Bona I, Vial C, Brunet P, Couturier J C, Girard-Madoux M, Bady B, Guibaud P
Laboratoire d'Electromyographie, Hôpital Neurologique, Lyon.
Electromyogr Clin Neurophysiol. 1994 Dec;34(8):471-5.
Carpal tunnel syndrome (CTS) is very uncommon in childhood. Sixty-five cases are reported in the literature, principally due to metabolic diseases. In Mucopolysaccharidoses, prospective studies (Wraith and Alani, 1990) found a bilateral CTS in about 90%. We report four cases of Mucopolysaccharidoses, diagnosed on clinical and biological data (two cases of Hurler disease, and two cases of Hunter disease), in children aged less than five years. Each child had claw hands, without thenar atrophy. Median nerve conduction studies and electromyography confirm the CTS. Motor and sensory nerve conductions are normal in other nerves. Concentric needle studies show in two cases, on abductor pollicis brevis, spontaneous activities as repetitive discharges, fasciculations and multiplets. Median nerve stimulations reveal responses with late potentials during 70 ms due to reinnervation. The physiopathology of those carpal tunnel syndromes is discussed.
腕管综合征（CTS）在儿童中非常罕见。文献报道了65例，主要病因是代谢性疾病。在黏多糖贮积症中，前瞻性研究（Wraith和Alani，1990年）发现约90%的患者存在双侧CTS。我们报告了4例黏多糖贮积症患儿，年龄均小于5岁，根据临床和生物学资料确诊（2例Hurler病，2例Hunter病）。每个患儿均有爪形手，无鱼际肌萎缩。正中神经传导研究和肌电图证实了CTS。其他神经的运动和感觉神经传导正常。在两例患儿中，对拇短展肌进行同心针电极检查显示有自发电活动，如重复放电、肌束震颤和复合波。正中神经刺激显示由于神经再支配，在70毫秒期间出现伴有晚电位的反应。本文对这些腕管综合征的病理生理学进行了讨论。
