Kinboshi Masato, Inoue Manabu, Kojima Yasuhiro, Nakagawa Tomokazu, Kanda Masutaro, Shibasaki Hiroshi
Department of Neurology, Ijinkai Takeda General Hospital.
Rinsho Shinkeigaku. 2012;52(1):25-9. doi: 10.5692/clinicalneurol.52.25.
A 61-year-old Japanese female was admitted with sudden onset of choreic movements of the right extremities. MRI demonstrated no abnormality suggestive of acute infarcts. Cerebral angiography revealed high-grade stenosis of bilateral middle cerebral arteries at the origin and abnormal vascular network compatible with moyamoya disease. Administration of low-dose haloperidol rapidly resolved the choreic movements. SPECT obtained one month after the clinical onset demonstrated increase of the regional cerebral blood flow (rCBF) in the left basal ganglia. Moyamoya disease presenting chorea as its initial symptom was only infrequently reported in the elderly. In the present case, increased rCBF in the basal ganglia and remarkable effect of a dopamine D2 blocker suggest functional abnormality of the corresponding striatum as an underlying cause of hemichorea.
一名61岁的日本女性因突发右上肢舞蹈样动作入院。磁共振成像（MRI）未显示提示急性梗死的异常。脑血管造影显示双侧大脑中动脉起始处高度狭窄，以及与烟雾病相符的异常血管网。给予小剂量氟哌啶醇后，舞蹈样动作迅速缓解。临床发病后1个月进行的单光子发射计算机断层扫描（SPECT）显示左侧基底节区局部脑血流量（rCBF）增加。以舞蹈症为首发症状的烟雾病在老年人中报道较少。在本病例中，基底节区rCBF增加以及多巴胺D2受体阻滞剂的显著疗效提示相应纹状体的功能异常是偏侧舞蹈症的潜在病因。
