肌萎缩侧索硬化感觉神经电生理和病理特点

Electrophysiology and pathology of sensory nerve in amyotrophic lateral sclerosis

目的探讨肌萎缩侧索硬化感觉神经即腓肠神经的临床电生理与病理改变特点,以明确肌萎缩侧索硬化是否伴有感觉神经的损害。方法选择确诊或拟诊的肌萎缩侧索硬化患者10例,行腓肠神经神经电生理及活检,观察神经传导速度及病理改变的特点。结果①电生理肌电图检查证实存在广泛神经源性损害,部分患者可见运动传导速度的远端潜伏期延长,正中神经为(3.21±0.76)ms,尺神经为(3.64±0.76)ms;复合肌肉动作电位(CMAP)波幅降低,正中神经为(8.62±7.20)mV,尺神经为(8.39±7.33)mV;CMAP波幅面积降低,正中神经为(18.12±10.21)ms×mV,尺神经为(17.22±16.91)ms×mV;所有患者感觉传导速度及感觉神经动作电位(SNAP)波幅均正常。②周围神经活检病理检查感觉神经完全正常,或存在轻度轴索变性、有髓神经纤维脱失的病理改变。结论肌萎缩侧索硬化患者电生理检查感觉神经正常,但病理检查可证实部分病例存在感觉神经轴索变性以及脱髓鞘改变。

To identify if there were lesions of sensory nerve in amyotrophic lateral sclerosis （ALS） and to approach the clinical electrophysiologic and pathologic characters of the sensory nerve （sural nerve） in patients of ALS. Methods 10 patients with clinically definite or probable ALS were selected and electrophysiologic examination and biopsies of sural nerve were performed to determine the changes of nerve conduction velocity and the consistency of sensory nerve pathology in ALS. Results （1） In the motor conduction study, we found prolonged distal latency, lowered complex muscle action potential （CMAP） amplitude and reduced area of CMAP amplitude in the patient of ALS. The distal latency of median nerve and cubital nerve were（3.21±0.76）ms and （3.64±0.76）ms respectively. CMAP amplitude of median nerve and cubital nerve were（8.62±7.20）mV and （8.39±7.33）mV respectively. CMAP amplitude area of median nerve and cubital nerve were （18.12±10.21） and （17.22±16.91） respectively. While the sensory conduction velocities and sensory nerve action potential （SNAP） of all the ALS patients were absolutely normal. （2） Superficial peroneal nerve biopsy performed in all patients showed absolutely normal or pathologic cbanges of mildly axonal degeneration and demyelination of sensory nerves. Conclusions Electrophysiology of sensory nerve is normal in amyotrophic lateral sclerosis, but the biopsy confirns that axonal degeneration and demyelination exist in some patients.