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Italian recommendations for the diagnosis and treatment of myasthenia gravis

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Abstract

Myasthenia gravis is a well-treatable disease, in which a prompt diagnosis and an adequate management can achieve satisfactory control of symptoms in the great majority of patients. Improved knowledge of the disease pathogenesis has led to recognition of patient subgroups, according to associated antibodies, age at onset and thymus pathology, and to a more personalized treatment. When myasthenia gravis is suspected on clinical grounds, diagnostic confirmation relies mainly on the detection of specific antibodies. Neurophysiological studies and, to a lesser extent, clinical response to cholinesterase inhibitors support the diagnosis in seronegative patients. In these cases, the differentiation from congenital myasthenia can be challenging. Treatment planning must consider weakness extension and severity, disease subtype, thymus pathology, together with patient characteristics and comorbidities. Since most subjects with myasthenia gravis require long-term immunosuppressive therapy, surveillance of expected and potential adverse events is critical. For patients refractory to conventional immunosuppression, the use of biologic agents is highly promising. These recommendations are addressed to non-experts on neuromuscular transmission disorders. The diagnostic procedures and therapeutic approaches hereafter described are largely accessible in Italy.

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Authors and Affiliations

  1. Institute of Neurology, Fondazione Policlinico “A. Gemelli” IRCCS, Università Cattolica del S. Cuore, Largo A. Gemelli 8, 00168, Rome, Italy

    Amelia Evoli

  2. Università Cattolica del Sacro Cuore, Rome, Italy

    Amelia Evoli

  3. Department of Neurosciences, Mental Health and Sensory Organs NESMOS, Università di Roma “La Sapienza”, Rome, Italy

    Giovanni Antonini

  4. Department of Neuroimmunology and Neuromuscular Diseases, Fondazione Istituto Neurologico C. Besta, Milan, Italy

    Carlo Antozzi & Renato Mantegazza

  5. Centro Regionale Malattie Neuromuscolari, Ospedale Clinicizzato “SS Annunziata”, Chieti, Italy

    Antonio DiMuzio

  6. UOC Neurofisiopatologia AORN Cardarelli, Naples, Italy

    Francesco Habetswallner

  7. Neurology Unit, Sant’Eugenio Hospital, Rome, Italy

    Cesare Iani

  8. Centro Malattie Rare Neuromuscolari, Dipartimento di Neuroscienze Umane, Università di Roma “La Sapienza”, Rome, Italy

    Maurizio Inghilleri

  9. Dipartimento di Scienze Biomediche e Neuromotorie, Università di Bologna, Bologna, Italy

    Rocco Liguori

  10. IRCCS Istituto delle Scienze Neurologiche di Bologna, Bologna, Italy

    Rocco Liguori

  11. Unit Malattie Neuromuscolari, Dipartimento di Medicina dei Sistemi, Università di Roma “Tor Vergata”, Rome, Italy

    Roberto Massa

  12. Department of Neurosciences, University of Padova, Padova, Italy

    Elena Pegoraro

  13. Department of Clinical and Experimental Medicine, Neurology Unit, University of Pisa, Pisa, Italy

    Roberta Ricciardi

  14. Unit of Neurology and Neuromuscular Diseases, University of Messina, Messina, Italy

    Carmelo Rodolico

Authors
  1. Amelia Evoli
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  2. Giovanni Antonini
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  3. Carlo Antozzi
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  6. Cesare Iani
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  7. Maurizio Inghilleri
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  8. Rocco Liguori
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  9. Renato Mantegazza
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  10. Roberto Massa
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  11. Elena Pegoraro
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  12. Roberta Ricciardi
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Corresponding author

Correspondence to Amelia Evoli.

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Conflict of interest

Amelia Evoli served on an advisory board for Alexion and as jury member for research grant award for Grifols.

Giovanni Antonini received conference honoraries from Kedrion, Sanofi-Genzyme and travel grants from Kedrion, Sanofi-Genzyme, Pfizer, Almylan.

Carlo Antozzi has no conflicts of interest to disclose.

Antonio Di Muzio has no conflicts of interest to disclose.

Francesco Habetswallner has no conflicts of interest to disclose.

Cesare Iani has no conflicts of interest to disclose.

Maurizio Inghilleri has no conflicts of interest to disclose.

Rocco Liguori served on advisory boards for Biogen, Sanofi-Genzyme, Argon Healthcare s.r.l., Editree Eventi s.r.l., received Lecture fees from Dynamicom Education, SIMG Service, Adnkronos Salute unipersonale s.r.l., DOC Congress s.r.l., First Class s.r.l., and is a consultant for Alfasigma and Amicus Therapeutics s.r.l.

Renato Mantegazza has received funding for travel or meeting attendance from: Sanofi-Aventis, Teva, Biomarin, Alexion, Argenx, Novartis, Catalyst, Merck, and for advisory boards or SSC from: Biomarin, Alexion, Argenx.

Roberto Massa received Travel grants from Sanofi-Genzyme, CSL-Behring, Pfizer, Almylan.

Elena Pegoraro reports personal fees from PTC Therapeutics, Santhera, Roche and Sanofi-Genzyme.

Roberta Ricciardi has no conflicts of interest to disclose.

Carmelo Rodolico has no conflicts of interest to disclose.

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This document takes into consideration recent advances in the field and international guidelines. It represents an update of diagnostic procedures and therapeutic approaches used in Italian neurological departments with experience in neuromuscular transmission (NMT) disorders.

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Evoli, A., Antonini, G., Antozzi, C. et al. Italian recommendations for the diagnosis and treatment of myasthenia gravis. Neurol Sci 40, 1111–1124 (2019). https://doi.org/10.1007/s10072-019-03746-1

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