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b/docs_md/articles/abnormal-shape-configuration-of-corpus-callosum_238ca32d-6bc6-4f5a-81b1-6601dd605856.md @@ -0,0 +1,421 @@ +--- +title: "Abnormal Shape/Configuration of Corpus Callosum" +docid: "238ca32d-6bc6-4f5a-81b1-6601dd605856" +authors: + - key: "47381de4-c9fd-4999-8dd0-1808cd72db6b" + value: "Luke L. Linscott, MD" +breadcrumbs: + - + name: "Pediatrics" + slug: "pediatrics" + treeNodeId: "a915965c-d436-44cf-ae65-2f22e7246ea4" + - + name: "Differential Diagnosis" + slug: "differential-diagnosis" + treeNodeId: "9e45b379-de58-40ea-b8b0-9877f7ebe994" + - + name: "Brain" + slug: "brain" + treeNodeId: "0ad2e6d5-5dc7-4db0-a2f9-de0cf62fd0de" + - + name: "Abnormal Shape/Configuration of Corpus Callosum" + slug: "abnormal-shapeconfiguration-of-cor-" + treeNodeId: null +category: "Pediatrics" +documentVersionId: "bea8db40-78a1-4745-a1f7-c18bbf5f3f30" +imageCount: 53 +lastUpdated: "08/16/19" +pageDescription: "Abnormal Shape/Configuration of Corpus Callosum" +pageKeywords: "Pediatrics, Differential Diagnosis, Brain, Abnormal Shape/Configuration of Corpus Callosum" +pageTitle: "Abnormal Shape/Configuration of Corpus Callosum | STATdx" +enhancedTitle: "Abnormal Shape/Configuration of Corpus Callosum" +type: "DDX" +references: true +breadcrumbs: + - "Pediatrics" + - "Differential Diagnosis" + - "Brain" + - "Abnormal Shape/Configuration of Corpus Callosum" +--- +# ESSENTIAL INFORMATION + +- ## Key Differential Diagnosis Issues + + + - Clinical features to consider + - Normal corpus callosum (CC) varies in thickness & shape + - Associated anomalies portend worse prognosis + - If not congenital anomaly, clinical history is crucial + - Prior surgical history, prematurity etc. + - Corpus callosotomy, shunt placement, endoscopic 3rd ventriculostomy + - Imaging features to consider + - Isolated callosal dysgenesis is not common → additional malformations in > 50% + - Malformations of cortical development + - Noncallosal midline anomalies + - Abnormal brainstem or cerebellum + - Look for parenchymal abnormalities to identify etiology + - White matter (WM) volume loss, prior infarction, diffuse axonal injury + - Ventricular abnormalities are common + - Colpocephaly → CC agenesis/dysgenesis, Chiari 2 + - Enlarged, angular ventricles → periventricular leukomalacia + - Modality considerations + - CT: Helpful to distinguish cellular vs. inflammatory + - ↑ density → cellular mass, hemorrhage + - ↓ density → edema, inflammation + - MR: Best spatial & contrast resolution + - Sagittal imaging is critical for evaluation of CC + - 3D acquisitions allow multiplanar reconstructions +- ## Helpful Clues for Common Diagnoses + + + - **Normal Variant** + - Immature CC is thin + - Gradually thickens with progressive myelination + - Size, shape, & thickness of normal CC vary + - Splenium & genu are largest parts of CC + - Normal narrowing at junction of body & splenium ("isthmus") + - Dorsal surface of fully developed, normally myelinated CC is often "wavy" + - **Thin Corpus Callosum** + - Many causes (congenital, acquired) + - All may result in focal or diffuse callosal thinning + - **Periventricular leukomalacia** + - Premature infant is at greatest risk + - Acute findings: US → ↑ periventricular WM echogenicity + - MR: Diffusion restriction, ↑ T1, ↓ T2 + - Subacute findings: Cavitation, periventricular cysts + - Chronic findings: ↓ volume of periventricular WM + - Thin posterior body & splenium are most common + - Ventricular enlargement shows angular margins + - **Hypoxic-ischemic encephalopathy** + - Loss of cerebral WM → thin CC + - May occur with profound or partial prolonged injury + - Profound: Often perirolandic → posterior body CC + - Partial prolonged: Watershed injury → entire CC + - **Chronic cerebral infarction** + - Axonal loss → focal/diffuse thinning of CC + - **Obstructive hydrocephalus** + - Look for obstructing lesion (e.g., tumor, aqueductal stenosis) or sequelae of prior hemorrhage/infection + - Acute: CC stretched, bowed upward + - Chronic: Thinned, irregular (sequela of CC impingement against falx & chronic WM injury from hydrocephalus) + - **Chemotherapy & radiation therapy** + - WM injury with volume loss + - e.g., chronic methotrexate toxicity + - **Postsurgical Defects** + - **Corpus c****allosotomy** + - Surgical disruption for intractable epilepsy + - Isolated callosotomy or part of functional hemispherotomy + - Often imaged in postoperative setting to detect residual neuronal connections across midline + - Best seen on sagittal or coronal MR + - **Ventricular drainage catheter tract** + - Small defect in paramidline CC + - Typically with overlying WM parenchymal tract & postoperative skull focus + - May see hypointense intracranial catheter ± hyperintense fluid-filled extracranial components + - **Endoscopic 3rd ventriculostomy** + - Small defect in CC represents scope tract, typically with overlying WM parenchymal tract & postoperative skull focus +- ## Helpful Clues for Less Common Diagnoses + + + - **Callosal Agenesis** + - Absent WM bridging cerebral hemispheres + - Absent septum pellucidum + - Absent cingulate gyrus with vertically oriented parasagittal sulci radiating to high-riding 3rd ventricle + - Lateral ventricles: Colpocephaly, upturned frontal horns + - Probst bundles (WM tracts that would have formed CC) lie along medial aspects of lateral ventricles + - **Primary Callosal Dysgenesis** + - Absence of 1 or all segments + - Rostrum & splenium are most likely to be deficient + - Remnants vary in size, shape, configuration + - Look for other associated malformations + - "Micro" CC: Small but well formed, often syndromic + - "Mega" CC: Megalencephalic (bulky WM) vs. small to normal brain (syndromic) + - **Chiari 2 Malformation** + - Constellation of intracranial findings secondary to open neural tube defect (e.g., myelomeningocele) + - Small posterior fossa, towering cerebellum that wraps around dorsal brainstem, small elongated 4th ventricle, vermian/tonsillar extension into upper cervical spine, "beaked" tectum, scalloped clivus + - Degree of callosal dysgenesis is highly variable + - Correlates with severity of hydrocephalus + - **Neoplasm** + - **Glioblastoma** + - Common in adults, uncommon in children + - "Butterfly" glioma crosses CC + - Central necrosis + thick, irregular rim enhancement + - **Lymphoma** + - NECT: Hyperdense + - Strong, uniform enhancement + - **Pericallosal Lipoma** + - 40-50% occur in interhemispheric fissure + - Almost always located in subarachnoid space; blood vessels & cranial nerves course through lipoma + - 2 morphologic types + - Bulky, mass-like ("tubulonodular" type) + - Thin, dorsal to body/splenium ("curvilinear" type) + - Midline lipomas may be part of more general midline developmental disorder; CC is often deficient + - **Neurofibromatosis Type 1 (NF1)** + - Patients with NF1 have ↑ CC volume + - Sometimes markedly so & qualitatively evident + - Nonenhancing lesions of NF1 can occur in CC + - If focal lesions of CC enhance, suggest low-grade tumor + - **Holoprosencephaly** + - Alobar + - CC absent + - "Pancake" anterior cerebral tissue + - Monoventricle with large dorsal "cyst" + - Semilobar + - Frontal lobe fusion/hypoplasia; caudate head fusion + - Splenium may be present + - Lobar + - Genu sometimes present; GM often crosses with genu + - Absent anterior midline falx & fissure + - Middle interhemispheric variant (a.k.a. syntelencephaly) + - Splenium & genu present, body deficient + - Middle CC body "dips" + - GM crosses midline in expected location of CC body + - ± bilateral perisylvian polymicrogyria +- ## Helpful Clues for Rare Diagnoses + + + - **Hypomyelination** + - Primary pathologic hypomyelination is rare + - e.g., Pelizaeus-Merzbacher, *TUBB4A* disorders + - **Inherited Metabolic Leukodystrophies** + - **Metachromatic leukodystrophy** + - Entire CC affected, genu & splenium worst + - **X-linked adrenoleukodystrophy** + - Most commonly involves splenium + - **Alexander disease** + - Frontal lobe & genu involvement + - **Krabbe disease** + - Central WM + deep gray nuclei (especially thalamus) + +## References + +# Selected References + +1. [Al-Hashim AH et al: Corpus callosum abnormalities: neuroradiological and clinical correlations. Dev Med Child Neurol. 58(5):475-84, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=26661037%5Bpmid%5D) +1. [Edwards TJ et al: Clinical, genetic and imaging findings identify new causes for corpus callosum development syndromes. Brain. 137(Pt 6):1579-1613, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=24477430%5Bpmid%5D) +1. [Battal B et al: Corpus callosum: normal imaging appearance, variants and pathologic conditions. J Med Imaging Radiat Oncol. 54(6):541-9, 2010](http://www.ncbi.nlm.nih.gov/pubmed/?term=21199431%5Bpmid%5D) +1. [Bourekas EC et al: Lesions of the corpus callosum: MR imaging and differential considerations in adults and children. AJR Am J Roentgenol. 179(1):251-7, 2002](http://www.ncbi.nlm.nih.gov/pubmed/?term=12076946%5Bpmid%5D) + + +## Images + + +### Selected Images + +![Midline sagittal T1 MR in a normal term neonate shows a thin, unmyelinated corpus callosum (CC) . The CC will gradually thicken as it myelinates from posterior to anterior. Note that the entire pituitary gland normally shows T1 shortening in the 1st few weeks of life.](images/app.statdx.com_image_thumbnail_770c0ebd-7c6f-4197-a62b-704c6b7d6bb6_annotated_true_size_900_quality_90_56dafce6_20251018T142218Z.jpg) +**Normal Variant** +*Midline sagittal T1 MR in a normal term neonate shows a thin, unmyelinated corpus callosum (CC) . The CC will gradually thicken as it myelinates from posterior to anterior. Note that the entire pituitary gland normally shows T1 shortening in the 1st few weeks of life.* + +![Midline sagittal T1 MR in a normal term neonate shows a thin, unmyelinated corpus callosum (CC) . The CC will gradually thicken as it myelinates from posterior to anterior. Note that the entire pituitary gland normally shows T1 shortening in the 1st few weeks of life.](images/app.statdx.com_image_thumbnail_770c0ebd-7c6f-4197-a62b-704c6b7d6bb6_size_174_quality_85_d61e2530_20251018T125015Z.jpg) +**Normal Variant** +*Midline sagittal T1 MR in a normal term neonate shows a thin, unmyelinated corpus callosum (CC) . The CC will gradually thicken as it myelinates from posterior to anterior. Note that the entire pituitary gland normally shows T1 shortening in the 1st few weeks of life.* + +![Midline sagittal T1 MR in a 13 year old with headaches shows a normal variant morphology of the CC with relative thinning of the posterior body . This should not be mistaken for a sign of white matter (WM) volume loss.](images/app.statdx.com_image_thumbnail_259efe0c-d735-48aa-a8a4-102adb752c04_annotated_true_size_900_quality_90_44e855c1_20251018T142218Z.jpg) +**Normal Variant** +*Midline sagittal T1 MR in a 13 year old with headaches shows a normal variant morphology of the CC with relative thinning of the posterior body . This should not be mistaken for a sign of white matter (WM) volume loss.* + +![Axial FLAIR MR in a 7 year old with a history of prematurity & periventricular leukomalacia (PVL) shows severe WM volume loss with relatively little signal abnormality. Also note the angular margins of the expanded ventricular occipital horns, consistent with PVL related to extreme prematurity.](images/app.statdx.com_image_thumbnail_fdffb43b-acf7-4645-ae14-03f4bc8c461c_annotated_true_size_900_quality_90_a9dd954a_20251018T142218Z.jpg) +**Periventricular Leukomalacia** +*Axial FLAIR MR in a 7 year old with a history of prematurity & periventricular leukomalacia (PVL) shows severe WM volume loss with relatively little signal abnormality. Also note the angular margins of the expanded ventricular occipital horns, consistent with PVL related to extreme prematurity.