Skip to main content

Main menu

  • Home
  • Our journals
    • Clinical Medicine
    • Future Healthcare Journal
  • Subject collections
  • About the RCP
  • Contact us

Clinical Medicine Journal

  • ClinMed Home
  • Content
    • Current
    • Ahead of print
    • Archive
  • Author guidance
    • Instructions for authors
    • Submit online
  • About ClinMed
    • Scope
    • Editorial board
    • Policies
    • Information for reviewers
    • Advertising

User menu

  • Log in

Search

  • Advanced search
RCP Journals
Home
  • Log in
  • Home
  • Our journals
    • Clinical Medicine
    • Future Healthcare Journal
  • Subject collections
  • About the RCP
  • Contact us
Advanced

Clinical Medicine Journal

clinmedicine Logo
  • ClinMed Home
  • Content
    • Current
    • Ahead of print
    • Archive
  • Author guidance
    • Instructions for authors
    • Submit online
  • About ClinMed
    • Scope
    • Editorial board
    • Policies
    • Information for reviewers
    • Advertising

Myasthenia mimicry

Stephanie Teh Siu-Hoong
Download PDF
DOI: https://doi.org/10.7861/clinmed.22-4-s35
Clin Med July 2022
Stephanie Teh Siu-Hoong
AWrexham Maelor Hospital, Betsi Cadwaladr University Health Board, North Wales
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
  • Article
  • Info & Metrics
Loading

Background

Bickerstaff's encephalitis is a rare GQ1b-mediated polyneuropathy, considered to be a brainstem variant of Guillain–Barré syndrome and Miller Fisher syndrome. It is classically differentiated from Miller Fisher syndrome by an altered sensorium but similarly demonstrates ophthalmoplegia and ataxia. Other commonly described features include peripheral involvement and bulbar weakness. Myasthenia gravis is more common, a chronic autoimmune condition primarily characterised by muscle weakness and fatiguability, and is an important cause of neuromuscular respiratory failure.

Case presentation

This case reports a man aged in his late 60s who presented with dysphagia to solids and headaches, double vision and an unsteady gait. He had a history of an antecedent viral upper respiratory tract illness a week prior to the onset of his symptoms. On arrival at the emergency department, he was assessed to be ataxic, ophthalmoplegic and was mildly confused (scoring 8/10 on AMT-10) but with no other neurological signs. Shortly after arrival, he became floridly confused and agitated with marked intermittent stridor. An arterial blood gas demonstrated severe respiratory acidosis secondary to hypercapnic respiratory failure. He was intubated for airway protection and subsequent investigations including direct visualisation of his vocal cords, oesophago-gastro-duodenoscopy, computed tomography and magnetic resonance imaging all did not find a cause for his symptoms. Cerebro-spinal fluid (CSF) analysis showed albuminocytological dissociation and nerve conduction studies demonstrated absent proximal motor responses and possible conduction block.

The combination of ataxia, altered sensorium, ophthalmoplegia, bulbar palsy, nerve conduction and CSF results prompted an initial working diagnosis of brainstem encephalitis, Bickerstaff's: a GQ1B-mediated Guillain–Barré variant. Autoantibodies were sent for GQ1B, myasthenia and voltage-gated calcium channels (VGCC).

He had a stormy clinical course with two failed primary extubations in his first week of presentation, and seeming improvement post-intravenous immunoglobulins (IVIG), with a recurrence of his symptoms 1 month later. A second IVIG course was restarted and the autoantibody results were found later positive for anti-acetylcholine receptor, confirming a diagnosis of late onset myasthenia. Anti-GQ1B, VGCC and GM1 were all negative.

Key points

This case critically highlights the overlap of key GQ1B and myasthenia features and demonstrates the challenge of distinguishing between them in clinical practice. The concurrent presentation of both stridor and his postural worsening of symptoms is suggestive of both a diaphragmatic and bulbar palsy while his history of weight loss with negative investigations may relate to a more insidious course of tongue and palate muscle weakness.

  • © Royal College of Physicians 2022. All rights reserved.
Back to top
Previous articleNext article

Article Tools

Download PDF
Article Alerts
Sign In to Email Alerts with your Email Address
Citation Tools
Myasthenia mimicry
Stephanie Teh Siu-Hoong
Clinical Medicine Jul 2022, 22 (Suppl 4) 35; DOI: 10.7861/clinmed.22-4-s35

Citation Manager Formats

  • BibTeX
  • Bookends
  • EasyBib
  • EndNote (tagged)
  • EndNote 8 (xml)
  • Medlars
  • Mendeley
  • Papers
  • RefWorks Tagged
  • Ref Manager
  • RIS
  • Zotero
Share
Myasthenia mimicry
Stephanie Teh Siu-Hoong
Clinical Medicine Jul 2022, 22 (Suppl 4) 35; DOI: 10.7861/clinmed.22-4-s35
del.icio.us logo Digg logo Reddit logo Twitter logo Facebook logo Google logo Mendeley logo
  • Tweet Widget
  • Facebook Like
  • Google Plus One

Jump to section

  • Article
    • Background
    • Case presentation
    • Key points
  • Info & Metrics

Related Articles

  • No related articles found.
  • PubMed
  • Google Scholar

Cited By...

  • No citing articles found.
  • Google Scholar

More in this TOC Section

  • A rare case report of granulomatosis with polyangiitis presenting with thrombus of the ascending aorta
  • Pancreatic cancer with multiple liver metastasis complicating multi-organ infarcts from marantic endocarditis and Trousseau's syndrome
  • Age-adjusted versus cut-off for D-dimer to exclude pulmonary embolism audit
Show more Clinical

Similar Articles

FAQs

  • Difficulty logging in.

There is currently no login required to access the journals. Please go to the home page and simply click on the edition that you wish to read. If you are still unable to access the content you require, please let us know through the 'Contact us' page.

  • Can't find the CME questionnaire.

The read-only self-assessment questionnaire (SAQ) can be found after the CME section in each edition of Clinical Medicine. RCP members and fellows (using their login details for the main RCP website) are able to access the full SAQ with answers and are awarded 2 CPD points upon successful (8/10) completion from:  https://cme.rcplondon.ac.uk

Navigate this Journal

  • Journal Home
  • Current Issue
  • Ahead of Print
  • Archive

Related Links

  • ClinMed - Home
  • FHJ - Home
clinmedicine Footer Logo
  • Home
  • Journals
  • Contact us
  • Advertise
HighWire Press, Inc.

Follow Us:

  • Follow HighWire Origins on Twitter
  • Visit HighWire Origins on Facebook

Copyright © 2021 by the Royal College of Physicians