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Last Update: 04/01/2024 02:31 AM
Current Deck: Neurology::RITE Images
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What condition is this image consistent with? {{c1::Fahr Disease (FD) }}
Notes
Also Known As: Cerebrovascular Ferrocalcinosis, Bilateral Striopallidodentate Calcification
Description:
- Rare degenerative neurological disorder
- Extensive bilateral basal ganglia (BG) calcifications (Ca++)
- May include progressive dystonia, parkinsonism, and neuropsychiatric manifestations
Imaging:
- Bilateral symmetric Ca++ deposits in BG, thalami, dentate nuclei, and cerebral white matter on CT scans
Top Differential Diagnoses:
- Normal (physiologic): Symmetrical BG Ca++ in middle-aged and elderly patients
- Pathologic BG Ca++ (e.g., endocrinological)
- Fahr Disease is often familial but exhibits heterogeneity
- Characteristic feature: Diffuse neurofibrillary tangles with calcification (a.k.a. Fahr-type calcification)
Clinical Issues:
- Most common signs/symptoms include:
- Neuropsychiatric disturbance
- Cognitive impairment (subcortical dementia)
- Extrapyramidal movement disorders
- Ca++/P metabolism and parathyroid hormone levels are normal
- Bimodal pattern of clinical onset:
- Early adulthood (schizophrenic-like psychosis)
- 6th decade (extrapyramidal syndrome, subcortical dementia)
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