ABSTRACT
Familial dysautonomia [FD], also known as Riley-Day syndrome, is a disorder of the autonomic nervous system that results in loss of demyelinated nerve fibers of sensory, sympathetic and parasympathetic neurons. Individuals with FD have variable clinical symptoms that may include insensitivity to pain, inability to produce tears, poor oral intake during infancy, repeated vomiting, failure to thrive, wide fluctuations in body temperature, and episodic hypertension and hypotension. These paroxysmal crises are due to dysfunction of the autonomic system with an elevation of both norepinephrine and dopamine levels. Clonidine, an alpha 2-adrenergic agonist, has been previously demonstrated to be an effective pharmacological agent in the treatment of dysautonomic crises related to FD. Dexmedetomidine is an alpha 2-adrenergic agonist with an alpha 2:alpha 1 specificity that is almost 8 times that of clonidine. The authors present the perioperative use of dexmedetomidine in a patient with FD. Previous reports of the use of dexmedetomidine in patients with FD are reviewed and the beneficial physiologic effects discussed