论文部分内容阅读
目的 探讨小儿脱髓鞘疾病的临床特征和糖皮质激素的治疗效果。方法 总结 11例脱髓鞘患儿的临床资料和磁共振 (MRI)特点 ,观察其临床转归和糖皮质激素的治疗效应。结果 11例患儿 ,以急性或亚急性起病 ,7例起病前 1～ 4周有发热、呼吸道或消化道感染症状 ,出现神经系统异常时感染征象已消失。主要表现有乏力、嗜睡、头痛、颅神经异常和肢体瘫痪。头部MRI检查全部异常 ,表现为白质、基底节、脑干和小脑T1WI等信号、低信号病灶和T2WI高信号病灶。甲基泼尼松龙或泼尼松治疗 4～ 7天症状好转 ,4～ 12周后治愈 6例 ,好转 5例。其中 3例有复发。结论 儿童急性脱髓鞘疾病 :①多数与感染有关 ;②临床多样化 ,颅神经受累和肢体瘫痪是主要神经系统表现 ;③MRI为诊断本病的主要辅助手段之一 ;④糖皮质激素治疗有效 ,但部分患儿有复发
Objective To investigate the clinical features of children with demyelinating diseases and the therapeutic effect of glucocorticoids. Methods The clinical data and magnetic resonance (MRI) features of 11 children with demyelination were summarized and their clinical outcomes and therapeutic effects of glucocorticoid were observed. Results 11 cases of children with acute or subacute onset, 7 cases 1 to 4 weeks before onset fever, respiratory or gastrointestinal symptoms, signs of infection in the presence of nervous system has disappeared. The main manifestations of fatigue, drowsiness, headache, cranial nerve abnormalities and limb paralysis. All head MRI abnormalities, manifested as white matter, basal ganglia, brain stem and cerebellar T1WI and other signals, low signal lesions and T2WI high signal lesions. Methylprednisolone or prednisone 4 to 7 days treatment of symptoms improved, 4 to 12 weeks after the cure in 6 cases, improved in 5 cases. Three of them had recurrence. Conclusions Acute demyelinating diseases in children: ① Most are related to infection; ② Clinical diversification, cranial nerve involvement and limb paralysis are the main neurological manifestations; ③ MRI is one of the main auxiliary methods for diagnosis of this disease; ④ Glucocorticoid treatment is effective, However, some children have recurrence