Kappers M H W, Swaak A J G, Zuidgeest D M H, Dees A
Erasmus MC-Dijkzigt, afd. Interne Geneeskunde, Postbus 2040, 3000 CA Rotterdam.
Ned Tijdschr Geneeskd. 2006 Feb 18;150(7):387-92.
A 41-year-old woman who had suffered from systemic lupus erythematosus (SLE) for 22 years presented with signs of neurological deficits. CT-scanning of the brain revealed hypodense lesions that suggested cerebral infarction due to vasculitis in SLE. However, in spite of intensified immunosuppressive therapy, she showed rapid neurological deterioration. After extensive, additional examinations and tests, the diagnosis was finally changed to progressive multifocal leukoencephalopathy, caused by an opportunistic infection by the JC polyomavirus. Neurological and psychiatric symptoms frequently occur in patients with SLE. The differential diagnosis of these symptoms in SLE is extensive and includes, on the one hand, primary neurological and psychiatric diseases related to direct involvement of the nervous system by SLE, and on the other hand, secondary syndromes arising as a result of complications of the SLE or the immunosuppressive treatment. Opportunistic infections are often an important secondary cause of neurological and psychiatric syndromes in patients with SLE. The clinical symptoms and radiological cerebral signs are non-specific and usually do not suffice to differentiate between the various syndromes. Since each syndrome requires its own specific clinical approach and treatment, extensive diagnostics are mandatory before the diagnosis 'cerebral lupus' can be made and immunosuppressive therapy can be started or intensified.
一名患有系统性红斑狼疮（SLE）22年的41岁女性出现神经功能缺损症状。脑部CT扫描显示低密度病变，提示SLE血管炎所致脑梗死。然而，尽管强化了免疫抑制治疗，她的神经功能仍迅速恶化。经过广泛的进一步检查和测试，最终诊断改为进行性多灶性白质脑病，由JC多瘤病毒的机会性感染引起。神经和精神症状在SLE患者中经常出现。SLE中这些症状的鉴别诊断范围广泛，一方面包括与SLE直接累及神经系统相关的原发性神经和精神疾病，另一方面包括SLE或免疫抑制治疗并发症导致的继发性综合征。机会性感染往往是SLE患者神经和精神综合征的重要继发性原因。临床症状和脑部影像学体征不具有特异性，通常不足以区分各种综合征。由于每种综合征都需要其特定的临床方法和治疗，在做出“脑狼疮”诊断并开始或强化免疫抑制治疗之前，必须进行广泛的诊断。
