. 2024 May;31(5):e16248.

doi: 10.1111/ene.16248. Epub 2024 Feb 20.

Assessment of diagnostic criteria for multifocal motor neuropathy in patients included in the Italian database

Pietro Emiliano Doneddu^{1

2}, Luca Gentile³, Dario Cocito⁴, Raffaella Fazio⁵, Marco Luigetti^{6

7}, Chiara Briani⁸, Massimiliano Filosto⁹, Gabriele Siciliano¹⁰, Luana Benedetti¹¹, Giovanni Antonini¹², Sabrina Matà¹³, Girolama Alessandra Marfia¹⁴, Maurizio Inghilleri^{15

16}, Fiore Manganelli¹⁷, Giuseppe Cosentino¹⁸, Filippo Brighina¹⁹, Marinella Carpo²⁰, Francesca Carta¹, Anna Mazzeo³, Erdita Peci²¹, Camilla Strano⁵, Angela Romano^{6

7}, Marta Campagnolo⁸, Stefano Cotti-Piccinelli⁹, Divina Valeria Viola¹⁰, Francesco Germano¹¹, Luca Leonardi¹², Martina Sperti¹³, Giorgia Mataluni¹⁴, Marco Ceccanti¹⁵, Emanuele Spina¹⁷, Elisa Vegezzi¹⁸, Vincenzo Di Stefano¹⁹, Eduardo Nobile-Orazio^{1

22}

Affiliations

¹ Neuromuscular and Neuroimmunology Unit, IRCCS Humanitas Research Hospital, Rozzano, Italy.
² Department of Biomedical Sciences, Humanitas University, Pieve Emanuele, Italy.
³ Department of Clinical and Experimental Medicine, Unit of Neurology, University of Messina, Messina, Italy.
⁴ Department of Clinical and Biological Sciences, University of Torino, Torino, Italy.
⁵ Division of Neuroscience, Department of Neurology, Institute of Experimental Neurology (INSPE), San Raffaele Scientific Institute, Milano, Italy.
⁶ Fondazione Policlinico Universitario Agostino Gemelli IRCCS, UOC Neurologia, Roma, Italy.
⁷ Università Cattolica del Sacro Cuore, Sede di Roma, Roma, Italy.
⁸ Neurology Unit, Department of Neuroscience, University of Padova, Padova, Italy.
⁹ Department of Clinical and Experimental Sciences, University of Brescia; NeMO-Brescia Clinical center for Neuromuscular Diseases, Brescia, Italy.
¹⁰ Neurology Unit, Department of Clinical and Experimental Medicine, University of Pisa, Pisa, Italy.
¹¹ IRCCS Ospedale Policlinico San Martino, Genova, Italy.
¹² Unit of Neurophysiopathology, Department of Neurology Mental Health and Sensory Organs (NESMOS), Faculty of Medicine and Psychology, Sant'Andrea Hospital, Sapienza' University of Rome, Roma, Italy.
¹³ Department of Neurological and Psychiatric Sciences, Azienda Ospedaliero-Universitaria di Careggi, Florence, Italy.
¹⁴ Dysimmune Neuropathies Unit, Department of Systems Medicine, Tor Vergata University of Rome, Roma, Italy.
¹⁵ Department of Human Neurosciences, Sapienza University of Rome, Rome, Italy.
¹⁶ IRCCS Neuromed, Pozzilli, Italy.
¹⁷ Department of Neuroscience, Reproductive Sciences and Odontostomatology, University of Naples 'Federico II', Napoli, Italy.
¹⁸ Department of Brain and Behavioral Sciences, IRCCS Mondino Foundation, University of Pavia, Pavia, Italy.
¹⁹ Azienda Ospedaliera Universitaria Policlinico Paolo Giaccone, Palermo, Italy.
²⁰ ASST Bergamo Ovest-Ospedale Treviglio, Treviglio, Italy.
²¹ Presidio Sanitario Major, Istituti Clinici Scientifici Maugeri, Torino, Italy.
²² Department of Medical Biotechnology and Translational Medicine, Milan University, Milano, Italy.

PMID: 38376074
PMCID: PMC11235749
DOI: 10.1111/ene.16248

Assessment of diagnostic criteria for multifocal motor neuropathy in patients included in the Italian database

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

. 2024 May;31(5):e16248.

doi: 10.1111/ene.16248. Epub 2024 Feb 20.

Authors

Affiliations

¹ Neuromuscular and Neuroimmunology Unit, IRCCS Humanitas Research Hospital, Rozzano, Italy.
² Department of Biomedical Sciences, Humanitas University, Pieve Emanuele, Italy.
³ Department of Clinical and Experimental Medicine, Unit of Neurology, University of Messina, Messina, Italy.
⁴ Department of Clinical and Biological Sciences, University of Torino, Torino, Italy.
⁵ Division of Neuroscience, Department of Neurology, Institute of Experimental Neurology (INSPE), San Raffaele Scientific Institute, Milano, Italy.
⁶ Fondazione Policlinico Universitario Agostino Gemelli IRCCS, UOC Neurologia, Roma, Italy.
⁷ Università Cattolica del Sacro Cuore, Sede di Roma, Roma, Italy.
⁸ Neurology Unit, Department of Neuroscience, University of Padova, Padova, Italy.
⁹ Department of Clinical and Experimental Sciences, University of Brescia; NeMO-Brescia Clinical center for Neuromuscular Diseases, Brescia, Italy.
¹⁰ Neurology Unit, Department of Clinical and Experimental Medicine, University of Pisa, Pisa, Italy.
¹¹ IRCCS Ospedale Policlinico San Martino, Genova, Italy.
¹² Unit of Neurophysiopathology, Department of Neurology Mental Health and Sensory Organs (NESMOS), Faculty of Medicine and Psychology, Sant'Andrea Hospital, Sapienza' University of Rome, Roma, Italy.
¹³ Department of Neurological and Psychiatric Sciences, Azienda Ospedaliero-Universitaria di Careggi, Florence, Italy.
¹⁴ Dysimmune Neuropathies Unit, Department of Systems Medicine, Tor Vergata University of Rome, Roma, Italy.
¹⁵ Department of Human Neurosciences, Sapienza University of Rome, Rome, Italy.
¹⁶ IRCCS Neuromed, Pozzilli, Italy.
¹⁷ Department of Neuroscience, Reproductive Sciences and Odontostomatology, University of Naples 'Federico II', Napoli, Italy.
¹⁸ Department of Brain and Behavioral Sciences, IRCCS Mondino Foundation, University of Pavia, Pavia, Italy.
¹⁹ Azienda Ospedaliera Universitaria Policlinico Paolo Giaccone, Palermo, Italy.
²⁰ ASST Bergamo Ovest-Ospedale Treviglio, Treviglio, Italy.
²¹ Presidio Sanitario Major, Istituti Clinici Scientifici Maugeri, Torino, Italy.
²² Department of Medical Biotechnology and Translational Medicine, Milan University, Milano, Italy.

