Autonomic system and amyotrophic lateral sclerosis

Ezequiel A. Piccione, David M. Sletten, Nathan P. Staff, Phillip A. Low

Research output: Contribution to journalArticlepeer-review

35 Scopus citations


Introduction: 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.

Original languageEnglish (US)
Pages (from-to)676-679
Number of pages4
JournalMuscle and Nerve
Issue number5
StatePublished - May 1 2015


  • Amyotrophic lateral sclerosis
  • Autonomic impairment
  • Composite Autonomic Severity Score
  • Motor neuron disease
  • Sudomotor

ASJC Scopus subject areas

  • Physiology
  • Clinical Neurology
  • Cellular and Molecular Neuroscience
  • Physiology (medical)


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