Krasenbrink I, Fühlhuber V, Juhasz-Boess I, Stolz E, Hahn A, Kaps M, Hero B, Blaes F
Department of Neurology, Justus-Liebig University Giessen, Giessen, Germany.
Neuropediatrics. 2007 Jun;38(3):114-6. doi: 10.1055/s-2007-985906.
Opsoclonus-myoclonus syndrome (OMS) is a rare neurological disease in childhood which can be associated with neuroblastoma. Since autoantibodies have been detected in some patients with OMS, an autoimmune etiology is suspected. We compared the prevalence of autoimmune disorders and autoantibodies in parents of children with OMS and in a group of controls of same age and sex. Autoimmune diseases were found in 15.8% of the parents of OMS children, but only in 2.0% of the controls (p<0.001) There was also an increased prevalence of autoantibodies in the OMS parents (42.8% vs. 8.0%, p<0.001). Thyroid diseases were the most frequent autoimmune diseases found, followed by inflammatory rheumatic diseases. Interestingly, the OMS parents also had significantly more autoantibodies against CNS structures than the controls (p<0.01). These findings support the autoimmune hypothesis of childhood OMS and may also hint to a genetic susceptibility for OMS.
眼阵挛-肌阵挛综合征（OMS）是一种罕见的儿童期神经系统疾病，可能与神经母细胞瘤有关。由于在一些OMS患者中检测到自身抗体，因此怀疑其病因与自身免疫有关。我们比较了OMS患儿父母与一组年龄和性别相同的对照组中自身免疫性疾病和自身抗体的患病率。在OMS患儿的父母中，15.8%患有自身免疫性疾病，而对照组中这一比例仅为2.0%（p<0.001）。OMS患儿父母中自身抗体的患病率也有所增加（42.8%对8.0%，p<0.001）。甲状腺疾病是最常见的自身免疫性疾病，其次是炎性风湿性疾病。有趣的是，OMS患儿父母中针对中枢神经系统结构的自身抗体也明显多于对照组（p<0.01）。这些发现支持了儿童OMS的自身免疫假说，也可能暗示了OMS的遗传易感性。
