Bouché P, Gherardi R, Cathala H P, Lhermitte F, Castaigne P
J Neurol Sci. 1983 Oct-Nov;61(3):389-99. doi: 10.1016/0022-510x(83)90172-7.
144 patients with the clinical syndrome of peroneal muscular atrophy or Charcot-Marie-Tooth disease were studied. Thirteen were recognized as the spinal form of the disease since all had normal motor nerve conduction velocity and sensory nerve action potential. The remaining patients could be classified according to their values of motor conduction velocity for the median nerve. Two main groups were therefore identified: 55 patients whose nerve conduction was below 30 m/s belonged to group I, which corresponds to the previously reported hypertrophic form or hereditary motor sensory neuropathy (HMSN) type I. Sixty-four patients whose nerve conduction was above 40 m/s belonged to group II which corresponds in the majority of cases to the neuronal form or HMSN type II. Twelve patients could not be classified since the motor nerve conduction velocity for median nerve ranged between 30 and 40 m/s. These could belong to either of the two main groups or might form an intermediate group, the existence of which is discussed. Clinical genetic and electrophysiological features of the two main groups are discussed and compared.
对144例患有腓骨肌萎缩症或夏科-马里-图思病临床综合征的患者进行了研究。其中13例被认定为该病的脊髓型，因为他们的运动神经传导速度和感觉神经动作电位均正常。其余患者可根据正中神经的运动传导速度值进行分类。因此确定了两个主要组：55例神经传导速度低于30米/秒的患者属于第一组，这与先前报道的肥厚型或遗传性运动感觉神经病（HMSN）I型相对应。64例神经传导速度高于40米/秒的患者属于第二组，在大多数情况下，这与神经元型或HMSN II型相对应。12例患者无法分类，因为正中神经的运动神经传导速度在30至40米/秒之间。这些患者可能属于两个主要组中的任何一组，也可能构成一个中间组，文中对该中间组的存在进行了讨论。对两个主要组的临床、遗传和电生理特征进行了讨论和比较。
