【摘要】 目的探讨儿童视神经脊髓炎谱系疾病(p-NMOSD)的临床特点,提高对p-NMOSD的认识。方法回顾性分析2015年10月1日至2019年5月1日于吉林大学第一医院小儿神经科诊断为p-NMOSD的16例患儿临床资料,包括一般资料、血和脑脊液实验室检查、影像学改变及治疗、随访情况。结果 16例患儿均为首次确诊病例,首发症状中,视神经损害者7例,脊髓损害者6例,大脑损害者3例。脑脊液常规生化改变无特异性,水通道蛋白4抗体(AQP4-Ig G)阳性4例(4/16例),髓鞘少突胶质细胞抗体(MOG-Ig G)阳性3例(3/6例)。除1例患儿脊髓MRI正常外,其余15例患儿存在长节段脊髓炎,以颈胸受累为主。除2例患儿头部MRI正常外,其余14例患儿头部MRI异常,均存在幕上病变,以近皮层及皮层下白质病灶多见。经激素及人免疫球蛋白治疗,所有患儿均缓解,但部分患儿存在复发。结论 p-NMOSD患儿常以视神经损害起病,长节段横贯性脊髓病灶具有特征性,此类患儿应常规行AQP4-Ig G及MOG-Ig G检查,及早干预,减少疾病复发。更多还原

【Abstract】 Objective To investigate the clinical characteristics of pediatric neuromyelitis optica spectrum disorder(p-NMOSD) so as to help raise awareness of p-NMOSD. Me thods A retrospective analysis of 16 children diagnosed with p-NMOSD in our department from October 2015 to May 2019 was performed. The clinical data including general information, blood and cerebrospinal fluid examinations, imaging tests, therapy and prognosis were reviewed. Re s ults All 16 cases were diagnosed for the first time. Among the initial symptoms,there were 7 cases of optic nerve damage, 6 cases of spinal cord damage and 3 cases of brain damage. There were no specific biochemical changes in CSF. Four cases( 4/16 cases) were positive for aquaporin-4 antibody(AQP4-IgG) and 3 cases(3/6 cases) positive for myelin oligodendrocyte glycoprotein antibody(MOG-IgG).The spinal MRI was normal in 1 patient, but showed longitudinally extensive transverse myelitis, mainly involving the neck and chest in the rest 15 patients. The cranial MRI was normal in 2 cases, but showed upratentorial lesions in the rest 14 head MRI, most of which were proximal and subcortical white matter lesions. After hormone and human immunoglobulin treatment, all children were relieved, but some relapsed. Conclus ion The p-NMOSD often begins with optic nerve damage, and longitudinally extensive transverse myelitis is its main feature. These patients should be routinely examined for AQP4-IgG and MOG-IgG for early intervention to reduce the recurrence rate.更多还原