Gabriele Monte1, Gregorio Spagni2, Valentina Damato2,3, Raffaele Iorio3, Mariapaola Marino4, Amelia Evoli2,3. 1. Istituto di Neurologia, Università Cattolica del Sacro Cuore, Largo Agostino Gemelli, 8, 00168, Rome, Italy. gabriele.monte91@gmail.com. 2. Istituto di Neurologia, Università Cattolica del Sacro Cuore, Largo Agostino Gemelli, 8, 00168, Rome, Italy. 3. Istituto di Neurologia, Fondazione Policlinico Universitario "A. Gemelli" IRCCS, Largo Agostino Gemelli, 8, 00168, Rome, Italy. 4. Dipartimento di Medicina e Chirurgia Traslazionale, Fondazione Policlinico Universitario "A. Gemelli" IRCCS, Università Cattolica del Sacro Cuore, Rome, Italy.
Abstract
BACKGROUND: Anti-acetylcholine receptor antibodies (AChR Abs) are detected in 85% of myasthenia gravis (MG) patients, at higher rates in patients with late-onset disease. AChR Ab frequency is generally thought to be much lower in ocular MG (OMG), although recent studies reported positivity rates higher than 70%. We hypothesized that the improved AChR Ab diagnostic yield in OMG could be related to an increased frequency of late-onset disease, as observed in generalized MG. METHODS: We compared OMG patients, with disease onset before or after 1998, for the age of onset, sex, presence of thymoma, immunosuppressive therapy rate, AChR Ab positivity, and follow-up duration. All patients had a follow-up ≥ 2 years. AChR Abs were tested by radioimmunoassay. RESULTS: The study included 133 patients. Disease onset occurred before 1998 in 54/133 cases (41%). Age of onset, the proportion of late-onset patients, and AChR Ab positivity rate were significantly increased in the more recent population. Thymoma frequency was similar in the two series. On multivariate analysis, the only variable predicting AChR Ab positivity was the age at onset ≥ 50 years (OR = 6.50, 95% CI = 2.70-15.63, p < 0.0001). CONCLUSIONS: Our results confirm that current AChR Ab positivity in OMG may be higher than generally thought. In our population, this finding was associated with an increased frequency of late-onset cases.
BACKGROUND: Anti-acetylcholine receptor antibodies (AChR Abs) are detected in 85% of myasthenia gravis (MG) patients, at higher rates in patients with late-onset disease. AChR Ab frequency is generally thought to be much lower in ocular MG (OMG), although recent studies reported positivity rates higher than 70%. We hypothesized that the improved AChR Ab diagnostic yield in OMG could be related to an increased frequency of late-onset disease, as observed in generalized MG. METHODS: We compared OMG patients, with disease onset before or after 1998, for the age of onset, sex, presence of thymoma, immunosuppressive therapy rate, AChR Ab positivity, and follow-up duration. All patients had a follow-up ≥ 2 years. AChR Abs were tested by radioimmunoassay. RESULTS: The study included 133 patients. Disease onset occurred before 1998 in 54/133 cases (41%). Age of onset, the proportion of late-onset patients, and AChR Ab positivity rate were significantly increased in the more recent population. Thymoma frequency was similar in the two series. On multivariate analysis, the only variable predicting AChR Ab positivity was the age at onset ≥ 50 years (OR = 6.50, 95% CI = 2.70-15.63, p < 0.0001). CONCLUSIONS: Our results confirm that current AChR Ab positivity in OMG may be higher than generally thought. In our population, this finding was associated with an increased frequency of late-onset cases.
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 Date: 2020-02-18 Impact factor: 9.910
Authors: Crandall E Peeler; Lindsey B De Lott; Lina Nagia; Joao Lemos; Eric R Eggenberger; Wayne T Cornblath Journal: JAMA Neurol Date: 2015-10 Impact factor: 18.302