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Case Report

Severe bilateral amyotrophic neuralgia associated with major dysphagia secondary to acute hepatitis E

[version 1; peer review: 4 approved]
PUBLISHED 26 Nov 2013
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Abstract

Introduction: Several acute neurological syndromes can be triggered by immune events. Hepatitis E virus (HEV), an emerging infectious disease, can be one of these triggers.
Case report: We report the case of a 36-year-old man that presented nausea and a dull abdominal pain for a week and then felt an acute neuralgic pain involving both shoulders that lasted for 8 to 10 hours. Immediately after, the patient presented a severe bilateral muscular weakness of the proximal part of both upper limbs, corresponding to an amyotrophic neuralgia. Two days after the shoulder pain, the patient presented a dysphagia necessitating tube feeding.  A blood sample confirmed hepatitis caused by hepatitis E virus (HEV; genotype 3F). Oral feeding resumed progressively after five months. The patient was fully independent for the activities of daily living but was still unable to work after six months.
Conclusion: Amyotrophic neuralgia and hepatitis E are both under-diagnosed. It is noteworthy that HEV can trigger amyotrophic neuralgia. Antiviral drugs, oral steroids and intravenous immunoglobulins can be proposed, but the optimal treatment has  not yet been determined.

Keywords

Amyotrophic neuralgia, Hepatitis E, dysphagia

Introduction

Neurological syndromes such as Guillain-Barré Syndrome, transverse myelitis, encephalitis or amyotrophic neuralgia can be triggered by immune events. Hepatitis E virus (HEV), discovered in the 1980s, can be one of these triggers. Epidemics of hepatitis E occur periodically throughout the developing world, but autochthonous HEV infections have also been reported in most developed countries during the last decade. Several HEV-associated neurological syndromes have been described but are probably under-diagnosed1.

Case report

We report the case of a 36-year-old French, Caucasian truck driver, without any significant medical history. The clinical symptoms started in May 2012 with nausea and a dull abdominal pain. No sign of chronic liver disease or of portal hypertension was noted. High liver enzymes were diagnosed after assay for: alanine aminotransferase (ALT) 1707 µmol/L (normal range: N<78), aspartate aminotransferase (AST) 554 µmol/L (N<37), gamma-glutamyltranspeptidase (GGT) 737 U/L (N<95) and alkaline phosphatase at 311 U/L (N<136). Total bilirubin level was at 54 µmol/L (N<17). There was no hepatitis A, B or C, no HIV and no sign of autoimmune disease. Liver ultrasound was normal.

Around one week after the first digestive symptoms, the patient felt an acute neuralgic pain involving both shoulders that lasted for 8 to 10 hours. Immediately after, the patient presented a severe bilateral muscular weakness of the proximal part of both upper limbs. Two days after the shoulder pain, the patient presented with hypophonia and dysphagia. The MRI did not show any brain abnormality. The spinal cord and the brachial plexus were unharmed. The cerebrospinal fluid (CSF) was normal (2 white blood cells/mm3; CSF Protein= 0.37 g/L) and there was no intrathecal antibody synthesis. Antiganglioside antibodies were negative (GM1, GM2, GD1a, GD1b, GQ1b). Electromyography (EMG) and nerve conduction studies (NCS) showed normal amplitudes and conduction velocity but bilateral denervation in the supraspinatus, infraspinatus, subscapularis and deltoid muscles. An acute hepatitis E infection was suspected due to the presence of IgM and confirmed by PCR (genotype 3f). The initial serum HEV RNA count was 5.2 log-copies/ml. The PCR was negative in the CSF.

A treatment with intravenous immunoglobulins (Tegeline®, LFB laboratory, France; 0.4 g/kg/day) was given for 5 days. Ribavirin (600 mg/day) was also introduced. Nine days after ribavirin initiation, the PCR showed 2.02 log-copies/ml and was negative after 18 days. After three weeks, the patient still required nasogastric tube-feeding and a gastric feeding tube was placed endoscopically. There was no contraction of the shoulder girdle muscles. Oral feeding resumed progressively after five months. After six months follow-up and intensive rehabilitation, there was a 3/5 muscular strength in the affected muscles, corresponding to a movement possible against gravity, but not against resistance by the examiner. The patient was fully independent for the activities of daily living but still unable to work.

