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. 2022 Feb;29(2):593-604.
doi: 10.1111/ene.15170. Epub 2021 Nov 24.

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

Jelte Helfferich  1 , Joyce Roodbol  2 , Marie-Claire de Wit  3 , Oebele F Brouwer  1 , Bart C Jacobs  4 ; 2016 Enterovirus D68 Acute Flaccid Myelitis Working Group and the Dutch Pediatric GBS Study Group
Collaborators, Affiliations

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

Jelte Helfferich et al. Eur J Neurol. 2022 Feb.

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.

Keywords: Brighton criteria; Guillain-Barré syndrome; acute flaccid myelitis.

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Conflict of interest statement

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.

Figures

FIGURE 1
FIGURE 1
(a) Duration of mechanical ventilation in the acute flaccid myelitis (AFM) and Guillain–Barré syndrome (GBS) cohorts. Three patients from the AFM cohort were still intubated at 200 days. The difference between the duration of intubation between the two groups was based on log‐rank test. (b) Long‐term prognosis, indicating time until the ability to walk unaided. Included were the patients who were unable to walk unaided at nadir (GBS disability score >2). The long‐term follow‐up was available until 1 year after onset of weakness. The difference between the duration until independent walking between the two groups was based on log‐rank test
FIGURE 2
FIGURE 2
Performance of the Brighton diagnostic criteria for Guillain–Barré syndrome (GBS) in the acute flaccid myelitis (AFM) and GBS cohorts. Percentage of patients fulfilling the various levels of the GBS diagnostic criteria from the Brighton Collaboration (except for the criterion to exclude an alternative diagnosis in the AFM cohort) are shown [5]. Level 1: Patient fulfills all criteria. Level 2: All items of Level 1 except the cerebrospinal fluid findings are not required. Level 3: All items of Level 2 except nerve conduction studies findings are not required. Level 4: No alternative diagnosis can be present; all other criteria are not required. For the AFM patients, this criteria was excluded
FIGURE 3
FIGURE 3
Venn diagram illustrating overlapping and differentiating features of acute flaccid myelitis (AFM) and Guillain–Barré syndrome (GBS). The indicated features are suggestive of either diagnosis, but they are not necessarily present or exclusive. CSF, cerebrospinal fluid; EMG, electromyography; MRI, magnetic resonance imaging
See this image and copyright information in PMC

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