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.


CONCLUSIONS
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.
Kivela
Jonathon E
JE
Departments of Anesthesiology, College of Medicine, Mayo Clinic, Rochester, Minnesota, USA.
Sprung
Juraj
J
Southorn
Peter A
PA
Watson
James C
JC
Weingarten
Toby N
TN
D000328:Adult; D000368:Aged; D000768:Anesthesia, General / Q000009:adverse effects / Q000379:methods; D000777:Anesthetics / Q000009:adverse effects; D005260:Female; D006801:Humans; D006816:Huntington Disease / Q000188:drug therapy; D007430:Intraoperative Care; D008297:Male; D008875:Middle Aged; D011229:Preanesthetic Medication; D011619:Psychotropic Drugs / Q000627:therapeutic use; D013514:Surgical Procedures, Operative
D000777:Anesthetics; D011619:Psychotropic Drugs