Dubuisson Nicolas J, Van Pesch Vincent, Van Den Bergh Peter Y K
Neuromuscular Reference Center, Cliniques universitaires Saint-Luc Avenue Hippocrate 10/13.11 1200 Brussels, Belgium.
Department of Neurology, University Hospitals St-Luc, University of Louvain, Brussels, Belgium.
Muscle Nerve. 2017 Oct;56(4):828-832. doi: 10.1002/mus.25528. Epub 2017 Mar 23.
Cramp-fasciculation syndrome is a peripheral nerve hyperexcitability disorder, which could be caused by inflammatory neuropathy.
We describe a 51-year-old woman who presented with a 4- to 5-year history of fasciculations and painful cramping of the right thenar eminence.
Electrophysiological studies showed motor conduction block in the right median nerve between the axilla and the elbow with fasciculation potentials and cramp discharges on electromyography in the right abductor pollicis brevis muscle. High titers of serum anti-GM1 immunoglobulin M antibodies were detected.
Monofocal motor neuropathy of the right median nerve was diagnosed. Intravenous immunoglobulin treatment led to significant improvement of symptoms and signs. Although fasciculations and cramps have been reported in multifocal motor neuropathy and are considered supporting criteria for the diagnosis, the occurrence of cramp-fasciculation syndrome as the presenting feature and predominant manifestation in monofocal motor neuropathy, a variant of multifocal motor neuropathy, is unique. Muscle Nerve 56: 828-832, 2017.
痛性肌束震颤综合征是一种周围神经兴奋性亢进性疾病，可由炎性神经病引起。
我们描述了一名51岁女性，她有4至5年的右手鱼际肌束震颤和疼痛性痉挛病史。
电生理研究显示右侧正中神经在腋窝和肘部之间存在运动传导阻滞，右侧拇短展肌肌电图显示有肌束震颤电位和痉挛放电。检测到高滴度的血清抗GM1免疫球蛋白M抗体。
诊断为右侧正中神经单灶性运动神经病。静脉注射免疫球蛋白治疗使症状和体征有显著改善。虽然在多灶性运动神经病中已报道有肌束震颤和痉挛，且被认为是诊断的支持标准，但痛性肌束震颤综合征作为多灶性运动神经病的一种变异型——单灶性运动神经病的首发特征和主要表现则较为独特。《肌肉与神经》56: 828 - 832, 2017年。