* + +![Midline sagittal T1 MR in the same patient shows marked thinning of the posterior body & splenium of the CC due to WM volume loss. This is the most common area of CC involvement in PVL.](images/app.statdx.com_image_thumbnail_419e3589-55cb-4187-abe4-34701b078398_annotated_true_size_900_quality_90_521cfe60_20251018T142218Z.jpg) +**Periventricular Leukomalacia** +*Midline sagittal T1 MR in the same patient shows marked thinning of the posterior body & splenium of the CC due to WM volume loss. This is the most common area of CC involvement in PVL.* + +![Axial T2 MR in a 9 year old with a history of hypoxic-ischemic encephalopathy (HIE) at birth shows extensive gliosis & encephalomalacia causing WM volume & signal abnormality in a watershed distribution . This results in marked CC thinning.](images/app.statdx.com_image_thumbnail_601e0a1e-2d56-4c42-8215-e2d7a9357c1c_annotated_true_size_900_quality_90_0ed7b3c3_20251018T142240Z.jpg) +**Hypoxic-Ischemic Encephalopathy** +*Axial T2 MR in a 9 year old with a history of hypoxic-ischemic encephalopathy (HIE) at birth shows extensive gliosis & encephalomalacia causing WM volume & signal abnormality in a watershed distribution . This results in marked CC thinning.* + +![Midline sagittal T1 MR in the same patient shows marked thinning of the CC secondary to WM loss as a consequence of the remote HIE injury.](images/app.statdx.com_image_thumbnail_62a0bdfd-2c15-4578-8548-30dbd6278948_annotated_true_size_900_quality_90_665f8b2a_20251018T142240Z.jpg) +**Hypoxic-Ischemic Encephalopathy** +*Midline sagittal T1 MR in the same patient shows marked thinning of the CC secondary to WM loss as a consequence of the remote HIE injury.* + +![Midline sagittal T2 MR in a neonate with posthemorrhagic hydrocephalus shows a stretched & thinned CC . Note the enlarged lateral , 3rd , & 4th ventricles as well as thin T2 hypointensity along the brainstem, consistent with hemosiderin deposition.](images/app.statdx.com_image_thumbnail_a124c2db-747c-4ab8-885e-95a07831790d_annotated_true_size_900_quality_90_967e57cd_20251018T142240Z.jpg) +**Obstructive Hydrocephalus** +*Midline sagittal T2 MR in a neonate with posthemorrhagic hydrocephalus shows a stretched & thinned CC . Note the enlarged lateral , 3rd , & 4th ventricles as well as thin T2 hypointensity along the brainstem, consistent with hemosiderin deposition.* + +![Midline sagittal T1 MR in the same patient 1 year after shunting shows a thinned & dysmorphic CC as well as numerous thin, pencil-like gyri (stenogyria) .](images/app.statdx.com_image_thumbnail_2f23b513-918a-4b07-b08f-a03f25edaae2_annotated_true_size_900_quality_90_cd311d46_20251018T142240Z.jpg) +**Obstructive Hydrocephalus** +*Midline sagittal T1 MR in the same patient 1 year after shunting shows a thinned & dysmorphic CC as well as numerous thin, pencil-like gyri (stenogyria) .* + +![Coronal FLAIR MR shows changes of a left functional hemispherotomy with a WM disconnection & insular decortication . Corpus callosotomy may be performed in isolation or as part of a more extensive functional hemispherotomy, as in this patient.](images/app.statdx.com_image_thumbnail_b0668126-045a-4375-a13d-8336f6f18cc8_annotated_true_size_900_quality_90_ef6279e6_20251018T142242Z.jpg) +**Corpus Callosotomy** +*Coronal FLAIR MR shows changes of a left functional hemispherotomy with a WM disconnection & insular decortication . Corpus callosotomy may be performed in isolation or as part of a more extensive functional hemispherotomy, as in this patient.* + +![Coronal T2 MR shows absence of the midline CC with persistent paramidline callosal tissue , consistent with an isolated surgical callosotomy.](images/app.statdx.com_image_thumbnail_62167fbf-3d3c-4c29-b456-b457c903d157_annotated_true_size_900_quality_90_27e4bdc5_20251018T142242Z.jpg) +**Corpus Callosotomy** +*Coronal T2 MR shows absence of the midline CC with persistent paramidline callosal tissue , consistent with an isolated surgical callosotomy.* + +![Paramidline sagittal T1 MR in a 12 year old with Chiari 2 malformation shows a ventricular shunt catheter tract in the anterior body of the CC. Note the caudal migration of the cerebellum & brainstem , consistent with Chiari 2.](images/app.statdx.com_image_thumbnail_5b3bafb7-d57f-4111-bd8b-1a122c74d4e2_annotated_true_size_900_quality_90_9b2efa1d_20251018T142242Z.jpg) +**Ventricular Drainage Catheter Tract** +*Paramidline sagittal T1 MR in a 12 year old with Chiari 2 malformation shows a ventricular shunt catheter tract in the anterior body of the CC. Note the caudal migration of the cerebellum & brainstem , consistent with Chiari 2.* + +![Paramidline sagittal T2 MR in a teenager with a history of a prior endoscopic 3rd ventriculostomy shows a linear defect in the parasagittal body of the CC. The defect represents the site of surgical access for the scope to enter the 3rd ventricle.](images/app.statdx.com_image_thumbnail_dd083e9e-beac-4e49-8c01-df6dcb4e4e72_annotated_true_size_900_quality_90_e58b8787_20251018T142242Z.jpg) +**Endoscopic 3rd Ventriculostomy** +*Paramidline sagittal T2 MR in a teenager with a history of a prior endoscopic 3rd ventriculostomy shows a linear defect in the parasagittal body of the CC. The defect represents the site of surgical access for the scope to enter the 3rd ventricle.* + +![Coronal T2 MR in a 4 year old with callosal agenesis shows widely spaced upturned lateral ventricular frontal horns , a high-riding 3rd ventricle , & bilateral Probst bundles . Also note the extensive periventricular gray matter (GM) heterotopia .](images/app.statdx.com_image_thumbnail_82c23417-cc0d-47b9-a72c-9253390c8669_annotated_true_size_900_quality_90_7081e987_20251018T142242Z.jpg) +**Callosal Agenesis** +*Coronal T2 MR in a 4 year old with callosal agenesis shows widely spaced upturned lateral ventricular frontal horns , a high-riding 3rd ventricle , & bilateral Probst bundles . Also note the extensive periventricular gray matter (GM) heterotopia .* + +![Midline sagittal T1 MR in a 5 month old with isolated callosal dysgenesis shows a very short & thin CC with no evident rostrum or splenium. Isolated callosal dysgenesis is uncommon. Associated anomalies should be carefully sought.](images/app.statdx.com_image_thumbnail_5ba333fc-5752-4158-9879-eb9fba5607f7_annotated_true_size_900_quality_90_67a96337_20251018T142242Z.jpg) +**Callosal Dysgenesis** +*Midline sagittal T1 MR in a 5 month old with isolated callosal dysgenesis shows a very short & thin CC with no evident rostrum or splenium. Isolated callosal dysgenesis is uncommon. Associated anomalies should be carefully sought.* + +![Midline sagittal T1 MR in a 7 year old with multiple anomalies shows a short, thin, & dysmorphic CC with a poorly formed splenium & rostrum .](images/app.statdx.com_image_thumbnail_f86d2115-e3b9-421e-ad82-19c308b0bbe4_annotated_true_size_900_quality_90_ba2a85fb_20251018T142242Z.jpg) +**Callosal Dysgenesis** +*Midline sagittal T1 MR in a 7 year old with multiple anomalies shows a short, thin, & dysmorphic CC with a poorly formed splenium & rostrum .* + +![Midline sagittal T2 MR in a child with a repaired myelomeningocele & Chiari 2 malformation (with beaked tectum , small 4th ventricle , & scalloped clivus ) shows a thinned & dysmorphic CC .](images/app.statdx.com_image_thumbnail_acee92fd-3247-482d-97d6-df3fe96c1863_annotated_true_size_900_quality_90_246ff2ac_20251018T142242Z.jpg) +**Chiari 2 Malformation** +*Midline sagittal T2 MR in a child with a repaired myelomeningocele & Chiari 2 malformation (with beaked tectum , small 4th ventricle , & scalloped clivus ) shows a thinned & dysmorphic CC .* + +![Coronal T2 MR in a 10 year old with glioblastoma shows mass-like infiltrative signal crossing the midline through an expanded CC. Infiltrative high-grade glial neoplasms should be considered whenever such a finding is encountered, as they commonly spread along WM tracts, such as the CC.](images/app.statdx.com_image_thumbnail_d7178b25-99a0-48e5-b5d1-4ba745e31ffd_annotated_true_size_900_quality_90_c0b535a4_20251018T142242Z.jpg) +**Glioblastoma** +*Coronal T2 MR in a 10 year old with glioblastoma shows mass-like infiltrative signal crossing the midline through an expanded CC. Infiltrative high-grade glial neoplasms should be considered whenever such a finding is encountered, as they commonly spread along WM tracts, such as the CC.* + +![Midline sagittal T2 MR shows expansion & increased signal in the rostrum & anterior genu of the CC , consistent with tumor infiltration/edema in this patient with CNS lymphoma.](images/app.statdx.com_image_thumbnail_4e887fcd-b71a-4ea0-9a32-a0c92831bd3c_annotated_true_size_900_quality_90_3b5f0c6f_20251018T142242Z.jpg) +**Lymphoma** +*Midline sagittal T2 MR shows expansion & increased signal in the rostrum & anterior genu of the CC , consistent with tumor infiltration/edema in this patient with CNS lymphoma.* + +![Midline sagittal T1 MR in a 4 month old shows a T1-hyperintense lipoma along the dorsal CC with associated absence of the splenium .](images/app.statdx.com_image_thumbnail_254a4187-4098-4461-a132-5f320ed115cc_annotated_true_size_900_quality_90_4d84f230_20251018T142242Z.jpg) +**Pericallosal Lipoma** +*Midline sagittal T1 MR in a 4 month old shows a T1-hyperintense lipoma along the dorsal CC with associated absence of the splenium .* + +![Midline sagittal T1 MR in a 15 year old with neurofibromatosis type 1 (NF1) shows diffuse marked thickening of the entire CC, a finding that can be seen in NF1. Look for associated findings of NF1, such as nonenhancing signal abnormalities of the globus pallidus & medial cerebellum, optic pathway gliomas, & plexiform neurofibromas.](images/app.statdx.com_image_thumbnail_2cdae2ee-21a2-4d37-86b8-ec8c63300332_annotated_true_size_900_quality_90_def17346_20251018T142242Z.jpg) +**Neurofibromatosis Type 1** +*Midline sagittal T1 MR in a 15 year old with neurofibromatosis type 1 (NF1) shows diffuse marked thickening of the entire CC, a finding that can be seen in NF1. Look for associated findings of NF1, such as nonenhancing signal abnormalities of the globus pallidus & medial cerebellum, optic pathway gliomas, & plexiform neurofibromas.* + +![Midline sagittal T2 MR shows absence of the CC in a patient with alobar holoprosencephaly. There is continuity of frontal WM & GM across the midline with a large dorsal cyst that communicates with a monoventricle . Note the lack of a vermian primary fissure due to associated rhombencephalosynapsis.](images/app.statdx.com_image_thumbnail_87401e50-17f7-4c3a-a13d-25585cf8587c_annotated_true_size_900_quality_90_3fda2aba_20251018T142242Z.jpg) +**Holoprosencephaly** +*Midline sagittal T2 MR shows absence of the CC in a patient with alobar holoprosencephaly. There is continuity of frontal WM & GM across the midline with a large dorsal cyst that communicates with a monoventricle . Note the lack of a vermian primary fissure due to associated rhombencephalosynapsis.* + +![Midline sagittal T1 MR in a 2 year old with semilobar holoprosencephaly shows absence of a normal CC & extension of cortical GM across the midline.](images/app.statdx.com_image_thumbnail_26c5225d-e501-4ac2-8b64-dcad9220c0cc_annotated_true_size_900_quality_90_9af45eb7_20251018T142242Z.jpg) +**Holoprosencephaly** +*Midline sagittal T1 MR in a 2 year old with semilobar holoprosencephaly shows absence of a normal CC & extension of cortical GM across the midline.* + +![Midline sagittal T1 MR in a teenager with the middle interhemispheric variant of holoprosencephaly shows an intact CC anteriorly & posteriorly but abnormal extension of GM across the midline in the expected location of the CC body. The abnormal body of the CC typically "dips" down toward the interthalamic adhesion.](images/app.statdx.com_image_thumbnail_f06b5e0c-4fa2-4141-90e9-b3ff276df084_annotated_true_size_900_quality_90_02851778_20251018T142242Z.jpg) +**Holoprosencephaly** +*Midline sagittal T1 MR in a teenager with the middle interhemispheric variant of holoprosencephaly shows an intact CC anteriorly & posteriorly but abnormal extension of GM across the midline in the expected location of the CC body. The abnormal body of the CC typically "dips" down toward the interthalamic adhesion.