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Juvenile Huntington chorea

Clinical, ultrastructural, and biochemical studies

Division of Neuropathology and Department of Neurology, University of Göttingen, West Germany; and the Departments of Medicine and Pathology (Neuropathology Division), Albert Einstein College of Medicine, Bronx, New York.

A brain biopsy from a 20-year-old patient whose clinical course was marked by progressive dementia and chorea since age 10 years showed increased amounts of lipofuscin, abnormal mitochondria, and other organelles in cortical neurons, neurites, and astrocytes. Juvenile Huntington chorea was confirmed at autopsy. High levels of three histone-like proteins (molecular weight 10,000 to 16,000) in the microsomal fraction of purified neurons were found by SDS-polyacrylamide gel electrophoresis. Fatty acids were abnormal in white matter sphingomyelin. These ultrastructural and biochemical findings conformed to those established in adult Huntington chorea, thus strengthening the concept of a uniform pathologic process in adult and juvenile Huntington diseases in spite of some clinical and histologic differences.

Requests for reprints should be addressed to Dr. I. Tellez-Nagel, Department of Medicine, Albert Einstein College of Medicine of Yeshiva University, 1300 Morris Park Avenue, Bronx, NY 10461.

This paper was presented in part at the annual meeting of the American Association of Neuropathologists, San Francisco, June 1976.

These investigations were supported by Deutsch Forschungsgemeinschaft (SFB 33) and NIH - GM-19100-4836 and NS-08180.

Accepted for publication January 31, 1977.

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