title: Multiple system atrophy with prolonged survival: is late onset of dysautonomia the clue?
abstract: Multiple system atrophy (MSA) is a neurodegenerative disease characterised by cardiovascular autonomic failure and/or urinary dysfunctions, associated with parkinsonism, cerebellar and/or corticospinal signs, usually leading to death after an average of 7 years. We describe the disease course of five patients diagnosed with probable MSA (4 with predominant parkinsonism and 1 with predominant cerebellar ataxia) who survived for more than 15 years and were followed throughout the disease course at our department. Cardiovascular autonomic dysfunction of any severity occurred late (mean latency from disease onset 9.4 ± 5 years) in this subgroup of MSA patients. The time of involvement of the urogenital system was more variable (from 0 to 14 years after disease onset) and manifested with symptoms of storage disorders (urinary urgency, frequency and incontinence) and erectile dysfunction in men. Conversely complains suggestive of urinary voiding dysfunction (incomplete bladder emptying and urinary retention) were not recorded and patients required catheterization only late in the disease course. In conclusion, our study showed that late onset of both cardiovascular autonomic failure and urinary voiding disorders may be positive prognostic factors in MSA irrespective of the MSA subtype.
authors.affiliation: IRCCS, Istituto delle Scienze Neurologiche e Dipartimento di Scienze Biomediche e Neuromotorie (DIBINEM), Università di Bologna, c/o Ospedale Bellaria, Via Altura n 3, 40139, Bologna, Italy.
authors.forename: Giovanna
authors.initials: G
authors.lastname: Calandra-Buonaura
authors.forename: Pietro
authors.initials: P
authors.lastname: Guaraldi
authors.forename: Luisa
authors.initials: L
authors.lastname: Sambati
authors.forename: Giovanna
authors.initials: G
authors.lastname: Lopane
authors.forename: Annagrazia
authors.initials: A
authors.lastname: Cecere
authors.forename: Giorgio
authors.initials: G
authors.lastname: Barletta
authors.forename: Federica
authors.initials: F
authors.lastname: Provini
authors.forename: Manuela
authors.initials: M
authors.lastname: Contin
authors.forename: Paolo
authors.initials: P
authors.lastname: Martinelli
authors.forename: Pietro
authors.initials: P
authors.lastname: Cortelli
mesh_terms: D017668:Age of Onset; D000368:Aged; D018450:Disease Progression; D005260:Female; D006801:Humans; D008137:Longitudinal Studies; D008297:Male; D019578:Multiple System Atrophy / Q000150:complications* / Q000175:diagnosis / Q000401:mortality*; D054969:Primary Dysautonomias / Q000150:complications*; D012189:Retrospective Studies