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NEUROLOGY 1975;25:1126
© 1975 American Academy of Neurology

Monozygotic twins with Huntington's disease in a family expressing the rigid variant

THOMAS D. BIRD, M.D. and GILBERT S. OMENN, M.D., Ph.D.

Department of Medicine, Divisions of Medical Genetics and Neurology, University of Washington School of Medicine, Seattle.

A set of male monozygotic twins concordant for Huntington's disease is described. The monozygosity of the twins and the diagnosis of Huntington's disease are well established. The twins are now age 30, and although the severity of their chorea differs, they have a similar degree of mental deficit. This family is of additional interest because the daughter of one of the twins has childhood Huntington's disease, and the mother of the twins had the adult-onset rigid variant of the disease. Such unusual families afford some insight into the variability of the clinical manifestations of this hereditary disease.

Supported by NIH grants 1 F22 NS01561–01, GM15253 and Research Career Development Award GM43122 (G.S.O.).

Received for publication October 9, 1974.

Dr. Bird's address is Division of Neurology and Medical Genetics, University of Washington, Seattle, WA 98195.




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