Monitored anesthesia care for a patient with advanced Huntington's chorea.

Huntington’s disease (HD), a rare, autosomal dominant disorder of the central nervous system, has been associated at times with unusual responses to anesthetic agents such as thiopental, midazolam, succinylcholine, and nondepolarizing neuromuscular blocking drugs. We describe the anesthetic management of a 50 year-old female with advanced HD, complicated by chorea, dementia, dysphagia, and dysarthria, undergoing percutaneous endoscopic gastrostomy (PEG) placement. To the best of our knowledge, there have not been any prior reports describing the use of propofol for sedation in a patient with Huntington’s disease.