Skip to main content
Advertisement
  • Neurology.org
  • Journals
    • Neurology
    • Clinical Practice
    • Genetics
    • Neuroimmunology & Neuroinflammation
    • Education
  • Online Sections
    • COVID-19
    • Inclusion, Diversity, Equity, Anti-racism, & Social Justice (IDEAS)
    • Innovations in Care Delivery
    • Practice Buzz
    • Practice Current
    • Residents & Fellows
    • Without Borders
  • Collections
    • Topics A-Z
    • Disputes & Debates
    • Health Disparities
    • Infographics
    • Patient Pages
    • Null Hypothesis
    • Translations
  • Podcast
  • CME
  • About
    • About the Journals
    • Contact Us
    • Editorial Board
  • Authors
    • Submit a Manuscript
    • Author Center

Advanced Search

Main menu

  • Neurology.org
  • Journals
    • Neurology
    • Clinical Practice
    • Genetics
    • Neuroimmunology & Neuroinflammation
    • Education
  • Online Sections
    • COVID-19
    • Inclusion, Diversity, Equity, Anti-racism, & Social Justice (IDEAS)
    • Innovations in Care Delivery
    • Practice Buzz
    • Practice Current
    • Residents & Fellows
    • Without Borders
  • Collections
    • Topics A-Z
    • Disputes & Debates
    • Health Disparities
    • Infographics
    • Patient Pages
    • Null Hypothesis
    • Translations
  • Podcast
  • CME
  • About
    • About the Journals
    • Contact Us
    • Editorial Board
  • Authors
    • Submit a Manuscript
    • Author Center
  • Home
  • Latest Articles
  • Current Issue
  • Past Issues
  • Residents & Fellows

User menu

  • Subscribe
  • My Alerts
  • Log in

Search

  • Advanced search
Neurology
Home
The most widely read and highly cited peer-reviewed neurology journal
  • Subscribe
  • My Alerts
  • Log in
Site Logo
  • Home
  • Latest Articles
  • Current Issue
  • Past Issues
  • Residents & Fellows

Share

February 22, 2022; 98 (8) Resident & Fellow Section

Pearls & Oy-sters: Delayed Diagnosis of Acute Motor Axonal Neuropathy With Cardiac Arrest

View ORCID ProfileSina Marzoughi, Laura Marulanda, Dian Ngo, View ORCID ProfileTychicus Chen
First published December 14, 2021, DOI: https://doi.org/10.1212/WNL.0000000000013220
Sina Marzoughi
From the Division of Neurology (S.M., L.M., T.C.), Department of Medicine, University of British Columbia; and School of Pharmacy (D.N.), Department of Pharmacy, University of British Columbia, Vancouver, Canada.
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
  • ORCID record for Sina Marzoughi
Laura Marulanda
From the Division of Neurology (S.M., L.M., T.C.), Department of Medicine, University of British Columbia; and School of Pharmacy (D.N.), Department of Pharmacy, University of British Columbia, Vancouver, Canada.
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Dian Ngo
From the Division of Neurology (S.M., L.M., T.C.), Department of Medicine, University of British Columbia; and School of Pharmacy (D.N.), Department of Pharmacy, University of British Columbia, Vancouver, Canada.
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Tychicus Chen
From the Division of Neurology (S.M., L.M., T.C.), Department of Medicine, University of British Columbia; and School of Pharmacy (D.N.), Department of Pharmacy, University of British Columbia, Vancouver, Canada.
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
  • ORCID record for Tychicus Chen
Full PDF
Citation
Pearls & Oy-sters: Delayed Diagnosis of Acute Motor Axonal Neuropathy With Cardiac Arrest
Sina Marzoughi, Laura Marulanda, Dian Ngo, Tychicus Chen
Neurology Feb 2022, 98 (8) 336-339; DOI: 10.1212/WNL.0000000000013220

Citation Manager Formats

  • BibTeX
  • Bookends
  • EasyBib
  • EndNote (tagged)
  • EndNote 8 (xml)
  • Medlars
  • Mendeley
  • Papers
  • RefWorks Tagged
  • Ref Manager
  • RIS
  • Zotero
Permissions

