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September 1982

Computed Tomographic Visualization of Extensive Calcinosis in a Patient With Idiopathic Familial Basal Ganglia Calcification

Author Affiliations

Department of Neurology St Louis Veterans Administration Medical Center and St Louis University School of Medicine South Grand Boulevard St Louis, MO 63125
Departments of Neurology and Radiology University of Pittsburgh School of Medicine Pittsburgh, PA 15240

Arch Neurol. 1982;39(9):603. doi:10.1001/archneur.1982.00510210073030

To the Editor.—  We wish to describe the computed tomographic (CT) manifestations of a patient with idiopathic familial basal ganglia calcification. A detailed history of this patient and his family can be found in earlier reports.1,2

Report of a Case.—  A 45-year-old man was brought to the St Louis Veterans Administration Hospital in June 1976. Progressive slowing of mentation had started when he was 36 years old. For several years before admission, grand mal seizures had been frequent. He had become irritable and irrational.When he was 39 years old, bilateral basal ganglia calcification was found by skull roentgenography. His mother had similar symptoms and was also found to have basal ganglia calcification. His children and his sister's children also had roentgenographic evidence of basal ganglia calcification, but none of them had displayed neurologic deficits.On examination, the patient appeared dull and unkempt. His recent memory was markedly impaired.

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