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Neuropediatrics 2025; 56(04): 274-277
DOI: 10.1055/a-2532-4172
Short Communication

EBV and Concomitant Acute Motor and Sensory Axonal Neuropathy in a Healthy 15-Year-Old Female

Rebecca E. Basch 
1   Division of General Pediatrics, Connecticut Children's, Hartford, Connecticut, United States
2   Department of Pediatrics, University of Connecticut School of Medicine, Farmington, Connecticut, United States
,
McKenna Tierney
1   Division of General Pediatrics, Connecticut Children's, Hartford, Connecticut, United States
2   Department of Pediatrics, University of Connecticut School of Medicine, Farmington, Connecticut, United States
,
Lila Worden
2   Department of Pediatrics, University of Connecticut School of Medicine, Farmington, Connecticut, United States
3   Division of Neurology, Connecticut Children's, Hartford, Connecticut, United States
,
Sara Sanders
2   Department of Pediatrics, University of Connecticut School of Medicine, Farmington, Connecticut, United States
4   Division of Critical Care, Connecticut Children's, Hartford, Connecticut, United States
,
Elizabeth A. Ng
2   Department of Pediatrics, University of Connecticut School of Medicine, Farmington, Connecticut, United States
3   Division of Neurology, Connecticut Children's, Hartford, Connecticut, United States
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Funding None.
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Abstract

Acute motor and sensory axonal neuropathy (AMSAN) is a rare and severe form of acute axonal injury caused by immune damage to the axonal membrane. AMSAN is an axonal variant of GBS. GBS occurs from immune injury to the myelin sheath, axonal variants of GBS (AMSAN and AMAN) differ in that insult is to the axonal membrane. AMSAN is seldom seen, especially in pediatric and adolescent patients. Unlike acute motor axonal neuropathy (AMAN), which has been well-described in literature to be secondary to Campylobacter jejuni infection, there is no known etiology of AMSAN. Here, we present a case of an otherwise healthy 15-year-old female who presented with new-onset facial and bulbar weakness that rapidly progressed to functional paralysis requiring intubation. With no clear diagnosis and after failure in improvement with high-dose steroids, Intravenous Immunoglobulin (IVIG), and plasma exchange transfusion, diagnosis was finally made with electromyography (EMG) and nerve conduction study (NCS). In addition, extensive laboratory work was completed and was only notable for primary acute EBV infection. This case represents a new presenting symptom of AMSAN, a unique finding of concomitant primary EBV infection, the possibility of primary Epstein–Barr virus (EBV) infection as the triggering event in AMSAN and stresses the importance of EMG and NCS when evaluating patients with weakness.

Keywords

acute motor and sensory axonal neuropathy - acute inflammatory demyelinating polyneuropathy - Epstein–Barr virus - acute motor axonal neuropathy


Publication History

Received: 08 November 2024

Accepted: 03 February 2025

Accepted Manuscript online:
04 February 2025

Article published online:
20 February 2025

© 2025. Thieme. All rights reserved.

Georg Thieme Verlag KG
Oswald-Hesse-Straße 50, 70469 Stuttgart, Germany

 

  • References

  • 1 Hahn AF. Guillain-Barré syndrome. Lancet 1998; 352 (9128): 635-641
    Search in Google Scholar
  • 2 Rees JH, Soudain SE, Gregson NA, Hughes RA. Campylobacter jejuni infection and Guillain-Barré syndrome. N Engl J Med 1995; 333 (21) 1374-1379
    Search in Google Scholar
  • 3 Hughes RA, Cornblath DR. Guillain-Barré syndrome. Lancet 2005; 366 (9497): 1653-1666
    Search in Google Scholar
  • 4 Van den Berg B, Walgaard C, Drenthen J, Fokke C, Jacobs B, van Doorn P. Gillian-Barre syndrome: pathogenesis, diagnosis, treatment, and prognosis. Nat Rev Neurol 2014; 10: 469-482
    Search in Google Scholar
  • 5 Singh S, Gupta N, Gupta AM, Chandel AS, Waghela S, Saple P. Clinical profile and predictors for outcome in children presenting with Guillain-Barré syndrome. J Family Med Prim Care 2020; 9 (10) 5316-5319
    Search in Google Scholar
  • 6 Rho YI. Overlapping Guillain-Barré syndrome and Bickerstaff's brainstem encephalitis associated with Epstein Barr virus. Korean J Pediatr 2014; 57 (10) 457-460
    Search in Google Scholar
  • 7 Surve RM, Sharma P, Nisal R, Chakrabarti D, Raghavendra K, Kulkarni GB, Kamath S. Clinical characteristics and functional outcomes of pediatric Guillain-Barré syndrome admitted to the Neuro-intensive care unit: a decade-long retrospective observational study. Neurol Sci 2025; 46 (03) 1369-1377
    Search in Google Scholar
  • 8 Chaweekulrat P, Sanmaneechai O. Prognostic model for time to achieve independent walking in children with Guillain-Barré syndrome. Pediatr Res 2022; 92 (05) 1417-1422
    Search in Google Scholar
  • 9 Florian IA, Lupan I, Sur L, Samasca G, Timiș TL. To be, or not to be… Guillain-Barré Syndrome. Autoimmun Rev 2021; 20 (12) 102983
    Search in Google Scholar