Nanri Kazunori, Yoshikura Nobuaki, Kimura Akio, Nakayama Sayaka, Otomo Takayuki, Shimohata Takayoshi, Terashi Hiroo, Sato Tatsuya, Yamada Junji
Tokyo Medical University Hachioji Medical Center, Department of Neurology, Tokyo Medical University Hachioji Medical Center.
Brain Nerve. 2018 Apr;70(4):371-382. doi: 10.11477/mf.1416201010.
The cerebellum is one of the main targets in the central nervous system for autoimmunity. Immune-mediated cerebellar ataxias include gluten ataxia, GAD antibody-associated cerebellar ataxia, Hashimoto's encephalopathy, and paraneoplastic cerebellar degeneration. Autoimmune cerebellar ataxia may be of either insidious or subacute onset, and vertigo or transient neurological symptoms occur in some patients before the onset of the disease, in contrast to spinocerebellar degeneration. If autoimmune cerebellar ataxia is suspected, early diagnosis and introduction of treatment are very important. For diagnosis, testing for gliadin antibody, TG6 antibody, GAD antibody, thyroid antibody, and anti-neuronal antibodies, including mGluR1, is useful. Magnetic resonance imaging voxel-based morphometry is also useful because it can detect cortical cerebellar atrophy of autoimmune cerebellar ataxia, different from spinocerebellar ataxia. As for treatment, it is important to remove autoimmune triggering factors (e.g.,dietary gluten or neoplasm). When the ataxia symptoms are causing hindrances in the daily life, it is worth considering immunotherapy including IVIg, steroid therapy and so on.
小脑是自身免疫在中枢神经系统中的主要靶点之一。免疫介导的小脑性共济失调包括麸质共济失调、GAD抗体相关小脑性共济失调、桥本脑病和副肿瘤性小脑变性。自身免疫性小脑性共济失调可能隐匿起病或亚急性起病,与脊髓小脑变性不同,部分患者在疾病发作前会出现眩晕或短暂性神经症状。如果怀疑是自身免疫性小脑性共济失调,早期诊断和开始治疗非常重要。对于诊断,检测麦醇溶蛋白抗体、TG6抗体、GAD抗体、甲状腺抗体以及包括mGluR1在内的抗神经元抗体很有用。基于磁共振成像体素的形态测量也很有用,因为它可以检测到自身免疫性小脑性共济失调的小脑皮质萎缩,这与脊髓小脑性共济失调不同。至于治疗,去除自身免疫触发因素(如饮食中的麸质或肿瘤)很重要。当共济失调症状对日常生活造成妨碍时,值得考虑包括静脉注射免疫球蛋白、类固醇治疗等在内的免疫疗法。