Autonomic system and amyotrophic lateral sclerosis

Muscle Nerve. 2015 May;51(5):676-9. doi: 10.1002/mus.24457. Epub 2015 Mar 31.

Abstract

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.

Keywords: Composite Autonomic Severity Score; amyotrophic lateral sclerosis; autonomic impairment; motor neuron disease; sudomotor.

Publication types

  • Comparative Study
  • Research Support, N.I.H., Extramural

MeSH terms

  • Adult
  • Aged
  • Aged, 80 and over
  • Amyotrophic Lateral Sclerosis / diagnosis*
  • Amyotrophic Lateral Sclerosis / physiopathology*
  • Autonomic Nervous System / physiopathology*
  • Cross-Sectional Studies
  • Female
  • Humans
  • Male
  • Middle Aged
  • Motor Neuron Disease / diagnosis
  • Motor Neuron Disease / physiopathology
  • Neural Conduction / physiology
  • Retrospective Studies
  • Severity of Illness Index