论文部分内容阅读
目的探讨儿童多灶性运动神经病(MMN)的特点及临床与电生理诊断标准。方法对1例临床表现为慢性进行性、非对称性肢体无力的MMN患儿进行临床、肌电图神经传导检查及腓肠神经活检。结果该MMN患儿为8岁男童,临床主要表现为慢性非对称性、进行性肢体无力3a,以右上肢为重,受累肢体肌肉萎缩,肌容积减少,上肢重于下肢,远端重于近端,感觉检查未见异常。右尺神经和右胫神经复合肌肉动作电位负波面积近端较远端均减少50%以上,2条神经出现波形离散,提示运动神经传导阻滞。腓肠神经活检未见特征性病理改变,患儿经用大剂量IVIG治疗后右手力量增加,遗留有明显的肌肉萎缩和轻度握力减弱,但无明显感觉减退。结论 MMN虽多见于成人,但亦可在儿童起病,神经传导检查存在运动神经传导阻滞是确诊的重要依据,本病需与慢性炎症性脱髓鞘性多神经病、Lewis-Sumner综合征及轴索型MMN等鉴别,治疗上首选大剂量IVIG。
Objective To investigate the characteristics of children with multifocal motor neuropathy (MMN) and clinical and electrophysiological diagnostic criteria. Methods A clinical, electromyogram nerve conduction test and sural nerve biopsy were performed in 1 patient with MMN with chronic progressive and asymmetric limb weakness. Results The MMN children were 8 years old. The main clinical manifestations were chronic asymmetry, progressive limb weakness 3a, right upper limb weight, affected limb muscle atrophy, decreased muscle volume, upper limbs heavier than lower limbs, and distal heavier Near the end, no abnormal feeling check. Right foot ulnar nerve and right tibial nerve complex muscle action potential negative side of the proximal more than 50% reduction in the distal part of the two neurological waveform discrete, suggesting that motor nerve block. Sural nerve biopsy showed no characteristic pathological changes in children treated with high-dose IVIG right hand strength increased, leaving a clear muscle atrophy and mild grip weakening, but no significant loss of feeling. Conclusion MMN is more common in adults, but also in children onset, nerve conduction check for the presence of motor nerve block is an important basis for the diagnosis, the disease with chronic inflammatory demyelinating polyneuropathy, Lewis-Sumner syndrome and Axonal MMN identification, the treatment of choice for high-dose IVIG.