Monday, January 16, 2012

Near and Dear

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?

Image shown under Fair Use.


Reflex Hammer said...

Huntington's Disease?

Anonymous said...

Hunington's ?

Anonymous said...

fahr syndrome

Anonymous said...

Fahr's syndrome. I had to look a long time for this one. Good case!

Craig Chen said...

Oh! Huntington's is a good guess, but this is indeed Fahr's syndrome. Good job!
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.