Prevalence of Autoantibodies and the Efficacy of Immunotherapy for Autoimmune Cerebellar Ataxia

Abstract

Objective Autoimmune cerebellar ataxias were recently reported to be treatable. However, the proportion of patients with cortical cerebellar atrophy of unknown etiology with autoimmune-associated cerebellar ataxia and the actual effectiveness of immunotherapy in these diseases remain unknown. Methods We measured the level of autoantibodies (including anti-gliadin antibody, anti-glutamic acid decarboxylase (GAD) antibody, and anti-thyroid antibody) in 58 Japanese patients with cerebellar ataxia, excluding those with multiple system atrophy, hereditary spinocerebellar ataxia, cancer, or those who were receiving phenytoin, and the efficacy of immunotherapy was assessed. Results Thirty-one of 58 (53%) patients were positive for anti-GAD antibody, anti-gliadin antibody, or antithyroid antibody. Seven of the 12 anti-gliadin antibody-positive patients, three of the four anti-GAD antibody-positive patients, and three of the six anti-thyroid antibody-positive patients responded well to immunotherapy, indicating that 59% of patients with ataxia-associated antibody-positive cerebellar ataxia undergoing immunotherapy responded well. Conclusion Some patients with cerebellar ataxia have autoimmune conditions and diagnosing autoimmune cerebellar ataxia is therefore an important component in the care of patients with this disease entity.

Immune-Mediated Cerebellar Ataxias: Clinical Diagnosis and Treatment Based on Immunological and Physiological Mechanisms

“…More recently, the prevalence of GA in Asian countries has been found to be higher than was previously estimated. 22,23 For example, patients with GA have recently been reported in Japan. 23 A survey in China also showed that the prevalence rates of positive-anti-transglutaminase 6 (TG6) IgA were 35% in 100 sporadic CA patients and 9.8% in 51 healthy controls (p = 0.001).…”

Steroid-responsive Nivolumab-induced Involuntary Movement with Anti-thyroid Antibodies

“…Hashimoto's encephalopathy may present with elevated protein levels in the CSF and slow waves on EEG; however, cerebellar-type is unlikely to show such abnormalities (7). Hashimoto's encephalopathy is the most prevalent cause of autoimmune ataxia without tumors (51%) and is unlikely to include nystagmus, as observed in anti-GAD antibody-positive cerebellar ataxia or gluten ataxia (8). Cerebellar syndrome is a major type of PNS, but our patient had no antibodies for PNS; furthermore, ataxia caused by PNS does not typically disappear with the use of steroids alone.…”

A Steady Cause of Unsteadiness: A Case of Thyroid-Associated Cerebellar Ataxia

“…Neuroimaging studies are nonrevealing; however, increased level of protein in the CSF has usually been found in these cases. The nature of the autoantibodies involved in the pathogenesis of autoimmune thyroid disease and the mechanisms that cross anti-thyroid autoimmunity and encephalopathy are still undefined [7,8]. In most of the cases, the diagnosis was based on neurological symptoms, negative CSF evidence of viral or bacterial infection and presence of anti-thyroid antibodies.…”