Objective: To describe a case of acute onset chorea in a patient with significant right carotid stenosis with resolution of symptoms post transcarotid artery revascularization (TCAR).

Background: Chorea is a hyperkinetic movement disorder that can affect the face, trunk, and limbs and is characterized by jerky, irregular, and non-patterned movements. While the differential diagnosis is quite broad, underlying cerebrovascular disease is an important etiology to keep in mind with lesions typically occurring in the basal ganglia, subthalamic nucleus and thalamus. However, even in the absence of structural lesions, significant cerebral hypoperfusion should be kept in mind as an underlying etiology for new onset chorea.

Method: Case report

Results: A 77 year-old female presented to the emergency department with acute onset oral-lingual dyskinesias and chorea. Pertinent past medical history included prominent cerebrovascular disease including subarachnoid hemorrhage (s/p aneurysmal clipping in the 1990’s in India), stroke (w/ residual right hemiparesis), hypertension, and diabetes. She underwent an extensive inpatient work up evaluating for various infectious, inflammatory, and autoimmune etiologies which were all unrevealing. Unfortunately, due to previous aneurysm clipping placed in India, MRI Brain was unable to be obtained, however head CT was without evidence of a large structural lesion to explain her symptoms. PET-CT obtained without evidence of malignancy. Genetic testing for Huntington disease as well as a movement disorders genetic testing panel were negative. As part of her initial work up, the patient was found to have significant right sided carotid stenosis (90%) for which she was referred for neurosurgical evaluation. Despite the presumed asymptomatic nature of her stenosis, given the degree of stenosis and the potential for an underlying vascular etiology to her symptoms, she was deemed a candidate for transcarotid artery revascularization (TCAR) which she underwent successfully and resulted in almost complete resolution of her hyperkinetic movement disorder.

Conclusion: This case highlights the importance of including carotid stenosis in the differential diagnosis of new onset chorea and including pertinent vessel imaging with CTA or carotid ultrasound in the diagnostic work up. For patients with significant stenosis and new onset chorea, revascularization should be considered as an important management strategy.

References: Chung SJ, Im JH, Lee MC, Kim JS. Hemichorea after stroke: clinical-radiological correlation. J Neurol. 2004 Jun;251(6):725-9. doi: 10.1007/s00415-004-0412-5. PMID: 15311349.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/improvement-in-chorea-after-transcarotid-artery-revascularization-a-case-report/