298 lines
29 KiB
Markdown
298 lines
29 KiB
Markdown
---
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title: "Basal Ganglia Calcification"
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docid: "f8dc8f27-f256-480d-9393-7ec3495a3d27"
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authors:
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- key: "8d5254e9-8dda-478b-8f08-bdee97a32c79"
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value: "Karen L. Salzman, MD, FACR"
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breadcrumbs:
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-
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name: "Brain"
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slug: "brain"
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treeNodeId: "6d8829f1-14d7-45af-8675-255189aa526a"
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-
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name: "Differential Diagnosis"
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slug: "differential-diagnosis"
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treeNodeId: "a7fdd139-664e-4bb8-8d18-400e4733ff60"
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-
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name: "Supratentorial Brain Parenchyma"
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slug: "supratentorial-brain-parenchyma"
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treeNodeId: "6683b329-de24-4726-a77a-bf760698fa6a"
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-
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name: "Anatomically Based Differentials"
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slug: "anatomically-based-differentials"
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treeNodeId: "205377bb-d221-4519-aec2-85bad8ed08de"
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-
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name: "Basal Ganglia Calcification"
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slug: "basal-ganglia-calcification"
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treeNodeId: null
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category: "Brain"
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cmeTopicId: "b45f0261-eda5-4a33-a468-2c2632ec25af"
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documentVersionId: "c3a08182-fe6e-42b1-a8a1-bf4b64c51892"
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imageCount: 28
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lastUpdated: "02/01/23"
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pageDescription: "Basal Ganglia Calcification"
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pageKeywords: "Brain, Differential Diagnosis, Supratentorial Brain Parenchyma, Anatomically Based Differentials, Basal Ganglia Calcification"
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pageTitle: "Basal Ganglia Calcification | STATdx"
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enhancedTitle: "Basal Ganglia Calcification"
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type: "DDX"
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references: true
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breadcrumbs:
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- "Brain"
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- "Differential Diagnosis"
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- "Supratentorial Brain Parenchyma"
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- "Anatomically Based Differentials"
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- "Basal Ganglia Calcification"
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---
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# ESSENTIAL INFORMATION
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- ## Key Differential Diagnosis Issues
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- Basal ganglia (BG) Ca⁺⁺ is end result of multiple toxic, metabolic, inflammatory, & infectious insults
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- Location of Ca⁺⁺ helpful to determine underlying cause [globus pallidus (GP) vs. putamen vs. caudate]
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- Patient age may impact differential diagnosis
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- ## Helpful Clues for Common Diagnoses
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- **Aging B****rain, Normal**
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- Commonly affects GP more than putamen
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- Seen in aging brain as normal variant
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- Typically in patients older than 30 years
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- If occurs with other Ca⁺⁺, consider pathologic condition
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- **Neurocysticercosis**
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- May occur anywhere in brain
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- Convexity subarachnoid spaces most common
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- Imaging varies with pathologic stage
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- Ca⁺⁺ in nodular calcified (healed) stage
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- ## Helpful Clues for Less Common Diagnoses
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- **Fahr Disease**
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- Bilateral symmetric BG Ca⁺⁺, often with Ca⁺⁺ in other locations