Make Comment

See Comments

Downloads
499

Share

  • Article
  • Figures & Data
  • Info & Disclosures
Loading

We present the case of a 53-year-old woman who presented with right lower extremity weakness with preceding systemic symptoms including fever and chest pain. She developed rapid quadriparesis over 24 hours and had ventricular fibrillation with cardiac arrest. Examination demonstrated tetraplegia, facial diplegia with spared extraocular movements, and areflexia. Electrodiagnostic studies including nerve conduction studies and EMG were consistent with acute motor axonal neuropathy. This case highlights an atypical asymmetric presentation with initially preserved reflexes, rapid progression, and cardiac dysfunction that can occur independent of dysautonomia. Treatment options include IV immunoglobulin (IVIg) or plasmapheresis as well as supportive care and long-term multidisciplinary rehabilitation and communication strategies.

Pearls

  • Asymmetric onset and rapidly progressive weakness should raise the suspicion for Guillain-Barré syndrome (GBS) and its variants.

  • Although uncommon, acute motor axonal neuropathy (AMAN) can be complicated by dysautonomia, requiring close monitoring for cardiac arrhythmia and rarely ventricular fibrillation.

Oy-sters

  • Preserved reflexes do not exclude the possibility of AMAN, where areflexia can occur later on.

  • Although CSF albuminocytologic dissociation is a classic finding in GBS, mild pleocytosis may also occur.

Case

A 53-year-old woman presented to hospital for sudden onset of right leg weakness. After dropping her children to school, she was unable to support her body on her right leg with difficulty walking back to her car. She was able to get into the car, and ankle strength was sufficient to operate the pedals. On getting out of the car, her right leg buckled again. She was unable to stand and called an ambulance. She endorsed some low back pain without any radiating pattern. Her medical history was significant for hypothyroidism and dyslipidemia, treated with levothyroxine and rosuvastatin accordingly.

On initial examination, she was afebrile with normal vital signs. She was alert and oriented, with normal language testing. Pupils were equal and reactive to light. Visual fields and extraocular movements were full. She had full facial sensation and strength. Tongue and palate were midline. Shoulders and neck were strong. Upper extremities and left leg demonstrated normal strength, sensation, deep tendon reflexes, and coordination. Right leg demonstrated grade 2/5 strength in hip flexion and 3/5 in knee flexion and extension, but otherwise 5/5 in ankle movements with normal sensation. Her Achilles reflexes were present bilaterally, but patellar reflexes were absent. Plantar response was flexor bilaterally.

An urgent CT of the head and angiography did not reveal early ischemic changes or occlusion. ECG showed normal sinus rhythm. Serum creatine kinase and TSH were normal. On serial assessments in the emergency department, there were fluctuations in the motor strength of the right leg, prompting speculation that she may have functional weakness. The patient was admitted to hospital for further investigations, namely an MRI of the lumbar spine. Within the next 24 hours, she developed rapid quadriparesis and dysphagia to liquids and solids. She was found unresponsive without a pulse with ventricular fibrillation, and CPR was initiated. She achieved return of spontaneous circulation after 1 shock and 6 minutes of CPR. She was intubated and was transferred to the intensive care unit. Repeat ECG showed sinus rhythm with T-wave inversions, mildly prolonged QTc at 500 msec; troponin and B-type natriuretic peptide were unremarkable. The cardiology team was not able to identify a specific cause or trigger for the ventricular fibrillation including no significant electrolyte imbalances, toxic exposure, or structural cardiac abnormalities.

After discontinuation of anesthetics, she remained ventilator dependent with absent cough and gag. She was alert with spontaneous eye movements and blinking. Her pupils remained equal and reactive to light. Extraocular movements remained full, and she had clear bifacial weakness with incomplete eye closure and scleral show left more than right and no frontalis or orbicularis oris activation. She had flaccid tone and no motor activity or response throughout the extremities. Deep tendon reflexes were globally absent. She was able to reliably answer yes/no questions with eye movements, and she reported preservation of light touch, pain, and vibration sensation in all extremities. Lumbar puncture revealed elevated protein (0.70 g/L; ref 0.15–0.45) and lymphocytic (69%) pleocytosis (9 x 106/L; ref 0–5), with otherwise normal cytology and negative viral testing. Plasma porphyrin and urinary porphobilinogen were normal. MRI of the brain and spine was unremarkable. Nerve conduction studies performed 8 days after presentation revealed normal sensory responses for the superficial radial, ulnar dorsal cutaneous, and sural sensory nerves with absent median, ulnar, fibular, and tibial motor responses, and EMG showed no spontaneous activity or motor unit activation (Table).

