
- Primary empty sella
- Stalk, gland enhance normally
- No other abnormalities
- Secondary empty sella
- Gland and stalk may be adhesed/distorted
# DIFFERENTIAL DIAGNOSIS
- [Idiopathic Intracranial Hypertension](/document/idiopathic-intracranial-hypertensi-/d7a0a1b6-1d94-473c-9fe9-021443969f9f)
- Often not truly "idiopathic" (e.g., dural venous sinus stenosis)
- Usually obese female, 20-40 years
- Headache, papilledema
- Intraoptic protrusion of optic nerve head
- Enlarged optic nerve sheaths ± empty sella
- Ventricles may appear slit-like
- Subarachnoid spaces (cisterns, surface sulci) may be small
- ## Secondary Intracranial Hypertension
- Increased intracranial pressure caused by
- Obstructive hydrocephalus (intra-/extraventricular)
- Mass (neoplasm, etc.)
- Dilated anterior recesses of 3rd ventricle herniate into sella
- Look for mass, evidence for transependymal CSF migration
- [Arachnoid Cyst](/document/arachnoid-cyst/d25aaeb3-5b3c-4483-99dc-2757468eedb9)
- Suprasellar arachnoid cyst may herniate into bony sella
- Bony sella often enlarged, eroded/expanded
- Look for 3rd ventricle or optic chiasm displaced by CSF-containing mass
- Cyst walls may be visible on thin-section imaging
- [Pituitary Apoplexy](/document/pituitary-apoplexy/43efc995-d33c-4ac1-be70-e3237eec9fc9)
- Acute: Pituitary gland usually enlarged, not small
- Usually hemorrhagic
- Look for rim enhancement around periphery of enlarged, nonenhancing gland
- Chronic: May cause empty sella
- [Pituitary Anomalies](/document/pituitary-anomalies/09ca9b54-a3d9-43fd-a9cc-4c0212b578a1)
- Ectopic posterior pituitary "bright spot"
- May cause small pituitary gland
- Infundibular stalk short, "stubby"
- Bony sella often small, shallow appearing
- Sella can appear partially empty
- Persisting embryonal infundibular recess of 3rd ventricle
- Can mimic empty sella (rare)
- Pituitary stalk duplication
- Rare
- Look for 2 thin stalks
- Sella may appear partially empty
- [Sheehan Syndrome](/document/pituitary-apoplexy/43efc995-d33c-4ac1-be70-e3237eec9fc9)
- Original clinical description
- Postpartum hemorrhage
- Pituitary necrosis
- Lactation failure
- Hypopituitarism
- Anterior pituitary necrosis
- Leaves small residual pituitary gland
- Result = empty sella
- May occur years after pregnancy
- Slow clinical progression over years suggests factors other than ischemia may be involved
- Necrosis may be caused by antihypothalamus, antipituitary antibodies
- Pituitary autoimmunity may perpetuate hypopituitarism
- [Epidermoid Cyst](/document/epidermoid-cyst/704c5ddf-e1f7-4a5d-a1b8-5b0e603170d9)
- True intrasellar epidermoid cyst very rare
- Off midline > midline
- Usually extension from cerebellopontine angle epidermoid
# PATHOLOGY
- ## General Features
- ### Etiology
- Primary empty sella
- Deficient diaphragma sellae
- Dural covering of sella is incomplete (widened)
- Leaves widened dural opening for infundibular stalk
- Allows intrasellar herniation of arachnoid with CSF from suprasellar subarachnoid cistern above
- Compresses pituitary gland against sellar floor
- Traction on infundibular stalk may cause alteration in visual system
- Pulsatile CSF may gradually enlarge sella
- Secondary empty sella
- Common: Surgery, bromocriptine therapy, radiation
- Less common: Pituitary apoplexy, pituitary abscess
- Rare: Pituitary necrosis in viral hemorrhagic fever (e.g., hanta)
- ## Gross Pathologic & Surgical Features
- Diaphragma sellae appears widened, gaping
- Intrasellar herniation of arachnoid-containing CSF
# CLINICAL ISSUES
- ## Presentation
- ### Most common signs/symptoms
- Incidental, usually asymptomatic
- Headache
- Visual disturbances 1-15%
- Idiopathic intracranial hypertension (IIH)
- Optic chiasm herniation into ES may cause visual symptoms
- Endocrine disturbances
- 20% of adults have subtle laboratory abnormalities
- Majority (70%) of children with ES have endocrine abnormalities
- ## Demographics
- ### Age
- Peak incidence between 50-60 years
- Increased CSF pressure presents earlier (30-40 years)
- ### Sex
- F:M = 5:1
- ### Epidemiology
- 10-15% found incidentally on imaging
- ## Natural History & Prognosis
- Both primary and secondary empty sella usually benign, do not require treatment
- If related to IIH, can result in vision loss or CSF leak
- Hormonal replacement therapy may be required in some cases
- Surgery (rare)
- "Chiasmapexy" to elevate optic chiasm if severe visual disturbances caused by inferior displacement of optic chiasm into empty sella
- CSF rhinorrhea may require surgical intervention
# DIAGNOSTIC CHECKLIST
- ## Consider
- Incidental, normal variant in older adults
- Additional findings of IIH in younger females (e.g., dilated optic nerve sheaths, papilledema, dural venous sinus narrowing)
- Look for endocrine abnormalities in children
- ## Image Interpretation Pearls
- Intrasellar fluid follows CSF **exactly**on all sequences
8bb93cd6-d836-4878-89c3-865ebc070aea
## References
# Selected References
1. [Byrne N et al: Symptomatic primary tethered optic chiasm: Technical case report. Oper Neurosurg (Hagerstown). ePub, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=32386310%5Bpmid%5D)
1. [Chen H et al: A case report of empty Sella syndrome secondary to Hantaan virus infection and review of the literature. Medicine (Baltimore). 99(14):e19734, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=32243412%5Bpmid%5D)
1. [Guinto G et al: Osseous remodeling technique of the sella turcica: a new surgical option for primary empty sella syndrome. World Neurosurg. 126:e953-8, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=30877013%5Bpmid%5D)
1. [Kirigin Biloš LS et al: Empty sella in the making. World Neurosurg. 128:366-70, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31128314%5Bpmid%5D)
1. [Rehder D: Idiopathic intracranial hypertension: Review of clinical syndrome, imaging findings, and treatment. Curr Probl Diagn Radiol. ePub, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31056359%5Bpmid%5D)
1. [Seo YS et al: Bitemporal hemianopsia associated with empty sella syndrome. J Craniofac Surg. 30(8):2660-1, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31449212%5Bpmid%5D)
1. [Atci IB et al: Prognosis of hormonal deficits in empty sella syndrome using neuroimaging. Asian J Neurosurg. 13(3):737-41, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=30283536%5Bpmid%5D)
1. [Auer MK et al: Primary empty sella syndrome and the prevalence of hormonal dysregulation. Dtsch Arztebl Int. 115(7):99-105, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=29510819%5Bpmid%5D)
1. [Barzaghi LR et al: Treatment of empty sella associated with visual impairment: a systematic review of chiasmapexy techniques. Pituitary. 21(1):98-106, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=29027644%5Bpmid%5D)
1. [Chiloiro S et al: Diagnosis of endocrine disease: Primary empty sella: A comprehensive review. Eur J Endocrinol. ePub, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=28780516%5Bpmid%5D)
1. [Kyung SE et al: Enlargement of the sella turcica in pseudotumor cerebri. J Neurosurg. 120(2):538-42, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=24313606%5Bpmid%5D)
1. [Saindane AM et al: Factors determining the clinical significance of an "empty" sella turcica. AJR Am J Roentgenol. 200(5):1125-31, 2013](http://www.ncbi.nlm.nih.gov/pubmed/?term=23617499%5Bpmid%5D)
## Images
### Selected Images
![Sagittal graphic shows an empty sella (ES). The extension of arachnoid with CSF through the diaphragma sellae <img src='img/arrows/WS.png'/> flattens and displaces the pituitary gland <img src='img/arrows/BO.png'/> posteroinferiorly against the sellar floor.](images/app.statdx.com_image_thumbnail_6004ed79-2fa0-49f7-940c-bd93c8468a05_size_168_quality_85_f648e337_20251014T204947Z.jpg)
*Sagittal graphic shows an empty sella (ES). The extension of arachnoid with CSF through the diaphragma sellae <img src='img/arrows/WS.png'/> flattens and displaces the pituitary gland <img src='img/arrows/BO.png'/> posteroinferiorly against the sellar floor.*
![Sagittal graphic shows an empty sella (ES). The extension of arachnoid with CSF through the diaphragma sellae <img src='img/arrows/WS.png'/> flattens and displaces the pituitary gland <img src='img/arrows/BO.png'/> posteroinferiorly against the sellar floor.](images/app.statdx.com_image_thumbnail_6004ed79-2fa0-49f7-940c-bd93c8468a05_size_174_quality_85_c0f2247b.jpg)
*Sagittal graphic shows an empty sella (ES). The extension of arachnoid with CSF through the diaphragma sellae <img src='img/arrows/WS.png'/> flattens and displaces the pituitary gland <img src='img/arrows/BO.png'/> posteroinferiorly against the sellar floor.*
![Sagittal T1 MR in a 59-year-old woman with blurry vision and a primary hypopituitarism shows an enlarged, mostly CSF-filled sella turcica <img src='img/arrows/WC.png'/>. The pituitary gland appears flattened along the sellar floor and a posterior pituitary &quot;bright spot&quot; is absent.](images/app.statdx.com_image_thumbnail_f16f23ba-0444-41be-a643-31d4421376f1_size_168_quality_85_17ebc893_20251014T204947Z.jpg)
*Sagittal T1 MR in a 59-year-old woman with blurry vision and a primary hypopituitarism shows an enlarged, mostly CSF-filled sella turcica <img src='img/arrows/WC.png'/>. The pituitary gland appears flattened along the sellar floor and a posterior pituitary &quot;bright spot&quot; is absent.*
![Sagittal T1 C+ FS MR in the same patient shows a thin rim of enhancing pituitary gland <img src='img/arrows/WO.png'/> compressed against the expanded sellar floor. The infundibulum is kinked over the dorsum sellae <img src='img/arrows/WC.png'/>.](images/app.statdx.com_image_thumbnail_497bac8b-b19c-47fb-9f2e-73b3c20c7db8_size_168_quality_85_287c11b8_20251014T204947Z.jpg)
*Sagittal T1 C+ FS MR in the same patient shows a thin rim of enhancing pituitary gland <img src='img/arrows/WO.png'/> compressed against the expanded sellar floor. The infundibulum is kinked over the dorsum sellae <img src='img/arrows/WC.png'/>.*
![Coronal T2 MR in the same patient shows the expanded sella has a thin rim of compressed pituitary gland lining the sellar floor <img src='img/arrows/WO.png'/>. The sella is filled with CSF exactly the same signal intensity as the fluid in Meckel caves and lateral ventricles. The diagnosis was primary ES.](images/app.statdx.com_image_thumbnail_f8594f4b-c6a7-4b8c-bcff-98361c087d66_size_168_quality_85_5a29d33e_20251014T204947Z.jpg)
*Coronal T2 MR in the same patient shows the expanded sella has a thin rim of compressed pituitary gland lining the sellar floor <img src='img/arrows/WO.png'/>. The sella is filled with CSF exactly the same signal intensity as the fluid in Meckel caves and lateral ventricles. The diagnosis was primary ES.*
![Sagittal T1 MR shows an incidental finding of a partially ES <img src='img/arrows/WC.png'/>.](images/app.statdx.com_image_thumbnail_b6dc3f3c-5d53-4c29-b91d-6fac6c61019c_size_168_quality_85_6ebec5d3_20251014T204947Z.jpg)
*Sagittal T1 MR shows an incidental finding of a partially ES <img src='img/arrows/WC.png'/>.*
![Coronal T1 C+ MR shows the enhancing infundibular stalk and pituitary gland <img src='img/arrows/WS.png'/>. This configuration with the stalk in the midline and a curvilinear pituitary gland has been called the anchor sign because of its resemblance to a ship's anchor <img src='img/arrows/WC.png'/>.](images/app.statdx.com_image_thumbnail_f2bd9c0e-0713-4235-adaa-5fc1035bcc0f_size_168_quality_85_d55ccbd8_20251014T204947Z.jpg)
*Coronal T1 C+ MR shows the enhancing infundibular stalk and pituitary gland <img src='img/arrows/WS.png'/>. This configuration with the stalk in the midline and a curvilinear pituitary gland has been called the anchor sign because of its resemblance to a ship's anchor <img src='img/arrows/WC.png'/>.*
![Sagittal T1 MR shows a 40-year-old man with an incidentally found partially ES. No endocrine laboratory abnormalities were reported. Notice the enlarged, bony sella with thin rim of pituitary tissue <img src='img/arrows/CO.png'/>.](images/app.statdx.com_image_thumbnail_b128cfaf-e8be-44d2-bde3-997afb4245e8_size_168_quality_85_e5a5e2b9_20251014T204947Z.jpg)
*Sagittal T1 MR shows a 40-year-old man with an incidentally found partially ES. No endocrine laboratory abnormalities were reported. Notice the enlarged, bony sella with thin rim of pituitary tissue <img src='img/arrows/CO.png'/>.*
![Axial T2 MR in the same patient shows the bony sella is expanded and filled with CSF <img src='img/arrows/WO.png'/>. A normal pituitary infundibulum <img src='img/arrows/BC.png'/> is present in the midline.](images/app.statdx.com_image_thumbnail_d9289536-d0de-4c2b-9553-2983a5375758_size_168_quality_85_97f163d7_20251014T204947Z.jpg)
*Axial T2 MR in the same patient shows the bony sella is expanded and filled with CSF <img src='img/arrows/WO.png'/>. A normal pituitary infundibulum <img src='img/arrows/BC.png'/> is present in the midline.*
![Sagittal T1 C+ FS MR shows an ES secondary to surgery for pituitary macroadenoma. There is little pituitary tissue apparent along the enlarged sellar floor <img src='img/arrows/CO.png'/>.](images/app.statdx.com_image_thumbnail_78e22665-2399-4ec9-b3a4-caa71dddb27a_size_168_quality_85_0f2d43d0_20251014T204947Z.jpg)
*Sagittal T1 C+ FS MR shows an ES secondary to surgery for pituitary macroadenoma. There is little pituitary tissue apparent along the enlarged sellar floor <img src='img/arrows/CO.png'/>.*
![Coronal T2 MR of a secondary ES in the same patient demonstrates that the sella is filled with CSF <img src='img/arrows/CO.png'/>. Note the thinned optic chiasm <img src='img/arrows/CS.png'/> retracted downward toward the sella.](images/app.statdx.com_image_thumbnail_adb74bf5-9d6c-4d1a-9d19-a37df5eb661e_size_168_quality_85_9eebf5fc_20251014T204947Z.jpg)
*Coronal T2 MR of a secondary ES in the same patient demonstrates that the sella is filled with CSF <img src='img/arrows/CO.png'/>. Note the thinned optic chiasm <img src='img/arrows/CS.png'/> retracted downward toward the sella.*
### Additional Images
![Axial gross pathology shows a primary empty sella found incidentally at autopsy. Note the wide opening of the diaphragma sellae <img src='img/arrows/BO.png'/> and CSF <img src='img/arrows/BC.png'/> largely filling the bony sella. (Courtesy M. Sage, MD.)](images/app.statdx.com_image_thumbnail_45b8f767-808f-48ab-81a5-e0430efa67d4_size_168_quality_85_7e78824d_20251014T204947Z.jpg)
*Axial gross pathology shows a primary empty sella found incidentally at autopsy. Note the wide opening of the diaphragma sellae <img src='img/arrows/BO.png'/> and CSF <img src='img/arrows/BC.png'/> largely filling the bony sella. (Courtesy M. Sage, MD.)*
![Low-power micropathology shows primary empty sella with downward herniation of CSF-filled subarachnoid space <img src='img/arrows/BS.png'/> into the sella. The pituitary gland <img src='img/arrows/BO.png'/> is flattened against the sellar floor. (Courtesy W. Kucharczyk, MD.)](images/app.statdx.com_image_thumbnail_064b097f-a1e6-43cb-ace5-1f9286121aef_size_168_quality_85_241b06a6_20251014T204947Z.jpg)
*Low-power micropathology shows primary empty sella with downward herniation of CSF-filled subarachnoid space <img src='img/arrows/BS.png'/> into the sella. The pituitary gland <img src='img/arrows/BO.png'/> is flattened against the sellar floor. (Courtesy W. Kucharczyk, MD.)*
![Coronal T2 MR at 3T shows CSF within the sella turcica <img src='img/arrows/CS.png'/> surrounding the infundibular stalk <img src='img/arrows/CC.png'/>. The pituitary gland <img src='img/arrows/CO.png'/> is flattened against the sellar floor in this patient with a primary empty sella.](images/app.statdx.com_image_thumbnail_48de841a-aa52-4a23-9d05-d3489b4332fd_size_168_quality_85_8d02aadd_20251014T204947Z.jpg)
*Coronal T2 MR at 3T shows CSF within the sella turcica <img src='img/arrows/CS.png'/> surrounding the infundibular stalk <img src='img/arrows/CC.png'/>. The pituitary gland <img src='img/arrows/CO.png'/> is flattened against the sellar floor in this patient with a primary empty sella.*
![Sagittal T1 MR demonstrates a thinned pituitary gland <img src='img/arrows/CS.png'/> along the floor of the mostly empty sella in a 34-year-old woman who had postpartum anterior pituitary gland necrosis (Sheehan syndrome) 10 years prior to imaging.](images/app.statdx.com_image_thumbnail_879f291a-c195-4e96-a229-8198ebbb09a8_size_168_quality_85_4589ec3f_20251014T204947Z.jpg)
*Sagittal T1 MR demonstrates a thinned pituitary gland <img src='img/arrows/CS.png'/> along the floor of the mostly empty sella in a 34-year-old woman who had postpartum anterior pituitary gland necrosis (Sheehan syndrome) 10 years prior to imaging.*
![Coronal T2 MR in the same patient shows a thin, nearly inapparent pituitary gland remnant along the sellar floor <img src='img/arrows/CS.png'/>. The history distinguishes Sheehan syndrome from an incidental finding of partial empty sella.](images/app.statdx.com_image_thumbnail_5a4c3bfe-473e-44c8-8a0a-4bb0385ecfba_size_168_quality_85_001db1f0_20251014T204947Z.jpg)
*Coronal T2 MR in the same patient shows a thin, nearly inapparent pituitary gland remnant along the sellar floor <img src='img/arrows/CS.png'/>. The history distinguishes Sheehan syndrome from an incidental finding of partial empty sella.*
![Sagittal T1 C+ MR shows a 71-year-old woman with a sellar and suprasellar arachnoid cyst mimicking an empty sella. Note the normally enhancing pituitary infundibulum <img src='img/arrows/CS.png'/> and pituitary tissue <img src='img/arrows/CO.png'/> displaced anteriorly by the CSF intensity arachnoid cyst.](images/app.statdx.com_image_thumbnail_018ed755-930e-45c6-8df5-a51132bfc156_size_168_quality_85_8a2100f0_20251014T204947Z.jpg)
*Sagittal T1 C+ MR shows a 71-year-old woman with a sellar and suprasellar arachnoid cyst mimicking an empty sella. Note the normally enhancing pituitary infundibulum <img src='img/arrows/CS.png'/> and pituitary tissue <img src='img/arrows/CO.png'/> displaced anteriorly by the CSF intensity arachnoid cyst.*
![Sagittal T2 MR in the same patient shows the lack of CSF flow artifact within the cyst <img src='img/arrows/CS.png'/> compared to the flow within the suprasellar and interpeduncular cisterns <img src='img/arrows/CO.png'/>.](images/app.statdx.com_image_thumbnail_b2ea973a-5da3-4acf-8b1d-2f2e0e92b9ed_size_168_quality_85_e93c7292_20251014T204947Z.jpg)
*Sagittal T2 MR in the same patient shows the lack of CSF flow artifact within the cyst <img src='img/arrows/CS.png'/> compared to the flow within the suprasellar and interpeduncular cisterns <img src='img/arrows/CO.png'/>.*
![Coronal T2 MR shows primary empty sella seen as incidental finding on screening IAC MR. The sella is filled with CSF and the pituitary gland <img src='img/arrows/WS.png'/> is flattened against the sellar floor.](images/app.statdx.com_image_thumbnail_19ea42e4-a0d1-4571-952b-843dc54e0011_size_168_quality_85_9174edff_20251014T204947Z.jpg)
*Coronal T2 MR shows primary empty sella seen as incidental finding on screening IAC MR. The sella is filled with CSF and the pituitary gland <img src='img/arrows/WS.png'/> is flattened against the sellar floor.*
docs_md/articles/enhancing-suprasellar-mass_528eaea9-095f-4fab-ad42-5ad84e215a2d.md
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---
# ESSENTIAL INFORMATION
- ## Key Differential Diagnosis Issues
- Effect of age on differential diagnosis important
- Common lesions ("big 5") account for > 75% of all suprasellar masses
- Most other lesions < 1-2% each
- Differential diagnosis narrows if mass confined to infundibulum
- ## Helpful Clues for Common Diagnoses
- **Pituitary Macroadenoma vs. Meningioma**
- Macroadenoma: Gland cannot be identified separate from mass
- Meningioma: Mass distinct from gland (diaphragma sellae separates mass above from pituitary below)
- **S****accular****Aneurysm**
- Coronal plane helps distinguish aneurysm from pituitary
- Look for phase artifact, flow void on MR
- **Craniopharyngioma**
- Adamantinomatous: 90% Ca⁺⁺, 90% cystic, 90% enhance
- Papillary may be solid, noncalcified, enhance strongly
- **Pilocytic Astrocytoma**
- More common in children than adults
- T2 hyperintense, variable enhancement
- Expands hypothalamus, optic chiasm
- May extend into optic nerves/tracts
- **Pilomyxoid Astrocytoma**
- Subtype of pilocytic astrocytoma, more aggressive
- Infant > child
- Large, bulky tumor with lateral extension to temporal lobe common
- Hemorrhage in 20-25%
- ## Helpful Clues for Less Common Diagnoses
- **Pituitary Hyperplasia**
- May be physiologic (pregnancy, lactation, puberty)
- Pathologic in end-organ failure (thyroid, ovarian, etc.)
- **Neurosarcoid**
- Older patients, enhancing mass
- **Langerhans Cell Histiocytosis**
- Children and young adults; enhancing mass
- May cause thickened infundibulum
- Absence of pituitary "bright spot"
- **Germinoma**
- Children > > adults; enhancing mass
- CT hyperdense
- May cause thickened infundibulum
- Absence of the pituitary "bright spot"
- **Lymphocytic Hypophysitis**
- Can mimic pituitary adenoma on imaging
- Most common in peripartum female
- May be autoimmune, inflammatory, granulomatous process, drug related
- May be secondary to checkpoint inhibitor therapy
- **Astrocytoma, IDH-Mutant**
- May affect hypothalamic and suprasellar regions
- T2 hyperintense; variable enhancement
- WHO grades 2-4
- ## Helpful Clues for Rare Diagnoses
- Solitary**metastasis** to gland &/or stalk rare
- **Lymphoma**, **leukemia** usually with systemic disease
- **Pituicytoma, g****ranular cell tumor**, and **spindle cell oncocytoma** are rare tumors, which may occur along infundibulum &/or posterior pituitary or may mimic macroadenoma
- ## Alternative Differential Approaches
- **Adult**: Consider adenoma, meningioma, and aneurysm
- **Child**: Craniopharyngioma, astrocytoma, Langerhans cell histiocytosis, and germinoma
## References
# Selected References
1. [Kinoshita Y et al: Natural course of Rathke's cleft cysts and risk factors for progression. J Neurosurg. 1-7, 2022](http://www.ncbi.nlm.nih.gov/pubmed/?term=36057119%5Bpmid%5D)
1. [Pascual JM et al: Duct-like recess in the infundibular portion of third ventricle craniopharyngiomas: an MRI sign identifying the papillary type. AJNR Am J Neuroradiol. 43(9):1333-40, 2022](http://www.ncbi.nlm.nih.gov/pubmed/?term=35953277%5Bpmid%5D)
1. [Poyuran R et al: Nonneoplastic and noninfective cysts of the central nervous system: a histopathological study. Neuropathology. ePub, 2022](http://www.ncbi.nlm.nih.gov/pubmed/?term=36210745%5Bpmid%5D)
1. [Jipa A et al: Imaging of the sellar and parasellar regions. Clin Imaging. 77:254-75, 2021](http://www.ncbi.nlm.nih.gov/pubmed/?term=34153590%5Bpmid%5D)
1. [Kurokawa R et al: MRI findings of immune checkpoint inhibitor-induced hypophysitis: possible association with fibrosis. AJNR Am J Neuroradiol. 41(9):1683-9, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=32763900%5Bpmid%5D)
1. [Go JL et al: Imaging of the sella and parasellar region. Radiol Clin North Am. 55(1):83-101, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=27890190%5Bpmid%5D)
1. [Seeburg DP et al: Imaging of the sella and parasellar region in the pediatric population. Neuroimaging Clin N Am. 27(1):99-121, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=27889026%5Bpmid%5D)
1. [Zamora C et al: Sellar and parasellar imaging. Neurosurgery. 80(1):17-38, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=28362892%5Bpmid%5D)
1. [Patel KS et al: Intraoperative magnetic resonance imaging assessment of non-functioning pituitary adenomas during transsphenoidal surgery. Pituitary. 19(2):222-31, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=26323592%5Bpmid%5D)
1. [Petrakakis I et al: The sellar and suprasellar region: a "hideaway" of rare lesions. Clinical aspects, imaging findings, surgical outcome and comparative analysis. Clin Neurol Neurosurg. 149:154-65, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=27540757%5Bpmid%5D)
1. [Wang J et al: The clinicopathological features of pituicytoma and the differential diagnosis of sellar glioma. Neuropathology. 36(5):432-440, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=26919073%5Bpmid%5D)
1. [Wu AW et al: Chondroid chordoma of the sella turcica mimicking a pituitary adenoma. Ear Nose Throat J. 94(10-11):E47-9, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=26535833%5Bpmid%5D)
1. DW Louis, et. al (Editors). WHO Classification of Tumours of the Central Nervous System. International Agency for Research on Cancer, Lyon, 2016. Chapter 17: Tumours of the sellar region, pp 323-334.
## Images
### Selected Images
![Coronal T1 C+ MR shows an enhancing intrasellar and suprasellar mass <img src='img/arrows/CS.png'/>. The pituitary gland cannot be separated from the mass and indeed is the mass. Note the superior displacement of the optic chiasm <img src='img/arrows/WS.png'/> by the macroadenoma.](images/app.statdx.com_image_thumbnail_1cbcbdf8-0e55-4af0-b4ff-011551b12f25_annotated_true_size_900_quality_90_c8f5de23.jpg)
**Pituitary Macroadenoma**
*Coronal T1 C+ MR shows an enhancing intrasellar and suprasellar mass <img src='img/arrows/CS.png'/>. The pituitary gland cannot be separated from the mass and indeed is the mass. Note the superior displacement of the optic chiasm <img src='img/arrows/WS.png'/> by the macroadenoma.*
![Coronal T1 C+ MR shows an enhancing intrasellar and suprasellar mass <img src='img/arrows/CS.png'/>. The pituitary gland cannot be separated from the mass and indeed is the mass. Note the superior displacement of the optic chiasm <img src='img/arrows/WS.png'/> by the macroadenoma.](images/app.statdx.com_image_thumbnail_1cbcbdf8-0e55-4af0-b4ff-011551b12f25_size_174_quality_85_81a0e030.jpg)
**Pituitary Macroadenoma**
*Coronal T1 C+ MR shows an enhancing intrasellar and suprasellar mass <img src='img/arrows/CS.png'/>. The pituitary gland cannot be separated from the mass and indeed is the mass. Note the superior displacement of the optic chiasm <img src='img/arrows/WS.png'/> by the macroadenoma.*
![Coronal T1 C+ FS MR shows a suprasellar enhancing mass <img src='img/arrows/CO.png'/> separate from the normal pituitary gland below <img src='img/arrows/WC.png'/>. Meningiomas often have associated hyperostosis of the adjacent bone. Additionally, these benign tumors enhance homogeneously and may have an associated dural tail.](images/app.statdx.com_image_thumbnail_e113dc69-0754-4224-952a-c02882a414df_annotated_true_size_900_quality_90_3104d9f2.jpg)
**Meningioma**
*Coronal T1 C+ FS MR shows a suprasellar enhancing mass <img src='img/arrows/CO.png'/> separate from the normal pituitary gland below <img src='img/arrows/WC.png'/>. Meningiomas often have associated hyperostosis of the adjacent bone. Additionally, these benign tumors enhance homogeneously and may have an associated dural tail.*
![Axial T1 C+ MR shows a suprasellar enhancing mass <img src='img/arrows/WS.png'/> with prominent phase artifact <img src='img/arrows/WO.png'/> caused by large basilar tip aneurysm with slow intraluminal flow. Aneurysms are the 3rd most common suprasellar mass in an adult.](images/app.statdx.com_image_thumbnail_67a712f3-90f1-4ab8-b4c4-c2c5a25c9e4a_annotated_true_size_900_quality_90_1ca9fa52.jpg)
**Saccular Aneurysm**
*Axial T1 C+ MR shows a suprasellar enhancing mass <img src='img/arrows/WS.png'/> with prominent phase artifact <img src='img/arrows/WO.png'/> caused by large basilar tip aneurysm with slow intraluminal flow. Aneurysms are the 3rd most common suprasellar mass in an adult.*
![Sagittal T1 C+ MR shows an enhancing, partially solid and cystic suprasellar mass <img src='img/arrows/WS.png'/> related to an adamantinomatous craniopharyngioma. These WHO grade 1 tumors are the most common pediatric intracranial tumor of nonglial origin.](images/app.statdx.com_image_thumbnail_f6f7ba93-35d2-452a-a93b-59fd3ecbf369_annotated_true_size_900_quality_90_7d308f19.jpg)
**Craniopharyngioma**
*Sagittal T1 C+ MR shows an enhancing, partially solid and cystic suprasellar mass <img src='img/arrows/WS.png'/> related to an adamantinomatous craniopharyngioma. These WHO grade 1 tumors are the most common pediatric intracranial tumor of nonglial origin.*
![Coronal T1 C+ FS MR shows a heterogeneously enhancing suprasellar mass <img src='img/arrows/CO.png'/> in a child that was found to represent a pilomyxoid astrocytoma, WHO grade 1. Pilomyxoid astrocytoma is a subtype of pilocytic astrocytoma that is often more aggressive.](images/app.statdx.com_image_thumbnail_865ed7c4-c2cb-420f-8b61-cacdfa6fa69f_annotated_true_size_900_quality_90_dd99cab0.jpg)
**Pilomyxoid Astrocytoma**
*Coronal T1 C+ FS MR shows a heterogeneously enhancing suprasellar mass <img src='img/arrows/CO.png'/> in a child that was found to represent a pilomyxoid astrocytoma, WHO grade 1. Pilomyxoid astrocytoma is a subtype of pilocytic astrocytoma that is often more aggressive.*
![Axial T1 C+ FS MR shows a lobular, enhancing suprasellar mass <img src='img/arrows/CO.png'/> related to neurosarcoid. Patients with neurosarcoid often have associated pulmonary hilar adenopathy.](images/app.statdx.com_image_thumbnail_f161ad8d-60f1-49e1-9115-266cfd01a65d_annotated_true_size_900_quality_90_6a020174.jpg)
**Neurosarcoid**
*Axial T1 C+ FS MR shows a lobular, enhancing suprasellar mass <img src='img/arrows/CO.png'/> related to neurosarcoid. Patients with neurosarcoid often have associated pulmonary hilar adenopathy.*
![Axial T1 C+ FS MR shows a diffusely enhancing suprasellar mass <img src='img/arrows/CO.png'/> in a young adult who first presented with diabetes insipidus and a thick, enhancing infundibulum with absence of a posterior pituitary bright spot. Nine months later, patient returned with a much larger suprasellar mass.](images/app.statdx.com_image_thumbnail_64976a79-72bf-485e-a4dc-14450487874d_annotated_true_size_900_quality_90_1bbcaf1c.jpg)
**Langerhans Cell Histiocytosis**
*Axial T1 C+ FS MR shows a diffusely enhancing suprasellar mass <img src='img/arrows/CO.png'/> in a young adult who first presented with diabetes insipidus and a thick, enhancing infundibulum with absence of a posterior pituitary bright spot. Nine months later, patient returned with a much larger suprasellar mass.*
![Coronal T2 MR in a pregnant patient with acute vision problems shows a sellar and suprasellar mass <img src='img/arrows/CO.png'/> with superior displacement of the optic chiasm <img src='img/arrows/CS.png'/>. Imaging of lymphocytic hypophysitis may mimic a pituitary macroadenoma.](images/app.statdx.com_image_thumbnail_0276cc0e-edd9-4087-b2e9-e34c7b8357e9_annotated_true_size_900_quality_90_38ff17be.jpg)
**Lymphocytic Hypophysitis**
*Coronal T2 MR in a pregnant patient with acute vision problems shows a sellar and suprasellar mass <img src='img/arrows/CO.png'/> with superior displacement of the optic chiasm <img src='img/arrows/CS.png'/>. Imaging of lymphocytic hypophysitis may mimic a pituitary macroadenoma.*
### Additional Images
![Coronal T1 C+ MR shows an inhomogeneously enhancing intra- and suprasellar mass <img src='img/arrows/WS.png'/>; the pituitary gland cannot be found separate from it and indeed is the mass.](images/app.statdx.com_image_thumbnail_39de1cf7-b4c6-4e2c-bd5f-1a3c3569676b_annotated_true_size_900_quality_90_ec755e9e.jpg)
**Pituitary Macroadenoma**
*Coronal T1 C+ MR shows an inhomogeneously enhancing intra- and suprasellar mass <img src='img/arrows/WS.png'/>; the pituitary gland cannot be found separate from it and indeed is the mass.*
![Coronal T1 C+ FS MR shows a suprasellar enhancing mass <img src='img/arrows/WO.png'/> separated from the normal pituitary gland below <img src='img/arrows/WC.png'/> by a thin black line of diaphragma sellae <img src='img/arrows/WS.png'/>.](images/app.statdx.com_image_thumbnail_247d91b3-9c11-483a-8868-9bae8ccfafe0_annotated_true_size_900_quality_90_e6aeeb67.jpg)
**Meningioma**
*Coronal T1 C+ FS MR shows a suprasellar enhancing mass <img src='img/arrows/WO.png'/> separated from the normal pituitary gland below <img src='img/arrows/WC.png'/> by a thin black line of diaphragma sellae <img src='img/arrows/WS.png'/>.*
![Coronal T1 C+ MR in a child shows a intra-/suprasellar mass. An apical nonenhancing portion is surrounded by a thin enhancing rim <img src='img/arrows/WC.png'/>. The solid portion enhances strongly but heterogeneously <img src='img/arrows/WS.png'/>.](images/app.statdx.com_image_thumbnail_86351338-7652-4b99-974a-ca19377a7151_annotated_true_size_900_quality_90_3e5677ce.jpg)
**Craniopharyngioma**
*Coronal T1 C+ MR in a child shows a intra-/suprasellar mass. An apical nonenhancing portion is surrounded by a thin enhancing rim <img src='img/arrows/WC.png'/>. The solid portion enhances strongly but heterogeneously <img src='img/arrows/WS.png'/>.*
![Sagittal T1 C+ FS MR shows a papillary craniopharyngioma in an adult man. Papillary craniopharyngiomas are most often solid masses.](images/app.statdx.com_image_thumbnail_999670ec-804a-4625-8f6d-5cc8870f28d5_annotated_true_size_900_quality_90_fcc2f2b4.jpg)
**Craniopharyngioma**
*Sagittal T1 C+ FS MR shows a papillary craniopharyngioma in an adult man. Papillary craniopharyngiomas are most often solid masses.*
![Coronal T1 C+ MR shows an enhancing suprasellar mass <img src='img/arrows/WS.png'/> separate from the pituitary gland below <img src='img/arrows/WO.png'/>. Note involvement of the optic chiasm <img src='img/arrows/WC.png'/>.](images/app.statdx.com_image_thumbnail_ec568e64-0e9e-4b1f-aecd-f1a34604ede5_annotated_true_size_900_quality_90_796aa48d.jpg)
**Astrocytoma, IDH-Mutant**
*Coronal T1 C+ MR shows an enhancing suprasellar mass <img src='img/arrows/WS.png'/> separate from the pituitary gland below <img src='img/arrows/WO.png'/>. Note involvement of the optic chiasm <img src='img/arrows/WC.png'/>.*
![Sagittal T1 C+ MR in a 3-year-old boy with rotary nystagmus and vomiting shows a large suprasellar mass <img src='img/arrows/WS.png'/> clearly separate from the pituitary gland below <img src='img/arrows/BO.png'/>.](images/app.statdx.com_image_thumbnail_ff3cfeac-da02-4993-b553-b3c4956a9a56_annotated_true_size_900_quality_90_da128db3.jpg)
**Pilocytic Astrocytoma**
*Sagittal T1 C+ MR in a 3-year-old boy with rotary nystagmus and vomiting shows a large suprasellar mass <img src='img/arrows/WS.png'/> clearly separate from the pituitary gland below <img src='img/arrows/BO.png'/>.*
![Axial T1 C+ FS MR shows an enhancing suprasellar mass involving the optic chiasm <img src='img/arrows/WS.png'/> and hypothalamus <img src='img/arrows/WO.png'/> with pial enhancement along the inferior frontal lobe <img src='img/arrows/WC.png'/>. The lesion also involved the optic nerve.](images/app.statdx.com_image_thumbnail_0af02be6-d5be-4512-b078-070b8d786e32_annotated_true_size_900_quality_90_37aefa5d.jpg)
**Neurosarcoid**
*Axial T1 C+ FS MR shows an enhancing suprasellar mass involving the optic chiasm <img src='img/arrows/WS.png'/> and hypothalamus <img src='img/arrows/WO.png'/> with pial enhancement along the inferior frontal lobe <img src='img/arrows/WC.png'/>. The lesion also involved the optic nerve.*
![Coronal T1 C+ FS MR in 22-year-old man with diabetes insipidus shows hydrocephalus and an enhancing sellar/suprasellar mass. Germinoma commonly presents with diabetes insipidus. A macroadenoma often has visual defects.](images/app.statdx.com_image_thumbnail_21a4e733-abbb-4549-a086-866e1b3c94d3_annotated_true_size_900_quality_90_d56cd73d.jpg)
**Germinoma**
*Coronal T1 C+ FS MR in 22-year-old man with diabetes insipidus shows hydrocephalus and an enhancing sellar/suprasellar mass. Germinoma commonly presents with diabetes insipidus. A macroadenoma often has visual defects.*
![Coronal T1 C+ MR shows a diffusely enhancing suprasellar mass <img src='img/arrows/CO.png'/> that involves the infundibulum. A pituicytoma was diagnosed at resection. These rare tumors are WHO grade 1 and usually arise along the infundibulum or neurohypophysis. (Courtesy A.V. Hasso, MD.)](images/app.statdx.com_image_thumbnail_163414a6-9c5e-42a5-af16-2800a1f2006c_annotated_true_size_900_quality_90_5044c664.jpg)
**Rare Sellar Region Tumor (Pituicytoma, Granular Cell Tumor, Spindle Cell Oncocytoma)**
*Coronal T1 C+ MR shows a diffusely enhancing suprasellar mass <img src='img/arrows/CO.png'/> that involves the infundibulum. A pituicytoma was diagnosed at resection. These rare tumors are WHO grade 1 and usually arise along the infundibulum or neurohypophysis. (Courtesy A.V. Hasso, MD.)*
![Axial T1 C+ FS MR shows a cystic and solid suprasellar enhancing mass <img src='img/arrows/CS.png'/>. In an adult, craniopharyngiomas may be adamantinomatous or papillary types. Adamantinomatous craniopharyngiomas enhance, calcify, and have cysts. Papillary craniopharyngiomas are commonly solid and enhancing. These are WHO grade 1 tumors.](images/app.statdx.com_image_thumbnail_0b04c2f6-f090-4827-ba56-400f1bb52a17_annotated_true_size_900_quality_90_59ceca3c.jpg)
**Craniopharyngioma**
*Axial T1 C+ FS MR shows a cystic and solid suprasellar enhancing mass <img src='img/arrows/CS.png'/>. In an adult, craniopharyngiomas may be adamantinomatous or papillary types. Adamantinomatous craniopharyngiomas enhance, calcify, and have cysts. Papillary craniopharyngiomas are commonly solid and enhancing. These are WHO grade 1 tumors.*
![Coronal T1 C+ FS MR in 25-year-old woman with visual complaints shows an enhancing suprasellar mass <img src='img/arrows/WS.png'/> that partially encases the optic chiasm <img src='img/arrows/WC.png'/>. There is also a parasellar cystic portion <img src='img/arrows/WO.png'/> of the mass. Pilocytic astrocytomas often present as a solid and cystic cerebellar mass in a child or young adult. These WHO grade 1 tumors are typically T2 hyperintense with variable enhancement.](images/app.statdx.com_image_thumbnail_219d69b0-067c-41c4-8aa4-89ca5bf2cb0b_annotated_true_size_900_quality_90_e0824e5a.jpg)
**Pilocytic Astrocytoma**
*Coronal T1 C+ FS MR in 25-year-old woman with visual complaints shows an enhancing suprasellar mass <img src='img/arrows/WS.png'/> that partially encases the optic chiasm <img src='img/arrows/WC.png'/>. There is also a parasellar cystic portion <img src='img/arrows/WO.png'/> of the mass. Pilocytic astrocytomas often present as a solid and cystic cerebellar mass in a child or young adult. These WHO grade 1 tumors are typically T2 hyperintense with variable enhancement.*
docs_md/articles/enlarged-pituitary-gland_65e54fd1-aecf-463c-bd85-97504c542051.md
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---
title: "Enlarged Pituitary Gland"
docid: "65e54fd1-aecf-463c-bd85-97504c542051"
authors:
- key: "8d5254e9-8dda-478b-8f08-bdee97a32c79"
value: "Karen L. Salzman, MD, FACR"
- key: "5cff4116-3654-4b3a-bb75-5ebe0b8c9850"
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slug: "brain"
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name: "Differential Diagnosis"
slug: "differential-diagnosis"
treeNodeId: "a7fdd139-664e-4bb8-8d18-400e4733ff60"
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name: "Sella/Juxtasellar, Pineal Region"
slug: "sellajuxtasellar-pineal-region"
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documentVersionId: "6624d150-d588-4aed-b1dd-621505522f70"
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lastUpdated: "01/27/23"
pageDescription: "Enlarged Pituitary Gland"
pageKeywords: "Brain, Differential Diagnosis, Sella/Juxtasellar, Pineal Region, Anatomically Based Differentials, Enlarged Pituitary Gland"
pageTitle: "Enlarged Pituitary Gland | STATdx"
enhancedTitle: "Enlarged Pituitary Gland"
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breadcrumbs:
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- "Differential Diagnosis"
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---
# ESSENTIAL INFORMATION
- ## Key Differential Diagnosis Issues
- Not all "enlarged pituitary glands" abnormal
- Size/height varies with sex, age
- Children: 6 mm
- Males, postmenopausal females: 8 mm
- Young, menstruating females: 10-12 mm (can bulge upward)
- Pregnant, postpartum, lactating females: 12-14 mm
- Enhances strongly, uniformly
- 15-20% have incidental cyst or nonfunctioning microadenoma (pituitary "incidentaloma")
- Variants/mimics of "enlarged pituitary"
- Pseudoenlargement secondary to unusually shallow bony sella or "kissing carotids"
- Medially positioned cavernous internal carotid arteries ("kissing carotids") may make gland appear enlarged
- ## Helpful Clues for Common Diagnoses
- **Pituitary****H****yperplasia**
- Can be normal (young, menstruating females)
- Enlarged gland ± upward bulging
- May be related to end-organ failure (i.e., hypothyroidism, ovarian) or neuroendocrine tumors
- **Pituitary****M****icroadenoma**
- May enlarge gland
- Best identified with dynamic, contrast-enhanced MR
- **Pituitary****M****acroadenoma**
- Pituitary gland cannot be distinguished from mass
- Enhances strongly, often heterogeneously
- Typically sellar and suprasellar mass
- ## Helpful Clues for Less Common Diagnoses
- **Lymphocytic Hypophysitis**
- May enlarge pituitary gland, ± infundibulum
- May mimic macroadenoma
- Common in pregnant and postpartum females
- Different types: Autoimmune, granulomatous, IgG4 related, drug related (i.e., ipilimumab)
- **Neurosarcoid**
- Affects hypothalamic-pituitary axis in 50%
- Diffusely enhancing lesion involving stalk ± gland
- **Langerhans Cell Histiocytosis**
- Loss of posterior pituitary bright spot
- Enhancing infundibulum ± gland, hypothalamus
- Young patients
- ## Other Essential Information
- Venous congestion (intracranial hypotension, dural arteriovenous fistula) can enlarge gland
- Caution: Child or young adolescent male with "pituitary adenoma" most likely has pituitary hyperplasia; less likely LCH or germinoma, **not** adenoma
- Evaluate for end-organ failure (e.g., hypothyroidism)
- In **child/young adult**, consider pituitary hyperplasia, LCH, lymphocytic hypophysitis, anatomic variants
- In **adult**, consider pituitary adenoma, hyperplasia, hypophysitis, neurosarcoid, lymphoma, metastatic disease
## References
# Selected References
1. [Bashari WA et al: Modern imaging in Cushing's disease. Pituitary. 25(5):709-12, 2022](http://www.ncbi.nlm.nih.gov/pubmed/?term=35666391%5Bpmid%5D)
1. [Chalif EJ et al: Pituitary adenoma in the elderly: surgical outcomes and treatment trends in the United States. J Neurosurg. 1-12, 2022](http://www.ncbi.nlm.nih.gov/pubmed/?term=35535847%5Bpmid%5D)
1. [Potorac I et al: Pituitary MRI features in acromegaly resulting from ectopic ghrh secretion from a neuroendocrine tumor: analysis of 30 cases. J Clin Endocrinol Metab. 107(8):e3313-20, 2022](http://www.ncbi.nlm.nih.gov/pubmed/?term=35512251%5Bpmid%5D)
1. [Villemaire A et al: Is systematic gadolinium injection relevant during MRI follow-up for non-functioning pituitary macroadenomas? J Neuroradiol. ePub, 2022](http://www.ncbi.nlm.nih.gov/pubmed/?term=36055429%5Bpmid%5D)
1. [Altshuler DB et al: Imaging errors in distinguishing pituitary adenomas from other sellar lesions. J Neuroophthalmol. 41(4):512-18, 2021](http://www.ncbi.nlm.nih.gov/pubmed/?term=33630780%5Bpmid%5D)
1. [Huang J et al: Noncontrast MRI protocol for selected pediatric pituitary endocrinopathies: a procedure with high diagnostic yield and potential to reduce anesthesia and gadolinium-based contrast exposure. AJNR Am J Neuroradiol. 42(10):1884-90, 2021](http://www.ncbi.nlm.nih.gov/pubmed/?term=34475192%5Bpmid%5D)
1. [Mittal PK et al: Role of imaging in the evaluation of male infertility. Radiographics. 160125, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=28410062%5Bpmid%5D)
1. [Zamora C et al: Sellar and parasellar imaging. Neurosurgery. 80(1):17-38, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=28362892%5Bpmid%5D)
1. [Kocova M et al: Diagnostic approach in children with unusual symptoms of acquired hypothyroidism. When to look for pituitary hyperplasia? J Pediatr Endocrinol Metab. 29(3):297-303, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=26587741%5Bpmid%5D)
1. [Fujita A et al: IgG4-related disease of the head and neck: CT and MR imaging manifestations. Radiographics. 32(7):1945-58, 2012](http://www.ncbi.nlm.nih.gov/pubmed/?term=23150850%5Bpmid%5D)
## Images
### Selected Images
![Sagittal T1 MR in a teenager with hypothyroidism shows marked enlargement of the pituitary gland <img src='img/arrows/CS.png'/> related to pituitary hyperplasia. The pituitary gland returned to normal size after the hypothyroidism was treated.](images/app.statdx.com_image_thumbnail_b49dfe71-0acd-4da7-896d-252d39c6afa9_annotated_true_size_900_quality_90_3429edd0.jpg)
**Pituitary Hyperplasia**
*Sagittal T1 MR in a teenager with hypothyroidism shows marked enlargement of the pituitary gland <img src='img/arrows/CS.png'/> related to pituitary hyperplasia. The pituitary gland returned to normal size after the hypothyroidism was treated.*
![Sagittal T1 MR in a teenager with hypothyroidism shows marked enlargement of the pituitary gland <img src='img/arrows/CS.png'/> related to pituitary hyperplasia. The pituitary gland returned to normal size after the hypothyroidism was treated.](images/app.statdx.com_image_thumbnail_b49dfe71-0acd-4da7-896d-252d39c6afa9_size_174_quality_85_5ea31d79.jpg)
**Pituitary Hyperplasia**
*Sagittal T1 MR in a teenager with hypothyroidism shows marked enlargement of the pituitary gland <img src='img/arrows/CS.png'/> related to pituitary hyperplasia. The pituitary gland returned to normal size after the hypothyroidism was treated.*