PMID: 38376074
PMCID: PMC11235749
DOI: 10.1111/ene.16248

Abstract

Background and purpose: This study aimed to assess the diagnostic criteria, ancillary investigations and treatment response using real-life data in multifocal motor neuropathy (MMN) patients.

Methods: Clinical and laboratory data were collected from 110 patients enrolled in the Italian MMN database through a structured questionnaire. Twenty-six patients were excluded due to the unavailability of nerve conduction studies or the presence of clinical signs and symptoms and electrodiagnostic abnormalities inconsistent with the MMN diagnosis. Analyses were conducted on 73 patients with a confirmed MMN diagnosis and 11 patients who did not meet the diagnostic criteria.

Results: The European Federation of Neurological Societies/Peripheral Nerve Society (EFNS/PNS) diagnostic criteria were variably applied.

Author: When applying the American Association of Electrodiagnostic Medicine criteria, an additional 17% of patients fulfilled the criteria for probable/definite diagnosis whilst a further 9.5% missed the diagnosis. In 17% of the patients only compound muscle action potential amplitude, but not area, was measured and subsequently recorded in the database by the treating physician. Additional investigations, including anti-GM1 immunoglobulin M antibodies, cerebrospinal fluid analysis, nerve ultrasound and magnetic resonance imaging, supported the diagnosis in 46%-83% of the patients. Anti-GM1 immunoglobulin M antibodies and nerve ultrasound demonstrated the highest sensitivity. Additional tests were frequently performed outside the EFNS/PNS guideline recommendations.

Conclusions: This study provides insights into the real-world diagnostic and management strategies for MMN, highlighting the challenges in applying diagnostic criteria.

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

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Conflict of interest statement

Pietro Emiliano Doneddu has received travel grants to attend scientific meetings from CSL Behring and Kedrion. Fiore Manganelli reports personal fees for scientific events from CSL Behring and has received travel grants to attend scientific meetings from CSL Behring and Kedrion. Dario Cocito has received honoraria for lecturing from Shire, CSL Behring and Kedrion and travel grants to attend scientific meetings from Shire, Kedrion and CSL Behring. Raffaella Fazio has served on scientific advisory boards for CSL Behring and has received travel grants from Kedrion and CSL Behring to attend scientific meetings. Marco Luigetti received financial grants from Ackea, Alnylam, Sobi and Pfizer, and travel grants from Ackea, Alnylam, Sobi, Pfizer, Kedrion and Grifols. Chiara Briani has served on scientific advisory boards for Pfizer, Alnylam and Akcea, and has received travel grants from Kedrion and CSL Behring to attend scientific meetings. Giovanni Antonini served on advisory boards and received travel grants from Alnylam, Alexion, UCB, Kedrion, and honoraria for lecturing from Kedrion. Anna Mazzeo has received travel grants from Kedrion and CSL Behring to attend scientific meetings. Massimiliano Filosto 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. Giuseppe Cosentino has received travel grants to attend scientific meetings from CSL Behring and Kedrion. Girolama Alessandra Marfia has received travel grants to attend scientific meetings from CSL Behring and Kedrion. Maurizio Inghilleri has received travel grants to attend scientific meetings from CSL Behring and Alexion. Erdita Peci has received travel grants to attend scientific meetings from CSL Behring. Eduardo Nobile‐Orazio reports personal fees for advisory or scientific board from ArgenX (Belgium), Takeda (Italy and USA), CSL Behring (Italy and USA), Janssen (USA), Kedrion (Italy), LFB (France), Roche (Switzerland), Sanofi (USA). The other authors declare no conflict of interest.

Figures

**FIGURE 1**
Flowchart of the Italian MMN database. AAEM, American Association of Electrodiagnostic Medicine diagnostic criteria for MMN; EFNS/PNS, European Federation of Neurological Societies/Peripheral Nerve Society diagnostic criteria for MMN; MMN, multifocal motor neuropathy; NCS, nerve conduction studies.

**FIGURE 2**
Alluvial diagram illustrating the change in diagnosis using the EFNS/PNS and the AAEM diagnostic criteria for MMN in real life. AAEM, American Association of Electrodiagnostic Medicine diagnostic criteria for MMN; EFNS/PNS, European Federation of Neurological Societies/Peripheral Nerve Society diagnostic criteria for MMN; MMN, multifocal motor neuropathy.

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Assessment of diagnostic criteria for multifocal motor neuropathy in patients included in the Italian database

Affiliations

Assessment of diagnostic criteria for multifocal motor neuropathy in patients included in the Italian database

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