Discussion

This is the first report of both severe bilateral amyotrophic neuralgia and dysphagia caused by an acute hepatitis E infection. Amyotrophic neuralgia (AN) is a peripheral neuropathy consisting of multiple symptoms including abrupt onset of shoulder pain, usually unilaterally, followed by motor weakness, with an annual incidence above 2 per 100,000 inhabitants2. Concomitant involvement of other peripheral nervous system structures (such as the lumbosacral plexus or phrenic nerve) is described. AN can be triggered by immune events but also by trauma or surgery. Many patients are left with residual disabilities that affect their ability to work and their everyday life. It is noteworthy that a particularly severely affected subgroup presents sign of liver dysfunction, as seen in HEV infections2. The only validated treatment is corticosteroids but this may have been dangerous in this case of acute hepatitis E3. Some authors have also reported a positive effect of intravenous immunoglobulins and this was the option we selected46.

HEV-associated neurological syndromes include both central and peripheral nervous system involvement7. Such cases have been described in the Asian sub-continent (probably due to HEV1) but also in Western Europe with acute and chronic HEV3 infection. For patients with chronic HEV infections, neurological symptoms completely resolved or significantly improved when viral clearance was achieved1. This is the reason why we tried antiviral treatment. Unfortunately, although viral clearance was achieved quickly, this did not lead to fast clinical improvements.

An alternative diagnosis of pharyngeal-cervical-brachial variant of Guillain-Barré syndrome could have been made. This pathology is characterized by oropharyngeal, neck, and upper limb muscle involvement. However, in the present case, this diagnosis was excluded (no neck involvement, atypical EMG, no albuminocytologic dissociation of the cerebrospinal fluid and negative GQ1b antibody).

Conclusion

Post-infectious neurological diseases following HEV infection must be recognized to avoid unnecessary and potentially invasive procedures. Further studies are needed in order to determine the optimal treatment. In the meantime, antiviral drugs, steroids and IV-immunoglobulins are all possibilities.

Consent

Written informed consent for publication of clinical details was obtained from the patient.

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Version 2
VERSION 2 PUBLISHED 26 Nov 2013
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CITE
how to cite this article
Moisset X, Vitello N, Bicilli E et al. Severe bilateral amyotrophic neuralgia associated with major dysphagia secondary to acute hepatitis E [version 1; peer review: 4 approved]. F1000Research 2013, 2:259 (https://doi.org/10.12688/f1000research.2-259.v1)
NOTE: If applicable, it is important to ensure the information in square brackets after the title is included in all citations of this article.
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Open Peer Review

Current Reviewer Status: ?
Key to Reviewer Statuses VIEW
ApprovedThe paper is scientifically sound in its current form and only minor, if any, improvements are suggested
Approved with reservations A number of small changes, sometimes more significant revisions are required to address specific details and improve the papers academic merit.
Not approvedFundamental flaws in the paper seriously undermine the findings and conclusions
Version 1
VERSION 1
PUBLISHED 26 Nov 2013
Views
58
Cite
Reviewer Report 23 Dec 2013
Michelle Cheung, King's College Hospital, London, UK 
Approved
VIEWS 58
This is a report of an emerging viral infection with well-documented but still uncommon neurological symptoms, and adds to the knowledge of the natural history and potential treatment of this clinical syndrome. It is clearly written and the case is ... Continue reading
CITE
CITE
HOW TO CITE THIS REPORT
Cheung M. Reviewer Report For: Severe bilateral amyotrophic neuralgia associated with major dysphagia secondary to acute hepatitis E [version 1; peer review: 4 approved]. F1000Research 2013, 2:259 (https://doi.org/10.5256/f1000research.2926.r2571)
NOTE: it is important to ensure the information in square brackets after the title is included in all citations of this article.
  • Author Response 31 Dec 2013
    Xavier Moisset, CHU Clermont-Ferrand, Service de Neurologie, CHU Gabriel Montpied, Clermont-Ferrand, F-63000, France
    31 Dec 2013
    Author Response
    We want to thank Dr Cheung for the comments she made that have helped us to improve the quality of our text.
    1.  Dr Cheung is right; the Cochrane review identified one
    ... Continue reading
COMMENTS ON THIS REPORT
  • Author Response 31 Dec 2013
    Xavier Moisset, CHU Clermont-Ferrand, Service de Neurologie, CHU Gabriel Montpied, Clermont-Ferrand, F-63000, France
    31 Dec 2013
    Author Response
    We want to thank Dr Cheung for the comments she made that have helped us to improve the quality of our text.
    1.  Dr Cheung is right; the Cochrane review identified one
    ... Continue reading
Views
41
Cite
Reviewer Report 20 Dec 2013
José Manuel Echevarrı́a, Department of Virology, National Centre of Microbiology, Carlos III Health Institute Majadahonda, Madrid, Spain 
Approved
VIEWS 41
The manuscript describes the association of a convincingly diagnosed case of acute hepatitis E due to HEV genotype 3 infection with the development of severe amyotrophic neuralgia and major dysphagia in the patient. Reports of neurological complications among otherwise healthy ... Continue reading
CITE
CITE
HOW TO CITE THIS REPORT
Echevarrı́a JM. Reviewer Report For: Severe bilateral amyotrophic neuralgia associated with major dysphagia secondary to acute hepatitis E [version 1; peer review: 4 approved]. F1000Research 2013, 2:259 (https://doi.org/10.5256/f1000research.2926.r2868)
NOTE: it is important to ensure the information in square brackets after the title is included in all citations of this article.
  • Author Response 31 Dec 2013
    Xavier Moisset, CHU Clermont-Ferrand, Service de Neurologie, CHU Gabriel Montpied, Clermont-Ferrand, F-63000, France
    31 Dec 2013
    Author Response
    Dr Echevarrı́a is right; anti-HEV antibody testing of CSF was not performed and this is now clearly specified in our version 2. Indeed, it would be of interest in future ... Continue reading
COMMENTS ON THIS REPORT
  • Author Response 31 Dec 2013
    Xavier Moisset, CHU Clermont-Ferrand, Service de Neurologie, CHU Gabriel Montpied, Clermont-Ferrand, F-63000, France
    31 Dec 2013
    Author Response
    Dr Echevarrı́a is right; anti-HEV antibody testing of CSF was not performed and this is now clearly specified in our version 2. Indeed, it would be of interest in future ... Continue reading
Views
32
Cite
Reviewer Report 20 Dec 2013
Thierry Coton, Service de Pathologie Digestive, Hôpital d’instruction des Armées Laveran, Marseille, France 
Approved
VIEWS 32
This is a very interesting article about an emerging complication of acute hepatitis E.

