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.
Original language | English (US) |
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Pages (from-to) | 676-679 |
Number of pages | 4 |
Journal | Muscle and Nerve |
Volume | 51 |
Issue number | 5 |
DOIs | |
State | Published - May 1 2015 |
Keywords
- Amyotrophic lateral sclerosis
- Autonomic impairment
- Composite Autonomic Severity Score
- Motor neuron disease
- Sudomotor
ASJC Scopus subject areas
- Physiology
- Clinical Neurology
- Cellular and Molecular Neuroscience
- Physiology (medical)