Anesthetic management of patients with Huntington disease.

BACKGROUND: Huntington disease (HD) is a rare autosomal dominant disease with symptoms of chorea, dystonia, incoordination, cognitive decline, and behavioral difficulties. Abnormal responses to anesthesia have been reported in case reports and raised concerns regarding the safety of anesthesia in this patient population. METHODS: We performed a computerized search of the Mayo Clinic medical records database searching for patients with HD who underwent general anesthesia. Medical records were reviewed for anesthetic technique, medications used, and postoperative complications. RESULTS: We identified 11 patients with genetically confirmed HD who underwent 17 general anesthetics. Psychiatric medication use was common, with 6 patients using antipsychotics, 7 patients using antidepressants, and 3 patients using benzodiazepines. Succinylcholine was used in 7 anesthetics, and nondepolarizing neuromuscular blocking drugs in 11 anesthetics, all without adverse effects. Patients had normal responses to induction and maintenance of anesthesia without adverse effects. Serious postoperative complications did not occur. CONCLUSION: Contrary to previous case reports, we found that patients with HD have normal responses to general anesthesia. However, the anesthesiologist should be aware of interactions between anesthetics and psychiatric medications frequently used by these patients. Measures should also be taken to minimize the risk of pulmonary aspiration because bulbar dysfunction may be a manifestation of this disease.

Regional uptake of meal fatty acids in humans.

Two protocols were performed to study meal fatty acid metabolism. In protocol 1, 14 patients scheduled for elective intra-abdominal surgery (11 undergoing bariatric surgery for severe obesity) consumed a meal containing [3H]triolein in the evening before surgery. This allowed us to measure adipose tissue lipid specific activity (SA) in mesenteric and omental, deep and superficial abdominal subcutaneous adipose tissue. Intra-abdominal adipose tissue lipid SA was greater than subcutaneous lipid SA. There were no significant differences between mesenteric and omental or between deep and superficial abdominal subcutaneous adipose tissue. In protocol 2, meal fatty acid oxidation and uptake into subcutaneous and omental adipose tissue ([3H]triolein) were measured in six normal, healthy volunteers. Meal fatty acid oxidation (3H2O generation) plus that remaining in plasma ( approximately 1%) plus uptake into upper body subcutaneous, lower body subcutaneous, and visceral fat allowed us to account for 98 +/- 6% of meal fatty acids 24 h after meal ingestion. We conclude that omental fat is a good surrogate for visceral fat and that abdominal subcutaneous fat depots are comparable with regard to meal fatty acid metabolic studies. Using [3H]triolein, we were able to account for virtually 100% of meal fatty acids 24 h after meal ingestion. These results support the meal fatty acid tracer model as a way to study the metabolic fate of dietary fat.