View this table:
  • View inline
  • View popup
  • Download powerpoint
Table

Nerve Conduction Study and EMG Demonstrating Findings Consistent With a Severe Motor Axonal Variant of Guillain-Barré Syndrome

She was treated with IV immunoglobulin (IVIG) 2 g/kg over 5 days and had minimal response. Four weeks after her last dose of IVIG, she received 5 sessions of plasma exchange. Over the next several weeks, she had some improvement in terms of neck flexion and extension strength. Examined at 7-month follow-up, she has also made improvements with regard to extraocular movements, lateral head movements, and full strength at the trapezius bilaterally. She is now also able to speak with a flaccid dysarthria.

Data Availability

All data presented or referenced in the report below can be provided in full on request.

Discussion

GBS is a demyelinating immune-mediated polyneuropathy, an important cause of acquired neuromuscular weakness.1 It encompasses several subtypes, most commonly acute inflammatory demyelinating polyneuropathy (AIDP).1 Another variant of GBS is acute motor axonal neuropathy (AMAN). AMAN is characterized as a pure motor axonal subtype of GBS with a more rapid progression and earlier nadir than demyelinating GBS. Unlike the typical form of AIDP, reflexes in AMAN can be preserved and autonomic manifestations are relatively rare.1,-,3

The pathophysiology is mediated by molecular mimicry with findings in the serum of patients to various antiganglioside antibodies, most commonly anti-GM1, anti-GD1a, and anti-GD1b.4 In comparison to AIDP, patients with AMAN less commonly involve the cranial nerves and sensory deficits. In addition, recovery from axonal regeneration takes much longer compared with recovery from demyelinating processes. Two patterns of recovery are typically seen in patients with AMAN: a subset of patients who recover within days, whereas others have a very prolonged time of recovery.5

Electrodiagnostic studies play a critical role in the diagnosis of AMAN, as they do in AIDP. Although the latter is characterized by slowing of conduction velocity, conduction block or temporal dispersion indicative of demyelination in 2 or more motor nerves, AMAN is characterized by reduced amplitudes of distally evoked compound muscle action potentials without significant reduction in conduction velocity, with normal sensory nerve conduction studies.1,6 Axonal damage is typically associated with positive sharp waves and fibrillations but can take up to 3 weeks to appear. Given that nerve conduction studies and EMG were performed 8 days after presentation in our case, this may explain the absence of spontaneous activity seen on EMG.

Autonomic dysfunction in GBS has a wide spectrum of manifestations, most commonly sinus arrhythmias, labile blood pressure, urinary retention, pupillary abnormalities, and sudomotor dysfunction.6 In contrast with other variants of GBS, patients with AMAN rarely have the autonomic dysfunction.1 Dysautonomia is more common in patients with more severe disease such as those with quadriparesis, neck flexor, and bulbar weakness and on mechanical ventilation.7 Patients with dysautonomia have been found to have more major complications that have not been necessarily related to the dysautonomia; this includes cardiac arrhythmias with ventricular tachycardia, atrial fibrillation with rapid ventricular response, neurogenic stunned myocardium, cardiomyopathy, syndrome of inappropriate antidiuretic hormone secretion, and posterior reversible encephalopathy syndrome.7,8 CSF evaluation in GBS can reveal albuminocytologic dissociation, mild pleocytosis, or normal CSF studies, particularly during the first few weeks of presentation for both classic AIDP and variants such as AMAN while demyelinating forms may show higher levels of protein.9

We present a case of AMAN with several educational features for clinicians. For one, the presence or even exaggeration of reflexes should not rule out a diagnosis of AMAN as about 10% of patients have preserved reflexes throughout the disease course.4 In addition, initial physical examination features of our case may have appeared as functional weakness including knee buckling and give-way weakness on initial strength examination. This highlights the importance of serial physical examination and careful observation of the patient moving around their environment.