![Coronal T1 C+ FS MR in a 55-year-old woman shows a mildly enlarged right pituitary gland <img src='img/arrows/CO.png'/> related to a prolactin secreting microadenoma. Microadenomas are benign tumors of the adenohypophysis that are often seen best on dynamic contrast MR.](images/app.statdx.com_image_thumbnail_be60f3b2-a8bc-48ae-95a6-670bacdc91fd_annotated_true_size_900_quality_90_032e1eaa.jpg)
**Pituitary Microadenoma**
*Coronal T1 C+ FS MR in a 55-year-old woman shows a mildly enlarged right pituitary gland <img src='img/arrows/CO.png'/> related to a prolactin secreting microadenoma. Microadenomas are benign tumors of the adenohypophysis that are often seen best on dynamic contrast MR.*
![Coronal T1 C+ MR shows an enlarged pituitary gland <img src='img/arrows/CS.png'/> related to a macroadenoma. Note displacement of the pituitary infundibulum <img src='img/arrows/WS.png'/> to the contralateral side, typical of adenomas.](images/app.statdx.com_image_thumbnail_2c33b2c1-7174-4b33-8f92-868eb2bd2217_annotated_true_size_900_quality_90_57f0b26a.jpg)
**Pituitary Macroadenoma**
*Coronal T1 C+ MR shows an enlarged pituitary gland <img src='img/arrows/CS.png'/> related to a macroadenoma. Note displacement of the pituitary infundibulum <img src='img/arrows/WS.png'/> to the contralateral side, typical of adenomas.*
![Sagittal T1 C+ FS MR shows marked enlargement of the pituitary gland <img src='img/arrows/CS.png'/> and infundibular stalk <img src='img/arrows/CO.png'/> in this patient being treated with Ipilimumab for metastatic melanoma. The drug-related hypophysitis resolved after the therapy was discontinued.](876d829c-f20b-420b-bb3d-3e5d8a9c9a94)
**Lymphocytic Hypophysitis**
*Sagittal T1 C+ FS MR shows marked enlargement of the pituitary gland <img src='img/arrows/CS.png'/> and infundibular stalk <img src='img/arrows/CO.png'/> in this patient being treated with Ipilimumab for metastatic melanoma. The drug-related hypophysitis resolved after the therapy was discontinued.*
![Sagittal T1 C+ MR shows a diffusely enlarged, enhancing pituitary gland <img src='img/arrows/CO.png'/> in a young female with pituitary dysfunction. Neurosarcoid was diagnosed at resection. Imaging mimics the much more common pituitary adenoma.](886ff2c8-29ae-436f-9c76-1f6328a6bbe3)
**Neurosarcoid**
*Sagittal T1 C+ MR shows a diffusely enlarged, enhancing pituitary gland <img src='img/arrows/CO.png'/> in a young female with pituitary dysfunction. Neurosarcoid was diagnosed at resection. Imaging mimics the much more common pituitary adenoma.*
![Sagittal T1 C+ MR shows a uniformly enlarged, enhancing pituitary gland <img src='img/arrows/CO.png'/> in this young adult with known Langerhans cell histiocytosis (LCH). LCH often presents with diabetes insipidus and loss of the normal posterior pituitary bright spot.](2aa9bac7-8e69-4ec5-b4d7-f667c779886b)
**Langerhans Cell Histiocytosis**
*Sagittal T1 C+ MR shows a uniformly enlarged, enhancing pituitary gland <img src='img/arrows/CO.png'/> in this young adult with known Langerhans cell histiocytosis (LCH). LCH often presents with diabetes insipidus and loss of the normal posterior pituitary bright spot.*
![Coronal T2 MR shows enlargement of the pituitary gland by a mass <img src='img/arrows/CS.png'/> with extension to the left cavernous sinus <img src='img/arrows/CO.png'/>. Lymphoma is often infiltrative, DWI positive, and diffusely enhances. Pituitary involvement is rare.](1b383775-5014-4020-9538-68c02c80c8ed)
**Lymphoma, Primary CNS**
*Coronal T2 MR shows enlargement of the pituitary gland by a mass <img src='img/arrows/CS.png'/> with extension to the left cavernous sinus <img src='img/arrows/CO.png'/>. Lymphoma is often infiltrative, DWI positive, and diffusely enhances. Pituitary involvement is rare.*
![Sagittal T1 MR shows a sagging midbrain <img src='img/arrows/WC.png'/> with a decreased mamillopontine distance and a prominent pituitary gland <img src='img/arrows/CO.png'/>. The optic chiasm is draped over the gland <img src='img/arrows/WS.png'/> in this patient with intractable headaches. Diffuse dural enhancement is common in intracranial hypotension.](c45b5a47-2632-43f3-882a-c79666e5be0c)
**Intracranial Hypotension**
*Sagittal T1 MR shows a sagging midbrain <img src='img/arrows/WC.png'/> with a decreased mamillopontine distance and a prominent pituitary gland <img src='img/arrows/CO.png'/>. The optic chiasm is draped over the gland <img src='img/arrows/WS.png'/> in this patient with intractable headaches. Diffuse dural enhancement is common in intracranial hypotension.*
### Additional Images
![Sagittal T1 MR shows a sagging midbrain <img src='img/arrows/WC.png'/> and a prominent pituitary gland <img src='img/arrows/CO.png'/>. The optic chiasm is draped over the gland <img src='img/arrows/WS.png'/> in this patient with postural hypotension and intractable headaches. Inferiorly displaced tonsils are common in intracranial hypotension.](5b5f931f-8650-4b93-a933-72004dc96246)
**Intracranial Hypotension**
*Sagittal T1 MR shows a sagging midbrain <img src='img/arrows/WC.png'/> and a prominent pituitary gland <img src='img/arrows/CO.png'/>. The optic chiasm is draped over the gland <img src='img/arrows/WS.png'/> in this patient with postural hypotension and intractable headaches. Inferiorly displaced tonsils are common in intracranial hypotension.*
![Sagittal T1 MR shows a very shallow bony sella <img src='img/arrows/WO.png'/> with the optic chiasm <img src='img/arrows/WS.png'/> draped over the pituitary gland. The gland measures 9 mm, which is normal in 19-year-old women. Although the gland appears enlarged, it is actually a pseudoenlarged gland.](393d3f0d-6203-4610-84e4-b27d428165d5)
**Anatomic Variants (Small Sella Turcica, "Kissing Carotids")**
*Sagittal T1 MR shows a very shallow bony sella <img src='img/arrows/WO.png'/> with the optic chiasm <img src='img/arrows/WS.png'/> draped over the pituitary gland. The gland measures 9 mm, which is normal in 19-year-old women. Although the gland appears enlarged, it is actually a pseudoenlarged gland.*
![Sagittal T1 MR shows a diffusely enlarged pituitary gland <img src='img/arrows/CO.png'/> in a young female with pituitary dysfunction. Neurosarcoid was diagnosed at resection. Imaging mimics the much more common pituitary adenoma.](97ef3277-0166-4cd0-946e-95cb80f25136)
**Neurosarcoid**
*Sagittal T1 MR shows a diffusely enlarged pituitary gland <img src='img/arrows/CO.png'/> in a young female with pituitary dysfunction. Neurosarcoid was diagnosed at resection. Imaging mimics the much more common pituitary adenoma.*
![Coronal T1 C+ MR shows an enlarged pituitary gland <img src='img/arrows/CS.png'/> with a heterogeneous macroadenoma. Pituitary apoplexy can be considered in patients with acute clinical features, including headache, visual defects &amp;/or altered mental status, and a heterogeneous pituitary mass.](images/app.statdx.com_image_thumbnail_aebca945-5ce2-4e03-a492-f9849279cd77_annotated_true_size_900_quality_90_9a5de2b7.jpg)
**Pituitary Macroadenoma**
*Coronal T1 C+ MR shows an enlarged pituitary gland <img src='img/arrows/CS.png'/> with a heterogeneous macroadenoma. Pituitary apoplexy can be considered in patients with acute clinical features, including headache, visual defects &amp;/or altered mental status, and a heterogeneous pituitary mass.*
![Coronal T1 C+ MR shows a diffusely enlarged, enhancing pituitary gland <img src='img/arrows/CO.png'/> in a 32-year-old female patient with pituitary dysfunction. Neurosarcoid was diagnosed at resection. Imaging mimics the much more common pituitary adenoma.](4f91f04b-f6de-420c-b03b-e181e180ee2c)
**Neurosarcoid**
*Coronal T1 C+ MR shows a diffusely enlarged, enhancing pituitary gland <img src='img/arrows/CO.png'/> in a 32-year-old female patient with pituitary dysfunction. Neurosarcoid was diagnosed at resection. Imaging mimics the much more common pituitary adenoma.*
![Coronal T1 C+ MR shows a uniformly enlarged, enhancing pituitary gland <img src='img/arrows/CO.png'/> with upward displacement of the optic chiasm <img src='img/arrows/WS.png'/> in this child with known LCH. LCH often presents with diabetes insipidus and loss of the normal posterior pituitary bright spot.](2b5942ff-526a-4a30-ab3f-c95fc52c6f0b)
**Langerhans Cell Histiocytosis**
*Coronal T1 C+ MR shows a uniformly enlarged, enhancing pituitary gland <img src='img/arrows/CO.png'/> with upward displacement of the optic chiasm <img src='img/arrows/WS.png'/> in this child with known LCH. LCH often presents with diabetes insipidus and loss of the normal posterior pituitary bright spot.*
![Sagittal T1 C+ FS MR in a 53-year-old man shows a mildly enlarged pituitary gland with a focal area of less enhancement <img src='img/arrows/CO.png'/> related to a microadenoma. Microadenomas are benign tumors of the adenohypophysis that are often seen best on dynamic CEMR.](images/app.statdx.com_image_thumbnail_7f111e65-b465-4ace-9919-1c8847832f5d_annotated_true_size_900_quality_90_723d2769.jpg)
**Pituitary Microadenoma**
*Sagittal T1 C+ FS MR in a 53-year-old man shows a mildly enlarged pituitary gland with a focal area of less enhancement <img src='img/arrows/CO.png'/> related to a microadenoma. Microadenomas are benign tumors of the adenohypophysis that are often seen best on dynamic CEMR.*
![Sagittal T1 MR shows an enlarged pituitary gland <img src='img/arrows/WS.png'/> that elevates the optic chiasm <img src='img/arrows/WO.png'/>. This enlarged gland is almost isointense with the brain in this example of classic macroadenoma.](images/app.statdx.com_image_thumbnail_b35f6d9d-dc76-47a2-ab05-794a986e650e_annotated_true_size_900_quality_90_ac5cb6a5.jpg)
**Pituitary Macroadenoma**
*Sagittal T1 MR shows an enlarged pituitary gland <img src='img/arrows/WS.png'/> that elevates the optic chiasm <img src='img/arrows/WO.png'/>. This enlarged gland is almost isointense with the brain in this example of classic macroadenoma.*
![Sagittal T1 C+ MR shows thickened, enhancing infundibulum <img src='img/arrows/WS.png'/> and a slightly enlarged pituitary gland <img src='img/arrows/BO.png'/> in a patient with known metastatic disease.](aba31fd7-4534-4da4-90ed-3cf2be69331d)
**Metastases to Gland/Stalk**
*Sagittal T1 C+ MR shows thickened, enhancing infundibulum <img src='img/arrows/WS.png'/> and a slightly enlarged pituitary gland <img src='img/arrows/BO.png'/> in a patient with known metastatic disease.*
![Coronal T1 C+ MR shows an enhancing suprasellar mass <img src='img/arrows/WS.png'/> in a patient with known metastatic lung cancer and diabetes insipidus.](feddffd3-bfd0-414a-80a4-b1392717e4e3)
**Metastases to Gland/Stalk**
*Coronal T1 C+ MR shows an enhancing suprasellar mass <img src='img/arrows/WS.png'/> in a patient with known metastatic lung cancer and diabetes insipidus.*
![Coronal T1 MR shows an enlarged pituitary gland <img src='img/arrows/WO.png'/> with hyperintense focus <img src='img/arrows/WS.png'/>. Preoperative diagnosis was Rathke cleft cyst. Granular cell tumor (type of pituicytoma) was found at surgery.](6526a407-172a-4352-bf3b-4e5ba2620970)
**Pituicytoma**
*Coronal T1 MR shows an enlarged pituitary gland <img src='img/arrows/WO.png'/> with hyperintense focus <img src='img/arrows/WS.png'/>. Preoperative diagnosis was Rathke cleft cyst. Granular cell tumor (type of pituicytoma) was found at surgery.*
![Coronal T1 C+ MR shows an enlarged pituitary gland <img src='img/arrows/BC.png'/> and stalk <img src='img/arrows/WS.png'/>. Note dural thickening <img src='img/arrows/WO.png'/>. Scan was normal after intravenous steroid administration.](c2edc186-8789-4015-a2b6-cfb390741aed)
**Pseudotumor, Intracranial**
*Coronal T1 C+ MR shows an enlarged pituitary gland <img src='img/arrows/BC.png'/> and stalk <img src='img/arrows/WS.png'/>. Note dural thickening <img src='img/arrows/WO.png'/>. Scan was normal after intravenous steroid administration.*
![Coronal T1 C+ MR shows a mass diffusely infiltrating the pituitary gland <img src='img/arrows/WS.png'/>. Extension into the cavernous sinus <img src='img/arrows/BO.png'/> helps identify more aggressive pathology.](d4c9c906-249d-4de9-b2a9-711ad28f958a)
**Lymphoma, Primary CNS**
*Coronal T1 C+ MR shows a mass diffusely infiltrating the pituitary gland <img src='img/arrows/WS.png'/>. Extension into the cavernous sinus <img src='img/arrows/BO.png'/> helps identify more aggressive pathology.*
![Coronal T1 C+ MR shows a physiologically enlarged pituitary gland <img src='img/arrows/WS.png'/> in this 28-year-old lactating woman. The gland measures nearly 12 mm in height. Follow-up scan 1 year later was normal.](images/app.statdx.com_image_thumbnail_e5a30f38-5e24-4bd5-ba3a-44766e56eccf_annotated_true_size_900_quality_90_19330edf.jpg)
**Pituitary Hyperplasia**
*Coronal T1 C+ MR shows a physiologically enlarged pituitary gland <img src='img/arrows/WS.png'/> in this 28-year-old lactating woman. The gland measures nearly 12 mm in height. Follow-up scan 1 year later was normal.*
![Coronal T1 C+ MR in a 51-year-old man shows a mildly enlarged pituitary gland <img src='img/arrows/WS.png'/> measuring 11 mm in height. Note the faint area of slightly less enhancement <img src='img/arrows/BO.png'/>. An 8-mm microadenoma was found at surgery.](images/app.statdx.com_image_thumbnail_51377171-afe9-4af1-a4c6-e935ba3b3208_annotated_true_size_900_quality_90_60da5b4f.jpg)
**Pituitary Microadenoma**
*Coronal T1 C+ MR in a 51-year-old man shows a mildly enlarged pituitary gland <img src='img/arrows/WS.png'/> measuring 11 mm in height. Note the faint area of slightly less enhancement <img src='img/arrows/BO.png'/>. An 8-mm microadenoma was found at surgery.*
docs_md/articles/epidermoid-cyst_704c5ddf-e1f7-4a5d-a1b8-5b0e603170d9.md
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---
title: "Epidermoid Cyst"
docid: "704c5ddf-e1f7-4a5d-a1b8-5b0e603170d9"
authors:
- key: "5cff4116-3654-4b3a-bb75-5ebe0b8c9850"
value: "Anne G. Osborn, MD, FACR"
breadcrumbs:
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name: "Brain"
slug: "brain"
treeNodeId: "6d8829f1-14d7-45af-8675-255189aa526a"
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name: "Diagnosis"
slug: "diagnosis"
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name: "Pathology-Based Diagnoses"
slug: "pathology-based-diagnoses"
treeNodeId: "d9d3a8ed-f21b-4831-8c77-591a3500ef77"
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name: "Primary Nonneoplastic Cysts"
slug: "primary-nonneoplastic-cysts"
treeNodeId: "8037bffe-f61e-4433-b841-a263bcfbe056"
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name: "Epidermoid Cyst"
slug: "epidermoid-cyst"
treeNodeId: null
category: "Brain"
documentVersionId: "4092685b-a22f-42ca-b14b-6a21122b651f"
imageCount: 22
lastUpdated: "06/12/20"
pageDescription: "Epidermoid Cyst"
pageKeywords: "Brain, Diagnosis, Pathology-Based Diagnoses, Primary Nonneoplastic Cysts, Epidermoid Cyst"
pageTitle: "Epidermoid Cyst | STATdx"
enhancedTitle: "Epidermoid Cyst"
type: "DX"
references: true
breadcrumbs:
- "Brain"
- "Diagnosis"
- "Pathology-Based Diagnoses"
- "Primary Nonneoplastic Cysts"
- "Epidermoid Cyst"
---
# KEY FACTS
- ## Terminology
- Intracranial epidermoids ("pearly" tumor)
- Congenital ectodermal inclusion cysts, not true neoplasm
- ## Imaging
- CSF-like mass that insinuates cisterns and encases neurovascular structures
- Morphology: Lobulated, irregular, cauliflower-like mass with "fronds"
- FLAIR: Usually does not completely null
- DWI: Diffusion restriction definitively distinguishes from arachnoid cyst
- ## Top Differential Diagnoses
- Arachnoid cyst
- Inflammatory cyst (i.e., neurocysticercosis)
- Cystic neoplasm
- Dermoid cyst
- ## Pathology
- Arise from ectodermal inclusions during neural tube closure, 3rd to 5th week of embryogenesis
- ## Clinical Issues
- Symptoms depend on location and effect on adjacent neurovascular structures
- Most common symptom: Headache
- Cranial nerves V, VII, VIII neuropathy common
- 0.2-1.8% of all primary intracranial tumors
- Rare malignant degeneration into squamous cell carcinoma
- Treatment: Microsurgical resection
- Recurrence common if incompletely removed
- ## Diagnostic Checklist
- Insinuates CSF spaces, surrounds arteries, CNs with minimal displacement
- Incomplete nulling on FLAIR; DWI hyperintense
# TERMINOLOGY
- ## Synonyms
- Ectodermal inclusion cyst
- "Pearly" tumor
- ## Definitions
- Intracranial epidermoids are congenital inclusion cysts (not "tumors")
- Benign, slow-growing congenital lesions derived from ectodermal remnants sequestrated during embryogenesis
# IMAGING
- ## General Features
- ### Best diagnostic clue
- CSF-like mass that insinuates cisterns and encases neurovascular structures
- ### Location
- Intradural (90%), strong predilection for basal cisterns
- Cerebellopontine angle (CPA) (40-50%)
- 4th ventricle (17%)
- Parasellar/middle cranial fossa/sylvian fissure (10-15%)
- Parenchymal (i.e., cerebral hemispheres) rare (1.5%)
- Brainstem exceedingly rare
- Extradural (10%)
- Skull (intradiploic within frontal, parietal, occipital, sphenoid skull) as well as spine
- ### Size
- Variable; extradural intradiploic variants can become huge with minimal/no neurologic deficits
- Grow slowly by desquamation of normal cells into cystic cavity
- ### Morphology
- Lobulated, irregular, cauliflower-like excrescences
- Insinuates without mass effect unless large
- ## Radiographic Findings
- ### Radiography
- Diploic space epidermoids
- May alter scalp, outer/inner skull tables, and epidural space appearance
- Typically round or lobulated
- Well delineated with sclerotic rim
- ## CT Findings
- ### NECT
- Round/lobulated mass
- > 95% hypodense, resembling CSF
- 10-25% contain calcifications
- Rare variant = "dense" epidermoid
- 3% of intracranial epidermoids
- Secondary to hemorrhage, high protein, saponification of cyst debris to calcium soaps or iron-containing pigment
- ### CECT
- Usually none, though margin of cyst may show minimal enhancement
- ### Bone CT
- May have bony erosion; sharply corticated margins when intradiploic
- ## MR Findings
- ### T1WI
- Often (~ 75%) slightly hyperintense to CSF
- Lobulated periphery may be slightly more hyperintense than center
- Uncommonly hyperintense to brain ("white epidermoid") due to high triglycerides and unsaturated fatty acids
- Uncommonly hypointense to CSF ("black epidermoid")
- Presence of solid crystal cholesterol and keratin
- Lack of triglycerides and unsaturated fatty acids
- ### T2WI
- Often isointense (65%) to slightly hyperintense (35%) to CSF
- Very rarely hypointense due to calcification, ↓ hydration, viscous secretions, and iron pigments
- ### FLAIR
- Usually does not completely null
- ### DWI
- Characteristic hyperintensity
- High fractional anisotropy due to diffusion along 2D geometric plane
- Attributed to microstructure of parallel-layered keratin filaments and flakes
- In comparison to white matter, which also shows high fractional anisotropy, due to diffusion along single direction
- ADC = brain parenchyma
- ### T1WI C+
- Usually none, though margin of cyst may show minimal enhancement (25%)
- Enhancing tumor is sign of malignant degeneration
- ### MRS
- Resonances from lactate
- No NAA, choline, or lipid
- ## Angiographic Findings
- Conventional
- Depending on location and size, may show avascular mass effect
- ## Nonvascular Interventions
- ### Myelography
- Cisternography contrast delineates irregular lobulated tumor borders, extends into interstices
- ## Imaging Recommendations
- ### Best imaging tool
- MR
- ### Protocol advice
- FLAIR will often distinguish, whereas conventional sequences may not
- Diffusion restriction definitively distinguishes from arachnoid cyst
# DIFFERENTIAL DIAGNOSIS
- [Arachnoid Cyst](/document/arachnoid-cyst/d25aaeb3-5b3c-4483-99dc-2757468eedb9)
- Usually isointense to CSF on all standard sequences
- Completely nulls on FLAIR
- Hypointense diffusion: Contains highly mobile CSF, ADC = stationary water
- Rather than insinuate and engulf local structures, arachnoid cysts displace them
- Smooth surface, unlike lobulations of epidermoids
- [Inflammatory Cyst](/document/neurocysticercosis/6a45835f-6d7c-443e-874a-f33131d3def1)
- i.e., neurocysticercosis
- Often enhances
- Density/signal intensity usually not precisely like CSF
- Adjacent edema, gliosis common
- [Cystic Neoplasm](/document/pilocytic-astrocytoma/7eca92f5-6caa-4300-9afe-1b733b4473b2)
- Attenuation/signal intensity not that of CSF
- Often enhances
- [Dermoid Cyst](/document/dermoid-cyst/9b7aeb04-2cb3-405d-8c51-dd13297dd67c)
- Usually at or near midline
- Resembles fat, not CSF, and contains dermal appendages; often ruptured
# PATHOLOGY
- ## General Features
- ### Etiology
- Congenital: Embryology
- Arise from ectodermal inclusions during neural tube closure, 3rd to 5th week of embryogenesis
- Congenital intradural CPA epidermoids derived from cells of 1st branchial groove
- Acquired: Develop as result of trauma
- Uncommon etiology for intracranial tumors
- More common as spine etiology following LP
- ### Genetics
- Sporadic
- ### Associated abnormalities
- May have occipital/nasofrontal dermal sinus tract
- ## Gross Pathologic & Surgical Features
- Outer surface often has shiny, glistening, mother-of-pearl appearance ("beautiful tumor")
- Soft and pliable
- Conforms to shape of adjacent local structures/spaces
- Lobulated excrescences
- May invaginate into brain
- Insinuating growth pattern, extends through cisterns, surrounds and encases vessels/nerves
- Cyst filled with soft, waxy, creamy, or flaky keratinaceous material
- ## Microscopic Features
- Cyst wall = internal layer of simple stratified cuboidal squamous epithelium covered by fibrous capsule
- Cyst contents = solid crystalline cholesterol, keratinaceous debris; no dermal appendages
- Grows by progressive desquamation with conversion to keratin/cholesterol crystals, forming concentric lamellae
# CLINICAL ISSUES
- ## Presentation
- ### Most common signs/symptoms
- Symptoms depend on location and effect on adjacent neurovascular structures
- Most common symptom: Headache
- Cranial nerves V, VII, VIII neuropathy common
- 4th ventricular cerebellar signs common, yet increased intracranial pressure rare
- Less commonly hypopituitarism, diabetes insipidus
- Seizures if in sylvian fissure/temporal lobe
- May remain clinically silent for many years
- ## Demographics
- ### Age
- Presents between 20-60 years with peak at 40 years
- Presentation is uncommon in childhood
- ### Sex
- M = F
- CT hyperdense variant lesions have female predominance (M:F = 1:2.5)
- ### Epidemiology
- Epidermoids make up 0.2-1.8% of all primary intracranial tumors
- Much more common than dermoid cyst (4-9x higher incidence)
- Most common congenital intracranial tumor
- 3rd most common CPA/IAC mass, after vestibular schwannoma and meningioma
- ## Natural History & Prognosis
- Grows slowly: Epithelial component growth rate commensurate to that of normal epithelium
- Chemical meningitis possible from content leakage
- Rare malignant degeneration into squamous cell carcinoma (SCCa) reported
- Postulated prolonged or reparative process from foreign material leads to cellular atypia and neoplasia
- Often predated by frequent recurrences
- May occur years after surgical resection
- Mean age at presentation: 52 years with male preponderance
- ## Treatment
- Microsurgical resection
- Complicated by investment of local structures
- Recurrence common if cyst capsule incompletely removed
- Subarachnoid dissemination of contents may occur during operative/postoperative course
- May cause chemical meningitis
- CSF seeding and implantation reported
- Rare malignant degeneration of resection bed into SCCa reported
# DIAGNOSTIC CHECKLIST
- ## Consider
- Epidermoid if insinuates CSF spaces, surrounds arteries/CNs with minimal displacement
- ## Image Interpretation Pearls
- Resembles CSF on imaging studies, except usually incomplete nulling on FLAIR
- DWI hyperintensity is diagnostic
da5731a9-6c09-4bf5-99d9-fdefe09d5800
## References
# Selected References
1. [Pons Escoda A et al: Imaging of skull vault tumors in adults. Insights Imaging. 11(1):23, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=32056014%5Bpmid%5D)
1. [Bobeff EJ et al: Suprasellar Epidermoid Cyst: Case Report of Extended Endoscopic Transsphenoidal Resection and Systematic Review of the Literature. World Neurosurg. 128:514-26, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31121364%5Bpmid%5D)
1. [Hitti FL et al: Endoscopic Resection of a Cerebellopontine Angle Epidermoid Cyst via a Retrosigmoid Approach. J Neurol Surg B Skull Base. 80(Suppl 3):S330, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31143618%5Bpmid%5D)
1. [Ma J et al: Primary intradiploic epidermoid cyst: a case report with literature review. Clin Neuropathol. 38(1):28-32, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=30526818%5Bpmid%5D)
1. [Badat N et al: Malignant transformation of epidermoid cyst with diffuse leptomeningeal carcinomatosis on skull base and trigeminal perineural spread. J Neuroradiol. 45(5):337-40, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=30036548%5Bpmid%5D)
1. [Gollapudi PR et al: A frontal giant intradiploic giant pearl (epidermoid cyst) with intracranial and extracranial extension: a rare entity. J Pediatr Neurosci. 13(4):480-2, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=30937095%5Bpmid%5D)
1. [Twede JV et al: Intraosseous epidermoid cyst of the skull: case study and radiological imaging considerations. Dermatol Online J. 24(7), 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=30261578%5Bpmid%5D)
1. [Vaz-Guimaraes F et al: Endoscopic endonasal surgery for epidermoid and dermoid cysts: a 10-year experience. J Neurosurg. 1-11, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=29547084%5Bpmid%5D)
1. [Aboud E et al: Giant intracranial epidermoids: is total removal feasible? J Neurosurg. 1-14, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25594324%5Bpmid%5D)
1. [Law EK et al: Atypical intracranial epidermoid cysts: rare anomalies with unique radiological features. Case Rep Radiol. 2015:528632, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25667778%5Bpmid%5D)
1. [Demir MK et al: Rare and challenging extra-axial brain lesions: CT and MRI findings with clinico-radiological differential diagnosis and pathological correlation. Diagn Interv Radiol. 20(5):448-52, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=25010368%5Bpmid%5D)
1. [Vellutini EA et al: Malignant transformation of intracranial epidermoid cyst. Br J Neurosurg. 28(4):507-9, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=24345076%5Bpmid%5D)
1. [Velamati R et al: Meningitis secondary to ruptured epidermoid cyst: case-based review. Pediatr Ann. 42(6):248-51, 2013](http://www.ncbi.nlm.nih.gov/pubmed/?term=23718247%5Bpmid%5D)
1. [Ren X et al: Clinical, radiological, and pathological features of 24 atypical intracranial epidermoid cysts. J Neurosurg. 116(3):611-21, 2012](http://www.ncbi.nlm.nih.gov/pubmed/?term=22175719%5Bpmid%5D)
1. [Li F et al: Hyperdense intracranial epidermoid cysts: a study of 15 cases. Acta Neurochir (Wien). 149(1):31-9; discussion 39, 2007](http://www.ncbi.nlm.nih.gov/pubmed/?term=17151831%5Bpmid%5D)
## Images
### Selected Images
![Sagittal graphic shows a multilobulated epidermoid primarily within the prepontine cistern. Significant mass effect displaces the pons, cervicomedullary junction, and upper cervical spine.](images/app.statdx.com_image_thumbnail_ad974b01-ea05-4b6d-a64c-5ce72250a4ff_annotated_true_size_900_quality_90_e9ea0788.jpg)
*Sagittal graphic shows a multilobulated epidermoid primarily within the prepontine cistern. Significant mass effect displaces the pons, cervicomedullary junction, and upper cervical spine.*
![Gross pathology shows an epidermoid cyst extending anterosuperiorly from the cerebellopontine angle (CPA) cistern, insinuating within the prepontine cistern and encasing the basilar artery <img src='img/arrows/BO.png'/>. Note its typical pearly appearance. (Courtesy E. Hedley-Whyte, MD.)](images/app.statdx.com_image_thumbnail_69c8e599-c4d6-4096-8762-d50c4ff210a7_annotated_true_size_900_quality_90_ab2a4510.jpg)
*Gross pathology shows an epidermoid cyst extending anterosuperiorly from the cerebellopontine angle (CPA) cistern, insinuating within the prepontine cistern and encasing the basilar artery <img src='img/arrows/BO.png'/>. Note its typical pearly appearance. (Courtesy E. Hedley-Whyte, MD.)*
![Axial NECT shows a typical epidermoid cyst (EC) in an expanded right CPA cistern. Note that the EC <img src='img/arrows/WS.png'/> is slightly more dense than the adjacent CSF <img src='img/arrows/WC.png'/> and has a frothy, cauliflower-like surface.](images/app.statdx.com_image_thumbnail_0e8c9ff7-efdd-4f6b-953d-f9c6005d0d3b_annotated_true_size_900_quality_90_53ed4f8a.jpg)
*Axial NECT shows a typical epidermoid cyst (EC) in an expanded right CPA cistern. Note that the EC <img src='img/arrows/WS.png'/> is slightly more dense than the adjacent CSF <img src='img/arrows/WC.png'/> and has a frothy, cauliflower-like surface.*
![MR shows CPA epidermoid cyst that resembles CSF on T1 and T2 <img src='img/arrows/WS.png'/> but typically does not suppress on FLAIR <img src='img/arrows/BO.png'/> and demonstrates moderate restricted diffusion <img src='img/arrows/WO.png'/>.](images/app.statdx.com_image_thumbnail_9fe7a5f9-a971-4bfa-a40d-71b4c44a98a1_annotated_true_size_900_quality_90_d3fb2e8a.jpg)
*MR shows CPA epidermoid cyst that resembles CSF on T1 and T2 <img src='img/arrows/WS.png'/> but typically does not suppress on FLAIR <img src='img/arrows/BO.png'/> and demonstrates moderate restricted diffusion <img src='img/arrows/WO.png'/>.*
![Sagittal T1 MR in a 39-year-old woman with a history of attempted resection of a posterior fossa EC shows a large mass infiltrating the prepontine cistern <img src='img/arrows/BO.png'/> and wrapping around the cervicomedullary junction <img src='img/arrows/WC.png'/>. The mass is nearly isointense with CSF.](images/app.statdx.com_image_thumbnail_2644b3de-28ee-4fa5-883f-b9e8d96d399a_annotated_true_size_900_quality_90_79640b33.jpg)
*Sagittal T1 MR in a 39-year-old woman with a history of attempted resection of a posterior fossa EC shows a large mass infiltrating the prepontine cistern <img src='img/arrows/BO.png'/> and wrapping around the cervicomedullary junction <img src='img/arrows/WC.png'/>. The mass is nearly isointense with CSF.*
![Axial PD MR in the same patient shows a lobulated mass <img src='img/arrows/BS.png'/> in the right CPA cistern that is nearly isointense with CSF in the cistern and 4th ventricle <img src='img/arrows/BO.png'/>.](images/app.statdx.com_image_thumbnail_8cda8365-0903-4b43-b366-135ee0875419_annotated_true_size_900_quality_90_a50364f4.jpg)
*Axial PD MR in the same patient shows a lobulated mass <img src='img/arrows/BS.png'/> in the right CPA cistern that is nearly isointense with CSF in the cistern and 4th ventricle <img src='img/arrows/BO.png'/>.*
![Axial T2 MR in the same patient shows the mass <img src='img/arrows/WS.png'/> is nearly as hyperintense as fluid in the CPA cistern and 4th ventricle <img src='img/arrows/WO.png'/>.](images/app.statdx.com_image_thumbnail_f20393a7-5782-47e8-880e-f467a8f3c11c_annotated_true_size_900_quality_90_6d5256be.jpg)
*Axial T2 MR in the same patient shows the mass <img src='img/arrows/WS.png'/> is nearly as hyperintense as fluid in the CPA cistern and 4th ventricle <img src='img/arrows/WO.png'/>.*
![Coronal T2 MR shows the lobulated, hyperintense mass <img src='img/arrows/WS.png'/> encases and displaces the basilar artery <img src='img/arrows/WO.png'/>. CSF in the right middle fossa <img src='img/arrows/WC.png'/> is a cavity from prior attempted resection.](images/app.statdx.com_image_thumbnail_6a23a09b-6c4c-4ac6-ada1-a720f8a5f064_annotated_true_size_900_quality_90_6c5a9759.jpg)
*Coronal T2 MR shows the lobulated, hyperintense mass <img src='img/arrows/WS.png'/> encases and displaces the basilar artery <img src='img/arrows/WO.png'/>. CSF in the right middle fossa <img src='img/arrows/WC.png'/> is a cavity from prior attempted resection.*
![Axial FLAIR MR demonstrates that the lobulated, cauliflower-like mass <img src='img/arrows/WS.png'/> in the right CPA and prepontine cistern does not suppress.](images/app.statdx.com_image_thumbnail_14f37934-1d70-4179-b233-51fb60c93e21_annotated_true_size_900_quality_90_f5e93dbc.jpg)
*Axial FLAIR MR demonstrates that the lobulated, cauliflower-like mass <img src='img/arrows/WS.png'/> in the right CPA and prepontine cistern does not suppress.*
![The mass <img src='img/arrows/WS.png'/> restricts on DWI MR. This is a classic EC that infiltrates and insinuates CSF cisterns, encasing vessels (like the basilar artery) and cranial nerves (in this case, cranial nerves VII and VIII).](a277e245-7d2d-4420-b94a-ac271d9a5409)
*The mass <img src='img/arrows/WS.png'/> restricts on DWI MR. This is a classic EC that infiltrates and insinuates CSF cisterns, encasing vessels (like the basilar artery) and cranial nerves (in this case, cranial nerves VII and VIII).*
### Additional Images
![Gross pathology nicely shows the typical shiny, glistening, pearly appearance of an epidermoid residing within the prepontine cistern, which was also encasing the basilar artery (not shown).](a46d66f8-e14d-4727-9b2b-f8f6b85c7576)
*Gross pathology nicely shows the typical shiny, glistening, pearly appearance of an epidermoid residing within the prepontine cistern, which was also encasing the basilar artery (not shown).*
![Axial T2 MR shows a nearly CSF isointense epidermoid within the left anterior middle cranial fossa.](c21dff32-df8f-447f-8a48-a416706ab0da)
*Axial T2 MR shows a nearly CSF isointense epidermoid within the left anterior middle cranial fossa.*
![Axial DWI MR shows restricted diffusion within a left anterior middle cranial fossa epidermoid.](f691b2ec-6d58-43f6-9ba5-1b15b4ce0bbf)
*Axial DWI MR shows restricted diffusion within a left anterior middle cranial fossa epidermoid.*
![Axial T2 MR shows an extraaxial left occipital mass that scallops the skull <img src='img/arrows/WO.png'/> and displaces the dura inwardly <img src='img/arrows/WS.png'/>.](304d953f-4a15-4ce9-9512-90ee41f3d8ed)
*Axial T2 MR shows an extraaxial left occipital mass that scallops the skull <img src='img/arrows/WO.png'/> and displaces the dura inwardly <img src='img/arrows/WS.png'/>.*
![Axial T1 C+ FS MR in the same patient shows the mass does not enhance <img src='img/arrows/WS.png'/>. DWI (not shown) clinched the diagnosis as an EC. This is an atypical location for epidermoid.](9231e856-cc1b-4061-829f-7d5ef265999f)
*Axial T1 C+ FS MR in the same patient shows the mass does not enhance <img src='img/arrows/WS.png'/>. DWI (not shown) clinched the diagnosis as an EC. This is an atypical location for epidermoid.*
![Axial bone CT in the same patient reveals significant yet benign-appearing remodeling and scalloping of the inner calvarial table <img src='img/arrows/WO.png'/>.](45cb0593-e36a-4b7e-bd11-1907abdb220a)
*Axial bone CT in the same patient reveals significant yet benign-appearing remodeling and scalloping of the inner calvarial table <img src='img/arrows/WO.png'/>.*
![Bone CT in a 16-year-old girl with nonspecific headaches shows a smoothly marginated, lytic, expansile mass <img src='img/arrows/WC.png'/> centered on the diploic space of the calvarium.](3aa95b7f-2ac8-434a-a637-dc4940725fef)
*Bone CT in a 16-year-old girl with nonspecific headaches shows a smoothly marginated, lytic, expansile mass <img src='img/arrows/WC.png'/> centered on the diploic space of the calvarium.*
![Axial NECT in the same patient shows the calvarial cyst is hypodense relative to cortex. This is a classic EC of the skull and was an incidental finding in this asymptomatic patient.](b1956fad-d04d-44a9-931d-7703e06981d1)
*Axial NECT in the same patient shows the calvarial cyst is hypodense relative to cortex. This is a classic EC of the skull and was an incidental finding in this asymptomatic patient.*
![Axial bone CT demonstrates the typical appearance of a large intradiploic epidermoid as an expansile lesion with sharply corticated margins <img src='img/arrows/BS.png'/>.](1215b8d8-e92c-4051-a8f8-a0b45c0f3bf8)
*Axial bone CT demonstrates the typical appearance of a large intradiploic epidermoid as an expansile lesion with sharply corticated margins <img src='img/arrows/BS.png'/>.*
![Axial T2 MR in the same patient shows the lobulated, hyperintense mass scallops the skull <img src='img/arrows/WS.png'/>. Intradiploic EC was removed at surgery.](e846bf74-df16-4398-b84e-ffc505ac7f61)
*Axial T2 MR in the same patient shows the lobulated, hyperintense mass scallops the skull <img src='img/arrows/WS.png'/>. Intradiploic EC was removed at surgery.*
![Axial T1 (upper left), T2 (upper right) and FLAIR (lower left) MR show an extensive intradiploid EC. Coronal T1 C+ MR (lower right) shows mild enhancement <img src='img/arrows/WS.png'/> around the cyst margin.](617b6714-cbb8-45c2-bbec-a97ec0a17679)
*Axial T1 (upper left), T2 (upper right) and FLAIR (lower left) MR show an extensive intradiploid EC. Coronal T1 C+ MR (lower right) shows mild enhancement <img src='img/arrows/WS.png'/> around the cyst margin.*
![Axial T2 (upper left), FLAIR (upper right), T1 C+ (lower left), and DWI (lower right) MR show a recurrent EC that appears intraaxial, but the original surgery 24 years prior disclosed an EC of the quadrigeminal cistern.](2b1c5f5e-003c-4af5-8e86-5286add4ec63)
*Axial T2 (upper left), FLAIR (upper right), T1 C+ (lower left), and DWI (lower right) MR show a recurrent EC that appears intraaxial, but the original surgery 24 years prior disclosed an EC of the quadrigeminal cistern.*
docs_md/articles/epilepsy-adult_c936f9e1-b6c6-4c4a-afc6-f2e1a968a7b0.md
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---
title: "Epilepsy, Adult"
docid: "c936f9e1-b6c6-4c4a-afc6-f2e1a968a7b0"
authors:
- key: "f184750a-90b4-47a7-907b-23b05d70357a"
value: "Chang Yueh Ho, MD"
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value: "Bronwyn E. Hamilton, MD"
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slug: "brain-parenchyma-general"
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name: "Epilepsy, Adult"
slug: "epilepsy-adult"
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lastUpdated: "01/27/23"
pageDescription: "Epilepsy, Adult"
pageKeywords: "Brain, Differential Diagnosis, Brain Parenchyma, General, Clinically Based Differentials, Epilepsy, Adult"
pageTitle: "Epilepsy, Adult | STATdx"
enhancedTitle: "Epilepsy, Adult"
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breadcrumbs:
- "Brain"
- "Differential Diagnosis"
- "Brain Parenchyma, General"
- "Clinically Based Differentials"
- "Epilepsy, Adult"
---
# ESSENTIAL INFORMATION
- ## Key Differential Diagnosis Issues
- Adult-onset seizures are more likely acquired
- Acute symptomatic seizure ≤ 1 week of brain injury
- Remote symptomatic seizure: Beyond 1 week
- Encephalomalacia and gliosis can also cause seizures
- Epilepsy: When 2 or more seizures occur 24 hours apart
- ## Helpful Clues for Common Diagnoses
- **Trauma**
- Diffuse axonal injury and contusions
- Intracranial hemorrhage (subdural hemorrhage and subarachnoid hemorrhage) can also present with seizures without parenchymal findings
- **Stroke**
- Most common cause in older adults
- **Infection**
- All cerebral and meningeal infection can cause seizure
- Meningitis, encephalitis, and abscess
- Look for herpes encephalitis
- CNS tuberculosis and neurocysticercosis are common causes outside of USA
- **Drug Use and Withdrawal**
- Withdrawal from alcohol, benzodiazepines, barbiturates
- Illicit drug use and drugs, which lower seizure threshold
- **Metabolic**
- Hyper- or hypoglycemia
- Hyponatremia, hypocalcinemia, hypomagnesemia, hypothyroidism
- Hyperammonemia from hepatic encephalopathy
- Uremic encephalopathy
- **Neoplasms**
- Glioblastoma most common in older adults
- Cortically based tumors primarily in older children to young adults
- **Neurodegenerative Disease**
- Dementia: Alzheimer
- Demyelinating disease: Multiple sclerosis
- ## Helpful Clues for Less Common Diagnoses
- **Oligodendroglioma, IDH-Mutant and 1p/19q-Co-Deleted**
- 70-90% present with seizures
- Cortically based T2-hyperintense mass, rare enhancement
- **Mesial Temporal Sclerosis**
- Most common cause of intractable temporal lobe seizures
- Hippocampal atrophy and sclerosis
- May see ipsilateral mammillary body and forniceal atrophy
- **Paraneoplastic and Autoimmune Encephalitis**
- Both paraneoplastic and nonparaneoplastic
- 80% have bilateral/unilateral edema of temporal lobes
- Limbic system most common, also brainstem, cerebellum, and spinal cord
- **Posterior Reversible Encephalopathy Syndrome**
- 60-75% present with seizure
- Patchy parietooccipital cortical/subcortical T2/FLAIR hyperintense edema
- Associated with hypertension, chemotherapy, high-dose steroids, immunomodulation, sepsis, kidney failure, preeclampsia/eclampsia, autoimmune disease
- ## Helpful Clues for Rare Diagnoses
- **Pleomorphic Xanthoastrocytoma**
- 75% of patients present with seizures
- Cortical cyst + enhancing nodule is classic
- Reactive involvement of adjacent meninges typical: Dural tail
## References
# Selected References
1. [Akrami H et al: Neuroanatomic markers of posttraumatic epilepsy based on MR imaging and machine learning. AJNR Am J Neuroradiol. 43(3):347-53, 2022](http://www.ncbi.nlm.nih.gov/pubmed/?term=35210268%5Bpmid%5D)
1. [Tierney TS et al: Initial experience with magnetic resonance-guided focused ultrasound stereotactic surgery for central brain lesions in young adults. J Neurosurg. ePub, 2022](http://www.ncbi.nlm.nih.gov/pubmed/?term=35171812%5Bpmid%5D)
1. [DeSalvo MN et al: Contralateral preoperative resting-state functional MRI network integration is associated with surgical outcome in temporal lobe epilepsy. Radiology. 294(3):622-7, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=31961245%5Bpmid%5D)
1. [Kaur S et al: Adult onset seizures: clinical, etiological, and radiological profile. J Family Med Prim Care. 7(1):191-7, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=29915758%5Bpmid%5D)
1. [Berg AT et al: Revised terminology and concepts for organization of seizures and epilepsies: report of the ILAE Commission on Classification and Terminology, 2005-2009. Epilepsia. 51(4):676-85, 2010](http://www.ncbi.nlm.nih.gov/pubmed/?term=20196795%5Bpmid%5D)
## Images
### Selected Images
![Coronal NECT shows hyperdense acute hemorrhage in the inferior frontal lobes in a patient with a history of motor vehicle collision. The inferior frontal and anterior temporal lobes are the most common locations for traumatic contusions.](images/app.statdx.com_image_thumbnail_d8928373-1988-431b-ad93-b4649cc33628_size_168_quality_85_ca966ee0_20251014T204349Z.jpg)
**Trauma**
*Coronal NECT shows hyperdense acute hemorrhage in the inferior frontal lobes in a patient with a history of motor vehicle collision. The inferior frontal and anterior temporal lobes are the most common locations for traumatic contusions.*
![Coronal NECT shows hyperdense acute hemorrhage in the inferior frontal lobes in a patient with a history of motor vehicle collision. The inferior frontal and anterior temporal lobes are the most common locations for traumatic contusions.](images/app.statdx.com_image_thumbnail_d8928373-1988-431b-ad93-b4649cc33628_size_174_quality_85_dbd40ce8.jpg)
**Trauma**
*Coronal NECT shows hyperdense acute hemorrhage in the inferior frontal lobes in a patient with a history of motor vehicle collision. The inferior frontal and anterior temporal lobes are the most common locations for traumatic contusions.*
![Axial NECT shows loss of gray-white differentiation of the frontal and temporal operculum as well as the insular cortex consistent with infarct <img src='img/arrows/CS.png'/>. Note the hyperdensity in the sylvian fissure from thrombosed middle cerebral artery (MCA) branches <img src='img/arrows/CC.png'/>.](images/app.statdx.com_image_thumbnail_bfbb1626-c8fd-4e2a-8ec6-fe3428a3719f_size_168_quality_85_fd8b83c0_20251014T204349Z.jpg)
**Stroke**
*Axial NECT shows loss of gray-white differentiation of the frontal and temporal operculum as well as the insular cortex consistent with infarct <img src='img/arrows/CS.png'/>. Note the hyperdensity in the sylvian fissure from thrombosed middle cerebral artery (MCA) branches <img src='img/arrows/CC.png'/>.*
![Sagittal T1 C+ MR shows abnormal enhancement in the suprasellar and prepontine cistern <img src='img/arrows/CS.png'/>, inferior frontal lobe <img src='img/arrows/CO.png'/>, and quadrigeminal plate cistern <img src='img/arrows/CC.png'/>, consistent with tuberculomas. CNS tuberculosis is the most common cause of seizures from infection worldwide.](images/app.statdx.com_image_thumbnail_46b5932b-7e89-4fe6-bb70-3e1fe7f07d42_size_168_quality_85_57bde864_20251014T204349Z.jpg)
**Infection**
*Sagittal T1 C+ MR shows abnormal enhancement in the suprasellar and prepontine cistern <img src='img/arrows/CS.png'/>, inferior frontal lobe <img src='img/arrows/CO.png'/>, and quadrigeminal plate cistern <img src='img/arrows/CC.png'/>, consistent with tuberculomas. CNS tuberculosis is the most common cause of seizures from infection worldwide.*
![Axial b=1000 DWI MR shows increased signal in the tail of the hippocampi <img src='img/arrows/CS.png'/>, medial thalami <img src='img/arrows/CO.png'/>, insular cortex <img src='img/arrows/CC.png'/>, and cingulate cortex <img src='img/arrows/BS.png'/> in a patient with hyperammonemia from hepatic encephalopathy.](images/app.statdx.com_image_thumbnail_af3507db-e71b-45e1-b538-155678f68c36_size_168_quality_85_3b861d49_20251014T204349Z.jpg)
**Metabolic**
*Axial b=1000 DWI MR shows increased signal in the tail of the hippocampi <img src='img/arrows/CS.png'/>, medial thalami <img src='img/arrows/CO.png'/>, insular cortex <img src='img/arrows/CC.png'/>, and cingulate cortex <img src='img/arrows/BS.png'/> in a patient with hyperammonemia from hepatic encephalopathy.*
