狼疮舞蹈症。磁共振成像的研究。脉冲可的松耐药后血浆置换的成功[j]。

F Nectoux, L Euller-Ziegler, C Grisot, J F Quaranta, H Duplay, G Ziegler
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引用次数: 0

摘要

舞蹈病作为SLE的一种表现并不常见(占所有SLE病例的1%)。本文报道一例新病例。患者为17岁女性,入院时左半身体有一周的舞蹈动作史,双手腕关节炎。生物学检查结果证实SLE的诊断存在抗凝血酶原循环抗凝剂。脑部CT及核磁共振检查无明显异常。尽管每日剂量为1mg /kg的皮质类固醇与三次800mg甲基强的松龙治疗,临床症状仍恶化。给予大剂量抗精神病药物治疗,并进行三次血浆交换。临床症状和生物学异常的显著改善在随访期间发生并持续了一年多。MRI异常的缺失提示slel相关舞蹈病的发病机制涉及免疫复合物激活功能性神经元;血浆置换的显著效果可能使对皮质类固醇无效的患者无需使用免疫抑制剂。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
[Lupus chorea revealing. Study in magnetic resonance imaging. Success of plasma exchanges after resistance to pulsed cortisone].

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.

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