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Peer-reviewedCC BY-NC-NDEuropean journal of neurology

Changes in Demographic and Clinical Characteristics of MuSK Myasthenia Gravis.

  1. 1Department of Neuroscience, Università Cattolica del Sacro Cuore, Rome, Italy.
  2. 2Neurology Unit, Fondazione Policlinico Universitario Agostino Gemelli IRCCS, Rome, Italy.

Abstract

Background

Over the last two decades, an increasing number of late- and very-late onset myasthenia gravis cases (VLOMG) with acetylcholine receptor antibodies (AChR-MG) have been reported, whereas demographic studies on muscle-specific tyrosine kinase antibodies MG (MuSK-MG) are lacking. We investigated whether the proportion of patients with MuSK-MG with onset ≥ 50 years has increased and whether these patients show distinct clinical characteristics.Methods

We retrospectively enrolled consecutive patients with MuSK-MG onset ≥ 16 years followed between 1980 and 2025 at our tertiary center. Demographic characteristics and maximum disease severity, classified according to Myasthenia Gravis Foundation of America classification, were analyzed. Patients were stratified into three age-at-onset subgroups.Results

Overall, 120 patients were included. A significant increase in the median age at MuSK-MG onset was observed, from 36 years (IQR, 23-45.75) in the 1980s to 47 years (IQR, 29-69) in the 2013-2025 period (p = 0.033). Patients with onset ≥ 50 years increased from 2/17 (11.8%) to 19/39 (48.7%) (p = 0.015), including 12 VLOMG cases. Males increased from 23.5% to 35.9%. Maximum MG severity decreased over time, with a remarkable reduction of respiratory crises (p = 0.004), with no significant differences across age-at-onset groups.Conclusions

Demographic changes observed in AChR-MG also occur, though less prominently, in MuSK-MG.

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BibTeX
@article{falso2026changes,
  title = {{Changes in Demographic and Clinical Characteristics of MuSK Myasthenia Gravis.}},
  author = {Silvia Falso and Sofia Marini and Martina Marini and Federico Habetswallner and Amelia Evoli and Raffaele Iorio},
  journal = {European journal of neurology},
  year = {2026},
  month = oct,
  doi = {10.1111/ene.70785},
  url = {https://europepmc.org/article/MED/42963134},
  note = {CC BY-NC-ND},
}
RIS
TY  - JOUR
TI  - Changes in Demographic and Clinical Characteristics of MuSK Myasthenia Gravis.
AU  - Silvia Falso
AU  - Sofia Marini
AU  - Martina Marini
AU  - Federico Habetswallner
AU  - Amelia Evoli
AU  - Raffaele Iorio
PY  - 2026
DA  - 2026/10/30
JO  - European journal of neurology
DO  - 10.1111/ene.70785
UR  - https://europepmc.org/article/MED/42963134
AB  - Background  Over the last two decades, an increasing number of late- and very-late onset myasthenia gravis cases (VLOMG) with acetylcholine receptor antibodies (AChR-MG) have been reported, whereas demographic studies on muscle-specific tyrosine kinase antibodies MG (MuSK-MG) are lacking. We investigated whether the proportion of patients with MuSK-MG with onset ≥ 50 years has increased and whether these patients show distinct clinical characteristics.Methods  We retrospectively enrolled consecutive patients with MuSK-MG onset ≥ 16 years followed between 1980 and 2025 at our tertiary center. Demographic characteristics and maximum disease severity, classified according to Myasthenia Gravis Foundation of America classification, were analyzed. Patients were stratified into three age-at-onset subgroups.Results  Overall, 120 patients were included. A significant increase in the median age at MuSK-MG onset was observed, from 36 years (IQR, 23-45.75) in the 1980s to 47 years (IQR, 29-69) in the 2013-2025 period (p = 0.033). Patients with onset ≥ 50 years increased from 2/17 (11.8%) to 19/39 (48.7%) (p = 0.015), including 12 VLOMG cases. Males increased from 23.5% to 35.9%. Maximum MG severity decreased over time, with a remarkable reduction of respiratory crises (p = 0.004), with no significant differences across age-at-onset groups.Conclusions  Demographic changes observed in AChR-MG also occur, though less prominently, in MuSK-MG.
SN  - 1351-5101
SN  - 1468-1331
ER  -