Clinical and therapeutic features of myasthenia gravis in adults based on age at onset.

Abstract

OBJECTIVE:

To describe the characteristics of patients with very-late-onset myasthenia gravis (MG).

METHODS:

This observational cross-sectional multicenter study was based on information in the neurologist-driven Spanish Registry of Neuromuscular Diseases (NMD-ES). All patients were >18 years of age at onset of MG and onset occurred between 2000 and 2016 in all cases. Patients were classified into 3 age subgroups: early-onset MG (age at onset <50 years), late-onset MG (onset ≥50 and <65 years), and very-late-onset MG (onset ≥65 years). Demographic, immunologic, clinical, and therapeutic data were reviewed.

RESULTS:

A total of 939 patients from 15 hospitals were included: 288 (30.7%) had early-onset MG, 227 (24.2%) late-onset MG, and 424 (45.2%) very-late-onset MG. The mean follow-up was 9.1 years (SD 4.3). Patients with late onset and very late onset were more frequently men (p < 0.0001). Compared to the early-onset and late-onset groups, in the very-late-onset group, the presence of anti-acetylcholine receptor (anti-AChR) antibodies (p < 0.0001) was higher and fewer patients had thymoma (p < 0.0001). Late-onset MG and very-late-onset MG groups more frequently had ocular MG, both at onset (<0.0001) and at maximal worsening (p = 0.001). Although the very-late-onset group presented more life-threatening events (Myasthenia Gravis Foundation of America IVB and V) at onset (p = 0.002), they required fewer drugs (p < 0.0001) and were less frequently drug-refractory (p < 0.0001).

CONCLUSIONS:

Patients with MG are primarily ≥65 years of age with anti-AChR antibodies and no thymoma. Although patients with very-late-onset MG may present life-threatening events at onset, they achieve a good outcome with fewer immunosuppressants when diagnosed and treated properly.

• Authors: Elena Cortés-Vicente, Rodrigo Álvarez-Velasco, Sonia Segovia, Carmen Paradas, Carlos Casasnovas, Antonio Guerrero-Sola, Julio Pardo, Alba Ramos-Fransi, Teresa Sevilla, Adolfo López de Munain, Maria Teresa Gómez, Ivonne Jericó, Gerardo Gutiérrez-Gutiérrez, Ana Lara Pelayo-Negro, María Asunción Martín, María Dolores Mendoza, Germán Morís, Ricard Rojas-Garcia, Jordi Díaz-Manera, Luis Querol, Eduard Gallardo, Beatriz Vélez, María Antonia Albertí, Lucía Galán, Tania García-Sobrino, Alicia Martínez-Piñeiro, Ana Lozano-Veintimilla, Roberto Fernández-Torrón, Ángel Cano-Abascal, Isabel Illa
• Journal: Neurology (Neurology) Vol. 94 Issue 11 Pg. e1171-e1180 (03 17 2020) ISSN: 1526-632X [Electronic] United States
• PMID: 32071167 (Publication Type: Journal Article, Multicenter Study, Observational Study, Research Support, Non-U.S. Gov't)
• Copyright: © 2020 American Academy of Neurology.

Topics

• Adult
• Age of Onset
• Aged
• Cross-Sectional Studies
• Female
• Humans
• Male
• Middle Aged
• Myasthenia Gravis (complications, drug therapy, immunology)
• Treatment Outcome