* + +![Coronal T2 MR in the same patient with syntelencephaly shows abnormal GM crossing the midline along the CC WM . Also note the azygous internal carotid artery (ICA) .](images/app.statdx.com_image_thumbnail_45dda608-de0b-4b0c-b752-7a53664c3cf2_annotated_true_size_900_quality_90_5f982aeb_20251018T142242Z.jpg) +**Holoprosencephaly** +*Coronal T2 MR in the same patient with syntelencephaly shows abnormal GM crossing the midline along the CC WM . Also note the azygous internal carotid artery (ICA) .* + +![Coronal T2 MR in a 13-year-old female patient with metachromatic leukodystrophy shows symmetric extensive WM signal abnormality with preservation of the subcortical WM . Note the marked thinning of the CC .](images/app.statdx.com_image_thumbnail_ab751550-37f4-4c06-8aa3-704d77ade93f_annotated_true_size_900_quality_90_edf1566f_20251018T142242Z.jpg) +**Metachromatic Leukodystrophy** +*Coronal T2 MR in a 13-year-old female patient with metachromatic leukodystrophy shows symmetric extensive WM signal abnormality with preservation of the subcortical WM . Note the marked thinning of the CC .* + +![Axial FLAIR MR in a 14-year-old male patient with X-linked adrenoleukodystrophy (ALD) shows symmetric increased FLAIR signal intensity that crosses the splenium of the CC. This is the most common distribution of signal abnormality in X-linked ALD.](images/app.statdx.com_image_thumbnail_5b506ba1-f44e-4b24-bd43-d4b66ddd0f74_annotated_true_size_900_quality_90_51994411_20251018T142242Z.jpg) +**X-Linked Adrenoleukodystrophy** +*Axial FLAIR MR in a 14-year-old male patient with X-linked adrenoleukodystrophy (ALD) shows symmetric increased FLAIR signal intensity that crosses the splenium of the CC. This is the most common distribution of signal abnormality in X-linked ALD.* + + +### Additional Images + +![Midline sagittal 3D SSFP MR with a close-up view of the corpus callosum shows normal "wavy" dorsal surface. Note the focal thinning along the posterior body , a common normal finding.](images/app.statdx.com_image_thumbnail_4febdaa6-7169-4512-a441-b7ab601c6290_annotated_true_size_900_quality_90_98e8fade_20251018T142218Z.jpg) +**Normal Variant** +*Midline sagittal 3D SSFP MR with a close-up view of the corpus callosum shows normal "wavy" dorsal surface. Note the focal thinning along the posterior body , a common normal finding.* + +![Midline sagittal T1 MR shows a normal neonatal corpus callosum , thin due to an age-appropriate lack of myelin. The cingulate gyrus is normal.](images/app.statdx.com_image_thumbnail_2148930a-f910-449f-8f04-9b59c8919068_annotated_true_size_900_quality_90_4e19bec0_20251018T142218Z.jpg) +**Normal Variant** +*Midline sagittal T1 MR shows a normal neonatal corpus callosum , thin due to an age-appropriate lack of myelin. The cingulate gyrus is normal.* + +![Midline sagittal T1 MR shows diffuse thinning of the posterior corpus callosum , greater than typically seen. The thinning of the corpus callosum is secondary to loss of commissural fibers, damaged by periventricular leukomalacia.](images/app.statdx.com_image_thumbnail_46f727cc-b7d9-4ffc-a993-b5616685d0c1_annotated_true_size_900_quality_90_3e8787d9_20251018T142218Z.jpg) +**Periventricular Leukomalacia** +*Midline sagittal T1 MR shows diffuse thinning of the posterior corpus callosum , greater than typically seen. The thinning of the corpus callosum is secondary to loss of commissural fibers, damaged by periventricular leukomalacia.* + +![Axial T2 MR in the same child shows marked loss of the right periventricular parenchyma at the site of a prior grade 4 hemorrhage. The posterior white matter loss correlates with the focal corpus callosum atrophy .](images/app.statdx.com_image_thumbnail_702f029e-c743-4233-a437-58f7445a29ce_annotated_true_size_900_quality_90_7e055645_20251018T142218Z.jpg) +**Periventricular Leukomalacia** +*Axial T2 MR in the same child shows marked loss of the right periventricular parenchyma at the site of a prior grade 4 hemorrhage. The posterior white matter loss correlates with the focal corpus callosum atrophy .* + +![Midline sagittal T1 MR shows marked callosal thinning in a child whose hydrocephalus follows unilateral grade 4 intraventricular hemorrhage. Note the more severe callosal volume loss posteriorly .](images/app.statdx.com_image_thumbnail_8d204964-2869-4537-b851-89072fefa22b_annotated_true_size_900_quality_90_08fdf3d7_20251018T142240Z.jpg) +**Periventricular Leukomalacia** +*Midline sagittal T1 MR shows marked callosal thinning in a child whose hydrocephalus follows unilateral grade 4 intraventricular hemorrhage. Note the more severe callosal volume loss posteriorly .* + +![Midline sagittal T1 MR shows thinning of the body & splenium of the corpus callosum following neonatal parietooccipital ischemia & gliosis from a combination of hypoxic ischemic encephalopathy & hypoglycemia.](images/app.statdx.com_image_thumbnail_3122fe3d-7f2a-4446-b348-8f14efa4f4a2_annotated_true_size_900_quality_90_5c9ae416_20251018T142240Z.jpg) +**Chronic Cerebral Infarction** +*Midline sagittal T1 MR shows thinning of the body & splenium of the corpus callosum following neonatal parietooccipital ischemia & gliosis from a combination of hypoxic ischemic encephalopathy & hypoglycemia.* + +![Coronal T2 MR shows parietal ulegyria & marked thinning of the posterior corpus callosum .](images/app.statdx.com_image_thumbnail_efe29b1b-0a22-4a79-a164-306b4f9d09cc_annotated_true_size_900_quality_90_a66d8937_20251018T142240Z.jpg) +**Chronic Cerebral Infarction** +*Coronal T2 MR shows parietal ulegyria & marked thinning of the posterior corpus callosum .* + +![Midline sagittal T2 MR shows mild stretching & thinning of the corpus callosum due to hydrocephalus. There is obstruction of the aqueduct of Sylvius by a tectal glioma .](images/app.statdx.com_image_thumbnail_554904c6-222f-4f20-b05e-3581543671af_annotated_true_size_900_quality_90_878a7aa0_20251018T142240Z.jpg) +**Obstructive Hydrocephalus** +*Midline sagittal T2 MR shows mild stretching & thinning of the corpus callosum due to hydrocephalus. There is obstruction of the aqueduct of Sylvius by a tectal glioma .* + +![Coronal FLAIR MR shows thinning & gliosis of the corpus callosum & surrounding white matter following therapy for acute lymphoblastic leukemia (ALL).](b0aae959-3335-40ec-abef-68abd744cfdb) +**Chemotherapy & Radiation Therapy** +*Coronal FLAIR MR shows thinning & gliosis of the corpus callosum & surrounding white matter following therapy for acute lymphoblastic leukemia (ALL).* + +![Midline sagittal T1 MR shows a focal defect at the junction of the genu & body of the corpus callosum , which had been the site of a prior surgical approach to this child's suprasellar tumor .](images/app.statdx.com_image_thumbnail_45d959b2-20ac-493b-a132-b5a1c2354b55_annotated_true_size_900_quality_90_0e22670a_20251018T142242Z.jpg) +**Postsurgical Defects** +*Midline sagittal T1 MR shows a focal defect at the junction of the genu & body of the corpus callosum , which had been the site of a prior surgical approach to this child's suprasellar tumor .* + +![Paramidline sagittal T1 MR in a 7 year old with intractable epilepsy shows near-complete absence of the corpus callosum due to surgical discontinuity.](images/app.statdx.com_image_thumbnail_d5ade043-0a79-4e06-a806-2a6fd3d1509f_annotated_true_size_900_quality_90_3a494990_20251018T142242Z.jpg) +**Corpus Callosotomy** +*Paramidline sagittal T1 MR in a 7 year old with intractable epilepsy shows near-complete absence of the corpus callosum due to surgical discontinuity.* + +![Midline sagittal T2 MR in an 11 year old with intractable epilepsy who had undergone an isolated corpus callosotomy shows absence of the corpus callosum but presence of a cingulate gyrus . The presence of a cingulate gyrus would not be expected with congenital agenesis of the corpus callosum.](images/app.statdx.com_image_thumbnail_126a8ad4-dc8d-4abf-832d-87fd23880c01_annotated_true_size_900_quality_90_5dbfb155_20251018T142242Z.jpg) +**Corpus Callosotomy** +*Midline sagittal T2 MR in an 11 year old with intractable epilepsy who had undergone an isolated corpus callosotomy shows absence of the corpus callosum but presence of a cingulate gyrus . The presence of a cingulate gyrus would not be expected with congenital agenesis of the corpus callosum.* + +![Midline sagittal T1 MR shows complete absence of the corpus callosum with associated absence of the cingulate gyrus. Note the radial arrangement of parasagittal gyri/sulci , which point toward the 3rd ventricle.](images/app.statdx.com_image_thumbnail_82054e81-0249-4fb3-8216-0739f38441a7_annotated_true_size_900_quality_90_7162eb50_20251018T142242Z.jpg) +**Callosal Agenesis** +*Midline sagittal T1 MR shows complete absence of the corpus callosum with associated absence of the cingulate gyrus. Note the radial arrangement of parasagittal gyri/sulci , which point toward the 3rd ventricle.* + +![Axial T1 MR in a patient with callosal agenesis shows parallel lateral ventricles with colpocephaly , resulting in a typical tear-drop shape.](images/app.statdx.com_image_thumbnail_4cc06316-602d-4975-b9dd-ad30fd02d052_annotated_true_size_900_quality_90_cc24fcf3_20251018T142242Z.jpg) +**Callosal Agenesis** +*Axial T1 MR in a patient with callosal agenesis shows parallel lateral ventricles with colpocephaly , resulting in a typical tear-drop shape.* + +![Midline sagittal T1 MR shows only a residual genu of the corpus callosum with absence of the body & splenium as well as truncation of the rostrum.](images/app.statdx.com_image_thumbnail_28f4c3c1-8c1c-4e6c-8815-4824e939b53e_annotated_true_size_900_quality_90_4a62f400_20251018T142242Z.jpg) +**Primary Callosal Dysgenesis** +*Midline sagittal T1 MR shows only a residual genu of the corpus callosum with absence of the body & splenium as well as truncation of the rostrum.* + +![Midline sagittal T1 MR in a child with severe microcephaly shows a short, thick corpus callosum .](images/app.statdx.com_image_thumbnail_0ecf80f2-8db2-4f59-a30b-4b1b5b71ab10_annotated_true_size_900_quality_90_32c3bdbd_20251018T142242Z.jpg) +**Primary Callosal Dysgenesis** +*Midline sagittal T1 MR in a child with severe microcephaly shows a short, thick corpus callosum .* + +![Midline sagittal T1 MR shows an abnormal corpus callosum with an absent rostrum, small deformed genu, thick body , & absent splenium in this child with a Chiari 2 malformation due to a myelomeningocele. Note the prominent massa intermedia , inferiorly beaked tectum , & caudally displaced elongated 4th ventricle with flattening of the fastigium .](images/app.statdx.com_image_thumbnail_4e121da9-31c4-476f-8048-b690b404c2e8_annotated_true_size_900_quality_90_2fe43416_20251018T142242Z.jpg) +**Chiari 2 Malformation** +*Midline sagittal T1 MR shows an abnormal corpus callosum with an absent rostrum, small deformed genu, thick body , & absent splenium in this child with a Chiari 2 malformation due to a myelomeningocele. Note the prominent massa intermedia , inferiorly beaked tectum , & caudally displaced elongated 4th ventricle with flattening of the fastigium .* + +![Axial T2 MR shows a prominent massa intermedia & colpocephalic lateral ventricles with periventricular white matter deficiency in Chiari 2. The genu of the corpus callosum, usually seen on axial images, is absent.](images/app.statdx.com_image_thumbnail_106f9fc3-f917-413c-b862-0fca057f2e8b_annotated_true_size_900_quality_90_345ea79c_20251018T142242Z.jpg) +**Chiari 2 Malformation** +*Axial T2 MR shows a prominent massa intermedia & colpocephalic lateral ventricles with periventricular white matter deficiency in Chiari 2. The genu of the corpus callosum, usually seen on axial images, is absent.* + +![Midline sagittal T2 MR in a 15 month old with Chiari 2 malformation shows a severely thinned & dysmorphic corpus callosum . Note the typical Chiari 2 features, including a small posterior fossa with caudal herniation of the brainstem & cerebellum, clival scalloping , elongated 4th ventricle , & beaked tectum .](images/app.statdx.com_image_thumbnail_cd7db346-bbb1-4ace-8084-e6b609ffd3a4_annotated_true_size_900_quality_90_1324558b_20251018T142242Z.jpg) +**Chiari 2 Malformation** +*Midline sagittal T2 MR in a 15 month old with Chiari 2 malformation shows a severely thinned & dysmorphic corpus callosum . Note the typical Chiari 2 features, including a small posterior fossa with caudal herniation of the brainstem & cerebellum, clival scalloping , elongated 4th ventricle , & beaked tectum .