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成人斯蒂尔病相关自身免疫性脑膜脑炎 - 病例报告。

Autoimmune meningitis and encephalitis in adult-onset still disease - Case report.

机构信息

1st Department of Neurology, Institute of Psychiatry and Neurology, Warsaw, Poland.

Department of Neuropathology, Institute of Psychiatry and Neurology, Warsaw, Poland.

出版信息

Neurol Neurochir Pol. 2017 Sep-Oct;51(5):421-426. doi: 10.1016/j.pjnns.2017.06.006. Epub 2017 Jul 8.

Abstract

INTRODUCTION

Adult-onset Still disease (AOSD) is a rare systemic inflammatory disease of unknown cause. Its symptoms usually include persistent fever, fugitive salmon-colored rash, arthritis, sore throat (not specific), but it may also lead to internal organs' involvement, which presents with enlargement of the liver and spleen, swollen lymph nodes, carditis or pleuritis - potentially life-threatening complications. In rare cases, AOSD can cause aseptic meningitis or/and encephalitis.

CASE PRESENTATION

We report a case of 31-year-old male patient, who was referred to neurological department for extending diagnostics of frontal lobes lesions with involvement of adjacent meninges. Abnormalities have been revealed in brain MRI, which was performed due to persistent headaches, visual disturbances, fever, fatigue and cognitive decline. Wide differential diagnosis was performed including laboratory findings, contrast enhanced MRI, MR spectroscopy, flow cytometry and finally brain biopsy to exclude neoplastic or infectious origin. Final diagnosis of autoimmune meningoencephalitis in adult-onset Still disease has been made.

CONCLUSION

Adult-onset Still disease is a rare cause of inflammatory changes in central nervous system, which if diagnosed, may be treated successfully with steroids (commonly available agent), intravenous immunoglobulins or more specific immunomodulating regiments.

摘要

介绍

成人Still 病(AOSD)是一种病因不明的罕见全身性炎症性疾病。其症状通常包括持续发热、一过性鲑鱼斑疹、关节炎、咽痛(不具特异性),但也可能导致内脏器官受累,表现为肝脾肿大、淋巴结肿大、心肌炎或胸膜炎——可能危及生命的并发症。在极少数情况下,AOSD 可引起无菌性脑膜炎和/或脑炎。

病例介绍

我们报告了一例 31 岁男性患者,因额叶病变伴邻近脑膜受累,被转诊至神经科进行进一步诊断。由于持续性头痛、视力障碍、发热、疲劳和认知能力下降,进行了脑部 MRI 检查,结果显示异常。进行了广泛的鉴别诊断,包括实验室检查、增强 MRI、磁共振波谱、流式细胞术,最终进行了脑活检以排除肿瘤或感染性病变。最终诊断为成人Still 病中的自身免疫性脑膜脑炎。

结论

成人Still 病是中枢神经系统炎症性改变的罕见病因,如果确诊,可成功采用类固醇(常用药物)、静脉注射免疫球蛋白或更特异的免疫调节药物进行治疗。

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