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Autonomic impairment in primary lateral sclerosis
Hebatallah R Rashed1,2, Nathan P Staff1, Margherita Milone1
1Department of Neurology, Mayo Clinic, Rochester, MN, USA.
Summary
Autonomic dysfunction is common in primary lateral sclerosis (PLS), typically mild. This multisystem disease affects the autonomic nervous system, similar to amyotrophic lateral sclerosis (ALS).
Area of Science:
- Neurology
- Autonomic Neuroscience
Background:
- Autonomic nervous system (ANS) involvement is reported in motor neuron diseases.
- Previous studies suggest more severe autonomic dysfunction in upper motor neuron predominant syndromes.
Purpose of the Study:
- To characterize the extent and nature of autonomic impairment in patients with primary lateral sclerosis (PLS).
Main Methods:
- Retrospective analysis of neurological evaluations, thermoregulatory sweat tests, and autonomic reflex screens in 34 PLS patients.
- Exclusion of patients with other causes of autonomic failure or artifact-compromised test results.
Main Results:
- 17 out of 34 patients reported autonomic symptoms, most commonly orthostatic lightheadedness, bladder, bowel, and erectile dysfunction.
- Autonomic reflex screens were abnormal in 20/33 patients (60.6%), with sudomotor abnormalities most frequent (90%).
- Composite Autonomic Severity Score indicated mild to moderate impairment in 60.6% of patients.
Conclusions:
- Primary lateral sclerosis (PLS) frequently exhibits autonomic dysfunction, generally mild.
- The pattern of autonomic dysfunction suggests preganglionic/ganglionic involvement.
- PLS, like amyotrophic lateral sclerosis (ALS), is a multisystem disease impacting the autonomic nervous system.
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