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

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