![Axial T1 C+ MR shows an irregular ring-enhancing mass in the left medial temporal lobe and occipital lobe. This was a glioblastoma, IDH-wildtype at biopsy.](images/app.statdx.com_image_thumbnail_9b80b063-2a68-44e5-9cc4-784e3b34fd0f_size_168_quality_85_e073a8af_20251014T204349Z.jpg)
**Neoplasms**
*Axial T1 C+ MR shows an irregular ring-enhancing mass in the left medial temporal lobe and occipital lobe. This was a glioblastoma, IDH-wildtype at biopsy.*
![Axial FLAIR MR shows a large, T2-hyperintense mass in the left frontal lobe extending across the corpus callosum to the right frontal lobe and centrally to involve the basal ganglia. This was a 1p/19q co-deleted oligodendroglioma at biopsy. These tumors are often calcified and located in the frontal lobe.](9d79274b-c751-481d-8593-de92a88444fd)
**Neoplasms**
*Axial FLAIR MR shows a large, T2-hyperintense mass in the left frontal lobe extending across the corpus callosum to the right frontal lobe and centrally to involve the basal ganglia. This was a 1p/19q co-deleted oligodendroglioma at biopsy. These tumors are often calcified and located in the frontal lobe.*
![Coronal FLAIR MR shows increased T2 signal and relative volume loss of the right hippocampal formation <img src='img/arrows/CS.png'/>, consistent with mesial temporal sclerosis in this patient with temporal lobe seizures.](images/app.statdx.com_image_thumbnail_9644fd19-2c08-493a-b6c0-86829d88616e_size_168_quality_85_351aab5c_20251014T204349Z.jpg)
**Mesial Temporal Sclerosis**
*Coronal FLAIR MR shows increased T2 signal and relative volume loss of the right hippocampal formation <img src='img/arrows/CS.png'/>, consistent with mesial temporal sclerosis in this patient with temporal lobe seizures.*
![Axial FLAIR MR shows bilateral T2 hyperintensity in the occipital lobe cortex and subcortical white matter. In this patient with hypertension and renal failure, PRES was diagnosed. The DWI images were negative. Imaging of PRES often completely resolves when hypertension is controlled.](4017a3e8-d8cf-4b15-bee3-e7fe81e93fc0)
**Posterior Reversible Encephalopathy Syndrome**
*Axial FLAIR MR shows bilateral T2 hyperintensity in the occipital lobe cortex and subcortical white matter. In this patient with hypertension and renal failure, PRES was diagnosed. The DWI images were negative. Imaging of PRES often completely resolves when hypertension is controlled.*
### Additional Images
![Coronal T1 C+ MR shows central heterogeneous enhancement of a low- intensity tumor involving the cortex of the posterior frontal lobe, consistent with oligodendroglioma.](images/app.statdx.com_image_thumbnail_ac3fd4f1-6d7c-4979-8c8e-22ec6d3c7e27_size_168_quality_85_0be67a4a_20251014T204349Z.jpg)
**Oligodendroglioma, IDH-Mutant and 1p/19q-Co-Deleted**
*Coronal T1 C+ MR shows central heterogeneous enhancement of a low- intensity tumor involving the cortex of the posterior frontal lobe, consistent with oligodendroglioma.*
![Axial FLAIR MR shows bilateral hyperintensity of the hippocampi and medial temporal lobes <img src='img/arrows/CS.png'/>. In this patient with a history of lung cancer, this is consistent with autoimmune encephalitis.](images/app.statdx.com_image_thumbnail_e08a1aef-9ebb-4a33-8ea7-4d7c44b75c73_size_168_quality_85_8b30e647_20251014T204349Z.jpg)
**Paraneoplastic and Autoimmune Encephalitis**
*Axial FLAIR MR shows bilateral hyperintensity of the hippocampi and medial temporal lobes <img src='img/arrows/CS.png'/>. In this patient with a history of lung cancer, this is consistent with autoimmune encephalitis.*
![Axial FLAIR MR shows bilateral hyperintensity of the insular cortex <img src='img/arrows/CS.png'/> in this patient with ovarian cancer, consistent with autoimmune paraneoplastic encephalitis.](ec2a7de9-065a-4e3e-bd42-0b1af21c97a5)
**Paraneoplastic and Autoimmune Encephalitis**
*Axial FLAIR MR shows bilateral hyperintensity of the insular cortex <img src='img/arrows/CS.png'/> in this patient with ovarian cancer, consistent with autoimmune paraneoplastic encephalitis.*
![Coronal FLAIR MR shows a cortical hyperintense mass in the posterior frontal lobe with a focal cyst <img src='img/arrows/CS.png'/>. This was a pleomorphic xanthoastrocytoma at surgery.](1345d1aa-7c33-4b8b-954c-cfe17b967da3)
**Pleomorphic Xanthoastrocytoma**
*Coronal FLAIR MR shows a cortical hyperintense mass in the posterior frontal lobe with a focal cyst <img src='img/arrows/CS.png'/>. This was a pleomorphic xanthoastrocytoma at surgery.*
![Sagittal T2 MR shows a well-circumscribed T2-hyperintense mass involving the posterior frontal cortex <img src='img/arrows/CS.png'/>. This is a typical location and appearance for oligodendroglioma. 70-90% of patients with this tumor present with seizures due to its cortical nature.](d55a6a70-1a01-44e0-8ca2-38abc0981c88)
**Oligodendroglioma, IDH-Mutant and 1p/19q-Co-Deleted**
*Sagittal T2 MR shows a well-circumscribed T2-hyperintense mass involving the posterior frontal cortex <img src='img/arrows/CS.png'/>. This is a typical location and appearance for oligodendroglioma. 70-90% of patients with this tumor present with seizures due to its cortical nature.*
![Coronal FLAIR MR shows atrophy and hyperintensity of the left hippocampus <img src='img/arrows/CS.png'/>, consistent with left mesial temporal sclerosis. There is also loss of the normal internal architecture of the left hippocampus and ex vacuo dilatation of the left temporal horn <img src='img/arrows/CO.png'/>.](aa0b092b-064f-459a-9efd-8f878004a054)
**Mesial Temporal Sclerosis**
*Coronal FLAIR MR shows atrophy and hyperintensity of the left hippocampus <img src='img/arrows/CS.png'/>, consistent with left mesial temporal sclerosis. There is also loss of the normal internal architecture of the left hippocampus and ex vacuo dilatation of the left temporal horn <img src='img/arrows/CO.png'/>.*
![Axial CBF map from arterial spin labeling shows relative hypoperfusion of the left temporal lobe <img src='img/arrows/CS.png'/> compared to the right in this patient with left mesial temporal sclerosis. This is consistent with interictal seizure focus.](6de5dc2d-fa1c-49db-98ed-a3fda780dba6)
**Mesial Temporal Sclerosis**
*Axial CBF map from arterial spin labeling shows relative hypoperfusion of the left temporal lobe <img src='img/arrows/CS.png'/> compared to the right in this patient with left mesial temporal sclerosis. This is consistent with interictal seizure focus.*
![Axial T1 C+ MR shows a cyst <img src='img/arrows/CS.png'/> and heterogeneously enhancing nodule <img src='img/arrows/CO.png'/> involving the cortex. There is an incidental developmental venous anomaly <img src='img/arrows/CC.png'/>.](7e9f029e-0fb4-48da-afd3-cf5b752062b0)
**Pleomorphic Xanthoastrocytoma**
*Axial T1 C+ MR shows a cyst <img src='img/arrows/CS.png'/> and heterogeneously enhancing nodule <img src='img/arrows/CO.png'/> involving the cortex. There is an incidental developmental venous anomaly <img src='img/arrows/CC.png'/>.*
![Coronal FLAIR MR shows hyperintensity and swelling of the right hippocampus <img src='img/arrows/CS.png'/> and bilateral parahippocampal gyri <img src='img/arrows/CC.png'/>. In a patient with acute encephalopathy, seizures, and fever, herpes encephalitis must be excluded.](acb5e817-1a3c-4979-bdcd-be26118da2ec)
**Infection**
*Coronal FLAIR MR shows hyperintensity and swelling of the right hippocampus <img src='img/arrows/CS.png'/> and bilateral parahippocampal gyri <img src='img/arrows/CC.png'/>. In a patient with acute encephalopathy, seizures, and fever, herpes encephalitis must be excluded.*
![Axial T2 FS MR shows bilateral hippocampal hyperintensity and edema <img src='img/arrows/CS.png'/>. Herpes encephalitis typically involves the medial temporal lobes asymmetrically and the insular cortex. The basal ganglia is usually spared, and there is deceased diffusion of the cortex early in the disease.](faeb3244-f4ae-42cd-b75f-6d90c365ee6a)
**Infection**
*Axial T2 FS MR shows bilateral hippocampal hyperintensity and edema <img src='img/arrows/CS.png'/>. Herpes encephalitis typically involves the medial temporal lobes asymmetrically and the insular cortex. The basal ganglia is usually spared, and there is deceased diffusion of the cortex early in the disease.*
![Axial NECT shows a heterogeneous mass causing a seizure in the right frontal lobe extending to the basal ganglia with midline shift. There are areas of hyperdensity <img src='img/arrows/CS.png'/> suggesting a high-grade neoplasm. This was a glioblastoma at biopsy.](efaf4f17-91c0-47d6-b338-13ddefefb91c)
**Neoplasms**
*Axial NECT shows a heterogeneous mass causing a seizure in the right frontal lobe extending to the basal ganglia with midline shift. There are areas of hyperdensity <img src='img/arrows/CS.png'/> suggesting a high-grade neoplasm. This was a glioblastoma at biopsy.*
![Axial FLAIR MR in this patient with ovarian cancer shows T2 hyperintensity of the temporal lobes <img src='img/arrows/CS.png'/>, consistent with autoimmune, paraneoplastic, limbic encephalitis. Compared to herpes encephalitis, autoimmune encephalitis is more likely to be bilateral, symmetric without decreased diffusion. The basal ganglia are more commonly involved.](01f65532-afcb-4ef7-b05b-25b67af8c6da)
**Paraneoplastic and Autoimmune Encephalitis**
*Axial FLAIR MR in this patient with ovarian cancer shows T2 hyperintensity of the temporal lobes <img src='img/arrows/CS.png'/>, consistent with autoimmune, paraneoplastic, limbic encephalitis. Compared to herpes encephalitis, autoimmune encephalitis is more likely to be bilateral, symmetric without decreased diffusion. The basal ganglia are more commonly involved.*
![Axial FLAIR MR shows bilateral T2 hyperintensity in the parietal lobe cortex and subcortical white matter. In this patient with malignant hypertension, PRES was suspected.](0a69bb1c-f819-4ebe-a267-afb05719b3ca)
**Posterior Reversible Encephalopathy Syndrome**
*Axial FLAIR MR shows bilateral T2 hyperintensity in the parietal lobe cortex and subcortical white matter. In this patient with malignant hypertension, PRES was suspected.*
docs_md/articles/epilepsy-child_a342e5b5-5b98-4003-a437-6d42a483b40e.md
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---
title: "Epilepsy, Child"
docid: "a342e5b5-5b98-4003-a437-6d42a483b40e"
authors:
- key: "47381de4-c9fd-4999-8dd0-1808cd72db6b"
value: "Luke L. Linscott, MD"
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name: "Brain"
slug: "brain"
treeNodeId: "6d8829f1-14d7-45af-8675-255189aa526a"
-
name: "Differential Diagnosis"
slug: "differential-diagnosis"
treeNodeId: "a7fdd139-664e-4bb8-8d18-400e4733ff60"
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name: "Brain Parenchyma, General"
slug: "brain-parenchyma-general"
treeNodeId: "e79be97b-28c0-4023-be87-334c0579d35d"
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name: "Clinically Based Differentials"
slug: "clinically-based-differentials"
treeNodeId: "108519f7-93d7-4662-85dd-2239f2422821"
-
name: "Epilepsy, Child"
slug: "epilepsy-child"
treeNodeId: null
category: "Brain"
documentVersionId: "1b459176-1e44-4579-9ed7-e9f2d138d9ad"
imageCount: 65
lastUpdated: "01/27/23"
pageDescription: "Epilepsy, Child"
pageKeywords: "Brain, Differential Diagnosis, Brain Parenchyma, General, Clinically Based Differentials, Epilepsy, Child"
pageTitle: "Epilepsy, Child | STATdx"
enhancedTitle: "Epilepsy, Child"
type: "DDX"
references: true
breadcrumbs:
- "Brain"
- "Differential Diagnosis"
- "Brain Parenchyma, General"
- "Clinically Based Differentials"
- "Epilepsy, Child"
---
# ESSENTIAL INFORMATION
- ## Key Differential Diagnosis Issues
- Generalized seizure disorders usually nonlocalizing
- Partial complex (focal) epilepsy usually due to focal structural abnormality [i.e., focal cortical dysplasia (FCD)]
- Correlate with EEG results
- High-resolution & 3T MR helpful for subtle lesions
- 1-mm isotropic T1 for gray matter evaluation
- 3D FLAIR imaging helpful for identifying FCD
- PET & SPECT are often complimentary to MR in identifying seizure focus prior to surgical intervention
- PET: Decreased interictal metabolism in seizure focus
- SPECT: Increased ictal blood flow in seizure focus
- ## Helpful Clues for Common Diagnoses
- **Idiopathic Epilepsy**
- No structural cause found on MR
- Generalized: May be inherited
- Partial: Partial motor seizures, may resolve by puberty
- **Acquired Causes**
- Febrile seizure: Most common cause of seizure in children < 5 years
- Simple febrile seizure < 15 minutes without recurrence does not require imaging
- Trauma, remote stroke, or infection results in encephalomalacia &/or gliosis, which may cause epilepsy
- Benign & malignant tumors
- Toxic, metabolic, & drug abuse
- **Vascular Malformation**: Arteriovenous & cavernous malformations with hemorrhage
- **Mesial Temporal Sclerosis**
- Most common cause of intractable temporal lobe epilepsy in adults
- 2-hit hypothesis suggests initial injury with inherent vulnerability to neuronal injury
- Hippocampal atrophy & gliosis
- May see ipsilateral mammillary body & forniceal atrophy
- Look for associated FCD, especially in the ipsilateral temporal lobe (FCD type IIIa)
- **Migrational Anomalies**
- **Focal cortical dysplasia**
- Newest classification Blumcke et al 2011
- Type I: Mild blurring of gray-white junction with mild increased T2 signal of subcortical white matter
- More common in temporal lobes, difficult to detect
- Type II: Moderate blurring of gray-white junction & increased T2 signal of subcortical white matter
- Typically frontal lobes
- Type IIb includes more dysmorphia & balloon cells: Highly associated with transmantle sign & easier to detect on MR
- Transmantle sign: T2-hyperintense comet tail from ventricle to cortex; best seen on FLAIR
- Type III: Associated with another lesion: Mesial temporal sclerosis, tumor, vascular malformation, acquired injury
- **Polymicrogyria**
- Small, pebbly, cobblestone, or micronodular-appearing gyri
- Common migrational malformation with heterogeneous causes
- TORCH infection (particularly CMV) is common cause of polymicrogyria & seizures
- Diffuse or bilateral polymicrogyria more likely genetic/syndromic
- **Heterotopic gray matter**
- Gray matter nodules within deep white matter follow gray matter signal on all MR sequences
- Subependymal most common location
- Can be found incidentally in patients without seizures
- Diffuse subependymal heterotopia is X-linked
- **Schizencephaly**
- Cleft extending from cortical surface to ventricular ependyma, gray matter lined
- Outpouching or "dimpling" of lateral ventricular contour "points" to cleft
- May be unilateral or bilateral
- Open lipped: CSF in cleft; commonly bilateral
- Closed lipped: No CSF with apposed walls, usually unilateral
- **Septo-Optic Dysplasia Plus Syndrome**
- Septum pellucidum absence + optic nerve hypoplasia ± pituitary dysfunction
- When SOD is associated with schizencephaly &/or polymicrogyria, it is referred to as SOD Plus
- **Tuberous Sclerosis Complex**
- Burden of cortical dysplasias (i.e., tubers) correlates with seizure burden
- T2-hyperintense cortical/subcortical tubers: Similar imaging to type IIb FCD
- Cortical tubers also similar in histology to FCD type IIb with balloon cells
- Subependymal nodules can enhance & calcify
- 10-15% develop subependymal giant cell astrocytoma at foramen of Monro
- ## Helpful Clues for Less Common Diagnoses
- **Cortically Based Glioneuronal Tumors**
- Associated cortical dysplasia with tumor common (type IIIb)
- **Ganglioglioma**
- Most common cause of tumor-associated temporal lobe epilepsy
- Cystic/solid cortically based mass
- Ca⁺⁺ (~ 50%); enhancement (~ 50%)
- Temporal lobe most common site
- **Dysembryoplastic neuroepithelial tumor**
- Discrete T2-hyperintense "bubbly" cortical mass
- Medial temporal lobe most common
- Enhancement may occur (~ 10%) but is less common than ganglioglioma
- **Holoprosencephaly**
- Spectrum of failure of cleavage of midline cerebral hemispheres & telencephalon from diencephalon
- Monoventricle due to absence of septum pellucidum
- Complete to partial absence of other midline structures: Falx & corpus callosum
- Fusion of fornices, thalami, & basal ganglia
- Incomplete separation of frontal lobes
- More severe cases may include large dorsal cyst
- **Hemimegalencephaly**
- Unilateral hemispheric overgrowth
- Dysplastic enlarged ipsilateral ventricle
- Associated with genetic/syndromic diseases
- **Sturge****-****Weber Syndrome**
- Unilateral trigeminal (V1 & V2) distribution facial port-wine stain
- Ipsilateral malformation of cortical & pial veins = leptomeningeal enhancement
- Ipsilateral enlarged choroid plexus, hemiatrophy late finding
- Gyriform Ca⁺⁺ increases over time
- **Status Epilepticus**
- Seizure > 5 minutes or > 1 seizure within 5-minute period
- Higher likelihood for irreversible brain damage
- 1/2 are associated with known history of epilepsy
- Increased T2 signal of predominantly cortex with swelling & possible decreased diffusion
- ## Helpful Clues for Rare Diagnoses
- **Lissencephaly Type 1: Subcortical Band Heterotopia**
- "Smooth" brain lacking normal gyri; thick cortex
- Can see subcortical smooth gray matter band in many cases
- LIS1: Posterior predilection of lissencephaly
- DCX (double cortex): X-linked
- Females: Primarily diffuse band heterotopia
- Males: Diffuse lissencephaly, more severe phenotype
- **Lissencephaly Type 2**
- Congenital muscular dystrophy: Walker-Warburg, Fukuyama, & muscle-eye-brain
- Diffuse polymicrogyria (cobblestone lissencephaly) particularly frontal lobes & sylvian fissures
- Cerebellar polymicrogyria, cysts, vermian hypoplasia, hypomyelination, & eye abnormalities can be seen
- **Rasmussen Encephalitis**
- Likely autoimmune inflammation of unilateral cerebral hemisphere
- Typically at least mesial temporal lobe & insula affected
- Hemiatrophy late
## References
# Selected References
1. [Najm I et al: The ILAE consensus classification of focal cortical dysplasia: an update proposed by an ad hoc task force of the ILAE diagnostic methods commission. Epilepsia. 63(8):1899-919, 2022](http://www.ncbi.nlm.nih.gov/pubmed/?term=35706131%5Bpmid%5D)
1. [Jayalakshmi S et al: Focal cortical dysplasia and refractory epilepsy: role of multimodality imaging and outcome of surgery. AJNR Am J Neuroradiol. 40(5):892-8, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31000525%5Bpmid%5D)
1. [Duncan JS et al: Brain imaging in the assessment for epilepsy surgery. Lancet Neurol. 15(4):420-33, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=26925532%5Bpmid%5D)
1. [Blümcke I et al: The clinicopathologic spectrum of focal cortical dysplasias: a consensus classification proposed by an ad hoc task force of the ILAE Diagnostic Methods Commission. Epilepsia. 52(1):158-74. 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=21219302%5Bpmid%5D)
1. [Leventer RJ et al: Clinical and imaging heterogeneity of polymicrogyria: a study of 328 patients. Brain. 133(Pt 5):1415-27, 2010](http://www.ncbi.nlm.nih.gov/pubmed/?term=20403963%5Bpmid%5D)
## Images
### Selected Images
![Coronal T2 MR in a victim of remote abusive head trauma now with seizures shows extensive areas of encephalomalacia <img src='img/arrows/CS.png'/>, greater on the left. Any pathology leading to injury of the cortex puts a patient at risk for development of seizures.](images/app.statdx.com_image_thumbnail_a546195c-a62e-4c95-80b3-93c46a3736b1_size_168_quality_85_e7f27443_20251014T204358Z.jpg)
**Acquired Causes**
*Coronal T2 MR in a victim of remote abusive head trauma now with seizures shows extensive areas of encephalomalacia <img src='img/arrows/CS.png'/>, greater on the left. Any pathology leading to injury of the cortex puts a patient at risk for development of seizures.*
![Coronal T2 MR in a victim of remote abusive head trauma now with seizures shows extensive areas of encephalomalacia <img src='img/arrows/CS.png'/>, greater on the left. Any pathology leading to injury of the cortex puts a patient at risk for development of seizures.](images/app.statdx.com_image_thumbnail_a546195c-a62e-4c95-80b3-93c46a3736b1_size_174_quality_85_7fea666a.jpg)
**Acquired Causes**
*Coronal T2 MR in a victim of remote abusive head trauma now with seizures shows extensive areas of encephalomalacia <img src='img/arrows/CS.png'/>, greater on the left. Any pathology leading to injury of the cortex puts a patient at risk for development of seizures.*
![Axial T2 MR in a patient with right mesial temporal sclerosis (not shown) demonstrates ipsilateral volume loss of right mammillary body <img src='img/arrows/CS.png'/>. The medial limbic (Papez) circuit connects these structures.](images/app.statdx.com_image_thumbnail_1aca2366-85fc-45ad-b265-4614bcde435c_size_168_quality_85_3f84c074_20251014T204358Z.jpg)
**Mesial Temporal Sclerosis**
*Axial T2 MR in a patient with right mesial temporal sclerosis (not shown) demonstrates ipsilateral volume loss of right mammillary body <img src='img/arrows/CS.png'/>. The medial limbic (Papez) circuit connects these structures.*
![Coronal oblique FLAIR MR in this 11-year-old patient with chronic epilepsy shows an atrophic &amp; hyperintense left hippocampus <img src='img/arrows/CS.png'/>. Careful evaluation of the more anterior left temporal lobe showed an area of focal cortical dysplasia (FCD), making this an FCD type IIIa.](8143c44e-886d-44e6-bbec-1a693c25b8bb)
**Mesial Temporal Sclerosis**
*Coronal oblique FLAIR MR in this 11-year-old patient with chronic epilepsy shows an atrophic &amp; hyperintense left hippocampus <img src='img/arrows/CS.png'/>. Careful evaluation of the more anterior left temporal lobe showed an area of focal cortical dysplasia (FCD), making this an FCD type IIIa.*
![Coronal oblique T2 MR in the same patient shows significant volume loss &amp; loss of internal architecture of the left hippocampus <img src='img/arrows/CS.png'/>. Also note the decreased size of the left fornix <img src='img/arrows/CO.png'/>, often associated with mesial temporal sclerosis.](3b3d6e6a-8333-4948-9750-2508939bc826)
**Mesial Temporal Sclerosis**
*Coronal oblique T2 MR in the same patient shows significant volume loss &amp; loss of internal architecture of the left hippocampus <img src='img/arrows/CS.png'/>. Also note the decreased size of the left fornix <img src='img/arrows/CO.png'/>, often associated with mesial temporal sclerosis.*
![Coronal FLAIR MR images in a 3-year-old with FCD type IIb <img src='img/arrows/CS.png'/> with varying contrast windowing are shown. Note how FCD is much more conspicuous in the image on the right with windowing emphasizing greater contrast. Windowing is an important means to increasing detection of subtle FCD lesions.](images/app.statdx.com_image_thumbnail_1a14c951-38f8-45d3-850f-183742f1305f_size_168_quality_85_fd23609b_20251014T204358Z.jpg)
**Focal Cortical Dysplasia**
*Coronal FLAIR MR images in a 3-year-old with FCD type IIb <img src='img/arrows/CS.png'/> with varying contrast windowing are shown. Note how FCD is much more conspicuous in the image on the right with windowing emphasizing greater contrast. Windowing is an important means to increasing detection of subtle FCD lesions.*
![Axial T1 MR in a 4-month-old with seizures shows an area of increased signal <img src='img/arrows/CS.png'/> within the frontal lobes that are not yet myelinated. FCD is most conspicuous within the first few months of life &amp; after myelination (~ 2 years).](8c63e262-76dc-4f3b-80d4-b4ef8f0599e3)
**Focal Cortical Dysplasia**
*Axial T1 MR in a 4-month-old with seizures shows an area of increased signal <img src='img/arrows/CS.png'/> within the frontal lobes that are not yet myelinated. FCD is most conspicuous within the first few months of life &amp; after myelination (~ 2 years).*
![Parasagittal T1 MR in a 7-year-old boy with polymicrogyria shows irregularity &amp; nodularity of the cortical surface &amp; gray matter(GM)-white matter (WM) junction, typical of polymicrogyria.](images/app.statdx.com_image_thumbnail_7e4aab24-eb74-4d58-9145-0de12dda4b18_size_168_quality_85_01f69a52_20251014T204358Z.jpg)
**Polymicrogyria**
*Parasagittal T1 MR in a 7-year-old boy with polymicrogyria shows irregularity &amp; nodularity of the cortical surface &amp; gray matter(GM)-white matter (WM) junction, typical of polymicrogyria.*
![Coronal T2 MR in a 7-year-old with seizures shows extensive subependymal GM heterotopia <img src='img/arrows/CS.png'/> along the left lateral ventricular margin with overlying polymicrogyria <img src='img/arrows/CO.png'/>.](images/app.statdx.com_image_thumbnail_8399e301-4d15-4285-9b2a-0d37bebdae08_size_168_quality_85_4866c944_20251014T204358Z.jpg)
**Heterotopic Gray Matter**
*Coronal T2 MR in a 7-year-old with seizures shows extensive subependymal GM heterotopia <img src='img/arrows/CS.png'/> along the left lateral ventricular margin with overlying polymicrogyria <img src='img/arrows/CO.png'/>.*
![Coronal STIR MR shows a small, open-lipped left hemisphere cleft lined by GM with thickened gyri of pachygyria <img src='img/arrows/CS.png'/> &amp; outpouching of ventricular margin <img src='img/arrows/CO.png'/>, pointing to schizencephaly. Contralateral forme fruste schizencephaly does not reach ventricular margin <img src='img/arrows/CC.png'/>.](images/app.statdx.com_image_thumbnail_4d21eacd-4097-4246-9fb8-bd4fb1c0edb5_size_168_quality_85_9b6193d8_20251014T204358Z.jpg)
**Schizencephaly**
*Coronal STIR MR shows a small, open-lipped left hemisphere cleft lined by GM with thickened gyri of pachygyria <img src='img/arrows/CS.png'/> &amp; outpouching of ventricular margin <img src='img/arrows/CO.png'/>, pointing to schizencephaly. Contralateral forme fruste schizencephaly does not reach ventricular margin <img src='img/arrows/CC.png'/>.*
![Axial T2 MR in septo-optic dysplasia plus shows bilateral perisylvian polymicrogyria <img src='img/arrows/CS.png'/>, absent septum pellucidum <img src='img/arrows/CO.png'/>, &amp; optic nerve hypoplasia. Note extensive areas of irregular cortical thickening with shallow sulci &amp; irregular GM-WM junction.](images/app.statdx.com_image_thumbnail_c82ed510-8ba2-4e55-9495-9462e4a8df41_size_168_quality_85_fbc11d39_20251014T204358Z.jpg)
**Septo-Optic Dysplasia Plus Syndrome**
*Axial T2 MR in septo-optic dysplasia plus shows bilateral perisylvian polymicrogyria <img src='img/arrows/CS.png'/>, absent septum pellucidum <img src='img/arrows/CO.png'/>, &amp; optic nerve hypoplasia. Note extensive areas of irregular cortical thickening with shallow sulci &amp; irregular GM-WM junction.*
![Axial T2 MR shows a severe burden of hyperintense cortical dysplastic lesions (i.e., tubers) <img src='img/arrows/CS.png'/>, some of which display associated dark areas of mineralization <img src='img/arrows/CC.png'/>. Also note the calcified subependymal nodule <img src='img/arrows/CO.png'/> along the left lateral ventricle margin.](images/app.statdx.com_image_thumbnail_d9a54453-c5a3-47f5-b8ce-1c0f943beccd_size_168_quality_85_b16e1718_20251014T204358Z.jpg)
**Tuberous Sclerosis Complex**
*Axial T2 MR shows a severe burden of hyperintense cortical dysplastic lesions (i.e., tubers) <img src='img/arrows/CS.png'/>, some of which display associated dark areas of mineralization <img src='img/arrows/CC.png'/>. Also note the calcified subependymal nodule <img src='img/arrows/CO.png'/> along the left lateral ventricle margin.*
![Axial T2 MR shows a T2-hyperintense mass with indistinct margins in the left temporal lobe <img src='img/arrows/CS.png'/>. Increased T2 signal of the solid component of the mass suggests a low-grade tumor.](cdd814ca-8e38-4c52-86c2-bcce5095d5f3)
**Ganglioglioma**
*Axial T2 MR shows a T2-hyperintense mass with indistinct margins in the left temporal lobe <img src='img/arrows/CS.png'/>. Increased T2 signal of the solid component of the mass suggests a low-grade tumor.*
![Coronal T1 C+ FS MR shows intense &amp; amorphous enhancement of the left medial temporal lobe mass <img src='img/arrows/CS.png'/>. Gangliogliomas typically enhance, may have associated cysts, &amp; are most likely to occur in the temporal lobe.](2a483c98-f57a-4436-a632-b31d62f6c5f8)
**Ganglioglioma**
*Coronal T1 C+ FS MR shows intense &amp; amorphous enhancement of the left medial temporal lobe mass <img src='img/arrows/CS.png'/>. Gangliogliomas typically enhance, may have associated cysts, &amp; are most likely to occur in the temporal lobe.*
![Coronal T2 MR in a 10-year-old boy shows a bubbly, cystic lesion <img src='img/arrows/CS.png'/> replacing the left parasagittal frontal cortex without mass effect. This is a typical appearance for a dysembryoplastic neuroepithelial tumor (DNET).](99f6fb71-5106-413e-bdd7-4c1b817c036e)
**Dysembryoplastic Neuroepithelial Tumor**
*Coronal T2 MR in a 10-year-old boy shows a bubbly, cystic lesion <img src='img/arrows/CS.png'/> replacing the left parasagittal frontal cortex without mass effect. This is a typical appearance for a dysembryoplastic neuroepithelial tumor (DNET).*
![Axial T2 MR shows severe semilobar holoprosencephaly with fused basal ganglia <img src='img/arrows/CS.png'/> &amp; frontal lobes <img src='img/arrows/CO.png'/> with a large dorsal cyst communicating with monoventricle <img src='img/arrows/CC.png'/>. Seizures are a symptom of this Dx.](04d7cf7f-75a0-42c0-993d-f7f6b61afa47)
**Holoprosencephaly**
*Axial T2 MR shows severe semilobar holoprosencephaly with fused basal ganglia <img src='img/arrows/CS.png'/> &amp; frontal lobes <img src='img/arrows/CO.png'/> with a large dorsal cyst communicating with monoventricle <img src='img/arrows/CC.png'/>. Seizures are a symptom of this Dx.*
![Axial T2 MR in a 6-month-old girl with hemimegalencephaly (HME) shows asymmetric enlargement, cortical thickening <img src='img/arrows/CS.png'/>, poor sulcation, &amp; abnormal WM signal <img src='img/arrows/CO.png'/> within the left cerebral hemisphere. Enlargement of the lateral ventricle of the affected hemisphere is common in HME.](63d7808c-1c56-4cac-bd09-396bac754e48)
**Hemimegalencephaly**
*Axial T2 MR in a 6-month-old girl with hemimegalencephaly (HME) shows asymmetric enlargement, cortical thickening <img src='img/arrows/CS.png'/>, poor sulcation, &amp; abnormal WM signal <img src='img/arrows/CO.png'/> within the left cerebral hemisphere. Enlargement of the lateral ventricle of the affected hemisphere is common in HME.*
![Axial T1 C+ MR in a young patient shows leptomeningeal angiomatosis <img src='img/arrows/CS.png'/> &amp; prominent collateral medullary venous collateral <img src='img/arrows/CO.png'/> drainage, characteristic of Sturge-Weber syndrome. This vascular malformation results in poor perfusion of the cortex, chronic ischemia, &amp; eventual neuronal death.](33609040-5e46-4418-8bbd-92991e935f93)
**Sturge-Weber Syndrome**
*Axial T1 C+ MR in a young patient shows leptomeningeal angiomatosis <img src='img/arrows/CS.png'/> &amp; prominent collateral medullary venous collateral <img src='img/arrows/CO.png'/> drainage, characteristic of Sturge-Weber syndrome. This vascular malformation results in poor perfusion of the cortex, chronic ischemia, &amp; eventual neuronal death.*
![Axial NECT in the same patient shows geographic cortical &amp; subcortical calcification <img src='img/arrows/CS.png'/> in areas of permanently injured brain. Calcification develops over time &amp; is a reflection of disease severity, including severity of seizure symptoms.](12112af9-c83a-4ef8-a9eb-5bed0f88ddcf)
**Sturge-Weber Syndrome**
*Axial NECT in the same patient shows geographic cortical &amp; subcortical calcification <img src='img/arrows/CS.png'/> in areas of permanently injured brain. Calcification develops over time &amp; is a reflection of disease severity, including severity of seizure symptoms.*
![Coronal FLAIR MR shows a band of subcortical GM <img src='img/arrows/CS.png'/> intensity involving the posterior frontal lobes &amp; insula with associated pachygyria. Note T2 hyperintensity &amp; swelling of the right hippocampal gyrus <img src='img/arrows/CO.png'/> in this patient in status epilepticus.](1d64f955-fb75-45ea-807c-7b7f46a94bb4)
**Lissencephaly Type 1: Subcortical Band Heterotopia**
*Coronal FLAIR MR shows a band of subcortical GM <img src='img/arrows/CS.png'/> intensity involving the posterior frontal lobes &amp; insula with associated pachygyria. Note T2 hyperintensity &amp; swelling of the right hippocampal gyrus <img src='img/arrows/CO.png'/> in this patient in status epilepticus.*
![Axial DWI MR of a patient in status epilepticus with lissencephaly type 1 shows decreased diffusion of the hippocampal gyrus <img src='img/arrows/CS.png'/>. This is potentially reversible, but does put the patient at risk for developing hippocampal sclerosis in the future.](16c1e1ac-6b0e-4383-abe5-9b0697384f04)
**Lissencephaly Type 1: Subcortical Band Heterotopia**
*Axial DWI MR of a patient in status epilepticus with lissencephaly type 1 shows decreased diffusion of the hippocampal gyrus <img src='img/arrows/CS.png'/>. This is potentially reversible, but does put the patient at risk for developing hippocampal sclerosis in the future.*
![Axial T2 FS MR shows pachygyria with a frontal lobe predominance associated with a band of subcortical GM. This distribution is suggestive of the DCX mutation within the type 1 lissencephalies. Note the thin strip of WM in between the subcortical band &amp; the cortex <img src='img/arrows/CS.png'/>.](9230201e-f2bb-49a8-a9b2-aa4ff249a9b7)
**Lissencephaly Type 1: Subcortical Band Heterotopia**
*Axial T2 FS MR shows pachygyria with a frontal lobe predominance associated with a band of subcortical GM. This distribution is suggestive of the DCX mutation within the type 1 lissencephalies. Note the thin strip of WM in between the subcortical band &amp; the cortex <img src='img/arrows/CS.png'/>.*
![Axial T2 MR shows multiple cysts &amp; bizarre architecture of the cerebellar hemispheres <img src='img/arrows/CS.png'/> from cerebellar polymicrogyria. This appearance can be seen with congenital muscular dystrophies.](74b3cd2b-cc8b-4e83-a9d5-4622c310f257)
**Lissencephaly Type 2**
*Axial T2 MR shows multiple cysts &amp; bizarre architecture of the cerebellar hemispheres <img src='img/arrows/CS.png'/> from cerebellar polymicrogyria. This appearance can be seen with congenital muscular dystrophies.*
![Axial T2 MR shows bilateral symmetric frontal polymicrogyria <img src='img/arrows/CS.png'/>, yielding a cobblestone lissencephaly appearance. The type 2 lissencephalies include the dystroglycanopathies, which all have bilateral polymicrogyria as a brain malformation.](89357e4c-13d2-4948-8e34-2f0586650cdf)
**Lissencephaly Type 2**
*Axial T2 MR shows bilateral symmetric frontal polymicrogyria <img src='img/arrows/CS.png'/>, yielding a cobblestone lissencephaly appearance. The type 2 lissencephalies include the dystroglycanopathies, which all have bilateral polymicrogyria as a brain malformation.*
![Sagittal T1 MR shows the mild brainstem hypoplasia, which can be seen with type 2 lissencephalies. Note the disorganized cerebellar polymicrogyria <img src='img/arrows/CS.png'/> &amp; frontal lobe polymicrogyria <img src='img/arrows/CO.png'/>.](872bce5d-e139-4171-af84-e67a8a602804)
**Lissencephaly Type 2**
*Sagittal T1 MR shows the mild brainstem hypoplasia, which can be seen with type 2 lissencephalies. Note the disorganized cerebellar polymicrogyria <img src='img/arrows/CS.png'/> &amp; frontal lobe polymicrogyria <img src='img/arrows/CO.png'/>.*
![Coronal FLAIR shows T2 hyperintensity indicating gliosis of the cortex &amp; subcortical WM of the insula &amp; frontal temporal opercula with associated atrophy of the left hemisphere. This is consistent with Rasmussen encephalitis, which is thought to be an autoimmune process.](01dabac7-7f3c-462d-a805-1ae3cfc3a4df)
**Rasmussen Encephalitis**
*Coronal FLAIR shows T2 hyperintensity indicating gliosis of the cortex &amp; subcortical WM of the insula &amp; frontal temporal opercula with associated atrophy of the left hemisphere. This is consistent with Rasmussen encephalitis, which is thought to be an autoimmune process.*
![Coronal oblique FLAIR MR in an 8-year-old with chronic epilepsy shows the late hemiatrophy &amp; signal change seen in Rasmussen encephalitis. Also note the abnormal signal within the ipsilateral hippocampus <img src='img/arrows/CS.png'/>.](9aeb1c6e-7b60-40cd-8af1-1876126d9e44)
**Rasmussen Encephalitis**
*Coronal oblique FLAIR MR in an 8-year-old with chronic epilepsy shows the late hemiatrophy &amp; signal change seen in Rasmussen encephalitis. Also note the abnormal signal within the ipsilateral hippocampus <img src='img/arrows/CS.png'/>.*
### Additional Images
![Axial T1 MR shows hyperintensity related to recent hemorrhage in a cavernous malformation <img src='img/arrows/WS.png'/>. Seizures are often the presenting symptom for vascular lesions, such as a cavernoma or arteriovenous malformation.](b8a4f597-9dcc-4602-b827-3c4f811d7ec3)
**Acquired Causes**
*Axial T1 MR shows hyperintensity related to recent hemorrhage in a cavernous malformation <img src='img/arrows/WS.png'/>. Seizures are often the presenting symptom for vascular lesions, such as a cavernoma or arteriovenous malformation.*
![Axial T2* GRE MR shows susceptibility artifact in this cavernous malformation <img src='img/arrows/WS.png'/> with recent hemorrhage. GRE/SWI MR is helpful to search for additional lesions that may be occult on other sequences.](5a70b17b-bc36-40f9-9db7-c25f025bfbbe)
**Acquired Causes**
*Axial T2* GRE MR shows susceptibility artifact in this cavernous malformation <img src='img/arrows/WS.png'/> with recent hemorrhage. GRE/SWI MR is helpful to search for additional lesions that may be occult on other sequences.*
![Coronal FLAIR MR shows high signal in the right hippocampus <img src='img/arrows/WS.png'/> related to this patient's mesial temporal sclerosis. The primary MR features are T2-hyperintense signal, atrophy of the hippocampus, &amp; loss of internal architecture.](53b6482e-b5dc-4668-9f78-7753b8315909)
**Mesial Temporal Sclerosis**
*Coronal FLAIR MR shows high signal in the right hippocampus <img src='img/arrows/WS.png'/> related to this patient's mesial temporal sclerosis. The primary MR features are T2-hyperintense signal, atrophy of the hippocampus, &amp; loss of internal architecture.*
![Coronal T1 MR shows the typical decreased parenchymal volume <img src='img/arrows/WS.png'/> of the hippocampus in mesial temporal sclerosis. Internal architecture remains preserved in this case. Mild enlargement of the adjacent temporal horn is common.](62219e35-ff97-4ede-9527-523c77e84de0)
**Mesial Temporal Sclerosis**
*Coronal T1 MR shows the typical decreased parenchymal volume <img src='img/arrows/WS.png'/> of the hippocampus in mesial temporal sclerosis. Internal architecture remains preserved in this case. Mild enlargement of the adjacent temporal horn is common.*
![Coronal FLAIR MR shows atrophy &amp; increased signal of the right hippocampi <img src='img/arrows/CS.png'/>, consistent with gliosis/sclerosis of mesial temporal sclerosis. Also note the ex vacuo dilatation of the temporal horn <img src='img/arrows/CO.png'/> &amp; asymmetric volume loss of the amygdala <img src='img/arrows/CC.png'/> &amp; parahippocampal gyrus <img src='img/arrows/WO.png'/>.](ea2c6d86-0699-4ac4-a848-0babbd8f6049)
**Mesial Temporal Sclerosis**
*Coronal FLAIR MR shows atrophy &amp; increased signal of the right hippocampi <img src='img/arrows/CS.png'/>, consistent with gliosis/sclerosis of mesial temporal sclerosis. Also note the ex vacuo dilatation of the temporal horn <img src='img/arrows/CO.png'/> &amp; asymmetric volume loss of the amygdala <img src='img/arrows/CC.png'/> &amp; parahippocampal gyrus <img src='img/arrows/WO.png'/>.*
![Coronal STIR MR shows the loss of internal architecture &amp; asymmetric atrophy of the right hippocampal gyrus <img src='img/arrows/CS.png'/>. Note the ipsilateral atrophy of the right fornix <img src='img/arrows/CO.png'/>.](a76b8f74-6c20-4ad9-8a17-aa9d22357236)
**Mesial Temporal Sclerosis**
*Coronal STIR MR shows the loss of internal architecture &amp; asymmetric atrophy of the right hippocampal gyrus <img src='img/arrows/CS.png'/>. Note the ipsilateral atrophy of the right fornix <img src='img/arrows/CO.png'/>.*
![Coronal FLAIR MR shows a single focus of mild gyral expansion <img src='img/arrows/WC.png'/> &amp; a classic thin, high-signal seam <img src='img/arrows/WS.png'/> extending to the ventricle <img src='img/arrows/WO.png'/>.](a86736ce-1a11-4135-960a-350f46ac1b92)
**Focal Cortical Dysplasia**
*Coronal FLAIR MR shows a single focus of mild gyral expansion <img src='img/arrows/WC.png'/> &amp; a classic thin, high-signal seam <img src='img/arrows/WS.png'/> extending to the ventricle <img src='img/arrows/WO.png'/>.*
![Coronal FLAIR MR shows the comet-tail transmantle sign <img src='img/arrows/CS.png'/>, pointing from the cortex to the ventricle, which is indicative of a type IIb cortical dysplasia. The transmantle sign reflects radial glial migrational lines from the periventricular germinal matrix to the cortex.](b71c13a0-d669-4d91-9e35-3046cf959c4e)
**Focal Cortical Dysplasia**
*Coronal FLAIR MR shows the comet-tail transmantle sign <img src='img/arrows/CS.png'/>, pointing from the cortex to the ventricle, which is indicative of a type IIb cortical dysplasia. The transmantle sign reflects radial glial migrational lines from the periventricular germinal matrix to the cortex.*
![Coronal T2 MR shows classic findings in Taylor dysplasia, demonstrating juxtacortical high signal <img src='img/arrows/WS.png'/> with a thin &quot;seam&quot; of high signal <img src='img/arrows/WO.png'/> tracking along the expected course of the radial glial fibers to the subependymal margin. FLAIR is often more sensitive to these dysplasias.](69b6402b-9e59-41ec-b0d5-8d4aaeece8aa)
**Focal Cortical Dysplasia**
*Coronal T2 MR shows classic findings in Taylor dysplasia, demonstrating juxtacortical high signal <img src='img/arrows/WS.png'/> with a thin &quot;seam&quot; of high signal <img src='img/arrows/WO.png'/> tracking along the expected course of the radial glial fibers to the subependymal margin. FLAIR is often more sensitive to these dysplasias.*
![Coronal T1 MR shows thickened, ill-defined frontal cortex <img src='img/arrows/WS.png'/> with mild blurring of the gray-white junctions related to focal cortical dysplasia. Such findings should be confirmed with multiplanar imaging or isovoxel reconstructions.](3264e453-e9e8-4234-88d1-b66ae26ad3fa)
**Focal Cortical Dysplasia**
*Coronal T1 MR shows thickened, ill-defined frontal cortex <img src='img/arrows/WS.png'/> with mild blurring of the gray-white junctions related to focal cortical dysplasia. Such findings should be confirmed with multiplanar imaging or isovoxel reconstructions.*
![Coronal T1 MR shows subtle cortical thickening <img src='img/arrows/WS.png'/> &amp; mild blurring of the gray-white junction related to focal cortical dysplasia.](277c79ef-2960-4c7b-b8e6-fa1b7888212a)
**Focal Cortical Dysplasia**
*Coronal T1 MR shows subtle cortical thickening <img src='img/arrows/WS.png'/> &amp; mild blurring of the gray-white junction related to focal cortical dysplasia.*
![Coronal T2 MR shows an ill-defined, thickened cortex <img src='img/arrows/WS.png'/>. Note the normal, thin, sharply demarcated contralateral cortex <img src='img/arrows/WO.png'/>. Such findings should be confirmed with multiplanar imaging or isovoxel reconstructions to ensure no contribution from volume averaging of off axis cortex.](8982f985-0bf9-4ae1-a56a-d71e379c3909)
**Focal Cortical Dysplasia**
*Coronal T2 MR shows an ill-defined, thickened cortex <img src='img/arrows/WS.png'/>. Note the normal, thin, sharply demarcated contralateral cortex <img src='img/arrows/WO.png'/>. Such findings should be confirmed with multiplanar imaging or isovoxel reconstructions to ensure no contribution from volume averaging of off axis cortex.*
![Axial T2 MR shows bilateral diffuse subependymal GM heterotopia <img src='img/arrows/CS.png'/>. Note similar intensity with GM. These do not have enhancement or calcification commonly seen in tuberous sclerosis.](78fba6e9-4259-4a60-a352-164c4d6b0433)
**Heterotopic Gray Matter**
*Axial T2 MR shows bilateral diffuse subependymal GM heterotopia <img src='img/arrows/CS.png'/>. Note similar intensity with GM. These do not have enhancement or calcification commonly seen in tuberous sclerosis.*
![Axial T1 MR shows multifocal GM nodules lining both lateral ventricles <img src='img/arrows/WS.png'/>. Note also heterotopic GM within the left frontal lobe WM <img src='img/arrows/WC.png'/>. Heterotopic GM follows GM signal on all MR sequences &amp; does not enhance.](9199e577-c4b0-4040-8ac9-0222b52af2da)
**Heterotopic Gray Matter**
*Axial T1 MR shows multifocal GM nodules lining both lateral ventricles <img src='img/arrows/WS.png'/>. Note also heterotopic GM within the left frontal lobe WM <img src='img/arrows/WC.png'/>. Heterotopic GM follows GM signal on all MR sequences &amp; does not enhance.*
![Coronal T2 MR shows multiple foci of cortical GM lining the ependymal margin of both lateral ventricles <img src='img/arrows/WS.png'/>. These may be associated with seizures or may be asymptomatic.](6ab94581-99c3-4932-a74b-fbc7412994b7)
**Heterotopic Gray Matter**
*Coronal T2 MR shows multiple foci of cortical GM lining the ependymal margin of both lateral ventricles <img src='img/arrows/WS.png'/>. These may be associated with seizures or may be asymptomatic.*
