After stumbling over a CT of a slightly parkinsonian woman with typical calcifications, I tried to read up on Fahr’s disease aka striopallidodentate calcinosis. Tried – i.e., unsuccessfully. Here is an article about it. And here is a review, which I cannot download. I did not find a proper entry in my standard textbooks the goes further than to add it to the list of metabolic symptomatic Parkinson syndromes. There are a lot of case reports along the lines “We saw it and then we read up on it”, but nothing really systematic.
Here are the highlights:
- Require calcifications in the dentate nucleus and the basal ganglia, possibly more.
- Expect Parkinsonism and ataxia, possibly more.
- There is a connection to Ca metabolism disorders, in particular hypoparathyroidism and pseudohypothyroidism, possibly also due to the treatment of these disorders.
- Guess what: there are familial and sporadic forms.
- Guess what: no proper treatment.