Chronic hepatitis B infection presenting with chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS): a case report

Journal of Medical Case Reports

Horng-Huei Liou²  Fei-Chih Liu²  Ya-Fang Chen¹  Ding-Cheng Chan³  Ching-Fu Weng⁴ 

[1] Department of Medical Imaging, National Taiwan University Hospital, Taipei, Taiwan;Department of Neurology, National Taiwan University Hospital, No.1, Changde Street, Zhongzheng District, Taipei City 10048, Taiwan;Department of Internal Medicine, National Taiwan University Hospital, Taipei, Taiwan;Department of Gerontology and Geriatrics, National Taiwan University Hospital, Taipei, Taiwan

关键词: Regulatory T cell;    Hepatitis B;    Encephalomyelitis;    Demyelinating disease;    CLIPPERS;    Autoimmunity;   

Others  :  1235879
DOI  :  10.1186/s13256-015-0750-1

 received in 2015-05-11, accepted in 2015-10-26,  发布年份 2015

【 摘 要 】

Introduction

Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids is a brainstem disorder characterized by perivascular pathologic reaction with lymphocyte infiltration and leading to diplopia, facial palsy, dysarthria, and gait ataxia. It was thought to be an autoimmune disorder without distinct pathogenesis. Chronic hepatitis B virus infection has been proposed in correlation with autoimmune diseases, including central nervous system demyelinating disease. Patients with chronic hepatitis B infection may develop the syndrome of chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids.

Case presentation

A 34-year-old Taiwanese man who had been a hepatitis B virus carrier for a decade presented to our emergency room. He had influenza symptoms and progressive symptoms of left hemifacial numbness, double vision, and an unsteady gait of 2 days’ duration. Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids was diagnosed, with increased hepatitis B viral load at the same time. He had no past history of similar neurologic deficits, and his liver function tests had been within normal limits before this episode. After corticosteroid and entecavir treatments, his neurological deficits and neuroimaging anomalies improved and his serum hepatitis B virus DNA viral load normalized.

Conclusions

Hepatitis B virus infection may induce central nervous system autoimmune reactions, including chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids. In such cases, concomitant administration of corticosteroids and antiviral agent was helpful. We suggest further investigations in patients with regulatory T cell dysfunction, which may assist in clarifying a loss of immune tolerance in patients with such disorders.

【 授权许可】

   
2015 Weng et al.

【 图 表 】

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