Autonomic Dysfunction in Neuromuscular Disorders

The autonomic nervous system extends throughout the human body with a combination of central and peripheral nerves, nuclei, ganglia, and neurotransmitters in order to maintain system homeostasis. The autonomic nervous system modulates blood pressure, heart rate, thermoregulation, motility of the gastrointestinal system, micturition, pupillary function, and salivary gland secretion, among other things. Dysfunction of one, or all, of these processes may occur in neuromuscular disorders that affect the autonomic nervous system. This chapter briefly reviews the anatomic structure and clinical implications of autonomic dysfunction, the technical evaluation of the autonomic nervous system through neurophysiologic testing, and the neuromuscular diseases that result in autonomic disturbances and discusses treatment options in patients with dysautonomia.