Anesthetic Management of a Patient with
Huntington’s Disease (HD): A Case report
David McEnerney D.O, Mohamad El Churafa D.O and Giuseppe Giuratrabocchetta M.D*
Department of Anesthesiology, University of Florida, UF Health, USA
Submission: August 17 2021; Published: October 07, 2021
*Corresponding author: Marco Gruss, Department of Anaesthesiology, Intensive Care Medicine and Pain Therapy, Klinikum Hanau GmbH, Leimenstrasse 20, Hanau D-63450, Germany, Tel: 0049-6181-296 2410
How to cite this article: Giuseppe Giuratrabocchetta M.D., Department of Anesthesiology, University of Florida, UF Health, UF College of Medicine-Jacksonville, 2nd Floor, Clinical Center 655 West 8th Street, C72, Jacksonville, FL 32209, USA J Anest & Inten care med. 2021; 11(3): 555813. DOI 10.19080/JAICM.2021.11.555813
Huntington disease (HD) is a rare autosomal dominant disease affecting the central nervous system (CNS) that is attributed with distinct anesthetic challenges and implications. We present a patient with HD who underwent general anesthesia for two different orthopedic surgeries using, respectively, total intravenous anesthesia (TIVA) and inhalational anesthesia; neuromuscular blockade reversal was obtained utilizing, respectively, traditional agents and Sugammadex. There have been no prospective studies on anesthetic management of patients with HD, and clinicians rely solely on case reports or prior experience in assessing general anesthetic safety in these patients. This case report presents different methods in the anesthetic management of the same patient with HD.
Huntington disease (HD) is an autosomal dominant disease caused by cytosine-adenine-guanine (CAG) trinucleotide repeat in the Huntingtin gene on chromosome 4p. The protein, Huntingtin, is present throughout the body but symptomatology is primarily due to central nervous system (CNS) dysfunction. Pathological destruction of neurons in the basal ganglia, particularly in the putamen and caudate nucleus, is evident . Symptoms manifest in the form of progressively worsening cognitive deterioration, involuntary choreiform movements, psychiatric disturbances, and dysphagia . Death typically occurs 10-30 years from onset of disease and is generally due to aspiration complications . Age of onset is generally between ages 30-45 but is dependent on the number of trinucleotide repeats found in the gene. Greater than 40 repeats demonstrates full penetrance with earlier onset, while less than 28 repeats yield no symptoms. Anticipation is commonly seen between generations with HD. It is associated with people of caucasian ancestry due to its prevalence of approximately 5.7 per 100,000 in North America, and 0.4 per 100,000 in Asia and 2.7 per 100,000 worldwide .
The anesthetic management of patients with HD has been complicated by pharyngeal muscle involvement, prolonged apnea, generalized tonic spasm, postoperative fever, prolonged recovery, increased sensitivity to benzodiazepines, hypertonia,and postoperative shivering . The optimal anesthetic management of these patients has yet to be established due to the increased risk of aspiration, although multiple different agents have been successfully used for induction, muscle relaxation, and maintenance of anesthesia. In this report, we present our intraoperative anesthetic management of the same patient with HD who underwent two orthopedic operations.
A 63-year-old, caucasian female weighing 55.3 kilograms with PMH of HD, seizures, dementia, anxiety, depression, hyperlipidemia, and adult failure to thrive presented for open reduction internal fixation (ORIF) of a right proximal humerus fracture due to a fall from her bed. Physical examination did not demonstrate any choreiform movements, but she did have difficulty communicating. Her home medications included Amantadine, Buspirone, Donepezil, Fluoxetine, Levetiracetam, Lorazepam, Quetiapine, and Risperidone. She was brought to the OR, and, after pre-oxygenation, a modified rapid sequence induction was performed with 100 mcg of Fentanyl, 50 mg of Lidocaine, 60 mg of Propofol, and 50 mg of Rocuronium. The patient was intubated with a Miller 2 blade with a grade 1 view. Anesthesia was maintained with TIVA using Propofol and intermittent boluses of Fentanyl. She received Dexamethasone, IV Acetaminophen, and Ketorolac. Neuromuscular blockade was reversed with 100 mg of
Sugammadex and extubation was without issue. She tolerated the
procedure well without complications.
