title: Major motor-functional determinants associated with poor self-reported health-related quality of life in myasthenia gravis patients.
abstract: Myasthenia gravis (MG) is an autoimmune neuromuscular disorder in which disabling muscle weakness may affect health-related quality of life (HRQoL). The aim of this study was to investigate which common motor-functional deficits and corresponding severity are most determinant of poor HRQoL in these patients. In 41 patients, the dichotomized first item of the Italian Myasthenia Gravis Questionnaire (IMGQ), categorizing patients who report "good" and "poor" HRQoL, was chosen as dependent-outcome variable. All items composing the myasthenia gravis-specific scale (MG-ADL), i.e. talking, chewing, swallowing, breathing, impairment of ability to brush teeth or comb hair, impairment of ability to rise from chair, double vision, and eyelid droop were acquired as independent variables and dichotomized. Stepwise backward LR multivariable logistic regression analysis was performed. In addition, the main characteristics of patients were compared. MG-ADL items "chewing" ≥1, i.e. "fatigue chewing solid food", and "breathing" ≥2, i.e. "shortness of breath at rest" proved to be significant determinants. Higher dose of corticosteroid therapy was significantly (p = 0.027; r s  = -0.35), correlated with poor HRQoL. At diagnosis, a decremental response to repetitive nerve stimulation (RNS) from the abductor pollicis brevis was significantly more frequent in patients with poor HRQoL. In conclusion, impaired "chewing" and "breathing" functions indicate the need for careful planning of rehabilitation, re-education and patient management. Moreover, decremental response to RNS at diagnosis may identify patients at risk for poor HRQoL.
authors.affiliation: U.O.P. Professioni della Riabilitazione, Azienda Ospedaliera Universitaria Senese, Siena, Italy. cioncoloni9@unisi.it.
authors.forename: David
authors.initials: D
authors.lastname: Cioncoloni
authors.affiliation: Dipartimento di Scienze Mediche, Chirurgiche e Neuroscienze, Azienda Ospedaliera Universitaria Senese, Università di Siena, Siena, Italy.
authors.forename: Stefania
authors.initials: S
authors.lastname: Casali
authors.affiliation: Dipartimento di Scienze Mediche, Chirurgiche e Neuroscienze, Azienda Ospedaliera Universitaria Senese, Università di Siena, Siena, Italy.
authors.forename: Federica
authors.initials: F
authors.lastname: Ginanneschi
authors.affiliation: Dipartimento di Scienze Mediche, Chirurgiche e Neuroscienze, Azienda Ospedaliera Universitaria Senese, Università di Siena, Siena, Italy.
authors.forename: Marisa
authors.initials: M
authors.lastname: Carone
authors.affiliation: Dipartimento di Scienze Mediche, Chirurgiche e Neuroscienze, Azienda Ospedaliera Universitaria Senese, Università di Siena, Siena, Italy.
authors.forename: Boni
authors.initials: B
authors.lastname: Veronica
authors.affiliation: Dipartimento di Scienze Mediche, Chirurgiche e Neuroscienze, Azienda Ospedaliera Universitaria Senese, Università di Siena, Siena, Italy.
authors.forename: Alessandro
authors.initials: A
authors.lastname: Rossi
authors.affiliation: Dipartimento di Scienze Mediche, Chirurgiche e Neuroscienze, Azienda Ospedaliera Universitaria Senese, Università di Siena, Siena, Italy.
authors.forename: Fabio
authors.initials: F
authors.lastname: Giannini
mesh_terms: D000203:Activities of Daily Living; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D015331:Cohort Studies; D005260:Female; D015438:Health Behavior; D006801:Humans; D008297:Male; D008875:Middle Aged; D009069:Movement Disorders / Q000209:etiology*; D009157:Myasthenia Gravis / Q000150:complications* / Q000523:psychology*; D009460:Neurologic Examination; D011788:Quality of Life / Q000523:psychology*; D012372:ROC Curve; D057566:Self Report
keywords: Activities of daily living; Myasthenia gravis; Outcome and process assessment; Prognosis; Quality of life; Rehabilitation