* + +![Coronal T1 C+ MR shows a classic "butterfly" glioblastoma multiforme of the corpus callosum . Central necrosis with an irregular rind of enhancing tumor is typical.](images/app.statdx.com_image_thumbnail_807b1f61-4777-4bc3-8f59-ecdd2b8d484d_annotated_true_size_900_quality_90_42f971bd_20251018T142242Z.jpg) +**Glioblastoma** +*Coronal T1 C+ MR shows a classic "butterfly" glioblastoma multiforme of the corpus callosum . Central necrosis with an irregular rind of enhancing tumor is typical.* + +![Axial T1 C+ MR shows a primary CNS lymphoma involving the splenium of the corpus callosum. There is avid, solid enhancement of the tumor with extension into the adjacent parenchymal white matter.](images/app.statdx.com_image_thumbnail_92ac059a-cc5f-46fb-bcec-54b20c03f7d2_annotated_true_size_900_quality_90_9f582aba_20251018T142242Z.jpg) +**Lymphoma** +*Axial T1 C+ MR shows a primary CNS lymphoma involving the splenium of the corpus callosum. There is avid, solid enhancement of the tumor with extension into the adjacent parenchymal white matter.* + +![Coronal oblique T1 C+ MR in an 11 year old with CNS lymphoma shows bifrontal areas of enhancement , which corresponded to hyperdense areas on CT (not shown). Note the abnormally thickened corpus callosum that is infiltrated by a nonenhancing tumor.](images/app.statdx.com_image_thumbnail_f6e3e0fd-f4ab-4e5c-a4d0-2f1abdb32e13_annotated_true_size_900_quality_90_29400ff9_20251018T142242Z.jpg) +**Lymphoma** +*Coronal oblique T1 C+ MR in an 11 year old with CNS lymphoma shows bifrontal areas of enhancement , which corresponded to hyperdense areas on CT (not shown). Note the abnormally thickened corpus callosum that is infiltrated by a nonenhancing tumor.* + +![Midline sagittal T1 MR shows a large pericallosal lipoma with severe dysgenesis of the corpus callosum .](images/app.statdx.com_image_thumbnail_99d1900d-a440-4c87-a4d7-a63b771cb20e_annotated_true_size_900_quality_90_093fee8d_20251018T142242Z.jpg) +**Pericallosal Lipoma** +*Midline sagittal T1 MR shows a large pericallosal lipoma with severe dysgenesis of the corpus callosum .* + +![Axial FLAIR MR shows a large midline lipoma. Two smaller lipomatous masses protrude into the lateral ventricles.](images/app.statdx.com_image_thumbnail_05a43764-0e56-43a7-a397-c6985ccf2f90_annotated_true_size_900_quality_90_fb43a056_20251018T142242Z.jpg) +**Pericallosal Lipoma** +*Axial FLAIR MR shows a large midline lipoma. Two smaller lipomatous masses protrude into the lateral ventricles.* + +![Axial T1 MR in a patient with holoprosencephaly shows the lack of a midline fissure. White matter is in continuity across the midline. The small basal ganglia approximate each other. Note the monoventricle communicating with a dorsal cyst .](images/app.statdx.com_image_thumbnail_65c833a7-4e18-443c-9f90-1a5b1878c31e_annotated_true_size_900_quality_90_e3eebedd_20251018T142242Z.jpg) +**Holoprosencephaly** +*Axial T1 MR in a patient with holoprosencephaly shows the lack of a midline fissure. White matter is in continuity across the midline. The small basal ganglia approximate each other. Note the monoventricle communicating with a dorsal cyst .* + +![Midline sagittal T1 MR shows both white & gray matter crossing midline anterior & posterior to the "dip" in the corpus callosum, where only gray matter traverses. This is a middle interhemispheric variant of holoprosencephaly (syntelencephaly).](images/app.statdx.com_image_thumbnail_c3835c36-b514-487d-9e87-fb2c809411b8_annotated_true_size_900_quality_90_3cb56218_20251018T142242Z.jpg) +**Holoprosencephaly** +*Midline sagittal T1 MR shows both white & gray matter crossing midline anterior & posterior to the "dip" in the corpus callosum, where only gray matter traverses. This is a middle interhemispheric variant of holoprosencephaly (syntelencephaly).* + +![Axial T1 MR in the same patient shows gray & white matter traversing the midline in the expected location of the splenium. Gray matter also protrudes into the ventricular system. The septum pellucidum is absent.](images/app.statdx.com_image_thumbnail_e972264a-0edb-41ef-827c-f0a12c2068f5_annotated_true_size_900_quality_90_a49b1938_20251018T142242Z.jpg) +**Holoprosencephaly** +*Axial T1 MR in the same patient shows gray & white matter traversing the midline in the expected location of the splenium. Gray matter also protrudes into the ventricular system. The septum pellucidum is absent.* + diff --git a/docs_md/articles/adem_a3fafeb7-5861-4364-beb8-c0e30220564e.md b/docs_md/articles/adem_a3fafeb7-5861-4364-beb8-c0e30220564e.md index 8fb7912..f21352a 100644 --- a/docs_md/articles/adem_a3fafeb7-5861-4364-beb8-c0e30220564e.md +++ b/docs_md/articles/adem_a3fafeb7-5861-4364-beb8-c0e30220564e.md @@ -409,9 +409,6 @@ breadcrumbs: ![Axial FLAIR MR shows peripheral, confluent areas of hyperintensity predominantly involving the subcortical white matter (WM) in this child with ADEM. The bilateral but asymmetric pattern is typical of ADEM.](images/app.statdx.com_image_thumbnail_deba3310-8e09-466c-97d2-1e6bccb28edf_size_168_quality_85_3dfef960_20251018T064936Z.jpg) *Axial FLAIR MR shows peripheral, confluent areas of hyperintensity predominantly involving the subcortical white matter (WM) in this child with ADEM. The bilateral but asymmetric pattern is typical of ADEM.* -![Axial FLAIR MR shows peripheral, confluent areas of hyperintensity predominantly involving the subcortical white matter (WM) in this child with ADEM. The bilateral but asymmetric pattern is typical of ADEM.](images/app.statdx.com_image_thumbnail_deba3310-8e09-466c-97d2-1e6bccb28edf_size_174_quality_85_6f9ba8b5_20251018T122441Z.jpg) -*Axial FLAIR MR shows peripheral, confluent areas of hyperintensity predominantly involving the subcortical white matter (WM) in this child with ADEM. The bilateral but asymmetric pattern is typical of ADEM.* - ![Axial T1 C+ MR in the same patient shows marked, irregular enhancement of nearly all lesions. As ADEM is a monophasic illness, enhancement of the majority of lesions is typical; all lesions have a similar time course. Enhancement of multiple sclerosis (MS) lesions is more variable.](images/app.statdx.com_image_e18458d3-5f6e-43fd-ab6d-72c7a86dca92_0f3893d6_20251018T064949Z.jpg) *Axial T1 C+ MR in the same patient shows marked, irregular enhancement of nearly all lesions. As ADEM is a monophasic illness, enhancement of the majority of lesions is typical; all lesions have a similar time course. Enhancement of multiple sclerosis (MS) lesions is more variable.* diff --git a/docs_md/articles/basal-ganglia-calcification_f8dc8f27-f256-480d-9393-7ec3495a3d27.md b/docs_md/articles/basal-ganglia-calcification_f8dc8f27-f256-480d-9393-7ec3495a3d27.md index 7abc5b3..bcdd6db 100644 --- a/docs_md/articles/basal-ganglia-calcification_f8dc8f27-f256-480d-9393-7ec3495a3d27.md +++ b/docs_md/articles/basal-ganglia-calcification_f8dc8f27-f256-480d-9393-7ec3495a3d27.md @@ -180,7 +180,7 @@ breadcrumbs: **Aging Brain, Normal** *Axial NECT shows typical basal ganglia (BG) Ca⁺⁺ in this 75-year-old man who presented after minor trauma. Note the location within the globus pallidus (GP) , typical for normal aging brain. Physiologic Ca⁺⁺ is typically seen in adults over 30 years.* -![Axial NECT shows typical basal ganglia (BG) Ca⁺⁺ in this 75-year-old man who presented after minor trauma. Note the location within the globus pallidus (GP) , typical for normal aging brain. Physiologic Ca⁺⁺ is typically seen in adults over 30 years.](images/app.statdx.com_image_thumbnail_04a1f1d8-6210-4030-84ff-4f60f3f7f7bf_size_174_quality_85_63332bdc_20251018T115114Z.jpg) +![Axial NECT shows typical basal ganglia (BG) Ca⁺⁺ in this 75-year-old man who presented after minor trauma. Note the location within the globus pallidus (GP) , typical for normal aging brain. Physiologic Ca⁺⁺ is typically seen in adults over 30 years.](images/app.statdx.com_image_thumbnail_04a1f1d8-6210-4030-84ff-4f60f3f7f7bf_size_174_quality_85_02fa8f20_20251018T142330Z.jpg) **Aging Brain, Normal** *Axial NECT shows typical basal ganglia (BG) Ca⁺⁺ in this 75-year-old man who presented after minor trauma. Note the location within the globus pallidus (GP) , typical for normal aging brain. Physiologic Ca⁺⁺ is typically seen in adults over 30 years.* diff --git a/docs_md/articles/images/app.statdx.com_image_thumbnail_04a1f1d8-6210-4030-84ff-4f60f3f7f7bf_size_174_quality_85_02fa8f20_20251018T142330Z.jpg b/docs_md/articles/images/app.statdx.com_image_thumbnail_04a1f1d8-6210-4030-84ff-4f60f3f7f7bf_size_174_quality_85_02fa8f20_20251018T142330Z.jpg new file mode 100644 index 0000000..86679bc Binary files /dev/null and b/docs_md/articles/images/app.statdx.com_image_thumbnail_04a1f1d8-6210-4030-84ff-4f60f3f7f7bf_size_174_quality_85_02fa8f20_20251018T142330Z.jpg differ diff --git a/docs_md/articles/images/app.statdx.com_image_thumbnail_05a43764-0e56-43a7-a397-c6985ccf2f90_annotated_true_size_900_quality_90_fb43a056_20251018T142242Z.jpg b/docs_md/articles/images/app.statdx.com_image_thumbnail_05a43764-0e56-43a7-a397-c6985ccf2f90_annotated_true_size_900_quality_90_fb43a056_20251018T142242Z.jpg new file mode 100644 index 0000000..5ecd61d Binary files /dev/null and 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Woodward, MD, FSRU', 'bookmarked': False, 'bookmarkUrl': '/document/bookmark/daf8e6c7-c462-456a-ae66-ba4c913c42d3', 'category': 'Ultrasound', 'compareUrl': '/compare/document/daf8e6c7-c462-456a-ae66-ba4c913c42d3/related-anatomy/treeNode?subContext=Vertebral Column and Spinal Cord', 'documentId': 'daf8e6c7-c462-456a-ae66-ba4c913c42d3', 'documentType': 'ANATOMY', 'documentUrl': '/document/vertebral-column-and-spinal-cord/daf8e6c7-c462-456a-ae66-ba4c913c42d3', 'enhancedTitle': 'Vertebral Column and Spinal Cord', 'entryDate': '12/20/17', 'imageCount': 24, 'imageUrl': '/image/thumbnail/2a4b84c1-8e69-43c0-8bad-e08e8f3d0991?size=174&quality=85', 'inCompareCart': False, 'rank': 2, 'referenceCount': 0, 'showCompareButton': False, 'title': 'Vertebral Column and Spinal Cord'}" +cases: 2 +breadcrumbs: + - "Spine" + - "Diagnosis" + - "Infection and Inflammatory Disorders" + - "Inflammatory and Autoimmune Disorders" + - "Multiple Sclerosis" +--- +# KEY FACTS + +- ## Terminology + + + - Primary demyelinating disease of CNS with multiple lesions disseminated over time & space + - Concomitant intracranial lesions in periventricular, subcallosal, brainstem, or cerebellar white matter +- ## Imaging + + + - Isolated spinal cord disease (10-20%) + - Cervical segment is most commonly affected + - Dorsolateral aspect of cord + - < 1/2 of cross-sectional area of spinal cord + - < 2 vertebral segments in length + - Sagittal & axial T1WI/T2WI sequences with gadolinium + - Lesions typically oval, peripheral, & asymmetric + - Discrete vs. vague hyperintense lesions + - Enhancement lasts 1-2 months but does not reflect disease progression +- ## Top Differential Diagnoses + + + - Intramedullary neoplasm + - Idiopathic transverse myelitis + - Neuromyelitis optica +- ## Pathology + + + - Autoimmune, cell-mediated inflammatory process focused on CNS myelin +- ## Clinical Issues + + + - Peak onset: 20-40 years + - Adult females more susceptible than males (1.7:1) + - Relapsing remitting (RR) + - Secondary progressive (SP) + - Primary progressive (PP) + - Progressive relapsing (PR) +- ## Diagnostic Checklist + + + - Imaging findings must be correlated with clinical & laboratory features to confirm diagnosis + +# TERMINOLOGY + +- ## Abbreviations + + + - Spinal cord multiple sclerosis (MS) +- ## Definitions + + + - Primary demyelinating disease of CNS with multiple lesions disseminated over time & space + +# IMAGING + +- ## General Features + + + - ### Best diagnostic clue + + + - Concomitant T2 lesions in ≥ 2 of 4 areas of CNS: Periventricular, cortical