AUTOANTIBODIES TO GLUTAMATE-DECARBOXYLASE IN A PATIENT WITH CEREBELLAR CORTICAL ATROPHY, PERIPHERAL NEUROPATHY, AND SLOW EYE-MOVEMENTS

Objective: To study the existence of autoimmunity against the cerebell um in patients with sporadic cortical cerebellar atrophy. Design: The presence of autoantibodies against the cerebellum in the serum and cer ebrospinal fluid samples that were obtained from patients with sporadi c cortical cerebellar atrophy and control patients was investigated by using immunohistochemical techniques. Setting: University hospital an d research laboratory in Lyons, France. Patients: Eight patients with cortical cerebellar atrophy that was associated with or without other neurological symptoms; 350 patients with various neurological diseases ; and 33 normal, healthy subjects. Outcome Measures: Serum and cerebro spinal fluid anti-cerebellar autoantibodies were investigated by using indirect immunofluorescence techniques in rat cerebellum. To characte rize antigen labeled by patient's serum, we used an immunotrapping enz yme activity assay of glutamate decarboxylase. Results: Serum and cere brospinal fluid samples that were taken from one patient with sporadic cortical cerebellar atrophy associated with peripheral neuropathy and slow eye movements contained anti-glutamate decarboxylase autoantibod ies. Conclusions: These results suggest a participation of autoimmunit y in the pathogenesis of some cases of sporadic cerebellar cortical at rophy and the involvement of the cerebellar gamma-aminobutyric acid-er gic system in the pathogenesis of this disease.