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NEUROLOGY 1984;34:187
© 1984 American Academy of Neurology

Neurochemical abnorrnalities in a patient with ataxia- telangiectasia

Thomas L. Perry, MD, Stephen J. Kish, PhD, David Hinton, MD, Shirley Hansen, BA, Laurence E. Becker, MD and Erwin W. Gelfand, MD

From the Department of Pharmacology (Dr. Perry and Mrs. Hansen), University of British Columbia, Vancouver, Canada, The Clarke Institute of Psychiatry (Dr. Kish), and the Hospital for Sick Children (Drs. Hinton, Becker, and Gelfand), Toronto, Canada.

We describe biochemical abnormalities found in autopsied brain of a patient with ataxia-telangiectasia. Neuropathologic changes were limited to the cerebellum and spinal cord. The atrophic cerebellum showed marked loss of Purkinje's and granule cells, and moderate loss of stellate and basket cells. Glutmic acid content was markedly reduced, and taurine content somewhat reduced in the cerebellar cortex, while {gamma}-aminobutyric acid (GABA) content was greatly reduced in the dentate nucleus. GABA receptor binding was reduced by 70% in cerebellar cortex. Phosphoethanolamine content was greatly reduced in the cerebellar cortex and inferior olivary nucleus. This compound was also deficient in 10 other brain regions and was the only extracerebellar neurochemical abnormality observed.

Address correspondence and reprint requests to Dr. Perry, Department of Pharmacology, Faculty of Medicine, University of British Columbia, Vancouver, Canada, V6T 1W5.

Supported by the Medical Research Council of Canada, the Huntington Society of Canada, and by the Clarke Institute of Psychiatry. S.J.K. is a career scientist of the Ontario Ministry of Health.

Accepted for publication May 23, 1983.




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