or juxtacortical, infratentorial, & spinal cord + - ### Location + + + - Isolated spinal cord disease in 10-20% + - Cervical segment is most commonly affected + - 2/3 of cord lesions + - Dorsolateral aspect of cord + - Does not respect gray-white boundary + - ### Size + + + - < 1/2 of cross-sectional area of spinal cord + - < 2 vertebral segments in length + - ### Morphology + + + - Wedge-shaped on axial MR + - Apex directed centrally +- ## MR Findings + + + - ### T1WI + + + - Iso- to hypointense lesions + - In cord (unlike brain), rarely visible as hypointense + - 30% of brain lesions are dark, "black holes" + - ### T2WI + + + - Discrete or ill-defined hyperintense lesions + - May be related to extent of demyelination + - Lesions ↑ in size due to edema associated with inflammatory infiltrates → reach max size at 4 weeks + - Slow ↓ in size over 6-8 weeks as edema resolves ± remyelination + - Lesions typically oval, peripheral, & asymmetric + - ### PD/intermediate + + + - Hyperintense lesions + - ### STIR + + + - Improved lesion detection + - ### FLAIR + + + - Lower sensitivity compared to STIR in cord imaging + - ### DWI + + + - ↑ mean diffusivity, ↓ fractional anisotropy in plaques & areas without T2 abnormality + - ### T1WI C+ + + + - Variable + - Homogeneous, nodular, or ring enhancement during acute or subacute phase + - Enhancement lasts 1-2 months + - Does not reflect disease progression + - No enhancement during chronic phase + - ### MRS + + + - ↓ N-acetylaspartate level + - ↑ choline levels, even in normal-appearing white matter + - Solitary or multifocal lesions + - Larger lesions formed by coalescence of smaller plaques + - Normal or mild focal cord expansion + - Cord edema + - Resolves after 6-8 weeks + - Cord atrophy + - Usually in late stage + - May be seen in early disease course + - Useful for monitoring disease progression & therapeutic efficacy + - Correlates with clinical disability + - fMRI + - Tactile-associated cervical cord fMRI activity ↑ in relapse-onset MS patients + - Overactivation more prominent in patients with more severe locomotor disability + - Suggests abnormality of cord functional properties may be among factors associated with clinical status of MS patient +- ## Nonvascular Interventions + + + - ### Myelography + + + - Nonspecific mild cord expansion +- ## Other Modality Findings + + + - Magnetization transfer (MT) imaging + - ↓ MT ratio in spinal cord + - Better correlation with disability & axonal loss + - ↓ MT ratios in enhancement patterns in which myelin known to be ↓ histopathologically +- ## Imaging Recommendations + + + - ### Best imaging tool + + + - T1WI/T2WI spinal cord MR in sagittal & axial planes with gadolinium + +# DIFFERENTIAL DIAGNOSIS + +- [Intramedullary Neoplasm](/document/spinal-cord-astrocytoma/43d5efa2-7a6d-4972-bfc6-c300cc31f9af) + - Cord expansion with holocord involvement + - Peritumoral edema + - Diffuse or partial enhancement + - Cystic ± hemorrhagic components +- [Spinal Cord Infarction](/document/spinal-cord-infarction/5afcaea7-09a0-49b7-8f23-f93734d627fb) + - Sudden onset of symptoms + - Posterior columns typically spared in anterior spinal infarct +- [Idiopathic Transverse Myelitis](/document/idiopathic-acute-transverse-myelit-/6e82fa47-19b7-45ac-b195-3c21687fa648) + - Lesion centrally located + - 3-4 segments in length + - Involving > 2/3 of cord cross-sectional area + - Variable enhancement + - No associated intracranial lesions + - Diagnosis of exclusion +- [Syringohydromyelia](/document/syringomyelia/fb362df4-5033-4f7c-9f4d-01d701ebab84) + - Central cystic lesion + - CSF intensity on all sequences + - No abnormal enhancement +- [Neuromyelitis Optica Spectrum Disorder (NMOSD)](/document/neuromyelitis-optica/11d42d8d-e7bb-4ecf-85ce-d96f0afeb076) + - Autoimmune inflammatory disorder involving myelin of neurons of optic nerves & spinal cord + - Limited brain involvement + - Longitudinally extensive transverse myelitis (LETM) (> 3 vertebral segments), T2 hyperintensity within cord + enhancement of optic nerves + - T2 abnormality involves entire cross section of cord +- ## Myelin Oligodendrocyte Glycoprotein Autoantibody Myelitis (MOGAD) + + + - LETM similar to AQP4 (+) NMOSD but more often involving conus + - H pattern of cord involvement + - Children often present with ADEM phenotype +- ## Parainfectious myelitis (Viral) + + + - Long segment of T2 signal abnormality + - Usually prominent irregular enhancement + +# PATHOLOGY + +- ## General Features + + + - ### Etiology + + + - Autoimmune, cell-mediated inflammatory process focused on CNS myelin + - Infectious agents may play primary or secondary role + - Humoral mechanism: Cross reactivity between infectious & self-epitopes + - ### Genetics + + + - MS may be inherited as complex multifactorial disorder resulting from interaction of genetic & environmental factors + - Estimated risk to siblings of proband ~ 3.0-5.0%, ↑ to 29.5% if 1 or both parents have MS + - Risk to offspring of person with MS is 2.0-3.0% & higher if both parents have MS + - ### Associated abnormalities + + + - 90% incidence of associated intracranial lesions + - Different serum thyroid hormone & complement C3, C4, & CH50 levels in neuromyelitis optica vs. MS + - Thyroid hormones may play different role in modulating complement activation in MS & neuromyelitis optica + - Focal regions of demyelination of varying size & age scattered throughout CNS white matter +- ## Staging, Grading, & Classification + + + - Dissemination in space (DIS) + - Demonstrated by ≥ 1 T2-hyperintense lesions that are characteristic of MS in ≥ 2 of 4 areas of CNS + - Periventricular, cortical or juxtacortical, infratentorial brain regions, & spinal cord + - Dissemination in time (DIT) + - Simultaneous presence of gadolinium-enhancing & nonenhancing lesions at any time + - New T2-hyperintense or gadolinium-enhancing lesion on follow-up MR, with reference to baseline scan, irrespective of timing of baseline MR +- ## Microscopic Features + + + - Discrete lesions of myelin destruction + - Active lesions with macrophages & lymphocytes + - Chronic lesions with gliosis & cavitation + - Perivascular cuffs of lymphocytes & mononuclear cells + - Involvement of dorsal horns common + +# CLINICAL ISSUES + +- ## Presentation + + + - ### Most common signs/symptoms + + + - Cord lesions may be asymptomatic + - Paresthesia + - ### Other signs/symptoms + + + - Muscle weakness, hyperreflexia, gait disturbance + - Bladder/bowel dysfunction + - Surveillance includes periodic neurologic examination to track disease progression & periodic brain & spinal cord MRs to monitor disease activity + - Additional examination techniques → ambulation index, 25-foot timed walk, & 25-foot walk combined with 9-hole peg test & paced serial auditory addition test +- ## Demographics + + + - ### Age + + + - Peak onset: 20-40 years + - Onset: < 18 years in 3-5% of MS cases + - ### Sex + + + - Women more susceptible than men (1.7:1) + - Men more likely to have progressive relapsing (PR) & secondary progressive (SP) MS + - Women more likely to have relapsing remitting (RR) MS + - Both sexes equally affected in primary progressive (PP) MS + - ### Ethnicity + + + - Western Europeans have higher risk + - ### Epidemiology + + + - Increasing prevalence further north from equator + - 30-80 per 100,000 in northern USA & Europe + - 6-14 per 100,000 in southern USA & Europe + - 1 per 100,000 in equatorial regions +- ## Natural History & Prognosis + + + - Benign (20%) + - Complete recovery after 1-2 attacks + - Some may experience progressive MS after 10-15 years + - RR (25%) + - Distinct periods of new or worsening symptoms alternating with complete or partial recovery + - 90% will evolve into progressive MS after 25 years + - SP (40%) + - From RR MS + - Worsening deficits & disabilities + - Incomplete & infrequent remission + - PP (12%) + - Steady progression of symptoms + - Motor dysfunction common + - Primary cord involvement; no distinct attacks + - PR (3%) + - Similar to PP MS + - Distinct periods of exacerbation but without recovery + - High mortality rate +- ## Treatment + + + - Traditional disease-modifying therapies (DMTs) + - Interferon β (several types, such as interferon β-1b, interferon β-1a) + - Enhancing suppressor T-cell activity & reducing proinflammatory cytokine production + - Glatiramer acetate + - Synthetic protein similar to myelin protein + - Shifts immune response from proinflammatory to antiinflammatory state + - S1P receptor modulators + - Fingolimod, siponimod, ozanimod + - Small-molecule oral agents, which prevent lymphocytes from leaving lymph nodes & decreasing number in peripheral blood that may cross to CNS + - Fumarates + - Dimethyl fumarate, diroximel fumarate, monomethyl fumarate + - Increases antioxidant activity + - Teriflunomide + - Inhibits proliferation of rapidly dividing T & B cells & leaving resting lymphocytes unaltered + - Early aggressive treatment strategy + - Natalizumab + - Blocks α-4 integrin & ability of leukocytes to cross blood-brain barrier + - Increased risk of progressive multifocal leukoencephalopathy (PML) + - Anti-CD20 monoclonal antibodies + - Rituximab, ocrelizumab, ublituximab, ofatumumab + - Selectively deplete CD20-expressing B cells + - Alemtuzumab + - Binds to surface antigen CD52, which is expressed on T & B lymphocytes resulting in antibody-dependent cellular cytolysis + - Cladribine + - Interferes with DNA synthesis & repair of T & B lymphocytes resulting in lymphocyte depletion + - Physical therapy + +# DIAGNOSTIC CHECKLIST + +- ## Consider + + + - Brain MR, including high-resolution fast spin-echo T2 through corpus callosum + - Periventricular, subcallosal, brainstem, or cerebellar white matter lesions suggest MS + - Gray matter atrophy correlates with disability +- ## Image Interpretation Pearls + + + - Imaging findings must be correlated with clinical & laboratory features to confirm diagnosis + - Acute MS can mimic cord neoplasm + + 23e58f52-8b8e-4c96-9a03-de07a43975cd + +## References + +# Selected References + +1. [Hernandez J: Multiple sclerosis treatment review for primary care providers. Nurse Pract. 49(7):38-47, 2024](http://www.ncbi.nlm.nih.gov/pubmed/?term=38915149%5Bpmid%5D) +1. [Solomon AJ et al: Differential diagnosis of suspected multiple sclerosis: an updated consensus approach. Lancet Neurol. 22(8):750-68, 2023](http://www.ncbi.nlm.nih.gov/pubmed/?term=37479377%5Bpmid%5D) +1. [Ciccarelli O et al: Spinal cord involvement in multiple sclerosis and neuromyelitis optica spectrum disorders. Lancet Neurol. 18(2):185-97, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=30663608%5Bpmid%5D) +1. [Thompson AJ et al: Diagnosis of multiple sclerosis: 2017 revisions of the McDonald criteria. Lancet Neurol. 17(2):162-73, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=29275977%5Bpmid%5D) +1. [Bigi S et al: Outcomes after early administration of plasma exchange in pediatric central nervous system inflammatory demyelination. J Child Neurol. 30(7):874-80, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25246301%5Bpmid%5D) +1. [Kearney H et al: Spinal cord grey matter abnormalities are associated with secondary progression and physical disability in multiple sclerosis. J Neurol Neurosurg Psychiatry. 86(6):608-14, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25097217%5Bpmid%5D) +1. [Riederer I et al: Double inversion recovery sequence of the cervical spinal cord in multiple sclerosis and related inflammatory diseases. AJNR Am J Neuroradiol. 36(1):219-25, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25169924%5Bpmid%5D) +1. [Russi AE et al: The meninges: new therapeutic targets for multiple sclerosis. Transl Res. 165(2):255-69, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25241937%5Bpmid%5D) +1. [De Stefano N et al: Spinal cord imaging in multiple sclerosis: filling the gap with the brain. Neurology. 