![Axial T1 MR shows a classically located perisylvian open-lip schizencephaly with a wide CSF cleft <img src='img/arrows/WO.png'/> lined with GM <img src='img/arrows/WS.png'/>. The cleft margins do not touch in open-lip schizencephaly.](45ef6c69-5fa8-483f-be89-0695ae967af3)
**Schizencephaly**
*Axial T1 MR shows a classically located perisylvian open-lip schizencephaly with a wide CSF cleft <img src='img/arrows/WO.png'/> lined with GM <img src='img/arrows/WS.png'/>. The cleft margins do not touch in open-lip schizencephaly.*
![Axial T2 MR shows a closed-lip schizencephaly lined by dysplastic GM <img src='img/arrows/WS.png'/>. Note the characteristic ventricular outpouching <img src='img/arrows/BO.png'/>. Flow voids from embryonic vessels lay adjacent to the lateral margin of the schizencephalic cleft <img src='img/arrows/WC.png'/>.](23b38e57-d072-4651-969f-deadadd738bc)
**Schizencephaly**
*Axial T2 MR shows a closed-lip schizencephaly lined by dysplastic GM <img src='img/arrows/WS.png'/>. Note the characteristic ventricular outpouching <img src='img/arrows/BO.png'/>. Flow voids from embryonic vessels lay adjacent to the lateral margin of the schizencephalic cleft <img src='img/arrows/WC.png'/>.*
![Coronal T1 MR shows hyperintense nodule in the hypothalamus, consistent with an ectopic neurohypophysis <img src='img/arrows/CS.png'/>. There is also absence of septum pellucidum <img src='img/arrows/CO.png'/>. Hypoplastic optic nerves &amp; the pituitary gland within the sella was also noted (not shown).](a3253a1a-eaf4-456b-9610-2c1193649f20)
**Septo-Optic Dysplasia Plus Syndrome**
*Coronal T1 MR shows hyperintense nodule in the hypothalamus, consistent with an ectopic neurohypophysis <img src='img/arrows/CS.png'/>. There is also absence of septum pellucidum <img src='img/arrows/CO.png'/>. Hypoplastic optic nerves &amp; the pituitary gland within the sella was also noted (not shown).*
![Axial T2 MR shows an abnormal deep cleft <img src='img/arrows/WS.png'/> lined with dysplastic thickened GM <img src='img/arrows/WO.png'/>, extending to the ventricular margin, consistent with a closed-lip-type schizencephaly. These findings are commonly associated with septo-optic dysplasia and polymicrogyria.](31db2889-9975-4248-8852-80ee73000e86)
**Schizencephaly**
*Axial T2 MR shows an abnormal deep cleft <img src='img/arrows/WS.png'/> lined with dysplastic thickened GM <img src='img/arrows/WO.png'/>, extending to the ventricular margin, consistent with a closed-lip-type schizencephaly. These findings are commonly associated with septo-optic dysplasia and polymicrogyria.*
![Sagittal T1 MR shows small, disorganized perisylvian gyri <img src='img/arrows/WS.png'/> with a cobblestone appearance, characteristic for polymicrogyria. Other areas of cortex appear thickened <img src='img/arrows/WO.png'/> &amp; indistinct related to pachygyria.](e942ac12-b24e-4d68-9725-6c1583dc93ea)
**Polymicrogyria**
*Sagittal T1 MR shows small, disorganized perisylvian gyri <img src='img/arrows/WS.png'/> with a cobblestone appearance, characteristic for polymicrogyria. Other areas of cortex appear thickened <img src='img/arrows/WO.png'/> &amp; indistinct related to pachygyria.*
![Axial T2 MR shows diffuse cortical migrational anomaly with areas of pachygyria <img src='img/arrows/CS.png'/> &amp; polymicrogyria <img src='img/arrows/CO.png'/>. TORCH infections in the 1st trimester, most commonly CMV, are a common cause of nonsyndromic migrational anomalies &amp; resulting seizures.](24d1c778-116d-4f6a-8f14-03fa2c419e4e)
**Polymicrogyria**
*Axial T2 MR shows diffuse cortical migrational anomaly with areas of pachygyria <img src='img/arrows/CS.png'/> &amp; polymicrogyria <img src='img/arrows/CO.png'/>. TORCH infections in the 1st trimester, most commonly CMV, are a common cause of nonsyndromic migrational anomalies &amp; resulting seizures.*
![Axial T2 MR shows dysplastic cerebellar cortex bilaterally. This finding is often incidental &amp; not usually associated with seizure syndromes.](55b9a92c-ad20-467c-98e5-a32b6e30188f)
**Polymicrogyria**
*Axial T2 MR shows dysplastic cerebellar cortex bilaterally. This finding is often incidental &amp; not usually associated with seizure syndromes.*
![Coronal T2 MR shows septo-optic dysplasia with a small optic chiasm <img src='img/arrows/BO.png'/> &amp; absent septum pellucidum <img src='img/arrows/BS.png'/>. Note that the sella is also small <img src='img/arrows/WS.png'/>. These patients frequently also have pituitary hypofunction.](af3ebeaf-a528-4597-a016-9a3b58f84ddd)
**Septo-Optic Dysplasia Plus Syndrome**
*Coronal T2 MR shows septo-optic dysplasia with a small optic chiasm <img src='img/arrows/BO.png'/> &amp; absent septum pellucidum <img src='img/arrows/BS.png'/>. Note that the sella is also small <img src='img/arrows/WS.png'/>. These patients frequently also have pituitary hypofunction.*
![Coronal T2WI MR shows right perisylvian polymicrogyria <img src='img/arrows/WS.png'/> in this septo-optic dysplasia patient, a common association. However, schizencephaly is nearly always associated with polymicrogyria, adjacent to the schizencephalic cleft.](084fc89b-4ebe-4c06-b60a-8b2b0cb4e7a7)
**Septo-Optic Dysplasia Plus Syndrome**
*Coronal T2WI MR shows right perisylvian polymicrogyria <img src='img/arrows/WS.png'/> in this septo-optic dysplasia patient, a common association. However, schizencephaly is nearly always associated with polymicrogyria, adjacent to the schizencephalic cleft.*
![Sagittal T1 MR shows thinning of the optic chiasm <img src='img/arrows/WS.png'/> &amp; hypoplastic appearance of the anterior pituitary <img src='img/arrows/BO.png'/>, characteristic of septo-optic dysplasia. An absent septum pellucidum was also present, seen best on coronal imaging.](41662122-61cd-411c-a6a7-026e88bc5c5f)
**Septo-Optic Dysplasia Plus Syndrome**
*Sagittal T1 MR shows thinning of the optic chiasm <img src='img/arrows/WS.png'/> &amp; hypoplastic appearance of the anterior pituitary <img src='img/arrows/BO.png'/>, characteristic of septo-optic dysplasia. An absent septum pellucidum was also present, seen best on coronal imaging.*
![Axial T1 C+ MR shows a subependymal giant cell astrocytoma <img src='img/arrows/WS.png'/>, seen in 10-15% of patients with tuberous sclerosis complex. Note the associated ventriculomegaly. Multifocal subcortical tubers <img src='img/arrows/WC.png'/> are seen in the left hemisphere.](6b0eb9d3-39c7-4fa2-9f82-d1ad8eda6c88)
**Tuberous Sclerosis Complex**
*Axial T1 C+ MR shows a subependymal giant cell astrocytoma <img src='img/arrows/WS.png'/>, seen in 10-15% of patients with tuberous sclerosis complex. Note the associated ventriculomegaly. Multifocal subcortical tubers <img src='img/arrows/WC.png'/> are seen in the left hemisphere.*
![Coronal STIR MR shows multiple hyperintensities of the subcortical WM <img src='img/arrows/CS.png'/>. With the addition of a calcified subependymal nodule <img src='img/arrows/CO.png'/>, this is pathognomonic for tuberous sclerosis. Cortical tubers have a similar imaging and histological appearance with cortical dysplasia.](440c862a-9ed0-4263-9f65-980087ad9a92)
**Tuberous Sclerosis Complex**
*Coronal STIR MR shows multiple hyperintensities of the subcortical WM <img src='img/arrows/CS.png'/>. With the addition of a calcified subependymal nodule <img src='img/arrows/CO.png'/>, this is pathognomonic for tuberous sclerosis. Cortical tubers have a similar imaging and histological appearance with cortical dysplasia.*
![Coronal FLAIR MR shows numerous subcortical hyperintensities, consistent with tubers <img src='img/arrows/WS.png'/> in this tuberous sclerosis complex patient. Several subependymal nodules <img src='img/arrows/WO.png'/> are also present. Before they calcify, subependymal nodules follow WM signal.](409f1f47-c2f5-4b43-96ae-b3a10048a980)
**Tuberous Sclerosis Complex**
*Coronal FLAIR MR shows numerous subcortical hyperintensities, consistent with tubers <img src='img/arrows/WS.png'/> in this tuberous sclerosis complex patient. Several subependymal nodules <img src='img/arrows/WO.png'/> are also present. Before they calcify, subependymal nodules follow WM signal.*
![Axial T1 C+ MR shows a circumscribed cystic WM solid mass in the anterior temporal lobe <img src='img/arrows/WC.png'/>. This well-differentiated neuronal-glial tumor is the most common tumor to cause temporal lobe epilepsy.](bd480c76-c647-4c25-855b-bbff20b122e6)
**Ganglioglioma**
*Axial T1 C+ MR shows a circumscribed cystic WM solid mass in the anterior temporal lobe <img src='img/arrows/WC.png'/>. This well-differentiated neuronal-glial tumor is the most common tumor to cause temporal lobe epilepsy.*
![Axial FLAIR MR shows a tumor of the left frontal cortex with T2-hyperintense rim <img src='img/arrows/CS.png'/> &amp; heterogeneous central signal suppression <img src='img/arrows/CO.png'/>. This is an exaggerated appearance of the FLAIR rim sign &amp; microcystic variant of DNET.](9b2b925f-a6e0-4ed7-b9a7-38456d87d4b5)
**Dysembryoplastic Neuroepithelial Tumor**
*Axial FLAIR MR shows a tumor of the left frontal cortex with T2-hyperintense rim <img src='img/arrows/CS.png'/> &amp; heterogeneous central signal suppression <img src='img/arrows/CO.png'/>. This is an exaggerated appearance of the FLAIR rim sign &amp; microcystic variant of DNET.*
![Coronal T1 MR shows a nearly cystic-appearing mass in the mesial right temporal lobe <img src='img/arrows/WO.png'/>. This was a proven DNET, a neuronal tumor commonly associated with dysplastic cortex.](9cbdaa36-9731-4042-af92-b5891c66f9cf)
**Dysembryoplastic Neuroepithelial Tumor**
*Coronal T1 MR shows a nearly cystic-appearing mass in the mesial right temporal lobe <img src='img/arrows/WO.png'/>. This was a proven DNET, a neuronal tumor commonly associated with dysplastic cortex.*
![Coronal T1 MR show an enlarged right hemisphere and ventricle <img src='img/arrows/WS.png'/> compared to the left. Note ipsilateral, dysplastic-appearing GM <img src='img/arrows/WC.png'/> in this hemimegalencephaly patient.](aec66053-e0e5-4a01-a29a-ca7f7a794e04)
**Hemimegalencephaly**
*Coronal T1 MR show an enlarged right hemisphere and ventricle <img src='img/arrows/WS.png'/> compared to the left. Note ipsilateral, dysplastic-appearing GM <img src='img/arrows/WC.png'/> in this hemimegalencephaly patient.*
![Axial T2 MR shows diffuse enlargement of the left cerebral hemisphere with pachygyria and a dark band in the subcortical WM. Calcification can sometimes occur with cortical dysplasia. Note the ipsilateral ventricular enlargement <img src='img/arrows/CS.png'/>, typical of hemimegalencephaly.](9c8da539-2580-472b-9ac9-48223e173553)
**Hemimegalencephaly**
*Axial T2 MR shows diffuse enlargement of the left cerebral hemisphere with pachygyria and a dark band in the subcortical WM. Calcification can sometimes occur with cortical dysplasia. Note the ipsilateral ventricular enlargement <img src='img/arrows/CS.png'/>, typical of hemimegalencephaly.*
![Coronal T1 C+ MR shows right hemiatrophy, pial enhancement and angiomatosis <img src='img/arrows/WS.png'/> of CSF spaces. This congenital malformation has failure of cortical venous development that leads to progressive venous occlusion and ischemia.](056e47c5-5c6c-47dc-b3b2-116749a58627)
**Sturge-Weber Syndrome**
*Coronal T1 C+ MR shows right hemiatrophy, pial enhancement and angiomatosis <img src='img/arrows/WS.png'/> of CSF spaces. This congenital malformation has failure of cortical venous development that leads to progressive venous occlusion and ischemia.*
![Coronal T1 C+ FS MR shows ipsilateral enlarged choroid plexus glomus <img src='img/arrows/CS.png'/> to the facial port-wine stain. This is associated with asymmetric pial enhancement <img src='img/arrows/CO.png'/>. Hemispheric atrophy occurs late in the disease, which is not yet seen with this patient.](8ffd26ec-2013-464c-979c-e67ab14d378e)
**Sturge-Weber Syndrome**
*Coronal T1 C+ FS MR shows ipsilateral enlarged choroid plexus glomus <img src='img/arrows/CS.png'/> to the facial port-wine stain. This is associated with asymmetric pial enhancement <img src='img/arrows/CO.png'/>. Hemispheric atrophy occurs late in the disease, which is not yet seen with this patient.*
![Axial T1 C+ MR shows asymmetric leptomeningeal enhancement <img src='img/arrows/CS.png'/> of the right temporal occipital lobe, representing abnormal pial vessels. Note the right eye proptosis from an orbital vascular malformation &amp; an enhancing retinal angioma <img src='img/arrows/CO.png'/>, also seen with Sturge-Weber syndrome.](e0076b2c-b27c-4973-a501-e248f09c2b97)
**Sturge-Weber Syndrome**
*Axial T1 C+ MR shows asymmetric leptomeningeal enhancement <img src='img/arrows/CS.png'/> of the right temporal occipital lobe, representing abnormal pial vessels. Note the right eye proptosis from an orbital vascular malformation &amp; an enhancing retinal angioma <img src='img/arrows/CO.png'/>, also seen with Sturge-Weber syndrome.*
![Coronal FLAIR MR shows marked hyperintensity involving temporal cortex &amp; adjacent subcortical WM <img src='img/arrows/WS.png'/> in a patient with persistent status epilepticus. These changes resolved slowly over the following weeks.](709e7a08-fa3e-47ae-a4fd-7f2c2a74d1ca)
**Status Epilepticus**
*Coronal FLAIR MR shows marked hyperintensity involving temporal cortex &amp; adjacent subcortical WM <img src='img/arrows/WS.png'/> in a patient with persistent status epilepticus. These changes resolved slowly over the following weeks.*
![Axial T2 MR shows a thin band of GM in the deep WM of both hemispheres <img src='img/arrows/WS.png'/> in a 6- month-old. Some consider band heterotopia to be in the GM heterotopia spectrum.](1a239fd8-0e72-4edb-a345-f98d73480dba)
**Lissencephaly Type 1: Subcortical Band Heterotopia**
*Axial T2 MR shows a thin band of GM in the deep WM of both hemispheres <img src='img/arrows/WS.png'/> in a 6- month-old. Some consider band heterotopia to be in the GM heterotopia spectrum.*
![Coronal T2 MR shows decreased sulcation, primitive sylvian fissures <img src='img/arrows/WO.png'/>, &amp; thick bands of incompletely migrated cortex <img src='img/arrows/WS.png'/> consistent with band heterotopia (&quot;double cortex&quot;). Note that the thickness of the overlying cortex is inversely proportional to band heterotopia.](6eb47e9e-2727-40ef-890f-13aebf4f5c43)
**Lissencephaly Type 1: Subcortical Band Heterotopia**
*Coronal T2 MR shows decreased sulcation, primitive sylvian fissures <img src='img/arrows/WO.png'/>, &amp; thick bands of incompletely migrated cortex <img src='img/arrows/WS.png'/> consistent with band heterotopia (&quot;double cortex&quot;). Note that the thickness of the overlying cortex is inversely proportional to band heterotopia.*
docs_md/articles/fragile-x-associated-tremor-ataxia-fxtas_4778fafe-9873-4c28-8f4f-299c00c72b50.md
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---
title: "Fragile X-Associated Tremor/Ataxia (FXTAS)"
docid: "4778fafe-9873-4c28-8f4f-299c00c72b50"
authors:
- key: "a25c450b-3d34-4f64-bba3-cc0834813df6"
value: "Miral D. Jhaveri, MD, MBA"
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pageDescription: "Fragile X-Associated Tremor/Ataxia (FXTAS)"
pageKeywords: "Brain, Diagnosis, Pathology-Based Diagnoses, Acquired Toxic/Metabolic/Degenerative Disorders, Dementias and Degenerative Disorders, Fragile X-Associated Tremor/Ataxia (FXTAS)"
pageTitle: "Fragile X-Associated Tremor/Ataxia (FXTAS) | STATdx"
enhancedTitle: "Fragile X-Associated Tremor/Ataxia (FXTAS)"
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breadcrumbs:
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- "Fragile X-Associated Tremor/Ataxia (FXTAS)"
---
# KEY FACTS
- ## Terminology
- Fragile X-associated tremor/ataxia syndrome (FXTAS)
- X-linked progressive neurodegenerative disorder characterized by 55-200 CGG trinucleotide repeats in *FMR1* gene
- ## Imaging
- Ventricular and sulcal prominence: Global volume loss
- WM and brainstem hyperintensities
- MCP atrophy with symmetric hyperintensities: MCP sign
- Decreased MCP width may be first notable sign
- Splenium of corpus callosum atrophy with hyperintensity: Corpus callosum splenium sign
- High sensitivity but lower specificity than MCP sign
- ## Top Differential Diagnoses
- **Middle cerebellar peduncle sign**
- Neurodegenerative, metabolic, cerebrovascular, inflammatory and demyelinating disorders
- **Corpus callosum splenium sign**
- Normal aging, radiation therapy
- Cytotoxic/transient splenial lesions
- ## Pathology
- Premutation expansions (55-200 CGG repeats) in 5' untranslated region of *FMR1* gene, located on X-chromosome
- **Radiological criteria**
- Major: White matter lesions in brainstem or MCP sign
- Minor: Cerebral white matter lesions, moderate to severe generalized brain atrophy
- ## Clinical Issues
- Kinetic tremor, cerebellar gait ataxia, cognitive dysfunction
- Usually > age 50
- Diagnosis confirmed by molecular genetic testing
- Progressive and severe neurodegenerative disease
# TERMINOLOGY
- ## Abbreviations
- Fragile X-associated tremor/ataxia syndrome (FXTAS)
- ## Definitions
- X-linked progressive neurodegenerative disorder characterized by 55-200 CGG trinucleotide repeats in *FMR1* gene
# IMAGING
- ## General Features
- ### Best diagnostic clue
- Generalized brain atrophy with hyperintensities in brainstem or middle cerebellar peduncle (MCP)
- ### Location
- Brainstem and middle cerebellar peduncles
- Cerebral white matter (WM), corpus callosum
- ### Size
- MCP atrophy, global atrophy
- ## CT Findings
- ### NECT
- Moderate to severe generalized brain atrophy
- MCP atrophy with subtle hypodensities
- ## MR Findings
- ### T1WI
- Subtle hypointensity in MCP with atrophy
- Corpus callosum splenium hypointensity
- ### FLAIR
- Ventricular and sulcal prominence: Global volume loss
- WM and brainstem hyperintensities
- Putaminal rim hyperintensity
- MCP atrophy with symmetric hyperintensities: MCP sign
- Decreased MCP width may be first notable sign
- Splenium of corpus callosum atrophy with hyperintensity: Corpus callosum splenium sign
- High sensitivity but lower specificity than MCP sign
- ### T2* GRE
- No hemorrhage
- ### T1WI C+
- No enhancement
- ### MRS
- Decreased NAA/Cr and Ch/Cr in MCP
- DTI
- Reduced fractional anisotropy (FA) in corpus callosum
- Associated with increasing FXTAS symptom severity
- Reduced FA in MCP
- ## Imaging Recommendations
- ### Best imaging tool
- Brain MR without contrast
- ### Protocol advice
- Add coronal T2/FLAIR MR
# DIFFERENTIAL DIAGNOSIS
- ## Middle Cerebellar Peduncle Sign
- **Neurodegenerative**
- Multiple systemic atrophy (MSA), olivopontocerebellar atrophy, spinocerebellar ataxia
- **Metabolic**
- Adrenoleukodystrophy, Wilson disease, hypoglycemia
- **Cerebrovascular**
- Infarcts, PRES, pontine infarct with wallerian degeneration of MCPs
- **Inflammatory and demyelinating**
- Multiple sclerosis, ADEM, Behçet disease
- ## Corpus Callosum Splenium Sign
- Normal aging, radiation therapy
- Cytotoxic /transient splenial lesions
- Seizure- &/or drug-related, viral encephalitis, metabolic derangement
# PATHOLOGY
- ## General Features
- ### Genetics
- Premutation expansions (55-200 CGG repeats) in untranslated region of *FMR1* gene, located on X-chromosome
- Full mutation (> 200 CGG repeats) leads to neurodevelopmental disease fragile X syndrome (FXS)
- 2 main molecular mechanisms
- Toxic gain of function of expanded CGG-repeat *FMR1*mRNA → binding/sequestration of CGG-binding proteins
- CGG repeat-associated non-AUG-initiated (RAN) translation → polyglycine peptide toxic to cells
- ## Staging, Grading, & Classification
- Diagnostic criteria for FXTAS: Clinical, radiological, and pathological
- **Radiological criteria**
- Major criteria: WM lesions in brainstem or MCP sign
- Minor criteria: Cerebral WM lesions, moderate to severe generalized brain atrophy
- ## Microscopic Features
- Diffuse, spongy degeneration of WM
- Eosinophilic intranuclear inclusions in neurons and astrocytes with cortex and cerebellum
# CLINICAL ISSUES
- ## Presentation
- ### Most common signs/symptoms
- Kinetic tremor, cerebellar gait ataxia, cognitive dysfunction
- ### Other signs/symptoms
- Psychiatric disorders common
- ## Demographics
- Usually > age 50
- More common among male patients who are hemizygous for premutation (40%) than female patients who are heterozygous for premutation (8-16%)
- ## Natural History & Prognosis
- Diagnosis confirmed by molecular genetic testing
- Progressive and severe neurodegenerative disease
- ## Treatment
- Symptomatic and supportive
# DIAGNOSTIC CHECKLIST
- ## Consider
- Patient presenting with ataxia and tremor with symmetric middle cerebellar peduncle hyperintensities (MCP sign)
863ab378-3ef7-4994-a374-6fc4bb8249e5
## References
# Selected References
1. [Cvejic RC et al: Reduced caudate volume and cognitive slowing in men at risk of fragile X-associated tremor ataxia syndrome. Brain Imaging Behav. ePub, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=30046972%5Bpmid%5D)
1. [Famula JL et al: Presence of middle cerebellar peduncle sign in FMR1 premutation carriers without tremor and ataxia. Front Neurol. 9:695, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=30186228%5Bpmid%5D)
1. [Mascalchi M et al: Neuroimaging Applications in Chronic Ataxias. Int Rev Neurobiol. 143:109-162, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=30473193%5Bpmid%5D)
1. [Hall DA et al: The corpus callosum splenium sign in fragile X-associated tremor ataxia syndrome. Mov Disord Clin Pract. 4(3):383-8, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=30363360%5Bpmid%5D)
1. [Fasano A et al: MRI-guided focused ultrasound thalamotomy in fragile X-associated tremor/ataxia syndrome. Neurology. 87(7):736-8, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=27440151%5Bpmid%5D)
1. [Filley CM: Fragile X tremor ataxia syndrome and white matter dementia. Clin Neuropsychol. 30(6):901-12, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=27356088%5Bpmid%5D)
## Images
### Selected Images
![Sagittal T1 MR in a 56-year-old man with fragile X-associated tremor/ataxia syndrome (FXTAS) demonstrates atrophy of the corpus callosum <img src='img/arrows/CS.png'/>, brainstem <img src='img/arrows/CC.png'/>, and cerebellum <img src='img/arrows/CO.png'/>.](images/app.statdx.com_image_thumbnail_bd354cab-3b2c-4a44-834d-8a782f46ee4d_size_168_quality_85_ce881543_20251014T204705Z.jpg)
*Sagittal T1 MR in a 56-year-old man with fragile X-associated tremor/ataxia syndrome (FXTAS) demonstrates atrophy of the corpus callosum <img src='img/arrows/CS.png'/>, brainstem <img src='img/arrows/CC.png'/>, and cerebellum <img src='img/arrows/CO.png'/>.*
![Axial FLAIR MR in the same patient shows decreased width of the middle cerebellar peduncles with symmetric hyperintensities <img src='img/arrows/CC.png'/>, consistent with the MCP sign. This sign can be seen in a number of conditions, particularly neurodegenerative diseases, and is most commonly associated with FXTAS.](images/app.statdx.com_image_thumbnail_6e85ed35-5fce-4b42-8e4b-e22ad8384616_size_168_quality_85_6c416419_20251014T204705Z.jpg)
*Axial FLAIR MR in the same patient shows decreased width of the middle cerebellar peduncles with symmetric hyperintensities <img src='img/arrows/CC.png'/>, consistent with the MCP sign. This sign can be seen in a number of conditions, particularly neurodegenerative diseases, and is most commonly associated with FXTAS.*
![Axial FLAIR MR in a patient with tremor, cerebellar ataxia, and cognitive decline shows diffuse parenchymal volume loss, WM hyperintensities <img src='img/arrows/CC.png'/>, and hyperintensity in the splenium of the corpus callosum <img src='img/arrows/CS.png'/> (corpus callosum splenium sign).](images/app.statdx.com_image_thumbnail_139d26cb-4438-4af5-b15f-e814163805de_size_168_quality_85_ca5b409b_20251014T204705Z.jpg)
*Axial FLAIR MR in a patient with tremor, cerebellar ataxia, and cognitive decline shows diffuse parenchymal volume loss, WM hyperintensities <img src='img/arrows/CC.png'/>, and hyperintensity in the splenium of the corpus callosum <img src='img/arrows/CS.png'/> (corpus callosum splenium sign).*
![Axial T2 MR in the same patient shows atrophy of the middle cerebellar peduncles with symmetric hyperintensities <img src='img/arrows/CC.png'/> (MCP sign). The patient was diagnosed with definite FXTAS and had 166 CGG repeats on molecular genetic testing.](images/app.statdx.com_image_thumbnail_163bf33d-0795-49b8-ac7b-393d1289a00e_size_168_quality_85_e69bac40_20251014T204705Z.jpg)
*Axial T2 MR in the same patient shows atrophy of the middle cerebellar peduncles with symmetric hyperintensities <img src='img/arrows/CC.png'/> (MCP sign). The patient was diagnosed with definite FXTAS and had 166 CGG repeats on molecular genetic testing.*
docs_md/articles/frontotemporal-dementia_9f9eda8c-7e3c-4292-9861-4b8abc2c6474.md
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---
title: "Frontotemporal Dementia"
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---
# KEY FACTS
- ## Terminology
- Frontotemporal dementia (FTD): Progressive neurodegenerative disorder of frontal/anterior temporal lobes
- ## Imaging
- F-18 FDG PET
- Helps differentiate between FTD and other causes of dementia
- Glucose hypometabolism
- Initially in frontal lobes progressing to temporal lobes
- Anterior cingulate also commonly hypometabolic
- Left-sided asymmetry could suggest underlying primary progressive aphasia (PPA)
- Hypometabolism within motor strip (precentral gyrus) could suggest motor neuron disease FTD (FTD-MND)
- Perfusion SPECT
- Similar pattern of frontal hypoperfusion as F-18 FDG PET
- Potentially less sensitive than F-18 FDG PET
- ## Clinical Issues
- Progressive changes in behavior, language, or motor function depending upon subtype
- Memory is less dominant clinical feature in FTDs
- No current disease-modifying treatment for FTD, only symptomatic therapy
- ## Diagnostic Checklist
- Clinical therapy decisions depend on proper diagnosis
- FTD important to distinguish from Alzheimer disease (AD) because AD medications do not slow progression of FTD and can worsen symptoms
- Amyloid-targeting therapies (ATTs) have no role in treating FTDs
- Image analysis
- If hypometabolism is anterior-predominant (e.g., frontal, anterior temporal, anterior cingulate), this favors FTD
- Amyloid PET can also help exclude AD pathology
# TERMINOLOGY
- ## Abbreviations
- Frontotemporal dementia (FTD)
- Alzheimer disease (AD)
- ## Definitions
- Progressive neurodegenerative disorder of frontal/anterior temporal lobes
- Typically subdivided into categories based on underlying molecular aggregates [TDP-43 (50%), tauopathy without amyloid (40%), and FET protein family (10%)] and main functional deficit (cognitive and behavior, language, or motor)
- Behavioral variant FTD (bvFTD) (formerly Pick disease)
- Commonly characterized by behavioral disinhibition, apathy, loss of sympathy, hyperorality, executive deficits
- Most commonly seen with TDP-43 aggregation, initially described with intracellular τ inclusions (Pick bodies)
- Prosopagnosia (inability to recognize familiar faces) has been described with right temporal variant FTD
- Language variant FTD [FTD-primary progressive aphasia (PPA)]
- Includes 2/3 subtypes of PPA, semantic variant PPA (svPPA) and nonfluent agrammatic variant PPA (nfvPPA)
- svPPA is predominantly seen with TDP-43 pathology and nfvPPA with τ pathology
- Other PPA variant, logopenic variant (lvPPA), is atypical AD variant (amyloid and τ positive)
- Motor neuron disease FTD (FTD-MND)
- Includes amyotrophic lateral sclerosis (FTD-ALS; 95% TDP-43) as well as atypical parkinsonian syndromes of progressive supranuclear palsy (PSP; tauopathy) and corticobasal degeneration (CBD; tauopathy)
- ALS classically presents with both upper motor neuron signs (spasticity, rigidity, hyperreflexia), lower motor neuron signs (muscle fasciculations, muscle atrophy), and nonmotor signs (behavioral disturbances), as seen with bvFTD (30% of cases)
- PSP usually presents with bradykinesia, rigidity, **vertical gaze palsy**, dysphagia, dysarthria
- CBD presents with parkinsonism, dystonia, apraxia, executive dysfunction, aphasia, "alien limb" phenomenon
# IMAGING
- ## Nuclear Medicine Findings
- F-18 FDG PET/CT
- Glucose hypometabolism initially in frontal lobes with progression to include regions of temporal/parietal lobes
- Anterior cingulate cortex, frontal insula, caudate nuclei, thalamus may also have hypometabolism bilaterally
- Relative sparing of motor cortex, except in FTD-MNDs, specifically ALS and CBD, which can show hypometabolism
- Hemispheric metabolic asymmetry may be present
- Hypometabolism occurs before atrophy visually evident on CT/MR
- Most sensitive diagnostic tool currently available
- Glucose hypometabolism worsens with disease progression
- May be used to distinguish between FTD and AD
- AD often shows hypometabolism in posterior cingulate/temporoparietal regions, spared with FTD
- 50% of patients with behavioral or dysexecutive AD variant do not show typical parietal and posterior cingulate hypometabolism
- Amyloid PET can be helpful in this situation if considering amyloid-targeting therapies (ATTs)
- Attenuation correction CT can show
- Preferential atrophy of frontal/temporal lobes
- Increased CSF space surrounding medial temporal lobes
- Enlargement of lateral ventricles
- Perfusion SPECT
- Pattern is similar to F-18 FDG PET with decreased radiotracer activity in frontal/temporal lobes
- SPECT generally has less sensitivity and quantitative potential compared to PET
- More sensitive than structural MR in detecting early changes
- ## Imaging Recommendations
- ### Best imaging tool
- F-18 FDG PET helps to differentiate between FTD and other causes of dementia, e.g., AD and Lewy body dementia (LBD)
- Correlates with disease progression
- Amyloid PET can help exclude AD variants that can mimic FTDs, such as lvPPA
- I-123 ioflupane scan can help in cases of suspected PSP or CBD, which will show decreased/abnormal uptake
- CT/MR documents atrophy of mainly frontal/temporal lobe structures
- Look for reversible causes of dementia, e.g., normal-pressure hydrocephalus
- Motor band sign with T2* hypointensity of precentral gyri or T2/FLAIR hyperintensity of corticospinal tracts in ALS
- F-18 FDG PET
- Patient preparation
- Patient should fast, stop IV fluids containing dextrose, and stop parenteral feeding for 4-6 hours
- Blood sugar should be < 150-200 (mg/dL)
- Patient should be placed in quiet, dimly lit room prior to and after injection (30 min)
- Radiopharmaceutical: F-18 FDG
- Dose: 5-20 mCi (185-740 MBq)
- Dosimetry: Urinary bladder receives largest dose
- Image acquisition: Image 30-60 min after injection
- SPECT
- 2nd-line study if F-18 FDG PET is not available/reimbursed
- Patient preparation
- Patient should be placed in quiet, dimly lit room prior to and after injection (30 min)
- Radiopharmaceutical
- Tc-99m exametazime (HMPAO)
- Tc-99m ethyl cysteinate dimer (ECD)
- Dose: 15-30 mCi (555 MBq to 1.1 GBq)
- Dosimetry
- Tc-99m HMPAO: Kidneys receive highest dose
- Tc-99m ECD: Bladder wall receives highest dose
- Image acquisition
- Optimal imaging time for Tc-99m HMPAO: 90 min post injection
- Optimal imaging time for Tc-99m ECD: 45 min post injection
- ## Artifacts and Quality Control
- Immobilize patient's head to decrease motion, attenuation correction artifacts
# DIFFERENTIAL DIAGNOSIS
- [Alzheimer Disease](/document/alzheimer-disease/2aad3ac4-44fd-43e5-8e50-a86987483af3)
- Most common cause of dementia generally leading to impairments in episodic memory and other cognitive domains
- Related to aggregation of amyloid-β and τ proteins; therefore, positive on amyloid PET
- Behavioral and dysexecutive variants of AD
- Clinical presentation with less memory impairment and more behavioral disinhibition/loss of executive function
- Can be clinically indistinguishable from bvFTD
- 50% of cases show F-18 FDG hypometabolism in precuneus and posterior cingulate gyrus (similar to classic AD); 50% show frontal hypometabolism with parietal sparing similar to FTD
- Must consider amyloid PET in these cases; prerequisite for ATTs
- lvPPA
- Predominant language loss with spared memory that can mimic other PPAs
- Sentence repetition and single-word meaning usually preserved
- Often have mild cognitive impairments outside of language (more than seen with svPPA or nfvPPA)
- F-18 FDG hypometabolism shows more parietal involvement than other PPAs; amyloid PET can be helpful if considering ATTs
- ## Limbic-Predominant Age-Related TDP-43 Encephalopathy
- Newly recognized neurodegenerative disease with predominant memory deficits related to hippocampal dysfunction
- Typically in patients > 80 years
- Often slower decline than AD, though can commonly be comorbid with AD, accelerating disease progression
- F-18 FDG PET shows marked hippocampal hypometabolism with less severe involvement of precuneus and posterior cingulate
- MR often shows marked hippocampal sclerosis
- ## Vascular Dementia
- 2nd most common cause of dementia
- Caused by impaired blood supply to brain regions
- Global atrophy with diffuse white matter lesions (infarcts)
- Lesions generally correlate with cognitive symptoms
- ## Lewy Body Dementia
- Commonly presents with hallucinations, sleep disturbances, and parkinsonian motor features
- F-18 FDG PET hypometabolism in occipital cortex
- Cardiac MIBG demonstrates sympathetic denervation (CBD and PSP do not demonstrate denervation)
- Positive α-synuclein skin test (CBD and PSP are tauopathies)
- ## Psychiatric Illness
- Bipolar disorder, schizophrenia, obsessive compulsive disorder
- ## Reversible Dementias
- Mass lesions (brain tumor), head trauma, normal-pressure hydrocephalus, vitamin B12 deficiency, hypothyroidism, infections (neurosyphilis, Lyme disease)
# PATHOLOGY
- ## General Features
- ### Etiology
- Heterogeneous pathologic and clinical subtypes
- Resulted from disease naming related to clinical presentation, before etiology/pathology was well understood
- Pathologic subtypes of FTD are classified based upon pattern of protein accumulation in groups encompassing disorders of frontotemporal lobar degeneration
- Etiology uncertain but associated with 3 major protein aggregates
- τ (microtubule-associated protein)
- TDP-43 (transactive response DNA binding protein of 43kD)
- FET protein family, including FUS (tumor-associated protein; fused in sarcoma)
- ### Genetics
- Autosomal dominant inheritance in 10-25% of FTD cases
- c9orf72 most common genetic mutation in familial FTD and familial ALS
- *SOD1*(superoxide dismutase) gene mutation in 10% of familial ALS with specific treatment, intrathecal tofersen
- Positive family history of FTD is only known risk factor
- 30-50% of individuals with bvFTD have positive family history
# CLINICAL ISSUES
- ## Presentation
- ### Most common signs/symptoms
- Progressive changes in behavior, personality, language, &/or motor function, depending upon clinical subtype
- Disinhibition, apathy, loss of sympathy, hyperorality, dysexecutive behaviors
- Bradykinesia, rigidity, tremor, spasticity, hyperreflexia, fasciculations, muscle atrophy in motor subtypes
- Loss of language comprehension, including word meaning or agrammatism in language subtypes
- ### Clinical profile
- FTD is composed of 3 main clinical subtypes
- bvFTD
- Most common, accounting for ~ 1/2 of cases
- Progressive decline in social function with personality changes, often with disinhibition
- Language presentation (PPA)
- Including variants svPPA and nfvPPA
- Motor presentation
- ALS, PSP, CBD
- ## Demographics
- ### Age
- Mean of onset: 50-60 years
- ~ 10% > 70 years
- Younger onset than AD, which is generally > 65 years
- ## Natural History & Prognosis
- Insidious onset of behavioral and cognitive dysfunction
- More significant behavioral, language, executive functioning impairment than memory
- Slowly progressive with eventual functional impairment
- Median survival ~ 8-10 years after diagnosis; varies widely based upon underlying pathology
- Median survival in ALS is 2-5 years
- ## Treatment
- No current disease-modifying treatment for FTD
- ALS with *SOD1*mutation can be treated with intrathecal tofersen (6.5-month increase in median survival at 3 years)
# DIAGNOSTIC CHECKLIST
- ## Consider
- FTD important to distinguish from AD because AD medications do not slow progression of FTD and can worsen symptoms
- FTDs are not amenable to ATTs due to lack of underlying amyloid pathology
- ## Image Interpretation Pearls
- When analyzing images, use surface projections and normative dataset comparison to increase sensitivity
c96633b0-f435-4b0a-9f06-702857dfe4c4
## References
# Selected References
1. [Nelson PT: New criteria to predict LATE-NC in the clinical setting: probable/possible LATE and LANS. J Neuropathol Exp Neurol. 84(1):2-7, 2025](http://www.ncbi.nlm.nih.gov/pubmed/?term=39441698%5Bpmid%5D)
1. [Antonioni A et al: Frontotemporal dementia, where do we stand? A narrative review. Int J Mol Sci. 24(14):11732, 2023](http://www.ncbi.nlm.nih.gov/pubmed/?term=37511491%5Bpmid%5D)
1. [Paganoni S et al: Long-term survival of participants in the CENTAUR trial of sodium phenylbutyrate-taurursodiol in amyotrophic lateral sclerosis. Muscle Nerve. 63(1):31-9, 2021](http://www.ncbi.nlm.nih.gov/pubmed/?term=33063909%5Bpmid%5D)
1. [Bergeron D et al: The behavioral/dysexecutive variant of Alzheimer's disease: a case series with clinical, neuropsychological, and FDG-PET characterization. Dement Geriatr Cogn Disord. 49(5):518-25, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=33207355%5Bpmid%5D)
1. [Wilson H et al: Dementia spectrum disorders: lessons learnt from decades with PET research. J Neural Transm (Vienna). 126(3):233-51, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=30762136%5Bpmid%5D)
1. [Vijverberg EG et al: Diagnostic accuracy of MRI and additional [18F]FDG-PET for behavioral variant frontotemporal dementia in patients with late onset behavioral changes. J Alzheimers Dis. 53(4):1287-97, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=27372646%5Bpmid%5D)
1. [Brown RK et al: Brain PET in suspected dementia: patterns of altered FDG metabolism. Radiographics. 34(3):684-701, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=24819789%5Bpmid%5D)
## Images
### Selected Images
![Axial F-18 FDG PET shows findings associated with frontotemporal dementia (FTD), predominantly frontal <img src='img/arrows/WS.png'/> and temporal lobe hypometabolism. Functional changes may occur prior to CT or MR changes.](images/app.statdx.com_image_thumbnail_01274ce7-fce5-4f42-bd69-b5a99e6a4930_size_168_quality_85_6a0c97a6_20251014T185357Z.jpg)
*Axial F-18 FDG PET shows findings associated with frontotemporal dementia (FTD), predominantly frontal <img src='img/arrows/WS.png'/> and temporal lobe hypometabolism. Functional changes may occur prior to CT or MR changes.*
![Axial F-18 FDG PET shows findings associated with frontotemporal dementia (FTD), predominantly frontal <img src='img/arrows/WS.png'/> and temporal lobe hypometabolism. Functional changes may occur prior to CT or MR changes.](images/app.statdx.com_image_thumbnail_01274ce7-fce5-4f42-bd69-b5a99e6a4930_size_168_quality_85_8de4c587_20251014T193351Z.jpg)
*Axial F-18 FDG PET shows findings associated with frontotemporal dementia (FTD), predominantly frontal <img src='img/arrows/WS.png'/> and temporal lobe hypometabolism. Functional changes may occur prior to CT or MR changes.*
![Axial F-18 FDG PET shows findings associated with frontotemporal dementia (FTD), predominantly frontal <img src='img/arrows/WS.png'/> and temporal lobe hypometabolism. Functional changes may occur prior to CT or MR changes.](images/app.statdx.com_image_thumbnail_01274ce7-fce5-4f42-bd69-b5a99e6a4930_size_174_quality_85_2d76707e_20251014T190917Z.jpg)
*Axial F-18 FDG PET shows findings associated with frontotemporal dementia (FTD), predominantly frontal <img src='img/arrows/WS.png'/> and temporal lobe hypometabolism. Functional changes may occur prior to CT or MR changes.*
![Axial F-18 FDG PET shows findings associated with frontotemporal dementia (FTD), predominantly frontal <img src='img/arrows/WS.png'/> and temporal lobe hypometabolism. Functional changes may occur prior to CT or MR changes.](images/app.statdx.com_image_thumbnail_01274ce7-fce5-4f42-bd69-b5a99e6a4930_size_174_quality_85_39bfb958_20251014T185333Z.jpg)
*Axial F-18 FDG PET shows findings associated with frontotemporal dementia (FTD), predominantly frontal <img src='img/arrows/WS.png'/> and temporal lobe hypometabolism. Functional changes may occur prior to CT or MR changes.*
![Axial F-18 FDG PET shows findings associated with frontotemporal dementia (FTD), predominantly frontal <img src='img/arrows/WS.png'/> and temporal lobe hypometabolism. Functional changes may occur prior to CT or MR changes.](images/app.statdx.com_image_thumbnail_01274ce7-fce5-4f42-bd69-b5a99e6a4930_size_174_quality_85_9adcd758_20251014T193347Z.jpg)
*Axial F-18 FDG PET shows findings associated with frontotemporal dementia (FTD), predominantly frontal <img src='img/arrows/WS.png'/> and temporal lobe hypometabolism. Functional changes may occur prior to CT or MR changes.*
![Axial F-18 FDG PET shows findings associated with frontotemporal dementia (FTD), predominantly frontal <img src='img/arrows/WS.png'/> and temporal lobe hypometabolism. Functional changes may occur prior to CT or MR changes.](images/app.statdx.com_image_thumbnail_01274ce7-fce5-4f42-bd69-b5a99e6a4930_size_174_quality_85_aa4b0e9b_20251014T204456Z.jpg)
*Axial F-18 FDG PET shows findings associated with frontotemporal dementia (FTD), predominantly frontal <img src='img/arrows/WS.png'/> and temporal lobe hypometabolism. Functional changes may occur prior to CT or MR changes.*
![Axial CT shows classic morphologic changes of FTD. Note advanced bilateral frontal lobe atrophy <img src='img/arrows/BS.png'/>. In this case, there is also advanced bilateral temporal lobe atrophy (not shown).](images/app.statdx.com_image_thumbnail_2009b267-4975-4e43-93d0-a25ffbdae3b7_size_168_quality_85_aef47456_20251014T193351Z.jpg)
*Axial CT shows classic morphologic changes of FTD. Note advanced bilateral frontal lobe atrophy <img src='img/arrows/BS.png'/>. In this case, there is also advanced bilateral temporal lobe atrophy (not shown).*
![Axial CT shows classic morphologic changes of FTD. Note advanced bilateral frontal lobe atrophy <img src='img/arrows/BS.png'/>. In this case, there is also advanced bilateral temporal lobe atrophy (not shown).](images/app.statdx.com_image_thumbnail_2009b267-4975-4e43-93d0-a25ffbdae3b7_size_168_quality_85_c17622e9_20251014T185357Z.jpg)
*Axial CT shows classic morphologic changes of FTD. Note advanced bilateral frontal lobe atrophy <img src='img/arrows/BS.png'/>. In this case, there is also advanced bilateral temporal lobe atrophy (not shown).*
![Axial F-18 FDG PET shows findings associated with FTD, predominantly frontal <img src='img/arrows/WS.png'/> and temporal lobe hypometabolism, the latter not shown.](images/app.statdx.com_image_thumbnail_26b3d890-8b8a-4e21-88af-0ec0cf4db55f_size_168_quality_85_70f69db3_20251014T193351Z.jpg)
*Axial F-18 FDG PET shows findings associated with FTD, predominantly frontal <img src='img/arrows/WS.png'/> and temporal lobe hypometabolism, the latter not shown.*
![Axial F-18 FDG PET shows findings associated with FTD, predominantly frontal <img src='img/arrows/WS.png'/> and temporal lobe hypometabolism, the latter not shown.](images/app.statdx.com_image_thumbnail_26b3d890-8b8a-4e21-88af-0ec0cf4db55f_size_168_quality_85_7a117327_20251014T185357Z.jpg)
*Axial F-18 FDG PET shows findings associated with FTD, predominantly frontal <img src='img/arrows/WS.png'/> and temporal lobe hypometabolism, the latter not shown.*
![Axial fused F-18 FDG PET/CTs in the same patient show predominantly frontal hypometabolism in purple/blue areas <img src='img/arrows/WS.png'/>, signifying regions with a z-score &gt; -2, or 2 standard deviations less than expected in normal controls.](images/app.statdx.com_image_thumbnail_3007ad36-74c0-4dfa-9d76-28263d830da3_size_168_quality_85_8228902a_20251014T185357Z.jpg)
*Axial fused F-18 FDG PET/CTs in the same patient show predominantly frontal hypometabolism in purple/blue areas <img src='img/arrows/WS.png'/>, signifying regions with a z-score &gt; -2, or 2 standard deviations less than expected in normal controls.*
![Axial fused F-18 FDG PET/CTs in the same patient show predominantly frontal hypometabolism in purple/blue areas <img src='img/arrows/WS.png'/>, signifying regions with a z-score &gt; -2, or 2 standard deviations less than expected in normal controls.](images/app.statdx.com_image_thumbnail_3007ad36-74c0-4dfa-9d76-28263d830da3_size_168_quality_85_eec9766a_20251014T193351Z.jpg)
*Axial fused F-18 FDG PET/CTs in the same patient show predominantly frontal hypometabolism in purple/blue areas <img src='img/arrows/WS.png'/>, signifying regions with a z-score &gt; -2, or 2 standard deviations less than expected in normal controls.*
![Sagittal F-18 FDG PET in a patient with FTD shows predominantly frontal hypometabolism <img src='img/arrows/WS.png'/>.](images/app.statdx.com_image_thumbnail_798f0fe3-db0e-4f0c-8224-00baa631ceaa_size_168_quality_85_2d108137_20251014T185357Z.jpg)
*Sagittal F-18 FDG PET in a patient with FTD shows predominantly frontal hypometabolism <img src='img/arrows/WS.png'/>.*
![Sagittal F-18 FDG PET in a patient with FTD shows predominantly frontal hypometabolism <img src='img/arrows/WS.png'/>.](images/app.statdx.com_image_thumbnail_798f0fe3-db0e-4f0c-8224-00baa631ceaa_size_168_quality_85_ceb2c50c_20251014T193351Z.jpg)
*Sagittal F-18 FDG PET in a patient with FTD shows predominantly frontal hypometabolism <img src='img/arrows/WS.png'/>.*
