Mano Y, Nakamuro T, Ikoma K, Takayanagi T, Mayer R F
Department of Neurology, Nara Medical University, Japan.
Electromyogr Clin Neurophysiol. 1993 Mar;33(2):101-7.
Central and peripheral motor nerve conduction were analyzed in 13 patients with hereditary demyelinating motor and sensory neuropathy using central magnetic stimulation and peripheral electrodiagnostic techniques. All patients showed a marked decrease in peripheral nerve conduction velocity. In 11 patients, the central motor conduction time was slightly prolonged but in 2 it was markedly prolonged suggesting dysfunction of the corticospinal tract. These two patients exhibited marked weakness and atrophy of distal muscles without clinical signs of upper motor neuron dysfunction, which was considered to be masked by the lower motor neuron disorder. This study suggests that in some patients with hereditary demyelinating polyneuropathy central as well as peripheral nerve fibers may be affected.
采用中枢磁刺激和外周电诊断技术，对13例遗传性脱髓鞘运动和感觉神经病患者的中枢和外周运动神经传导进行了分析。所有患者外周神经传导速度均显著降低。11例患者中枢运动传导时间稍有延长，但2例显著延长，提示皮质脊髓束功能障碍。这2例患者表现为远端肌肉明显无力和萎缩，无临床上运动神经元功能障碍的体征，认为是被下运动神经元疾病掩盖。本研究提示，在一些遗传性脱髓鞘性多发性神经病患者中，中枢和外周神经纤维可能均受影响。
