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Reversible hemichorea due to contralateral anterior cerebral artery territory hypoperfusion

S. Adukia, P. Shah, M. Shrivastava, G. Sangani, D. Sanghvi, M. Bhatt, A. Aggarwal (Mumbai, India)

Meeting: MDS Virtual Congress 2021

Abstract Number: 49

Keywords: Chorea (also see specific diagnoses, Huntingtons disease, etc): Pathophysiology, Chorea (also see specific diagnoses, Huntingtons disease, etc): Treatment, Hemichorea

Category: Choreas (Non-Huntington's Disease)

Objective: We report hemichorea due to hypoperfusion of the contralateral anterior cerebral artery (ACA) territory (including caudate nucleus and its connections) that reversed with revascularization.

Background: Hemichorea is often caused by strokes in the contralateral subthalamic nucleus, striatum, thalamus, and frontal, parietal or insular cortices. Cerebral hypoperfusion has also been associated with hemichorea.

Method: –

Results: A 42-year woman had developed acute right hemichorea twelve days earlier. She had well-controlled grade 1 hypertension and had had 2 uneventful pregnancies 14 years ago. There was no history of limb weakness, behavioural problems, fever or other systemic symptoms, neuroleptic drug use, or relevant family history. Except for moderate right hemichorea, the neurological exam was normal. Extensive work-up for chorea including haemogram, erythrocyte sedimentation rate, routine metabolic tests, panels for autoimmune, vasculitis, infections, and paraneoplastic disorders, echocardiography, whole-body PET-CT and test for Huntington’s disease, was negative. Brain MRI was normal. MR angiogram and DSA revealed severe stenosis of A1 segment of the left ACA and moderate stenosis of M1 segment of the left middle cerebral artery (MCA) due to atherosclerosis. MR perfusion study showed hypoperfusion in the distal left ACA and ACA/MCA watershed territories. In absence of an alternative aetiology, we hypothesized that hypoperfusion of the contralateral ACA and /or MCA territories (possibly the caudate or its connections) was leading to the hemichorea. Therefore, best medical management for atherosclerosis and tetrabenazine for symptomatic treatment of chorea was commenced. There was initial improvement but over the next 10 months, the hemichorea worsened. Repeat DSA showed worsening of left ACA stenosis. In consultation with the patient and her family, we planned endovascular revascularization of the more severely stenosed left ACA. The hemichorea improved within the few days of the left ACA stenting and the improvement has been sustained over the last six months.

Conclusion: Our report suggests that acute hemichorea may result from hypoperfusion of the contralateral ACA territory (that includes caudate and its connections) and the chorea may be reversed by revascularization. Our report reiterates cerebral hypoperfusion as a cause of reversible hemichorea.

To cite this abstract in AMA style:

S. Adukia, P. Shah, M. Shrivastava, G. Sangani, D. Sanghvi, M. Bhatt, A. Aggarwal. Reversible hemichorea due to contralateral anterior cerebral artery territory hypoperfusion [abstract]. Mov Disord. 2021; 36 (suppl 1). https://www.mdsabstracts.org/abstract/reversible-hemichorea-due-to-contralateral-anterior-cerebral-artery-territory-hypoperfusion/. Accessed June 15, 2025.
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