Stiff limb syndrome, first case report in Palestine

Mustafa Hammad , Wasef Alhroub , Alhareth Amro , Rama Rije , Mohammad Abu Saif , Mohammad Abo-Ghosh
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Abstract

Background

Stiff person syndrome (SPS) is a rare autoimmune neurological disorder characterized by progressive muscle rigidity and spasms. Stiff limb syndrome (SLS) is a rare, more localized variant of SPS.

Case presentation

This case report describes a 52-year-old woman who presented with a 2-year history of slowly progressive right leg stiffness causing severe spasms and difficulty in walking. Neurological examination showed severe spasticity of the right leg, increased deep tendon reflexes in all extremities, worse on the right leg and inability to stand due to the severity of the pain and spasticity of the right leg. Laboratory findings revealed elevated glutamic acid decarboxylase (GAD) antibodies, and positive pancreatic islet cells antibodies (ICA). Electromyography demonstrated continuous motor unit activity, that improved after administration of a benzodiazepine. The patient was treated with a benzodiazepine, which led to significant improvement in her symptoms.

Discussion

This article documented the first reported case of SLS in Palestine, highlighting the importance of early recognition and appropriate treatment of SLS to minimize functional impairment in patients. Detailed clinical features, laboratory findings, and the patient's response to therapy are discussed to emphasize the diagnostic challenges and management strategies for this rare neurological condition.
肢体僵硬综合征,巴勒斯坦首例报告病例
背景:僵直人综合征(SPS)是一种罕见的自身免疫性神经系统疾病,以进行性肌肉僵硬和痉挛为特征。肢体僵硬综合征(SLS)是一种罕见的,更局部变异的SPS。病例报告:该病例报告描述了一名52岁女性,其表现为2年缓慢进行性右腿僵硬史,导致严重痉挛和行走困难。神经学检查显示右腿严重痉挛,四肢深腱反射增加,右腿更严重,由于右腿疼痛和痉挛的严重程度,无法站立。实验室结果显示谷氨酸脱羧酶(GAD)抗体升高,胰岛细胞抗体(ICA)阳性。肌电图显示持续的运动单元活动,在给予苯二氮卓类药物后得到改善。患者接受苯二氮卓类药物治疗,症状明显改善。本文记录了巴勒斯坦首例报道的SLS病例,强调了早期识别和适当治疗SLS的重要性,以尽量减少患者的功能损害。详细的临床特征,实验室结果和患者对治疗的反应进行了讨论,以强调诊断挑战和管理策略,这种罕见的神经系统疾病。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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