Nectoux F, Euller-Ziegler L, Grisot C, Quaranta J F, Duplay H, Ziegler G
Service de Rhumatologie, Hôpital de l'Archet, Nice.
Rev Rhum Mal Osteoartic. 1992 Jun;59(6):436-8.
Chorea as a manifestation of SLE is infrequent (1% of all cases of SLE). A new case is reported herein. The patient, a seventeen-year-old female, was admitted with a one-week history of choreic movements of the left half of the body and arthritis of both wrists. Biologic findings confirmed the diagnosis of SLE with presence of an antiprothrombinase circulating anticoagulant. Findings upon cerebral CT scan and magnetic resonance imaging were normal. Clinical symptoms worsened despite corticosteroids in a daily dosage of 1 mg/kg with three pulses of 800 mg methylprednisolone. High-dose neuroleptic therapy was given and three plasma exchanges were performed. A dramatic improvement in clinical symptoms and biological anomalies occurred and persisted during follow-up which now exceeds one year. The lack of MRI anomalies suggests that the pathogenesis of SLE-associated chorea involves functional neurone activation by immune complexes; the dramatic effectiveness of plasma exchanges may obviate the need for using immunosuppressant agents in patients who fail to respond to corticosteroids.
舞蹈症作为系统性红斑狼疮(SLE)的一种表现并不常见(占所有SLE病例的1%)。本文报告了一例新病例。该患者为一名17岁女性,因左侧身体舞蹈样动作及双腕关节炎病史一周入院。生物学检查结果通过循环抗凝抗凝血酶原酶的存在确诊为SLE。脑部CT扫描和磁共振成像结果正常。尽管每日给予1mg/kg的皮质类固醇及三次800mg甲泼尼龙冲击治疗,临床症状仍恶化。给予大剂量抗精神病药物治疗并进行了三次血浆置换。临床症状和生物学异常显著改善,并在随访超过一年的时间里持续存在。MRI无异常表明,SLE相关舞蹈症的发病机制涉及免疫复合物对功能性神经元的激活;血浆置换的显著疗效可能使对皮质类固醇无反应的患者无需使用免疫抑制剂。