Bilateral striopallidodentate calcification (Fahr's syndrome) and multiple system atrophy in a patient with longstanding hypoparathyroidism.

Neuropathology 2007 Oct;27(5):453-6

Institute of Neurology, Medical University of Vienna, Vienna, Austria.

Recently, a family with idiopathic brain calcification was reported, in which one family member was diagnosed with multiple system atrophy (MSA) at autopsy. We report here a case showing similar neuropathological features in a patient with longstanding hypoparathyroidism. Our female patient had a history of hypoparathyroidism with hypocalcaemia and tetany since the age of 9 years. In her 50s she developed dementia and parkinsonism. She died of myocardial infarction aged 65 years. Neuropathology showed severe brain calcifications of the Fahr type in the basal ganglia, thalami, cerebral and cerebellar white matter and dentate nuclei. Additionally, there was prominent alpha-synucleinopathy of the multiple system atrophy type (MSA). The patient has a healthy identical twin and there is no family history of hypoparathyroidism or neurological disease. Data on alpha-synuclein accumulation in various cases of Fahr's syndrome are needed to establish the correlation between alpha-synucleinopathy and bilateral striopallidodentate calcification.

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http://dx.doi.org/10.1111/j.1440-1789.2007.00790.xDOI Listing
October 2007
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