Huntington’s Disease (HD) was first described by Paracelsus in the 16th century, when he created the term “chorea” to describe the characteristic choreiform movements associated with the disease. The word originates from the Greek term “khoreia” meaning “to dance in unison.” The disease was definitively described by George Huntington in his paper “On Chorea” in 1872, which helped to establish its role as a heritable illness. In North America and Europe, HD is thought to occur between 5-7 per 100,000 people, but is not unlikely to be encountered by anesthesiologists. There are important implications for perioperative management that the anesthesiologists should be aware of and be prepared to manage, particularly with motor function, esophageal dysmotility, dystonia, muscle rigidity, inability to cooperate, and case reports of adverse drug reactions.
This case report presents the successful general anesthetic of a 57 year old, 50 kg female with diagnosed HD, presenting for hip hemiarthroplasty following a fall. Her manifestations of the disease are dementia, well controlled chorea, poorly coordinated oropharyngeal movements and poor motor response to commands. She is induced with 70mg propofol, 50mg lidocaine and 100mcg of fentanyl, and maintained on TIVA of propofol and remifentanil 20mcg/mL. She is extubated at the end of the case and has no further complications from anesthesia.
Case reports exist citing use of succinylcholine and thiopental as cause for prolonged emergence, however there have been larger studies conducted that find no increased incidence of prolonged emergence with these drugs. It is hypothesized that populations with HD may have low levels of atypical pseudocholinesterases, however this remains controversial. Additionally, there have been recent studies citing maintenance of GA with isoflurane in rat models with disease progression. Overall, general anesthesia appears to be largely safe for those with HD, as long as the anesthesiologist is aware of the risks associated with the manifestations of the disease, drug interactions and can guide the patient and family through safe decision making.
