Top

Journal of Medical Case Reports

Published in:

Open Access 01-12-2015 | Case report

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

Authors: Ching-Fu Weng, Ding-Cheng Chan, Ya-Fang Chen, Fei-Chih Liu, Horng-Huei Liou

Published in: Journal of Medical Case Reports | Issue 1/2015

Abstract

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.

Pittock SJ, Debruyne J, Krecke KN, Giannini C, van den Ameele J, De Herdt V, et al. Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS). Brain. 2010;133(9):2626–34.CrossRefPubMed

Kastrup O, van de Nes J, Gasser T, Keyvani K. Three cases of CLIPPERS: a serial clinical, laboratory and MRI follow-up study. J Neurol. 2011;258(12):2140–6.CrossRefPubMed

Simon NG, Parratt JD, Barnett MH, Buckland ME, Gupta R, Hayes MW, et al. Expanding the clinical, radiological and neuropathological phenotype of chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS). J Neurol Neurosurg Psychiatry. 2012;83(1):15–22.CrossRefPubMed

Limousin N, Praline J, Motica O, Cottier JP, Rousselot-Denis C, Mokhtari K, et al. Brain biopsy is required in steroid-resistant patients with chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS). J Neurooncol. 2012;107(1):223–4.CrossRefPubMed

Tohge R, Nagao M, Yagishita A, Matsubara S. A case of chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) in East Asia. Intern Med. 2012;51(9):1115–9.CrossRefPubMed

Hillesheim PB, Parker JR, Parker Jr JC, Escott E, Berger JR. Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids following influenza vaccination. Arch Pathol Lab Med. 2012;136(6):681–5.CrossRefPubMed

Maddison P, Damian MS, Sewry C, McGorrian C, Winer JB, Odgerel Z, et al. Clinical and myopathological characteristics of desminopathy caused by a mutation in desmin tail domain. Eur Neurol. 2012;68(5):279–86.CrossRefPubMedPubMedCentral

Bárcena Marugán R, García GS. DNA-guided hepatitis B treatment, viral load is essential, but not sufficient. World J Gastroenterol. 2009;15(4):423–30.CrossRefPubMed

Matsui M, Kakigi R, Watanabe S, Kuroda Y. Recurrent demyelinating transverse myelitis in a high titer HBs-antigen carrier. J Neurol Sci. 1996;139(2):235–7.CrossRefPubMed

10.

Ichiki Y, He XS, Shimoda S, Ishibashi H, Keeffe EB, Rossaro L, et al. T cell immunity in hepatitis B and hepatitis C virus infection: implications for autoimmunity. Autoimmun Rev. 2005;4(2):82–95.CrossRefPubMed

11.

Maya R, Gershwin ME, Shoenfeld Y. Hepatitis B virus (HBV) and autoimmune disease. Clin Rev Allergy Immunol. 2008;34(1):85–102.CrossRefPubMed

12.

Chastain EM, Miller SD. Molecular mimicry as an inducing trigger for CNS autoimmune demyelinating disease. Immunol Rev. 2012;245(1):227–38.CrossRefPubMedPubMedCentral

13.

Fujinami RS, Oldstone MB. Amino acid homology between the encephalitogenic site of myelin basic protein and virus: mechanism for autoimmunity. Science. 1985;230(4729):1043–5.CrossRefPubMed

14.

Georgiadou SP, Zachou K, Liaskos C, Gabeta S, Rigopoulou EI, Dalekos GN. Occult hepatitis B virus infection in patients with autoimmune liver diseases. Liver Int. 2009;29(3):434–42.CrossRefPubMed

15.

Xu D, Fu J, Jin L, Zhang H, Zhou C, Zou Z, et al. Circulating and liver resident CD4⁺CD25⁺ regulatory T cells actively influence the antiviral immune response and disease progression in patients with hepatitis B. J Immunol. 2006;177(1):739–47.CrossRefPubMed

16.

Stoop JN, van der Molen RG, Baan CC, van der Laan LJ, Kuipers EJ, Kusters JG, et al. Regulatory T cells contribute to the impaired immune response in patients with chronic hepatitis B virus infection. Hepatology. 2005;41(4):771–8.CrossRefPubMed

17.

Costantino CM, Baecher-Allan CM, Hafler DA. Human regulatory T cells and autoimmunity. Eur J Immunol. 2008;38(4):921–4.CrossRefPubMedPubMedCentral

Title: Chronic hepatitis B infection presenting with chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS): a case report
Authors: Ching-Fu Weng
Ding-Cheng Chan
Ya-Fang Chen
Fei-Chih Liu
Horng-Huei Liou
Publication date: 01-12-2015
Publisher: BioMed Central
Published in: Journal of Medical Case Reports / Issue 1/2015
Electronic ISSN: 1752-1947
DOI: https://doi.org/10.1186/s13256-015-0750-1

At a glance: The ONWARDS insulin icodec trials

Springer Medicine

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

Abstract

Introduction

Case presentation

Conclusions

At a glance: The ONWARDS insulin icodec trials

Springer Medicine

Abstract

Introduction

Case presentation

Conclusions

Please log in to get access to this content

Other articles of this Issue 1/2015

The importance of good history taking: a case report

Single-incision assisted laparoscopic surgery (SILS) in the treatment of an intussusception induced by a solitary hamartomatous polyp: a case report and review of the literature

Simultaneous occurrence of a severe Morel-Lavallée lesion and gluteal muscle necrosis as a sequela of transcatheter angiographic embolization following pelvic fracture: a case report

Pancreatic and biliary obstruction years after retention of a swallowed coin in a duodenal diverticulum: a case report

Interleukin-1 receptor antagonist deficiency with a novel mutation; late onset and successful treatment with canakinumab: a case report

Reactivation of hepatitis B (reverse seroconversion) after melphalan/dexamethasone therapy for primary amyloidosis: a case report