This is a rare neurodegenerative condition with a characteristic non-contrast CT scan shown above. Its phenotypic presentation is variable with features of parkinsonism, chorea, dystonia, cognitive impairment, and/or ataxia. Patients are between 20 and 60, and there is a familial form inherited as an autosomal dominant disease.
Challenge: What is it?
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Huntington's Disease?
ReplyDeleteHunington's ?
ReplyDeletefahr syndrome
ReplyDeleteFahr's syndrome. I had to look a long time for this one. Good case!
ReplyDeleteOh! Huntington's is a good guess, but this is indeed Fahr's syndrome. Good job!
ReplyDelete-
Near and Dear
Fahr’s Disease or idiopathic basal ganglia calcification or bilateral striatopallidodentate calcinosis is characterized by accumulation of calcium in the basal ganglia.
Sources: UpToDate; LearningRadiology.