Department of Cerebrovascular Surgery, Saitama Medical University International Medical Center, Hidaka, Japan.
Department of Cerebrovascular Surgery, Saitama Medical University International Medical Center, Hidaka, Japan.
Neurochirurgie. 2023 Jul;69(4):101449. doi: 10.1016/j.neuchi.2023.101449. Epub 2023 May 11.
Although hypoperfusion of the basal ganglia or the frontal subcortical matter is suspected, the pathology of chorea in moyamoya disease remains unclarified. Herein, we report a case of moyamoya disease presenting with hemichorea and evaluate pre- and postoperative perfusion using single photon emission computed tomography with N-isopropyl-p-I-iodoamphetamine (I-IMP SPECT).
An 18-year-old woman presented with choreic movement of her left limbs. Magnetic resonance imaging revealed an ivy sign, and I-IMP SPECT demonstrated decreased cerebral blood flow (CBF) and cerebral vascular reserve (CVR) values in the right hemisphere. The patient underwent direct and indirect revascularization surgery to improve cerebral hemodynamic impairment. The choreic movements entirely resolved immediately after surgery. Although CBF and CVR values in the ipsilateral hemisphere demonstrated by quantitative SPECT increased, these did not reach the normal values threshold.
Choreic movement in moyamoya disease may be related to cerebral hemodynamic impairment. Further studies are required to elucidate its pathophysiological mechanisms.
尽管基底节或额皮质下低灌注被怀疑与舞蹈病有关，但烟雾病的舞蹈病病理仍不清楚。本文报告 1 例以偏侧舞蹈病为表现的烟雾病患者，并采用单光子发射计算机断层扫描结合 N-异丙基-p-I-碘安非他命（I-IMP SPECT）评估其术前和术后灌注情况。
18 岁女性，出现左侧肢体舞蹈样运动。磁共振成像显示 Ivy 征，I-IMP SPECT 显示右侧半球脑血流（CBF）和脑血管储备（CVR）值降低。患者接受直接和间接血运重建手术以改善脑血流动力学障碍。术后舞蹈样运动完全消失。尽管定量 SPECT 显示同侧半球的 CBF 和 CVR 值增加，但未达到正常阈值。
烟雾病的舞蹈样运动可能与脑血流动力学障碍有关。需要进一步研究以阐明其病理生理机制。
