Autoantibodies to glutamate decarboxylase in a patient with cerebellar cortical atrophy, peripheral neuropathy, and slow eye movements

J. Honnorat, P. Trouillas, C. Thivolet, M. Aguera and M. F. Belin
Ataxia Research Center, Hopital Neurologique, Lyons, France.

OBJECTIVE: To study the existence of autoimmunity against the cerebellum in patients with sporadic cortical cerebellar atrophy. DESIGN: The presence of autoantibodies against the cerebellum in the serum and cerebrospinal fluid samples that were obtained from patients with sporadic cortical cerebellar atrophy and control patients was investigated by using immunohistochemical techniques. SETTING: University hospital and 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 cerebrospinal fluid anti-cerebellar autoantibodies were investigated by using indirect immunofluorescence techniques in rat cerebellum. To characterize antigen labeled by patient's serum, we used an immunotrapping enzyme activity assay of glutamate decarboxylase. RESULTS: Serum and cerebrospinal 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 autoantibodies. CONCLUSIONS: These results suggest a participation of autoimmunity in the pathogenesis of some cases of sporadic cerebellar cortical atrophy and the involvement of the cerebellar gamma-aminobutyric acid-ergic system in the pathogenesis of this disease.