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

Autonomic system and amyotrophic lateral sclerosis.

Author information

1
Department of Neurology, Mayo Clinic, 200 First Street SW, Rochester, Minnesota, USA, 55905.

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

PMID:
25211238
PMCID:
PMC4362936
DOI:
10.1002/mus.24457
[Indexed for MEDLINE]
Free PMC Article

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