View Abstract

A6881 - An Off-Label Use of Dexmedetomidine in Treatment of Dysautonomic Crisis in Familial Dysautonomia (Riley-Day Syndrome)
Author Block: A. Subedi, I. A. Lalani, S. M. Weiner; Monmouth Medical Center, Long Branch, NJ, United States.
Introduction: Familial Dysautonomia (Riley-Day Syndrome) is a rare genetic disorder characterized by recurrent, refractory, paroxysmal episodes of hypertension, tachycardia, flushing, vomiting, sweating, swallowing dysfunction and speech dysfunction known as ""dysautonomic crisis"". The mainstay of treatment of such dysautonomic crisis has been benzodiazepines and clonidine. Those cases refractory to the treatment with benzodiazepines and clonidine have also been treated with carbidopa. We present a case of dysautonomic crisis in familial dysautonomia who was treated effectively with dexmedetomidine after no response to labetalol, diazepam and clonidine. Case description: A 19 year-old-male with medical history of familial dysautonomia with frequent autonomic crisis, Obstructive Sleep Apnea on home CPAP, corneal abrasion, history of febrile seizure was brought to the Emergency Department (ED) by his parents because of intractable nausea and vomiting for 6 hours. The patient has had such symptoms during autonomic crisis. He has on an average 2-3 crises per week which would resolve with clonidine and labetalol at home. The night of presentation, his symptoms did not resolve even after 3 treatments with clonidine and labetalol at home so was brought to the ED. In the ED, he continued to have nausea and vomiting with BP of 206/158 mm Hg and tachycardic with heart rate up to 110bpm. He was treated with intravenous Labetalol and intravenous fluids which did not resolve his symptoms. He was then admitted to Intensive Care Unit (ICU) and was started on Dexmedetomidine drip. Within few hours, the patient's symptoms improved and was back to baseline and the patient was discharged to home from the ICU. Discussion: Dysautonomic crises is the hallmark of Familial Dysautonomia. It is believed to be caused by high norepinephrine and dopamine levels triggered by emotional and physical stress. Dexmedetomidine is a centrally acting α2 adrenergic agonist which acts as a sedative and anesthetic by acting in the G-protein in the brainstem that leads to inhibition of norepinephrine release. In case of failure of the conventional therapy for crises, which is benzodiazepines and clonidine, suppression of norepinephrine by dexmedetomidine seems to be effective in treating the crisis theoretically and in this case, in practice. There have been reported cases and studies about the treatment with dexmedetomidine. In this case, the crisis was safely and effectively treated by dexmedetomidine.