Work

Autonomic system and amyotrophic lateral sclerosis

Year: 2014

Type: article

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.

Source: Muscle & Nerve

DOI: 10.1002/mus.24457

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

Institution: Mayo Clinic

Language: English

Topic: Amyotrophic Lateral Sclerosis Research

Subfield: Neurology

Field: Medicine

Domain: Health Sciences

SDG: Good health and well-being

Open Access status: green

Funders: National Institutes of Health, National Cancer Institute, National Institute of Neurological Disorders and Stroke, National Center for Advancing Translational Sciences