自己免疫性小脳性運動失調症からのアプローチ

Approach from a Perspective of Autoimmune Cerebellar Ataxia

本邦から提唱された特発性小脳失調症（IDCA）には，多様な疾患が含まれる可能性がある。われわれはIDCAと診断された患者の一部の血清から抗小脳抗体が検出され，IDCAの中に自己免疫性小脳性運動失調症が含まれていることを確認した。今後，治療が困難と考えられてきた患者の中から，抗小脳抗体陽性例を見出し，免疫療法の有効性を確認する必要がある。

Abstract

Recently, the diagnostic criteria for idiopathic cerebellar ataxia (IDCA) have been proposed in Japan as a diagnosis to replace the clinical concept of cortical cerebellar atrophy, which was originally described as a neuropathological disorder. However, IDCA proposed in Japan may include various diseases such as multiple system atrophy with early stage, rare hereditary ataxias, and autoimmune-mediated cerebellar ataxia. We tackled this significant clinical challenge by detecting anti-cerebellar autoantibodies in patients' sera and identifying their target antigens. We detected anti-cerebellar autoantibodies in the sera of some patients diagnosed with IDCA in Japan. In the future, it will be necessary to confirm the efficacy of immunotherapy for anti-cerebellar autoantibody-positive cases among patients who were thought to be difficult to treat.