Tashiro K, Inoue I, Ohyagi Y, Osoegawa M, Fujimoto M, Furuya H, Yamada T, Kira J
Department of Neurology, Neurological Institute, Graduate School of Medical Sciences, Kyushu University.
Rinsho Shinkeigaku. 2000 Jul;40(7):732-5.
A 72-year-old man noticed progressive weakness of both upper limbs, more severe on the left side, 9 months after an electric shock of a 20,000 V alternating current. He had diffuse scars of superficial burns with skin graft in four limbs, more on the right side. A neurological examination revealed diffuse muscle atrophy, weakness and fasciculation in both upper limbs, predominantly on the left side, hyper-reflexia in four limbs with mildly exaggerated jaw jerk, left Babinski sign, and mild decrease of touch and pain sensation in the right C6 and C7 segments. Painful dysesthesia was present in the left hand and right lower limb. The search for serum antibodies against GM1, GM2, GM3, GD1a, Gd1b, GQ1b, GA1, and GT1b was negative. No abnormality except mild cervical spondylotic changes was evident in the magnetic resonance imaging of the brain and spinal cord. The upper limb motor evoked potentials (MEPs) were not elicited by the left cortical stimulation and the central motor conduction time by the right cortical stimulation was remarkably prolonged in the upper limb MEPs. Nerve conduction study showed a delay of motor conduction velocity and distal latency in the right median and bilateral ulnar nerves with low amplitude and delayed velocity of sensory nerves of those nerves. Needle EMG revealed diffuse ongoing denervation potentials in bilateral upper limbs and giant motor unit potentials in the right triceps and first dorsal interossei muscles. These findings indicate that the delayed motor neuron syndrome induced by electrical shock is characteristic for having demyelination as well as axonal changes in both central and peripheral nervous systems.
一名72岁男性在遭受20000伏交流电电击9个月后,发现双侧上肢进行性无力,左侧更为严重。他四肢有弥漫性浅表烧伤瘢痕,右侧更多处进行了皮肤移植。神经系统检查发现双侧上肢弥漫性肌肉萎缩、无力和肌束震颤,以左侧为主,四肢腱反射亢进,下颌反射轻度亢进,左侧巴宾斯基征阳性,右侧C6和C7节段触觉和痛觉轻度减退。左手和右下肢存在疼痛性感觉异常。检测血清中抗GM1、GM2、GM3、GD1a、Gd1b、GQ1b、GA1和GT1b抗体均为阴性。脑和脊髓的磁共振成像除轻度颈椎病改变外无明显异常。左侧皮层刺激未引出上肢运动诱发电位(MEP),右侧皮层刺激的上肢MEP中枢运动传导时间显著延长。神经传导研究显示右侧正中神经和双侧尺神经运动传导速度延迟、远端潜伏期延长,这些神经的感觉神经波幅低且速度延迟。针极肌电图显示双侧上肢弥漫性失神经电位,右侧肱三头肌和第一背侧骨间肌出现巨大运动单位电位。这些发现表明,电击诱发的迟发性运动神经元综合征的特征是中枢和周围神经系统均存在脱髓鞘以及轴突改变。