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  • Title: Adult onset idiopathic familial brain calcifications.
    Author: Harati Y, Jackson JA, Benjamin E.
    Journal: Arch Intern Med; 1984 Dec; 144(12):2425-7. PubMed ID: 6508450.
    Abstract:
    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.
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