缺血性增殖型脑血管病2例报道及回顾性分析

Ischemic Cerebral Proliferative Angiopathy: Two Cases Report with Retrospective Study

目的报道2例增殖型脑血管病(CPA)病例,探讨CPA的临床表现、血流动力学特点、诊断和鉴别诊断要点以及治疗方式。方法回顾性分析2例CPA患者的病史、体格检查、辅助检查、治疗方式及随访结果,结合文献进行分析讨论。结果 2例患者均有不同程度的脑缺血症状,并经DSA诊断明确。2例患者局部脑畸形血管网及周围脑组织在单光子发射计算机断层成像术(SPECT)脑血流灌注中均显示低血流灌注信号,但同时在MRI脑血流灌注图像上显示局部脑血流量增加,呈现出高血流量低灌注状态。其中1例短暂性脑缺血发作症状较明显的患儿经脑硬脑膜-颞肌-血管融合术(EDMS)治疗后症状缓解明显,随访DSA示部分畸形血管网消失,来自颈外动脉的重建血流向颅内代偿良好;复查SPECT示局部灌注改善明显,且随访MRI灌注示畸形血管网内血流量下降。结论 CPA可因"盗血"等机制而导致局部脑缺血,EDMS治疗对于缺血明显的CPA患者是一种合理有效的方式。

Aim To investigate cerebral proliferative angiopathy＇s （CPA） clinical presentation, hemodynamic characteristics and its treatment method. Methods The history, physical examination, clinical exams, treatment and follow-up results of 2 cases were retrospectively analyzed, and related literatures were reviewed. Results Both 2 patients presented ischemic symptoms in varying degrees, and were diagnosed by digital subtraction angiography （DSA）. Preoperative single photon emission computed tomography （SPECT） demonstrated hypoperfusion signal at abnormal vascular network and surrounding brain tissue in both 2 cases. However, magnetic resonance （MR） perfusion weighted imaging （PWI） showed both renal cerebral flow （rCBF） of the area increased significantly. One pediatric patient with more obvious TIA symptoms accepted encephalo-duro-myo-synangiosis （EDMS）, and the clinical symptom relieved significantly at the follow-up period. Follow-up DSA demonstrated partial vascular malformation disappeared, while reconstructed blood flow from external carotid artery formed a well compensation of these areas. In addition, follow-up SPECT revealed perfusion of the area improved significantly, and follow- tip PW1 showed rCBF decreased apparently in abnormal vascular networks. Conclusion CPA might lead to brain ischemia by ＂blood steal phenomenon＂ , and EDMS may be a reasonable and effective treatment for CPA patients with severe brain ischemia.