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NEUROLOGY 1985;35:533
© 1985 American Academy of Neurology

Dystonia and calcification of the basal ganglia

T. Andreo Larsen, MD, PhD, Henry G. Dunn, MB, FRCP(C), FRCP(Lond), James E. Jan, MD, FRCP(C) and Donald B. Calne, DM, FRCP

Division of Neurology, Department of Medicine (Drs. Larsen and Calne) and Pediatrics (Drs. Dunn and Jan), University oC British Columbia, Vancouver, BC, Canada.

We studied a family with dominantly inherited dystonia and intracranial calcification. Thirty-seven members were examined; 13 were affected by dystonia that was segmental in most patients, affecting especially the voice, face, neck, and limbs. Intracranial calcification involved the putamen, pallidum, cerebral white matter and cortex, and cerebellar nuclei. Several patients had both dystonia and calcification, but others had either dystonia or calcification alone.

Address correspondence and reprint requests to Dr. Calne, U.B.C. Health Sciences Centre Hospital, 2211 Wesbrook Mall, Vancouver, BC, V6T 1W5, Canada.

Supported by the Dystonia Medical Research Foundation and the Medical Research Council of Canada.

Accepted for publication August 6, 1984.




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