Hajjioui Abderrazak, Benbouazza Karima, Faris Mohamed El Alaoui, Missaoui Amina, Hassouni Najia Hajjaj
Physical Medicine and Rehabilitation, El Ayachi Hospital, Mohamed V University, Coast Road Street, Sale, 11000, Morocco.
Cases J. 2010 Feb 12;3:60. doi: 10.1186/1757-1626-3-60.
Stiff limb syndrome is a clinical feature of the stiff person syndrome, which is a rare and disabling neurologic disorder characterized by muscle rigidity and episodic spasms that involve axial and limb musculature. It is an autoimmune disorder resulting in a malfunction of aminobutyric acid mediated inhibitory networks in the central nervous system. We describe a patient diagnosed by neurological symptoms of stiff limb syndrome with a good outcome after treatment, and a review of the related literature.
A 49-year-old male patient presented with a progressive stiffness and painful spasms of his both legs resulting in a difficulty of standing up and walking. The diagnosis of stiff limb syndrome was supported by the dramatically positive response to treatment using diazepam 25 mg/day and baclofen 30 mg/day.
This clinical case highlights the importance of a therapeutic test to confirm the diagnosis of stiff limb syndrome especially when there is a high clinical suspicion with unremarkable electromyography.
僵肢综合征是僵人综合征的一种临床特征,僵人综合征是一种罕见的致残性神经系统疾病,其特征为肌肉僵硬和发作性痉挛,累及躯干和四肢肌肉。它是一种自身免疫性疾病,导致中枢神经系统中γ-氨基丁酸介导的抑制网络功能失调。我们描述了一名经僵肢综合征神经症状诊断的患者,治疗后效果良好,并对相关文献进行了综述。
一名49岁男性患者出现双下肢进行性僵硬和疼痛性痉挛,导致站立和行走困难。使用地西泮25mg/天和巴氯芬30mg/天治疗后出现显著阳性反应,支持僵肢综合征的诊断。
本临床病例强调了治疗性试验对确诊僵肢综合征的重要性,尤其是在临床高度怀疑但肌电图无明显异常的情况下。