儿童急性运动轴索性多发性神经病临床-电生理特征及预后

Clinical and electrophysiological characteristics, and prognosis of acute motor axonal neuropathy in children

摘要:

目的：探讨中国南方儿童急性运动轴索性多发性神经病（acute motor axonal neuropathy, AMAN）的临床特征、电生理特点及其预后。方法：对6例AMAN患儿的临床和神经电生理资料进行分析和随访。结果：（1）患儿平均发病年龄为4.4岁，均来自农村，多有前驱感染史（5例），发病时间无明显季节性差异。肌无力为主要首发症状，病情达高峰的平均时间为4.2 d。（2）神经电生理检查：所有患儿神经传导检测均表现为运动神经反应电位波幅显著减低，降低至（22.3%～73.4%）正常值低限，四肢远端神经受损程度较近端重（P<0.05），而上、下肢神经受损程度无明显差异（P>0.05），运动神经电位传导速度和感觉神经传导正常。全部患儿给予静脉丙种球蛋白（IVIG）治疗。（3）随访3个月至1年,4例患儿已能独立行走。结论：儿童AMAN好发于农村，全年均可发病；最常见的首发症状为肌无力，多在病程早期达到疾病高峰；神经电生理对AMAN的诊断、鉴别诊断提供了客观依据；部分患儿起病后1年能恢复独立行走能力；尽早IVIG治疗有利于促进临床神经功能恢复。

Abstract:

OBJECTIVE: To investigate the clinical and electrophysiological characteristics and prognosis of acute motor axonal neuropathy (AMAN) in children in South China. METHODS: The clinical and electrophysiological data of 6 children with AMAN was analyzed, and they were followed up. RESULTS: The mean age of onset was 4.4 years. Most patients came from rural areas and 5 cases had a history of prodromal infection. There were no seasonal differences in clinical onset among the patients. The most common first symptom was muscle weakness, and the mean time from onset to the most severe disease status was 4.2 days. Nerve conduction test results revealed that all patients showed significantly lower amplitude of motor nerve action potential, only 22.3%-73.4% of the lower limit of normal. Injury to the nerves of distal extremities was more serious than injury to the nerves of proximal extremities (P<0.05), while there was no significant difference in the injury to the nerves of upper and lower extremities (P>0.05). Motor nerve conduction velocity and sensory nerve conduction velocity were normal. All patients received intravenous immunoglobulin (IVIG). Of the 6 AMAN patients, 4 could walk independently after a follow-up of 3 months to 1 year. CONCLUSIONS: AMAN in children occurs mostly in rural areas. There is no seasonal difference in the clinical onset of the disease. Muscle weakness is the most common first symptom and the worst status of AMAN appears in the early stage of the disease. Electrophysiological examination provides important information for the diagnosis of AMAN. Some children with AMAN regain the ability to walk independently 1 year after onset. Early application of IVIG treatment may help recovery of neural function.