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- GP is most common site of Ca⁺⁺ (lateral > medial)
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- Other locations: Putamen, caudate, thalami, dentate nuclei of cerebellum, cerebral white matter (WM), internal capsule
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- Associated abnormalities: Parkinsonism in autosomal dominant Fahr disease (FD)
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- **Hypoxic-Ischemic Injury**
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- **Term**: Profound acute injury results in decreased BG & thalamic density ± hemorrhage acutely
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- Lateral thalami & posterior putamen typical
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- May show Ca⁺⁺ in chronic phase
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- **Adults**: Putamen > GP typically
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- May have history of anoxic event
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- MR > CT for acute changes
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- May show Ca⁺⁺ in chronic phase
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- **Mitochondrial Disorders**
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- Mitochondrial myopathy, encephalopathy, lactic acidosis, & stroke-like episodes (MELAS): BG Ca⁺⁺ in child or young adult with cortical lesions (parietooccipital > temporoparietal)
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- Myoclonic epilepsy with ragged red fibers (MERRF): BG Ca⁺⁺ with watershed ischemia
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- **Congenital Infections**
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- **HIV, congenital**
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- Symmetric BG Ca⁺⁺ & cerebral atrophy
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- GP & putamen > caudate
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- Subcortical WM Ca⁺⁺ common
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- Ca⁺⁺ occurs in fairly symmetric fashion, result of calcific vasculopathy of medium & small arteries
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- **CMV, congenital**
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- Periventricular Ca⁺⁺, microcephaly, & cortical dysplasia
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- Periventricular > > BG Ca⁺⁺
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- **Endocrinologic Disorders**
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- Imaging of hyperparathyroidism, hypoparathyroidism, pseudohypoparathyroidism, pseudopseudohypoparathyroidism, hypothyroidism in nearly indistinguishable
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- Bilateral BG: GP & putamen, dentate nuclei, thalami, subcortical areas
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- Ca⁺⁺ in primary hypoparathyroidism is more diffuse than in other etiologies of Ca⁺⁺
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- **Toxoplasmosis, Acquired**
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- Typically multifocal, but BG common site (up to 75%)
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- Enhancing lesion most common acutely
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- Post therapy, Ca⁺⁺ is common
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- **Leigh Syndrome**
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- Bilateral, symmetric ↑ T2/FLAIR putamina & periaqueductal gray matter
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- Putamen > caudate > GP, Ca⁺⁺ when chronic
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- **Tuberculosis**
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- Typically causes tuberculous meningitis &/or localized CNS infection, tuberculoma
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- ~ 20% of tuberculomas calcify
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- **Radiation****&****Chemotherapy**
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- Mineralizing microangiopathy causes BG & subcortical WM Ca⁺⁺, atrophy
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- Mineralizing microangiopathy common with chemotherapy & XRT
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- Typically occurs 2 or more years after XRT
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- **Cavernous Malformation (Mimic)**
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- Hyperdense mass (Ca⁺⁺ & blood products) may occur in any location
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- **Vascular Calcification (Mimic)**
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- May relate to physiologic vascular calcification, atherosclerosis, aneurysm, or vascular mass
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- **Tuberous Sclerosis Complex (Mimic)**
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- Subependymal nodules are typically calcified; occur along caudothalamic groove, periventricular
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- ## Helpful Clues for Rare Diagnoses
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- **Developmental Venous Anomaly**
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- Congenital cerebral vascular malformation with mature venous elements
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- "Medusa head" with many small veins joining into collector vein
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- Seen on contrast CT/MR, CTA/CTV, MRV, DSA, SWI
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- Unilateral BG/thalami Ca⁺⁺ rare