![F-18 FDG PET shows reference maps (1st row), healthy older person map of glucose metabolism (2nd row), and patient's glucose metabolism (3rd row), which demonstrates strikingly diminished metabolism in the frontal and temporal lobes. Z-score map (4th row) illustrates areas of hypometabolism (compared to normal controls) in the frontal <img src='img/arrows/WS.png'/> and temporal lobe <img src='img/arrows/WO.png'/>. (Courtesy University of Utah Medical Center.)](images/app.statdx.com_image_thumbnail_914739ca-b8fe-41c7-8562-951cbb701c39_size_168_quality_85_5d924cc2_20251014T185357Z.jpg)
*F-18 FDG PET shows reference maps (1st row), healthy older person map of glucose metabolism (2nd row), and patient's glucose metabolism (3rd row), which demonstrates strikingly diminished metabolism in the frontal and temporal lobes. Z-score map (4th row) illustrates areas of hypometabolism (compared to normal controls) in the frontal <img src='img/arrows/WS.png'/> and temporal lobe <img src='img/arrows/WO.png'/>. (Courtesy University of Utah Medical Center.)*
![F-18 FDG PET shows reference maps (1st row), healthy older person map of glucose metabolism (2nd row), and patient's glucose metabolism (3rd row), which demonstrates strikingly diminished metabolism in the frontal and temporal lobes. Z-score map (4th row) illustrates areas of hypometabolism (compared to normal controls) in the frontal <img src='img/arrows/WS.png'/> and temporal lobe <img src='img/arrows/WO.png'/>. (Courtesy University of Utah Medical Center.)](images/app.statdx.com_image_thumbnail_914739ca-b8fe-41c7-8562-951cbb701c39_size_168_quality_85_bc8fb2f1_20251014T193351Z.jpg)
*F-18 FDG PET shows reference maps (1st row), healthy older person map of glucose metabolism (2nd row), and patient's glucose metabolism (3rd row), which demonstrates strikingly diminished metabolism in the frontal and temporal lobes. Z-score map (4th row) illustrates areas of hypometabolism (compared to normal controls) in the frontal <img src='img/arrows/WS.png'/> and temporal lobe <img src='img/arrows/WO.png'/>. (Courtesy University of Utah Medical Center.)*
![Axial fused FDG PET/MR in a 61-year-old man with apathy, disinhibition, and lack of empathy shows subtle decreased metabolism in the medial frontal lobes <img src='img/arrows/WS.png'/>. Not pictured is decreased activity in the right temporal lobe.](images/app.statdx.com_image_thumbnail_d62aedb5-495e-4202-bd30-5b9ec678aefc_size_168_quality_85_83c2a2bf_20251014T185357Z.jpg)
*Axial fused FDG PET/MR in a 61-year-old man with apathy, disinhibition, and lack of empathy shows subtle decreased metabolism in the medial frontal lobes <img src='img/arrows/WS.png'/>. Not pictured is decreased activity in the right temporal lobe.*
![Axial fused FDG PET/MR in a 61-year-old man with apathy, disinhibition, and lack of empathy shows subtle decreased metabolism in the medial frontal lobes <img src='img/arrows/WS.png'/>. Not pictured is decreased activity in the right temporal lobe.](images/app.statdx.com_image_thumbnail_d62aedb5-495e-4202-bd30-5b9ec678aefc_size_168_quality_85_dd8c482b_20251014T193351Z.jpg)
*Axial fused FDG PET/MR in a 61-year-old man with apathy, disinhibition, and lack of empathy shows subtle decreased metabolism in the medial frontal lobes <img src='img/arrows/WS.png'/>. Not pictured is decreased activity in the right temporal lobe.*
![Surface projection in the same patient corroborates mild regional hypometabolism within the medial frontal lobes <img src='img/arrows/BS.png'/> and the bilateral temporal lobes <img src='img/arrows/WO.png'/> with preservation of the precuneus. This is suggestive of early FTD.](images/app.statdx.com_image_thumbnail_5da45f18-616e-4b1f-8c10-264f78644ec5_size_168_quality_85_98a02e63_20251014T193351Z.jpg)
*Surface projection in the same patient corroborates mild regional hypometabolism within the medial frontal lobes <img src='img/arrows/BS.png'/> and the bilateral temporal lobes <img src='img/arrows/WO.png'/> with preservation of the precuneus. This is suggestive of early FTD.*
![Surface projection in the same patient corroborates mild regional hypometabolism within the medial frontal lobes <img src='img/arrows/BS.png'/> and the bilateral temporal lobes <img src='img/arrows/WO.png'/> with preservation of the precuneus. This is suggestive of early FTD.](images/app.statdx.com_image_thumbnail_5da45f18-616e-4b1f-8c10-264f78644ec5_size_168_quality_85_fac41143_20251014T185357Z.jpg)
*Surface projection in the same patient corroborates mild regional hypometabolism within the medial frontal lobes <img src='img/arrows/BS.png'/> and the bilateral temporal lobes <img src='img/arrows/WO.png'/> with preservation of the precuneus. This is suggestive of early FTD.*
![Axial fused FDG PET/MR shows L &gt; R frontal hypometabolism <img src='img/arrows/WS.png'/> as well as left parietal hypometabolism <img src='img/arrows/CS.png'/> in a 51-year-old woman with severe frontal dysexecutive features.](images/app.statdx.com_image_thumbnail_55b4cbe7-2cbc-4320-87f8-c9734d543a1f_size_168_quality_85_817945c4_20251014T185357Z.jpg)
*Axial fused FDG PET/MR shows L &gt; R frontal hypometabolism <img src='img/arrows/WS.png'/> as well as left parietal hypometabolism <img src='img/arrows/CS.png'/> in a 51-year-old woman with severe frontal dysexecutive features.*
![Axial fused FDG PET/MR shows L &gt; R frontal hypometabolism <img src='img/arrows/WS.png'/> as well as left parietal hypometabolism <img src='img/arrows/CS.png'/> in a 51-year-old woman with severe frontal dysexecutive features.](images/app.statdx.com_image_thumbnail_55b4cbe7-2cbc-4320-87f8-c9734d543a1f_size_168_quality_85_fe5bfe2b_20251014T193351Z.jpg)
*Axial fused FDG PET/MR shows L &gt; R frontal hypometabolism <img src='img/arrows/WS.png'/> as well as left parietal hypometabolism <img src='img/arrows/CS.png'/> in a 51-year-old woman with severe frontal dysexecutive features.*
![Surface projection in the same patient shows focal hypometabolism within the L &gt; R precuneus and parietal lobes <img src='img/arrows/CS.png'/> as well as L &gt; R frontal and temporal lobes <img src='img/arrows/WS.png'/>, most consistent with early-onset dysexecutive Alzheimer disease (AD), rather than FTD.](images/app.statdx.com_image_thumbnail_9277ec72-9a19-46ec-a90b-440c881bdcab_size_168_quality_85_5afed927_20251014T185357Z.jpg)
*Surface projection in the same patient shows focal hypometabolism within the L &gt; R precuneus and parietal lobes <img src='img/arrows/CS.png'/> as well as L &gt; R frontal and temporal lobes <img src='img/arrows/WS.png'/>, most consistent with early-onset dysexecutive Alzheimer disease (AD), rather than FTD.*
![Surface projection in the same patient shows focal hypometabolism within the L &gt; R precuneus and parietal lobes <img src='img/arrows/CS.png'/> as well as L &gt; R frontal and temporal lobes <img src='img/arrows/WS.png'/>, most consistent with early-onset dysexecutive Alzheimer disease (AD), rather than FTD.](images/app.statdx.com_image_thumbnail_9277ec72-9a19-46ec-a90b-440c881bdcab_size_168_quality_85_f27510b2_20251014T193351Z.jpg)
*Surface projection in the same patient shows focal hypometabolism within the L &gt; R precuneus and parietal lobes <img src='img/arrows/CS.png'/> as well as L &gt; R frontal and temporal lobes <img src='img/arrows/WS.png'/>, most consistent with early-onset dysexecutive Alzheimer disease (AD), rather than FTD.*
docs_md/articles/frontotemporal-lobar-degeneration_49510d0e-acf7-45cb-9eb1-53f8193b0b6d.md
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title: "Frontotemporal Lobar Degeneration"
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value: "Santhosh Gaddikeri, MD"
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pageDescription: "Frontotemporal Lobar Degeneration"
pageKeywords: "Brain, Diagnosis, Pathology-Based Diagnoses, Acquired Toxic/Metabolic/Degenerative Disorders, Dementias and Degenerative Disorders, Frontotemporal Lobar Degeneration"
pageTitle: "Frontotemporal Lobar Degeneration | STATdx"
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---
# KEY FACTS
- ## Terminology
- Clinical subtypes
- Behavioral variant frontotemporal dementia **(bvFTD)**
- Primary progressive aphasia syndromes **(PPA)**
- Semantic variant **(sv-PPA)**: Previously known as semantic dementia
- Nonfluent/agrammatic variant **(nfv-PPA)**: Previously known as progressive nonfluent aphasia
- Logopenic variant **(lv-PPA)**
- Frontotemporal dementia with motor symptoms
- ## Imaging
- Early
- PET shows frontotemporal ↓ glucose metabolism
- Late: Frontotemporal atrophy with knife-like gyri on MR
- Subtypes have characteristic cortical atrophy patterns
- ## Top Differential Diagnoses
- Alzheimer dementia (AD)
- Vascular dementia
- Corticobasal ganglionic degeneration (CBD)
- Dementia with Lewy bodies (DLB)
- ## Clinical Issues
- Clinical syndromes (some overlap)
- **bvFTD**: Disinhibition, apathy & loss of empathy, hyperorality, & compulsive behavior
- **sv-PPA**: Impaired single-word comprehension & object naming with preserved fluency, repetition, & grammar
- **nfv-PPA**: Effortful speech production of phonemes (linguistic units of sound) & orofacial apraxia
- **lv-PPA**: Impaired word finding & repetition with errors in speech & naming
- Younger age group than AD
- FTLD most common cause of early-onset (< 65 years) dementia
- Median survival: 6-11 years from symptom onset & 3-4 years from diagnosis
# TERMINOLOGY
- ## Abbreviations
- Frontotemporal lobar degeneration (FTLD)
- Clinical subtypes
- Behavioral variant frontotemporal dementia **(bvFTD)**
- Primary progressive aphasia syndromes **(PPA)**
- Semantic variant **(sv-PPA)**: Previously known as semantic dementia
- Nonfluent/agrammatic variant **(nfv-PPA)**: Previously known as progressive nonfluent aphasia
- Logopenic variant**(lv-PPA)**: Has Alzheimer pathology & is not included as 1 of 3 clinical FTD syndromes
- Frontotemporal dementia (FTD) with motor symptoms
- Corticobasal degeneration (CBD)
- Progressive supranuclear palsy (PSP)
- FTD with motor neuron disease
- FTD with amyotrophic lateral sclerosis (ALS)
- ## Synonyms
- Pick disease no longer used
- Referred to pathologic variant with Pick bodies
- ## Definitions
- Heterogeneous family of neurodegenerative disorders characterized by focal lobar degeneration of frontal &/or temporal lobes
# IMAGING
- ## General Features
- ### Best diagnostic clue
- Structural & functional imaging are supportive but not diagnostic of FTD
- PET showing frontotemporal ↓ glucose metabolism
- Anterior frontotemporal atrophy with knife-like gyri
- ### Location
- Anterior temporal/frontal lobes, orbitofrontal cortex, medial temporal region
- Relative sparing of parietooccipital lobes
- ### Morphology
- Knife blade appearance of atrophic gyri
- ± marked asymmetry
- May have worst atrophy in dominant hemisphere
- ## CT Findings
- ### NECT
- Frontal lobe atrophy often most prominent feature
- ↑ size of frontal horns (larger than rest of lateral ventricles)
- ## MR Findings
- ### T1WI
- Atrophy of frontal & temporal lobes, often asymmetric
- Knife-like gyri with normal signal
- Dilated frontal sulci reflecting atrophy
- Relative sparing of parietooccipital lobes
- ### T2WI
- ± hyperintensity in frontotemporal white matter (WM)
- ### FLAIR
- ± hyperintensity in frontotemporal WM
- ### MRS
- ↓ NAA glutamate + glutamine (neuronal loss), ↑ myoinositol (↑ glial content) in frontal lobes
- ↓ NAA in posterior cingulate gyri
- Reflects ↓ neuronal population, viability
- ± lactate peak in frontal lobes
- MR voxel-based morphometry
- Subtypes have characteristic cortical atrophy patterns
- Frontal vs. temporal, left vs. right help discriminate
- **bvFTD**: Atrophy of frontal & temporal lobes
- Anterior insula, anterior cingulate, orbitofrontal cortex, & amygdala (early changes occur in right hemisphere)
- **sv-PPA**: Typically anterior temporal lobe atrophy (asymmetric to left)
- Entire temporal lobe can be involved
- Ventromedial & superior frontal lobes
- Right temporal atrophy as disease progresses
- **nfv-PPA**: Selective left posterior frontoinsular region
- **lv-PPA**: Predominant atrophy of left posterior temporal cortex & parietal lobe
- DTI
- Widespread damage to WM tracts reported
- **bvFTD**: Uncinate fasciculus, inferior longitudinal fasciculus, & anterior commissural fibers
- **sv-PPA**: Inferior longitudinal & uncinate fasciculi
- **nfv-FTD**: Left superior longitudinal fasciculus
- **lv-PPA**: Widespread dorsal & ventral WM tracts
- ## Nuclear Medicine Findings
- ### PET
- Functional imaging more sensitive than MR in early-stage disease
- FDG PET: ↓ metabolic activity in frontotemporal cortex
- Amyloid PET helps differentiate FTLD from Alzheimer disease (AD)
- HMPAO-SPECT
- Sensitive technique for early detection of FTD
- Occurs before atrophy is evident
- **bvFTD**: ↓ perfusion frontal & anterior temporal lobes
- Asymmetric, left or right dominant
- **sv-PPA**: Prominent anterior temporal hypoperfusion, left > right
- **nfv-PPA**: Asymmetric frontal hypoperfusion often involving insular cortex
- **lv-PPA**: ↓ perfusion in left parietal inferior lobule & posterolateral temporal lobe
- SPECT perfusion deficits predominantly in frontal & anterior temporal lobes with preserved perfusion posteriorly
- Helps distinguish FTD from AD
- Reduced frontal perfusion is not specific to FTD but also occurs in some cases of schizophrenia, depression, HIV encephalopathy, Creutzfeldt-Jakob disease, AD
- ## Imaging Recommendations
- ### Best imaging tool
- PET/SPECT; MR voxel-based morphometry
- ### Protocol advice
- Routine T1WI, T2WI, coronal T2WI MR
# DIFFERENTIAL DIAGNOSIS
- [Alzheimer Disease](/document/alzheimer-disease/f71f5cf5-b1af-4c6d-b145-b4c10eec7b58)
- Parietal & temporal cortical atrophy with disproportionate hippocampal volume loss
- Increased rate of atrophy in FTD compared to AD
- Often coexisting microvascular disease, WM hyperintensities, microhemorrhages
- Amyloid imaging (11C-labeled Pittsburgh Compound-B) helps to differentiate AD from other dementias
- [Vascular Dementia](/document/vascular-dementia/f59dab57-c511-4369-8fcc-592421a4b8d1)
- 2nd most common dementia (15-30%)
- WM & deep gray lacunae
- Hyperintense lesions on T2WI & focal atrophy is suggestive of chronic infarcts
- [Corticobasal Degeneration](/document/corticobasal-degeneration/23f97d4e-8724-4229-b9f8-08f63906ebd8)
- Prominent extrapyramidal, cortical symptoms
- Severe frontoparietal atrophy contralateral to more severely affected clinically
- Atrophy of paracentral structures
- [Dementia With Lewy Bodies](/document/dementia-with-lewy-bodies/e8e46d1d-46d2-4e5a-880f-f025a84c5871)
- Hypometabolism of entire brain, especially visual cortex
- Visual & auditory hallucinations, paranoid delusions
# PATHOLOGY
- ## General Features
- ### Etiology
- Tau protein (hyperphosphorylated microtubular protein) or TDP-43 (TAR DNA-binding protein-43)
- Rare cases change on fused-in-sarcoma (FUS) protein
- ### Genetics
- FTD is highly heritable without clear inheritance pattern
- 25-40% of FTD is familial, > 50% of bvFTD is autosomal dominant
- Mutations in following 3 genes together constitute 15% of FTD cases
- Most common: Hexanucleotide expansion in chromosome 9 open reading frame 72 (*C9orf72*) gene
- Microtubule-associated protein tau (*MAPT*) gene
- Granulin precursor (*GRN*) gene
- ## Staging, Grading, & Classification
- Histopathologic classification of FTLD based on abnormal inclusions
- FTLD-tau: Tau inclusion (hyperphosphorylated tau protein)/Pick bodies
- FTLD-TDP: Tau-negative & TDP-43-positive inclusions (subtypes: Type A, B, C, & D)
- FTLD-FUS: Tau-/TDP-negative & FUS-positive inclusions
- FTLD-ALS/dipeptide repeats (DPR): TDP-negative DPR protein aggregates
- FTLD-ni: No inclusions
- FTD clinical syndromes correlate with brain atrophy patterns & not with pathologic subtypes
- ## Gross Pathologic & Surgical Features
- Gross atrophy of frontal &/or anterior temporal lobes
- Firm cortical gray matter (gliosis) &/or basal ganglia atrophy
- Soft, retracted subcortical WM
- ## Microscopic Features
- Loss of pyramidal neurons & microvacuolar degeneration in layer II & III of frontal & temporal cortex
- Subjacent WM shows axonal & myelin loss
- FTLD-related tauopathies
- Pick disease: Prototypical tauopathy of FTLD
- Characterized by Pick bodies: Solitary, round or oval, argyrophilic inclusions in cytoplasm of neurons
- Commonly found in dentate gyrus of hippocampus, amygdala, frontal & temporal neocortex
# CLINICAL ISSUES
- ## Presentation
- ### Most common signs/symptoms
- Personality, behavior, & language changes
- Memory loss, confusion, cognitive & speech dysfunction, apathy, & abulia
- ### Clinical profile
- **bvFTD**: Disinhibition, apathy & loss of empathy, hyperorality, & compulsive behavior
- 15-20% may develop concomitant motor neuron disease (MND)
- **sv-PPA**: Impaired single-word comprehension & object naming with preserved fluency, repetition, & grammar
- **nfv-PPA**: Effortful speech production of phonemes (linguistic units of sound) & orofacial apraxia
- **lv-PPA**: Impaired word finding & repetition with errors in speech & naming
- ## Demographics
- ### Age
- More common cause of early-onset (midlife) dementia
- Mean age of onset is 58 years; rare < 40 & > 75 years
- Peak incidence 45-65 years
- ### Sex
- bvFTD & sv-FTD: Male preponderance
- nfv-PPA: Female predominance
- ### Ethnicity
- Familial forms of Pick complex dementias particularly common in people of Scandinavian origin
- ### Epidemiology
- FTLD more common cause of early-onset (< 65 years) dementia
- Age > 65 years account for 20-25% of cases of FTLD
- Prevalence: 3.5-15/100,000 person-years
- FTLD accounts for ~ 5% of all pathologic diagnoses in patients with dementia
- ## Natural History & Prognosis
- Insidious onset of behavioral & cognitive dysfunction
- Speech & language disturbance are often more profound than memory disorder
- Median survival 6-11 years from symptom onset & 3-4 years from diagnosis
- Currently no FDA-approved disease-modifying drugs available for treatment of FTD
- Some patients develop artistic talents during course of dementia (disinhibition of "creative" brain areas)
# DIAGNOSTIC CHECKLIST
- ## Consider
- Other common forms of dementia (AD, dementia with Lewy bodies)
- ## Image Interpretation Pearls
- Bilateral frontal lobe atrophy should make one consider diagnosis of FTD
- Bilateral asymmetric anterior temporal lobe atrophy: sv-PPA
- ## Reporting Tips
- Report pattern of cortical volume loss
52f7139c-657b-4ff0-8be5-0f3d7523130e
## References
# Selected References
1. [Ishii K: Diagnostic imaging of dementia with Lewy bodies, frontotemporal lobar degeneration, and normal pressure hydrocephalus. Jpn J Radiol. 38(1):64-76, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=31549279%5Bpmid%5D)
1. [Raji CA et al: Overview of MR imaging volumetric quantification in neurocognitive disorders. Top Magn Reson Imaging. 28(6):311-5, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31794503%5Bpmid%5D)
1. [Risacher SL et al: Neuroimaging in aging and neurologic diseases. Handb Clin Neurol. 167:191-227, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31753134%5Bpmid%5D)
1. [Shepherd TM et al: Clinical use of integrated positron emission tomography-magnetic resonance imaging for dementia patients. Top Magn Reson Imaging. 28(6):299-310, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31794502%5Bpmid%5D)
1. [Zukotynski K et al: PET/CT of dementia. AJR Am J Roentgenol. 211(2):246-59, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=29949415%5Bpmid%5D)
1. [Mann DM et al: Frontotemporal lobar degeneration: pathogenesis, pathology and pathways to phenotype. Brain Pathol. 27(6):723-36, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=28100023%5Bpmid%5D)
1. [Bang J et al: Frontotemporal dementia. Lancet. 386(10004):1672-82, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=26595641%5Bpmid%5D)
1. [Shivamurthy VK et al: Brain FDG PET and the diagnosis of dementia. AJR Am J Roentgenol. 204(1):W76-85, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25539279%5Bpmid%5D)
1. [Bott NT et al: Frontotemporal dementia: diagnosis, deficits and management. Neurodegener Dis Manag. 4(6):439-54, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=25531687%5Bpmid%5D)
1. [Chare L et al: New criteria for frontotemporal dementia syndromes: clinical and pathological diagnostic implications. J Neurol Neurosurg Psychiatry. 85(8):865-70, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=24421286%5Bpmid%5D)
1. [Diehl-Schmid J et al: Imaging frontotemporal lobar degeneration. Curr Neurol Neurosci Rep. 14(10):489, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=25171901%5Bpmid%5D)
1. [Bhogal P et al: The common dementias: a pictorial review. Eur Radiol. 23(12):3405-17, 2013](http://www.ncbi.nlm.nih.gov/pubmed/?term=24081643%5Bpmid%5D)
1. [Risacher SL et al: Neuroimaging biomarkers of neurodegenerative diseases and dementia. Semin Neurol. 33(4):386-416, 2013](http://www.ncbi.nlm.nih.gov/pubmed/?term=24234359%5Bpmid%5D)
1. [Gorno-Tempini ML et al: Classification of primary progressive aphasia and its variants. Neurology. 76(11):1006-14, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=21325651%5Bpmid%5D)
1. [Rascovsky K et al: Sensitivity of revised diagnostic criteria for the behavioural variant of frontotemporal dementia. Brain. 134(Pt 9):2456-77, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=21810890%5Bpmid%5D)
1. [Krueger CE et al: Longitudinal rates of lobar atrophy in frontotemporal dementia, semantic dementia, and Alzheimer's disease. Alzheimer Dis Assoc Disord. 24(1):43-8, 2010](http://www.ncbi.nlm.nih.gov/pubmed/?term=19571735%5Bpmid%5D)
1. [Mackenzie IR et al: Nomenclature and nosology for neuropathologic subtypes of frontotemporal lobar degeneration: an update. Acta Neuropathol. 119(1):1-4, 2010](http://www.ncbi.nlm.nih.gov/pubmed/?term=19924424%5Bpmid%5D)
1. [King RD et al: Characterization of atrophic changes in the cerebral cortex using fractal dimensional analysis. Brain Imaging Behav. 3(2):154-66, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=20740072%5Bpmid%5D)
1. [Lindberg O et al: Cortical morphometric subclassification of frontotemporal lobar degeneration. AJNR Am J Neuroradiol. 30(6):1233-9, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=19346314%5Bpmid%5D)
1. [Moon WJ et al: Atrophy measurement of the anterior commissure and substantia innominata with 3T high-resolution MR imaging: does the measurement differ for patients with frontotemporal lobar degeneration and Alzheimer disease and for healthy subjects? AJNR Am J Neuroradiol. 29(7):1308-13, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18436612%5Bpmid%5D)
1. [Mihara M et al: Magnetic resonance spectroscopic study of Alzheimer's disease and frontotemporal dementia/Pick complex. Neuroreport. 17(4):413-6, 2006](http://www.ncbi.nlm.nih.gov/pubmed/?term=16514368%5Bpmid%5D)
1. [Whitwell JL et al: Magnetic resonance imaging signatures of tissue pathology in frontotemporal dementia. Arch Neurol. 62(9):1402-8, 2005](http://www.ncbi.nlm.nih.gov/pubmed/?term=16157747%5Bpmid%5D)
## Tables
# Imaging Features for Various Clinical Subtypes of Frontotemporal Dementia
| Clinical Subtypes | Imaging Features |
| --- | --- |
| bvFTD | MR: Atrophy of frontal & temporal lobes; asymmetric right frontal &/or temporal lobe atrophy may occur NM: Decreased perfusion & metabolism in frontal &/or temporal lobes, usually asymmetric to right side |
| sv-PPA | MR: Typically atrophy of left anterior & inferior temporal lobe; right temporal lobe atrophies as disease progresses NM: Decreased perfusion & metabolism in anterior temporal lobes (L > > R) |
| nfv-PPA | MR: Selective left perisylvian & frontal atrophy NM: Asymmetric decreased perfusion & metabolism in frontal lobes (L > > R) often involving insular cortex |
| lv-PPA | MR: Prominent atrophy of left angular & middle temporal gyri NM: Decreased perfusion & metabolism in left parietal inferior lobule & posterolateral temporal lobe |
## Images
### Selected Images
![Graphic depicts the classic disproportionate frontal lobe atrophy of late-stage frontotemporal dementia (FTD). The sulci are widened &amp; gyri are knife-like <img src='img/arrows/CS.png'/>. Parietooccipital lobes are spared. Gyri around the central sulcus are normal.](images/app.statdx.com_image_thumbnail_ccf3ab42-d18d-44f4-9b3f-cc9fd9510d11_size_168_quality_85_4a79edbc_20251014T204712Z.jpg)
*Graphic depicts the classic disproportionate frontal lobe atrophy of late-stage frontotemporal dementia (FTD). The sulci are widened &amp; gyri are knife-like <img src='img/arrows/CS.png'/>. Parietooccipital lobes are spared. Gyri around the central sulcus are normal.*
![Graphic depicts the classic disproportionate frontal lobe atrophy of late-stage frontotemporal dementia (FTD). The sulci are widened &amp; gyri are knife-like <img src='img/arrows/CS.png'/>. Parietooccipital lobes are spared. Gyri around the central sulcus are normal.](images/app.statdx.com_image_thumbnail_ccf3ab42-d18d-44f4-9b3f-cc9fd9510d11_size_168_quality_85_c56af8d8_20251014T193509Z.jpg)
*Graphic depicts the classic disproportionate frontal lobe atrophy of late-stage frontotemporal dementia (FTD). The sulci are widened &amp; gyri are knife-like <img src='img/arrows/CS.png'/>. Parietooccipital lobes are spared. Gyri around the central sulcus are normal.*
![Graphic depicts the classic disproportionate frontal lobe atrophy of late-stage frontotemporal dementia (FTD). The sulci are widened &amp; gyri are knife-like <img src='img/arrows/CS.png'/>. Parietooccipital lobes are spared. Gyri around the central sulcus are normal.](images/app.statdx.com_image_thumbnail_ccf3ab42-d18d-44f4-9b3f-cc9fd9510d11_size_174_quality_85_014f1181_20251014T190917Z.jpg)
*Graphic depicts the classic disproportionate frontal lobe atrophy of late-stage frontotemporal dementia (FTD). The sulci are widened &amp; gyri are knife-like <img src='img/arrows/CS.png'/>. Parietooccipital lobes are spared. Gyri around the central sulcus are normal.*
![Graphic depicts the classic disproportionate frontal lobe atrophy of late-stage frontotemporal dementia (FTD). The sulci are widened &amp; gyri are knife-like <img src='img/arrows/CS.png'/>. Parietooccipital lobes are spared. Gyri around the central sulcus are normal.](images/app.statdx.com_image_thumbnail_ccf3ab42-d18d-44f4-9b3f-cc9fd9510d11_size_174_quality_85_17e77d5c_20251014T185332Z.jpg)
*Graphic depicts the classic disproportionate frontal lobe atrophy of late-stage frontotemporal dementia (FTD). The sulci are widened &amp; gyri are knife-like <img src='img/arrows/CS.png'/>. Parietooccipital lobes are spared. Gyri around the central sulcus are normal.*
![Graphic depicts the classic disproportionate frontal lobe atrophy of late-stage frontotemporal dementia (FTD). The sulci are widened &amp; gyri are knife-like <img src='img/arrows/CS.png'/>. Parietooccipital lobes are spared. Gyri around the central sulcus are normal.](images/app.statdx.com_image_thumbnail_ccf3ab42-d18d-44f4-9b3f-cc9fd9510d11_size_174_quality_85_6b1ba6fe_20251014T193347Z.jpg)
*Graphic depicts the classic disproportionate frontal lobe atrophy of late-stage frontotemporal dementia (FTD). The sulci are widened &amp; gyri are knife-like <img src='img/arrows/CS.png'/>. Parietooccipital lobes are spared. Gyri around the central sulcus are normal.*
![Graphic depicts the classic disproportionate frontal lobe atrophy of late-stage frontotemporal dementia (FTD). The sulci are widened &amp; gyri are knife-like <img src='img/arrows/CS.png'/>. Parietooccipital lobes are spared. Gyri around the central sulcus are normal.](images/app.statdx.com_image_thumbnail_ccf3ab42-d18d-44f4-9b3f-cc9fd9510d11_size_174_quality_85_c31cd059_20251014T204456Z.jpg)
*Graphic depicts the classic disproportionate frontal lobe atrophy of late-stage frontotemporal dementia (FTD). The sulci are widened &amp; gyri are knife-like <img src='img/arrows/CS.png'/>. Parietooccipital lobes are spared. Gyri around the central sulcus are normal.*
![Coronal NECT MPR through the frontal lobes of a 62-year-old man with behavioral variant frontotemporal dementia (bvFTD) shows asymmetric atrophy of right frontal lobe <img src='img/arrows/CS.png'/> with relatively preserved left frontal lobe volume <img src='img/arrows/CO.png'/>.](images/app.statdx.com_image_thumbnail_8cab5d42-f1b2-44ba-ba3b-b0c17fdb3217_size_168_quality_85_547ec7bb_20251014T204712Z.jpg)
*Coronal NECT MPR through the frontal lobes of a 62-year-old man with behavioral variant frontotemporal dementia (bvFTD) shows asymmetric atrophy of right frontal lobe <img src='img/arrows/CS.png'/> with relatively preserved left frontal lobe volume <img src='img/arrows/CO.png'/>.*
![Coronal NECT MPR through the frontal lobes of a 62-year-old man with behavioral variant frontotemporal dementia (bvFTD) shows asymmetric atrophy of right frontal lobe <img src='img/arrows/CS.png'/> with relatively preserved left frontal lobe volume <img src='img/arrows/CO.png'/>.](images/app.statdx.com_image_thumbnail_8cab5d42-f1b2-44ba-ba3b-b0c17fdb3217_size_168_quality_85_b08c6cb5_20251014T193509Z.jpg)
*Coronal NECT MPR through the frontal lobes of a 62-year-old man with behavioral variant frontotemporal dementia (bvFTD) shows asymmetric atrophy of right frontal lobe <img src='img/arrows/CS.png'/> with relatively preserved left frontal lobe volume <img src='img/arrows/CO.png'/>.*
![Coronal T2WI MR of a 55-year-old woman with nfv-PPA shows diffuse brain parenchymal atrophy, which is more pronounced in the left perisylvian region <img src='img/arrows/CS.png'/> due to atrophy of adjacent inferior &amp; lateral temporal convexity <img src='img/arrows/CC.png'/> and frontal operculum <img src='img/arrows/CO.png'/>.](images/app.statdx.com_image_thumbnail_04291565-16df-407f-8345-c6415c6edd9a_size_168_quality_85_4aced880_20251014T204712Z.jpg)
*Coronal T2WI MR of a 55-year-old woman with nfv-PPA shows diffuse brain parenchymal atrophy, which is more pronounced in the left perisylvian region <img src='img/arrows/CS.png'/> due to atrophy of adjacent inferior &amp; lateral temporal convexity <img src='img/arrows/CC.png'/> and frontal operculum <img src='img/arrows/CO.png'/>.*
![Coronal T2WI MR of a 55-year-old woman with nfv-PPA shows diffuse brain parenchymal atrophy, which is more pronounced in the left perisylvian region <img src='img/arrows/CS.png'/> due to atrophy of adjacent inferior &amp; lateral temporal convexity <img src='img/arrows/CC.png'/> and frontal operculum <img src='img/arrows/CO.png'/>.](images/app.statdx.com_image_thumbnail_04291565-16df-407f-8345-c6415c6edd9a_size_168_quality_85_7f280f46_20251014T193509Z.jpg)
*Coronal T2WI MR of a 55-year-old woman with nfv-PPA shows diffuse brain parenchymal atrophy, which is more pronounced in the left perisylvian region <img src='img/arrows/CS.png'/> due to atrophy of adjacent inferior &amp; lateral temporal convexity <img src='img/arrows/CC.png'/> and frontal operculum <img src='img/arrows/CO.png'/>.*
![Parasagittal T1WI MR through left (top) &amp; right (bottom) temporal lobes of a 55-year-old man with sv-PPA shows asymmetric temporal lobe atrophy on left <img src='img/arrows/CS.png'/> vs. right <img src='img/arrows/CO.png'/>. Also note relatively preserved frontal lobe <img src='img/arrows/CC.png'/> volume.](images/app.statdx.com_image_thumbnail_17f86b8c-57b0-45c4-b31e-ba4a1a5c1a4a_size_168_quality_85_ba2729b3_20251014T204712Z.jpg)
*Parasagittal T1WI MR through left (top) &amp; right (bottom) temporal lobes of a 55-year-old man with sv-PPA shows asymmetric temporal lobe atrophy on left <img src='img/arrows/CS.png'/> vs. right <img src='img/arrows/CO.png'/>. Also note relatively preserved frontal lobe <img src='img/arrows/CC.png'/> volume.*
![Parasagittal T1WI MR through left (top) &amp; right (bottom) temporal lobes of a 55-year-old man with sv-PPA shows asymmetric temporal lobe atrophy on left <img src='img/arrows/CS.png'/> vs. right <img src='img/arrows/CO.png'/>. Also note relatively preserved frontal lobe <img src='img/arrows/CC.png'/> volume.](images/app.statdx.com_image_thumbnail_17f86b8c-57b0-45c4-b31e-ba4a1a5c1a4a_size_168_quality_85_c7dd5ad8_20251014T193509Z.jpg)
*Parasagittal T1WI MR through left (top) &amp; right (bottom) temporal lobes of a 55-year-old man with sv-PPA shows asymmetric temporal lobe atrophy on left <img src='img/arrows/CS.png'/> vs. right <img src='img/arrows/CO.png'/>. Also note relatively preserved frontal lobe <img src='img/arrows/CC.png'/> volume.*
![FDG PET raw data &amp; 3D-SSP images of a 59-year-old man with sv-PPA demonstrate decreased metabolic activity in the anterior &amp; medial left temporal lobe <img src='img/arrows/CO.png'/> &amp; to a lesser degree in anterior &amp; medial temporal lobe <img src='img/arrows/CS.png'/>. (Courtesy M. Matesan, MD.)](images/app.statdx.com_image_thumbnail_036a9532-9728-498e-b5d3-6cd07753ffaa_size_168_quality_85_3735e01d_20251014T193509Z.jpg)
*FDG PET raw data &amp; 3D-SSP images of a 59-year-old man with sv-PPA demonstrate decreased metabolic activity in the anterior &amp; medial left temporal lobe <img src='img/arrows/CO.png'/> &amp; to a lesser degree in anterior &amp; medial temporal lobe <img src='img/arrows/CS.png'/>. (Courtesy M. Matesan, MD.)*
![FDG PET raw data &amp; 3D-SSP images of a 59-year-old man with sv-PPA demonstrate decreased metabolic activity in the anterior &amp; medial left temporal lobe <img src='img/arrows/CO.png'/> &amp; to a lesser degree in anterior &amp; medial temporal lobe <img src='img/arrows/CS.png'/>. (Courtesy M. Matesan, MD.)](images/app.statdx.com_image_thumbnail_036a9532-9728-498e-b5d3-6cd07753ffaa_size_168_quality_85_afad773e_20251014T204712Z.jpg)
*FDG PET raw data &amp; 3D-SSP images of a 59-year-old man with sv-PPA demonstrate decreased metabolic activity in the anterior &amp; medial left temporal lobe <img src='img/arrows/CO.png'/> &amp; to a lesser degree in anterior &amp; medial temporal lobe <img src='img/arrows/CS.png'/>. (Courtesy M. Matesan, MD.)*
![Axial T2WI MR in a patient with classic FTD shows predominantly frontal lobar atrophy with knife-like gyri <img src='img/arrows/CO.png'/>. In this case, an associated region of hyperintense white matter <img src='img/arrows/CC.png'/> is present.](images/app.statdx.com_image_thumbnail_28e1e664-c1ec-4854-81d7-6ca5041e397f_size_168_quality_85_225e742c_20251014T193509Z.jpg)
*Axial T2WI MR in a patient with classic FTD shows predominantly frontal lobar atrophy with knife-like gyri <img src='img/arrows/CO.png'/>. In this case, an associated region of hyperintense white matter <img src='img/arrows/CC.png'/> is present.*
![Axial T2WI MR in a patient with classic FTD shows predominantly frontal lobar atrophy with knife-like gyri <img src='img/arrows/CO.png'/>. In this case, an associated region of hyperintense white matter <img src='img/arrows/CC.png'/> is present.](images/app.statdx.com_image_thumbnail_28e1e664-c1ec-4854-81d7-6ca5041e397f_size_168_quality_85_b0e3ee0c_20251014T204712Z.jpg)
*Axial T2WI MR in a patient with classic FTD shows predominantly frontal lobar atrophy with knife-like gyri <img src='img/arrows/CO.png'/>. In this case, an associated region of hyperintense white matter <img src='img/arrows/CC.png'/> is present.*
![Axial FDG PET of a 55-year-old woman with sv-PPA (clinical subtype of FTD) shows decreased metabolic activity in the left temporal lobe <img src='img/arrows/CO.png'/> &amp; to a lesser degree in the right temporal lobe <img src='img/arrows/CS.png'/>. Note relative sparing of parietal &amp; occipital lobes <img src='img/arrows/BS.png'/>.](images/app.statdx.com_image_thumbnail_c6cdd9e8-f0d6-4aca-831a-511ed76ddc55_size_168_quality_85_8fd2489d_20251014T204712Z.jpg)
*Axial FDG PET of a 55-year-old woman with sv-PPA (clinical subtype of FTD) shows decreased metabolic activity in the left temporal lobe <img src='img/arrows/CO.png'/> &amp; to a lesser degree in the right temporal lobe <img src='img/arrows/CS.png'/>. Note relative sparing of parietal &amp; occipital lobes <img src='img/arrows/BS.png'/>.*
![Axial FDG PET of a 55-year-old woman with sv-PPA (clinical subtype of FTD) shows decreased metabolic activity in the left temporal lobe <img src='img/arrows/CO.png'/> &amp; to a lesser degree in the right temporal lobe <img src='img/arrows/CS.png'/>. Note relative sparing of parietal &amp; occipital lobes <img src='img/arrows/BS.png'/>.](images/app.statdx.com_image_thumbnail_c6cdd9e8-f0d6-4aca-831a-511ed76ddc55_size_168_quality_85_ecd4a464_20251014T193509Z.jpg)
*Axial FDG PET of a 55-year-old woman with sv-PPA (clinical subtype of FTD) shows decreased metabolic activity in the left temporal lobe <img src='img/arrows/CO.png'/> &amp; to a lesser degree in the right temporal lobe <img src='img/arrows/CS.png'/>. Note relative sparing of parietal &amp; occipital lobes <img src='img/arrows/BS.png'/>.*
![Axial FLAIR MR in the same patient shows asymmetric atrophy of bilateral temporal lobes (left &gt; right) with knife-like gyri <img src='img/arrows/CC.png'/>. (Courtesy M. Matesan, MD.)](images/app.statdx.com_image_thumbnail_c4f82872-aa8c-49e4-8011-feae72168700_size_168_quality_85_a969cd70_20251014T204712Z.jpg)
*Axial FLAIR MR in the same patient shows asymmetric atrophy of bilateral temporal lobes (left &gt; right) with knife-like gyri <img src='img/arrows/CC.png'/>. (Courtesy M. Matesan, MD.)*
![Axial FLAIR MR in the same patient shows asymmetric atrophy of bilateral temporal lobes (left &gt; right) with knife-like gyri <img src='img/arrows/CC.png'/>. (Courtesy M. Matesan, MD.)](images/app.statdx.com_image_thumbnail_c4f82872-aa8c-49e4-8011-feae72168700_size_168_quality_85_b4a09e47_20251014T193509Z.jpg)
*Axial FLAIR MR in the same patient shows asymmetric atrophy of bilateral temporal lobes (left &gt; right) with knife-like gyri <img src='img/arrows/CC.png'/>. (Courtesy M. Matesan, MD.)*
![Axial color-coded FDG PET in a 65-year-old man with apathy &amp; slowly progressive behavioral changes with clinical diagnosis of bvFTD shows decreased metabolic activity in bilateral anterior &amp; medial temporal lobes <img src='img/arrows/CS.png'/> with relative sparing of bilateral parietooccipital lobes <img src='img/arrows/CC.png'/>.](images/app.statdx.com_image_thumbnail_8c9e626d-2963-4da6-aca4-48224a40c519_size_168_quality_85_307de5ce_20251014T204712Z.jpg)
*Axial color-coded FDG PET in a 65-year-old man with apathy &amp; slowly progressive behavioral changes with clinical diagnosis of bvFTD shows decreased metabolic activity in bilateral anterior &amp; medial temporal lobes <img src='img/arrows/CS.png'/> with relative sparing of bilateral parietooccipital lobes <img src='img/arrows/CC.png'/>.*
![Axial color-coded FDG PET in a 65-year-old man with apathy &amp; slowly progressive behavioral changes with clinical diagnosis of bvFTD shows decreased metabolic activity in bilateral anterior &amp; medial temporal lobes <img src='img/arrows/CS.png'/> with relative sparing of bilateral parietooccipital lobes <img src='img/arrows/CC.png'/>.](images/app.statdx.com_image_thumbnail_8c9e626d-2963-4da6-aca4-48224a40c519_size_168_quality_85_def2a9fd_20251014T193509Z.jpg)
*Axial color-coded FDG PET in a 65-year-old man with apathy &amp; slowly progressive behavioral changes with clinical diagnosis of bvFTD shows decreased metabolic activity in bilateral anterior &amp; medial temporal lobes <img src='img/arrows/CS.png'/> with relative sparing of bilateral parietooccipital lobes <img src='img/arrows/CC.png'/>.*
![Sagittal reformat of color-coded FDG PET in the same patient shows decreased metabolic activity in the frontal lobe <img src='img/arrows/CO.png'/>. (Courtesy J. Singh, MD.)](images/app.statdx.com_image_thumbnail_773c7326-90ae-4c2f-a70e-925d2f05462f_size_168_quality_85_231dc901_20251014T204712Z.jpg)
*Sagittal reformat of color-coded FDG PET in the same patient shows decreased metabolic activity in the frontal lobe <img src='img/arrows/CO.png'/>. (Courtesy J. Singh, MD.)*
![Sagittal reformat of color-coded FDG PET in the same patient shows decreased metabolic activity in the frontal lobe <img src='img/arrows/CO.png'/>. (Courtesy J. Singh, MD.)](images/app.statdx.com_image_thumbnail_773c7326-90ae-4c2f-a70e-925d2f05462f_size_168_quality_85_8e7667e5_20251014T193509Z.jpg)
*Sagittal reformat of color-coded FDG PET in the same patient shows decreased metabolic activity in the frontal lobe <img src='img/arrows/CO.png'/>. (Courtesy J. Singh, MD.)*
### Additional Images
![Sagittal T1WI MR demonstrates marked atrophy of the frontal lobe in a patient with FTD.](images/app.statdx.com_image_thumbnail_790df043-0719-4119-9280-781607f42bd2_size_168_quality_85_84862d5b_20251014T193509Z.jpg)
*Sagittal T1WI MR demonstrates marked atrophy of the frontal lobe in a patient with FTD.*
![Sagittal T1WI MR demonstrates marked atrophy of the frontal lobe in a patient with FTD.](images/app.statdx.com_image_thumbnail_790df043-0719-4119-9280-781607f42bd2_size_168_quality_85_c343f921_20251014T204712Z.jpg)
*Sagittal T1WI MR demonstrates marked atrophy of the frontal lobe in a patient with FTD.*
![Coronal T1WI MR in a patient with FTD shows prominent atrophy of both frontal lobes, which appears more pronounced on the left side.](images/app.statdx.com_image_thumbnail_3fb2009a-d209-4d22-85bf-afe0374052da_size_168_quality_85_383ea19f_20251014T204712Z.jpg)
*Coronal T1WI MR in a patient with FTD shows prominent atrophy of both frontal lobes, which appears more pronounced on the left side.*
![Coronal T1WI MR in a patient with FTD shows prominent atrophy of both frontal lobes, which appears more pronounced on the left side.](images/app.statdx.com_image_thumbnail_3fb2009a-d209-4d22-85bf-afe0374052da_size_168_quality_85_594e0c8d_20251014T193509Z.jpg)
*Coronal T1WI MR in a patient with FTD shows prominent atrophy of both frontal lobes, which appears more pronounced on the left side.*
![Axial NECT demonstrates predominantly frontal lobe volume loss from FTD.](images/app.statdx.com_image_thumbnail_e6d2623e-d594-4e81-a71b-61f69f70c6a7_size_168_quality_85_29d4c923_20251014T204712Z.jpg)
*Axial NECT demonstrates predominantly frontal lobe volume loss from FTD.*
![Axial NECT demonstrates predominantly frontal lobe volume loss from FTD.](images/app.statdx.com_image_thumbnail_e6d2623e-d594-4e81-a71b-61f69f70c6a7_size_168_quality_85_782a6aa1_20251014T193509Z.jpg)
*Axial NECT demonstrates predominantly frontal lobe volume loss from FTD.*
![Axial T2WI MR in a patient with FTD shows marked frontal lobe atrophy.](images/app.statdx.com_image_thumbnail_fbbfa5c7-e203-41ff-bbb5-5af9f7d2600a_size_168_quality_85_d0ead675_20251014T193509Z.jpg)
*Axial T2WI MR in a patient with FTD shows marked frontal lobe atrophy.*
![Axial T2WI MR in a patient with FTD shows marked frontal lobe atrophy.](images/app.statdx.com_image_thumbnail_fbbfa5c7-e203-41ff-bbb5-5af9f7d2600a_size_168_quality_85_e967f940_20251014T204712Z.jpg)
*Axial T2WI MR in a patient with FTD shows marked frontal lobe atrophy.*
![Axial FLAIR MR shows frontal lobe volume loss as well as associated hyperintense signal in white matter.](images/app.statdx.com_image_thumbnail_59d9cb53-05a2-4d68-9d1a-5fd0d42e8c82_size_168_quality_85_b9bbfcd3_20251014T193509Z.jpg)
*Axial FLAIR MR shows frontal lobe volume loss as well as associated hyperintense signal in white matter.*
![Axial FLAIR MR shows frontal lobe volume loss as well as associated hyperintense signal in white matter.](images/app.statdx.com_image_thumbnail_59d9cb53-05a2-4d68-9d1a-5fd0d42e8c82_size_168_quality_85_eeeb42c9_20251014T204712Z.jpg)
*Axial FLAIR MR shows frontal lobe volume loss as well as associated hyperintense signal in white matter.*
![Axial T2WI MR in a patient with FTD demonstrates marked volume loss as well as associated hyperintense signal within bilateral temporal lobes.](images/app.statdx.com_image_thumbnail_56421700-7b64-4578-acbb-c469f4197fc5_size_168_quality_85_a3e428df_20251014T204712Z.jpg)
*Axial T2WI MR in a patient with FTD demonstrates marked volume loss as well as associated hyperintense signal within bilateral temporal lobes.*
![Axial T2WI MR in a patient with FTD demonstrates marked volume loss as well as associated hyperintense signal within bilateral temporal lobes.](images/app.statdx.com_image_thumbnail_56421700-7b64-4578-acbb-c469f4197fc5_size_168_quality_85_f18bf899_20251014T193509Z.jpg)
*Axial T2WI MR in a patient with FTD demonstrates marked volume loss as well as associated hyperintense signal within bilateral temporal lobes.*
![FDG PET in a patient with FTD &amp; dementia depicts glucose hypometabolism (green regions in the cortex) in the frontal lobes. (Courtesy N. Foster, MD &amp; the University of Michigan PET Center.)](images/app.statdx.com_image_thumbnail_e35070b5-6df7-4e12-9df6-071c7dd71fef_size_168_quality_85_02c3ea13_20251014T193509Z.jpg)
*FDG PET in a patient with FTD &amp; dementia depicts glucose hypometabolism (green regions in the cortex) in the frontal lobes. (Courtesy N. Foster, MD &amp; the University of Michigan PET Center.)*
![FDG PET in a patient with FTD &amp; dementia depicts glucose hypometabolism (green regions in the cortex) in the frontal lobes. (Courtesy N. Foster, MD &amp; the University of Michigan PET Center.)](images/app.statdx.com_image_thumbnail_e35070b5-6df7-4e12-9df6-071c7dd71fef_size_168_quality_85_88e63129_20251014T204712Z.jpg)
*FDG PET in a patient with FTD &amp; dementia depicts glucose hypometabolism (green regions in the cortex) in the frontal lobes. (Courtesy N. Foster, MD &amp; the University of Michigan PET Center.)*