Five days later, the patient presented to the emergency
department with dizziness, weakness, and a hemoglobin of 5.9 g/
dL with a hematoma over her surgical site. She was brought to the
operating room for a revision of ORIF and hardware removal of
the right proximal humerus and was taken care of by a different
anesthesia team. After pre-oxygenation the patient received 100
mg of Propofol, 50 mg of Lidocaine, 100 mcg of Fentanyl, and 30
mg of Rocuronium, but was found to have an infiltrated midline.
A second IV was placed and 100 mg Propofol, 50 mg of Fentanyl,
and 20 mg of Rocuronium were administered. Bag mask was easy
without an airway device. She was intubated with a Mac 3 blade
with a grade 2 view. Anesthesia was maintained with Sevoflurane
throughout the case. At the end of the case, she received
Ondansetron for PONV prophylaxis. Neuromuscular blockade was
reversed with 3 mg of Neostigmine and 0.4 mg of Glycopyrrolate.
She was again extubated without issues. She tolerated this
procedure well without complications.
HD is a rare disease, there have been no prospective studies on
anesthetic management, and we must solely rely on case reports
to assess general anesthetic safety in these patients. There are
numerous anesthetic considerations that must be considered.
Many HD patients are on antipsychotics, antidepressants,
antiepileptics, and benzodiazepines for management of psychiatric
disorders and chorea. Theoretically, there may be inhibition or
induction in the cytochrome P450 system leading to changes in
the medication(s) half-life. Anesthesiologists should be aware of
these medications and their potential to interact with anesthetic
Previous cases have reports of prolonged apnea after
administration of 50 mg of Succinylcholine, but the patient was
found to have a low plasma cholinesterase level. There have been
conflicting population studies that show a genetic connection
between HD and a rare plasma cholinesterase fluoride-resistant
allele. But in a report of the literature, there have been multiple
incidences of Succinylcholine use without apnea . In both of
our cases, muscle relaxation was asked for by the surgical team
and Rocuronium was used. Reversal of muscle relaxation was
treated by Sugammadex in the first case. In the second operation,
our patient did receive Glycopyrrolate without any deleterious
effects in post-operative recovery. There have been case reports
of Metoclopramide and Anticholinergic medications worsening
choreiform movements . It is preferred to utilize a quaternary
amine, Glycopyrrolate, to reverse muscular blockade because it
does not cross the blood-brain-barrier.
Prior studies report uses of 5mg/kg of Sodium Thiopental
causing prolonged apnea over 1 hour. Although this dose seems aggressive, as most HD patients are debilitated , Sodium
Thiopental is no longer in use as an induction agent. For both
our cases induction was performed with Fentanyl, Lidocaine, and
Propofol without complications. In the first case, a decision was
made to perform an RSI, which is generally used for patients with
HD, as late symptoms include dysphagia, which places HD patients
at an increased risk of aspiration. The most common cause of
death in this patient population is aspiration pneumonia. For the
second case the decision was made for an RSI, but an infiltrated
IV necessitated bag mask ventilation until a second IV could be
placed. In both operations, ventilation throughout the case was
normal and no signs of aspiration were noticed. There have been
case reports of HD patients requiring awake fiberoptic intubation
Maintenance of anesthesia has been safely described using
inhalation agents and total intravenous anesthesia. Kang et al. 
theorize that a TIVA with Propofol and Remifentanil would be ideal
for HD patients due to ease of titration and rapid recovery without
lingering effects potentially leading to airway compromise. In
this patient, both inhalational with Sevoflurane and TIVA with
Propofol were used without complications. Both cases used a
Bispectral Index for titration of anesthesia. Postoperative delirium
can be increased in HD patients after general anesthesia . Our
patient did not experience any alterations that affected her postoperative
care in either case.
Successful spinal anesthesia has been reported in the literature
twice. Due to the location of the operation this was not considered,
and although regional anesthesia was potentially an option, it was
not utilized [6,7].
There have been many case reports of patients with HD
showing successful anesthetic management, although it seems
none have shown two different anesthetic regimes on the same
patient in such short succession, as in our case report. The perfect
anesthetic is likely never going to be found and will be at the
discretion of the provider for each individual patient. Further
studies should be performed to help elucidate the best anesthetic
choices for these patients.
Nance M, Paulsen JS, Rosenblatt A, Wheelock V (2011) Overview and principles of treatment. In: A Physician's Guide to the Management of Huntington's Disease. (3rd edn), Huntington's Disease Society of America, p. 5.