Department of Neurosurgery, Iwate Medical University, 2-1-1 Idai-dori, Yahaba, Iwate, 028-3695, Japan.
Institute for Biomedical Sciences, School of Medicine, Iwate Medical University, 2-1-1 Idai-dori, Yahaba, Iwate, 028-3695, Japan.
Neurosurg Rev. 2022 Apr;45(2):1553-1561. doi: 10.1007/s10143-021-01677-0. Epub 2021 Oct 23.
Angiographic disease progression reportedly develops in adult moyamoya disease (MMD). However, more than half of patients analyzed underwent revascularization surgery. The present supplementary analysis of a 5-year prospective cohort with follow-up using magnetic resonance angiography (MRA) and cerebral blood flow (CBF) measurements was to elucidate the incidence and clinical features of angiographic disease progression in adult patients receiving medical management alone for ischemic MMD. Sixty-eight patients without misery perfusion in the symptomatic cerebral hemispheres underwent MRA and CBF measurement using brain perfusion single-photon emission computed tomography at inclusion and at the end of the 5-year follow-up. When neurological symptoms recurred or newly developed during the 5-year follow-up period, additional MRA and CBF measurements were also performed at that time. All four patients with further ischemic events during the 5-year follow-up period exhibited angiographic disease progression on MRA at such events. Of the remaining 64 patients without further events during the 5-year follow-up period, four exhibited angiographic disease progression on MRA at the end of the 5-year follow-up. CBF was significantly lower at the time of further ischemic events or at the end of the 5-year follow-up than at inclusion in eight patients with angiographic disease progression (p = 0.0117). The incidence of angiographic disease progression was 12% for 5 years in medically treated adult patients with ischemic MMD without cerebral misery perfusion. Patients with further ischemic events always exhibited angiographic disease progression. Cerebral perfusion was reduced in patients with angiographic disease progression even when further ischemic events did not occur.
据报道，成人烟雾病（MMD）会出现血管造影疾病进展。然而，分析的患者中超过一半接受了血运重建手术。本研究对一项 5 年前瞻性队列进行了补充分析，该队列使用磁共振血管造影（MRA）和脑血流（CBF）测量进行随访，旨在阐明单独接受药物治疗的缺血性 MMD 成人患者的血管造影疾病进展的发生率和临床特征。68 例症状性大脑半球无低灌注的患者在纳入时和 5 年随访结束时接受脑灌注单光子发射计算机断层扫描的 MRA 和 CBF 测量。在 5 年随访期间，如果出现神经系统症状复发或新出现，此时也会进行额外的 MRA 和 CBF 测量。在 5 年随访期间发生进一步缺血事件的所有 4 例患者在这些事件中均显示 MRA 上存在血管造影疾病进展。在 5 年随访期间无进一步事件的 64 例患者中，4 例在 5 年随访结束时显示 MRA 上存在血管造影疾病进展。在出现进一步缺血事件或 5 年随访结束时，8 例存在血管造影疾病进展的患者的 CBF 明显低于纳入时（p = 0.0117）。在无大脑低灌注的缺血性 MMD 成人患者中，5 年内药物治疗的血管造影疾病进展发生率为 12%。有进一步缺血事件的患者总是表现出血管造影疾病进展。即使没有发生进一步的缺血事件，存在血管造影疾病进展的患者的脑灌注也会减少。