![FDG PET in the same patient shows a decreased rate of glucose metabolism in the frontal &amp; temporal lobes, consistent with FTD. (Courtesy N. Foster, MD &amp; the University of Michigan PET Center.)](images/app.statdx.com_image_thumbnail_caf2cccf-43ff-4ffd-b7c9-a29edebdfdcf_size_168_quality_85_6733760b_20251014T193509Z.jpg)
*FDG PET in the same patient shows a decreased rate of glucose metabolism in the frontal &amp; temporal lobes, consistent with FTD. (Courtesy N. Foster, MD &amp; the University of Michigan PET Center.)*
![FDG PET in the same patient shows a decreased rate of glucose metabolism in the frontal &amp; temporal lobes, consistent with FTD. (Courtesy N. Foster, MD &amp; the University of Michigan PET Center.)](images/app.statdx.com_image_thumbnail_caf2cccf-43ff-4ffd-b7c9-a29edebdfdcf_size_168_quality_85_f8b4423b_20251014T204712Z.jpg)
*FDG PET in the same patient shows a decreased rate of glucose metabolism in the frontal &amp; temporal lobes, consistent with FTD. (Courtesy N. Foster, MD &amp; the University of Michigan PET Center.)*
![Axial T2WI MR in a 57-year-old woman with probable FTD shows temporal lobe atrophy with knife-like gyri <img src='img/arrows/WS.png'/>. The parietal &amp; occipital lobes are relatively well preserved.](images/app.statdx.com_image_thumbnail_4f435ef8-d0f0-4cbc-9a09-5b92318e8482_size_168_quality_85_2f2854c4_20251014T204712Z.jpg)
*Axial T2WI MR in a 57-year-old woman with probable FTD shows temporal lobe atrophy with knife-like gyri <img src='img/arrows/WS.png'/>. The parietal &amp; occipital lobes are relatively well preserved.*
![Axial T2WI MR in a 57-year-old woman with probable FTD shows temporal lobe atrophy with knife-like gyri <img src='img/arrows/WS.png'/>. The parietal &amp; occipital lobes are relatively well preserved.](images/app.statdx.com_image_thumbnail_4f435ef8-d0f0-4cbc-9a09-5b92318e8482_size_168_quality_85_84c3c6a3_20251014T193509Z.jpg)
*Axial T2WI MR in a 57-year-old woman with probable FTD shows temporal lobe atrophy with knife-like gyri <img src='img/arrows/WS.png'/>. The parietal &amp; occipital lobes are relatively well preserved.*
![FDG PET in a patient with FTD shows marked hypometabolism in the frontal <img src='img/arrows/WS.png'/> &amp; temporal <img src='img/arrows/WC.png'/> lobes. There is relative preservation of glucose metabolism in the occipital &amp; parietal lobes <img src='img/arrows/WO.png'/>.](images/app.statdx.com_image_thumbnail_96ca8a9c-4048-495b-8d92-22a54ed7e9de_size_168_quality_85_ae34a1a3_20251014T193509Z.jpg)
*FDG PET in a patient with FTD shows marked hypometabolism in the frontal <img src='img/arrows/WS.png'/> &amp; temporal <img src='img/arrows/WC.png'/> lobes. There is relative preservation of glucose metabolism in the occipital &amp; parietal lobes <img src='img/arrows/WO.png'/>.*
![FDG PET in a patient with FTD shows marked hypometabolism in the frontal <img src='img/arrows/WS.png'/> &amp; temporal <img src='img/arrows/WC.png'/> lobes. There is relative preservation of glucose metabolism in the occipital &amp; parietal lobes <img src='img/arrows/WO.png'/>.](images/app.statdx.com_image_thumbnail_96ca8a9c-4048-495b-8d92-22a54ed7e9de_size_168_quality_85_f979b064_20251014T204712Z.jpg)
*FDG PET in a patient with FTD shows marked hypometabolism in the frontal <img src='img/arrows/WS.png'/> &amp; temporal <img src='img/arrows/WC.png'/> lobes. There is relative preservation of glucose metabolism in the occipital &amp; parietal lobes <img src='img/arrows/WO.png'/>.*
![Sagittal SSP FDG PET scan in a 72-year-old man with FTD shows a normal elderly control map (2nd row), the patient's glucose metabolism (3rd row), &amp; Z-score statistical map (bottom row). The frontal lobes <img src='img/arrows/WS.png'/> are strikingly hypometabolic. The temporal lobes <img src='img/arrows/WO.png'/> are somewhat less severely affected.](images/app.statdx.com_image_thumbnail_e85cb1b8-3306-4485-8ed1-7418b2b5adec_size_168_quality_85_08efde5a_20251014T193509Z.jpg)
*Sagittal SSP FDG PET scan in a 72-year-old man with FTD shows a normal elderly control map (2nd row), the patient's glucose metabolism (3rd row), &amp; Z-score statistical map (bottom row). The frontal lobes <img src='img/arrows/WS.png'/> are strikingly hypometabolic. The temporal lobes <img src='img/arrows/WO.png'/> are somewhat less severely affected.*
![Sagittal SSP FDG PET scan in a 72-year-old man with FTD shows a normal elderly control map (2nd row), the patient's glucose metabolism (3rd row), &amp; Z-score statistical map (bottom row). The frontal lobes <img src='img/arrows/WS.png'/> are strikingly hypometabolic. The temporal lobes <img src='img/arrows/WO.png'/> are somewhat less severely affected.](images/app.statdx.com_image_thumbnail_e85cb1b8-3306-4485-8ed1-7418b2b5adec_size_168_quality_85_3726377b_20251014T204712Z.jpg)
*Sagittal SSP FDG PET scan in a 72-year-old man with FTD shows a normal elderly control map (2nd row), the patient's glucose metabolism (3rd row), &amp; Z-score statistical map (bottom row). The frontal lobes <img src='img/arrows/WS.png'/> are strikingly hypometabolic. The temporal lobes <img src='img/arrows/WO.png'/> are somewhat less severely affected.*
![Coronal T1WI MR in the same patient shows focal atrophy of the olfactory gyri <img src='img/arrows/WO.png'/>. This finding was initially overlooked &amp; the patient was given the imaging diagnosis of Alzheimer disease. Review &amp; subsequent clinical evaluation confirmed FTD.](images/app.statdx.com_image_thumbnail_2add422d-e710-47d4-86c1-8949ca5b3b22_size_168_quality_85_7c24ef97_20251014T204712Z.jpg)
*Coronal T1WI MR in the same patient shows focal atrophy of the olfactory gyri <img src='img/arrows/WO.png'/>. This finding was initially overlooked &amp; the patient was given the imaging diagnosis of Alzheimer disease. Review &amp; subsequent clinical evaluation confirmed FTD.*
![Coronal T1WI MR in the same patient shows focal atrophy of the olfactory gyri <img src='img/arrows/WO.png'/>. This finding was initially overlooked &amp; the patient was given the imaging diagnosis of Alzheimer disease. Review &amp; subsequent clinical evaluation confirmed FTD.](images/app.statdx.com_image_thumbnail_2add422d-e710-47d4-86c1-8949ca5b3b22_size_168_quality_85_daee6eb1_20251014T193509Z.jpg)
*Coronal T1WI MR in the same patient shows focal atrophy of the olfactory gyri <img src='img/arrows/WO.png'/>. This finding was initially overlooked &amp; the patient was given the imaging diagnosis of Alzheimer disease. Review &amp; subsequent clinical evaluation confirmed FTD.*
![Axial FDG PET in a patient with FTD shows decreased glucose metabolism in frontal <img src='img/arrows/WS.png'/> &amp; temporal <img src='img/arrows/WC.png'/> lobes. The parietal &amp; occipital lobes <img src='img/arrows/WO.png'/> are spared.](images/app.statdx.com_image_thumbnail_2d335b53-39a7-48da-adec-02860af3221c_size_168_quality_85_98eb7ff8_20251014T204712Z.jpg)
*Axial FDG PET in a patient with FTD shows decreased glucose metabolism in frontal <img src='img/arrows/WS.png'/> &amp; temporal <img src='img/arrows/WC.png'/> lobes. The parietal &amp; occipital lobes <img src='img/arrows/WO.png'/> are spared.*
![Axial FDG PET in a patient with FTD shows decreased glucose metabolism in frontal <img src='img/arrows/WS.png'/> &amp; temporal <img src='img/arrows/WC.png'/> lobes. The parietal &amp; occipital lobes <img src='img/arrows/WO.png'/> are spared.](images/app.statdx.com_image_thumbnail_2d335b53-39a7-48da-adec-02860af3221c_size_168_quality_85_f405d3d1_20251014T193509Z.jpg)
*Axial FDG PET in a patient with FTD shows decreased glucose metabolism in frontal <img src='img/arrows/WS.png'/> &amp; temporal <img src='img/arrows/WC.png'/> lobes. The parietal &amp; occipital lobes <img src='img/arrows/WO.png'/> are spared.*
![Sagittal FDG PET in the same patient with FTD depicts glucose hypometabolism in the frontal lobes <img src='img/arrows/WS.png'/>. The occipital lobes <img src='img/arrows/WO.png'/> are normal.](images/app.statdx.com_image_thumbnail_92769fca-fdfe-41cf-b1dc-9fb4b42588c2_size_168_quality_85_4b601536_20251014T193509Z.jpg)
*Sagittal FDG PET in the same patient with FTD depicts glucose hypometabolism in the frontal lobes <img src='img/arrows/WS.png'/>. The occipital lobes <img src='img/arrows/WO.png'/> are normal.*
![Sagittal FDG PET in the same patient with FTD depicts glucose hypometabolism in the frontal lobes <img src='img/arrows/WS.png'/>. The occipital lobes <img src='img/arrows/WO.png'/> are normal.](images/app.statdx.com_image_thumbnail_92769fca-fdfe-41cf-b1dc-9fb4b42588c2_size_168_quality_85_5921ae54_20251014T204712Z.jpg)
*Sagittal FDG PET in the same patient with FTD depicts glucose hypometabolism in the frontal lobes <img src='img/arrows/WS.png'/>. The occipital lobes <img src='img/arrows/WO.png'/> are normal.*
![Coronal FDG PET in a patient with FTD exhibits glucose hypometabolism in frontal <img src='img/arrows/WC.png'/> and temporal lobes <img src='img/arrows/WS.png'/>.](images/app.statdx.com_image_thumbnail_d6302d0c-7770-4398-b0b3-5fe7b86cd973_size_168_quality_85_519e43c5_20251014T193509Z.jpg)
*Coronal FDG PET in a patient with FTD exhibits glucose hypometabolism in frontal <img src='img/arrows/WC.png'/> and temporal lobes <img src='img/arrows/WS.png'/>.*
![Coronal FDG PET in a patient with FTD exhibits glucose hypometabolism in frontal <img src='img/arrows/WC.png'/> and temporal lobes <img src='img/arrows/WS.png'/>.](images/app.statdx.com_image_thumbnail_d6302d0c-7770-4398-b0b3-5fe7b86cd973_size_168_quality_85_5422ff62_20251014T204712Z.jpg)
*Coronal FDG PET in a patient with FTD exhibits glucose hypometabolism in frontal <img src='img/arrows/WC.png'/> and temporal lobes <img src='img/arrows/WS.png'/>.*
![Axial T2WI MR in a patient with semantic dementia subtype of FTLD shows marked atrophy of anterolateral temporal lobes <img src='img/arrows/WO.png'/>. Note the relative preservation of the hippocampi <img src='img/arrows/WS.png'/>.](images/app.statdx.com_image_thumbnail_e91a21ee-283e-4308-8549-88d99ff9ab25_size_168_quality_85_a913ddc2_20251014T193509Z.jpg)
*Axial T2WI MR in a patient with semantic dementia subtype of FTLD shows marked atrophy of anterolateral temporal lobes <img src='img/arrows/WO.png'/>. Note the relative preservation of the hippocampi <img src='img/arrows/WS.png'/>.*
![Axial T2WI MR in a patient with semantic dementia subtype of FTLD shows marked atrophy of anterolateral temporal lobes <img src='img/arrows/WO.png'/>. Note the relative preservation of the hippocampi <img src='img/arrows/WS.png'/>.](images/app.statdx.com_image_thumbnail_e91a21ee-283e-4308-8549-88d99ff9ab25_size_168_quality_85_f274ae6c_20251014T204712Z.jpg)
*Axial T2WI MR in a patient with semantic dementia subtype of FTLD shows marked atrophy of anterolateral temporal lobes <img src='img/arrows/WO.png'/>. Note the relative preservation of the hippocampi <img src='img/arrows/WS.png'/>.*
![Axial NECT in a 78-year-old patient diagnosed with late-stage FTD through the upper lateral ventricles shows striking frontal lobar atrophy with classic knife-like gyri <img src='img/arrows/WO.png'/> characteristic of FTD. In contrast, the parietal &amp; occipital lobes appear relatively spared.](images/app.statdx.com_image_thumbnail_f28013b4-4745-4f7c-b83a-e21cba7113b1_size_168_quality_85_2f660baa_20251014T193509Z.jpg)
*Axial NECT in a 78-year-old patient diagnosed with late-stage FTD through the upper lateral ventricles shows striking frontal lobar atrophy with classic knife-like gyri <img src='img/arrows/WO.png'/> characteristic of FTD. In contrast, the parietal &amp; occipital lobes appear relatively spared.*
![Axial NECT in a 78-year-old patient diagnosed with late-stage FTD through the upper lateral ventricles shows striking frontal lobar atrophy with classic knife-like gyri <img src='img/arrows/WO.png'/> characteristic of FTD. In contrast, the parietal &amp; occipital lobes appear relatively spared.](images/app.statdx.com_image_thumbnail_f28013b4-4745-4f7c-b83a-e21cba7113b1_size_168_quality_85_f550c809_20251014T204712Z.jpg)
*Axial NECT in a 78-year-old patient diagnosed with late-stage FTD through the upper lateral ventricles shows striking frontal lobar atrophy with classic knife-like gyri <img src='img/arrows/WO.png'/> characteristic of FTD. In contrast, the parietal &amp; occipital lobes appear relatively spared.*
![Axial FLAIR MR in a patient with early findings of FTD shows relatively mild frontal lobe volume loss <img src='img/arrows/WS.png'/> compared to the normal appearance of the parietal lobes.](images/app.statdx.com_image_thumbnail_77b51dfc-697e-42d6-bc39-0ab8e4d59ac6_size_168_quality_85_0d82e90c_20251014T204712Z.jpg)
*Axial FLAIR MR in a patient with early findings of FTD shows relatively mild frontal lobe volume loss <img src='img/arrows/WS.png'/> compared to the normal appearance of the parietal lobes.*
![Axial FLAIR MR in a patient with early findings of FTD shows relatively mild frontal lobe volume loss <img src='img/arrows/WS.png'/> compared to the normal appearance of the parietal lobes.](images/app.statdx.com_image_thumbnail_77b51dfc-697e-42d6-bc39-0ab8e4d59ac6_size_168_quality_85_fb7967b8_20251014T193509Z.jpg)
*Axial FLAIR MR in a patient with early findings of FTD shows relatively mild frontal lobe volume loss <img src='img/arrows/WS.png'/> compared to the normal appearance of the parietal lobes.*
![Axial FLAIR MR shows atrophy of both the frontal &amp; temporal lobes. Note compensatory enlargement of the temporal horns <img src='img/arrows/WS.png'/> due to temporal lobe volume loss.](images/app.statdx.com_image_thumbnail_606a11b2-5289-4bd0-86df-e4ca45437d69_size_168_quality_85_a3752e9f_20251014T204716Z.jpg)
*Axial FLAIR MR shows atrophy of both the frontal &amp; temporal lobes. Note compensatory enlargement of the temporal horns <img src='img/arrows/WS.png'/> due to temporal lobe volume loss.*
![Sagittal T1WI MR shows predominantly frontal &amp; temporal lobe atrophy. Some gyri demonstrate a classic knife-like appearance (<img src='img/arrows/WS.png'/>) reflecting severe atrophy of a gyrus.](images/app.statdx.com_image_thumbnail_a11d3c67-3524-4a6a-87cf-6c6b946c6d0f_size_168_quality_85_632a5cb8_20251014T204716Z.jpg)
*Sagittal T1WI MR shows predominantly frontal &amp; temporal lobe atrophy. Some gyri demonstrate a classic knife-like appearance (<img src='img/arrows/WS.png'/>) reflecting severe atrophy of a gyrus.*
docs_md/articles/granular-cell-tumor_da976b04-85a3-4bf8-ac81-e579f081293e.md
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---
title: "Granular Cell Tumor"
docid: "da976b04-85a3-4bf8-ac81-e579f081293e"
authors:
- key: "8d5254e9-8dda-478b-8f08-bdee97a32c79"
value: "Karen L. Salzman, MD, FACR"
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name: "Brain"
slug: "brain"
treeNodeId: "6d8829f1-14d7-45af-8675-255189aa526a"
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slug: "diagnosis"
treeNodeId: "51c00394-446e-4a38-94af-d3b1d14d34e8"
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name: "Anatomy-Based Diagnoses"
slug: "anatomy-based-diagnoses"
treeNodeId: "529d3e33-f508-498c-bc70-cf962e81e629"
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name: "Sella and Pituitary"
slug: "sella-and-pituitary"
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slug: "neoplasms"
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name: "Granular Cell Tumor"
slug: "granular-cell-tumor"
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category: "Brain"
documentVersionId: "16196366-3704-4511-b91f-73ac70ac21c1"
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lastUpdated: "08/05/20"
pageDescription: "Granular Cell Tumor"
pageKeywords: "Brain, Diagnosis, Anatomy-Based Diagnoses, Sella and Pituitary, Neoplasms, Granular Cell Tumor"
pageTitle: "Granular Cell Tumor | STATdx"
enhancedTitle: "Granular Cell Tumor"
type: "DX"
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breadcrumbs:
- "Brain"
- "Diagnosis"
- "Anatomy-Based Diagnoses"
- "Sella and Pituitary"
- "Neoplasms"
- "Granular Cell Tumor"
---
# KEY FACTS
- ## Terminology
- Neoplasms that arise from pituicytes, specialized glial cells of neurohypophysis or infundibulum
- Rare low-grade, nonendocrine neoplasms of sellar region
- Formerly called pituicytoma; granular cell tumor of neurohypophysis
- Part of 2017 WHO spectrum of thyroid transcription factor 1 (TTF-1) expressing pituitary tumors of posterior lobe
- ## Imaging
- Enhancing, well-circumscribed sellar/suprasellar or infundibular mass
- 1.5-6.0 cm
- CT: Sellar/suprasellar mass with hyperattenuation
- Rarely calcification may be present
- Best imaging tool: C+ MR with high-resolution imaging through sellar region
- Consider granular cell tumor if sellar/suprasellar mass appears separate from anterior pituitary gland
- ## Top Differential Diagnoses
- Pituitary macroadenoma
- Lymphocytic hypophysitis
- Pituicytoma
- Spindle cell oncocytoma
- Rathke cleft cyst
- ## Pathology
- WHO grade 1
- ## Clinical Issues
- Commonly asymptomatic (small lesions)
- Visual field deficit related to optic chiasm compression is most common presenting feature
- Less common symptoms: Panhypopituitarism, galactorrhea, amenorrhea, decreased libido, neuropsychological changes
- Typically present in adulthood, 5th-6th decades
- Generally benign clinical course
# TERMINOLOGY
- ## Abbreviations
- Granular cell tumor (GCT)
- ## Synonyms
- Formerly called pituicytoma; granular cell tumor of neurohypophysis
- ## Definitions
- Neoplasms that arise from pituicytes, specialized glia of neurohypophysis or infundibulum
- Rare low-grade, nonendocrine neoplasms of sellar region
- Part of 2017 WHO spectrum of thyroid transcription factor1 (TTF-1) expressing pituitary tumors of posterior lobe
# IMAGING
- ## General Features
- ### Best diagnostic clue
- Enhancing, well-circumscribed sellar/suprasellar or infundibular mass
- ### Location
- Sellar and suprasellar or infundibular mass
- ### Size
- 1.5-6.0 cm
- ### Morphology
- Lobulated and well circumscribed
- ## CT Findings
- ### NECT
- Sellar/suprasellar mass with hyperattenuation
- Rarely calcification may be present
- ## MR Findings
- ### T1WI
- Sellar/suprasellar mass isointense to gray matter
- ### T1WI C+
- Enhancement may be homogeneous or heterogeneous
- ## Imaging Recommendations
- ### Best imaging tool
- C+ MR with high-resolution imaging through sellar region
# DIFFERENTIAL DIAGNOSIS
- [Pituitary Macroadenoma](/document/pituitary-microadenoma/283f3068-d369-4f79-bf01-0f2b82c6e49b)
- Sellar and suprasellar enhancing mass
- Arises from adenohypophysis
- May be indistinguishable
- [Lymphocytic Hypophysitis](/document/lymphocytic-hypophysitis/f30774c3-cbd0-4ab3-b3d1-e0574106db1f)
- May be indistinguishable from macroadenoma
- May present as infundibular mass
- Typically pregnant or postpartum females
- [Pituicytoma](/document/pituicytoma/d6e481d5-7742-4354-8dfe-4f04286a709a)
- May be intrasellar or suprasellar mass
- May be separate from adenohypophysis
- [Spindle Cell Oncocytoma](/document/spindle-cell-oncocytoma/1557bfb2-8315-4782-9a1e-4e70d6d20c4e)
- Imaging mimics macroadenoma
- Enhancing sellar and suprasellar mass
- [Rathke Cleft Cyst](/document/rathke-cleft-cyst/8f1561f7-92a7-485c-a0ae-2e2d5c8c1628)
- Nonenhancing cystic sellar &/or suprasellar lesion
- Intracystic nodule in up to 75%
# PATHOLOGY
- ## General Features
- ### Associated abnormalities
- Granular cell tumors have been found in associated with adenomas
- Small granular cell clusters have been found in up to 17% of autopsy series
- ## Staging, Grading, & Classification
- WHO grade 1
- ## Gross Pathologic & Surgical Features
- Lobulated, well-circumscribed mass, soft but rubbery
- More firm than pituitary adenoma
- ## Microscopic Features
- Densely packed polygonal cells with abundant granular eosinophilic cytoplasm
- Electron microscopy: Cytoplasm is filled with phagolysosomes containing electron-dense material and membranous debris
# CLINICAL ISSUES
- ## Presentation
- ### Most common signs/symptoms
- Commonly asymptomatic (small lesions)
- Visual field deficit related to optic chiasm compression
- ### Other signs/symptoms
- Panhypopituitarism, galactorrhea, amenorrhea, decreased libido, neuropsychological changes
- Rarely diabetes insipidus
- ## Demographics
- ### Age
- Typically present in adulthood, 5th-6th decades
- ### Gender
- F:M = 2:1
- ## Natural History & Prognosis
- Rare (~ 150 reported cases)
- Generally benign clinical course
- ## Treatment
- Surgical resection
# DIAGNOSTIC CHECKLIST
- ## Image Interpretation Pearls
- Consider granular cell tumor if sellar/suprasellar mass appears separate from anterior pituitary gland
55b6bf2f-c975-435a-972b-a42c5b4cbe6d
## References
# Selected References
1. [Borg A et al: Tumors of the neurohypophysis: One unit's experience and literature review. World Neurosurg. 134:e968-e978, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=31734425%5Bpmid%5D)
1. [Guerrero-Pérez F et al: Posterior pituitary tumours: the spectrum of a unique entity. A clinical and histological study of a large case series. Endocrine. 63(1):36-43, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=30276594%5Bpmid%5D)
1. [Guerrero-Pérez F et al: Posterior pituitary tumours: the spectrum of a unique entity. A clinical and histological study of a large case series. Endocrine. 63(1):36-43, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=30276594%5Bpmid%5D)
1. [Shibuya M: Welcoming the new WHO classification of pituitary tumors 2017: revolution in TTF-1-positive posterior pituitary tumors. Brain Tumor Pathol. 35(2):62-70, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=29500747%5Bpmid%5D)
1. [Ahmed AK et al: Extent of surgical resection and tumor size predicts prognosis in granular cell tumor of the sellar region. Acta Neurochir (Wien). 159(11):2209-16, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=28948361%5Bpmid%5D)
1. [Jian F et al: Surgical biopsies in patients with central diabetes insipidus and thickened pituitary stalks. Endocrine. 47(1):325-35, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=24532100%5Bpmid%5D)
1. [Shizukuishi T et al: Granular cell tumor of the neurohypophysis with optic tract edema. Jpn J Radiol. 32(3):179-82, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=24414885%5Bpmid%5D)
1. [Mete O et al: Spindle cell oncocytomas and granular cell tumors of the pituitary are variants of pituicytoma. Am J Surg Pathol. 37(11):1694-9, 2013](http://www.ncbi.nlm.nih.gov/pubmed/?term=23887161%5Bpmid%5D)
1. [Saiegh L et al: Granular cell tumor of the neurohypophysis: case report and review of the literature. Neuro Endocrinol Lett. 34(5):331-8, 2013](http://www.ncbi.nlm.nih.gov/pubmed/?term=23922042%5Bpmid%5D)
1. [Covington MF et al: Pituicytoma, spindle cell oncocytoma, and granular cell tumor: clarification and meta-analysis of the world literature since 1893. AJNR Am J Neuroradiol. 32(11):2067-72, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=21960498%5Bpmid%5D)
1. [Mumert ML et al: Cystic granular cell tumor mimicking Rathke cleft cyst. J Neurosurg. 114(2):325-8, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=20509726%5Bpmid%5D)
1. [Menon G et al: Symptomatic granular cell tumour of the pituitary. Br J Neurosurg. 22(1):126-30, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=17952719%5Bpmid%5D)
1. Fuller GN et el: Granular cell tumour of the neurohypophysis In Louis, DN et el: WHO Classification of Tumors of the Central Nervous System. 4th Ed. Lyon: IARC. 241-2, 2007
## Images
### Selected Images
![Sagittal T1 C+ MR in a 65-year-old woman shows an enhancing suprasellar mass <img src='img/arrows/CO.png'/>.](images/app.statdx.com_image_thumbnail_5ca8b84a-b148-4d75-a721-86afb09900bf_size_168_quality_85_6152a149_20251014T205304Z.jpg)
*Sagittal T1 C+ MR in a 65-year-old woman shows an enhancing suprasellar mass <img src='img/arrows/CO.png'/>.*
![Sagittal T1 C+ MR in a 65-year-old woman shows an enhancing suprasellar mass <img src='img/arrows/CO.png'/>.](images/app.statdx.com_image_thumbnail_5ca8b84a-b148-4d75-a721-86afb09900bf_size_174_quality_85_93e878e8.jpg)
*Sagittal T1 C+ MR in a 65-year-old woman shows an enhancing suprasellar mass <img src='img/arrows/CO.png'/>.*
![Coronal T1 C+ MR in the same patient shows the suprasellar enhancing mass <img src='img/arrows/CO.png'/> contacting the optic chiasm <img src='img/arrows/WS.png'/>. Imaging mimics the much more common pituitary macroadenoma. Granular cell tumors are rare low-grade, nonendocrine neoplasms arising from the infundibulum or neurohypophysis. These tumors are part of the 2017 WHO spectrum of TTF-1 expressing pituitary tumors of the posterior lobe.](images/app.statdx.com_image_thumbnail_37b27bcd-5ca6-4d76-b72f-601736dcfe13_size_168_quality_85_89773be0_20251014T205304Z.jpg)
*Coronal T1 C+ MR in the same patient shows the suprasellar enhancing mass <img src='img/arrows/CO.png'/> contacting the optic chiasm <img src='img/arrows/WS.png'/>. Imaging mimics the much more common pituitary macroadenoma. Granular cell tumors are rare low-grade, nonendocrine neoplasms arising from the infundibulum or neurohypophysis. These tumors are part of the 2017 WHO spectrum of TTF-1 expressing pituitary tumors of the posterior lobe.*
![Sagittal T1 MR in a 31-year-old woman with headache and visual complaints shows a sellar and suprasellar mass <img src='img/arrows/WO.png'/> with superior displacement of the optic chiasm <img src='img/arrows/WS.png'/>. There is a hyperintense focus <img src='img/arrows/CC.png'/> along the inferior aspect of the mass.](images/app.statdx.com_image_thumbnail_e25dc2dc-35c8-4cde-85d2-2a4a82a86aae_size_168_quality_85_bd87c0e3_20251014T205304Z.jpg)
*Sagittal T1 MR in a 31-year-old woman with headache and visual complaints shows a sellar and suprasellar mass <img src='img/arrows/WO.png'/> with superior displacement of the optic chiasm <img src='img/arrows/WS.png'/>. There is a hyperintense focus <img src='img/arrows/CC.png'/> along the inferior aspect of the mass.*
![Sagittal T1 C+ MR in the same patient shows peripheral enhancement <img src='img/arrows/WO.png'/> of the mass. The region of T1 hyperintensity is also seen, resembling an intracystic nodule <img src='img/arrows/CC.png'/>, typically seen in a Rathke cleft cyst. Granular cell tumor of the neurohypophysis was diagnosed at resection.](images/app.statdx.com_image_thumbnail_a887a4ff-115c-4cf2-8604-74d6473eba9a_size_168_quality_85_1a5a4e25_20251014T205304Z.jpg)
*Sagittal T1 C+ MR in the same patient shows peripheral enhancement <img src='img/arrows/WO.png'/> of the mass. The region of T1 hyperintensity is also seen, resembling an intracystic nodule <img src='img/arrows/CC.png'/>, typically seen in a Rathke cleft cyst. Granular cell tumor of the neurohypophysis was diagnosed at resection.*
### Additional Images
![Sagittal T1 C+ MR shows an enhancing mass along the superior infundibulum and anterior 3rd ventricle <img src='img/arrows/CO.png'/>. Granular cell tumor was diagnosed at resection.](images/app.statdx.com_image_thumbnail_0fb060d9-7fd7-4e32-befc-534cb8d7d572_size_168_quality_85_c1d2f18a_20251014T205304Z.jpg)
*Sagittal T1 C+ MR shows an enhancing mass along the superior infundibulum and anterior 3rd ventricle <img src='img/arrows/CO.png'/>. Granular cell tumor was diagnosed at resection.*
![Coronal T1 C+ MR shows a large sellar and suprasellar enhancing mass contacting the left optic chiasm <img src='img/arrows/WO.png'/>. Granular cell tumor was diagnosed at resection. Imaging mimics the much more common pituitary macroadenoma.](images/app.statdx.com_image_thumbnail_51f9669f-be82-4dd4-89b7-27b6c5fda51a_size_168_quality_85_8f77246c_20251014T205304Z.jpg)
*Coronal T1 C+ MR shows a large sellar and suprasellar enhancing mass contacting the left optic chiasm <img src='img/arrows/WO.png'/>. Granular cell tumor was diagnosed at resection. Imaging mimics the much more common pituitary macroadenoma.*
docs_md/articles/hypertrophic-olivary-degeneration_78257543-6d52-4879-84b1-445f3611d996.md
+136 -29
View File
@@ -32,7 +32,6 @@ breadcrumbs:
slug: "hypertrophic-olivary-degeneration"
treeNodeId: null
category: "Brain"
cmeTopicId: "b70885e6-d7ea-4f0f-8b2c-c871245fd05c"
documentVersionId: "0c307ba9-ac00-479c-9a0f-4201c66bc1f1"
imageCount: 26
lastUpdated: "09/30/20"
@@ -333,7 +332,7 @@ breadcrumbs:
- Avoid misdiagnosis of tumor or multiple sclerosis
- Bilateral & symmetrical lesions in ION argue against subacute infarct & vertebral artery dissection
9342df55-d4e7-4743-8a78-b03338f00dc0
88b7142c-a73e-4046-a5d4-0c12bddb8133
## References
@@ -369,87 +368,195 @@ breadcrumbs:
### Selected Images
![Axial graphic of the upper medulla shows the medullary pyramids <img src='img/arrows/CC.png'/> on each side of the ventral median fissure. The olives <img src='img/arrows/CO.png'/> lie just posterior to the preolivary sulci <img src='img/arrows/CS.png'/>.](images/app.statdx.com_image_thumbnail_e2ab6980-4968-4bcb-9864-1fa85eceedca_annotated_true_size_900_quality_90_eafe6900_20251018T122607Z.jpg)
![Axial graphic of the upper medulla shows the medullary pyramids <img src='img/arrows/CC.png'/> on each side of the ventral median fissure. The olives <img src='img/arrows/CO.png'/> lie just posterior to the preolivary sulci <img src='img/arrows/CS.png'/>.](images/app.statdx.com_image_e2ab6980-4968-4bcb-9864-1fa85eceedca_6ebcdb95_20251014T204755Z.jpg)
*Axial graphic of the upper medulla shows the medullary pyramids <img src='img/arrows/CC.png'/> on each side of the ventral median fissure. The olives <img src='img/arrows/CO.png'/> lie just posterior to the preolivary sulci <img src='img/arrows/CS.png'/>.*
![Axial graphic of the upper medulla shows the medullary pyramids <img src='img/arrows/CC.png'/> on each side of the ventral median fissure. The olives <img src='img/arrows/CO.png'/> lie just posterior to the preolivary sulci <img src='img/arrows/CS.png'/>.](images/app.statdx.com_image_thumbnail_e2ab6980-4968-4bcb-9864-1fa85eceedca_size_174_quality_85_b06dd025_20251018T122603Z.jpg)
![Axial graphic of the upper medulla shows the medullary pyramids <img src='img/arrows/CC.png'/> on each side of the ventral median fissure. The olives <img src='img/arrows/CO.png'/> lie just posterior to the preolivary sulci <img src='img/arrows/CS.png'/>.](images/app.statdx.com_image_thumbnail_e2ab6980-4968-4bcb-9864-1fa85eceedca_size_168_quality_85_20601fcc_20251014T204754Z.jpg)
*Axial graphic of the upper medulla shows the medullary pyramids <img src='img/arrows/CC.png'/> on each side of the ventral median fissure. The olives <img src='img/arrows/CO.png'/> lie just posterior to the preolivary sulci <img src='img/arrows/CS.png'/>.*
![Coronal graphic of the midbrain, pons, and medulla is sectioned to depict the Guillain-Mollaret triangle (GMT). The GMT is composed of the ipsilateral inferior olivary nucleus (green), dentate nucleus (blue) of the contralateral cerebellum, and the ipsilateral red nucleus (RN, red).](images/app.statdx.com_image_thumbnail_87b7bf23-7410-4d44-a5f1-8dac3d81f82f_annotated_true_size_900_quality_90_5e71e83b_20251018T122607Z.jpg)
![Axial graphic of the upper medulla shows the medullary pyramids <img src='img/arrows/CC.png'/> on each side of the ventral median fissure. The olives <img src='img/arrows/CO.png'/> lie just posterior to the preolivary sulci <img src='img/arrows/CS.png'/>.](images/app.statdx.com_image_thumbnail_e2ab6980-4968-4bcb-9864-1fa85eceedca_size_168_quality_85_41df60ec_20251014T204719Z.jpg)
*Axial graphic of the upper medulla shows the medullary pyramids <img src='img/arrows/CC.png'/> on each side of the ventral median fissure. The olives <img src='img/arrows/CO.png'/> lie just posterior to the preolivary sulci <img src='img/arrows/CS.png'/>.*
![Coronal graphic of the midbrain, pons, and medulla is sectioned to depict the Guillain-Mollaret triangle (GMT). The GMT is composed of the ipsilateral inferior olivary nucleus (green), dentate nucleus (blue) of the contralateral cerebellum, and the ipsilateral red nucleus (RN, red).](images/app.statdx.com_image_87b7bf23-7410-4d44-a5f1-8dac3d81f82f_023985c7_20251014T204757Z.jpg)
*Coronal graphic of the midbrain, pons, and medulla is sectioned to depict the Guillain-Mollaret triangle (GMT). The GMT is composed of the ipsilateral inferior olivary nucleus (green), dentate nucleus (blue) of the contralateral cerebellum, and the ipsilateral red nucleus (RN, red).*
![Axial T2 MR of a 40-year-old woman with brainstem glioma and secondary hypertrophic olivary degeneration (HOD) shows a heterogeneous mass lesion involving midbrain <img src='img/arrows/CO.png'/> invading the RN <img src='img/arrows/CS.png'/> (R &gt; L). RN is a component of GMT.](images/app.statdx.com_image_thumbnail_6489d4cd-9c84-4d0a-8fc5-8e987f5d8077_annotated_true_size_900_quality_90_3eee82f5_20251018T122607Z.jpg)
![Coronal graphic of the midbrain, pons, and medulla is sectioned to depict the Guillain-Mollaret triangle (GMT). The GMT is composed of the ipsilateral inferior olivary nucleus (green), dentate nucleus (blue) of the contralateral cerebellum, and the ipsilateral red nucleus (RN, red).](images/app.statdx.com_image_thumbnail_87b7bf23-7410-4d44-a5f1-8dac3d81f82f_size_168_quality_85_7d2edc18_20251014T204719Z.jpg)
*Coronal graphic of the midbrain, pons, and medulla is sectioned to depict the Guillain-Mollaret triangle (GMT). The GMT is composed of the ipsilateral inferior olivary nucleus (green), dentate nucleus (blue) of the contralateral cerebellum, and the ipsilateral red nucleus (RN, red).*
![Coronal graphic of the midbrain, pons, and medulla is sectioned to depict the Guillain-Mollaret triangle (GMT). The GMT is composed of the ipsilateral inferior olivary nucleus (green), dentate nucleus (blue) of the contralateral cerebellum, and the ipsilateral red nucleus (RN, red).](images/app.statdx.com_image_thumbnail_87b7bf23-7410-4d44-a5f1-8dac3d81f82f_size_168_quality_85_87d93765_20251014T204754Z.jpg)
*Coronal graphic of the midbrain, pons, and medulla is sectioned to depict the Guillain-Mollaret triangle (GMT). The GMT is composed of the ipsilateral inferior olivary nucleus (green), dentate nucleus (blue) of the contralateral cerebellum, and the ipsilateral red nucleus (RN, red).*
![Axial T2 MR of a 40-year-old woman with brainstem glioma and secondary hypertrophic olivary degeneration (HOD) shows a heterogeneous mass lesion involving midbrain <img src='img/arrows/CO.png'/> invading the RN <img src='img/arrows/CS.png'/> (R &gt; L). RN is a component of GMT.](images/app.statdx.com_image_6489d4cd-9c84-4d0a-8fc5-8e987f5d8077_7638176e_20251014T204759Z.jpg)
*Axial T2 MR of a 40-year-old woman with brainstem glioma and secondary hypertrophic olivary degeneration (HOD) shows a heterogeneous mass lesion involving midbrain <img src='img/arrows/CO.png'/> invading the RN <img src='img/arrows/CS.png'/> (R &gt; L). RN is a component of GMT.*
![Axial T2 MR at the level of medulla in the same patient shows enlarged right inferior olivary nucleus with hyperintense signal <img src='img/arrows/CC.png'/> indicating HOD. Also note normal-appearing left olivary nucleus <img src='img/arrows/BC.png'/> and preolivary sulcus <img src='img/arrows/BS.png'/>.](images/app.statdx.com_image_thumbnail_9b10efdb-4747-4a85-adcb-e23c73ffe4cc_annotated_true_size_900_quality_90_de6979c0_20251018T122607Z.jpg)
![Axial T2 MR of a 40-year-old woman with brainstem glioma and secondary hypertrophic olivary degeneration (HOD) shows a heterogeneous mass lesion involving midbrain <img src='img/arrows/CO.png'/> invading the RN <img src='img/arrows/CS.png'/> (R &gt; L). RN is a component of GMT.](images/app.statdx.com_image_thumbnail_6489d4cd-9c84-4d0a-8fc5-8e987f5d8077_size_168_quality_85_128fac13_20251014T204719Z.jpg)
*Axial T2 MR of a 40-year-old woman with brainstem glioma and secondary hypertrophic olivary degeneration (HOD) shows a heterogeneous mass lesion involving midbrain <img src='img/arrows/CO.png'/> invading the RN <img src='img/arrows/CS.png'/> (R &gt; L). RN is a component of GMT.*
![Axial T2 MR of a 40-year-old woman with brainstem glioma and secondary hypertrophic olivary degeneration (HOD) shows a heterogeneous mass lesion involving midbrain <img src='img/arrows/CO.png'/> invading the RN <img src='img/arrows/CS.png'/> (R &gt; L). RN is a component of GMT.](images/app.statdx.com_image_thumbnail_6489d4cd-9c84-4d0a-8fc5-8e987f5d8077_size_168_quality_85_2f16fdc1_20251014T204754Z.jpg)
*Axial T2 MR of a 40-year-old woman with brainstem glioma and secondary hypertrophic olivary degeneration (HOD) shows a heterogeneous mass lesion involving midbrain <img src='img/arrows/CO.png'/> invading the RN <img src='img/arrows/CS.png'/> (R &gt; L). RN is a component of GMT.*
![Axial T2 MR at the level of medulla in the same patient shows enlarged right inferior olivary nucleus with hyperintense signal <img src='img/arrows/CC.png'/> indicating HOD. Also note normal-appearing left olivary nucleus <img src='img/arrows/BC.png'/> and preolivary sulcus <img src='img/arrows/BS.png'/>.](images/app.statdx.com_image_9b10efdb-4747-4a85-adcb-e23c73ffe4cc_8b39bbda_20251014T204801Z.jpg)
*Axial T2 MR at the level of medulla in the same patient shows enlarged right inferior olivary nucleus with hyperintense signal <img src='img/arrows/CC.png'/> indicating HOD. Also note normal-appearing left olivary nucleus <img src='img/arrows/BC.png'/> and preolivary sulcus <img src='img/arrows/BS.png'/>.*
![Axial FLAIR MR of a 58-year-old woman presenting with palatal myoclonus and a history of treated CNS lymphoma shows volume loss and hyperintense signal in left dentate nucleus (DN) <img src='img/arrows/CO.png'/> due to encephalomalacia (DN is a component of GMT).](images/app.statdx.com_image_thumbnail_45d7e872-98da-486f-ba07-c5778f1207a7_annotated_true_size_900_quality_90_14f511a6_20251018T122607Z.jpg)
![Axial T2 MR at the level of medulla in the same patient shows enlarged right inferior olivary nucleus with hyperintense signal <img src='img/arrows/CC.png'/> indicating HOD. Also note normal-appearing left olivary nucleus <img src='img/arrows/BC.png'/> and preolivary sulcus <img src='img/arrows/BS.png'/>.](images/app.statdx.com_image_thumbnail_9b10efdb-4747-4a85-adcb-e23c73ffe4cc_size_168_quality_85_1ba77311_20251014T204754Z.jpg)
*Axial T2 MR at the level of medulla in the same patient shows enlarged right inferior olivary nucleus with hyperintense signal <img src='img/arrows/CC.png'/> indicating HOD. Also note normal-appearing left olivary nucleus <img src='img/arrows/BC.png'/> and preolivary sulcus <img src='img/arrows/BS.png'/>.*
![Axial T2 MR at the level of medulla in the same patient shows enlarged right inferior olivary nucleus with hyperintense signal <img src='img/arrows/CC.png'/> indicating HOD. Also note normal-appearing left olivary nucleus <img src='img/arrows/BC.png'/> and preolivary sulcus <img src='img/arrows/BS.png'/>.](images/app.statdx.com_image_thumbnail_9b10efdb-4747-4a85-adcb-e23c73ffe4cc_size_168_quality_85_6aecad07_20251014T204719Z.jpg)
*Axial T2 MR at the level of medulla in the same patient shows enlarged right inferior olivary nucleus with hyperintense signal <img src='img/arrows/CC.png'/> indicating HOD. Also note normal-appearing left olivary nucleus <img src='img/arrows/BC.png'/> and preolivary sulcus <img src='img/arrows/BS.png'/>.*
![Axial FLAIR MR of a 58-year-old woman presenting with palatal myoclonus and a history of treated CNS lymphoma shows volume loss and hyperintense signal in left dentate nucleus (DN) <img src='img/arrows/CO.png'/> due to encephalomalacia (DN is a component of GMT).](images/app.statdx.com_image_45d7e872-98da-486f-ba07-c5778f1207a7_2e22a519_20251014T204802Z.jpg)
*Axial FLAIR MR of a 58-year-old woman presenting with palatal myoclonus and a history of treated CNS lymphoma shows volume loss and hyperintense signal in left dentate nucleus (DN) <img src='img/arrows/CO.png'/> due to encephalomalacia (DN is a component of GMT).*
![Axial T2 MR in the same patient at the level of medulla shows mild hypertrophy and increased signal involving bilateral inferior olivary nuclei <img src='img/arrows/CC.png'/> indicating HOD.](images/app.statdx.com_image_thumbnail_949d9854-f619-4e10-87e8-08b1674cd7b0_annotated_true_size_900_quality_90_732f280d_20251018T122607Z.jpg)
![Axial FLAIR MR of a 58-year-old woman presenting with palatal myoclonus and a history of treated CNS lymphoma shows volume loss and hyperintense signal in left dentate nucleus (DN) <img src='img/arrows/CO.png'/> due to encephalomalacia (DN is a component of GMT).](images/app.statdx.com_image_thumbnail_45d7e872-98da-486f-ba07-c5778f1207a7_size_168_quality_85_0a07159d_20251014T204719Z.jpg)
*Axial FLAIR MR of a 58-year-old woman presenting with palatal myoclonus and a history of treated CNS lymphoma shows volume loss and hyperintense signal in left dentate nucleus (DN) <img src='img/arrows/CO.png'/> due to encephalomalacia (DN is a component of GMT).*
![Axial FLAIR MR of a 58-year-old woman presenting with palatal myoclonus and a history of treated CNS lymphoma shows volume loss and hyperintense signal in left dentate nucleus (DN) <img src='img/arrows/CO.png'/> due to encephalomalacia (DN is a component of GMT).](images/app.statdx.com_image_thumbnail_45d7e872-98da-486f-ba07-c5778f1207a7_size_168_quality_85_e224aaf6_20251014T204754Z.jpg)
*Axial FLAIR MR of a 58-year-old woman presenting with palatal myoclonus and a history of treated CNS lymphoma shows volume loss and hyperintense signal in left dentate nucleus (DN) <img src='img/arrows/CO.png'/> due to encephalomalacia (DN is a component of GMT).*
![Axial T2 MR in the same patient at the level of medulla shows mild hypertrophy and increased signal involving bilateral inferior olivary nuclei <img src='img/arrows/CC.png'/> indicating HOD.](images/app.statdx.com_image_949d9854-f619-4e10-87e8-08b1674cd7b0_90f421ff_20251014T204803Z.jpg)
*Axial T2 MR in the same patient at the level of medulla shows mild hypertrophy and increased signal involving bilateral inferior olivary nuclei <img src='img/arrows/CC.png'/> indicating HOD.*
![Axial T2 of a 67-year-old man with left para median pontine cavernous malformation (CM) involving central tegmental tract resulting in ipsilateral HOD shows hyperintense popcorn lesion with rim of hemosiderin in left para median pons <img src='img/arrows/CS.png'/> due to CM.](images/app.statdx.com_image_thumbnail_7c52868b-ffa4-46aa-bf88-27a6ca7a5281_annotated_true_size_900_quality_90_28eaaafe_20251018T122607Z.jpg)
![Axial T2 MR in the same patient at the level of medulla shows mild hypertrophy and increased signal involving bilateral inferior olivary nuclei <img src='img/arrows/CC.png'/> indicating HOD.](images/app.statdx.com_image_thumbnail_949d9854-f619-4e10-87e8-08b1674cd7b0_size_168_quality_85_976e7715_20251014T204719Z.jpg)
*Axial T2 MR in the same patient at the level of medulla shows mild hypertrophy and increased signal involving bilateral inferior olivary nuclei <img src='img/arrows/CC.png'/> indicating HOD.*
![Axial T2 MR in the same patient at the level of medulla shows mild hypertrophy and increased signal involving bilateral inferior olivary nuclei <img src='img/arrows/CC.png'/> indicating HOD.](images/app.statdx.com_image_thumbnail_949d9854-f619-4e10-87e8-08b1674cd7b0_size_168_quality_85_d519f596_20251014T204754Z.jpg)
*Axial T2 MR in the same patient at the level of medulla shows mild hypertrophy and increased signal involving bilateral inferior olivary nuclei <img src='img/arrows/CC.png'/> indicating HOD.*
![Axial T2 of a 67-year-old man with left para median pontine cavernous malformation (CM) involving central tegmental tract resulting in ipsilateral HOD shows hyperintense popcorn lesion with rim of hemosiderin in left para median pons <img src='img/arrows/CS.png'/> due to CM.](images/app.statdx.com_image_7c52868b-ffa4-46aa-bf88-27a6ca7a5281_17405b87_20251014T204804Z.jpg)
*Axial T2 of a 67-year-old man with left para median pontine cavernous malformation (CM) involving central tegmental tract resulting in ipsilateral HOD shows hyperintense popcorn lesion with rim of hemosiderin in left para median pons <img src='img/arrows/CS.png'/> due to CM.*
![Axial FLAIR MR in the same patient shows enlarged left inferior olivary nucleus with hyperintense signal <img src='img/arrows/CO.png'/> due to HOD.](images/app.statdx.com_image_thumbnail_5f1f5911-5f58-460f-b712-eff1a657b51e_annotated_true_size_900_quality_90_351a3f56_20251018T122607Z.jpg)
![Axial T2 of a 67-year-old man with left para median pontine cavernous malformation (CM) involving central tegmental tract resulting in ipsilateral HOD shows hyperintense popcorn lesion with rim of hemosiderin in left para median pons <img src='img/arrows/CS.png'/> due to CM.](images/app.statdx.com_image_thumbnail_7c52868b-ffa4-46aa-bf88-27a6ca7a5281_size_168_quality_85_5c3b0041_20251014T204754Z.jpg)
*Axial T2 of a 67-year-old man with left para median pontine cavernous malformation (CM) involving central tegmental tract resulting in ipsilateral HOD shows hyperintense popcorn lesion with rim of hemosiderin in left para median pons <img src='img/arrows/CS.png'/> due to CM.*
![Axial T2 of a 67-year-old man with left para median pontine cavernous malformation (CM) involving central tegmental tract resulting in ipsilateral HOD shows hyperintense popcorn lesion with rim of hemosiderin in left para median pons <img src='img/arrows/CS.png'/> due to CM.](images/app.statdx.com_image_thumbnail_7c52868b-ffa4-46aa-bf88-27a6ca7a5281_size_168_quality_85_ab85dadf_20251014T204719Z.jpg)
*Axial T2 of a 67-year-old man with left para median pontine cavernous malformation (CM) involving central tegmental tract resulting in ipsilateral HOD shows hyperintense popcorn lesion with rim of hemosiderin in left para median pons <img src='img/arrows/CS.png'/> due to CM.*