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- May be related to venous congestion/ischemia
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- **Pantothenate Kinase-Associated Neurodegeneration**
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- Rare neurodegenerative disorder with brain iron accumulation
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- T2 MR characteristic: High signal within bilateral GP with surrounding low signal, eye of the tiger appearance
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- CT may show mineralization in GP
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- Formerly known as Hallervorden-Spatz
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- **Carbon Monoxide Poisoning**
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- Typically hypodense, symmetric GP on CT, T2 hyperintense
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- GP Ca⁺⁺ occurs as end result
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- **Parasites, Miscellaneous**
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- **Amebic encephalitis**: Supratentorial, frontal lobes, & BG
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- Typically enhancing lesions acutely, may calcify in chronic phase
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- **Malaria**: Predilection for BG, cortex
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- Hemorrhage, infarcts, & cerebral edema
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- May show Ca⁺⁺ in chronic phase
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- **Paragonimiasis**: Acutely often hemorrhage or infarct, followed by Ca⁺⁺ granulomas
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- ## Alternative Differential Approaches
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- BG Ca⁺⁺ in **child**
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- Mitochondrial encephalopathies: MELAS, MERRF, Leigh syndrome
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- Congenital infections: HIV, CMV
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- HIE, term
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- Associated with Down syndrome
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- Aicardi-Goutières syndrome (pseudo-TORCH)
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- Cockayne syndrome
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- Long-term complications of radiation therapy for childhood brain tumors & intrathecal chemotherapy
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## References
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# Selected References
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1. [de Brouwer EJ et al: Basal ganglia calcifications: no association with cognitive function. J Neuroradiol. S0150-9861(22)00066-9, 2022](http://www.ncbi.nlm.nih.gov/pubmed/?term=35134441%5Bpmid%5D)
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1. [Di Mascio D et al: Role of fetal magnetic resonance imaging in fetuses with congenital cytomegalovirus infection: a multicenter study. Ultrasound Obstet Gynecol. ePub, 2022](http://www.ncbi.nlm.nih.gov/pubmed/?term=36056700%5Bpmid%5D)
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1. [Patel J et al: Hyperkinetic choreiform movements secondary to basal ganglia calcification and underlying developmental venous anomaly. Cureus. 14(3):e22752, 2022](http://www.ncbi.nlm.nih.gov/pubmed/?term=35371891%5Bpmid%5D)
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1. [Zavatta G et al: Basal ganglia calcification is associated with local and systemic metabolic mechanisms in adult hypoparathyroidism. J Clin Endocrinol Metab. 106(7):1900-17, 2021](http://www.ncbi.nlm.nih.gov/pubmed/?term=33788935%5Bpmid%5D)
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1. [Batla A et al: Deconstructing Fahr's disease/syndrome of brain calcification in the era of new genes. Parkinsonism Relat Disord. 37:1-10, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=28162874%5Bpmid%5D)
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1. [Levine D et al: How does imaging of congenital Zika compare with imaging of other TORCH infections? Radiology. 285(3):744-61, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=29155634%5Bpmid%5D)
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1. [Yoshimoto K et al: Prevalence and clinicopathological features of H3.3 G34-mutant high-grade gliomas: a retrospective study of 411 consecutive glioma cases in a single institution. Brain Tumor Pathol. 34(3):103-12, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=28447171%5Bpmid%5D)
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1. [Donzuso G et al: Extensive bilateral striopallidodentate calcinosis: a 50 years history of hypoparathyroidism presenting like a parkinsonian syndrome. J Neurol. 263(9):1876-9, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=27485169%5Bpmid%5D)
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1. [Nunomura A: Idiopathic basal ganglia calcification (Fahr's disease) and dementia. Psychiatry Clin Neurosci. 70(3):129-30, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=26939914%5Bpmid%5D)
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1. [Saini AG et al: Teaching neuroimages: the syndrome of cutaneous photosensitivity, growth failure, and basal ganglia calcification. Neurology. 87(6):e56-7, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=27502967%5Bpmid%5D)
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1. [Gossner J: Basal ganglia calcifications on brain computed tomography are also common in other elderly populations. Geriatr Gerontol Int. 15(1):128, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25583396%5Bpmid%5D)
