Abstract：
This study aimed to explore the long-term course of posterior circulation changes and predictors in patients with moyamoya disease (MMD).
The authors retrospectively enrolled patients who were diagnosed with MMD and underwent encephaloduroarteriosynangiosis (EDAS) surgery at the authors\' department from December 2002 to September 2011. A comparative study between short-term (6-12 months) and long-term (≥ 9 years) follow-up angiography was conducted. The progression of lesions was defined from lower to higher stages of the posterior cerebral artery (PCA).
Eighty-eight patients who received indirect EDAS were enrolled in the study. The mean age at first surgery was 28.1 ± 15.0 years. Among these 88 patients with MMD, 39 (44.3%) exhibited transient ischemic attack and 27 (30.7%) exhibited infarction, comprising 5 with occipital lobe infarction, 14 (15.9%) with hemorrhagic symptoms, and 8 (9.1%) with atypical symptoms as the initial symptoms. Heterozygous mutations occurred significantly more frequently in the cases that presented with PCA involvement. During follow-up, stage progression of PCA was observed in 21 patients (28 hemispheres). At short-term follow-up, 21/176 (11.9%) hemispheres had progression of steno-occlusive lesions in the PCA. At long-term follow-up, 7 (4.0%) hemispheres had progression of steno-occlusive lesions in the PCA. At short-term follow-up, the progression of steno-occlusive lesions in the PCA was associated with progression of the internal carotid artery. Stage progression of PCA occurred significantly more frequently in the cases with PCA involvement on preoperative angiography. Nine strokes (10.2%) occurred in 88 patients during long-term follow-up. Four patients (4.5%) presented with ischemic stroke, including 2 with occipital lobe infarctions.
Progression of PCA stenosis is common in patients with MMD, even if the PCA is normal initially. Mutations of RNF213 p.R4810K may predict PCA involvement or progression. Follow-up of the PCA in MMD patients should be conducted, and timely surgical revascularization is needed.
The authors retrospectively enrolled patients who were diagnosed with MMD and underwent encephaloduroarteriosynangiosis (EDAS) surgery at the authors\' department from December 2002 to September 2011. A comparative study between short-term (6-12 months) and long-term (≥ 9 years) follow-up angiography was conducted. The progression of lesions was defined from lower to higher stages of the posterior cerebral artery (PCA).
Eighty-eight patients who received indirect EDAS were enrolled in the study. The mean age at first surgery was 28.1 ± 15.0 years. Among these 88 patients with MMD, 39 (44.3%) exhibited transient ischemic attack and 27 (30.7%) exhibited infarction, comprising 5 with occipital lobe infarction, 14 (15.9%) with hemorrhagic symptoms, and 8 (9.1%) with atypical symptoms as the initial symptoms. Heterozygous mutations occurred significantly more frequently in the cases that presented with PCA involvement. During follow-up, stage progression of PCA was observed in 21 patients (28 hemispheres). At short-term follow-up, 21/176 (11.9%) hemispheres had progression of steno-occlusive lesions in the PCA. At long-term follow-up, 7 (4.0%) hemispheres had progression of steno-occlusive lesions in the PCA. At short-term follow-up, the progression of steno-occlusive lesions in the PCA was associated with progression of the internal carotid artery. Stage progression of PCA occurred significantly more frequently in the cases with PCA involvement on preoperative angiography. Nine strokes (10.2%) occurred in 88 patients during long-term follow-up. Four patients (4.5%) presented with ischemic stroke, including 2 with occipital lobe infarctions.
Progression of PCA stenosis is common in patients with MMD, even if the PCA is normal initially. Mutations of RNF213 p.R4810K may predict PCA involvement or progression. Follow-up of the PCA in MMD patients should be conducted, and timely surgical revascularization is needed.
摘要：
目的：本研究旨在探讨烟雾病（MMD）患者后循环变化的长期过程和预测因素。
方法：作者回顾性地纳入了2002年12月至2011年9月在作者部门诊断为MMD并接受了脑动脉共病（EDAS）手术的患者。进行了短期（6-12个月）和长期（≥9年）随访血管造影的比较研究。病变的进展是从大脑后动脉（PCA）的较低阶段到较高阶段。
结果：88名接受间接EDAS的患者被纳入研究。首次手术的平均年龄为28.1±15.0岁。在这88例MMD患者中，39例(44.3%)出现短暂性脑缺血发作,27例(30.7%)出现梗死,包括5例枕叶梗塞，14例（15.9%）有出血症状，8例（9.1％）以非典型症状为初始症状。在涉及PCA的病例中，杂合突变的发生频率明显更高。随访期间,在21例患者（28个半球）中观察到PCA的分期进展。在短期随访中，21/176（11.9％）半球在PCA中出现狭窄闭塞性病变。在长期随访中，在PCA中，有7个（4.0％）半球的狭窄闭塞性病变进展。在短期随访中，PCA中狭窄闭塞性病变的进展与颈内动脉的进展相关.在术前血管造影术中涉及PCA的病例中，PCA的分期进展明显更频繁。在长期随访中，88例患者发生了9次中风（10.2％）。4例患者（4.5%）出现缺血性卒中，包括2例枕叶梗塞。
结论：PCA狭窄的进展在MMD患者中很常见，即使PCA最初是正常的。RNF213p.R4810K的突变可以预测PCA的参与或进展。应该对MMD患者的PCA进行随访，需要及时手术血运重建。
方法：作者回顾性地纳入了2002年12月至2011年9月在作者部门诊断为MMD并接受了脑动脉共病（EDAS）手术的患者。进行了短期（6-12个月）和长期（≥9年）随访血管造影的比较研究。病变的进展是从大脑后动脉（PCA）的较低阶段到较高阶段。
结果：88名接受间接EDAS的患者被纳入研究。首次手术的平均年龄为28.1±15.0岁。在这88例MMD患者中，39例(44.3%)出现短暂性脑缺血发作,27例(30.7%)出现梗死,包括5例枕叶梗塞，14例（15.9%）有出血症状，8例（9.1％）以非典型症状为初始症状。在涉及PCA的病例中，杂合突变的发生频率明显更高。随访期间,在21例患者（28个半球）中观察到PCA的分期进展。在短期随访中，21/176（11.9％）半球在PCA中出现狭窄闭塞性病变。在长期随访中，在PCA中，有7个（4.0％）半球的狭窄闭塞性病变进展。在短期随访中，PCA中狭窄闭塞性病变的进展与颈内动脉的进展相关.在术前血管造影术中涉及PCA的病例中，PCA的分期进展明显更频繁。在长期随访中，88例患者发生了9次中风（10.2％）。4例患者（4.5%）出现缺血性卒中，包括2例枕叶梗塞。
结论：PCA狭窄的进展在MMD患者中很常见，即使PCA最初是正常的。RNF213p.R4810K的突变可以预测PCA的参与或进展。应该对MMD患者的PCA进行随访，需要及时手术血运重建。