![Axial FLAIR MR in the same patient shows enlarged left inferior olivary nucleus with hyperintense signal <img src='img/arrows/CO.png'/> due to HOD.](images/app.statdx.com_image_5f1f5911-5f58-460f-b712-eff1a657b51e_bc24d3df_20251014T204806Z.jpg)
*Axial FLAIR MR in the same patient shows enlarged left inferior olivary nucleus with hyperintense signal <img src='img/arrows/CO.png'/> due to HOD.*
![Axial T2 MR at 1 day (top left), 4 months (top right), and 7 months (bottom left) postoperative follow-up show edema in left DN <img src='img/arrows/CS.png'/> and normal right olive <img src='img/arrows/CO.png'/>. Note light enlargement and ↑ signal in right olive <img src='img/arrows/CC.png'/>, progressive enlargement and ↑ signal in olive <img src='img/arrows/WC.png'/>, and lack of enhancement in olive <img src='img/arrows/WO.png'/> on postcontrast T1WI (bottom right).](images/app.statdx.com_image_thumbnail_bfd80fc4-8257-4305-be57-f6f17e5d2725_annotated_true_size_900_quality_90_0ec94d8f_20251018T122607Z.jpg)
![Axial FLAIR MR in the same patient shows enlarged left inferior olivary nucleus with hyperintense signal <img src='img/arrows/CO.png'/> due to HOD.](images/app.statdx.com_image_thumbnail_5f1f5911-5f58-460f-b712-eff1a657b51e_size_168_quality_85_2eaa4324_20251014T204754Z.jpg)
*Axial FLAIR MR in the same patient shows enlarged left inferior olivary nucleus with hyperintense signal <img src='img/arrows/CO.png'/> due to HOD.*
![Axial FLAIR MR in the same patient shows enlarged left inferior olivary nucleus with hyperintense signal <img src='img/arrows/CO.png'/> due to HOD.](images/app.statdx.com_image_thumbnail_5f1f5911-5f58-460f-b712-eff1a657b51e_size_168_quality_85_576c80ec_20251014T204719Z.jpg)
*Axial FLAIR MR in the same patient shows enlarged left inferior olivary nucleus with hyperintense signal <img src='img/arrows/CO.png'/> due to HOD.*
![Axial T2 MR at 1 day (top left), 4 months (top right), and 7 months (bottom left) postoperative follow-up show edema in left DN <img src='img/arrows/CS.png'/> and normal right olive <img src='img/arrows/CO.png'/>. Note light enlargement and ↑ signal in right olive <img src='img/arrows/CC.png'/>, progressive enlargement and ↑ signal in olive <img src='img/arrows/WC.png'/>, and lack of enhancement in olive <img src='img/arrows/WO.png'/> on postcontrast T1WI (bottom right).](images/app.statdx.com_image_bfd80fc4-8257-4305-be57-f6f17e5d2725_068d6772_20251014T204807Z.jpg)
*Axial T2 MR at 1 day (top left), 4 months (top right), and 7 months (bottom left) postoperative follow-up show edema in left DN <img src='img/arrows/CS.png'/> and normal right olive <img src='img/arrows/CO.png'/>. Note light enlargement and ↑ signal in right olive <img src='img/arrows/CC.png'/>, progressive enlargement and ↑ signal in olive <img src='img/arrows/WC.png'/>, and lack of enhancement in olive <img src='img/arrows/WO.png'/> on postcontrast T1WI (bottom right).*
![Axial graphic of the midbrain at the level of the hypoglossal nuclei shows the distinct wavy pattern of the olives <img src='img/arrows/CS.png'/> corresponding to the FLAIR hyperintensity in the previous image.](af8d64b8-e6d3-4f2c-bae7-0c7e3b0fba51)
![Axial T2 MR at 1 day (top left), 4 months (top right), and 7 months (bottom left) postoperative follow-up show edema in left DN <img src='img/arrows/CS.png'/> and normal right olive <img src='img/arrows/CO.png'/>. Note light enlargement and ↑ signal in right olive <img src='img/arrows/CC.png'/>, progressive enlargement and ↑ signal in olive <img src='img/arrows/WC.png'/>, and lack of enhancement in olive <img src='img/arrows/WO.png'/> on postcontrast T1WI (bottom right).](images/app.statdx.com_image_thumbnail_bfd80fc4-8257-4305-be57-f6f17e5d2725_size_168_quality_85_2d84ce83_20251014T204719Z.jpg)
*Axial T2 MR at 1 day (top left), 4 months (top right), and 7 months (bottom left) postoperative follow-up show edema in left DN <img src='img/arrows/CS.png'/> and normal right olive <img src='img/arrows/CO.png'/>. Note light enlargement and ↑ signal in right olive <img src='img/arrows/CC.png'/>, progressive enlargement and ↑ signal in olive <img src='img/arrows/WC.png'/>, and lack of enhancement in olive <img src='img/arrows/WO.png'/> on postcontrast T1WI (bottom right).*
![Axial T2 MR at 1 day (top left), 4 months (top right), and 7 months (bottom left) postoperative follow-up show edema in left DN <img src='img/arrows/CS.png'/> and normal right olive <img src='img/arrows/CO.png'/>. Note light enlargement and ↑ signal in right olive <img src='img/arrows/CC.png'/>, progressive enlargement and ↑ signal in olive <img src='img/arrows/WC.png'/>, and lack of enhancement in olive <img src='img/arrows/WO.png'/> on postcontrast T1WI (bottom right).](images/app.statdx.com_image_thumbnail_bfd80fc4-8257-4305-be57-f6f17e5d2725_size_168_quality_85_5ac0e6ac_20251014T204754Z.jpg)
*Axial T2 MR at 1 day (top left), 4 months (top right), and 7 months (bottom left) postoperative follow-up show edema in left DN <img src='img/arrows/CS.png'/> and normal right olive <img src='img/arrows/CO.png'/>. Note light enlargement and ↑ signal in right olive <img src='img/arrows/CC.png'/>, progressive enlargement and ↑ signal in olive <img src='img/arrows/WC.png'/>, and lack of enhancement in olive <img src='img/arrows/WO.png'/> on postcontrast T1WI (bottom right).*
![Axial graphic of the midbrain at the level of the hypoglossal nuclei shows the distinct wavy pattern of the olives <img src='img/arrows/CS.png'/> corresponding to the FLAIR hyperintensity in the previous image.](images/app.statdx.com_image_af8d64b8-e6d3-4f2c-bae7-0c7e3b0fba51_00a8db06_20251014T204808Z.jpg)
*Axial graphic of the midbrain at the level of the hypoglossal nuclei shows the distinct wavy pattern of the olives <img src='img/arrows/CS.png'/> corresponding to the FLAIR hyperintensity in the previous image.*
![Axial graphic of the midbrain at the level of the hypoglossal nuclei shows the distinct wavy pattern of the olives <img src='img/arrows/CS.png'/> corresponding to the FLAIR hyperintensity in the previous image.](images/app.statdx.com_image_thumbnail_af8d64b8-e6d3-4f2c-bae7-0c7e3b0fba51_size_168_quality_85_c50c0f98_20251014T204719Z.jpg)
*Axial graphic of the midbrain at the level of the hypoglossal nuclei shows the distinct wavy pattern of the olives <img src='img/arrows/CS.png'/> corresponding to the FLAIR hyperintensity in the previous image.*
![Axial graphic of the midbrain at the level of the hypoglossal nuclei shows the distinct wavy pattern of the olives <img src='img/arrows/CS.png'/> corresponding to the FLAIR hyperintensity in the previous image.](images/app.statdx.com_image_thumbnail_af8d64b8-e6d3-4f2c-bae7-0c7e3b0fba51_size_168_quality_85_e0e343e4_20251014T204754Z.jpg)
*Axial graphic of the midbrain at the level of the hypoglossal nuclei shows the distinct wavy pattern of the olives <img src='img/arrows/CS.png'/> corresponding to the FLAIR hyperintensity in the previous image.*
### Additional Images
![Axial T2WI MR demonstrates hypertrophy of both inferior olivary nuclei, which are also hyperintense <img src='img/arrows/CS.png'/>, secondary to HOD.](3e5effcb-b025-48ed-ba4b-2b20975d18d0)
![Axial T2WI MR demonstrates hypertrophy of both inferior olivary nuclei, which are also hyperintense <img src='img/arrows/CS.png'/>, secondary to HOD.](images/app.statdx.com_image_3e5effcb-b025-48ed-ba4b-2b20975d18d0_80366b9a_20251014T204809Z.jpg)
*Axial T2WI MR demonstrates hypertrophy of both inferior olivary nuclei, which are also hyperintense <img src='img/arrows/CS.png'/>, secondary to HOD.*
![Sagittal FLAIR MR shows abnormally ↑ signal intensity in an anterior medullary area <img src='img/arrows/CS.png'/> that corresponds to the inferior olivary nucleus.](2698fac6-694f-43bc-904f-c368445620a9)
![Axial T2WI MR demonstrates hypertrophy of both inferior olivary nuclei, which are also hyperintense <img src='img/arrows/CS.png'/>, secondary to HOD.](images/app.statdx.com_image_thumbnail_3e5effcb-b025-48ed-ba4b-2b20975d18d0_size_168_quality_85_e47b3356_20251014T204719Z.jpg)
*Axial T2WI MR demonstrates hypertrophy of both inferior olivary nuclei, which are also hyperintense <img src='img/arrows/CS.png'/>, secondary to HOD.*
![Axial T2WI MR demonstrates hypertrophy of both inferior olivary nuclei, which are also hyperintense <img src='img/arrows/CS.png'/>, secondary to HOD.](images/app.statdx.com_image_thumbnail_3e5effcb-b025-48ed-ba4b-2b20975d18d0_size_168_quality_85_f2db0be1_20251014T204754Z.jpg)
*Axial T2WI MR demonstrates hypertrophy of both inferior olivary nuclei, which are also hyperintense <img src='img/arrows/CS.png'/>, secondary to HOD.*
![Sagittal FLAIR MR shows abnormally ↑ signal intensity in an anterior medullary area <img src='img/arrows/CS.png'/> that corresponds to the inferior olivary nucleus.](images/app.statdx.com_image_thumbnail_2698fac6-694f-43bc-904f-c368445620a9_size_168_quality_85_39b0c1ce_20251014T204754Z.jpg)
*Sagittal FLAIR MR shows abnormally ↑ signal intensity in an anterior medullary area <img src='img/arrows/CS.png'/> that corresponds to the inferior olivary nucleus.*
![Axial FLAIR MR in the same patient who suffered midbrain hemorrhage (not shown) depicts bilateral hyperintense and hypertrophied inferior olivary nuclei <img src='img/arrows/CS.png'/>.](3b581539-62f7-42f9-8909-56a190ba83e1)
![Sagittal FLAIR MR shows abnormally ↑ signal intensity in an anterior medullary area <img src='img/arrows/CS.png'/> that corresponds to the inferior olivary nucleus.](images/app.statdx.com_image_thumbnail_2698fac6-694f-43bc-904f-c368445620a9_size_168_quality_85_9037044c_20251014T204719Z.jpg)
*Sagittal FLAIR MR shows abnormally ↑ signal intensity in an anterior medullary area <img src='img/arrows/CS.png'/> that corresponds to the inferior olivary nucleus.*
![Axial FLAIR MR in the same patient who suffered midbrain hemorrhage (not shown) depicts bilateral hyperintense and hypertrophied inferior olivary nuclei <img src='img/arrows/CS.png'/>.](images/app.statdx.com_image_thumbnail_3b581539-62f7-42f9-8909-56a190ba83e1_size_168_quality_85_36b746e4_20251014T204754Z.jpg)
*Axial FLAIR MR in the same patient who suffered midbrain hemorrhage (not shown) depicts bilateral hyperintense and hypertrophied inferior olivary nuclei <img src='img/arrows/CS.png'/>.*
![Axial FLAIR MR shows high signal intensity and asymmetric enlargement of right anterior medulla corresponding to the region of hypertrophic degeneration of the right inferior olivary nucleus <img src='img/arrows/CS.png'/> .](9dcb9590-4f66-4d9a-b58b-fd84da758053)
![Axial FLAIR MR in the same patient who suffered midbrain hemorrhage (not shown) depicts bilateral hyperintense and hypertrophied inferior olivary nuclei <img src='img/arrows/CS.png'/>.](images/app.statdx.com_image_thumbnail_3b581539-62f7-42f9-8909-56a190ba83e1_size_168_quality_85_a4513686_20251014T204719Z.jpg)
*Axial FLAIR MR in the same patient who suffered midbrain hemorrhage (not shown) depicts bilateral hyperintense and hypertrophied inferior olivary nuclei <img src='img/arrows/CS.png'/>.*
![Axial FLAIR MR shows high signal intensity and asymmetric enlargement of right anterior medulla corresponding to the region of hypertrophic degeneration of the right inferior olivary nucleus <img src='img/arrows/CS.png'/> .](images/app.statdx.com_image_thumbnail_9dcb9590-4f66-4d9a-b58b-fd84da758053_size_168_quality_85_4bf0f302_20251014T204754Z.jpg)
*Axial FLAIR MR shows high signal intensity and asymmetric enlargement of right anterior medulla corresponding to the region of hypertrophic degeneration of the right inferior olivary nucleus <img src='img/arrows/CS.png'/> .*
![Axial T2WI MR in the same patient shows a right pontine infarct, the primary lesion that led to right HOD.](c6ad71d6-76b6-407d-8d3f-5520dbb1bbca)
![Axial FLAIR MR shows high signal intensity and asymmetric enlargement of right anterior medulla corresponding to the region of hypertrophic degeneration of the right inferior olivary nucleus <img src='img/arrows/CS.png'/> .](images/app.statdx.com_image_thumbnail_9dcb9590-4f66-4d9a-b58b-fd84da758053_size_168_quality_85_9540ce82_20251014T204719Z.jpg)
*Axial FLAIR MR shows high signal intensity and asymmetric enlargement of right anterior medulla corresponding to the region of hypertrophic degeneration of the right inferior olivary nucleus <img src='img/arrows/CS.png'/> .*
![Axial T2WI MR in the same patient shows a right pontine infarct, the primary lesion that led to right HOD.](images/app.statdx.com_image_thumbnail_c6ad71d6-76b6-407d-8d3f-5520dbb1bbca_size_168_quality_85_25516031_20251014T204719Z.jpg)
*Axial T2WI MR in the same patient shows a right pontine infarct, the primary lesion that led to right HOD.*
![Axial T2WI MR shows bilateral symmetric hypertrophy with ↑ signal intensity confined to inferior olivary nuclei, with loss of pre- and postolivary sulci <img src='img/arrows/CS.png'/>.](d3122192-cad3-4f63-ae3b-d0bfe6f8b428)
![Axial T2WI MR in the same patient shows a right pontine infarct, the primary lesion that led to right HOD.](images/app.statdx.com_image_thumbnail_c6ad71d6-76b6-407d-8d3f-5520dbb1bbca_size_168_quality_85_dfe86ce5_20251014T204754Z.jpg)
*Axial T2WI MR in the same patient shows a right pontine infarct, the primary lesion that led to right HOD.*
![Axial T2WI MR shows bilateral symmetric hypertrophy with ↑ signal intensity confined to inferior olivary nuclei, with loss of pre- and postolivary sulci <img src='img/arrows/CS.png'/>.](images/app.statdx.com_image_thumbnail_d3122192-cad3-4f63-ae3b-d0bfe6f8b428_size_168_quality_85_ab9a5054_20251014T204754Z.jpg)
*Axial T2WI MR shows bilateral symmetric hypertrophy with ↑ signal intensity confined to inferior olivary nuclei, with loss of pre- and postolivary sulci <img src='img/arrows/CS.png'/>.*
![Axial T2WI MR in the same patient shows the primary midbrain lesion that caused the occurrence of bilateral HOD.](7f3adfa6-8b44-448b-acbc-7bec45f4c5f8)
![Axial T2WI MR shows bilateral symmetric hypertrophy with ↑ signal intensity confined to inferior olivary nuclei, with loss of pre- and postolivary sulci <img src='img/arrows/CS.png'/>.](images/app.statdx.com_image_thumbnail_d3122192-cad3-4f63-ae3b-d0bfe6f8b428_size_168_quality_85_bbf068ad_20251014T204719Z.jpg)
*Axial T2WI MR shows bilateral symmetric hypertrophy with ↑ signal intensity confined to inferior olivary nuclei, with loss of pre- and postolivary sulci <img src='img/arrows/CS.png'/>.*
![Axial T2WI MR in the same patient shows the primary midbrain lesion that caused the occurrence of bilateral HOD.](images/app.statdx.com_image_thumbnail_7f3adfa6-8b44-448b-acbc-7bec45f4c5f8_size_168_quality_85_18b8546d_20251014T204754Z.jpg)
*Axial T2WI MR in the same patient shows the primary midbrain lesion that caused the occurrence of bilateral HOD.*
![Axial T2WI MR in a patient who developed onset of dysarthria and upper extremity dysmetria 15 months following stereotaxic XRT for midbrain arteriovenous malformation shows mixed hyper-/hypointensity in the residual vascular malformation <img src='img/arrows/CO.png'/>.](717f7010-1fdc-42fa-adfe-f828d24b650a)
![Axial T2WI MR in the same patient shows the primary midbrain lesion that caused the occurrence of bilateral HOD.](images/app.statdx.com_image_thumbnail_7f3adfa6-8b44-448b-acbc-7bec45f4c5f8_size_168_quality_85_c64bfc5e_20251014T204719Z.jpg)
*Axial T2WI MR in the same patient shows the primary midbrain lesion that caused the occurrence of bilateral HOD.*
![Axial T2WI MR in a patient who developed onset of dysarthria and upper extremity dysmetria 15 months following stereotaxic XRT for midbrain arteriovenous malformation shows mixed hyper-/hypointensity in the residual vascular malformation <img src='img/arrows/CO.png'/>.](images/app.statdx.com_image_thumbnail_717f7010-1fdc-42fa-adfe-f828d24b650a_size_168_quality_85_8310efb4_20251014T204719Z.jpg)
*Axial T2WI MR in a patient who developed onset of dysarthria and upper extremity dysmetria 15 months following stereotaxic XRT for midbrain arteriovenous malformation shows mixed hyper-/hypointensity in the residual vascular malformation <img src='img/arrows/CO.png'/>.*
![Axial T2WI MR in the same patient shows bilateral inferior olivary hyperintensity and hypertrophy <img src='img/arrows/CS.png'/>.](81bec60a-3dee-42d5-bb77-cf3045ec6b06)
![Axial T2WI MR in a patient who developed onset of dysarthria and upper extremity dysmetria 15 months following stereotaxic XRT for midbrain arteriovenous malformation shows mixed hyper-/hypointensity in the residual vascular malformation <img src='img/arrows/CO.png'/>.](images/app.statdx.com_image_thumbnail_717f7010-1fdc-42fa-adfe-f828d24b650a_size_168_quality_85_9517d921_20251014T204754Z.jpg)
*Axial T2WI MR in a patient who developed onset of dysarthria and upper extremity dysmetria 15 months following stereotaxic XRT for midbrain arteriovenous malformation shows mixed hyper-/hypointensity in the residual vascular malformation <img src='img/arrows/CO.png'/>.*
![Axial T2WI MR in the same patient shows bilateral inferior olivary hyperintensity and hypertrophy <img src='img/arrows/CS.png'/>.](images/app.statdx.com_image_thumbnail_81bec60a-3dee-42d5-bb77-cf3045ec6b06_size_168_quality_85_00d7b27e_20251014T204754Z.jpg)
*Axial T2WI MR in the same patient shows bilateral inferior olivary hyperintensity and hypertrophy <img src='img/arrows/CS.png'/>.*
![Axial T2WI MR (CISS) shows the normal shape of the medullary olives <img src='img/arrows/CS.png'/>.](7cd64580-3d32-4887-900a-30be8b06e436)
![Axial T2WI MR in the same patient shows bilateral inferior olivary hyperintensity and hypertrophy <img src='img/arrows/CS.png'/>.](images/app.statdx.com_image_thumbnail_81bec60a-3dee-42d5-bb77-cf3045ec6b06_size_168_quality_85_5f3ff6c1_20251014T204719Z.jpg)
*Axial T2WI MR in the same patient shows bilateral inferior olivary hyperintensity and hypertrophy <img src='img/arrows/CS.png'/>.*
![Axial T2WI MR (CISS) shows the normal shape of the medullary olives <img src='img/arrows/CS.png'/>.](images/app.statdx.com_image_thumbnail_7cd64580-3d32-4887-900a-30be8b06e436_size_168_quality_85_01ed2b3f_20251014T204719Z.jpg)
*Axial T2WI MR (CISS) shows the normal shape of the medullary olives <img src='img/arrows/CS.png'/>.*
![Axial T2WI MR in a patient who developed palatal myoclonus ~ 6 months after resection of a midbrain CM shows hyperintensity and enlargement of both olives <img src='img/arrows/CS.png'/>. This pattern is typical in the subacute stage of HOD, which typically appears between 6 months and 3-4 years after injury to the dentato-rubro-olivary pathway.](842f79ca-da5e-4b88-a632-22de84d9f8df)
![Axial T2WI MR (CISS) shows the normal shape of the medullary olives <img src='img/arrows/CS.png'/>.](images/app.statdx.com_image_thumbnail_7cd64580-3d32-4887-900a-30be8b06e436_size_168_quality_85_e8047204_20251014T204754Z.jpg)
*Axial T2WI MR (CISS) shows the normal shape of the medullary olives <img src='img/arrows/CS.png'/>.*
![Axial T2WI MR in a patient who developed palatal myoclonus ~ 6 months after resection of a midbrain CM shows hyperintensity and enlargement of both olives <img src='img/arrows/CS.png'/>. This pattern is typical in the subacute stage of HOD, which typically appears between 6 months and 3-4 years after injury to the dentato-rubro-olivary pathway.](images/app.statdx.com_image_thumbnail_842f79ca-da5e-4b88-a632-22de84d9f8df_size_168_quality_85_a834833e_20251014T204719Z.jpg)
*Axial T2WI MR in a patient who developed palatal myoclonus ~ 6 months after resection of a midbrain CM shows hyperintensity and enlargement of both olives <img src='img/arrows/CS.png'/>. This pattern is typical in the subacute stage of HOD, which typically appears between 6 months and 3-4 years after injury to the dentato-rubro-olivary pathway.*
![Axial SWI MR demonstrates hemosiderin staining in the dorsal aspect of the brainstem <img src='img/arrows/CC.png'/> in the midline and to the right due to an old hemorrhage.](2c2646c5-4793-4f2e-9d9e-6600ed9bfc6f)
![Axial T2WI MR in a patient who developed palatal myoclonus ~ 6 months after resection of a midbrain CM shows hyperintensity and enlargement of both olives <img src='img/arrows/CS.png'/>. This pattern is typical in the subacute stage of HOD, which typically appears between 6 months and 3-4 years after injury to the dentato-rubro-olivary pathway.](images/app.statdx.com_image_thumbnail_842f79ca-da5e-4b88-a632-22de84d9f8df_size_168_quality_85_c66c4ac0_20251014T204754Z.jpg)
*Axial T2WI MR in a patient who developed palatal myoclonus ~ 6 months after resection of a midbrain CM shows hyperintensity and enlargement of both olives <img src='img/arrows/CS.png'/>. This pattern is typical in the subacute stage of HOD, which typically appears between 6 months and 3-4 years after injury to the dentato-rubro-olivary pathway.*
![Axial SWI MR demonstrates hemosiderin staining in the dorsal aspect of the brainstem <img src='img/arrows/CC.png'/> in the midline and to the right due to an old hemorrhage.](images/app.statdx.com_image_thumbnail_2c2646c5-4793-4f2e-9d9e-6600ed9bfc6f_size_168_quality_85_6d72ebd6_20251014T204754Z.jpg)
*Axial SWI MR demonstrates hemosiderin staining in the dorsal aspect of the brainstem <img src='img/arrows/CC.png'/> in the midline and to the right due to an old hemorrhage.*
![Axial FLAIR MR in the same patient at the level of the medulla shows mild hypertrophy with hyperintensity in the region of the right inferior olivary nucleus <img src='img/arrows/CS.png'/>. Findings are typical for HOD caused by primary lesions in dentato-rubro-olivary pathway (anatomical GMT).](f5a3dae8-1682-4b14-a65e-db91929e2ca2)
![Axial SWI MR demonstrates hemosiderin staining in the dorsal aspect of the brainstem <img src='img/arrows/CC.png'/> in the midline and to the right due to an old hemorrhage.](images/app.statdx.com_image_thumbnail_2c2646c5-4793-4f2e-9d9e-6600ed9bfc6f_size_168_quality_85_f6de8b6b_20251014T204719Z.jpg)
*Axial SWI MR demonstrates hemosiderin staining in the dorsal aspect of the brainstem <img src='img/arrows/CC.png'/> in the midline and to the right due to an old hemorrhage.*
![Axial FLAIR MR in the same patient at the level of the medulla shows mild hypertrophy with hyperintensity in the region of the right inferior olivary nucleus <img src='img/arrows/CS.png'/>. Findings are typical for HOD caused by primary lesions in dentato-rubro-olivary pathway (anatomical GMT).](images/app.statdx.com_image_thumbnail_f5a3dae8-1682-4b14-a65e-db91929e2ca2_size_168_quality_85_8c890454_20251014T204719Z.jpg)
*Axial FLAIR MR in the same patient at the level of the medulla shows mild hypertrophy with hyperintensity in the region of the right inferior olivary nucleus <img src='img/arrows/CS.png'/>. Findings are typical for HOD caused by primary lesions in dentato-rubro-olivary pathway (anatomical GMT).*
![Axial T2WI MR through the medulla shows that the ipsilateral olive is atrophic and hyperintense <img src='img/arrows/CS.png'/>. This patient also has crossed cerebellar atrophy <img src='img/arrows/CO.png'/> due to interruption of the ponto-cerebellar pathway.](a47e6de5-44e1-4f0f-9855-8278718b020f)
![Axial FLAIR MR in the same patient at the level of the medulla shows mild hypertrophy with hyperintensity in the region of the right inferior olivary nucleus <img src='img/arrows/CS.png'/>. Findings are typical for HOD caused by primary lesions in dentato-rubro-olivary pathway (anatomical GMT).](images/app.statdx.com_image_thumbnail_f5a3dae8-1682-4b14-a65e-db91929e2ca2_size_168_quality_85_d9562458_20251014T204754Z.jpg)
*Axial FLAIR MR in the same patient at the level of the medulla shows mild hypertrophy with hyperintensity in the region of the right inferior olivary nucleus <img src='img/arrows/CS.png'/>. Findings are typical for HOD caused by primary lesions in dentato-rubro-olivary pathway (anatomical GMT).*
![Axial T2WI MR through the medulla shows that the ipsilateral olive is atrophic and hyperintense <img src='img/arrows/CS.png'/>. This patient also has crossed cerebellar atrophy <img src='img/arrows/CO.png'/> due to interruption of the ponto-cerebellar pathway.](images/app.statdx.com_image_thumbnail_a47e6de5-44e1-4f0f-9855-8278718b020f_size_168_quality_85_9d9e41c9_20251014T204719Z.jpg)
*Axial T2WI MR through the medulla shows that the ipsilateral olive is atrophic and hyperintense <img src='img/arrows/CS.png'/>. This patient also has crossed cerebellar atrophy <img src='img/arrows/CO.png'/> due to interruption of the ponto-cerebellar pathway.*
![Axial T2WI MR in a patient who developed palatal myoclonus several months following midbrain surgery for CM. Imaging obtained 1 year later shows residual CM <img src='img/arrows/CO.png'/>.](47d78ac6-02eb-4674-bfda-316586ec456d)
![Axial T2WI MR through the medulla shows that the ipsilateral olive is atrophic and hyperintense <img src='img/arrows/CS.png'/>. This patient also has crossed cerebellar atrophy <img src='img/arrows/CO.png'/> due to interruption of the ponto-cerebellar pathway.](images/app.statdx.com_image_thumbnail_a47e6de5-44e1-4f0f-9855-8278718b020f_size_168_quality_85_f4dc3265_20251014T204754Z.jpg)
*Axial T2WI MR through the medulla shows that the ipsilateral olive is atrophic and hyperintense <img src='img/arrows/CS.png'/>. This patient also has crossed cerebellar atrophy <img src='img/arrows/CO.png'/> due to interruption of the ponto-cerebellar pathway.*
![Axial T2WI MR in a patient who developed palatal myoclonus several months following midbrain surgery for CM. Imaging obtained 1 year later shows residual CM <img src='img/arrows/CO.png'/>.](images/app.statdx.com_image_thumbnail_47d78ac6-02eb-4674-bfda-316586ec456d_size_168_quality_85_0c26fca7_20251014T204754Z.jpg)
*Axial T2WI MR in a patient who developed palatal myoclonus several months following midbrain surgery for CM. Imaging obtained 1 year later shows residual CM <img src='img/arrows/CO.png'/>.*
![Axial FLAIR MR in the same patient delineates the somewhat wavy appearance of the hyperintensity conforming to the configuration of the olives <img src='img/arrows/CS.png'/>. The pyramids <img src='img/arrows/CO.png'/> are spared, helping differentiate HOD from perforating artery infarction.](24cc4357-0f7a-464d-be25-3f85a555f29e)
![Axial T2WI MR in a patient who developed palatal myoclonus several months following midbrain surgery for CM. Imaging obtained 1 year later shows residual CM <img src='img/arrows/CO.png'/>.](images/app.statdx.com_image_thumbnail_47d78ac6-02eb-4674-bfda-316586ec456d_size_168_quality_85_490d2382_20251014T204719Z.jpg)
*Axial T2WI MR in a patient who developed palatal myoclonus several months following midbrain surgery for CM. Imaging obtained 1 year later shows residual CM <img src='img/arrows/CO.png'/>.*
![Axial FLAIR MR in the same patient delineates the somewhat wavy appearance of the hyperintensity conforming to the configuration of the olives <img src='img/arrows/CS.png'/>. The pyramids <img src='img/arrows/CO.png'/> are spared, helping differentiate HOD from perforating artery infarction.](images/app.statdx.com_image_thumbnail_24cc4357-0f7a-464d-be25-3f85a555f29e_size_168_quality_85_24074433_20251014T204719Z.jpg)
*Axial FLAIR MR in the same patient delineates the somewhat wavy appearance of the hyperintensity conforming to the configuration of the olives <img src='img/arrows/CS.png'/>. The pyramids <img src='img/arrows/CO.png'/> are spared, helping differentiate HOD from perforating artery infarction.*
![Axial FLAIR MR in the same patient delineates the somewhat wavy appearance of the hyperintensity conforming to the configuration of the olives <img src='img/arrows/CS.png'/>. The pyramids <img src='img/arrows/CO.png'/> are spared, helping differentiate HOD from perforating artery infarction.](images/app.statdx.com_image_thumbnail_24cc4357-0f7a-464d-be25-3f85a555f29e_size_168_quality_85_c8eb04b6_20251014T204754Z.jpg)
*Axial FLAIR MR in the same patient delineates the somewhat wavy appearance of the hyperintensity conforming to the configuration of the olives <img src='img/arrows/CS.png'/>. The pyramids <img src='img/arrows/CO.png'/> are spared, helping differentiate HOD from perforating artery infarction.*
docs_md/articles/hypothalamic-hamartoma_7f85487f-9497-44a9-b884-b98e50d41018.md
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---
title: "Hypothalamic Hamartoma"
docid: "7f85487f-9497-44a9-b884-b98e50d41018"
authors:
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value: "Karen L. Salzman, MD, FACR"
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pageDescription: "Hypothalamic Hamartoma"
pageKeywords: "Brain, Diagnosis, Anatomy-Based Diagnoses, Sella and Pituitary, Congenital, Hypothalamic Hamartoma"
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---
# KEY FACTS
- ## Terminology
- a.k.a. tuber cinereum hamartoma
- Nonneoplastic; congenital gray matter heterotopia
- ## Imaging
- Hypothalamic mass contiguous with tuber cinereum
- Located between mammillary bodies and infundibulum
- Can be sessile or pedunculated ("collar button")
- Size ranges from few mm to several cm
- Isointense with gray matter on T1WI
- Can be slightly hyperintense on T2/FLAIR
- Large lesions can be heterogeneous, contain cysts
- No enhancement on T1 C+
- ## Top Differential Diagnoses
- Chiasmatic/hypothalamic astrocytoma
- Craniopharyngioma
- Ectopic posterior pituitary
- Lipoma
- Germinoma
- Langerhans cell histiocytosis
- ## Pathology
- Mature but dysplastic neuronal ganglionic tissue
- ## Clinical Issues
- Infant with epilepsy or precocious puberty
- Cognitive, neuropsychiatric comorbidities common
- Older children with precocious puberty
- Often tall, overweight, with advanced bone age
- Shape, size of hamartoma often predicts symptoms, presentation
- Large, sessile lesions → seizures
- Small, pedunculated lesions → central precocious puberty
- ## Diagnostic Checklist
- If hypothalamic mass in seizure imaging, think hypothalamic hamartoma; if enhancement present, consider astrocytoma
# TERMINOLOGY
- ## Synonyms
- Tuber cinereum hamartoma, diencephalic hamartoma
- ## Definitions
- Nonneoplastic congenital gray matter heterotopia in region of tuber cinereum of hypothalamus
# IMAGING
- ## General Features
- ### Best diagnostic clue
- Nonenhancing hypothalamic mass contiguous with tuber cinereum
- ### Location
- Tuber cinereum of hypothalamus
- Located between pons/mammillary bodies and hypothalamic infundibulum
- ### Size
- Variable, few mm to giant (3-5 cm)
- ### Morphology
- Sessile or pedunculated mass
- Similar in density/intensity to gray matter
- ## Radiographic Findings
- ### Radiography
- ± suprasellar calcifications, eroded dorsum, enlarged sella (rare)
- ## CT Findings
- ### NECT
- Homogeneous suprasellar mass
- Isodense → slightly hypodense
- Cysts and calcification are uncommon
- ± patent craniopharyngeal canal (very rare)
- ### CECT
- No pathologic enhancement
- ## MR Findings
- ### T1WI
- Mass located between mammillary bodies and infundibulum
- Isointense → slightly hypointense to gray matter
- ### T2WI
- Isointense → slightly hyperintense (secondary to fibrillary gliosis)
- ### PD/intermediate
- Hyperintense to CSF, slightly hyperintense to gray matter
- ### FLAIR
- Isointense → slightly hyperintense to gray matter
- ### T1WI C+
- Nonenhancing; if enhancing, consider other diagnosis
- ### MRS
- ↓ NAA and NAA/Cr, mild ↑ Cho and Cho/Cr, ↑ myoinositol (mI) and mI/Cr
- ↓ NAA and ↑ Cho indicate reduced neuronal density and relative gliosis, respectively, compared to normal gray matter
- ↑ mI/Cr correlates with ↑ glial component and lesion T2 hyperintensity
- ## Imaging Recommendations
- ### Best imaging tool
- Multiplanar MR imaging
- ### Protocol advice
- Thin-section sagittal and coronal T2W1, T1WI C+ MR
# DIFFERENTIAL DIAGNOSIS
- [Craniopharyngioma](/document/craniopharyngioma/00e66680-6731-4287-b5a1-3f0b3f09053b)
- Most common suprasellar mass in children
- Variable signal intensity cysts (90%), calcifications (90%), and enhancement (90%)
- Longstanding lesion, frequently with short stature and pituitary abnormalities
- ## Chiasmatic/Hypothalamic Astrocytoma
- 2nd most common pediatric suprasellar mass [± neurofibromatosis type 1 (NF1)]
- Hyperintense on T2WI MR ± contrast enhancement (heterogeneous, often vigorous)
- Optic pathway or hypothalamus ± optic tract extension
- ## Ectopic Posterior Pituitary
- Ectopic hyperintense focus on T1WI MR
- Often located along median eminence of hypothalamus
- No normal orthotopic posterior pituitary hyperintensity
- [Germinoma](/document/germinoma/078b68a2-67de-457e-818a-63655cec95aa)
- Thickening, abnormal enhancement of pituitary stalk rather than tuber cinereum
- Diabetes insipidus common
- ± multicentric: Suprasellar, pineal, thalamus, basal ganglia
- Early leptomeningeal metastatic dissemination
- [Langerhans Cell Histiocytosis](/document/langerhans-cell-histiocytosis-skul-/5bfd61b0-b320-46f4-b785-6c69daa8523c)
- Thickening, abnormal enhancement of pituitary stalk rather than tuber cinereum
- Diabetes insipidus common
- Look for lytic bone lesions in typical locations
- [Lipoma](/document/lipoma-brain/1bdb974e-8346-4730-9b1c-dea7b70b844d)
- Hyperintense fat signal on T1WI MR
- Hypointense on STIR or fat-saturated sequences
# PATHOLOGY
- ## General Features
- ### Etiology
- Neuronal migration anomaly (occurs between gestational days 33-41)
- Affects normal hypothalamic regulation of autonomic, endocrine, neurologic, behavioral functions
- Pathogenesis of precocious puberty-induced sexual precocity
- ± luteinizing hormone-releasing hormone (LHRH) granules in hamartoma/connecting axons in some
- Activating astroglial-derived factors in tumors may stimulate endogenous LHRH secretion if no intratumoral LHRH granules
- Shape and size of hamartoma postulated to predict symptoms
- Large, sessile lesions → seizures
- Small, pedunculated lesions → central precocious puberty (CPP)
- Presentation with both seizures and CPP common
- ### Genetics
- *GLI3*mutation
- Pallister-Hall syndrome (PHS)
- Hamartoma or hamartoblastoma of tuber cinereum; often large mass
- Digital malformations (short metacarpals, syndactyly, polydactyly)
- Other midline (epiglottis/larynx) and cardiac/renal/anal anomalies
- Greig cephalopolysyndactyly syndrome (GCPS)
- ## Staging, Grading, & Classification
- Valdueza classification
- Pedunculated, CPP or asymptomatic
- Originates in tuber cinereum
- Originates in mammillary bodies
- Sessile, hypothalamus displaced, seizures
- More hypothalamic dysfunction and abnormal behavior
- ## Gross Pathologic & Surgical Features
- Mature neuronal ganglionic tissue projecting from hypothalamus, tuber cinereum, or mammillary bodies
- Pedunculated or sessile, rounded or nodular
- ## Microscopic Features
- Well-differentiated neurons interspersed with glial cells, myelinated/unmyelinated axons, variable amounts of fibrillary gliosis
- Hamartoblastomas include primitive undifferentiated cells
- Rare reports of cysts, necrosis, calcifications, fat
# CLINICAL ISSUES
- ## Presentation
- ### Most common signs/symptoms
- Luteinizing hormone-releasing hormone (LHRH) dependent CPP presenting at very young age
- Refractory symptomatic mixed seizure types, including gelastic seizures
- Gelastic seizures are recurrent automatic bursts of laughter without mirth
- Presentation usually encompasses both epileptic seizures and encephalopathy with behavioral cognitive impairment
- May progress to partial epilepsy, partial complex seizures, generalized tonic clonic seizures
- Rarely occur in conjunction with focal cortical dysplasia or hypothalamic astrocytoma
- Other seizure types frequent with hypothalamic hamartoma (HH); always look for HH in child with epilepsy
- ### Other signs/symptoms
- Depression, anxiety common in adult HH patients
- ### Clinical profile
- Infant with gelastic seizures or precocious puberty
- Older children with precocious puberty; tall, overweight, and advanced bone age
- ## Demographics
- ### Age
- Usually present between 1-3 years of age
- ### Sex
- No predilection; some reports M > F
- ### Ethnicity
- No predilection
- ### Epidemiology
- Of histologically verified lesions, 3/4 have precocious puberty and 1/2 have seizures
- Up to 33% of patients with CPP have HH
- ## Natural History & Prognosis
- Size should remain stable; if growth is detected, surgery/biopsy is indicated
- Postsurgical hypothalamic complications include headache, mental slowing, and weight gain
- Symptomatic lesions: Sessile > > pedunculated
- Sessile lesions nearly always symptomatic
- Syndromic patients generally do poorly
- ## Treatment
- Medical: Hormonal-suppressive therapy, treat seizures
- Surgical: If medical therapy failure or rapid lesion growth
- Endoscopic or transcallosal surgical resection
- Recent studies have shown stereotactic laser ablation to have equivalent efficacy to open surgery with fewer complications
- Stereotactic radiosurgery and Gamma Knife surgery also potential options
- Newer, less invasive techniques include magnetic resonance imaging-guided laser interstitial thermal therapy (MRgLITT)
# DIAGNOSTIC CHECKLIST
- ## Consider
- If hypothalamic mass identified in seizure imaging, think HH
- ## Image Interpretation Pearls
- Classic = nonenhancing hypothalamic mass
- Isointense to gray matter on T1WI, slightly ↑ signal on T2WI/FLAIR
- Hypothalamic astrocytoma, Langerhans cell histiocytosis (LCH), germ cell tumor all show some contrast enhancement
1e636dc4-6e35-473c-a03d-2ed82f71d5bc
## References
# Selected References
1. [Bourdillon P et al: Surgical treatment of hypothalamic hamartomas. Neurosurg Rev. ePub, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=32318922%5Bpmid%5D)
1. [Gadgil N et al: Staged magnetic resonance-guided laser interstitial thermal therapy for hypothalamic hamartoma: analysis of ablation volumes and morphological considerations. Neurosurgery. 86(6):808-16, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=31990344%5Bpmid%5D)
1. [Roodakker KR et al: Ecstatic and gelastic seizures relate to the hypothalamus. Epilepsy Behav Rep. 14:100358, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=32368731%5Bpmid%5D)
1. [Wang S et al: Stereotactic radiofrequency thermocoagulation and resective surgery for patients with hypothalamic hamartoma. J Neurosurg. 1-8, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=32302977%5Bpmid%5D)
1. [Youngerman BE et al: Magnetic resonance imaging-guided laser interstitial thermal therapy for epilepsy: systematic review of technique, indications, and outcomes. Neurosurgery. 86(4):E366-82, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=31980831%5Bpmid%5D)
1. [Ferrand-Sorbets S et al: Seizure outcome and prognostic factors for surgical management of hypothalamic hamartomas in children. Seizure. 75:28-33, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31865135%5Bpmid%5D)
1. [Fujita A et al: Pathogenic variants of DYNC2H1, KIAA0556, and PTPN11 associated with hypothalamic hamartoma. Neurology. 93(3):e237-51, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31197031%5Bpmid%5D)
1. [Ler GYL et al: Teaching NeuroImages: hypothalamic hamartoma and polydactyly: think Pallister-Hall syndrome. Neurology. 93(23):e1016-7, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31792112%5Bpmid%5D)
1. [Roland JL et al: Recent advances in the neurosurgical treatment of pediatric epilepsy: JNSPG 75th Anniversary Invited Review Article J Neurosurg Pediatr. 23(4):411-21, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=30970205%5Bpmid%5D)
1. [Fayed I et al: MR-guided laser interstitial thermal therapy for medically refractory lesional epilepsy in pediatric patients: experience and outcomes. Pediatr Neurosurg. 53(5):322-9, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=30110689%5Bpmid%5D)
1. [Du VX et al: Laser interstitial thermal therapy: a first line treatment for seizures due to hypothalamic hamartoma? Epilepsia. 58 Suppl 2:77-84, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=28591480%5Bpmid%5D)
1. [Démurger F et al: New insights into genotype-phenotype correlation for GLI3 mutations. Eur J Hum Genet. 23(1):92-102, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=24736735%5Bpmid%5D)
1. [Wu J et al: Mechanisms of intrinsic epileptogenesis in human gelastic seizures with hypothalamic hamartoma. CNS Neurosci Ther. 21(2):104-11, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25495642%5Bpmid%5D)
1. [Li CD et al: Classification of hypothalamic hamartoma and prognostic factors for surgical outcome. Acta Neurol Scand. 130(1):18-26, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=24382157%5Bpmid%5D)
1. [Alves C et al: Giant hypothalamic hamartoma: case report and literature review. Childs Nerv Syst. 29(3):513-6, 2013](http://www.ncbi.nlm.nih.gov/pubmed/?term=23314738%5Bpmid%5D)
1. [Mittal S et al: Hypothalamic hamartomas. Part 1. Clinical, neuroimaging, and neurophysiological characteristics. Neurosurg Focus. 34(6):E6, 2013](http://www.ncbi.nlm.nih.gov/pubmed/?term=23724840%5Bpmid%5D)
1. [Pati S et al: Diagnosis and management of epilepsy associated with hypothalamic hamartoma: an evidence-based systematic review. J Child Neurol. 28(7):909-16, 2013](http://www.ncbi.nlm.nih.gov/pubmed/?term=23670254%5Bpmid%5D)
1. [Parvizi J et al: Gelastic epilepsy and hypothalamic hamartomas: neuroanatomical analysis of brain lesions in 100 patients. Brain. 134(Pt 10):2960-8, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=21975589%5Bpmid%5D)
1. [Beggs J et al: Hypothalamic hamartomas associated with epilepsy: ultrastructural features. J Neuropathol Exp Neurol. 67(7):657-68, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18596547%5Bpmid%5D)
1. [Kos S et al: Hydrometrocolpos, postaxial polydactyly, and hypothalamic hamartoma in a patient with confirmed Pallister-Hall syndrome: a clinical overlap with McKusick-Kaufman syndrome. Pediatr Radiol. 38(8):902-6, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18478223%5Bpmid%5D)
1. [Ng YT et al: Endoscopic resection of hypothalamic hamartomas for refractory symptomatic epilepsy. Neurology. 70(17):1543-8, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18427070%5Bpmid%5D)
1. [Ng YT: Clarification of the term "status gelasticus" and treatment and prognosis of gelastic seizures. Pediatr Neurol. 38(4):300-1; author reply 301-2, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18358415%5Bpmid%5D)
1. [Parent AS et al: Gene expression profiling of hypothalamic hamartomas: a search for genes associated with central precocious puberty. Horm Res. 69(2):114-23, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18059092%5Bpmid%5D)
1. [Pleasure SJ et al: Hypothalamic hamartomas and hedgehogs: not a laughing matter. Neurology. 70(8):588-9, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18285533%5Bpmid%5D)
1. [Prigatano GP et al: Intellectual functioning in presurgical patients with hypothalamic hamartoma and refractory epilepsy. Epilepsy Behav. 13(1):149-55, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18375185%5Bpmid%5D)
1. [Romanelli P et al: Radiosurgery for hypothalamic hamartomas. Neurosurg Focus. 24(5):E9, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18447748%5Bpmid%5D)
1. [Schulze-Bonhage A et al: Outcome and predictors of interstitial radiosurgery in the treatment of gelastic epilepsy. Neurology. 71(4):277-82, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18645166%5Bpmid%5D)
1. [Shahar E et al: Intractable gelastic seizures during infancy: ictal positron emission tomography (PET) demonstrating epileptiform activity within the hypothalamic hamartoma. J Child Neurol. 23(2):235-9, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18160558%5Bpmid%5D)
1. [Shim KW et al: Treatment modality for intractable epilepsy in hypothalamic hamartomatous lesions. Neurosurgery. 62(4):847-56; discussion 856, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18496191%5Bpmid%5D)
1. [Castori M et al: Reassessment of holoprosencephaly-diencephalic hamartoblastoma (HDH) association. Am J Med Genet A. 143(3):277-84, 2007](http://www.ncbi.nlm.nih.gov/pubmed/?term=17230485%5Bpmid%5D)
1. [Castro LH et al: Epilepsy syndromes associated with hypothalamic hamartomas. Seizure. 16(1):50-8, 2007](http://www.ncbi.nlm.nih.gov/pubmed/?term=17157037%5Bpmid%5D)
1. [Cheung CS et al: Gelastic seizures: not always hypothalamic hamartoma. Epileptic Disord. 9(4):453-8, 2007](http://www.ncbi.nlm.nih.gov/pubmed/?term=18077234%5Bpmid%5D)
1. [Coons SW et al: The histopathology of hypothalamic hamartomas: study of 57 cases. J Neuropathol Exp Neurol. 66(2):131-41, 2007](http://www.ncbi.nlm.nih.gov/pubmed/?term=17278998%5Bpmid%5D)
1. [Homma J et al: Stereotactic radiofrequency thermocoagulation for hypothalamic hamartoma with intractable gelastic seizures. Epilepsy Res. 76(1):15-21, 2007](http://www.ncbi.nlm.nih.gov/pubmed/?term=17643965%5Bpmid%5D)
1. [Kerrigan JF et al: Hypothalamic hamartoma and infantile spasms. Epilepsia. 48(1):89-95, 2007](http://www.ncbi.nlm.nih.gov/pubmed/?term=17241213%5Bpmid%5D)
1. [Régis J et al: Gamma knife surgery for epilepsy related to hypothalamic hamartomas. Semin Pediatr Neurol. 14(2):73-9, 2007](http://www.ncbi.nlm.nih.gov/pubmed/?term=17544950%5Bpmid%5D)
1. [Rosenfeld JV et al: Hypothalamic hamartoma treatment: surgical resection with the transcallosal approach. Semin Pediatr Neurol. 14(2):88-98, 2007](http://www.ncbi.nlm.nih.gov/pubmed/?term=17544952%5Bpmid%5D)
1. [Schulze-Bonhage A et al: Treatment options for gelastic epilepsy due to hypothalamic hamartoma: interstitial radiosurgery. Semin Pediatr Neurol. 14(2):80-7, 2007](http://www.ncbi.nlm.nih.gov/pubmed/?term=17544951%5Bpmid%5D)
1. [Shahar E et al: Pediatric-onset gelastic seizures: clinical data and outcome. Pediatr Neurol. 37(1):29-34, 2007](http://www.ncbi.nlm.nih.gov/pubmed/?term=17628219%5Bpmid%5D)
