title: Autonomic system and amyotrophic lateral sclerosis.
abstract: BACKGROUND
  The aim of this study is to characterize autonomic impairment in motor neuron disease.
  
  
  METHODS
  Neurological evaluations and autonomic testing were analyzed retrospectively in 132 patients: 86 classic amyotrophic lateral sclerosis (ALS), 36 lower motor neuron (LMN), and 10 upper motor neuron (UMN) predominant disease.
  
  
  RESULTS
  One-third of patients were symptomatic; urinary urgency and constipation were the most frequent symptoms. Increased Composite Autonomic Severity Score (CASS) was present in 75% with mild impairment (CASS 1-3) in 85% and moderate (CASS 4-7) in 15%. The frequencies of testing abnormalities were: sudomotor 46%, cardiovagal 50%, and adrenergic 14%. The UMN group had significantly higher median CASS scores than the classic ALS (P = 0.021) and LMN group (P = 0.018).
  
  
  CONCLUSIONS
  We found predominantly mild autonomic impairment in ALS patients, with mostly cardiovagal and sudomotor involvement. Moderate autonomic failure occurred in 1 of 7 patients, especially those with an UMN presentation. Patients with selective corticospinal tract involvement may have more impairment of autonomic pathways.
authors.affiliation: Department of Neurology, Mayo Clinic, 200 First Street SW, Rochester, Minnesota, USA, 55905.
authors.forename: Ezequiel A
authors.initials: EA
authors.lastname: Piccione
authors.forename: David M
authors.initials: DM
authors.lastname: Sletten
authors.forename: Nathan P
authors.initials: NP
authors.lastname: Staff
authors.forename: Phillip A
authors.initials: PA
authors.lastname: Low
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000690:Amyotrophic Lateral Sclerosis / Q000175:diagnosis* / Q000503:physiopathology*; D001341:Autonomic Nervous System / Q000503:physiopathology*; D003430:Cross-Sectional Studies; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D016472:Motor Neuron Disease / Q000175:diagnosis / Q000503:physiopathology; D009431:Neural Conduction / Q000502:physiology; D012189:Retrospective Studies; D012720:Severity of Illness Index
keywords: Composite Autonomic Severity Score; amyotrophic lateral sclerosis; autonomic impairment; motor neuron disease; sudomotor