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Anesthetic management of patients with Huntington disease
Jonathon E Kivela1, Juraj Sprung, Peter A Southorn
1Departments of Anesthesiology, College of Medicine, Mayo Clinic, Rochester, Minnesota, USA.
Insights
Patients with Huntington disease (HD) tolerate general anesthesia well, contrary to prior concerns. Anesthesiologists should monitor for drug interactions and aspiration risk in HD patients.
Area of Science:
- Anesthesiology
- Neurology
- Genetics
Background:
- Huntington disease (HD) is a rare autosomal dominant neurodegenerative disorder.
- Symptoms include chorea, dystonia, cognitive decline, and behavioral issues.
- Previous case reports raised safety concerns regarding anesthesia in HD patients.
Purpose of the Study:
- To evaluate the safety and response of Huntington disease patients to general anesthesia.
- To identify potential anesthetic complications in this patient population.
Main Methods:
- Computerized search of Mayo Clinic medical records for HD patients undergoing general anesthesia.
- Review of medical records for anesthetic techniques, medications, and postoperative complications.
Main Results:
- Eleven genetically confirmed HD patients underwent 17 general anesthetics without adverse effects.
- Common use of psychiatric medications (antipsychotics, antidepressants, benzodiazepines) did not cause complications.
- Succinylcholine and nondepolarizing neuromuscular blocking drugs were used safely.
Conclusions:
- Patients with Huntington disease exhibit normal responses to general anesthesia.
- Awareness of anesthetic-psychiatric medication interactions is crucial.
- Minimizing pulmonary aspiration risk is important due to potential bulbar dysfunction in HD.
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.
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