Difficult to Treat Focal, Stiff Person Syndrome of the Left Upper Extremity
Difficult to Treat Focal, Stiff Person Syndrome of the Left Upper Extremity
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DOI：
10.1155/2017/2580620
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发表时间：
2017-01-01
影响因子：
0.9
通讯作者：
Ali, Sayed K.
中科院分区：
文献类型：
--
作者：
Esplin, Nathan E.;Stelzer, John W.;Ali, Sayed K.
背景。僵直人综合征（SPS）是一种罕见的以肌肉僵硬为特征的神经系统疾病。这是一种氨基丁酸活性降低的疾病，导致肌肉紧张度增加，经常出现疼痛性痉挛。它通常表现在轴向肌肉组织，但很少能累及单肢，通常是下肢。在极少数情况下，它可能是副肿瘤，通常在治疗基础肿瘤后消退。病例报告。46岁男性，霍奇金淋巴瘤病史缓解，左上肢疼痛继发于左上肢僵硬综合征。他以前曾接受血浆置换治疗，并在家中服用安定和巴氯芬，症状得到了较好的控制。SPS之前被诊断为肌电图和抗gad -65抗体滴度，并通过抗gad -65抗体滴度升高证实。他接受了血浆置换和包括肉毒杆菌毒素在内的最大剂量药物治疗，症状只有短暂的轻微改善。结论。这个病例是一种罕见的疾病，对所有已知的治疗方法都是难治的。它概述了进一步了解这种疾病的必要性，以便提供更好的对症治疗或潜在的更明确的护理。
Background. Stiff person syndrome (SPS) is a rare neurologic disorder characterized by muscle rigidity. It is a disorder of reduced GABA activity leading to increased muscle tone and often painful spasms. It generally presents in the axial musculature but rarely can involve only one limb, typically a lower extremity. In rare cases it can be paraneoplastic which generally resolves on treatment of the underlying neoplasm. Case Report. A 46-year-old male with a history of Hodgkin's Lymphoma in remission presented with left upper extremity pain secondary to a diagnosis of Stiff Person Syndrome limited to his left upper extremity. He had previously benefitted from plasmapheresis and was on diazepam and baclofen at home with relatively good control of his symptoms. SPS had previously been diagnosed with EMG and anti-GAD-65 antibody titers and was confirmed by an elevated anti-GAD-65 antibody titer. He was treated with plasmapheresis and maximum doses of medical treatment including botulinum toxin with only transient mild improvement in his symptoms. Conclusion. This case represents a case of a rare disease that was refractory to all known therapies. It outlines the need for further understanding of this disorder in order to provide better symptomatic treatment or potentially more definitive care.