Mano Y, Nakamuro T, Ikoma K, Takayanagi T
Department of Neurology, Nara Medical University.
Rinsho Shinkeigaku. 1990 May;30(5):487-91.
The motor system of 13 cases with hereditary motor sensory neuropathy (HMSN) type I were analysed by clinical neurophysiological method. The motor conduction velocity (MCV) and F wave latency in lower motor neurone were markedly delayed. The latency of the muscle evoked potential (MEP) by cortical magnetic stimulation were also markedly delayed. The central motor conduction times (CMCT) were calculated by two methods. CMCT-mag was calculated by subtraction of the MEP by cervical magnetic stimulation from the MEP by cortical magnetic stimulation. CMCT-f was calculated by subtraction of the [(F wave latency -1 + distal latency)/2] from the MEP by cortical magnetic stimulation. There were positive correlation between CMCT-f and CMCT-mag. CMCT of HMSN type I were divided to two groups. CMCT of the first group was markedly delayed. CMCT of the second group was mildly delayed or normal. The former group showed marked weakness in distal muscles clinically. The latter group showed mild or moderate weakness in distal muscles clinically. All these patients did not show any pyramidal tract signs, which could be covered by severe lower motor neurone involvements. The classification of HMSN type I by gene was well known, genetical analysis might be important to these groups in HMSN type I.
采用临床神经生理学方法对13例I型遗传性运动感觉神经病（HMSN）患者的运动系统进行了分析。下运动神经元的运动传导速度（MCV）和F波潜伏期明显延迟。皮层磁刺激诱发的肌肉动作电位（MEP）潜伏期也明显延迟。通过两种方法计算中枢运动传导时间（CMCT）。CMCT-mag通过皮层磁刺激的MEP减去颈磁刺激的MEP来计算。CMCT-f通过皮层磁刺激的MEP减去[（F波潜伏期-1 + 远端潜伏期）/2]来计算。CMCT-f与CMCT-mag之间存在正相关。I型HMSN的CMCT分为两组。第一组的CMCT明显延迟。第二组轻度延迟或正常。前一组临床上远端肌肉明显无力。后一组临床上远端肌肉轻度或中度无力。所有这些患者均未表现出任何锥体束征，这可能被严重的下运动神经元受累所掩盖。I型HMSN按基因分类是众所周知的，基因分析对这些I型HMSN患者群体可能很重要。
