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Anesthetic management of a parturient with Huntington’s disease
Abstract Number: S4C-7
Abstract Type: Case Report/Case Series
Introduction: Huntington’s disease is an inherited progressive neurodegenerative disorder characterized by choreoform movements, cognitive impairment, mood disturbances, and dementia. Though uncommon, it is an autosomal dominant condition with no known cure. The incidence of Huntington’s disease in pregnancy is rare, and guidelines for the management of these patients are not well established. Here, we discuss the management of a parturient with a strong family history of neurodegenerative disease, who presented to the labor and delivery unit in active labor.
Case report: 37 year old G7P4A2 at 39 weeks gestation presented to the labor and delivery unit with ruptured membranes and regular contractions. She had a strong family history of neurodegenerative disease – her mother died at 36 from presumed Huntington’s degeneration, and her three brothers suffered from the same condition. She presented to a neurologist during the second trimester of her pregnancy for “arm and leg jerking.” She underwent brain imaging, which was unremarkable. However, the neurologist believed that she had the early stages of Huntington’s disease based on her exam findings and family history. Our physical exam was notable for perioral twitching and sporadic choreoform arm movements.
The patient requested an epidural for labor analgesia. We performed an extensive literature search to determine whether a neuraxial anesthetic was safe in patients with Huntington’s disease. We found a few case reports in which neuraxial anesthesia was successfully administered without complications. In addition, we learned that patients with this disease are at increased risk of aspiration and are exquisitely sensitive to muscle relaxants. Based on these findings, we determined that epidural placement was the best option for labor analgesia and possible surgical anesthesia. An epidural catheter was placed and adequate labor analgesia was achieved with a 0.0625% bupivacaine solution.
The obstetrical team decided to proceed with a caesarean section for non-reassuring fetal heart tones. The patient’s labor epidural was dosed with 2% lidocaine and adequate surgical anesthesia was achieved. The procedure was uncomplicated, and required no adjuvants. She was discharged on post-op day 3 with no worsening of her neurological symptoms.
Conclusion: Huntington’s disease in parturients is very rare. There have been few reports in the literature regarding ideal analgesic and anesthetic techniques for obstetrical patients. This case illustrates one effective method – we successfully achieved adequate analgesia for caesarian section via epidural placement with no neurological complications during her stay.
1. Hoskins KE, Tita AT, et al. Pregnancy and active Huntington disease: a rare combination. J Perinatol. 2008 Feb; 28(2):156-7
2. Dayalu P, Albin RL. Huntington disease: pathogenesis and treatment. Neurol Clin. 2015 Feb; 3(1):101-14