83(15):1306-7, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=25186859%5Bpmid%5D) +1. [Makary MS et al: Tumefactive demyelinating disease with isolated spinal cord involvement. Acta Radiol Short Rep. 3(5):2047981614539324, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=25298871%5Bpmid%5D) +1. [Schlaeger R et al: Spinal cord gray matter atrophy correlates with multiple sclerosis disability. Ann Neurol. 76(4):568-80, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=25087920%5Bpmid%5D) +1. [Toosy AT et al: Voxel-based cervical spinal cord mapping of diffusion abnormalities in MS-related myelitis. Neurology. 83(15):1321-5, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=25186861%5Bpmid%5D) +1. [Simka M et al: Reinterpreting the magnetic resonance signs of hemodynamic impairment in the brains of multiple sclerosis patients from the perspective of a recent discovery of outflow block in the extracranial veins. J Neurosci Res. 88(9):1841-5, 2010](http://www.ncbi.nlm.nih.gov/pubmed/?term=20127806%5Bpmid%5D) +1. [Tallantyre EC et al: Clinico-pathological evidence that axonal loss underlies disability in progressive multiple sclerosis. Mult Scler. 16(4):406-11, 2010](http://www.ncbi.nlm.nih.gov/pubmed/?term=20215480%5Bpmid%5D) +1. [Valsasina P et al: Cervical cord functional MRI changes in relapse-onset MS patients. J Neurol Neurosurg Psychiatry. 81(4):405-8, 2010](http://www.ncbi.nlm.nih.gov/pubmed/?term=19965858%5Bpmid%5D) +1. [Zamboni P et al: Chronic cerebrospinal venous insufficiency in patients with multiple sclerosis. J Neurol Neurosurg Psychiatry. 80(4):392-9, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=19060024%5Bpmid%5D) +1. [Zhang B et al: Correlation between serum thyroxine and complements in patients with multiple sclerosis and neuromyelitis optica. Neuro Endocrinol Lett. 29(2):256-60, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18404143%5Bpmid%5D) +1. [Yukawa Y et al: MR T2 image classification in cervical compression myelopathy: predictor of surgical outcomes. Spine (Phila Pa 1976). 32(15):1675-8; discussion 1679, 2007](http://www.ncbi.nlm.nih.gov/pubmed/?term=17621217%5Bpmid%5D) +1. [Stüve O, Oksenberg J. Multiple sclerosis overview. 1993-, 2006](http://www.ncbi.nlm.nih.gov/pubmed/?term=20301492%5Bpmid%5D) +1. [International Working Group for Treatment Optimization in MS: Treatment optimization in multiple sclerosis: report of an international consensus meeting. Eur J Neurol. 11(1):43-7, 2004](http://www.ncbi.nlm.nih.gov/pubmed/?term=14692887%5Bpmid%5D) +1. [Pretorius PM et al: The role of MRI in the diagnosis of MS. Clin Radiol. 58(6):434-48, 2003](http://www.ncbi.nlm.nih.gov/pubmed/?term=12788312%5Bpmid%5D) +1. [Filippi M et al: Overview of diffusion-weighted magnetic resonance studies in multiple sclerosis. J Neurol Sci. 186 Suppl 1:S37-43, 2001](http://www.ncbi.nlm.nih.gov/pubmed/?term=11334988%5Bpmid%5D) +1. [Institute of Medicine (US) Committee on Multiple Sclerosis: current status and strategies for the future et al: 2001](http://www.ncbi.nlm.nih.gov/pubmed/?term=25057543%5Bpmid%5D) +1. [Poser CM et al: Diagnostic criteria for multiple sclerosis. Clin Neurol Neurosurg. 103(1):1-11, 2001](http://www.ncbi.nlm.nih.gov/pubmed/?term=11311469%5Bpmid%5D) +1. [Steiner I et al: Infection and the etiology and pathogenesis of multiple sclerosis. Curr Neurol Neurosci Rep. 1(3):271-6, 2001](http://www.ncbi.nlm.nih.gov/pubmed/?term=11898529%5Bpmid%5D) +1. [Bastianello S et al: MRI of spinal cord in MS. J Neurovirol. 6 Suppl 2:S130-3, 2000](http://www.ncbi.nlm.nih.gov/pubmed/?term=10871800%5Bpmid%5D) +1. [Hickman SJ et al: Imaging of the spine in multiple sclerosis. Neuroimaging Clin N Am. 10(4):689-704 ,viii, 2000](http://www.ncbi.nlm.nih.gov/pubmed/?term=11359719%5Bpmid%5D) +1. [Simon JH: Brain and spinal cord atrophy in multiple sclerosis. Neuroimaging Clin N Am. 10(4):753-70 ,ix, 2000](http://www.ncbi.nlm.nih.gov/pubmed/?term=11359723%5Bpmid%5D) +1. [Simon JH: The contribution of spinal cord MRI to the diagnosis and differential diagnosis of multiple sclerosis. J Neurol Sci. 172 Suppl 1:S32-5, 2000](http://www.ncbi.nlm.nih.gov/pubmed/?term=10606803%5Bpmid%5D) +1. [van Waesberghe JH et al: Magnetization transfer imaging of the spinal cord and the optic nerve in patients with multiple sclerosis. Neurology. 53(5 Suppl 3):S46-8, 1999](http://www.ncbi.nlm.nih.gov/pubmed/?term=10496211%5Bpmid%5D) +1. [McFarland HF: The lesion in multiple sclerosis: clinical, pathological, and magnetic resonance imaging considerations. J Neurol Neurosurg Psychiatry. 64 Suppl 1:S26-30, 1998](http://www.ncbi.nlm.nih.gov/pubmed/?term=9647281%5Bpmid%5D) +1. [Campi A et al: Acute transverse myelopathy: spinal and cranial MR study with clinical follow-up. AJNR Am J Neuroradiol. 16(1):115-23, 1995](http://www.ncbi.nlm.nih.gov/pubmed/?term=7900579%5Bpmid%5D) +1. [Miller DH: Magnetic resonance imaging and spectroscopy in multiple sclerosis. Curr Opin Neurol. 8(3):210-5, 1995](http://www.ncbi.nlm.nih.gov/pubmed/?term=7551120%5Bpmid%5D) +1. [Tartaglino LM et al: Multiple sclerosis in the spinal cord: MR appearance and correlation with clinical parameters. Radiology. 195(3):725-32, 1995](http://www.ncbi.nlm.nih.gov/pubmed/?term=7754002%5Bpmid%5D) +1. [Jeffery DR et al: Transverse myelitis. Retrospective analysis of 33 cases, with differentiation of cases associated with multiple sclerosis and parainfectious events. Arch Neurol. 50(5):532-5, 1993](http://www.ncbi.nlm.nih.gov/pubmed/?term=8489410%5Bpmid%5D) +1. [Thomas DJ et al: Magnetic resonance imaging of spinal cord in multiple sclerosis by fluid-attenuated inversion recovery. Lancet. 341(8845):593-4, 1993](http://www.ncbi.nlm.nih.gov/pubmed/?term=8094830%5Bpmid%5D) +1. [Maravilla KR et al: Magnetic resonance demonstration of multiple sclerosis plaques in the cervical cord. AJR Am J Roentgenol. 144(2):381-5, 1985](http://www.ncbi.nlm.nih.gov/pubmed/?term=3871287%5Bpmid%5D) + +## Differential diagnosis + +- {'authors': 'Bernadette L. Koch, MD', 'bookmarked': False, 'bookmarkUrl': '/document/bookmark/56579195-7385-4918-872c-c25d965e8486', 'category': 'Pediatrics', 'compareUrl': '/compare/document/56579195-7385-4918-872c-c25d965e8486/related-ddx/treeNode?subContext=Intramedullary Spinal Cord Lesion', 'documentId': '56579195-7385-4918-872c-c25d965e8486', 'documentType': 'DDX', 'documentUrl': '/document/intramedullary-spinal-cord-lesion/56579195-7385-4918-872c-c25d965e8486', 'enhancedTitle': 'Intramedullary Spinal Cord Lesion', 'entryDate': '07/10/19', 'imageCount': 21, 'imageUrl': '/image/thumbnail/d58903d8-1977-44cd-b85d-f87d4e999f1a?size=174&quality=85', 'inCompareCart': False, 'rank': 1, 'referenceCount': 4, 'showCompareButton': False, 'title': 'Intramedullary Spinal Cord Lesion'} + +## Anatomy + +- {'authors': 'Kevin R. Moore, MD', 'bookmarked': False, 'bookmarkUrl': '/document/bookmark/7bc73286-326e-4017-a9d8-eabc87b88ac9', 'category': 'Spine', 'compareUrl': '/compare/document/7bc73286-326e-4017-a9d8-eabc87b88ac9/related-anatomy/treeNode?subContext=Spinal Cord and Cauda Equina', 'documentId': '7bc73286-326e-4017-a9d8-eabc87b88ac9', 'documentType': 'ANATOMY', 'documentUrl': '/document/spinal-cord-and-cauda-equina/7bc73286-326e-4017-a9d8-eabc87b88ac9', 'enhancedTitle': 'Spinal Cord and Cauda Equina', 'entryDate': '10/20/20', 'imageCount': 24, 'imageUrl': '/image/thumbnail/e2e75362-7958-443d-aa70-f465fad198dd?size=174&quality=85', 'inCompareCart': False, 'rank': 1, 'referenceCount': 0, 'showCompareButton': False, 'title': 'Spinal Cord and Cauda Equina'} +- {'authors': 'Paula J. Woodward, MD, FSRU', 'bookmarked': False, 'bookmarkUrl': '/document/bookmark/daf8e6c7-c462-456a-ae66-ba4c913c42d3', 'category': 'Ultrasound', 'compareUrl': '/compare/document/daf8e6c7-c462-456a-ae66-ba4c913c42d3/related-anatomy/treeNode?subContext=Vertebral Column and Spinal Cord', 'documentId': 'daf8e6c7-c462-456a-ae66-ba4c913c42d3', 'documentType': 'ANATOMY', 'documentUrl': '/document/vertebral-column-and-spinal-cord/daf8e6c7-c462-456a-ae66-ba4c913c42d3', 'enhancedTitle': 'Vertebral Column and Spinal Cord', 'entryDate': '12/20/17', 'imageCount': 24, 'imageUrl': '/image/thumbnail/2a4b84c1-8e69-43c0-8bad-e08e8f3d0991?size=174&quality=85', 'inCompareCart': False, 'rank': 2, 'referenceCount': 0, 'showCompareButton': False, 'title': 'Vertebral Column and Spinal Cord'} + +## Cases + +- {'cases': [{'authors': [{'key': 'bee1f359-33fb-4cba-9e6b-ed1ca1842439', 'value': 'Jeffrey S. Ross, MD'}], 'caseVersionId': '84757fd8-c814-48a5-adad-10bddd50d9eb', 'description': 'Variability of plaque conspicuity with different sequences.\n\nSagittal T1 image (#1) is normal. Sagittal T2 image (#2) (TR 3100/TE 112) shows no definite abnormality within cord. Sagittal intermediate image (#3) (TR 4000/TE 12) show focal hyperintensity within cord at C3 and C5 levels (arrows). Sagittal STIR image (#4) (TR 3500/TE 13/TI 110) show best conspicuity of the plaques, with slightly less signal to noise.\n\nComment: Heavily T2 weighted images are not the optimum for evaluation of intrinsic cord lesions, since they tend to give an image with only two signals (CSF and soft tissue) and do not have good sensitivity to subtle changes in cord signal.', 'history': None, 'imagePoolId': '25f459bd-eb87-4b6a-bd90-4f6399b5cfd6', 'name': 'Comparison of sequences', 'teachingPoint': None}, {'authors': [{'key': 'bee1f359-33fb-4cba-9e6b-ed1ca1842439', 'value': 'Jeffrey S. Ross, MD'}], 'caseVersionId': '99626c28-2b97-4a0a-8140-b4aa51c1ad5d', 'description': 'Typical appearance of MS lesions in the cervical cord.\n\nMR study (#1-4) shows 2 focal lesions of increased signal on the STIR image (arrows, #1) that are less than 1 vertebral body in length. There is no definite cord expansion and no edema beyond the focal lesions. Axial GE image shows 1 of the lesions at C4 within the posterior aspect of the cord (arrow, #2). Following contrast, there is mild ill-defined enhancement of the C4 intramedullary plaque (arrows, #3,4). Incidental note is made of a small disc protrusion at C5-6.', 'history': 'Chronic myelopathy.', 'imagePoolId': 'fd0bbd9f-8683-4aec-8d0a-5a842598a071', 'name': 'Enhancing plaque', 'teachingPoint': None, 'demographics': '34 Years old female'}, {'authors': [{'key': 'bee1f359-33fb-4cba-9e6b-ed1ca1842439', 'value': 'Jeffrey S. Ross, MD'}], 'caseVersionId': 'a9ec521a-8d2e-4d27-9e87-79eaf424d678', 'description': 'Classic appearance of Devic disease, involving the optic nerves and spinal cord, with no brain parenchymal abnormalities.\n\nBrain examination (#1-4) shows normal FLAIR (#1), and markedly enhancing right optic nerve and chiasm (arrows, #3, 4). There is also abnormal T2 hyperintensity in the left optic nerve on the STIR image (open arrow, #2).\n\nEvaluation of the spinal cord (#5-10) shows a long segment of cord enlargement with T2 hyperintensity, and ill-defined enhancement (arrows).', 'history': 'Myelopathic and blind in both eyes.', 'imagePoolId': '98716ff1-9b49-4826-be9e-8d2b4073814e', 'name': 'Devic Disease (neuromyelitis optica)', 'teachingPoint': None}, {'authors': [{'key': 'bee1f359-33fb-4cba-9e6b-ed1ca1842439', 'value': 'Jeffrey S. Ross, MD'}], 'caseVersionId': '27b5fe08-9933-4472-bdbd-5ae4bfa5f107', 'description': 'Typical pattern of involvement in Devic disease.\n\nBrain study (#1) is normal. Evaluation of the optic nerves (#2-4) show abnormal T2 hyperintensity in the right optic nerve that shows pronounced enhancement (arrow, #3,4). The cervical cord shows a long segment (4 vertebral bodies in length) of T2 hyperintensity and mild diffuse cord enlargement (#5-7). Axial T2 image (#8) shows central cord hyperintensity. Following contrast administration (#9) there is minimal patchy enhancement of the cord. The extensive nature of the cord involvement is given the title "longitudinally extensive" cord signal abnormality. This is distinct from the much more focal abnormalities typically seen with cord involvement with MS.', 'history': None, 'imagePoolId': '90c40690-dacf-4d4d-9b7c-04e39a144bc0', 'name': 'Long segment cord demyelination', 'teachingPoint': None}, {'authors': [{'key': 'bee1f359-33fb-4cba-9e6b-ed1ca1842439', 'value': 'Jeffrey S. Ross, MD'}], 'caseVersionId': '4513b381-b5d0-4fbf-a315-5a265090e55f', 'description': 'Typical pattern of cord involvement.