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目的探讨儿童急性播散性脑脊髓炎(ADEM)的临床特征及复发因素。方法 2003年2月-2009年12月,本院神经内科共收治符合ADEM诊断标准并完成随访的48例患儿,分析其临床资料、实验室检查、神经影像学特征及随访结果,总结其临床特征及复发因素。结果 48例ADEM患儿中,男22例,女26例;发病年龄1岁1个月~12岁6个月,中位年龄6.3岁。发病前驱期有感染症状31例(占65%),疫苗接种史4例(占8%),无明显诱因13例(占27%)。临床主要以运动障碍(46例)、精神障碍(21例)、头痛头晕(21例)及意识障碍(15例)为主要表现。MRI以皮质下白质、半卵圆中心、侧脑室周围、基底核、脑干及脊髓损害为主,呈点片状或稍长T1、长T2信号。8例为第2次复发或多次复发。其复发与视神经症状、头颅MRI上显示幕上和幕下病灶同时存在、脊髓病灶及视觉诱发电位异常有关。随访结果:34例完全缓解,无任何神经系统后遗症;8例复发;14例遗留神经功能缺损,主要以运动障碍为主。结论儿童ADEM临床表现多样,视神经损害及磁共振成像显示病灶的部位对复发的判断有一定的临床价值。ADEM长期预后大多良好,少数患儿遗留神经功能缺损。
Objective To investigate the clinical features and relapse factors of childhood acute disseminated encephalomyelitis (ADEM). Methods From February 2003 to December 2009, 48 children with ADEM diagnostic criteria were followed up. The clinical data, laboratory tests, neuroimaging features and follow-up results were analyzed. The clinical results Features and recurrence factors. Results Among 48 ADEM children, there were 22 males and 26 females. The age of onset was from 1 year old to 12 years old and 6 months old. The median age was 6.3 years old. There were 31 cases (65%) of the symptoms of infection in the prodromal period, 4 cases (8%) of the vaccination history and 13 cases (27%) without the obvious predisposition. Clinical mainly with movement disorders (46 cases), mental disorders (21 cases), headache and dizziness (21 cases) and disturbance of consciousness (15 cases) as the main performance. MRI showed subcortical white matter, semi-oval center, lateral ventricle, basal ganglia, brainstem and spinal cord lesions with flaky or slightly longer T1 and longer T2 signals. 8 cases of the second recurrence or multiple recurrence. The recurrence and optic nerve symptoms, head MRI showed on the screen and infratentorial lesions exist, spinal lesions and visual evoked potential abnormalities. Follow-up results: 34 cases of complete remission, no neurological sequelae; 8 cases of recurrence; 14 cases of neurological deficits, mainly dyskinesia. Conclusion The clinical manifestations of ADEM in children are diverse. The optic nerve damage and magnetic resonance imaging show that the site of the lesion has certain clinical value in the judgment of recurrence. ADEM long-term prognosis are mostly good, a small number of children with neurological deficits.