Pediatric acute flaccid myelitis: Evaluation of diagnostic criteria and differentiation from other causes of acute flaccid paralysis

Jelte Helfferich*, Rinze F. Neuteboom, Marit M.A. de Lange, Kimberley S.M. Benschop, Coretta C. Van Leer-Buter, Adam Meijer, Dewi P. Bakker, Eva de Bie, Hilde M.H. Braakman, Rick Brandsma, Erik H. Niks, Jikke Mien Niermeijer, Vincent Roelfsema, Niels Schoenmaker, Lilian T. Sie, Hubert G. Niesters, Margreet J.M. te Wierik, Bart C. Jacobs, Oebele F. Brouwer

*Corresponding author for this work

Research output: Contribution to journalArticleAcademicpeer-review

Abstract

Background: Acute flaccid paralysis (AFP) is characterized by rapidly progressive limb weakness with low muscle tone. It has a broad differential diagnosis, which includes acute flaccid myelitis (AFM), a rare polio-like condition that mainly affects young children. Differentiation between AFM and other causes of AFP may be difficult, particularly at onset of disease. Here, we evaluate the diagnostic criteria for AFM and compare AFM to other causes of acute weakness in children, aiming to identify differentiating clinical and diagnostic features. Methods: The diagnostic criteria for AFM were applied to a cohort of children with acute onset of limb weakness. An initial classification based on positive diagnostic criteria was compared to the final classification, based on application of features suggestive for an alternative diagnosis and discussion with expert neurologists. Cases classified as definite, probable, or possible AFM or uncertain, were compared to cases with an alternative diagnosis. Results: Of 141 patients, seven out of nine patients initially classified as definite AFM, retained this label after further classification. For probable AFM, this was 3/11, for possible AFM 3/14 and for uncertain 11/43. Patients initially classified as probable or possible AFM were most commonly diagnosed with transverse myelitis (16/25). If the initial classification was uncertain, Guillain-Barré syndrome was the most common diagnosis (31/43). Clinical and diagnostic features not included in the diagnostic criteria, were often used for the final classification. Conclusion: The current diagnostic criteria for AFM usually perform well, but additional features are sometimes required to distinguish AFM from other conditions.

Original languageEnglish
Pages (from-to)28-36
Number of pages9
JournalEuropean Journal of Paediatric Neurology
Volume44
DOIs
Publication statusPublished - May 2023

Keywords

  • Acute flaccid myelitis
  • Acute flaccid paralysis
  • Enterovirus D68
  • Guillain-Barré syndrome
  • Transverse myelitis

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