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1. [Ghei SK et al: MR imaging of hypoxic-ischemic injury in term neonates: pearls and pitfalls. Radiographics. 34(4):1047-61, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=25019441%5Bpmid%5D)
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1. [Hegde AN et al: Differential diagnosis for bilateral abnormalities of the basal ganglia and thalamus. Radiographics. 31(1):5-30, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=21257930%5Bpmid%5D)
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## Images
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### Selected Images
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**Aging Brain, Normal**
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*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) <img src='img/arrows/CS.png'/>, typical for normal aging brain. Physiologic Ca⁺⁺ is typically seen in adults over 30 years.*
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**Aging Brain, Normal**
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*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) <img src='img/arrows/CS.png'/>, typical for normal aging brain. Physiologic Ca⁺⁺ is typically seen in adults over 30 years.*
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**Neurocysticercosis**
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*Axial CT shows multiple calcified nodules in the deep gray nuclei <img src='img/arrows/CS.png'/> & along the cortex related to the nodular, calcified (healed) stage of neurocysticercosis. This intracranial parasitic infection is caused by the pork tapeworm Taenia solium.*
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**Fahr Disease**
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*Axial NECT shows the typical CT appearance of Fahr disease (FD) with extensive calcifications present in the BG, cerebral white matter (WM), & at the subcortical gray matter-WM junctions.*
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**Hypoxic-Ischemic Injury**
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*Axial NECT shows calcification of thalami & BG <img src='img/arrows/WC.png'/> from status marmoratus. There is atrophy & a collapsed calvarium following remote mixed hypoxic-ischemic injury (HII) in this infant. Profound acute HII typically affects the BG.*
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**Mitochondrial Disorders**
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*Axial NECT in a teenager shows bilateral GP <img src='img/arrows/WS.png'/> Ca⁺⁺, a rare finding in patients < 30 years.*
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**Mitochondrial Disorders**
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*FLAIR MR (same patient) shows left frontal & parietal hyperintensity related to recent middle cerebral artery <img src='img/arrows/CS.png'/> & anterior cerebral artery <img src='img/arrows/CO.png'/> infarcts. Muscle biopsy showed myoclonic epilepsy with ragged-red fibers. This rare mitochondrial disorder often presents with myoclonus & seizures. Imaging mimics other mitochondrial disorders, incl. mitochondrial myopathy, encephalopathy, lactic acidosis, & stroke-like episodes (MELAS).*
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**Mitochondrial Disorders**
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*Axial NECT shows Ca⁺⁺ of the GP bilaterally <img src='img/arrows/WS.png'/> in this child with MELAS. Note the low density in the medial occipital lobes related to <img src='img/arrows/CO.png'/> infarcts. BG Ca⁺⁺ is abnormal in children & young adults.*
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**HIV, Congenital**
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*Axial NECT in a patient with congenital HIV shows bilateral symmetrical BG Ca⁺⁺ predominantly in the GP <img src='img/arrows/CS.png'/>. This Ca⁺⁺ is seen typically months after birth. With HIV, involvement of the lentiform nuclei Ca⁺⁺ is greater than the caudate heads.*
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**CMV, Congenital**
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*Axial NECT shows periventricular & BG Ca⁺⁺ as well as open Sylvian fissures & ventriculomegaly. Periventricular Ca⁺⁺, ventriculomegaly, & microcephaly strongly suggest congenital CMV infection.*
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**Endocrinologic Disorders**
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*Axial NECT in a patient with with hypothyroidism shows diffuse hyperdense Ca⁺⁺ within the BG, thalami, & subcortical WM. Ca⁺⁺ related to systemic disease is typically symmetric.*
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**Endocrinologic Disorders**
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*Axial NECT in a patient with pseudohypoparathyroidism shows dense Ca⁺⁺ within the BG & subcortical WM in a pseudohypoparathyroidism patient. There is significant imaging overlap between systemic diseases with abnormal calcium deposition.*
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**Toxoplasmosis, Acquired**
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*Axial T1 C+ MR shows an enhancing right BG mass <img src='img/arrows/CS.png'/> in an AIDS patient. Post therapy, enhancing lesions typically calcify. The BG is the most common location for toxoplasmosis followed by the thalamus, then the cerebral hemispheres.*
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**Leigh Syndrome**
