PT期刊文章AU - a .麦肯盟诉答:列侬AU - t . Lotze盟- s . Tenenbaum盟- j·m·洛克非盟- m . Rensel AU - n . l .王桂萍AU - j·p·弗莱尔AU - h .小礼帽盟- j .猎人盟- b . g . Weinshenker AU - k . Krecke盟- c . f . Lucchinetti盟s . j . Pittock TI - CNS aquaporin-4自身免疫的儿童援助wnl.0000314832.24682 10.1212/01.。c6 DP - 2008 7月08年TA -神经病首页学第六PG - 93 - 100 - 71 IP - 2 4099 - //www.ez-admanager.com/content/71/2/93.short 4100 - //www.ez-admanager.com/content/71/2/93.full所以Neurology2008 08年7月;71 AB -背景:在成人患者自身抗体针对水通道aquaporin-4 (AQP4)频谱中枢神经系统炎性脱髓鞘疾病的生物标志物与偏爱视神经和脊髓(视neuromyelitis[动])。在这里,我们描述了神经、血清学和影像学与中枢神经系统相关的AQP4自身免疫在童年。方法:共有88名连续血清反应阳性的儿童被确定为NMO-IgG通过服务评价。血清自身抗体共存的75测试。临床信息可供58岁。结果:42例(73%)是白人,非洲种族和20例(34%)。平均年龄在12年(范围4-18)症状出现。57例(98%)有攻击的视神经炎(n = 48;83%)或横向脊髓炎(n = 45; 78%), or both. Twenty-six (45%) had episodic cerebral symptoms (encephalopathy, ophthalmoparesis, ataxia, seizures, intractable vomiting, or hiccups). Thirty-eight (68%) had brain MRI abnormalities, predominantly involving periventricular areas (in descending order of frequency): the medulla, supratentorial and infratentorial white matter, midbrain, cerebellum, thalamus, and hypothalamus. Additional autoantibodies were detected in 57 of 75 patients (76%), and 16 of 38 (42%) had a coexisting autoimmune disorder recorded (systemic lupus erythematosus, Sjögren syndrome, juvenile rheumatoid arthritis, Graves disease). Attacks were recurrent in 54 patients (93%; median follow-up, 12 months). Forty-three of 48 patients (90%) had residual disability: 26 (54%) visual impairment and 21 (44%) motor deficits (median Expanded Disability Status Scale 4.0 at 12 months). Conclusions: Aquaporin-4 autoimmunity is a distinctive recurrent and widespread inflammatory CNS disease in children. ADEM=acute disseminated encephalomyelitis; ANA=antinuclear antibodies; AQP4=aquaporin-4; EDSS=Expanded Disability Severity Scale; EIA=enzyme immunoassay; GAD65=glutamic acid decarboxylase; GFP=green fluorescent protein; MS=multiple sclerosis; NMO=neuromyelitis optica; NMO-IgG=neuromyelitis optica autoantibody marker; SIADH=syndrome of inappropriate antidiuretic hormone secretion; SLE=systemic lupus erythematosus; SS=Sjögren syndrome.