\n\nMR study (#1-4) shows focal T2 hyperintensity within the cord at C5 level, which shows both central (arrow, #2,3) and peripheral involvement (curved arrow, #2-4). No cord expansion.', 'history': None, 'imagePoolId': 'df27b385-6074-49f9-bbcd-a963856b974f', 'name': '3T', 'teachingPoint': None}, {'authors': [{'key': 'bee1f359-33fb-4cba-9e6b-ed1ca1842439', 'value': 'Jeffrey S. Ross, MD'}], 'caseVersionId': '27734ddd-b837-4047-85bb-1eaecf654e82', 'description': 'Multiple foci of cord enhancement in multiple sclerosis, some well-defined, and other ill-defined.\n\nImages through the cervical and thoracic cord (#1-7) show multiple foci of T2 hyperintensity (arrows, #1, 4, 5) consistent with demyelinating disease. There are foci of enhancement which vary from focal (curved arrow, #2, 3) to ill-defined (open arrow, #2, 6, 7). \n\nComment: The multiplicity of lesions along with the lack of edema or significant cord expansion is typical for demyelinating disease.', 'history': None, 'imagePoolId': 'ff2932a9-1190-4f36-bcaa-37b354ccf75d', 'name': 'Enhancing plaques', 'teachingPoint': None}, {'authors': [{'key': '99e1aff7-f42c-43a0-95ae-d89c8551aa01', 'value': 'Kevin R. Moore, MD'}], 'caseVersionId': '12839b07-fb73-472c-9539-48a0b136dd84', 'description': 'Typical case of acute spinal cord demyelination.\n\nSagittal T1WI MR (#1) is normal. Sagittal T2WI MR (#2) and STIR MR (#3) demonstrate multiple hyperintense intramedullary lesions with focal cord enlargement, typical of demyelination. Although MS is often considered a white matter disease, in fact spinal cord and brain gray matter involvement is very common. Sagittal T1 C+ MR (#4) shows faint enhancement of several lesions (arrows). Axial T2WI MR (#5, 6) are useful to localize lesion in relation to cord somatotopy. Axial T1 C+ MR (#7, 8) confirm faint ring enhancement (arrows). Sagittal (#9) and axial (#10) FLAIR MR images of the brain demonstrate concurrent severe corpus callosum and white matter brain lesions typical of MS.', 'history': 'Patient presents with relapsing/remitting Multiple Sclerosis (MS) exacerbation, complaining of left arm weakness, facial numbness, and dysmetria.', 'imagePoolId': 'b3a41192-9565-42f9-9ffd-2b494611e94a', 'name': 'Classic', 'teachingPoint': None, 'demographics': '17 Years old female'}, {'authors': [{'key': '61869900-31b1-4db7-b4f5-d4da24fa86f3', 'value': 'Mark Z. Chen, MD'}], 'caseVersionId': '16a742ad-313d-439a-af55-981ce0aede3a', 'description': 'Typical case of thoracic intramedullary demyelinating plaque.\n\nSagittal T2WI FS MR (#1) demonstrates a focal intramedullary hyperintense lesion (arrow) expanding the thoracic spinal cord. Axial T2WI (#2) and T1WI MR (#3) confirm the eccentrically located intramedullary lesion (arrow). Axial T1 C+ MR (#4) reveals peripheral ring enhancement implying more acute demyelination (arrow). Sagittal T2WI MR through the brain (#5) shows a hyperintense lesion (arrow) involving the corpus callosum, typical of MS.', 'history': 'Multiple Sclerosis patient presents with myelopathic symptoms referable to the thoracic level. ', 'imagePoolId': '204e85f8-1235-414c-8d9e-227cca403070', 'name': 'Classic, acute intramedullary lesion', 'teachingPoint': None, 'demographics': '30 Years old female'}, {'authors': [{'key': '99e1aff7-f42c-43a0-95ae-d89c8551aa01', 'value': 'Kevin R. Moore, MD'}], 'caseVersionId': '4e0e98c9-4b87-4e35-be86-b63c45123852', 'description': 'Typical case of multiple intramedullary spinal cord demyelinating lesions.\n\nSagittal T1WI MR (#1, 2) show mild degenerative disc disease but are otherwise normal. Sagittal T2WI MR (#3, 4) reveal multiple T2 hyperintense intramedullary demyelinating lesions (arrows). Sagittal STIR MR (#5, 6) show the same lesions, as well as an additional dorsal spinal cord lesion not visualized on the T2WI MR images (open arrow). In general, STIR MR is the most sensitive imaging sequence for detecting spinal demyelinating lesions. Axial T2WI MR (#7, 8) are most useful to localize lesion center (arrows) in relationship to spinal cord long tracts.', 'history': 'Patient with known Multiple Sclerosis (MS) presents for imaging during acute MS exacerbation.', 'imagePoolId': '305fd70f-34c8-421d-bebf-71ef42f45d7c', 'name': 'Multiple intramedullary lesions', 'teachingPoint': None, 'demographics': '53 Years old female'}], 'caseType': 'typical', 'name': 'TYPICAL'} +- {'cases': [{'authors': [{'key': 'bee1f359-33fb-4cba-9e6b-ed1ca1842439', 'value': 'Jeffrey S. Ross, MD'}], 'caseVersionId': 'b975cd06-048d-477e-b75e-3d34569e5131', 'description': "Multiple sclerosis, neuromyelitis optica variant (Devic's disease).\n\nT2 weighted image (#1) shows mild fusiform thoracic cord enlargement with hyperintensity (arrows). Sagittal and axial images following contrast (#2-4) show a large area of ring enhancement within the cord, with small punctate cord peripheral enhancement caudal to the major lesion (open arrow). Axial images show near total transverse cord involvement with enhancement.", 'history': 'Patient developed waist and bilateral lower extremity numbness, progressed to weakness, and inability to walk with hospital\nadmission and treatment with IV steroids and plasma exchange therapy.\n', 'imagePoolId': 'b49b8ab0-c8e1-4046-9a41-0a45f103d8d7', 'name': "Devic's variant", 'teachingPoint': None, 'demographics': '46 Years old female'}, {'authors': [{'key': '99e1aff7-f42c-43a0-95ae-d89c8551aa01', 'value': 'Kevin R. Moore, MD'}], 'caseVersionId': 'c9a7e1ce-0904-4882-a79a-08eca9797985', 'description': 'Variant case depicts ring enhancement and intrinsic lesion T1 hyperintensity.\n\nSagittal T1WI MR (#1, 2) demonstrate mild peripheral "ring" T1 hyperintensity (arrow) within an intramedullary demyelinating lesion, which has been attributed to paramagnetic metal deposition. Sagittal T2WI (#3, 4) depict peripheral T2 hyperintensity (arrow) and mild cord swelling. Sagittal T1 C+ MR (#5, 6) reveal peripheral rim-enhancement (arrows) of the lesion margins, indicating the advancing "wave" of demyelination. Axial T2WI MR (#7) and T1 C+ MR (#8) confirm same findings within the right lateral spinal cord (arrow). This pattern of signal intensity on the previous sequences is commonly noted in brain lesions but unusual in the spinal cord.', 'history': 'Adult patient with known Multiple Sclerosis (MS) presents with acute myelopathy for imaging work-up.', 'imagePoolId': '926f079e-67f4-4591-96bc-030a70f10ea2', 'name': 'Ring lesion enhancement', 'teachingPoint': None, 'demographics': '43 Years old male'}], 'caseType': 'variant', 'name': 'VARIANT'} + + +## Images + + +### Selected Images + +![Sagittal graphic depicts multiple demyelinating plaques within the cervical spinal cord, which are < 2 vertebral bodies in length.](images/app.statdx.com_image_thumbnail_cdd1d309-5f88-4517-8434-da8f015ecf81_annotated_true_size_900_quality_90_d1dfab0c_20251018T142350Z.jpg) +*Sagittal graphic depicts multiple demyelinating plaques within the cervical spinal cord, which are < 2 vertebral bodies in length.* + +![Sagittal T2 (left) & T1 C+ MR (right) show an active plaque at the C6-C7 level with ring enhancement & focal T2 hyperintensity .](images/app.statdx.com_image_thumbnail_f7c6cfd4-7a27-4173-b60d-f813b61cf506_annotated_true_size_900_quality_90_94245c62_20251018T142350Z.jpg) +*Sagittal T2 (left) & T1 C+ MR (right) show an active plaque at the C6-C7 level with ring enhancement & focal T2 hyperintensity .* + +![Sagittal T2 (left), PD (middle), & STIR (right) MR show multiple short-segment multiple sclerosis (MS) plaques within the thoracic cord . Note the relatively improved conspicuity of the plaques on the PD & STIR relative to the routine T2 sequence.](images/app.statdx.com_image_thumbnail_6bfb1493-e878-43b1-a089-407b3f0efbba_annotated_true_size_900_quality_90_28a87781_20251018T142350Z.jpg) +*Sagittal T2 (left), PD (middle), & STIR (right) MR show multiple short-segment multiple sclerosis (MS) plaques within the thoracic cord . Note the relatively improved conspicuity of the plaques on the PD & STIR relative to the routine T2 sequence.* + +![Sagittal T2 (left), T2 (middle), & T1 C+ FS (right) MR of the thoracic spine show multiple short-segment foci of T2 hyperintensity in this patient with MS. Multiple lesions show solid enhancement .](images/app.statdx.com_image_thumbnail_b72c256f-7f30-4ff0-9b03-2848dbebf832_annotated_true_size_900_quality_90_8f2ca444_20251018T142350Z.jpg) +*Sagittal T2 (left), T2 (middle), & T1 C+ FS (right) MR of the thoracic spine show multiple short-segment foci of T2 hyperintensity in this patient with MS. Multiple lesions show solid enhancement .* + +![Axial T2 MR shows focal MS plaques as T2 hyperintensity within both the right & left lateral aspects of the cervical cord .](images/app.statdx.com_image_thumbnail_5d6a0b48-ad9b-482b-99e5-bb3acf61fd6d_annotated_true_size_900_quality_90_d2dc5fbb_20251018T142350Z.jpg) +*Axial T2 MR shows focal MS plaques as T2 hyperintensity within both the right & left lateral aspects of the cervical cord .* + +![Axial T1 C+ MR shows focal enhancement within the right & left lateral aspect of the cervical cord in this patient with active MS plaques.](images/app.statdx.com_image_thumbnail_65ddf85c-133c-416b-987b-f64f8360baea_annotated_true_size_900_quality_90_9dd80371_20251018T142350Z.jpg) +*Axial T1 C+ MR shows focal enhancement within the right & left lateral aspect of the cervical cord in this patient with active MS plaques.* + +![Sagittal T2 (left) & T1 C+ MR (right) show active enhancing plaque at the C2 level with both focal well-defined (enhancing) T2 focus & a small amount of surrounding nonenhancing edema .](images/app.statdx.com_image_thumbnail_927e7fcc-08ba-4599-a057-8c54ea03bfad_annotated_true_size_900_quality_90_c2a635e9_20251018T142350Z.jpg) +*Sagittal T2 (left) & T1 C+ MR (right) show active enhancing plaque at the C2 level with both focal well-defined (enhancing) T2 focus & a small amount of surrounding nonenhancing edema .* + +![Sagittal T2 (left) & T1 C+ FS (right) MR show several T2-hyperintense foci in the cervical cord in this patient with MS. 2 of the lesions enhance reflecting active demyelination .](images/app.statdx.com_image_thumbnail_ddda2a05-d65f-416c-9100-45a824c86f76_annotated_true_size_900_quality_90_a8ef1ebd_20251018T142350Z.jpg) +*Sagittal T2 (left) & T1 C+ FS (right) MR show several T2-hyperintense foci in the cervical cord in this patient with MS. 2 of the lesions enhance reflecting active demyelination .* + +![Axial T2WI MR shows focal T2-hyperintense demyelinating lesions with both central & peripheral involvement . T2-hyperintense lesions are not specific for plaque age, degree of myelin & axon loss, or amount of edema & inflammation.](images/app.statdx.com_image_thumbnail_e26b711f-9edd-4e0b-bf62-14e7fdc5e6e5_annotated_true_size_900_quality_90_c6c3c128_20251018T142350Z.jpg) +*Axial T2WI MR shows focal T2-hyperintense demyelinating lesions with both central & peripheral involvement . T2-hyperintense lesions are not specific for plaque age, degree of myelin & axon loss, or amount of edema & inflammation.* + +![Sagittal high-resolution GRE MR of the thoracic cord shows multiple areas of ↑ signal in this patient with MS. All lesions are ≤ 2 vertebral bodies in length, typical for MS.](c5b7cee6-36e2-4513-8201-dd8945569641) +*Sagittal high-resolution GRE MR of the thoracic cord shows multiple areas of ↑ signal in this patient with MS. All lesions are ≤ 2 vertebral bodies in length, typical for MS.* + + +### Additional Images + +![Sagittal T2WI MR shows diffuse cord enlargement & hyperintensity throughout cervical segment into upper thoracic cord in this case of acute MS exacerbation.](c54e8dc1-7734-4ed7-9b33-79ce219f52c6) +*Sagittal T2WI MR shows diffuse cord enlargement & hyperintensity throughout cervical segment into upper thoracic cord in this case of acute MS exacerbation.* + +![Sagittal T1WI C+ MR shows diffuse cord expansion & extensive multilevel cord enhancement in acute exacerbation of MS.](226aa411-0834-4f44-8e71-d7bd384946f2) +*Sagittal T1WI C+ MR shows diffuse cord expansion & extensive multilevel cord enhancement in acute exacerbation of MS.