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. 2024 Sep;31(9):e16335.
doi: 10.1111/ene.16335. Epub 2024 Jul 4.

Electrodiagnostic subtyping in Guillain-Barré syndrome patients in the International Guillain-Barré Outcome Study

Collaborators, Affiliations

Electrodiagnostic subtyping in Guillain-Barré syndrome patients in the International Guillain-Barré Outcome Study

Samuel Arends et al. Eur J Neurol. 2024 Sep.

Abstract

Background and purpose: Various electrodiagnostic criteria have been developed in Guillain-Barré syndrome (GBS). Their performance in a broad representation of GBS patients has not been evaluated. Motor conduction data from the International GBS Outcome Study (IGOS) cohort were used to compare two widely used criterion sets and relate these to diagnostic amyotrophic lateral sclerosis criteria.

Methods: From the first 1500 patients in IGOS, nerve conduction studies from 1137 (75.8%) were available for the current study. These patients were classified according to nerve conduction studies criteria proposed by Hadden and Rajabally.

Results: Of the 1137 studies, 68.3% (N = 777) were classified identically according to criteria by Hadden and Rajabally: 111 (9.8%) axonal, 366 (32.2%) demyelinating, 195 (17.2%) equivocal, 35 (3.1%) inexcitable and 70 (6.2%) normal. Thus, 360 studies (31.7%) were classified differently. The areas of differences were as follows: 155 studies (13.6%) classified as demyelinating by Hadden and axonal by Rajabally; 122 studies (10.7%) classified as demyelinating by Hadden and equivocal by Rajabally; and 75 studies (6.6%) classified as equivocal by Hadden and axonal by Rajabally. Due to more strictly defined cutoffs fewer patients fulfilled demyelinating criteria by Rajabally than by Hadden, making more patients eligible for axonal or equivocal classification by Rajabally. In 234 (68.6%) axonal studies by Rajabally the revised El Escorial (amyotrophic lateral sclerosis) criteria were fulfilled; in axonal cases by Hadden this was 1.8%.

Conclusions and discussion: This study shows that electrodiagnosis in GBS is dependent on the criterion set utilized, both of which are based on expert opinion. Reappraisal of electrodiagnostic subtyping in GBS is warranted.

Keywords: Guillain–Barré syndrome; amyotrophic lateral sclerosis; electrodiagnosis; nerve conduction studies; polyneuropathy.

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Conflict of interest statement

Dr. Dimachkie serves or recently served as a consultant for Abata/Third Rock, Abcuro, Amicus, ArgenX, Astellas, Cabaletta Bio, Catalyst, CNSA, Covance/Labcorp, CSL‐Behring, Dianthus, Horizon, EMD Serono/Merck, Ig Society, Inc, Ipsen, Janssen, Medlink, Nuvig, Octapharma, Priovant, Sanofi Genzyme, Shire Takeda, TACT/Treat NMD, UCB Biopharma, Valenza Bio and Wolters Kluwer Health/UpToDate.

Dr. Dimachkie received research grants or contracts or educational grants from Alexion/ AstraZeneca, Alnylam Pharmaceuticals, Amicus, Argenx, Bristol‐Myers Squibb, Catalyst, CSL‐Behring, FDA/OOPD, GlaxoSmithKline, Genentech, Grifols, Mitsubishi Tanabe Pharma, MDA, NIH, Novartis, Octapharma, Orphazyme, Ra Pharma/UCB, Sanofi Genzyme, Sarepta Therapeutics, Shire Takeda, Spark Therapeutics, The Myositis Association, and UCB Biopharma / RaPharma.

Figures

FIGURE 1
FIGURE 1
Distribution of ulnar nerve NCS variables in IGOS. Ulnar nerve NCS variables: (a) distal motor latency versus dCMAP amplitude; (b) motor conduction velocity versus dCMAP amplitude; (c) F‐wave minimal latency versus dCMAP amplitude; (d) distribution of dCMAP amplitude of the ulnar nerves. ULN, upper limit of normal; LLN, lower limit of normal. A line was drawn at the cutoffs for DML (130% ULN), MCV (70% LLN) and F‐wave latency (130% ULN) derived from the revised El Escorial (exclusion) criteria.

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