成人Still病合并脱髓鞘脑病一例

目的 探讨成人Still病合并脱髓鞘脑病的临床特点及诊断.方法 报道1例表现为脑病症状的成人Still病患者,并结合相关文献进行讨论.结果 成人Still病中枢神经系统受累可表现为头痛、偏瘫及一过性意识障碍,头部MRI呈大片状长T1长T2异常信号,周围呈袖套样水肿带,占位效应显著,类似肿瘤的影像表现,病理检查证实为中枢神经系统炎性脱髓鞘,经糖皮质激素联合免疫球蛋白治疗症状好转.随访4个月头部MRI病灶基本消失.结论 成人Still病可合并中枢神经脱髓鞘脑病,头部MRI呈类肿瘤样影像,糖皮质激素联合免疫球蛋白治疗效果良好.

Demyelinating encephalopathy in adult onset Still's disease

Objective To report clinical features,diagnosis and treatment in a case of adult onset Still's disease (AOSD) accompanied by demyelinating encephalopathy.Methods We reported a case of Stills disease with signs of encephalopathy.We also reviewed and discussed the literature on the neurological manifestations in AOSD.Results The 35-year-old patient had recurrent fever and arthralgias for 3 years,headache for 1 month and transient loss of consciousness.Laboratory tests showed non-specific immunological activity.MRI showed tumor-like lesions at left parietal and occipital lobes surrounded by sleeve-like edema.The lesion had significant occupation effect.Biopsy proved the presence of demyelinating changes.The patient recovered favorably after administration of corticosteroids and immunoglobulin.The lesions had almost disappeared on follow-up MRI 4 months later.Conclusions Demyelinating encephalopathy may develop in patient with AOSD.MRI may show tumor-like damage,which is rarely reported in the literature.Diagnosis depends on history,clinical manifestation and neuroimaging.Biopsy provides important information in making diagnosis.Treatment with corticosteroids and intravenous immunoglobulin was found to achieve good recovery.