Category: Choreas (Non-Huntington's Disease)
Objective: To present a case of combined contralateral internal carotid artery (ICA) occlusion and ipsilateral ICA critical stenosis presenting with isolated hemichorea.
Background: Hemichorea consists in flowing, involuntary, purposeless movements on one side of the body and it has a wide differential diagnosis. A few case reports advocate ICA stenosis should be considered in chorea workup, suggesting a lesion in the contralateral basal ganglia or related structures as key factor.
Method: Clinical case
Results: A 78 year-old male presented with a 1-year history of a subtle jerk of his right hand he called a tic. He had history of controlled HIV infection, low grade urothelial bladder carcinoma treated with mitomycin C, and right-hand trauma. On observation, he presented right hemichorea with brachial predominance sparing the face. The workup was unremarkable and there were no parenchymal alterations on brain MRI but MR angiographic study revealed a left ICA occlusion and a right ICA critical stenosis. Perfusion studies showed global and symmetrical hypoperfusion. Doppler US revealed bilateral dampened MCA velocities. After right ICA stenting procedure there was a significant and gradual improvement of chorea at 3 and 6 months. Finally, the patient maintains solely discrete movements with distractibility manoeuvres. Doppler US showed normalized velocity on right MCA and improvement on left MCA.
Conclusion: Carotid occlusive disease compromises blood flow in lenticulostriate branches possibly leading to basal ganglia ischemia or hypoperfusion. This was only suspected due to the angiographic findings, highlighting the need to include them in the workup of chorea of patients with vascular risk factors, at least, even in the absence of evidence of parenchymal ischemic lesions, as our case. Differential diagnosis include chorea associated with HIV or opportunistic infection or a paraneoplastic syndrome. These seemed unlikely due to the temporal gap, disease stability and mainly after the witnessed post stenting clinical improvement. The evolution supports the hypothesis of hemodynamic compromise in basal ganglia as the pathophysiology of hemichorea associated with carotid artery stenosis.
To cite this abstract in AMA style:M. Ferro, M. Gil Veiga, I. Fragata, C. Marques Matos. Critical Bilateral Carotid Disease presenting as Hemichorea [abstract]. Mov Disord. 2022; 37 (suppl 2). https://www.mdsabstracts.org/abstract/critical-bilateral-carotid-disease-presenting-as-hemichorea/. Accessed September 22, 2023.
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MDS Abstracts - https://www.mdsabstracts.org/abstract/critical-bilateral-carotid-disease-presenting-as-hemichorea/