中国伴有皮质下梗死和白质脑病的常染色体显性遗传性脑动脉病临床诊疗专家共识(2022版)  

Chinese expert consensus on the diagnosis and management of cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy 2022

作　　者：中华医学会神经病学分会 中华医学会神经病学分会脑血管病学组 朱以诚[2] 彭斌[2] 王拥军[3] 姚明[4] Chinese Society of Neurology;Chinese Stroke Society;Zhu Yicheng;Peng Bin;Wang Yongjun(不详;Department of Neurology,Peking Union Medical College Hospital,Peking Union Medical College,Chinese Academy of Medical Sciences,Beijing 100730,China;Department of Neurology,Beijing Tiantan Hospital,Capital Medical University,Beijing 100070,China)

机构地区：[1]不详 [2]中国医学科学院北京协和医院神经科,北京100730 [3]首都医科大学附属北京天坛医院神经内科,国家神经系统疾病临床医学研究中心,北京100070 [4]中国医学科学院北京协和医院

出　　处：《中华神经科杂志》2024年第5期419-436,共18页Chinese Journal of Neurology

摘　　要：伴有皮质下梗死和白质脑病的常染色体显性遗传性脑动脉病(CADASIL)是一种罕见的脑小血管病。为进一步提高临床医生对CADASIL的临床诊疗水平,促进临床实践的标准化,中华医学会神经病学分会脑血管病学组结合国内外相关领域研究进展,特制订《中国伴有皮质下梗死和白质脑病的常染色体显性遗传性脑动脉病临床诊疗专家共识(2022版)》。本共识基于当前可获得的国内外最佳证据,采用证据质量和推荐分级的评估、制订与评价系统,充分考虑患者的意愿和价值观,综合衡量安全性、有效性、可行性、可及性和成本-效益等因素后形成推荐意见,回答了11个CADASIL临床诊断、治疗、预后等方面的重要临床问题,以期指导临床医生规范诊断与治疗CADASIL。Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy(CADASIL)is a rare cerebral small vessel disease.In order to provide up-to-date recommendations for the clinical diagnosis and treatment of CADASIL,and thus further improve the standardization of clinical practice,the Chinese expert consensus on the diagnosis and management of CADASIL 2022 is approved by the Chinese Society of Neurology,Chinese Stroke Society.This expert consensus adopts the grading of recommendations assessment,development and evaluation system,fully considering the wishes and values of patients,and comprehensively weighing safety,effectiveness,feasibility,accessibility,cost-effectiveness and other factors to form corresponding recommendations for 11 clinical issues regrading CADASIL diagnosis,treatment and prognosis,hoping the formulation of this expert consensus will help to further standardize the diagnosis and treatment of CADASIL in China.

关 键 词：CADASIL 诊断 治疗 共识 

分 类 号：R743[医药卫生—神经病学与精神病学]