title: Progression of dysautonomia in multiple system atrophy: a prospective study of self-perceived impairment.
abstract: To assess severity and progression of self-perceived dysautonomia and their impact on health-related quality of life (Hr-QoL) in multiple system atrophy (MSA), twenty-seven patients were recruited by the European MSA Study Group (EMSA-SG). At baseline, all patients completed the Composite Autonomic Symptom Scale (COMPASS) and the 36 item Short Form Health Survey (SF-36), and they were assessed using the 3-point global disease severity scale (SS-3) and the Unified MSA Rating Scale (UMSARS). After 6 months follow-up, the self completed COMPASS Change Scale (CCS), the SF-36, SS-3, and UMSARS were obtained. MSA patients showed marked self-perceived dysautonomia at baseline visit and pronounced worsening of dysautonomia severity on the CCS at follow-up. Severity and progression of dysautonomia did not correlate with age, disease duration, motor impairment and overall disease severity at baseline. There were no significant differences between genders and motor subtypes. Baseline COMPASS scores were, however, inversely correlated with SF-36 scores. Progression of self-perceived dysautonomia did not correlate with global disease progression. Hr-QoL scores were stable during follow-up. This is the first study to investigate self-perceived dysautonomia severity in MSA and its evolution over time. Our data suggest that dysautonomia should be recognized as a key target for therapeutic intervention in MSA.
authors.affiliation: Clinical Department of Neurology, Innsbruck Medical University, Innsbruck, Austria.
authors.forename: M
authors.initials: M
authors.lastname: Köllensperger
authors.forename: M
authors.initials: M
authors.lastname: Stampfer-Kountchev
authors.forename: K
authors.initials: K
authors.lastname: Seppi
authors.forename: F
authors.initials: F
authors.lastname: Geser
authors.forename: C
authors.initials: C
authors.lastname: Frick
authors.forename: F
authors.initials: F
authors.lastname: Del Sorbo
authors.forename: A
authors.initials: A
authors.lastname: Albanese
authors.forename: T
authors.initials: T
authors.lastname: Gurevich
authors.forename: N
authors.initials: N
authors.lastname: Giladi
authors.forename: R
authors.initials: R
authors.lastname: Djaldetti
authors.forename: A
authors.initials: A
authors.lastname: Schrag
authors.forename: P A
authors.initials: PA
authors.lastname: Low
authors.forename: C J
authors.initials: CJ
authors.lastname: Mathias
authors.forename: W
authors.initials: W
authors.lastname: Poewe
authors.forename: G K
authors.initials: GK
authors.lastname: Wenning
mesh_terms: D000368:Aged; D000369:Aged, 80 and over; D001342:Autonomic Nervous System Diseases / Q000453:epidemiology / Q000503:physiopathology*; D018450:Disease Progression; D005260:Female; D005500:Follow-Up Studies; D006306:Health Surveys; D006801:Humans; D008297:Male; D008875:Middle Aged; D019578:Multiple System Atrophy / Q000453:epidemiology / Q000503:physiopathology*; D011446:Prospective Studies; D012649:Self Concept