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A possible mechanism for selective cerebellar damage in partial pyruvate dehydrogenase deficiency

From the departments of Psychiatry and Biological Chemistry and the Mental Retardation Research Center, UCLA School of Medicine, Los Angeles, California 90024.

In patients with partial deficiencies of pyruvate dehydrogenase, cerebellar ataxia has been the most prominent and sometimes the only neurologic abnormality. It is not clear how this generalized enzyme deficiency (with activity 15 to 30 percent of normal in several tissues) might lead to clinical signs referable to a limited part of the nervous system. We therefore compared the normal activity of pyruvate dehydrogenase with the normal rate of pyruvate oxidation in different parts of animal brains and then calculated the effect on pyruvate oxidation of partial deficiencies of the enzyme. The data indicate that pyruvate oxidation could be impaired in an area of anterior cerebellar vermis by deficiencies of pyruvate dehydrogenase too mild to affect pyruvate oxidation in the other areas of the brain we examined.

Requests for reprints should be addressed to Dr. Blass, Department of Psychiatry, UCLA School of Medicine, 760 Westwood Plaza, Los Angeles, CA 90024.

These studies were supported by grants HD 05061, HD 34504, GM 00364, and HD 461205 and by the California State Department of Mental Hygiene.

Received for publication October 10, 1975.

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