Acute flaccid myelitis and Guillain-Barré syndrome in children: A comparative study with evaluation of diagnostic criteria

Jelte Helfferich, Joyce Roodbol, 2016 Enterovirus D68 Acute Flaccid Myelitis Working Group and the Dutch Pediatric GBS Study Group, Marie Claire de Wit, Oebele F. Brouwer, Bart C. Jacobs*

*Corresponding author for this work

Research output: Contribution to journalArticleAcademicpeer-review

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Abstract

BACKGROUND AND PURPOSE: Differentiation between acute flaccid myelitis (AFM) and Guillain-Barré syndrome (GBS) can be difficult, particularly in children. Our objective was to improve the diagnostic accuracy by giving recommendations based on a comparison of clinical features and diagnostic criteria in children with AFM or GBS. METHODS: A cohort of 26 children with AFM associated with enterovirus D68 was compared to a cohort of 156 children with GBS. The specificity of the Brighton criteria, used for GBS diagnosis, was evaluated in the AFM cohort and the specificity of the Centers for Disease Control and Prevention (CDC) AFM diagnostic criteria in the GBS cohort. RESULTS: Children with AFM compared to those with GBS had a shorter interval between onset of weakness and nadir (3 vs. 8 days, p < 0.001), more often had asymmetric limb weakness (58% vs. 0%, p < 0.001), and less frequently had sensory deficits (0% vs. 40%, p < 0.001). In AFM, cerebrospinal fluid leukocyte counts were higher, whereas protein concentrations were lower. Spinal cord lesions on magnetic resonance imaging were only found in AFM patients. No GBS case fulfilled CDC criteria for definite AFM. Of the AFM cases, 8% fulfilled the Brighton criteria for GBS, when omitting the criterion of excluding an alternate diagnosis. CONCLUSIONS: Despite the overlap in clinical presentation, we found distinctive early clinical and diagnostic characteristics for differentiating AFM from GBS in children. Diagnostic criteria for AFM and GBS usually perform well, but some AFM cases may fulfill clinical diagnostic criteria for GBS. This underlines the need to perform diagnostic tests early to exclude AFM in children suspected of atypical GBS.

Original languageEnglish
Pages (from-to)593-604
Number of pages12
JournalEuropean Journal of Neurology
Volume29
Issue number2
Early online date24 Nov 2021
DOIs
Publication statusPublished - 1 Feb 2022

Bibliographical note

Funding Information:
M.C.d.W. has received honoraria paid to her institution by Novartis for serving on a steering committee and presenting at a conference, and has received research funding from the Epilepsiefonds (Dutch Epilepsy Foundation), Hersenstichting, and Sophia Foundation. B.C.J. has received funding for travel from Baxter International, and has received research funding from the Netherlands Organization for Health Research and Development, Erasmus MC, Prinses Beatrix Spierfonds, Stichting Spieren voor Spieren, CSL‐Behring, Grifols, Annexon, Hansa Biopharma, and the GBS‐CIDP Foundation International. None of the other authors has any conflict of interest to disclose.

Funding Information:
The Prinses Beatrix Spierfonds funded the PhD project of J.R. on GBS in children (project number: W.OR12-04)

Publisher Copyright:
© 2021 The Authors. European Journal of Neurology published by John Wiley & Sons Ltd on behalf of European Academy of Neurology.

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