The following information is missing however:
  1. the gender of the patient.
  2. if prothrombin time increased during hepatitis.
  3. which auto antibodies were screened for.
  4. if PCR was realised in stools.
  5. the duration of
... Continue reading
CITE
CITE
HOW TO CITE THIS REPORT
Coton T. Reviewer Report For: Severe bilateral amyotrophic neuralgia associated with major dysphagia secondary to acute hepatitis E [version 1; peer review: 4 approved]. F1000Research 2013, 2:259 (https://doi.org/10.5256/f1000research.2926.r2575)
NOTE: it is important to ensure the information in square brackets after the title is included in all citations of this article.
  • Author Response 31 Dec 2013
    Xavier Moisset, CHU Clermont-Ferrand, Service de Neurologie, CHU Gabriel Montpied, Clermont-Ferrand, F-63000, France
    31 Dec 2013
    Author Response
    We want to thank Dr Coton for the comments and suggestions he made that have helped us to improve the quality of our case report.
    1. The gender of the patient (male)
    ... Continue reading
COMMENTS ON THIS REPORT
  • Author Response 31 Dec 2013
    Xavier Moisset, CHU Clermont-Ferrand, Service de Neurologie, CHU Gabriel Montpied, Clermont-Ferrand, F-63000, France
    31 Dec 2013
    Author Response
    We want to thank Dr Coton for the comments and suggestions he made that have helped us to improve the quality of our case report.
    1. The gender of the patient (male)
    ... Continue reading
Views
30
Cite
Reviewer Report 04 Dec 2013
Bruce Brew, Department of Neurology, St Vincent's Hospital, Sydney, NSW, Australia 
Approved
VIEWS 30
This is an important case report highlighting HEV as a cause for ... Continue reading
CITE
CITE
HOW TO CITE THIS REPORT
Brew B. Reviewer Report For: Severe bilateral amyotrophic neuralgia associated with major dysphagia secondary to acute hepatitis E [version 1; peer review: 4 approved]. F1000Research 2013, 2:259 (https://doi.org/10.5256/f1000research.2926.r2574)
NOTE: it is important to ensure the information in square brackets after the title is included in all citations of this article.

Comments on this article Comments (0)

Version 2
VERSION 2 PUBLISHED 26 Nov 2013
Comment
Alongside their report, reviewers assign a status to the article:
Approved - the paper is scientifically sound in its current form and only minor, if any, improvements are suggested
Approved with reservations - A number of small changes, sometimes more significant revisions are required to address specific details and improve the papers academic merit.
Not approved - fundamental flaws in the paper seriously undermine the findings and conclusions
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