McDonald D G, Farrell M A, McMenamin J B
Department of Neurology, Our Lady's Hospital for Sick Children, Dublin, Ireland.
Eur J Paediatr Neurol. 2000;4(6):283-7. doi: 10.1053/ejpn.2000.0381.
A 9-year-old girl presented with a 4-week history of right upper limb weakness. Clinical assessment and neurophysiological studies suggested an atypical brachial plexitis. She re-presented 3 1/2 years later with progressive muscle weakness involving both legs and left arm and hand. There had been no interval improvement in her right upper limb. Clinical, neurophysiological and pathological findings were consistent with chronic inflammatory demyelinating polyneuropathy. She responded to a single course of intravenous immunoglobulin--and review more than 6 years after treatment confirms that she remains functionally normal. Focal upper limb neuropathy preceding a diffuse demyelinating process by several years has not been previously described in a child. Long-term follow-up of this patient allows us to comment on the natural history of her condition and the apparent long-term efficacy of intravenous immunoglobulin in this case.
一名9岁女孩出现右上肢无力4周的病史。临床评估和神经生理学研究提示为非典型臂丛神经炎。3年半后，她再次就诊，出现累及双腿及左臂和手部的进行性肌无力。其右上肢无力无好转。临床、神经生理学及病理学检查结果均符合慢性炎症性脱髓鞘性多发性神经病。她接受了一个疗程的静脉注射免疫球蛋白治疗，治疗后6年多的复查证实其功能仍正常。儿童中此前未见局灶性上肢神经病先于弥漫性脱髓鞘过程数年出现的情况。对该患者的长期随访使我们能够对其病情的自然史以及静脉注射免疫球蛋白在该病例中的明显长期疗效进行评论。