Another learning point is the significance of autonomic manifestations. Our case illustrates that patients with AMAN can have cardiac complications that may or may not be directly related to dysautonomia, for instance, the cardiac arrest and postarrest atrial fibrillation. Consequently, close monitoring with telemetry is indicated in these patients.

With regard to treatment, IVIG and plasma exchange have proved in numerous clinical trials to be efficacious for AIDP.8 However, as most of these trials were conducted in North America and Europe, most of the patients included were likely AIDP rather than patients with the axonal variant, and it is unclear whether these therapies are efficacious for AMAN. There have been 2 studies that suggest that IVIG may be better for AMAN compared with plasma exchange, although the findings have not been consistent.1 Given the lack of evidence for either treatment or both treatments in AMAN, our team had a discussion with the patient's family and ultimately decided to treat with plasma exchange in addition to IVIG approximately 4 weeks after the course of IVIG was finished. Treatment decisions for AMAN must be done on a case-by-case basis given the lack of large randomized trials with supporting evidence.

Finally, communication was a significant challenge given the severity of our patient's weakness; she initially only had ability to control her extraocular muscles. As such, finding strategies to help patients communicate (Figure) as well as keeping them engaged through entertainment, cognitive activities, and social interaction can be a critical part of patient care. In addition, this case highlights that reliable nonverbal communication provided confirmation of preserved sensory function on clinical examination, allowing a presumptive diagnosis of AMAN before electrodiagnostic testing.

Figure
  • Download figure
  • Open in new tab
  • Download powerpoint
Figure Illustration of the Method Used to Communicate With Our Patient Given Only Intact Extraocular Movements

To use this tool, the patient was asked which letter she is indicating by clarifying which box (“is it 1? 2? 3?...”) for which the patient would look up for “yes” and down for “no”. To proceed, she was then asked to clarify which letter in that box by being asked one at a time (“is it A? B? …”).

Study Funding

No targeted funding reported.

Disclosure

The authors report no disclosures relevant to the manuscript. Go to Neurology.org/N for full disclosures.

Appendix Authors

Table

Footnotes

  • Go to Neurology.org/N for full disclosures. Funding information and disclosures deemed relevant by the authors, if any, are provided at the end of the article.

  • © 2021 American Academy of Neurology

References

  1. 1.↵
    1. Kuwabara S,
    2. Yuki N
    . Axonal Guillain-Barré syndrome: concepts and controversies. Lancet Neurol. 2013;12(12):1180-1188.
    OpenUrlCrossRefPubMed
  2. 2.↵
    1. McKhann GM,
    2. Cornblath DR,
    3. Ho T, et al.
    Clinical and electrophysiological aspects of acute paralytic disease of children and young adults in northern China. Lancet. 1991;338(8767):593-597.
    OpenUrlCrossRefPubMed
  3. 3.↵
    1. Yuki N,
    2. Kokubun N,
    3. Kuwabara S, et al.
    Guillain-Barré syndrome associated with normal or exaggerated tendon reflexes. J Neurol. 2012;259(6):1181-1190.
    OpenUrlCrossRefPubMed
  4. 4.↵
    1. Magid-Bernstein J,
    2. Al-Mufti F,
    3. Merkler AE, et al
    . Unexpected rapid improvement and neurogenic stunned myocardium in a patient with acute motor axonal neuropathy: a case report and literature review. J Clin Neuromuscul Dis. 2016;17(3):135-141.
    OpenUrl
  5. 5.↵
    1. Hiraga A,
    2. Mori M,
    3. Ogawara K,
    4. Hattori T,
    5. Kuwabara S
    . Differences in patterns of progression in demyelinating and axonal Guillain-Barré syndromes. Neurology. 2003;61(4):471-474.
    OpenUrlAbstract/FREE Full Text
  6. 6.↵
    1. Sheikh KA
    . Guillain-Barré syndrome. Continuum (Minneap Minn). 2020;26(5):1184-1204.
    OpenUrl
  7. 7.↵
    1. Chakraborty T,
    2. Kramer CL,
    3. Wijdicks EFM,
    4. Rabinstein AA
    . Dysautonomia in Guillain–Barré syndrome: prevalence, clinical spectrum, and outcomes. Neurocrit Care. 2020;32(1):113-120.
    OpenUrl
  8. 8.↵
    1. Zaeem Z,
    2. Siddiqi ZA,
    3. Zochodne DW
    . Autonomic involvement in Guillain–Barré syndrome: an update. Clin Auton Res. 2019;29(3):289-299.
    OpenUrlPubMed
  9. 9.↵
    1. Dimachkie MM,
    2. Barohn RJ
    . Guillain-Barré syndrome and variants. Neurol Clin. 2013;31(2):491.
    OpenUrlPubMed
  10. 10.
    1. Leonhard SE,
    2. Mandarakas MR,
    3. Gondim FAA, et al
    . Diagnosis and management of Guillain-Barré syndrome in ten steps. Nat Rev Neurol. 2019;15(11):671-683.
    OpenUrlCrossRefPubMed

