GLYCOGEN deposits have been demonstrated with light and electron microscope in the hindbrain areas of rabbits affected with hereditary ataxia.1,2 This raises the possibility that the underlying specific hereditary defect is in some way related to carbohydrate metabolism. Consequently, a quantitative study has been made of glycogen and of major intermediates of glycolysis in brains of ataxic rabbits. In order to assess the glycolytic capacity and metabolic status, these substances, as well as adenosine triphosphate (ATP) and phosphocreatine, were measured in two samples of tissue from each animal. One sample was frozen as quickly as possible after removal from the animal; the second sample was frozen about one minute later, ie, after a measured period of ischemia. Three enzymes concerned with glycogen metabolism and two enzymes of the pentose pathway were also measured.
The lesions of hereditary ataxia develop in a predictable pattern.3 To take advantage of this,