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Comparative Study
. 2024 Dec;31(12):e16444.
doi: 10.1111/ene.16444. Epub 2024 Sep 5.

Comparison of the diagnostic accuracy of the 2010 European Federation of Neurological Societies/Peripheral Nerve Society and American Association of Electrodiagnostic Medicine diagnostic criteria for multifocal motor neuropathy

Pietro Emiliano Doneddu  1   2 , Chiara Gallo  1 , Luca Gentile  3 , Dario Cocito  4 , Yuri Falzone  5 , Vincenzo Di Stefano  6 , Maurizio Inghilleri  7   8 , Giuseppe Cosentino  9 , Sabrina Matà  10 , Anna Mazzeo  3 , Massimiliano Filosto  11   12 , Erdita Peci  13 , Benedetta Sorrenti  5 , Filippo Brighina  6 , Federica Moret  7   8 , Elisa Vegezzi  9 , Martina Sperti  10 , Barbara Risi  14 , Eduardo Nobile-Orazio  1   15 ; on the behalf of the Italian MMN Database Study Group
Affiliations
Comparative Study

Comparison of the diagnostic accuracy of the 2010 European Federation of Neurological Societies/Peripheral Nerve Society and American Association of Electrodiagnostic Medicine diagnostic criteria for multifocal motor neuropathy

Pietro Emiliano Doneddu et al. Eur J Neurol. 2024 Dec.

Abstract

Background and purpose: This study was undertaken to compare the sensitivity and specificity of the 2010 European Federation of Neurological Societies/Peripheral Nerve Society (EFNS/PNS) diagnostic criteria for multifocal motor neuropathy (MMN) with those of the American Association of Electrodiagnostic Medicine (AAEM).

Methods: Sensitivity and specificity of the two sets of criteria were retrospectively evaluated in 53 patients with MMN and 280 controls with axonal peripheral neuropathy, inflammatory demyelinating polyneuropathy, or amyotrophic lateral sclerosis. Comparison of the utility of nerve conduction studies with different numbers of nerves examined was also assessed.

Results: The 2010 EFNS/PNS criteria had a sensitivity of 47% for definite MMN and 57% for probable/definite MMN, whereas the AAEM criteria had a sensitivity of 28% for definite MMN and 53% for probable/definite MMN. The sensitivity of the AAEM criteria was higher when utilizing area compared to amplitude reduction to define conduction block. Using supportive criteria, the sensitivity of the 2010 EFNS/PNS criteria for probable/definite MMN increased to 64%, and an additional 36% patients fulfilled the criteria (possible MMN). Specificity values for definite and probable/definite MMN were slightly higher with the AAEM criteria (100%) compared to the EFNS/PNS criteria (98.5% and 97%). Extended nerve conduction studies yielded slightly increased diagnostic sensitivity for both sets of criteria without significantly affecting specificity.

Conclusions: In our patient populations, the 2010 EFNS/PNS criteria demonstrated higher sensitivity but slightly lower specificity compared to the AAEM criteria. Extended nerve conduction studies are advised to achieve slightly higher sensitivity while maintaining very high specificity.

Keywords: MMN; diagnosis; diagnostic criteria; guidelines; multifocal motor neuropathy.

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Conflict of interest statement

P.E.D. has received travel grants to attend scientific meetings from CSL Behring and Kedrion. D.C. has received honoraria for lecturing from Shire, CSL Behring, and Kedrion and travel grants to attend scientific meetings from Shire, Kedrion, and CSL Behring. A.M. has received travel grants from Kedrion and CSL Behring to attend scientific meetings. M.F. has served on scientific advisory boards for CSL Behring, Sanofi, and Amicus and has received travel grants from Sanofi, Biogen, Kedrion, and CSL Behring to attend scientific meetings. G.C. has received travel grants to attend scientific meetings from CSL Behring and Kedrion. M.I. has received travel grants to attend scientific meetings from CSL Behring and Alexion. E.P. has received travel grants to attend scientific meetings from CSL Behring. E.N.‐O. reports personal fees for advisory or scientific boards from ArgenX (Belgium), Takeda (Italy and USA), CSL‐Behring (Italy and USA), Janssen (USA), Kedrion (Italy), LFB (France), Roche (Switzerland), and Sanofi (USA). The other authors declare no conflict of interest.

Figures

FIGURE 1
FIGURE 1
Flowchart of patients' selection for sensitivity analyses of the 2010 European Federation of Neurological Societies/Peripheral Nerve Society (EFNS/PNS) and American Association of Electrodiagnostic Medicine (AAEM) criteria for multifocal motor neuropathy (MMN). CMAP, compound muscle action potential; NCS, nerve conduction study.
See this image and copyright information in PMC

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