2026 American Academy of Neurology Abstract Website

Roser Velasco Fargas¹, Jose Maria Cabrera-Maqueda², Elianet Fonseca², Carla Marco³, Juan Luis Chico-Garcia⁴, Arnau Llaurado⁵, Francisco Javier Gomez⁶, Cristina Izquierdo⁷, Francisco Javier Cabello⁸, Laura del Pino⁹, Elena Cortes-Vicente¹⁰, Alejandro Horga¹¹, Jaime Gallego¹², Carlota Jauregui¹³, Rocio Caldu¹⁴, Ana Lara Pelayo¹⁵, Marta Ros¹⁶, Hugo Hernandez Tost¹⁷, Jorge Alvarez-Troncoso¹⁸, Monica alaña¹⁹, Laura Torne²⁰, Gerardo Gutierrez-Gutierrez²¹, Cristina Gonzalez-Mingot²², Marta Garcia-Yepes²³, Alba Chavarria²⁴, Javier Riancho²⁵, Cristina Sanchez-Vizcaino²⁶, Patricia Rodrigo²⁷, Sarah Besora²⁸, Pablo Cabezudo-García²⁹, German Moris³⁰, Monica Guijarro³¹, Adrian Macias³², Sergio Muñiz³³, Alejandra Carvajal³⁴, Manuel Baron³⁵, Jessica Pouso³⁶, Almudena Boix³⁷, Josep Dalmau³⁸, Jordi Bruna¹, Eugenia Martinez-Hernandez²
¹Neurooncology, Catalan Institute of Oncology, ²Neurology, Hospital Clinic de Barcelona, ³Neurology, Hospital Universitario de Bellvitge, ⁴Neurology, Hospital Ramon y Cajal, ⁵Neurology, Hospital Vall d'Hebron, ⁶Neurology, Hospital Virgen del Rocio, ⁷Neurology, Hospital Germans Tries i Pujol, ⁸Neurology, Hospital La Fe, ⁹Neurology, Hospital Gregorio Marañon, ¹⁰Neurology, Hospital de la Santa Creu i Sant Pau, ¹¹Neurology, Hospital Clinico de Madrid, ¹²Neurology, Clinica Universitaria de Navarra, ¹³Neurology, Hospital Universitario de Donostia, ¹⁴Neurology, Hospital Lozano Blesa, ¹⁵Neurology, Hospital Marques de Valdecilla, ¹⁶Neurology, Hospital Parc Tauli, ¹⁷Neurology, Hospital Nuestra Señora de la Candelaria, ¹⁸Internal Medicine, Hospital La Paz, ¹⁹Complejo Asistencial Universitario de Salamanca, ²⁰Neurology, Hospital Universitario de Navarra, ²¹Neurology, Hospital Universitario Infanta Sofía, ²²Neurology, Hospital Arnau de Vilanova, ²³Neurology, Complejo Hospitalario Universitario Insular Materno Infantil, ²⁴Neurology, Hospital Clinico Universitario de Valladolid, ²⁵Neurology, Hospital Sierrallana, ²⁶Neurology, Hospital General Universitario Santa Lucía, ²⁷Neurology, Hospital Universitario Basurto, ²⁸Neurology, Hospital Mutua de Terrassa, ²⁹Neurology, Hospital Regional Universitario de Malaga, ³⁰Neurology, Hospital Central de Asturias, ³¹Neurology, Hospital Universitario de Lugo, ³²Neurology, Hospital del Mar, ³³Neurology, Hospital 12 de Octubre, ³⁴Neurology, Hospital Universitario Virgen de las Nieves, ³⁵Neurology, Hospital Universitario Fundacion Alcorcon, ³⁶Neurology, Complejo Hospitalario Universitario de Pontevedra, ³⁷Neurology, Hospital Josep Trueta, ³⁸Neuroimmunology, Institut d'Investigacions Biomediques August Pi i Sunyer - Caixa Research Institute

Objective:

To identify predictors of early mortality (within 30 days) in patients with myositis/myasthenia (MM) associated with immune checkpoint inhibitors (ICI).

Background:

MM shows heterogeneous clinical features and severity. Early recognition and immunosuppressive treatments may improve outcomes, but markers of rapid deterioration are lacking.

Design/Methods:

This nationwide retrospective study in Spain collected information on MM patients through March 2025 using a web-based structured questionnaire. Syndromes were centrally classified as definite, probable, or possible, and severity was assessed using Myasthenia Gravis Foundation of America (MGFA) classification.

Results:

We recruited 153 patients from 36 hospitals. Median age was 72 years [IQR 66–78] and 67% were male. By consensus definitions, 79 (52%) had definite/probable myositis, 14 (9%) definite/probable myasthenia, 57 (37%) fulfilled criteria for both disorders and 3 (2%) only possible myositis (excluded from analyses). At disease nadir 102/150 (68%) patients reached MGFA class III–V. Higher MGFA class correlated with elevated Troponin T (TnT, n=49, ρ=0.506, p=0.001) and CK (n=123, ρ=0.272, p=0.002), but not Troponin I (n=53, ρ=0.106, p=0.444). All patients discontinued ICI; 144/150 (96%) received immunosuppressants. At day +30, 108/150 (72%) had improved, 22 (15%) were unchanged and 20 (13%) had died. Patients who died early showed more frequently dyspnea (75% vs. 41%, p=0.007), palpitations (15% vs. 3%, p=0.050), dysphonia (65% vs. 45%, p=0.098), AChR-antibody positivity (65% vs. 28%, p=0.002) and concurrent myocarditis (76.5% vs. 46.5%, p=0.035), with higher TnT levels (1577 vs. 421 ng/L, p=0.013). In multivariable analysis, ventilatory support and AChR-positivity independently increased early mortality (OR=4.95; 95%CI 1.41-17.54, p=0.013 and OR=3.62; 95%CI 1.07-12.20, p=0.038 respectively).

Conclusions:

TnT and CK levels in the acute phase correlated with MGFA class, indicating potential as biomarkers of neuromuscular involvement and severity. Early mortality remains a major challenge, underscoring the need for timely management strategies.