Traba A, Esteban A
Sección de Neurofisiología Clínica, Hospital General Gregorio Marañón, Madrid.
Neurologia. 1993 Feb;8(2):59-65.
A series of 4 patients with pure, chronic and progressive motor neuropathy whose main clinical characteristics were asymmetric and distal weakness of the upper limbs, myokymia and fasciculations is presented. There were no sensory impairment and amyotrophy was observed in only one case. This picture suggested the diagnosis of motor neuron disease (MND). However, neurophysiologic examination demonstrated the presence of multifocal conduction blocks (CB) of the motor axons which were preferentially located in the proximal nerve segments and always at points atypical to nerve compression. The peripheral sensitive conductions and the somatosensory evoked potentials were normal, even through the nerve segments where the CB were located. Since this is a treatable potentially reversible syndrome, this motor neuropathy with CB should be included in the differential diagnosis of MND.
本文报告了4例单纯性、慢性进行性运动神经病患者，其主要临床特征为上肢不对称性远端无力、肌束震颤和肌纤维颤搐。无感觉障碍，仅1例观察到肌萎缩。此表现提示运动神经元病（MND）的诊断。然而，神经生理学检查显示运动轴突存在多灶性传导阻滞（CB），其优先位于近端神经节段，且总是在非典型的神经受压部位。即使在存在CB的神经节段，周围感觉传导和体感诱发电位仍正常。由于这是一种可治疗的潜在可逆综合征，这种伴有CB的运动神经病应纳入MND的鉴别诊断。