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*Axial T2WI MR shows symmetric T2 hyperintensity in the BG <img src='img/arrows/BS.png'/> bilaterally in this child with neurodegeneration. Ca⁺⁺ of the BG is seen in chronic cases.*
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**Radiation & Chemotherapy**
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*Axial NECT shows mineralizing microangiopathy related to radiation therapy & chemotherapy for a remote childhood neoplasm. Note the symmetric Ca⁺⁺ in the BG & subcortical WM. This typically occurs ~ 2 years after therapy with XRT & chemotherapy.*
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**Tuberous Sclerosis Complex (Mimic)**
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*Axial T2 MR shows calcified subependymal nodules in the foramen of Monro region <img src='img/arrows/CS.png'/> in this child with seizures, mimicking BG Ca⁺⁺. These nodules occur in 98% of patients with tuberous sclerosis.*
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**Developmental Venous Anomaly**
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*Axial NECT shows dense Ca⁺⁺ in right BG <img src='img/arrows/CS.png'/> & thalamus. CE images (not shown) revealed an underlying developmental venous anomaly. These are congenital cerebral vascular malformations with mature venous elements, which may rarely have Ca⁺⁺ possibly related to underlying venous congestion & ischemia.*
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### Additional Images
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**Fahr Disease**
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*Axial NECT shows a variant CT appearance of FD with extensive Ca⁺⁺ present in the BG, cerebral WM, & at the subcortical gray matter-WM junctions.*
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**Pantothenate Kinase-Associated Neurodegeneration**
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*Axial NECT shows globus pallidus mineralization bilaterally <img src='img/arrows/CS.png'/> in a patient with pantothenate kinase-associated neurodegeneration. CT is typically normal. T2 MR shows classic the eye of the tiger appearance with globus pallidus hypointensity related to iron accumulation with medial T2 hyperintensity.*
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**CMV, Congenital**
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*Axial NECT shows marked atrophy & minimal BG Ca⁺⁺ in this child with congenital CMV. The Ca⁺⁺ seen in CMV is typically asymmetric & associated with migrational abnormalities & microcephaly.*
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**Radiation & Chemotherapy**
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*Axial NECT shows mineralizing microangiopathy related to radiation therapy & chemotherapy for a posterior fossa medulloblastoma. Note the symmetric Ca⁺⁺ in the BG & subcortical WM.*
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**CMV, Congenital**
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*Axial NECT shows periventricular & BG Ca⁺⁺. Periventricular calcifications, ventriculomegaly, & microcephaly strongly suggest congenital CMV infection.*
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**Toxoplasmosis, Acquired**
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*Axial CECT shows an enhancing BG mass <img src='img/arrows/WS.png'/> in an AIDS patient. Post therapy, enhancing lesions typically calcify. BG is the most common location followed by thalamus, then hemispheres.*
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**Vascular Calcification (Mimic)**
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*Axial NECT shows intracranial atherosclerotic disease with extensive Ca⁺⁺ in internal carotid & middle cerebral arteries <img src='img/arrows/WS.png'/>, which mimics BG Ca⁺⁺. Posterior fossa aneurysm is partially visible.*
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**Tuberous Sclerosis Complex (Mimic)**
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*Axial NECT shows calcified subependymal nodules in the foramen of Monro & periventricular regions, which mimic BG Ca⁺⁺. These typically accompany cortical tubers <img src='img/arrows/WC.png'/>, better seen on MR.*
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**Parasites, Miscellaneous**
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*Axial CECT shows a case of paragonimiasis with a hyperdense left BG nodule <img src='img/arrows/WS.png'/>. This parasite often presents with conglomerated granulomas, which may hemorrhage. Multiple Ca⁺⁺ are common.*
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**Neurocysticercosis**
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*Axial CECT shows a calcified left putamen nodule <img src='img/arrows/CS.png'/> that represents the nodular, calcified (healed) stage of neurocysticercosis. Note the right external capsule cyst with a central "dot" representing a scolex.*
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**Pseudohypoparathyroidism**
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*Axial NECT shows diffuse calcifications within the BG & subcortical WM in a pseudohypoparathyroidism patient. There is significant imaging overlap between systemic diseases with abnormal calcium deposition.*
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**HIV, Congenital**
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*Axial NECT shows symmetric BG calcification with scattered foci of subcortical calcification. Note the typical HIV involvement of the lentiform nuclei is greater than the caudate heads.*
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