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Utility of Dissociated Intrinsic Hand Muscle Atrophy in the Diagnosis of Amyotrophic Lateral Sclerosis
Published on: March 4, 2014
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Autonomic system and amyotrophic lateral sclerosis
Ezequiel A Piccione1, David M Sletten, Nathan P Staff
1Department of Neurology, Mayo Clinic, 200 First Street SW, Rochester, Minnesota, USA, 55905.
Muscle & Nerve
|September 12, 2014
Summary
Autonomic nervous system impairment is common in motor neuron disease, primarily mild and affecting cardiovagal and sudomotor functions. Upper motor neuron presentations show greater autonomic dysfunction.
Area of Science:
- Neurology
- Autonomic Neuroscience
Background:
- Motor neuron disease (MND) encompasses a group of progressive neurodegenerative disorders.
- Autonomic dysfunction is increasingly recognized as a component of MND.
Purpose of the Study:
- To characterize the prevalence and patterns of autonomic impairment in patients with motor neuron disease.
- To investigate differences in autonomic dysfunction across subtypes of MND.
Main Methods:
- Retrospective analysis of neurological evaluations and autonomic function tests in 132 patients.
- Classified patients into classic amyotrophic lateral sclerosis (ALS), lower motor neuron (LMN), and upper motor neuron (UMN) predominant disease.
- Utilized the Composite Autonomic Severity Score (CASS) to quantify autonomic impairment.
Main Results:
- 75% of patients exhibited autonomic impairment, with mild (CASS 1-3) in 85% and moderate (CASS 4-7) in 15%.
- Sudomotor (46%) and cardiovagal (50%) functions were most frequently affected.
- UMN-predominant disease showed significantly higher CASS scores compared to classic ALS and LMN groups.
Conclusions:
- Motor neuron disease patients frequently experience mild autonomic impairment, predominantly affecting cardiovagal and sudomotor systems.
- Moderate autonomic failure occurs in approximately 14% of patients, particularly those with UMN involvement.
- Autonomic pathway impairment may be more pronounced in patients with selective corticospinal tract involvement.
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