Lievens I, Fournier E, Viala K, Maisonobe T, Bouche P, Léger J-M
Centre national de référence maladies neuromusculaires rares, bâtiment Babinski, hôpital Pitié-Salpêtrière, AP-HP, 47, boulevard de l'Hôpital, 75651 Paris cedex 13, France.
Rev Neurol (Paris). 2009 Mar;165(3):243-8. doi: 10.1016/j.neurol.2008.10.018. Epub 2008 Dec 31.
Multifocal motor neuropathy is a well described condition characterized by slowly progressive, predominantly distal, asymmetric limb weakness and wasting, predominantly in the arms within an anatomical distribution of individual motor nerves, with minimal or no sensory involvement.
The aim of this retrospective study was to look for a significant reduction of the amplitude of sensory potentials in a cohort of 21 patients with defined multifocal motor neuropathy according to the Workshop Report criteria [Workshop Report, 2001. 79th ENMC International Workshop. Multifocal motor neuropathy 14-15 April 2000, Hilversum. The Netherlands. Muscle Nerve 11, 309-314], within a follow-up of at least 3 years.
Thirteen patients (62%) (Group 1) had a reduction of the amplitude of at least one sensory potential, of whom four patients had abnormalities of two or more sensory potentials, while eight patients (Group 2) had no abnormality. No significant differences were found for gender, age at onset, number of involved motor nerves, CSF protein count, presence/absence of anti-GM1 serum antibodies and response to IgIV between the two groups.
This study underlines the difficulty in defining criteria for multifocal motor neuropathies capable of distinguishing them from other chronic acquired demyelinating polyneuropathies, and mainly from multifocal acquired demyelinating sensory and motor (MADSAM) neuropathy, also called Lewis-Sumner's syndrome.
多灶性运动神经病是一种已被充分描述的疾病,其特征为缓慢进展,主要是远端、不对称的肢体无力和萎缩,主要累及上肢,呈单个运动神经的解剖分布,感觉受累极少或无感觉受累。
本回顾性研究的目的是,根据研讨会报告标准[研讨会报告,2001年。第79届ENMC国际研讨会。多灶性运动神经病,2000年4月14 - 15日,荷兰希尔弗瑟姆。《肌肉与神经》11卷,第309 - 314页],在至少3年的随访期内,观察21例确诊为多灶性运动神经病的患者队列中感觉电位波幅是否有显著降低。
13例患者(62%)(第1组)至少有一个感觉电位波幅降低,其中4例患者有两个或更多感觉电位异常,而8例患者(第2组)无异常。两组在性别、发病年龄、受累运动神经数量、脑脊液蛋白计数、抗GM1血清抗体的有无以及对静脉注射免疫球蛋白的反应方面均未发现显著差异。
本研究强调了在定义多灶性运动神经病标准方面的困难,这些标准要能将其与其他慢性获得性脱髓鞘性多发性神经病区分开来,主要是与多灶性获得性脱髓鞘性感觉和运动(MADSAM)神经病(也称为刘易斯 - 萨姆纳综合征)区分开来。