Objective: To determine whether stenosis of internal carotid artery (ICA) impacts the activity and perfusion of basal ganglia (BG) in six hemichorea (HC) patients.

Background: HC is usually the result of a focal lesion in BG. Possible causes include acute stroke in BG and adjacent areas, neoplastic lesions, hyperglycemia [1] and ICA stenosis [2–8]. Our previous observation of HC in patients with ICA stenosis showed an association between HC, contralateral ICA stenosis and BG hyperactivity assessed by ¹⁸F-FDG PET on the side of the stenosis, which normalized after revascularization procedures [9]. We now present additional patients in whom we measured BG metabolic activity and perfusion.

Method: Six patients (four male; mean age 74.7 ± 9.9) with acute HC and contralateral ICA stenosis determined with carotid ultrasound or CT angiography underwent ¹⁸F-FDG PET brain scan to evaluate BG metabolism. Perfusion SPECT with ^99mTc-HMPAO was performed at different time points from HC onset in three patients to evaluate dynamics of BG perfusion. Two patients underwent revascularization and four were treated conservatively.

Results: ICA stenosis contralateral to HC ranged from 60% to 90%. HC significantly improved after treatment in all patients. ¹⁸F-FDG PET scans showed BG hyperactivity (compared to BG on the other side) contralateral to HC in all patients, which normalized in the two patients who underwent repeated ¹⁸F-FDG PET scans after treatment. In one patient, perfusion SPECT that was performed 1 month after HC onset showed contralateral BG hypoperfusion. Two patients who underwent perfusion SPECT 1-2 years after HC onset showed contralateral BG hyperperfusion.

Conclusion: The mechanisms of BG hyperactivity and variable perfusion in patients with HC and ICA stenosis are complex and not yet well understood. We observed relative BG hyperactivity in all HC patients ipsilateral to ICA stenosis, while perfusion in these hyperactive BG was either decreased or increased. We believe that the timing of perfusion imaging plays a role. This would corroborate the hypothesis that hypoperfusion develops in the acute phase of transient HC, while hyperperfusion develops in the recovery phase as a compensatory mechanism [10].

References: [1] Cardoso F, Seppi K, Mair KJ, Wenning GK, Poewe W. Seminar on choreas. Lancet Neurol. 2006; 5 (7): 589–602. [2] Pareés I, Pujadas F, Hernández-Vara J, Lorenzo-Bosquet C, Cuberas G, Munuera J, Alvarez-Sabín J. Reversible hemichorea associated with extracranial carotid artery stenosis. J Neurol Sci. 2011; 300 (1–2): 185–6. [3] Morigaki R, Uno M, Suzue A, Nagahiro S. Hemichorea due to hemodynamic ischemia associated with extracranial carotid artery stenosis. Report of two cases. J Neurosurg. 2006; 105 (1): 142–7. [4] Noda K, Nakajima S, Sasaki F, Ito Y, Kawajiri S, Tomizawa Y, Hattori N, Yamamoto T, Okuma Y. Middle Cerebral Artery Occlusion Presenting as Upper Limb Monochorea. J Stroke Cerebrovasc Dis. 2015; 24 (10): e291–3. [5] Kim DW, Ko Y, Jang SH, Yoon SJ, Oh GS, Lee SJ, Yun DJ. Acute hemichorea as an unusual presentation of internal carotid artery stenosis. J Mov Disord. 2013; 6 (1): 17–20. [6] Noda K, Ishimoto R, Hattori N, Okuma Y, Yamamoto T. Hemichorea improvement following endarterectomy for internal carotid artery stenosis. J Neurol Sci. 2016; 371: 45–7. [7] Irioka T, Ayabe J, Mizusawa H. Hemichorea improved by extracranial-intracranial bypass surgery for middle cerebral artery occlusion. J Neurol. 2010; 257 (10): 1756–8. [8] Galea I, Norwood F, Phillips MJ, Shearman C, McMonagle P, Gibb WR. Pearls & Oy-sters: resolution of hemichorea following endarterectomy for severe carotid stenosis. Neurology. 2008; 71 (24): e80–2. [9] Savsek L, Andlovic A, Ozimic N, Jensterle L, Tomse P, Grmek M, Pretnar Oblak J, Pirtosek Z, Trost M, Kojovic M. Metabolic pattern of acute hemichorea associated with contralateral carotid stenosis [abstract]. Mov Disord. 2017; 32 (suppl 2). [10] Ehrlich DJ, Walker RH. Functional neuroimaging and chorea: a systematic review. J Clin Mov Disord. 2017 Jun 21; 4: 8.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/metabolic-activity-and-perfusion-of-basal-ganglia-in-patients-with-hemichorea-and-carotid-stenosis/