Chad D A, Hammer K, Sargent J
Neurology. 1986 Sep;36(9):1260-3. doi: 10.1212/wnl.36.9.1260.
A 25-year-old man with multifocal weakness and fasciculation was thought to have motor neuron disease. Signs progressed for 1 year, plateaued, and 3 years later resolved almost completely. There was no evidence of paraproteinemia, lymphoproliferative disorder, or vasculitis, and myelography was normal. Electrodiagnostic study disclosed multifocal, acute and chronic denervation that evolved into a picture consistent with residuals of old multifocal radiculopathy without active denervation. Prolongation of F response, absence of H-reflex, and conduction block in a proximal nerve segment suggested multifocal demyelination. A proximal motor neuropathy, perhaps demyelinating, may cause some of the benign motor neuron syndromes that simulate motor neuron disease.
一名25岁男性,有多灶性肌无力和肌束震颤,曾被认为患有运动神经元病。症状进展了1年,之后趋于平稳,3年后几乎完全缓解。没有副蛋白血症、淋巴增殖性疾病或血管炎的证据,脊髓造影正常。电诊断研究显示多灶性急性和慢性去神经改变,演变成与陈旧性多灶性神经根病残留相符的表现,无活动性去神经改变。F波延长、H反射消失以及近端神经节段的传导阻滞提示多灶性脱髓鞘。近端运动神经病,可能是脱髓鞘性的,可能导致一些模拟运动神经元病的良性运动神经元综合征。