Fahr Syndrome, A Rare Neurodegenerative Disorder


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Abstract

Fahr syndrome is known by idiopathic basal ganglia and extrabasal ganglia calcification, which is clinically manifested by movement disorders, dementia, behavioral disturbances, and cognitive impairments. The pathogenesis of this syndrome is still a matter of debate. Although abnormalities in calcium-phosphorus metabolism have been proposed as the possible etiology, no evidence is still provided. We report on 3 patients with basal ganglia calcification without either a familial trait or hypoparathyroidism. The focus of this paper is to illustrate extensive calcification of white and gray matter of the cerebellum in Fahr syndrome, which has not yet been reported in the literature and also to emphasize epilepsy as a rare feature of this syndrome.

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