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Neurol Sci. 2010 Oct;31(5):665-7. doi: 10.1007/s10072-010-0380-8. Epub 2010 Jul 13.

Fahr's disease: variable presentations in a family.

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1
Neurology Department, Isfahan University of Medical Sciences, Isfahan, Iran.

Abstract

Fahr's disease or Striato-pallido-dentate calcification is a well-defined entity with familial or sporadic presentation and approximately two-thirds of the patients are symptomatic. Herein, we report on different manifestations and neuroimaging of six symptomatic members of a family with Fahr's disease. It is significant that three symptom-free members of this family also had brain calcification on brain CT scan. The patients' symptoms had started at different ages with diverse manifestations. The manifestations included extrapyramidal signs, behavioral abnormalities, memory deficits, atonic type of seizure, and ataxia. In further generations, symptoms started earlier.

PMID:
20625786
DOI:
10.1007/s10072-010-0380-8
[Indexed for MEDLINE]
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