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. Key words: Still's disease,adult-onset; Demyelinating diseases; Central nerve system diseases; Magnetic resonance imaging