Nanri Kazunori, Okuma Misaki, Sato Saki, Yoneda Makoto, Taguchi Takeshi, Mitoma Hiroshi, Yamada Junji, Unezaki Sakae, Nagatani Tetsuo, Otsubo Shiho, Sugawara Mayumi, Tanaka Nobuyuki, Mizusawa Hidehiro
Department of Neurology, Tokyo Medical University Hachioji Medical Center, Japan.
Intern Med. 2016;55(5):449-54. doi: 10.2169/internalmedicine.55.5156. Epub 2016 Mar 1.
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.
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.
Thirty-one of 58 (53%) patients were positive for anti-GAD antibody, anti-gliadin antibody, or anti-thyroid 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.
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.
最近有报道称自身免疫性小脑共济失调是可治疗的。然而，病因不明的皮质小脑萎缩伴自身免疫相关小脑共济失调患者的比例以及免疫疗法在这些疾病中的实际疗效仍不清楚。
我们检测了58例日本小脑共济失调患者（不包括多系统萎缩、遗传性脊髓小脑共济失调、癌症患者或正在接受苯妥英治疗的患者）的自身抗体水平（包括抗麦醇溶蛋白抗体、抗谷氨酸脱羧酶（GAD）抗体和抗甲状腺抗体），并评估了免疫疗法的疗效。
58例患者中有31例（53%）抗GAD抗体、抗麦醇溶蛋白抗体或抗甲状腺抗体呈阳性。12例抗麦醇溶蛋白抗体阳性患者中有7例、4例抗GAD抗体阳性患者中有3例、6例抗甲状腺抗体阳性患者中有3例对免疫疗法反应良好，这表明接受免疫疗法的共济失调相关抗体阳性小脑共济失调患者中有59%反应良好。
一些小脑共济失调患者存在自身免疫状况，因此诊断自身免疫性小脑共济失调是这类疾病患者护理中的重要组成部分。