* + +![Axial T1WI C+ MR with fat suppression of cervical cord in a different patient shows right peripheral nodular enhancement.](cc419118-3518-48c0-8d90-80cb7dbd02b0) +*Axial T1WI C+ MR with fat suppression of cervical cord in a different patient shows right peripheral nodular enhancement.* + +![Sagittal T2WI MR of cervical cord shows a more discrete demyelinating focus at C3-C4.](3105c954-4abb-4532-aff9-e78e3f6d136d) +*Sagittal T2WI MR of cervical cord shows a more discrete demyelinating focus at C3-C4.* + +![Axial T2WI MR of cervical cord in another patient shows a poorly defined, wedge-shaped, mildly hyperintense plaque within right lateral aspect of the cord.](804853d2-0057-48d2-b52f-39b3793c192b) +*Axial T2WI MR of cervical cord in another patient shows a poorly defined, wedge-shaped, mildly hyperintense plaque within right lateral aspect of the cord.* + +![Sagittal T2WI MR of cervical cord shows an ill-defined hyperintense intramedullary lesion at C5-C6.](56c5c510-2867-4016-8f60-bbe34396c8d8) +*Sagittal T2WI MR of cervical cord shows an ill-defined hyperintense intramedullary lesion at C5-C6.* + +![Sagittal STIR MR shows a focal hyperintense demyelinating plaque within the thoracic cord without significant cord expansion. STIR MR is more sensitive for lesion depiction than T2WI MR at the price of more artifacts.](9405a238-1166-43de-8950-2074beb972c6) +*Sagittal STIR MR shows a focal hyperintense demyelinating plaque within the thoracic cord without significant cord expansion. STIR MR is more sensitive for lesion depiction than T2WI MR at the price of more artifacts.* + +![Sagittal T2WI MR of the cervical spinal cord demonstrates multiple T2-hyperintense foci , some well defined & others ill defined. The multiplicity of lesions & lack of edema or significant cord expansion is typical for demyelinating disease.](b2baf340-fc75-42e1-adc6-15a627126baa) +*Sagittal T2WI MR of the cervical spinal cord demonstrates multiple T2-hyperintense foci , some well defined & others ill defined. The multiplicity of lesions & lack of edema or significant cord expansion is typical for demyelinating disease.* + +![Sagittal T1WI C+ MR shows multiple enhancing demyelinating lesions within the cervical spinal cord. Enhancement varies from focal to ill defined . The enhancement pattern changes with evolution of inflammation.](cb69d270-e7d2-4687-9271-05ebe61d8a77) +*Sagittal T1WI C+ MR shows multiple enhancing demyelinating lesions within the cervical spinal cord. Enhancement varies from focal to ill defined . The enhancement pattern changes with evolution of inflammation.* + +![T1WI C+ MR (sagittal on top, axial on bottom) illustrates an incomplete rim-enhancing lesion in the dorsal cervical cord at the C3-C4 level. A 2nd small enhancing focus is noted in the ventral cord at the C6 level .](4b13a220-0bbf-4d72-a532-462f0550b539) +*T1WI C+ MR (sagittal on top, axial on bottom) illustrates an incomplete rim-enhancing lesion in the dorsal cervical cord at the C3-C4 level. A 2nd small enhancing focus is noted in the ventral cord at the C6 level .* + +![Sagittal PD FSE MR of the cervical spinal cord demonstrates characteristic ovoid hyperintense intramedullary demyelinating lesions without significant cord expansion.](29475de3-ed4e-4218-a400-20a5098c4ea8) +*Sagittal PD FSE MR of the cervical spinal cord demonstrates characteristic ovoid hyperintense intramedullary demyelinating lesions without significant cord expansion.* + +![Axial T1 C+ MR of the cervical spinal cord depicts focal ring enhancement within an active MS demyelinating lesion.](b35e8b09-0c1e-4011-90bc-9f5987ecc2ca) +*Axial T1 C+ MR of the cervical spinal cord depicts focal ring enhancement within an active MS demyelinating lesion.* + diff --git a/docs_md/articles/pediatric-multiple-sclerosis-brain_f2592b04-f800-4235-9eea-a43f2bf4adfe.md b/docs_md/articles/pediatric-multiple-sclerosis-brain_f2592b04-f800-4235-9eea-a43f2bf4adfe.md index 26de533..0b23caa 100644 --- a/docs_md/articles/pediatric-multiple-sclerosis-brain_f2592b04-f800-4235-9eea-a43f2bf4adfe.md +++ b/docs_md/articles/pediatric-multiple-sclerosis-brain_f2592b04-f800-4235-9eea-a43f2bf4adfe.md @@ -376,7 +376,7 @@ breadcrumbs: - 95% with clinically definite MS have positive MR findings - a5089f2b-fbb1-4100-b013-c093925fe15e + 4f257a7a-e9b9-4b8e-81ab-2922fcef8a3b ## References diff --git a/scrapers/document_to_markdown.py b/scrapers/document_to_markdown.py index a24c476..0a23ee8 100644 --- a/scrapers/document_to_markdown.py +++ b/scrapers/document_to_markdown.py @@ -15,6 +15,7 @@ Output: a .md file next to the JSON (or in --output-dir) with the same base name from __future__ import annotations import argparse +import fnmatch import glob import json import os @@ -679,6 +680,135 @@ def process_file(path: str, out_dir: str, overwrite: bool = False) -> tuple[bool ) except Exception: pass + # DDX / Tables / Anatomy / Cases: detect and append to frontmatter and body + try: + # DDX: only consider cached DDX[docid]; if not present, ddx is treated as not existing + ddx_entry = None + ddx_html = None + ddx_list = None + if isinstance(DDX, dict) and docid and docid in DDX: + ddx_entry = DDX.get(docid) + logger.debug(f"Found DDX entry for docid {docid}: {ddx_entry}") + # ddx_entry may be HTML or structured list/dict + if isinstance(ddx_entry, dict): + ddx_html = ddx_entry.get("ddxHtml") + ddx_list = ddx_entry.get("ddx") or ddx_entry.get("differentialDiagnoses") or ddx_entry.get("differentials") + else: + # could be list or simple string + ddx_list = ddx_entry + else: + logger.debug(f"No cached DDX entry for docid {docid}") + logger.debug(f"DDX keys available: {list(DDX.keys())}") + + logger.debug(f"DDX entry for docid {docid}: {ddx_entry}") + if ddx_html and isinstance(ddx_html, str) and ddx_html.strip(): + try: + ddx_md = html_to_markdown(ddx_html) + md = md.rstrip() + "\n\n" + "## Differential diagnosis\n\n" + ddx_md + "\n" + front_lines.append(f"ddx: {json.dumps(True)}") + except Exception: + logger.debug("Failed to convert ddxHtml for %s", out_path) + elif ddx_list: + # render list representation + try: + front_lines.append(f"ddx: {json.dumps(True)}") + md = md.rstrip() + "\n\n" + "## Differential diagnosis\n\n" + if isinstance(ddx_list, list): + for item in ddx_list: + md += "- " + str(item).strip() + "\n" + else: + md += str(ddx_list).strip() + "\n" + md += "\n" + except Exception: + pass + except Exception: + pass + + try: + # Tables: only consider cached TABLES[docid] + tables_entry = None + tables_html = None + tables_list = None + if isinstance(TABLES, dict) and docid and docid in TABLES: + tables_entry = TABLES.get(docid) + if isinstance(tables_entry, dict): + tables_html = tables_entry.get("tableHtml") + tables_list = tables_entry.get("tables") + else: + tables_list = tables_entry + if tables_html and isinstance(tables_html, str) and tables_html.strip(): + try: + tbl_md = html_to_markdown(tables_html) + md = md.rstrip() + "\n\n" + "## Tables\n\n" + tbl_md + "\n" + front_lines.append(f"tables: {json.dumps(True)}") + except Exception: + logger.debug("Failed to convert tableHtml for %s", out_path) + elif tables_list: + try: + front_lines.append(f"tables: {json.dumps(len(tables_list) if isinstance(tables_list, list) else True)}") + md = md.rstrip() + "\n\n" + "## Tables\n\n" + if isinstance(tables_list, list): + for t in tables_list: + if isinstance(t, str) and "<" in t: + md += html_to_markdown(t) + "\n" + else: + md += str(t) + "\n\n" + else: + md += str(tables_list) + "\n" + except Exception: + pass + except Exception: + pass + + try: + # Anatomy: only consider cached ANATOMY[docid] + anatomy_entry = None + anatomy_data = None + if isinstance(ANATOMY, dict) and docid and docid in ANATOMY: + anatomy_entry = ANATOMY.get(docid) + anatomy_data = anatomy_entry + if anatomy_data: + try: + if isinstance(anatomy_data, list): + front_lines.append('anatomy:') + for a in anatomy_data: + front_lines.append(f" - {json.dumps(str(a))}") + md = md.rstrip() + "\n\n" + "## Anatomy\n\n" + for a in anatomy_data: + md += "- " + str(a).strip() + "\n" + md += "\n" + else: + front_lines.append(f"anatomy: {json.dumps(str(anatomy_data))}") + md = md.rstrip() + "\n\n" + "## Anatomy\n\n" + str(anatomy_data).strip() + "\n" + except Exception: + pass + except Exception: + pass + + try: + # Cases / caseStudies: only consider cached CASES[docid] + cases_entry = None + cases_data = None + if isinstance(CASES, dict) and docid and docid in CASES: + cases_entry = CASES.get(docid) + cases_data = cases_entry + if cases_data: + try: + front_lines.append(f"cases: {json.dumps(len(cases_data) if isinstance(cases_data, list) else True)}") + md = md.rstrip() + "\n\n" + "## Cases\n\n" + if isinstance(cases_data, list): + for c in cases_data: + if isinstance(c, str) and "<" in c: + md += html_to_markdown(c) + "\n" + else: + md += "- " + str(c).strip() + "\n" + md += "\n" + else: + md += str(cases_data).strip() + "\n" + except Exception: + pass + except Exception: + pass # collect breadcrumbs and render as YAML list if present crumbs = [] if docid: @@ -990,7 +1120,7 @@ def main(argv: Iterable[str] | None = None) -> int: help="Where to write .md files (defaults to input-dir) ", ) p.add_argument( - "--pattern", default="*.json", help="Glob pattern to find files in input dir" + "--pattern", default="*", help="Glob pattern to limit files processed" ) p.add_argument( "--overwrite", action="store_true", help="Overwrite existing .md files" @@ -1014,9 +1144,9 @@ def main(argv: Iterable[str] | None = None) -> int: # remove all .md files in output_dir shutil.rmtree(output_dir, ignore_errors=True) - files = sorted(glob.glob(os.path.join(input_dir, args.pattern))) + files = sorted(glob.glob(os.path.join(input_dir, "*.json"))) if not files: - print(f"No files found in {input_dir} matching {args.pattern}") + print(f"No files found in {input_dir} matching *.json") return 1 # Start by caching image group metadata @@ -1065,7 +1195,7 @@ def main(argv: Iterable[str] | None = None) -> int: with open(path, "r", encoding="utf-8") as f: ddx_data = json.load(f) - doc_id = base.split("_ddx_")[1].split("_")[0] + doc_id = base.split("_document_")[1].split("_")[0] DDX[doc_id] = ( ddx_data # logger.debug(f"Document summary {n}: {pformat(doc)}") @@ -1074,7 +1204,7 @@ def main(argv: Iterable[str] | None = None) -> int: with open(path, "r", encoding="utf-8") as f: tables_data = json.load(f) - doc_id = base.split("_tables_")[1].split("_")[0] + doc_id = base.split("_document_")[1].split("_")[0] TABLES[doc_id] = ( tables_data # logger.debug(f"Document summary {n}: {pformat(doc)}") @@ -1083,7 +1213,7 @@ def main(argv: Iterable[str] | None = None) -> int: with open(path, "r", encoding="utf-8") as f: anatomy_data = json.load(f) - doc_id = base.split("_anatomy_")[1].split("_")[0] + doc_id = base.split("_document_")[1].split("_")[0] ANATOMY[doc_id] = ( anatomy_data # logger.debug(f"Document summary {n}: {pformat(doc)}") @@ -1092,7 +1222,7 @@ def main(argv: Iterable[str] | None = None) -> int: with open(path, "r", encoding="utf-8") as f: cases_data = json.load(f) - doc_id = base.split("_cases_")[1].split("_")[0] + doc_id = base.split("_document_")[1].split("_")[0] CASES[doc_id] = ( cases_data # logger.debug(f"Document summary {n}: {pformat(doc)}") @@ -1102,10 +1232,19 @@ def main(argv: Iterable[str] | None = None) -> int: logger.debug( f"Cached {len(DOCUMENT_SUMMARYS)} document summaries from summary files." ) + logger.debug(f"Cached {len(DDX)} DDX entries from ddx files.") + logger.debug(f"Cached {len(TABLES)} Tables entries from tables files.") + logger.debug(f"Cached {len(ANATOMY)} Anatomy entries from anatomy files.") + logger.debug(f"Cached {len(CASES)} Cases entries from cases files.") ok = 0 for path in files: base = os.path.basename(path) + + if args.pattern and not fnmatch.fnmatch(base, args.pattern): + # if args.verbose: + # print(f"SKIP (pattern mismatch): {path}") + continue # only process files that match the desired prefix if ( not base.startswith("app.statdx.com_document_")