Objective: To report the first case of adult-onset post-pump chorea in Puerto Rico and raise awareness of our patient’s atypical age and onset of clinical symptoms while exploring the patient’s risks, possible pathogenesis, treatments, and outcomes.

Background: Chorea is a movement dysfunction characterized by involuntary, irregular, brief, non-rhythmic, and unpredictable movements of the limbs, neck, head, and/or face. Post-pump chorea (PPC) is the development of chorea after cardiopulmonary bypass (CPB). PPC is a rare complication predominantly observed in children with congenital heart disease undergoing CPB; however, adult-onset PPC is even less reported having an estimated prevalence of 0.4% [1] When it does occur in adults, patients have a mean age of 46.8 years [1], and the onset latency typically ranges from 3 to 12 days post CPB [1,2,3,4].

Method: Case report

Results:

A 66-year-old male ex-smoker with past medical history of coronary artery disease, hypertension, diabetes, hypercholesterolemia, hypothyroidism, and a prior stroke developed choreic movements of mouth, tongue, and to a lesser extent in limbs. Symptoms began 2 months after bovine aortic valve replacement with CPB. Postoperatively, he spent 9 days in the intensive care unit (ICU) due to not tolerating extubation. The patient denied antipsychotic or antiemetic use and had no family history of Huntington’s disease (HD) or other movement disorders. Workup, including antinuclear antibody (ANA), anti-double-stranded DNA (anti-dsna), anticardiolipin antibodies, and Human Immunodeficiency Virus (HIV) test was negative; thyroid stimulating hormone (TSH) levels were normal. Head CTs at hospitalization and two months post-surgery were unremarkable. Brain MRI was not possible due to an incompatible defibrillator. PPC was diagnosed based on history of CPB surgery, patient’s comorbidities, and workup excluding other disorders. Patient was successfully treated with deutetrabenazine for involuntary movements, but later developed a neurocognitive disorder.

Conclusion: This case presents a rare occurrence of late-onset post-pump chorea (PPC) in an elderly adult, emerging two months after CPB surgery. We propose the patient’s chorea resulted from the combination of his vascular risk factors, CPB surgery, and prolonged ICU stay.

References: [1] Ahn JH, Song J, Choi I, Youn J, Cho JW. Risk factors and prognosis of adult-onset post-pump chorea. J Neurol Sci. 2021;422:117328. doi:10.1016/j.jns.2021.117328

[2] Bisciglia M, London F, Hulin J, Peeters A, Ivanoiu A, Jeanjean A. Choreoathetotic syndrome following cardiac surgery. J Clin Anesth. 2017;36:59-61.doi:10.1016/j.jclinane.2016.08.043

[3] Gherpelli JL, Azeka E, Riso A, Atik E, Ebaid M, Barbero-Marcial M. Choreoathetosis after cardiac surgery with hypothermia and extracorporeal circulation. Pediatr Neurol. 1998;19(2):113-118. doi:10.1016/s0887-8994(98)00036-8

[4] Huntley DT, al-Mateen M, Menkes JH. Unusual dyskinesia complicating cardiopulmonary bypass surgery. Dev Med Child Neurol. 1993;35(7):631-636. doi:10.1111/j.1469-8749.1993.tb11701.x

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MDS Abstracts - https://www.mdsabstracts.org/abstract/adult-onset-post-pump-chorea-a-rare-manifestation-post-cardiac-surgery-in-a-puerto-rican-patient/