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Stiff person syndrome (SPS): Literature review and case report

机译:僵人综合症(SPS):文献复习和病例报告

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Stiff person syndrome (SPS) is a rare, debilitating condition which presents with progressive and inconsistent neurological features. The main symptoms are stiffness and intermittent, painful muscle spasms, triggered and exacerbated by stressful and emotional stimuli. The fluctuating clinical nature of SPS, and otherwise normal neurological examination, often lead to a misdiagnosis of conversion disorder. Psychiatric symptoms frequently accompany this disorder and patients are often first seen by psychiatrists. SPS is autoimmune-based: antibodies are directed against glutamate decarboxylase, resulting in dysregulation of gamma-aminobutyric acid (GABA) in the brain which is considered the cause of the neuropsychiatric symptomatology. SPS should be considered in the differential diagnosis of conversion disorder. Effective management requires early detection, a collaborative approach with GABA-ergic medication and intravenous immunoglobulins, and management of concomitant psychiatric disorders. We describe a patient with SPS. Only one other case has been reported in South Africa.
机译:僵硬人综合症(SPS)是一种罕见的使人衰弱的疾病,表现为进行性和不一致的神经系统特征。主要症状是僵硬和间歇性疼痛,肌肉痉挛,由压力和情绪刺激触发并加剧。 SPS的临床性质波动以及其他正常的神经系统检查,通常会导致对转化障碍的误诊。这种病症经常伴随着精神病症状,并且精神病医生通常首先见到患者。 SPS是基于自身免疫的:抗体针对谷氨酸脱羧酶,导致大脑中的γ-氨基丁酸(GABA)失调,这被认为是神经精神症状的原因。鉴别诊断转化障碍时应考虑使用SPS。有效的管理需要及早发现,采用GABA药物和静脉注射免疫球蛋白的协作方法,并应对伴随的精神疾病。我们描述了SPS患者。南非仅报告了另一例。

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