脑增生性血管病的研究进展

Research progress of cerebral proliferative angiopathy

脑增生性血管病(CPA)是一种极其罕见的缺血性血管畸形,其形态复杂,起源不明。CPA的自然演变不同于脑动静脉畸形,临床表现较为复杂,多数患者以癫痫、头痛和进行性神经功能缺损为主要症状,血管影像学特征是其确诊依据。CPA自然史表明该病不稳定,可能在某一时间点发展从而导致残疾甚至死亡。认识CPA有利于积极开展手术或其他干预,从而防止对正常脑组织造成的永久性损伤。本文拟对CPA流行病学、发病机制、临床表现、诊断与治疗等方面进展进行综述。

Cerebral proliferative angiopathy(CPA)is an extremely rare ischemic vascular malformation.Its morphology is complex and its origin is unknown.The natural evolution of CPA is different from arteriovenous malformations,and the clinical manifestations of arteriovenous malformations are different.Most patients have epilepsy,headache and progressive neurological deficits as their main symptoms.Its natural history indicates that the disease is unstable and may develop at some point in time.It is therefore important to recognize that this rare disease facilitates active surgery or other interventions to prevent permanent damage to normal brain tissue.Therefore,in order to better understand CPA,this article intends to review the progress of CPA epidemiology,pathogenesis,clinical manifestations,diagnosis and treatment.