TY - JOUR
T1 - Clinical and therapeutic features of myasthenia gravis in adults based on age at onset
AU - Cortés-Vicente, Elena
AU - Álvarez-Velasco, Rodrigo
AU - Segovia, Sonia
AU - Paradas, Carmen
AU - Casasnovas, Carlos
AU - Guerrero-Sola, Antonio
AU - Pardo, Julio
AU - Ramos-Fransi, Alba
AU - Sevilla, Teresa
AU - López De Munain, Adolfo
AU - Gómez, Maria Teresa
AU - Jericó, Ivonne
AU - Gutiérrez-Gutiérrez, Gerardo
AU - Pelayo-Negro, Ana Lara
AU - Martín, María Asunción
AU - Mendoza, María Dolores
AU - Morís, Germán
AU - Rojas-Garcia, Ricard
AU - Díaz-Manera, Jordi
AU - Querol, Luis
AU - Gallardo, Eduard
AU - Vélez, Beatriz
AU - Albertí, María Antonia
AU - Galán, Lucía
AU - García-Sobrino, Tania
AU - Martínez-Piñeiro, Alicia
AU - Lozano-Veintimilla, Ana
AU - Fernández-Torrón, Roberto
AU - Cano-Abascal, Ángel
AU - Illa, Isabel
N1 - Funding Information:
This study was funded by the Instituto de Salud Carlos III through the project 16/01440 (cofunded by the European Union ERDF [PI Isabel Illa] and AMES [the Spanish Association of Myasthenia Gravis patients]). Elena Cortés-Vicente was supported by a Río Hortega grant (CM16/00096) from the Fondo de Investigación en Salud, Instituto de Salud Carlos III, Ministry of Health (Spain), the European Social Fund, and CESCE Spain. The NMD-ES Project and Sonia Segovia (data curator) are partially funded by the Centro de Investigación Biomédica en Red de Enfermedades Raras (CIBERER).
Publisher Copyright:
© American Academy of Neurology.
PY - 2020/3/17
Y1 - 2020/3/17
N2 - ObjectiveTo describe the characteristics of patients with very-late-onset myasthenia gravis (MG).MethodsThis 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.ResultsA 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).ConclusionsPatients 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.
AB - ObjectiveTo describe the characteristics of patients with very-late-onset myasthenia gravis (MG).MethodsThis 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.ResultsA 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).ConclusionsPatients 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.
UR - http://www.scopus.com/inward/record.url?scp=85081927769&partnerID=8YFLogxK
U2 - 10.1212/WNL.0000000000008903
DO - 10.1212/WNL.0000000000008903
M3 - Article
C2 - 32071167
AN - SCOPUS:85081927769
SN - 0028-3878
VL - 94
SP - e1171-e1180
JO - Neurology
JF - Neurology
IS - 11
ER -