Cerebral amyloid angiopathy-related inflammation (CAA-RI) is a rare but increasingly recognized subtype of CAA. CAA-RI consists of two subtypes: inflammatory cerebral amyloid angiopathy and amyloid β (Aβ)-related an...Cerebral amyloid angiopathy-related inflammation (CAA-RI) is a rare but increasingly recognized subtype of CAA. CAA-RI consists of two subtypes: inflammatory cerebral amyloid angiopathy and amyloid β (Aβ)-related angiitis. Acute or subacute onset of cognitive decline or behavioral changes is the most common symptom of CAA-RI. Rapid progressive dementia, headache, seizures, or focal neurological deficits, with patchy or confluent hyperintensity on T2 or fluid-attenuated inversion recovery sequences and evidence of strictly lobar microbleeds or cortical superficial siderosis on susceptibility-weighted imaging imply CAA-RI. The gold standard for diagnosis is autopsy or brain biopsy. However, biopsy is invasive;consequently, most clinically diagnosed cases have been based on clinical and radiological data. Other diagnostic indexes include the apolipoprotein E ε4 allele, Aβ and anti-Aβ antibodies in cerebral spinal fluid and amyloid positron emission tomography. Many diseases with similar clinical manifestations should be carefully ruled out. Immunosuppressive therapy is effective both during initial presentation and in relapses. The use of glucocorticoids and immunosuppressants improves prognosis. This article reviews the pathology and pathogenesis, clinical and imaging manifestations, diagnostic criteria, treatment, and prognosis of CAA-RI, and highlights unsolved problems in the existing research.展开更多
脑小血管病(cerebral small vessel disease,CSVD)是导致缺血性卒中和血管性痴呆的重要原因,给家庭和社会带来沉重负担。CSVD的预防和治疗一直是研究热点,但其发病机制目前尚不完全清楚。文章对CSVD的发病机制进行综述,包括慢性脑灌注...脑小血管病(cerebral small vessel disease,CSVD)是导致缺血性卒中和血管性痴呆的重要原因,给家庭和社会带来沉重负担。CSVD的预防和治疗一直是研究热点,但其发病机制目前尚不完全清楚。文章对CSVD的发病机制进行综述,包括慢性脑灌注不足、血脑屏障功能障碍、血管内皮功能障碍、组织间液回流障碍、炎症反应和遗传因素,以期为CSVD的及早干预和治疗提供更充分的理论依据。展开更多
基金National Key Research and Development Program of China(No.2016YFC1300500-505)。
文摘Cerebral amyloid angiopathy-related inflammation (CAA-RI) is a rare but increasingly recognized subtype of CAA. CAA-RI consists of two subtypes: inflammatory cerebral amyloid angiopathy and amyloid β (Aβ)-related angiitis. Acute or subacute onset of cognitive decline or behavioral changes is the most common symptom of CAA-RI. Rapid progressive dementia, headache, seizures, or focal neurological deficits, with patchy or confluent hyperintensity on T2 or fluid-attenuated inversion recovery sequences and evidence of strictly lobar microbleeds or cortical superficial siderosis on susceptibility-weighted imaging imply CAA-RI. The gold standard for diagnosis is autopsy or brain biopsy. However, biopsy is invasive;consequently, most clinically diagnosed cases have been based on clinical and radiological data. Other diagnostic indexes include the apolipoprotein E ε4 allele, Aβ and anti-Aβ antibodies in cerebral spinal fluid and amyloid positron emission tomography. Many diseases with similar clinical manifestations should be carefully ruled out. Immunosuppressive therapy is effective both during initial presentation and in relapses. The use of glucocorticoids and immunosuppressants improves prognosis. This article reviews the pathology and pathogenesis, clinical and imaging manifestations, diagnostic criteria, treatment, and prognosis of CAA-RI, and highlights unsolved problems in the existing research.
文摘脑小血管病(cerebral small vessel disease,CSVD)是导致缺血性卒中和血管性痴呆的重要原因,给家庭和社会带来沉重负担。CSVD的预防和治疗一直是研究热点,但其发病机制目前尚不完全清楚。文章对CSVD的发病机制进行综述,包括慢性脑灌注不足、血脑屏障功能障碍、血管内皮功能障碍、组织间液回流障碍、炎症反应和遗传因素,以期为CSVD的及早干预和治疗提供更充分的理论依据。
