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[以单侧上肢为主的严重疼痛和肌束震颤为表现的抗电压门控钾通道复合物抗体相关疾病病例]

[Case of anti VGKC-complex antibody associated disorder presenting with severe pain and fasciculations predominant in unilateral upper extremity].

作者信息

Hara Kenju, Watanabe Osamu, Shibano Ken, Ishiguro Hideaki

机构信息

Department of Neurology, Akita Red Cross Hospital.

出版信息

Rinsho Shinkeigaku. 2012;52(9):677-80. doi: 10.5692/clinicalneurol.52.677.

Abstract

A 21-year-old man complained of severe pain and muscle twitching localized in his right arm. Neurological examination showed muscle fasciculations in his right forearm but no myokymia or myotonia. Needle electromyography revealed fibrillation potentials in his biceps brachii muscle and extensor carpi radialis muscle at rest but no myokymic discharges. His serum anti-voltage-gated potassium channel (VGKC)-complex antibody level was significantly high (194.2pM; controls <100pM). Although anticonvulsant therapy relieved his pain, he was readmitted to our hospital because of severe pain in his left arm and both thighs three months later. A high-dose intravenous immunoglobulin (IVIG) therapy followed by steroid pulse therapy relieved his pain. This case with neither muscle cramp nor myokymia expands the phenotype of anti VGKC-complex antibody associated disorder.

摘要

一名21岁男性主诉右臂出现严重疼痛和肌肉抽搐。神经系统检查显示其右前臂有肌肉束颤,但无肌纤维颤搐或肌强直。针极肌电图显示,静息时肱二头肌和桡侧腕伸肌有纤颤电位,但无肌纤维颤搐放电。他的血清抗电压门控钾通道(VGKC)复合物抗体水平显著升高(194.2pM;对照组<100pM)。尽管抗惊厥治疗缓解了他的疼痛,但3个月后他因左臂和双侧大腿剧痛再次入院。大剂量静脉注射免疫球蛋白(IVIG)治疗后再进行类固醇冲击治疗缓解了他的疼痛。该病例既无肌肉痉挛也无肌纤维颤搐,扩展了抗VGKC复合物抗体相关疾病的表型。

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