title: Amyotrophic lateral sclerosis and myasthenia gravis: association or chance occurrence?
abstract: Very few cases of patients with myasthenia gravis (MG) who later developed amyotrophic lateral sclerosis (ALS) have been described, although some studies showed that significantly more cases than expected have ALS associated with a prior diagnosis of autoimmune diseases. Our aim was to investigate whether the association of ALS and MG was higher than expected in a population-based study and to describe the clinical features characterizing these patients. In Emilia Romagna Region of Italy, a prospective registry has been collecting all incident ALS cases since 1.1.2009. For each patient, detailed clinical information is collected by caring physicians, including comorbidities. From 1.1.2009 to 31.12.2014, 671 patients were diagnosed with ALS; five patients (0.75%) were also affected by MG. Considering Western Countries incidence rates the occurrence of both the diseases should be a really exceptional event (7.5/109), compared to our findings (1.87/107) (p < 0.01). Patients with ALS and MG had more frequently a bulbar onset and a fast progressive course. These cases of ALS after MG raise the possibility of potential shared immunological dysfunctions, which may be expression of common pathogenic mechanisms, as well as of shared disease-course modulating events.
authors.affiliation: DIBINEM, Department of Biomedical and Neuromotor Sciences, Alma Mater Studiorum, University of Bologna, Via Ugo Foscolo 7, 40123, Bologna, Italy.
authors.forename: Silvia
authors.initials: S
authors.lastname: de Pasqua
authors.affiliation: Department of Neuroscience, St. Agostino Estense Hospital, University of Modena and Reggio Emilia, Via Giardini 1355, 41126, Modena, Italy.
authors.forename: Francesco
authors.initials: F
authors.lastname: Cavallieri
authors.affiliation: Department of Neuroscience, St. Orsola-Malpighi University Hospital, Via Pietro Albertoni 15, 40138, Bologna, Italy.
authors.forename: Roberto
authors.initials: R
authors.lastname: D'Angelo
authors.affiliation: IRCCS-Institute of Neurological Sciences of Bologna, Bellaria Hospital, Via Altura 3, 40139, Bologna, Italy.
authors.forename: Fabrizio
authors.initials: F
authors.lastname: Salvi
authors.affiliation: Department of Neuroscience, St. Agostino Estense Hospital, University of Modena and Reggio Emilia, Via Giardini 1355, 41126, Modena, Italy.
authors.forename: Nicola
authors.initials: N
authors.lastname: Fini
authors.affiliation: IRCCS-Institute of Neurological Sciences of Bologna, Bellaria Hospital, Via Altura 3, 40139, Bologna, Italy.
authors.forename: Roberto
authors.initials: R
authors.lastname: D'Alessandro
authors.affiliation: Department of Neuroscience, St. Orsola-Malpighi University Hospital, Via Pietro Albertoni 15, 40138, Bologna, Italy.
authors.forename: Rita
authors.initials: R
authors.lastname: Rinaldi
authors.affiliation: Department of Neuroscience, St. Agostino Estense Hospital, University of Modena and Reggio Emilia, Via Giardini 1355, 41126, Modena, Italy.
authors.forename: Antonio
authors.initials: A
authors.lastname: Fasano
authors.affiliation: Department of Neuroscience, St. Agostino Estense Hospital, University of Modena and Reggio Emilia, Via Giardini 1355, 41126, Modena, Italy. j.mandrioli@ausl.mo.it.
authors.forename: Jessica
authors.initials: J
authors.lastname: Mandrioli
mesh_terms: D000368:Aged; D000369:Aged, 80 and over; D000690:Amyotrophic Lateral Sclerosis / Q000453:epidemiology*; D015897:Comorbidity; D005260:Female; D006801:Humans; D015994:Incidence; D007558:Italy / Q000453:epidemiology; D008297:Male; D008875:Middle Aged; D009157:Myasthenia Gravis / Q000453:epidemiology*; D012042:Registries
keywords: Amyotrophic lateral sclerosis; Immune-mediated disorders; Immunological dysfunction; Myasthenia gravis; Regulatory T lymphocytes