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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).

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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.