2022 年 39 巻 3 号 p. 195-199
The involvement of immunological mechanisms in cerebellar ataxia has long been emphasized from the viewpoint of paraneoplastic syndromes, but with the expansion of the concept of immune–mediated neurological diseases and the identification of many new autoantibodies, immune–mediated cerebellar ataxia has recently attracted attention. Immune–mediated cerebellar ataxia is also an important differential disease in the diagnosis of idiopathic cerebellar ataxia (IDCA). Although immune–mediated cerebellar ataxia is a disease that requires early therapeutic intervention, the diagnosis of immune–mediated cerebellar ataxia is often difficult and depends largely on the clinical course and neurological examinations. There have been reports of cases in which intravenous methylprednisolone therapy and intravenous immunoglobulin therapy were effective and also cases in which biologic products were successful even though the response to those therapies was poor. In the future, there are many important points for the development of treatment for immune–mediated cerebellar ataxia in Japan including the establishment of a system that enables comprehensive and rapid measurement of anti–neuronal antibodies, the development of diagnostic criteria for immune–mediated cerebellar ataxia, and the establishment of a system that enables administration of medications that have been reported to be effective overseas. In this article, we summarize recent findings regarding immune–mediated cerebellar ataxia from the perspective of anti–neuronal antibodies, which play an important role in its diagnosis.
