Takeguchi Masafumi, Nanri Kazunori, Okita Mitsunori, Taguchi Takeshi, Ishiko Tomoko, Saitoh Hirohiko
Department of Neurology, Tokyo Medical University Hachioji Medical Center.
Rinsho Shinkeigaku. 2006 Jul;46(7):467-74.
In slowly progressive cerebellar atrophy, it has been difficult to suppress the progression of cerebellar symptoms because no effective therapeutic agents are available when the diagnosis of secondary cerebellar atrophy, such as drug-induced cerebellar atrophy or paraneoplastic syndrome, is denied. However, amongst the different forms of slowly progressive cerebellar atrophy, some may be associated with treatable immune abnormalities. Therefore, we investigated the therapeutic efficacy of intravenous immunoglobulin (IVIg) in 9 patients with slowly progressive cerebellar atrophy (4 sporadic atrophy; 5 hereditary atrophy). The results were as follows. With regard to the 4 cases of sporadic atrophy, gait ataxia and imbalance were markedly improved in 1 patient who had positive anti-GAD antibody. Moderate improvement was seen in 1 patient and slight improvement in 2. With regard to the 5 cases of hereditary atrophy, gait ataxia and imbalance were moderately improved in 2 patients with SCA3, although there were 3 non-responders. In conclusion, our study results suggested that not only patients with sporadic atrophy but also some with hereditary atrophy may respond to therapy. In cases of slowly progressive cerebellar atrophy in which the cause may be due to immune abnormality, we should consider instituting active immunotherapy when a pathological state caused by immune abnormality is suspected after extensive evaluations of autoantibodies, including anti-GAD, anti-thyroid and anti-gliadin antibody, malignancy, and so on.
在缓慢进展性小脑萎缩中，由于在排除继发性小脑萎缩（如药物性小脑萎缩或副肿瘤综合征）的诊断后，没有有效的治疗药物，因此很难抑制小脑症状的进展。然而，在不同形式的缓慢进展性小脑萎缩中，有些可能与可治疗的免疫异常有关。因此，我们研究了静脉注射免疫球蛋白（IVIg）对9例缓慢进展性小脑萎缩患者（4例散发性萎缩；5例遗传性萎缩）的治疗效果。结果如下。对于4例散发性萎缩患者，1例抗GAD抗体阳性的患者步态共济失调和平衡能力明显改善。1例患者有中度改善，2例有轻度改善。对于5例遗传性萎缩患者，2例SCA3患者的步态共济失调和平衡能力有中度改善，尽管有3例无反应。总之，我们的研究结果表明，不仅散发性萎缩患者，而且一些遗传性萎缩患者也可能对治疗有反应。在缓慢进展性小脑萎缩病例中，如果病因可能是免疫异常，在对包括抗GAD、抗甲状腺和抗麦醇溶蛋白抗体、恶性肿瘤等自身抗体进行广泛评估后，怀疑存在免疫异常引起的病理状态时，我们应考虑采取积极的免疫治疗。
