Elderly-Onset Moyamoya Disease Presenting with Hemichorea Associated with Basal Ganglia Hyperperfusion: Symptomatic Improvement after Revascularization Surgery.

IF 0.4 Q4 CLINICAL NEUROLOGY

Case Reports in Neurology Pub Date : 2026-06-29 eCollection Date: 2026-01-01 DOI:10.1159/000552937

Sho Takata, Tomoya Kamide, Takeshi Kidoguchi, Taishi Tsutsui, Kouichi Misaki, Mitsutoshi Nakada

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引用次数: 0

Abstract

Introduction: Chorea has been reported in pediatric moyamoya disease, which is commonly associated with ischemia of the basal ganglia. However, elderly patients are rare, and the underlying mechanisms remain unclear.

Case presentation: A 69-year-old woman presented with a 6-month history of involuntary hemichorea affecting her left upper and lower limbs. Magnetic resonance imaging showed no acute infarction but demonstrated right middle cerebral artery (MCA) occlusion. Digital subtraction angiography revealed moyamoya collateralization. The ¹²³I-iodoamphetamine single-photon emission computed tomography scan revealed increased perfusion of basal ganglia and impaired cerebrovascular reserve in the right MCA territory. The patient underwent right superficial temporal artery-MCA bypass combined with encephalo-duro-myo-synangiosis. Postoperatively, basal ganglia hyperperfusion persisted, but cerebrovascular reserve improved. Her hemichorea markedly subsided, and she was discharged without neurological deficits. This is one of the oldest reported cases of moyamoya syndrome presenting with hemichorea.

Conclusion: Our findings suggest that chorea associated with moyamoya disease may result not only from decreased, but also from imbalanced basal ganglia perfusion. Revascularization surgery may help restore this balance and alleviate symptoms, even in elderly patients.

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老年性烟雾病伴基底神经节高灌注的血漏：血运重建术后症状改善

导读：小儿烟雾病中有舞蹈病的报道，这通常与基底神经节缺血有关。然而，老年患者很少，潜在的机制尚不清楚。病例介绍：一名69岁女性，有6个月的不自主血凝史，影响其左上肢和下肢。磁共振显示无急性梗死，但显示右大脑中动脉闭塞。数字减影血管造影显示烟雾状侧支。123i -碘安非他命单光子发射计算机断层扫描显示基底节区灌注增加，右MCA区域脑血管储备受损。患者行右侧颞浅动脉- mca搭桥合并脑-硬膜-肌-synangiosis。术后基底节区持续高灌注，但脑血管储备改善。她的血瘀明显消退，出院时无神经功能缺损。这是一个最古老的烟雾综合征报告的情况下，表现为偏血。结论：我们的研究结果表明，与烟雾病相关的舞蹈病可能不仅是由基底神经节灌注减少引起的，而且是由基底神经节灌注不平衡引起的。血管重建术可以帮助恢复这种平衡并减轻症状，即使在老年患者中也是如此。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Case Reports in Neurology Medicine-Neurology (clinical)

CiteScore

1.50

自引率

0.00%

发文量

审稿时长

14 weeks

期刊介绍： This new peer-reviewed online-only journal publishes original case reports covering the entire spectrum of neurology. Clinicians and researchers are given a tool to disseminate their personal experience to a wider public as well as to review interesting cases encountered by colleagues all over the world. To complement the contributions supplementary material is welcomed. The reports are searchable according to the key words supplied by the authors; it will thus be possible to search across the entire growing collection of case reports with universally used terms, further facilitating the retrieval of specific information. Following the open access principle, the entire contents can be retrieved at no charge, guaranteeing easy access to this valuable source of anecdotal information at all times.