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September 1961

Idiopathic Nonarteriosclerotic Calcification of Cerebral Vessels: Fahr's Disease—A Clinical and Histochemical Study

Author Affiliations

From the Department of Neurology and the Laboratory of Neuropathology, University of Michigan Medical Center.

Arch Neurol. 1961;5(3):279-286. doi:10.1001/archneur.1961.00450150045006

The present article reports clinical data and a histochemical analysis of a typical case of Fahr's disease.1 Fahr's disease is an idiopathic nonarteriosclerotic symmetrical calcification of cerebral vessels, with predilection for certain regions of the brain, such as dentate nucleus, basal ganglia, cerebellar and cerebral cortex. The disease has been recognized as a histopathological entity and a considerable number of typical cases have been reported in the literature. Surveys are found in the articles of Weimann,2 Meyer,3 Volland,4 and Beyme.5 A familial occurrence of the disease has been reported (Fritzsche,6 and Laubenthal and Hallervorden7). Occasionally, the calcifications were observed in x-ray examinations (Fritzsche,6 Pierach,8 Chavany, and van Bogaert and Houdart9).

Clinical Features  Fahr's disease may present with numerous neurologic and psychiatric symptoms. In a review of 52 cases, Nayrac, Graux, Pruvot, and Descamps10 found that the disorder was most

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