1. [Veendrick-Meekes MJ et al: Neuropsychiatric aspects of patients with hypothalamic hamartomas. Epilepsy Behav. 11(2):218-21, 2007](http://www.ncbi.nlm.nih.gov/pubmed/?term=17606410%5Bpmid%5D)
1. [Vieira TC et al: Central precocious puberty associated with pituitary duplication and midline defects. J Pediatr Endocrinol Metab. 20(10):1141-4, 2007](http://www.ncbi.nlm.nih.gov/pubmed/?term=18051933%5Bpmid%5D)
1. [Ali S et al: Psychiatric comorbidity in adult patients with hypothalamic hamartoma. Epilepsy Behav. 9(1):111-8, 2006](http://www.ncbi.nlm.nih.gov/pubmed/?term=16697263%5Bpmid%5D)
1. [Amstutz DR et al: Hypothalamic hamartomas: correlation of MR imaging and spectroscopic findings with tumor glial content. AJNR Am J Neuroradiol. 27(4):794-8, 2006](http://www.ncbi.nlm.nih.gov/pubmed/?term=16611766%5Bpmid%5D)
1. [Maixner W: Hypothalamic hamartomas--clinical, neuropathological and surgical aspects. Childs Nerv Syst. 22(8):867-73, 2006](http://www.ncbi.nlm.nih.gov/pubmed/?term=16763856%5Bpmid%5D)
1. [Procaccini E et al: Surgical management of hypothalamic hamartomas with epilepsy: the stereoendoscopic approach. Neurosurgery. 59(4 Suppl 2):ONS336-44; discussion ONS344-6, 2006](http://www.ncbi.nlm.nih.gov/pubmed/?term=17041502%5Bpmid%5D)
1. [Trivin C et al: Presentation and evolution of organic central precocious puberty according to the type of CNS lesion. Clin Endocrinol (Oxf). 65(2):239-45, 2006](http://www.ncbi.nlm.nih.gov/pubmed/?term=16886967%5Bpmid%5D)
1. [Boudreau EA et al: Hypothalamic hamartomas and seizures: distinct natural history of isolated and Pallister-Hall syndrome cases. Epilepsia. 46(1):42-7, 2005](http://www.ncbi.nlm.nih.gov/pubmed/?term=15660767%5Bpmid%5D)
1. [Kizilkilic O et al: Hypothalamic hamartoma associated with a craniopharyngeal canal. AJNR Am J Neuroradiol. 26(1):65-7, 2005](http://www.ncbi.nlm.nih.gov/pubmed/?term=15661703%5Bpmid%5D)
1. [Saxonhouse MA et al: Neonatal hypothalamic hamartoma: a differentiating nonlethal hamartoblastoma. J Neurosurg. 103(3 Suppl):277-81, 2005](http://www.ncbi.nlm.nih.gov/pubmed/?term=16238084%5Bpmid%5D)
1. [Striano S et al: The clinical spectrum and natural history of gelastic epilepsy-hypothalamic hamartoma syndrome. Seizure. 14(4):232-9, 2005](http://www.ncbi.nlm.nih.gov/pubmed/?term=15911357%5Bpmid%5D)
1. [Yamura M et al: Evaluation of small hypothalamic hamartomas with 3D constructive interference in steady state (CISS) sequence. Neuroradiology. 47(3):204-8, 2005](http://www.ncbi.nlm.nih.gov/pubmed/?term=15731910%5Bpmid%5D)
1. [Booth TN et al: Pre- and postnatal MR imaging of hypothalamic hamartomas associated with arachnoid cysts. AJNR Am J Neuroradiol. 25(7):1283-5, 2004](http://www.ncbi.nlm.nih.gov/pubmed/?term=15313725%5Bpmid%5D)
1. [Freeman JL et al: MR imaging and spectroscopic study of epileptogenic hypothalamic hamartomas: analysis of 72 cases. AJNR Am J Neuroradiol. 25(3):450-62, 2004](http://www.ncbi.nlm.nih.gov/pubmed/?term=15037472%5Bpmid%5D)
1. [Voyadzis JM et al: Hypothalamic hamartoma secreting corticotropin-releasing hormone. Case report. J Neurosurg. 100(2 Suppl Pediatrics):212-6, 2004](http://www.ncbi.nlm.nih.gov/pubmed/?term=14758953%5Bpmid%5D)
1. [Freeman JL: The anatomy and embryology of the hypothalamus in relation to hypothalamic hamartomas. Epileptic Disord. 5(4):177-86, 2003](http://www.ncbi.nlm.nih.gov/pubmed/?term=14975786%5Bpmid%5D)
1. [Mullatti N et al: The clinical spectrum of epilepsy in children and adults with hypothalamic hamartoma. Epilepsia. 44(10):1310-9, 2003](http://www.ncbi.nlm.nih.gov/pubmed/?term=14510825%5Bpmid%5D)
1. [Boyko OB et al: Hamartomas of the tuber cinereum: CT, MR, and pathologic findings. AJNR Am J Neuroradiol. 12(2):309-14, 1991](http://www.ncbi.nlm.nih.gov/pubmed/?term=1902033%5Bpmid%5D)
## Images
### Selected Images
![Sagittal graphic shows a classic pedunculated tuber cinereum hamartoma <img src='img/arrows/BS.png'/> interposed between the infundibulum anteriorly and the mammillary bodies posteriorly. The mass resembles gray matter.](images/app.statdx.com_image_thumbnail_3ea95519-17d0-4323-9eac-873b26fc71fc_size_168_quality_85_c6f76a0e_20251014T204851Z.jpg)
*Sagittal graphic shows a classic pedunculated tuber cinereum hamartoma <img src='img/arrows/BS.png'/> interposed between the infundibulum anteriorly and the mammillary bodies posteriorly. The mass resembles gray matter.*
![Sagittal T1WI MR in a 2-year-old girl with precocious puberty shows a large, pedunculated &quot;collar button&quot; hypothalamic hamartoma (HH) <img src='img/arrows/CO.png'/>. The lesion is in the classic location between the infundibular stalk <img src='img/arrows/WC.png'/> anteriorly and the mammillary bodies posteriorly.](images/app.statdx.com_image_thumbnail_93d04362-fa22-4e48-99ea-b1e34b4daa8b_size_168_quality_85_5bced641_20251014T204851Z.jpg)
*Sagittal T1WI MR in a 2-year-old girl with precocious puberty shows a large, pedunculated &quot;collar button&quot; hypothalamic hamartoma (HH) <img src='img/arrows/CO.png'/>. The lesion is in the classic location between the infundibular stalk <img src='img/arrows/WC.png'/> anteriorly and the mammillary bodies posteriorly.*
![Sagittal T2WI MR in a patient presenting with precocious puberty reveals a pedunculated hypothalamic mass <img src='img/arrows/CS.png'/> located between the median eminence and mammillary bodies <img src='img/arrows/WO.png'/>. The mass is isointense with cortex, typical of a hamartoma.](images/app.statdx.com_image_thumbnail_2707b78c-b251-4c96-b60d-d71b1d894e13_size_168_quality_85_c0d67f71_20251014T204851Z.jpg)
*Sagittal T2WI MR in a patient presenting with precocious puberty reveals a pedunculated hypothalamic mass <img src='img/arrows/CS.png'/> located between the median eminence and mammillary bodies <img src='img/arrows/WO.png'/>. The mass is isointense with cortex, typical of a hamartoma.*
![Sagittal T1WI C+ MR in the same patient shows the mass <img src='img/arrows/CS.png'/> does not enhance. This is a classic pedunculated HH. If the lesion enhanced, considerations would include hypothalamic astrocytoma, Langerhans cell histiocytosis, and germ cell tumor.](images/app.statdx.com_image_thumbnail_04f01b97-872f-4548-aed3-2691e9cc0e72_size_168_quality_85_884a84ae_20251014T204851Z.jpg)
*Sagittal T1WI C+ MR in the same patient shows the mass <img src='img/arrows/CS.png'/> does not enhance. This is a classic pedunculated HH. If the lesion enhanced, considerations would include hypothalamic astrocytoma, Langerhans cell histiocytosis, and germ cell tumor.*
![Sagittal T1WI MR in a 28-year-old woman being evaluated for seizures shows a well-delineated mass <img src='img/arrows/CS.png'/> that appears to lie within the 3rd ventricle, isointense to gray matter.](images/app.statdx.com_image_thumbnail_35de31ee-d3b9-4e51-8e14-fa9226c88a50_size_168_quality_85_e46e6944_20251014T204851Z.jpg)
*Sagittal T1WI MR in a 28-year-old woman being evaluated for seizures shows a well-delineated mass <img src='img/arrows/CS.png'/> that appears to lie within the 3rd ventricle, isointense to gray matter.*
![Coronal T2WI MR in the same patient shows the sessile mass <img src='img/arrows/CS.png'/> is located in the hypothalamus and remains isointense with gray matter. The mass projects into, but does not obstruct, the 3rd ventricle. Patients with sessile lesions are more likely to present with gelastic seizures.](images/app.statdx.com_image_thumbnail_b32f9b56-8d22-407a-8e1f-abd33d5f91c8_size_168_quality_85_b97ac491_20251014T204851Z.jpg)
*Coronal T2WI MR in the same patient shows the sessile mass <img src='img/arrows/CS.png'/> is located in the hypothalamus and remains isointense with gray matter. The mass projects into, but does not obstruct, the 3rd ventricle. Patients with sessile lesions are more likely to present with gelastic seizures.*
![Axial FLAIR MR in a 2 year old with a tuber cinereum hamartoma shows a large suprasellar mass <img src='img/arrows/CS.png'/> that is isointense to the brain parenchyma.](images/app.statdx.com_image_thumbnail_9a29b2e2-2c61-4add-b119-6bb0e3eb7274_size_168_quality_85_ec3fce4b_20251014T204851Z.jpg)
*Axial FLAIR MR in a 2 year old with a tuber cinereum hamartoma shows a large suprasellar mass <img src='img/arrows/CS.png'/> that is isointense to the brain parenchyma.*
![Sagittal T1WI MR in an 8-year-old girl with precocious puberty shows a classic pedunculated &quot;collar button&quot; HH <img src='img/arrows/CO.png'/>. The lesion is interposed between the infundibular stalk <img src='img/arrows/WC.png'/> anteriorly and the mammillary bodies <img src='img/arrows/CS.png'/> posteriorly. HHs are classically isointense with gray matter.](images/app.statdx.com_image_thumbnail_12cb821b-7bc2-4b4d-a0db-8a8f47b6107f_size_168_quality_85_b3eebe18_20251014T204851Z.jpg)
*Sagittal T1WI MR in an 8-year-old girl with precocious puberty shows a classic pedunculated &quot;collar button&quot; HH <img src='img/arrows/CO.png'/>. The lesion is interposed between the infundibular stalk <img src='img/arrows/WC.png'/> anteriorly and the mammillary bodies <img src='img/arrows/CS.png'/> posteriorly. HHs are classically isointense with gray matter.*
![Sagittal T1WI MR in a child with precocious puberty shows a large, lobulated suprasellar mass <img src='img/arrows/CS.png'/> that is isointense with cortex and clearly separate from the pituitary gland and optic chiasm. The mass is so large it displaces the brainstem posteriorly.](images/app.statdx.com_image_thumbnail_c04976fc-adb9-4f54-8f3e-e8a2d370873f_size_168_quality_85_b2c9de70_20251014T204851Z.jpg)
*Sagittal T1WI MR in a child with precocious puberty shows a large, lobulated suprasellar mass <img src='img/arrows/CS.png'/> that is isointense with cortex and clearly separate from the pituitary gland and optic chiasm. The mass is so large it displaces the brainstem posteriorly.*
![Axial T2WI MR in the same patient shows the mass is heterogeneously hyperintense compared to cortex. HHs can be huge, measuring up to several cm in diameter. A mass of dysplastic, disorganized gray matter was removed at surgery and was consistent with HH.](images/app.statdx.com_image_thumbnail_3e6109b1-8fef-4502-b0de-717614feace9_size_168_quality_85_72b76777_20251014T204851Z.jpg)
*Axial T2WI MR in the same patient shows the mass is heterogeneously hyperintense compared to cortex. HHs can be huge, measuring up to several cm in diameter. A mass of dysplastic, disorganized gray matter was removed at surgery and was consistent with HH.*
### Additional Images
![Sagittal T1 C+ MR shows a nonenhancing sessile mass <img src='img/arrows/CS.png'/> arising in the hypothalamus and projecting in the posterior suprasellar cistern.](images/app.statdx.com_image_thumbnail_3c8b0a2c-772f-4dc0-b5c4-60d65caf8d5a_size_168_quality_85_8e9c88ca_20251014T204851Z.jpg)
*Sagittal T1 C+ MR shows a nonenhancing sessile mass <img src='img/arrows/CS.png'/> arising in the hypothalamus and projecting in the posterior suprasellar cistern.*
![Sagittal T2WI MR demonstrates that the lesion <img src='img/arrows/CS.png'/> is slightly bright.](images/app.statdx.com_image_thumbnail_88e40c1c-0096-4042-83a7-1126d58f5b2e_size_168_quality_85_3fecc4f2_20251014T204851Z.jpg)
*Sagittal T2WI MR demonstrates that the lesion <img src='img/arrows/CS.png'/> is slightly bright.*
![Axial T1 C+ MR reveals nonenhancing hamartoma <img src='img/arrows/CS.png'/> in the suprasellar cistern.](images/app.statdx.com_image_thumbnail_f64eabcb-add9-4262-bd79-942adba10156_size_168_quality_85_714b0844_20251014T204851Z.jpg)
*Axial T1 C+ MR reveals nonenhancing hamartoma <img src='img/arrows/CS.png'/> in the suprasellar cistern.*
![Coronal T1 C+ MR shows a left-sided nonenhancing hamartoma <img src='img/arrows/CS.png'/> in the substance of the hypothalamus.](images/app.statdx.com_image_thumbnail_438b033e-e49e-4334-b5da-215dbbee0d64_size_168_quality_85_422403d4_20251014T204851Z.jpg)
*Coronal T1 C+ MR shows a left-sided nonenhancing hamartoma <img src='img/arrows/CS.png'/> in the substance of the hypothalamus.*
![Axial T2WI MR confirms that the mass <img src='img/arrows/CS.png'/> is isointense to gray matter.](images/app.statdx.com_image_thumbnail_0050b9a5-487e-4aa6-92c6-af4f4aa12322_size_168_quality_85_229bcb0f_20251014T204851Z.jpg)
*Axial T2WI MR confirms that the mass <img src='img/arrows/CS.png'/> is isointense to gray matter.*
![Sagittal T1WI MR demonstrates a huge tuber cinereum hamartoma.](images/app.statdx.com_image_thumbnail_312a84c2-11bd-4301-bd90-f1a90e7efa9e_size_168_quality_85_4d6967c6_20251014T204851Z.jpg)
*Sagittal T1WI MR demonstrates a huge tuber cinereum hamartoma.*
![Axial NECT shows large atypical tuber cinereum hamartoma containing a calcification <img src='img/arrows/CO.png'/>.](images/app.statdx.com_image_thumbnail_9e9b5d71-13e1-4078-b8a8-8735e67ad814_size_168_quality_85_6ca57b7b_20251014T204851Z.jpg)
*Axial NECT shows large atypical tuber cinereum hamartoma containing a calcification <img src='img/arrows/CO.png'/>.*
![Sagittal T1WI MR in a 36-year-old woman being evaluated for seizures shows a well-delineated mass <img src='img/arrows/CS.png'/> that appears to lie within the 3rd ventricle.](images/app.statdx.com_image_thumbnail_06073cbf-d35b-413b-bc9c-addb209b8cb6_size_168_quality_85_304f7f4d_20251014T204851Z.jpg)
*Sagittal T1WI MR in a 36-year-old woman being evaluated for seizures shows a well-delineated mass <img src='img/arrows/CS.png'/> that appears to lie within the 3rd ventricle.*
![Coronal thin-section IR shows the mass <img src='img/arrows/CS.png'/> is actually in the hypothalamus. The mass is clearly isointense with gray matter.](images/app.statdx.com_image_thumbnail_ad539b29-b6f1-4e90-9036-fb48a31d263d_size_168_quality_85_9b6f8fc1_20251014T204851Z.jpg)
*Coronal thin-section IR shows the mass <img src='img/arrows/CS.png'/> is actually in the hypothalamus. The mass is clearly isointense with gray matter.*
![Coronal T2WI MR in the same patient shows the mass <img src='img/arrows/CS.png'/> remains isointense with gray matter and elevates, but does not obstruct, the 3rd ventricle.](images/app.statdx.com_image_thumbnail_a5fd0631-9985-47c1-82ff-aa339d415ce0_size_168_quality_85_2c3caa00_20251014T204851Z.jpg)
*Coronal T2WI MR in the same patient shows the mass <img src='img/arrows/CS.png'/> remains isointense with gray matter and elevates, but does not obstruct, the 3rd ventricle.*
![Coronal FLAIR in the same patient shows the lesion <img src='img/arrows/CS.png'/> appears slightly hyperintense relative to cortex. The mass did not enhance following contrast administration. This is a classic sessile HH.](images/app.statdx.com_image_thumbnail_0f590796-dcc1-470f-92c0-71596ea230db_size_168_quality_85_64a784c1_20251014T204851Z.jpg)
*Coronal FLAIR in the same patient shows the lesion <img src='img/arrows/CS.png'/> appears slightly hyperintense relative to cortex. The mass did not enhance following contrast administration. This is a classic sessile HH.*
![Sagittal FIESTA MR in a 2 year old with seizures and an HH shows the relationship of the hypothalamic mass <img src='img/arrows/CO.png'/> with adjacent structures. FIESTA eliminates CSF flow artifact and provides high spatial resolution evaluation of suprasellar lesions with a CSF interface.](images/app.statdx.com_image_thumbnail_0e9d8d98-11d1-4384-9a6a-8b24fcfa4d48_size_168_quality_85_76f40e1f_20251014T204851Z.jpg)
*Sagittal FIESTA MR in a 2 year old with seizures and an HH shows the relationship of the hypothalamic mass <img src='img/arrows/CO.png'/> with adjacent structures. FIESTA eliminates CSF flow artifact and provides high spatial resolution evaluation of suprasellar lesions with a CSF interface.*
docs_md/articles/hypothalamus-lesion_0fc29bff-9f20-4e19-a436-04c7791e3972.md
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---
title: "Hypothalamus Lesion"
docid: "0fc29bff-9f20-4e19-a436-04c7791e3972"
authors:
- key: "318f80ab-6abb-4067-a809-2ebdaa5a30c9"
value: "Kalen Riley, MD, MBA"
- key: "f184750a-90b4-47a7-907b-23b05d70357a"
value: "Chang Yueh Ho, MD"
- key: "5cff4116-3654-4b3a-bb75-5ebe0b8c9850"
value: "Anne G. Osborn, MD, FACR"
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name: "Brain"
slug: "brain"
treeNodeId: "6d8829f1-14d7-45af-8675-255189aa526a"
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name: "Differential Diagnosis"
slug: "differential-diagnosis"
treeNodeId: "a7fdd139-664e-4bb8-8d18-400e4733ff60"
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name: "Sella/Juxtasellar, Pineal Region"
slug: "sellajuxtasellar-pineal-region"
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name: "Hypothalamus Lesion"
slug: "hypothalamus-lesion"
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category: "Brain"
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lastUpdated: "03/15/23"
pageDescription: "Hypothalamus Lesion"
pageKeywords: "Brain, Differential Diagnosis, Sella/Juxtasellar, Pineal Region, Anatomically Based Differentials, Hypothalamus Lesion"
pageTitle: "Hypothalamus Lesion | STATdx"
enhancedTitle: "Hypothalamus Lesion"
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breadcrumbs:
- "Brain"
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- "Sella/Juxtasellar, Pineal Region"
- "Anatomically Based Differentials"
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---
# ESSENTIAL INFORMATION
- ## Key Differential Diagnosis Issues
- Anatomic essentials
- Hypothalamus lies below level of anterior, posterior commissures
- Inferior hypothalamus formed by
- Anterior recesses of 3rd ventricle
- Tuber cinereum
- Mammillary bodies
- Infundibular stalk
- ## Helpful Clues for Common Diagnoses
- **Astrocytoma**
- Most common primary neoplasm arising from hypothalamic-optic chiasm structures
- Usually low grade (pilocytic astrocytoma WHO grade1)
- Age < 5 years
- Endocrine dysfunction in 20%
- Look for evidence for neurofibromatosis type 1
- 20-50% of patients with pilocytic astrocytoma
- **Craniopharyngioma**
- **Adamantinomatous**
- Most common suprasellar mass in children
- Occurs anywhere from intrasellar to stalk to anteroinferior 3rd ventricle
- 90% calcify, 90% have multiple cysts (mixed signal intensity), 90% calcify
- **Papillary**
- Occurs along hypothalamic-pituitary axis, often infundibulum and tuber cinereum of 3rd ventricle floor
- Often solid, may be cystic and solid
- ## Helpful Clues for Less Common Diagnoses
- **Germ Cell Tumor**
- Can be primary in hypothalamus/stalk
- M = F (vs. male predominance in pineal gland)
- 10% "double" midline lesions (pineal and hypothalamus)
- Diabetes insipidus, diencephalic syndrome, precocious puberty common
- Thick, enhancing stalk, 3rd floor, absent posterior pituitary bright spot
- Pure germinoma demonstrates more homogeneous enhancement with rapid response to chemotherapy
- Intermediate and mixed germ cell tumors are more heterogeneous without rapid treatment response
- Mature teratomas may have calcification, fat, and other differentiated cell types (hair, teeth, etc.)
- **Neurosarcoid**
- Adult with stalk, meningeal lesions
- Other infectious/inflammatory lesions that can mimic sarcoid
- Granulomatosis with polyangiitis (GPA)
- Tuberculosis, syphilis
- **Langerhans Cell Histiocytosis**
- Stalk/hypothalamus lesion in child
- **Lipoma**
- Lipoma: Sessile T1-hyperintense lesion on subpial surface of hypothalamus
- Osteolipoma: Rare; fat-density/signal intensity and calcification
- **Lymphocytic****H****y****pophysitis**
- Peripartum female common; other etiologies include granulomatous, Ig-G4-related, drug-related, etc.
- Can mimic macroadenoma
- **Metastases**
- **Hypothalamic-pituitary axis metastases**
- 1-25% of systemic cancers at autopsy
- Less common at imaging
- Breast, lung most common primary tumors
- **Lymphoma**
- Pituitary/stalk/hypothalamus uncommon site
- Can be primary or metastatic
- **Tuber****C****inereum Hamartoma**
- Children with gelastic seizures, males with isosexual precocious puberty
- Can be pedunculated or sessile
- Density/signal intensity typically isointense with cortex
- No calcification, enhancement
- Sessile lesion may be difficult to distinguish from hypothalamic astrocytoma (no change on follow-up)
- **Ectopic Posterior Pituitary**
- Failure of neurohypophysis to migrate from hypothalamus
- T1-hyperintense lesion anywhere from hypothalamus through stalk
- Associated with septo-optic dysplasia
- **Dermoid Cyst**
- Inclusion of surface ectoderm with neural tube closure
- Includes fat: Fat-suppression MR techniques and negative HU on CT
- May rupture with leptomeningeal fat deposits
- Can cause chemical meningitis
- No fat with decreased diffusion = epidermoid
- **Rathke Cleft Cyst**
- Like craniopharyngioma, arises from squamous cell rests migrating through craniopharyngeal duct, forming sellar/suprasellar mass
- Nonenhancing cysts with central protein: Variable T1 hyperintensity and T2 hypointensity
- When large, can involve hypothalamus
- ## Helpful Clues for Rare Diagnoses
- **Other****G****liomas**
- **Chordoid g****lioma**
- Floor of 3rd ventricle
- Hyperintense with strong, uniform enhancement
- **Pilomyxoid a****strocytoma**
- Infant/young child
- H-shaped tumor of hypothalamus; extension into medial temporal lobes common
- Often large, bulky ± hemorrhage (rare in pilocytic astrocytoma)
- WHO grade1, subtype of pilocytic astrocytoma
- **Pituicytoma**
- Stalk, posterior pituitary lobe
- Low-grade astrocytoma
- Enhances strongly, uniformly
- **Ganglioglioma**
- Very rare in hypothalamus/chiasm
- Young adult (mean age = 20 years)
- **Wernicke Encephalopathy**
- Acute: Abnormal hyperintensity/enhancement of mammillary bodies, inferolateral walls of 3rd ventricle, periaqueductal gray matter
- Chronic: Mammillary atrophy
- Note: Occurs in both alcoholics, nonalcoholics (e.g., longstanding parenteral nutrition, hyperemesis, bariatric surgery)
- **Demyelinating****D****isease**
- Optic chiasm involvement > > hypothalamus
- Enhancing, slightly enlarged optic nerves/chiasm seen with both multiple sclerosis, ADEM
## References
# Selected References
1. [Pascual JM et al: Duct-like recess in the infundibular portion of third ventricle craniopharyngiomas: an MRI sign identifying the papillary type. AJNR Am J Neuroradiol. 43(9):1333-40, 2022](http://www.ncbi.nlm.nih.gov/pubmed/?term=35953277%5Bpmid%5D)
1. [Tierney TS et al: Initial experience with magnetic resonance-guided focused ultrasound stereotactic surgery for central brain lesions in young adults. J Neurosurg. 1-8, 2022](http://www.ncbi.nlm.nih.gov/pubmed/?term=35171812%5Bpmid%5D)
1. [Shields R et al: Magnetic resonance imaging of sellar and juxtasellar abnormalities in the paediatric population: an imaging review. Insights Imaging. 6(2):241-60, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25794595%5Bpmid%5D)
1. [Zhang Y et al: Hypothalamus syndrome in opticospinal multiple sclerosis. AJNR Am J Neuroradiol. 32(8):E153-5, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=21051517%5Bpmid%5D)
1. [Hamilton BE et al: Anatomic and pathologic spectrum of pituitary infundibulum lesions. AJR Am J Roentgenol. 188(3):W223-32, 2007](http://www.ncbi.nlm.nih.gov/pubmed/?term=17312027%5Bpmid%5D)
1. [Saleem SN et al: Lesions of the hypothalamus: MR imaging diagnostic features. Radiographics. 27(4):1087-108, 2007](http://www.ncbi.nlm.nih.gov/pubmed/?term=17620469%5Bpmid%5D)
## Images
### Selected Images
![Axial T2 MR shows a T2-hyperintense mass <img src='img/arrows/CS.png'/> centered in the hypothalamus and optic chiasm with associated cysts <img src='img/arrows/CO.png'/>. This was a pilocytic astrocytoma at resection. Low-grade astrocytomas typically have T2-hyperintense solid components.](images/app.statdx.com_image_thumbnail_05e9ecd8-142c-4ed8-9f8f-0848cc8337d9_annotated_true_size_900_quality_90_2a281ef1.jpg)
**Astrocytoma**
*Axial T2 MR shows a T2-hyperintense mass <img src='img/arrows/CS.png'/> centered in the hypothalamus and optic chiasm with associated cysts <img src='img/arrows/CO.png'/>. This was a pilocytic astrocytoma at resection. Low-grade astrocytomas typically have T2-hyperintense solid components.*
![Axial T2 MR shows a T2-hyperintense mass <img src='img/arrows/CS.png'/> centered in the hypothalamus and optic chiasm with associated cysts <img src='img/arrows/CO.png'/>. This was a pilocytic astrocytoma at resection. Low-grade astrocytomas typically have T2-hyperintense solid components.](images/app.statdx.com_image_thumbnail_05e9ecd8-142c-4ed8-9f8f-0848cc8337d9_size_174_quality_85_4c7b12ad.jpg)
**Astrocytoma**
*Axial T2 MR shows a T2-hyperintense mass <img src='img/arrows/CS.png'/> centered in the hypothalamus and optic chiasm with associated cysts <img src='img/arrows/CO.png'/>. This was a pilocytic astrocytoma at resection. Low-grade astrocytomas typically have T2-hyperintense solid components.*
![Sagittal T1 C+ MR shows a lobular, intensely enhancing suprasellar mass involving the hypothalamus and optic chiasm <img src='img/arrows/CO.png'/>. A nonenhancing cyst <img src='img/arrows/CS.png'/> is seen anteriorly to the mass. This was a pilocytic astrocytoma, WHO grade 1 at resection.](images/app.statdx.com_image_thumbnail_d7cd41c7-9f98-42c1-a9c7-cef3f4d8da82_annotated_true_size_900_quality_90_54be4a42.jpg)
**Astrocytoma**
*Sagittal T1 C+ MR shows a lobular, intensely enhancing suprasellar mass involving the hypothalamus and optic chiasm <img src='img/arrows/CO.png'/>. A nonenhancing cyst <img src='img/arrows/CS.png'/> is seen anteriorly to the mass. This was a pilocytic astrocytoma, WHO grade 1 at resection.*
![Sagittal NECT shows a cystic mass <img src='img/arrows/CS.png'/> with a solid and densely calcified component <img src='img/arrows/CO.png'/> in the sella of this child. The mass involves the entire hypothalamic pituitary axis and extends into the 3rd ventricle.](images/app.statdx.com_image_thumbnail_cda7e009-9633-45b9-b5fe-5d02acb1e12b_annotated_true_size_900_quality_90_86b09026.jpg)
**Craniopharyngioma**
*Sagittal NECT shows a cystic mass <img src='img/arrows/CS.png'/> with a solid and densely calcified component <img src='img/arrows/CO.png'/> in the sella of this child. The mass involves the entire hypothalamic pituitary axis and extends into the 3rd ventricle.*
![Sagittal T1 C+ MR shows rim enhancement <img src='img/arrows/CS.png'/> of the cystic component of the suprasellar mass with heterogeneous enhancement of the solid component in the sella <img src='img/arrows/CO.png'/>. The solid portion showed dense calcification on a prior CT scan, consistent with adamantinomatous craniopharyngioma.](images/app.statdx.com_image_thumbnail_4738c212-17e9-43a9-9c2f-4d054f22c562_annotated_true_size_900_quality_90_336b2fef.jpg)
**Craniopharyngioma**
*Sagittal T1 C+ MR shows rim enhancement <img src='img/arrows/CS.png'/> of the cystic component of the suprasellar mass with heterogeneous enhancement of the solid component in the sella <img src='img/arrows/CO.png'/>. The solid portion showed dense calcification on a prior CT scan, consistent with adamantinomatous craniopharyngioma.*
![Coronal T1 C+ FS MR shows a lobular, enhancing mass <img src='img/arrows/CS.png'/> centered in the hypothalamus and pituitary stalk. This was a germinoma at surgery. Suprasellar and pineal midline locations are the most common location for intracranial germinomas.](images/app.statdx.com_image_thumbnail_c41ad38b-6ffb-4793-b485-f2d62b6a8c49_annotated_true_size_900_quality_90_0e182843.jpg)
**Germ Cell Tumor**
*Coronal T1 C+ FS MR shows a lobular, enhancing mass <img src='img/arrows/CS.png'/> centered in the hypothalamus and pituitary stalk. This was a germinoma at surgery. Suprasellar and pineal midline locations are the most common location for intracranial germinomas.*
![Sagittal T1 C+ FS MR shows heterogeneous pial and parenchymal enhancement of the hypothalamus, anterior 3rd ventricle, lateral ventricles, and rostrum of the corpus callosum <img src='img/arrows/CS.png'/>. In addition, there is leptomeningeal enhancement <img src='img/arrows/CO.png'/> seen in the midline sulci.](73261ea0-fb3d-4e63-abd0-f385fc9be2f8)
**Neurosarcoid**
*Sagittal T1 C+ FS MR shows heterogeneous pial and parenchymal enhancement of the hypothalamus, anterior 3rd ventricle, lateral ventricles, and rostrum of the corpus callosum <img src='img/arrows/CS.png'/>. In addition, there is leptomeningeal enhancement <img src='img/arrows/CO.png'/> seen in the midline sulci.*
![Sagittal T1 C+ FS MR shows a heterogeneously enhancing hypothalamic mass <img src='img/arrows/CS.png'/> in a child with known Langerhans cell histiocytosis (LCH). This significantly decreased in size after treatment. An absent posterior pituitary &quot;bright spot&quot; is common in LCH.](8fff7426-bd04-4330-8077-6a3bd0c1bd25)
**Langerhans Cell Histiocytosis**
*Sagittal T1 C+ FS MR shows a heterogeneously enhancing hypothalamic mass <img src='img/arrows/CS.png'/> in a child with known Langerhans cell histiocytosis (LCH). This significantly decreased in size after treatment. An absent posterior pituitary &quot;bright spot&quot; is common in LCH.*
![Sagittal T1 MR shows an intrinsically T1- hyperintense hypothalamic/suprasellar mass, consistent with lipoma <img src='img/arrows/CS.png'/>. Fat-saturated images (not shown) demonstrated complete suppression of hyperintense signal.](a39529ba-6f3f-4b50-a4d9-4ab6bf1aac42)
**Lipoma**
*Sagittal T1 MR shows an intrinsically T1- hyperintense hypothalamic/suprasellar mass, consistent with lipoma <img src='img/arrows/CS.png'/>. Fat-saturated images (not shown) demonstrated complete suppression of hyperintense signal.*
![Sagittal T1 C+ FS MR in a postpartum female with lymphocytic hypophysitis shows an enhancing, heterogeneous lesion involving the pituitary infundibulum and anterior pituitary gland <img src='img/arrows/CS.png'/>. There is reactive thickening of adjacent meninges <img src='img/arrows/CO.png'/>.](efdecbe2-7e09-4ab8-9d5b-4a6a8b3a0b8d)
**Lymphocytic Hypophysitis**
*Sagittal T1 C+ FS MR in a postpartum female with lymphocytic hypophysitis shows an enhancing, heterogeneous lesion involving the pituitary infundibulum and anterior pituitary gland <img src='img/arrows/CS.png'/>. There is reactive thickening of adjacent meninges <img src='img/arrows/CO.png'/>.*
![Sagittal T1 C+ FS MR shows a newly developed, avidly enhancing hypothalamic mass <img src='img/arrows/CS.png'/> in a patient with known metastatic genitourinary malignancy. Lung and breast cancer are the most common primary tumors to metastasize to the hypothalamic region.](de757b75-f35f-424a-adf6-7854bfc8c485)
**Metastases**
*Sagittal T1 C+ FS MR shows a newly developed, avidly enhancing hypothalamic mass <img src='img/arrows/CS.png'/> in a patient with known metastatic genitourinary malignancy. Lung and breast cancer are the most common primary tumors to metastasize to the hypothalamic region.*
![Sagittal T1 C+ MR shows a nonenhancing mass arising from the tuber cinereum <img src='img/arrows/CS.png'/>, which follows brain parenchyma in intensity. In a child with gelastic seizures, this is a hypothalamic hamartoma.](c5a4e3c6-3d82-457f-bac5-e9a67be49ed8)
**Tuber Cinereum Hamartoma**
*Sagittal T1 C+ MR shows a nonenhancing mass arising from the tuber cinereum <img src='img/arrows/CS.png'/>, which follows brain parenchyma in intensity. In a child with gelastic seizures, this is a hypothalamic hamartoma.*
![Coronal T1 MR shows a hyperintense lesion in the hypothalamus in this infant with hormonal abnormality. This is consistent with ectopic posterior pituitary <img src='img/arrows/CS.png'/>. Note the intact septum pellucidum <img src='img/arrows/CO.png'/>, which does not suggest septo-optic dysplasia. Ectopic posterior pituitary is often associated with septo-optic dysplasia.](d37cf870-62ea-44d4-a539-f786408a3528)
**Ectopic Posterior Pituitary**
*Coronal T1 MR shows a hyperintense lesion in the hypothalamus in this infant with hormonal abnormality. This is consistent with ectopic posterior pituitary <img src='img/arrows/CS.png'/>. Note the intact septum pellucidum <img src='img/arrows/CO.png'/>, which does not suggest septo-optic dysplasia. Ectopic posterior pituitary is often associated with septo-optic dysplasia.*
![Sagittal T1 MR shows a cystic lesion with T1 hyperintensity involving the sella and stalk <img src='img/arrows/CS.png'/>. This demonstrates loss of signal on fat saturation technique (not shown) and is consistent with a dermoid cyst confirmed at surgery. No leptomeningeal fatty deposits were seen to suggest rupture.](6669894b-3e81-46d8-8a51-9d5181bb67bd)
**Dermoid Cyst**
*Sagittal T1 MR shows a cystic lesion with T1 hyperintensity involving the sella and stalk <img src='img/arrows/CS.png'/>. This demonstrates loss of signal on fat saturation technique (not shown) and is consistent with a dermoid cyst confirmed at surgery. No leptomeningeal fatty deposits were seen to suggest rupture.*
![Axial T2 FS MR shows a unilocular cyst involving the stalk and hypothalamus with a fluid-fluid level <img src='img/arrows/CS.png'/>. This was a Rathke cleft cyst at surgery. Fluid levels in a Rathke cleft cyst may be from layering protein or recent hemorrhage.](8bc21a58-fb5a-4c18-8b54-41bb869d1869)
**Rathke Cleft Cyst**
*Axial T2 FS MR shows a unilocular cyst involving the stalk and hypothalamus with a fluid-fluid level <img src='img/arrows/CS.png'/>. This was a Rathke cleft cyst at surgery. Fluid levels in a Rathke cleft cyst may be from layering protein or recent hemorrhage.*
![Axial T1 C+ MR shows a heterogeneous, enhancing mass involving the chiasm, hypothalamus, and left optic tract <img src='img/arrows/CS.png'/>. A cyst <img src='img/arrows/CO.png'/> is seen associated with the mass with effacement of the left temporal horn <img src='img/arrows/CC.png'/>. This was a ganglioglioma at resection.](2fd549e0-7f95-4a18-b982-f1ce27f76245)
**Ganglioglioma**
*Axial T1 C+ MR shows a heterogeneous, enhancing mass involving the chiasm, hypothalamus, and left optic tract <img src='img/arrows/CS.png'/>. A cyst <img src='img/arrows/CO.png'/> is seen associated with the mass with effacement of the left temporal horn <img src='img/arrows/CC.png'/>. This was a ganglioglioma at resection.*
![Sagittal FLAIR MR shows T2 hyperintensity and swelling involving the ependymal margins at the foramen of Monro <img src='img/arrows/CS.png'/> and hypothalamus/optic chiasm <img src='img/arrows/CO.png'/>. The patient later developed myelitis and was diagnosed with neuromyelitis optica.](3c6d205d-cc2b-474b-94de-66fae6ea11d8)
**Demyelinating Disease**
*Sagittal FLAIR MR shows T2 hyperintensity and swelling involving the ependymal margins at the foramen of Monro <img src='img/arrows/CS.png'/> and hypothalamus/optic chiasm <img src='img/arrows/CO.png'/>. The patient later developed myelitis and was diagnosed with neuromyelitis optica.*
### Additional Images
![Sagittal T1 C+ MR shows a newly developed enhancing solid and cystic suprasellar and hypothalamic mass <img src='img/arrows/CS.png'/> in a patient with known metastatic lung cancer.](a966af20-cc22-4d8e-bb15-40c37442d2fe)
**Metastases**
*Sagittal T1 C+ MR shows a newly developed enhancing solid and cystic suprasellar and hypothalamic mass <img src='img/arrows/CS.png'/> in a patient with known metastatic lung cancer.*
![Sagittal T1 C+ MR shows an enhancing, thickened infundibular stalk <img src='img/arrows/WS.png'/> in patient with known systemic cancer. This was the only intracranial lesion identified.](94f50504-c494-4027-aa5e-bf37a9da78b2)
**Metastases**
*Sagittal T1 C+ MR shows an enhancing, thickened infundibular stalk <img src='img/arrows/WS.png'/> in patient with known systemic cancer. This was the only intracranial lesion identified.*
![Sagittal T1 MR shows a lobulated, hyperintense lesion <img src='img/arrows/WS.png'/> extending posteriorly along the hypothalamus from the tuber cinereum to the mammillary bodies.](950bdd82-0eda-4000-bd0c-74a41b4a1e36)
**Lipoma**
*Sagittal T1 MR shows a lobulated, hyperintense lesion <img src='img/arrows/WS.png'/> extending posteriorly along the hypothalamus from the tuber cinereum to the mammillary bodies.*
![Sagittal T1 C+ MR shows small pituitary fossa <img src='img/arrows/BO.png'/> with absent posterior pituitary &quot;bright spot&quot; and bulbous enlargement of infundibulum <img src='img/arrows/WC.png'/> at its origin from the hypothalamus.](695b77be-38c6-4643-bd02-b2cc74047d87)
**Ectopic Posterior Pituitary**
*Sagittal T1 C+ MR shows small pituitary fossa <img src='img/arrows/BO.png'/> with absent posterior pituitary &quot;bright spot&quot; and bulbous enlargement of infundibulum <img src='img/arrows/WC.png'/> at its origin from the hypothalamus.*
![Axial FLAIR MR shows multiple subcortical white matter hyperintensities <img src='img/arrows/WS.png'/> with hyperintense hypothalamus and optic tracts <img src='img/arrows/WO.png'/> in a child with ADEM.](8da64dd1-b711-403a-a9e1-abc258cd7523)
**Demyelinating Disease**
*Axial FLAIR MR shows multiple subcortical white matter hyperintensities <img src='img/arrows/WS.png'/> with hyperintense hypothalamus and optic tracts <img src='img/arrows/WO.png'/> in a child with ADEM.*
![Sagittal T2 MR shows classic pilocytic astrocytoma <img src='img/arrows/WS.png'/> originating from hypothalamus and optic chiasm. (Courtesy P. Rodriguez, MD).](images/app.statdx.com_image_thumbnail_515fc21f-f42d-409c-afc8-76fc1d6d4070_annotated_true_size_900_quality_90_b0c03410.jpg)
**Astrocytoma**
*Sagittal T2 MR shows classic pilocytic astrocytoma <img src='img/arrows/WS.png'/> originating from hypothalamus and optic chiasm. (Courtesy P. Rodriguez, MD).*
![Sagittal T1 C+ MR shows an inhomogeneously enhancing mass in the anterior 3rd ventricle, hypothalamus <img src='img/arrows/WS.png'/>.](images/app.statdx.com_image_thumbnail_d4da8508-123e-44f5-9394-817f216bb0dd_annotated_true_size_900_quality_90_96da8382.jpg)
**Astrocytoma**
*Sagittal T1 C+ MR shows an inhomogeneously enhancing mass in the anterior 3rd ventricle, hypothalamus <img src='img/arrows/WS.png'/>.*
![Sagittal T1 MR shows large, hyperintense craniopharyngioma originating from the 3rd ventricle <img src='img/arrows/BS.png'/> and hypothalamus. Note sparing of the suprasellar cistern <img src='img/arrows/BO.png'/>.](images/app.statdx.com_image_thumbnail_00698622-649b-49d5-a2cf-587247acab43_annotated_true_size_900_quality_90_6e54bad0.jpg)
**Craniopharyngioma**
*Sagittal T1 MR shows large, hyperintense craniopharyngioma originating from the 3rd ventricle <img src='img/arrows/BS.png'/> and hypothalamus. Note sparing of the suprasellar cistern <img src='img/arrows/BO.png'/>.*
![Sagittal T1 C+ MR in 13-year-old boy with central diabetes insipidus shows an enhancing mass in the anterior 3rd ventricle/hypothalamus <img src='img/arrows/WS.png'/> displacing the pituitary stalk <img src='img/arrows/WC.png'/> anteriorly.](707ec5e7-4e75-45fc-91a2-f5bebc3e98ad)
**Germ Cell Tumor**
*Sagittal T1 C+ MR in 13-year-old boy with central diabetes insipidus shows an enhancing mass in the anterior 3rd ventricle/hypothalamus <img src='img/arrows/WS.png'/> displacing the pituitary stalk <img src='img/arrows/WC.png'/> anteriorly.*
![Sagittal T1 C+ MR shows an enhancing mass infiltrating the hypothalamus <img src='img/arrows/WS.png'/> and infundibular stalk. The patient is an adult who presented with diabetes insipidus (DI).](1ad25312-dfdc-4eae-bb8f-ec4cb3f91874)
**Neurosarcoid**
*Sagittal T1 C+ MR shows an enhancing mass infiltrating the hypothalamus <img src='img/arrows/WS.png'/> and infundibular stalk. The patient is an adult who presented with diabetes insipidus (DI).*
![Sagittal T1 C+ MR in child with DI shows enhancing mass <img src='img/arrows/WS.png'/> infiltrating the hypothalamus, tuber cinereum, infundibular stalk, and pituitary gland.](e822f905-eff2-4b22-8231-04e49357f357)
**Langerhans Cell Histiocytosis**
*Sagittal T1 C+ MR in child with DI shows enhancing mass <img src='img/arrows/WS.png'/> infiltrating the hypothalamus, tuber cinereum, infundibular stalk, and pituitary gland.*
![Sagittal T1 C+ MR shows enhancing mass in anterior 3rd ventricle, hypothalamus <img src='img/arrows/WS.png'/>. The pituitary stalk <img src='img/arrows/WO.png'/> is slightly thickened.](55f14ad7-1168-45df-a768-38bb65ab25b4)
**Lymphocytic Hypophysitis**
*Sagittal T1 C+ MR shows enhancing mass in anterior 3rd ventricle, hypothalamus <img src='img/arrows/WS.png'/>. The pituitary stalk <img src='img/arrows/WO.png'/> is slightly thickened.*
![Sagittal T1 C+ FS MR shows pituitary <img src='img/arrows/WS.png'/>, hypothalamic <img src='img/arrows/WO.png'/> masses in this patient with proven B-cell lymphoma.](13dd8ba0-0338-411e-b549-ddf6b260c3d4)
**Lymphoma**
*Sagittal T1 C+ FS MR shows pituitary <img src='img/arrows/WS.png'/>, hypothalamic <img src='img/arrows/WO.png'/> masses in this patient with proven B-cell lymphoma.*
![Sagittal T2WI MR in a 12-year-old child with gelastic seizures shows a sessile hypothalamic mass <img src='img/arrows/WS.png'/> with a cyst <img src='img/arrows/WO.png'/>. No enhancement was seen on T1 C+ MR Variant cases may mimic astrocytoma.](0f44d562-1b8d-4194-817a-2bfdf12cc80b)
**Tuber Cinereum Hamartoma**
*Sagittal T2WI MR in a 12-year-old child with gelastic seizures shows a sessile hypothalamic mass <img src='img/arrows/WS.png'/> with a cyst <img src='img/arrows/WO.png'/>. No enhancement was seen on T1 C+ MR Variant cases may mimic astrocytoma.*
![Sagittal T1 MR in this 65-year-old patient shows an isointense hypothalamic/3rd ventricular mass <img src='img/arrows/WS.png'/> displacing and compressing optic chiasm <img src='img/arrows/WC.png'/>. Intense homogeneous enhancement was seen on T1 C+ study.](038aacce-b74b-4dc4-a99d-9500ba650cb7)
**Chordoid Glioma**
*Sagittal T1 MR in this 65-year-old patient shows an isointense hypothalamic/3rd ventricular mass <img src='img/arrows/WS.png'/> displacing and compressing optic chiasm <img src='img/arrows/WC.png'/>. Intense homogeneous enhancement was seen on T1 C+ study.*
![Sagittal T2 MR in a 3-year-old child with neurofibromatosis type 1 and diencephalic syndrome shows a large, hyperintense hypothalamic mass bulging into the anterior 3rd ventricle <img src='img/arrows/BS.png'/>.](82dcf44d-4527-458f-8f1a-40825822f3d8)
**Pilomyxoid Astrocytoma**
*Sagittal T2 MR in a 3-year-old child with neurofibromatosis type 1 and diencephalic syndrome shows a large, hyperintense hypothalamic mass bulging into the anterior 3rd ventricle <img src='img/arrows/BS.png'/>.*
![Sagittal T1 C+ MR in a 22-year-old woman with hypopituitarism shows a large, enhancing hypothalamic/infundibular stalk mass <img src='img/arrows/WC.png'/>.](77bec59a-d7f2-41a4-89c4-59cc60f1aa08)
**Pituicytoma**
*Sagittal T1 C+ MR in a 22-year-old woman with hypopituitarism shows a large, enhancing hypothalamic/infundibular stalk mass <img src='img/arrows/WC.png'/>.*
![Sagittal FLAIR MR shows multifocal hyperintensities along the callososeptal interface and in the hemispheric white matter as well as optic chiasm/hypothalamus <img src='img/arrows/BO.png'/>. This is a known patient with multiple sclerosis.](b003778f-f961-4af7-a1db-b858f2f54d44)
**Demyelinating Disease**
*Sagittal FLAIR MR shows multifocal hyperintensities along the callososeptal interface and in the hemispheric white matter as well as optic chiasm/hypothalamus <img src='img/arrows/BO.png'/>. This is a known patient with multiple sclerosis.*
![Axial FLAIR MR in a patient with longstanding hyperalimentation shows hyperintensity in mammillary bodies as well as periaqueductal gray matter <img src='img/arrows/WS.png'/>.](353868cf-f797-449b-b065-f35d8757d6b9)
**Wernicke Encephalopathy**
*Axial FLAIR MR in a patient with longstanding hyperalimentation shows hyperintensity in mammillary bodies as well as periaqueductal gray matter <img src='img/arrows/WS.png'/>.*
![Axial T2 FS MR with fat saturation shows a hypointense hypothalamic mass <img src='img/arrows/CS.png'/>, which was hyperintense on noncontrast T1 images (not shown). Loss of signal with fat saturation is compatible with fatty composition in this hypothalamic lipoma.](abd4645d-1afc-43a6-a353-4bfc1a3fe222)
**Lipoma**
*Axial T2 FS MR with fat saturation shows a hypointense hypothalamic mass <img src='img/arrows/CS.png'/>, which was hyperintense on noncontrast T1 images (not shown). Loss of signal with fat saturation is compatible with fatty composition in this hypothalamic lipoma.*
![Coronal T1 C+ FS MR shows multiple heterogeneous masses, consistent with metastases in a patient with known lung cancer. A specific metastatic lesion involves the hypothalamus <img src='img/arrows/CS.png'/>. There is edema and midline shift <img src='img/arrows/CO.png'/> from another metastatic mass (not shown).](02015ea3-14d9-42d7-9828-557c90ce1ffa)
**Metastases**
*Coronal T1 C+ FS MR shows multiple heterogeneous masses, consistent with metastases in a patient with known lung cancer. A specific metastatic lesion involves the hypothalamus <img src='img/arrows/CS.png'/>. There is edema and midline shift <img src='img/arrows/CO.png'/> from another metastatic mass (not shown).*
![Sagittal T1 C+ FS MR shows enhancing mass of the stalk and pituitary gland extending to the hypothalamus <img src='img/arrows/CS.png'/>. In a child with diabetes insipidus, this is consistent with Langerhans cell histiocytosis. There is an incidental pineal cyst <img src='img/arrows/CO.png'/>.](c8f20dbd-13ef-4948-94b4-01617752a1d7)
**Langerhans Cell Histiocytosis**
*Sagittal T1 C+ FS MR shows enhancing mass of the stalk and pituitary gland extending to the hypothalamus <img src='img/arrows/CS.png'/>. In a child with diabetes insipidus, this is consistent with Langerhans cell histiocytosis. There is an incidental pineal cyst <img src='img/arrows/CO.png'/>.*
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