Maeda Kengo, Shimizu Yoshiki, Sugihara Yoshiko, Kanazawa Naomi, Iizuka Takahiro
Department of Neurology, National Hospital Organization Higashi-ohmi General Medical Center.
Department of Neurology, Kitasato University School of Medicine.
Rinsho Shinkeigaku. 2019 Feb 23;59(2):98-101. doi: 10.5692/clinicalneurol.cn-001239. Epub 2019 Jan 31.
A 48-year-old woman with a 3-month history of spontaneously resolving stiff leg symptom at the age of 43 years presented with progressive onset of leg rigidity, walking difficulty, and myoclonic jerks. On admission she had marked stiffness in her foot joints with symmetric sustained dorsiflexion of the ankles and toes, with spontaneous and reflex myoclonic jerks easily provoked by knee tendon tap. She appeared to have a spastic gait due to stiffness in her legs. Needle electromyogram (EMG) examination revealed continuous motor unit activity in the tibialis anterior muscle at rest even when voluntary contraction of the gastrocnemius muscle was instructed, but no myokimic discharge or acute denervation sign was seen. The laboratory tests were unremarkable, including glutamic acid decarboxylase antibody. Cerebrospinal fluid (CSF) examination was also normal, without oligoclonal bands or elevated IgG index. She was diagnosed with stiff-limb syndrome based on neurologic examination and needle EMG findings, and she was treated with intravenous high-dose methylprednisolone (500 mg/day, 3 days), resulting in marked improvement in her symptoms. Anti-glycine receptor antibodies were subsequently identified in her archived serum and CSF obtained before immunotherapy. She was then started on oral prednisolone (30 mg/day) and had been free of symptoms.
一名48岁女性,43岁时曾有过持续3个月的自行缓解的腿部僵硬症状,此次因逐渐出现腿部僵硬、行走困难和肌阵挛前来就诊。入院时,她的足部关节明显僵硬,踝关节和脚趾呈对称性持续背屈,膝腱轻敲容易诱发自发和反射性肌阵挛。由于腿部僵硬,她表现出痉挛性步态。针极肌电图(EMG)检查显示,即使在指示腓肠肌进行自主收缩时,静息状态下的胫前肌仍有持续的运动单位活动,但未发现肌纤维放电或急性失神经征象。实验室检查无异常,包括谷氨酸脱羧酶抗体检查。脑脊液(CSF)检查也正常,无寡克隆带或IgG指数升高。根据神经系统检查和针极肌电图结果,她被诊断为僵肢综合征,并接受了静脉注射大剂量甲泼尼龙治疗(500mg/天,共3天),症状明显改善。随后在免疫治疗前采集的存档血清和脑脊液中发现了抗甘氨酸受体抗体。之后她开始口服泼尼松龙(30mg/天),症状消失。