Department of Neurology, Sanggye Paik Hospital, Inje University College of Medicine, Seoul, Korea.
Mov Disord. 2010 Jul 30;25(10):1482-6. doi: 10.1002/mds.23130.
The aim of this study was to define the clinical characteristics of patients who developed movement disorders in association with moyamoya disease (MMD). Using PubMed and medical records of our hospital from 1985 to 2008, we searched for patients who developed movement disorders in association with MMD. This study included 38 patients described in previous studies and 4 patients found in the medical records. The onset of movement disorders was thought to be sudden. In 13 patients, the movement disorders were precipitated by hyperventilation or emotional stress. Twenty-seven of the 42 patients developed chorea, 4 patients developed dystonia, and 4 developed a mixture of both. The movement disorders of the remaining 7 patients were described as dyskinesia. A third of the 42 patients developed bilateral movement disorders, and their mean age was younger than that of those with unilateral movement disorders. In 37 of the 42 patients, brain imaging studies showed ischemic lesions, but the remaining 5 patients showed no parenchymal lesions. Cerebral perfusion studies showed hypoperfusion in the basal ganglia and in the cerebral cortical areas. Most patients improved whether they were treated or not. MMD must be included in the differential diagnosis of the sudden onset of dyskinesias, particularly chorea and focal dystonia. Even in patients with no parenchymal lesion in brain imaging studies, cerebral angiography and cerebral blood perfusion studies must be performed, if they develop a sudden onset or recurrent movement disorders preceded by emotional stress or hyperventilation.
本研究旨在明确与烟雾病（MMD）相关的运动障碍患者的临床特征。通过使用 PubMed 和我们医院 1985 年至 2008 年的病历记录，我们搜索了与 MMD 相关的运动障碍患者。本研究共纳入了之前研究中描述的 38 例患者和病历中发现的 4 例患者。运动障碍的发病被认为是突然的。在 13 例患者中，运动障碍是由过度通气或情绪应激诱发的。42 例患者中有 27 例出现舞蹈症，4 例出现肌张力障碍，4 例出现两者混合。其余 7 例患者的运动障碍被描述为运动障碍。42 例患者中有三分之一出现双侧运动障碍，且其平均年龄小于单侧运动障碍患者。在 42 例患者中，37 例脑影像学研究显示存在缺血性病灶，但其余 5 例患者未显示实质病变。脑灌注研究显示基底节和大脑皮质区域存在灌注不足。大多数患者无论是否接受治疗，病情都有所改善。当出现突发性运动障碍，特别是舞蹈症和局灶性肌张力障碍时，必须将 MMD 纳入鉴别诊断。即使在脑影像学研究中没有实质病变的患者，如果他们出现情绪应激或过度通气诱发的突发性或复发性运动障碍，也必须进行脑血管造影和脑血流灌注研究。
