A rare idiopathic disease which manifests in middle age characterized by punctate areas of non-arteriosclerotic calcination in parts of the gray and dentate nuclei, particularly of smaller brain vessels. The symptoms include mental and growth retardation, dystonic movements, and athetosis. May be caused by a malfunction of the glandula parathyreoidea. May occur as a familial autosomal dominant inherited condition.
The term Fahr triad consists of symmetrical calcification of the basal ganglia, neuropsychiatric symptoms, and hypofunction of the parathyroid gland.
- T. Fahr:
Idiopathische Verkalkung der Hirngefässe.
Zentralblatt für allgemeine Pathologie und pathologische Anatomie, 1930-1931, 50: 129-133.
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