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神经精神性系统性红斑狼疮:一个诊断难题。

Neuropsychiatric Systemic Lupus Erythematosus: A Diagnostic Conundrum.

作者信息

Joseph Vivek, Anil Rahul, Aristy Sary

机构信息

Department of Internal Medicine, Rutgers New Jersey Medical School, Rutgers University, 150 Bergen Street, Newark, NJ 07601, USA.

Department of Neurology, Yale School of Medicine, Yale University, 15 York Street, New Haven, CT 06510, USA.

出版信息

J Clin Med Res. 2016 Oct;8(10):757-9. doi: 10.14740/jocmr2698w. Epub 2016 Aug 30.

Abstract

A 70-year-old man presented with complaints of rapid cognitive decline and new onset leukopenia. The patient had a 17-year history of refractory seizures. Detailed review of symptoms and investigations revealed the patient met American College of Rheumatology (ACR) diagnostic criteria for systemic lupus erythematosus (SLE). The patient had high titer ANA with a strongly positive dsDNA. Immunosuppressive therapy with hydroxychloroquine and mycophenolate mofetil led to significant improvement in cognition and seizures. Neuropsychiatric SLE should be considered a potential differential diagnosis for patients presenting with seizures or cognitive decline. Moreover, neuropsychiatric manifestations especially seizures are an early event in the disease course of SLE. Hence, we believe that early diagnosis of SLE by neuropsychiatric manifestations will not only lead to better control of CNS symptoms but early immunosuppressive therapy could control the progression of the underlying autoimmune disease.

摘要

一名70岁男性因认知功能快速衰退和新发白细胞减少症前来就诊。该患者有17年难治性癫痫病史。对症状和检查结果的详细回顾显示,该患者符合美国风湿病学会(ACR)系统性红斑狼疮(SLE)的诊断标准。患者抗核抗体(ANA)滴度高,双链DNA(dsDNA)呈强阳性。使用羟氯喹和霉酚酸酯进行免疫抑制治疗后,认知功能和癫痫发作有显著改善。对于出现癫痫发作或认知功能衰退的患者,神经精神性SLE应被视为一种潜在的鉴别诊断。此外,神经精神性表现尤其是癫痫发作是SLE病程中的早期事件。因此,我们认为通过神经精神性表现早期诊断SLE不仅能更好地控制中枢神经系统症状,而且早期免疫抑制治疗可以控制潜在自身免疫性疾病的进展。

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Predictors of Incident Seizure in Systemic Lupus Erythematosus.系统性红斑狼疮中癫痫发作的预测因素
J Rheumatol. 2016 Mar;43(3):565-75. doi: 10.3899/jrheum.150135. Epub 2016 Jan 15.
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Neuropsychiatric syndromes in systemic lupus erythematosus: a meta-analysis.神经精神性狼疮的神经精神性综合征:一项荟萃分析。
Semin Arthritis Rheum. 2011 Aug;41(1):1-11. doi: 10.1016/j.semarthrit.2010.08.001. Epub 2010 Oct 20.
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Epileptic seizures in systemic lupus erythematosus.系统性红斑狼疮中的癫痫发作
Neurology. 2004 Nov 23;63(10):1808-12. doi: 10.1212/01.wnl.0000144178.32208.4f.

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