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Fahr's Disease: Variable Presentations in a Family Publisher Pubmed



Ashtari F1 ; Fatehi F1
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  1. 1. Neurology Department, Isfahan Neuroscience Research Center, Isfahan University of Medical Sciences, Isfahan, Iran

Source: Neurological Sciences Published:2010


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. © 2010 Springer-Verlag.
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