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December 1984

Adult Onset Idiopathic Familial Brain Calcifications

Author Affiliations

From the Department of Neurology, Baylor College of Medicine (Drs Harati and Benjamin); the Neurology Service, Veterans Administration Medical Center (Dr Harati), Houston; and the Department of Neurology, Louisiana State University Medical School, New Orleans (Dr Jackson).

Arch Intern Med. 1984;144(12):2425-2427. doi:10.1001/archinte.1984.00350220157034

• We treated a family with idiopathic calcifications of symmetric areas of the brain, including the basal ganglia, dentate, and cerebral white matter. Dementia, progressive dysarthria, incontinence, propulsive-ataxic gait, fixed facies, and cogwheel rigidity without dysmorphic features develop in affected persons. Calcium, phosphorus, and parathyroid hormone levels were normal In the two siblings tested. The literature is reviewed and five other families with a similar syndrome are identified. These six families seem to be clinically distinct from the larger group of idiopathic cerebral calcifications usually referred to as Fahr's disease.

(Arch Intern Med 1984;144:2425-2427)