Autonomic system and amyotrophic lateral sclerosis |
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| Authors: | Ezequiel A. Piccione MD David M. Sletten MBA Nathan P. Staff MDPhD Phillip A. Low MD |
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| Affiliation: | Department of Neurology, Mayo Clinic, Rochester, Minnesota, USA |
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| 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. Muscle Nerve 51 :676–679, 2015 |
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| Keywords: | amyotrophic lateral sclerosis autonomic impairment Composite Autonomic Severity Score motor neuron disease sudomotor |
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