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Autonomic system and amyotrophic lateral sclerosis
Author(s) -
Piccione Ezequiel A.,
Sletten David M.,
Staff Nathan P.,
Low Phillip A.
Publication year - 2015
Publication title -
muscle and nerve
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.025
H-Index - 145
eISSN - 1097-4598
pISSN - 0148-639X
DOI - 10.1002/mus.24457
Subject(s) - sudomotor , amyotrophic lateral sclerosis , pure autonomic failure , medicine , upper motor neuron , motor neuron , lower motor neuron , autonomic nervous system , cardiology , neurology , physical medicine and rehabilitation , disease , heart rate , orthostatic vital signs , psychiatry , blood pressure
: 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. Muscle Nerve 51 :676–679, 2015