Disputes & Debates: Rapid online correspondence

No comments have been published for this article.
Comment

REQUIREMENTS

If you are uploading a letter concerning an article:
You must have updated your disclosures within six months: http://submit.neurology.org

Your co-authors must send a completed Publishing Agreement Form to Neurology Staff (not necessary for the lead/corresponding author as the form below will suffice) before you upload your comment.

If you are responding to a comment that was written about an article you originally authored:
You (and co-authors) do not need to fill out forms or check disclosures as author forms are still valid
and apply to letter.

Submission specifications:

  • Submissions must be < 200 words with < 5 references. Reference 1 must be the article on which you are commenting.
  • Submissions should not have more than 5 authors. (Exception: original author replies can include all original authors of the article)
  • Submit only on articles published within 6 months of issue date.
  • Do not be redundant. Read any comments already posted on the article prior to submission.
  • Submitted comments are subject to editing and editor review prior to posting.

More guidelines and information on Disputes & Debates

Compose Comment

More information about text formats

Plain text

  • No HTML tags allowed.
  • Web page addresses and e-mail addresses turn into links automatically.
  • Lines and paragraphs break automatically.
Author Information
NOTE: The first author must also be the corresponding author of the comment.
First or given name, e.g. 'Peter'.
Your last, or family, name, e.g. 'MacMoody'.
Your email address, e.g. higgs-boson@gmail.com
Your role and/or occupation, e.g. 'Orthopedic Surgeon'.
Your organization or institution (if applicable), e.g. 'Royal Free Hospital'.
Publishing Agreement
NOTE: All authors, besides the first/corresponding author, must complete a separate Publishing Agreement Form and provide via email to the editorial office before comments can be posted.
CAPTCHA
This question is for testing whether or not you are a human visitor and to prevent automated spam submissions.

Vertical Tabs

You May Also be Interested in

Back to top
  • Article
    • Pearls
    • Oy-sters
    • Case
    • Discussion
    • Study Funding
    • Disclosure
    • Appendix Authors
    • Footnotes
    • References
  • Figures & Data
  • Info & Disclosures
Advertisement

Related Articles

  • Resident & Fellow Rounds: February 2022

Alert Me

  • Alert me when eletters are published
Neurology: 98 (21)

Articles

  • Ahead of Print
  • Current Issue
  • Past Issues
  • Popular Articles
  • Translations

About

  • About the Journals
  • Ethics Policies
  • Editors & Editorial Board
  • Contact Us
  • Advertise

Submit

  • Author Center
  • Submit a Manuscript
  • Information for Reviewers
  • AAN Guidelines
  • Permissions

Subscribers

  • Subscribe
  • Activate a Subscription
  • Sign up for eAlerts
  • RSS Feed
Site Logo
  • Visit neurology Template on Facebook
  • Follow neurology Template on Twitter
  • Visit Neurology on YouTube
  • Neurology
  • Neurology: Clinical Practice
  • Neurology: Genetics
  • Neurology: Neuroimmunology & Neuroinflammation
  • Neurology: Education
  • AAN.com
  • AANnews
  • Continuum
  • Brain & Life
  • Neurology Today

Wolters Kluwer Logo

Neurology | Print ISSN:0028-3878
Online ISSN:1526-632X

© 2022 American Academy of Neurology

  • Privacy Policy
  • Feedback
  • Advertise