[{"data":1,"prerenderedAt":-1},["ShallowReactive",2],{"detail-sidebar-cat-0-en-105":3,"doc-seo-436111-105":59,"doc-detail-436111-en":130},{"code":4,"msg":5,"data":6},0,"success",[7,13,18,23,28,33,38,43,48,51,55],{"id":8,"doc_module":4,"doc_module_name":9,"category_name":10,"show_sort_weight":11,"slug":12},1,"Document","Story & Novel",90,"story-novel",{"id":14,"doc_module":4,"doc_module_name":9,"category_name":15,"show_sort_weight":16,"slug":17},2,"Literature",80,"literature",{"id":19,"doc_module":4,"doc_module_name":9,"category_name":20,"show_sort_weight":21,"slug":22},4,"Exam",70,"exam",{"id":24,"doc_module":4,"doc_module_name":9,"category_name":25,"show_sort_weight":26,"slug":27},5,"Comic",60,"comic",{"id":29,"doc_module":4,"doc_module_name":9,"category_name":30,"show_sort_weight":31,"slug":32},6,"Technology",50,"technology",{"id":34,"doc_module":4,"doc_module_name":9,"category_name":35,"show_sort_weight":36,"slug":37},7,"Healthcare",40,"healthcare",{"id":39,"doc_module":4,"doc_module_name":9,"category_name":40,"show_sort_weight":41,"slug":42},8,"Research & Report",30,"research-report",{"id":44,"doc_module":4,"doc_module_name":9,"category_name":45,"show_sort_weight":46,"slug":47},9,"Religion & Spirituality",20,"religion-spirituality",{"id":46,"doc_module":4,"doc_module_name":9,"category_name":49,"show_sort_weight":46,"slug":50},"World Cup","world-cup",{"id":52,"doc_module":4,"doc_module_name":9,"category_name":53,"show_sort_weight":52,"slug":54},10,"Lifestyle","lifestyle",{"id":56,"doc_module":4,"doc_module_name":9,"category_name":57,"show_sort_weight":24,"slug":58},19,"General","general",{"code":4,"msg":60,"data":61},"ok",{"site_id":62,"language":63,"slug":64,"title":65,"keywords":66,"description":67,"schema_data":68,"social_meta":123,"head_meta":125,"extra_data":127,"updated_unix":129},105,"en","intracerebral-hemorrhage-in-cadasil-review-article","Intracerebral hemorrhage in CADASIL - Review Article","","Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is the most common hereditary cerebral small vessel disease driven by NOTCH3 gene mutations. This review focuses on intracerebral hemorrhage (ICH) as an increasingly recognized CADASIL manifestation, typically discussed alongside ischemic stroke and vascular dementia. Reported ICH prevalence ranges from 0.5% to 33.3%, varying mainly by ethnicity, with East Asian NOTCH3 mutations linked to higher hemorrhagic risk. Hypertension, cerebral microbleeds and microbleed load, and thalamus/basal ganglia localization are associated with greater ICH risk. CADASIL patients with ICH show higher morbidity, mortality, and recurrent stroke risk; management prioritizes rigorous blood pressure control and cautious antithrombotic use.",{"@graph":69,"@context":122},[70,84,105],{"@type":71,"itemListElement":72},"BreadcrumbList",[73,77,79,82],{"item":74,"name":75,"@type":76,"position":8},"https://docshare.wps.com","Home","ListItem",{"item":78,"name":9,"@type":76,"position":14},"https://docshare.wps.com/document/",{"item":80,"name":35,"@type":76,"position":81},"https://docshare.wps.com/document/healthcare/",3,{"item":83,"name":65,"@type":76,"position":19},"https://docshare.wps.com/document/intracerebral-hemorrhage-in-cadasil-review-article/436111/",{"url":83,"name":65,"@type":85,"image":86,"author":91,"headline":65,"publisher":94,"fileFormat":97,"inLanguage":63,"description":67,"dateModified":98,"datePublished":99,"encodingFormat":97,"isAccessibleForFree":100,"interactionStatistic":101},"DigitalDocument",{"url":87,"@type":88,"width":89,"height":90},"https://docshare.wps.com/thumbnails/intracerebral-hemorrhage-in-cadasil-review-article/436111.png","ImageObject",300,407,{"name":92,"@type":93},"Asher","Person",{"url":74,"name":95,"@type":96},"DocShare","Organization","application/pdf","2026-11-06","2026-09-29",true,{"@type":102,"interactionType":103,"userInteractionCount":44},"InteractionCounter",{"@type":104},"ViewAction",{"@type":106,"mainEntity":107},"FAQPage",[108,114,118],{"name":109,"@type":110,"acceptedAnswer":111},"What disease does CADASIL describe, and what genetic cause is most common?","Question",{"text":112,"@type":113},"CADASIL is a hereditary cerebral small vessel disease caused by mutations in the NOTCH3 gene, typically cysteine-altering variants. These mutations contribute to progressive degeneration of small brain vessels.","Answer",{"name":115,"@type":110,"acceptedAnswer":116},"How common is intracerebral hemorrhage (ICH) among CADASIL patients, and why does it vary?",{"text":117,"@type":113},"Reported ICH prevalence in CADASIL ranges from 0.5% to 33.3%. The variability is mainly influenced by ethnicity.",{"name":119,"@type":110,"acceptedAnswer":120},"Which factors in CADASIL are associated with higher ICH risk and worse outcomes?",{"text":121,"@type":113},"Hypertension, cerebral microbleeds (especially high CMB load), and ICH localization in the thalamus and basal ganglia are associated with increased ICH risk. Patients with ICH experience greater morbidity, higher mortality, and increased annual stroke recurrence risk.","https://schema.org",{"og:url":83,"og:type":124,"og:title":65,"og:site_name":95,"og:description":67},"article",{"robots":126,"canonical":83},"index,follow",{"doc_id":128,"site_id":62},436111,1790882791,{"code":4,"msg":5,"data":131},{"doc_id":128,"user_id":132,"nickname":92,"user_avatar":133,"doc_module":4,"category_id":34,"category_name":35,"doc_title":65,"doc_description":67,"doc_content":134,"file_id":135,"file_url":136,"file_type":137,"file_size":138,"view_count":44,"is_deleted":4,"is_public":8,"is_downloadable":8,"audit_status":8,"page_count":34,"language":139,"language_code":63,"site_id":62,"html_lang":63,"table_of_contents":140,"faqs":141,"seo_title":142,"seo_description":67,"update_tm":143,"read_time":144,"preview_page_count":4},687197207639,"https://ap-avatar.wpscdn.com/davatar_a8503ba1806abce46bf441b54a3ca4cd","J Chin MedAssoc  \nReview ARticle  \nIntracerebral hemorrhage in CADASIL  \nShao-Lun Hsua,b, Yi-Chu Liaoc,d,e, Chih-Ping Chungc,d,e, Masafumi Iharaf, Jay Chol Choig, Sung-Chun Tangh, Yi-Chung Leea,c,d,e,*  \naInstitute of Clinical Medicine, National Yang Ming Chiao Tung University, Taipei, Taiwan, ROC; bDepartment of Neurology, Fu Jen Catholic University Hospital, New Taipei City, Taiwan, ROC; cDepartment of Neurology, Taipei Veterans General Hospital, Taipei, Taiwan, ROC; dDepartment of Neurology, National Yang Ming Chiao Tung University School of Medicine, Taipei, Taiwan, ROC; eBrain Research Center, National Yang Ming Chiao Tung University, Taipei, Taiwan, ROC; fDepartment of Neurology, National Cerebral and Cardiovascular Center, Suita, Japan; gDepartment of Neurology, Jeju National University College of Medicine, Jeju National University Hospital, Jeju, Korea; hDepartment of Neurology, National Taiwan University Hospital, Taipei, Taiwan, ROC  \nAbstract  \nCerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is the most common hereditary cerebral small vessel disease caused by mutations in the NOTCH3 gene. This review highlights the increasing recognition of intracerebral hemorrhage (ICH) as a significant manifestation of CADASIL, often predominantly characterized by ischemic strokes and vascular dementia. Recent studies indicate that the prevalence of ICH in CADASIL patients ranges from 0.5% to 33.3%, the variability of which is mainly influenced by ethnicity. In East Asian cohorts, specific NOTCH3 mutations like p. R544Cand p. R75P are more prevalent and have been associated with a higher rate of ICH, suggesting a link between these mutations and the hemorrhagic risk. Hypertension, as with other etiologies of ICH, is a key risk factor in CADASIL patients, with 40% to 90% of those who experience ICH also having a history of hypertension. The presence of cerebral microbleeds (CMBs) and a high CMB load are strongly associated with an increased risk of ICH. Neuroimaging studies show that ICH in CADASIL patients predominantly occurs in the thalamus and basal ganglia. There is a notable spatial correlation between CMBs and subsequent ICH, suggesting that CMBs may serve as markers of microangiopathy in regions prone to vascular injury. CADASIL patients with ICH experience greater morbidity, higher mortality rates, and increased annual stroke recurrence risk compared with those with ischemic events. In summary, this review emphasizes the need for tailored management strategies that prioritize rigorous blood pressure control and the careful use of antithrombotic agents in CADASIL patients with a high burden of CMBs. By advancing our understanding of ICHin CADASIL, we aim to improve diagnostic and therapeutic approaches, ultimately enhancing patient outcomes and quality of life in this high-risk population.  \nKeywords: CADASIL; Cerebral small vessel disease; Intracerebral hemorrhage; NOTCH3 gene  \n[www.ejcma.org](www.ejcma.org) 189  \n\u003Cz10.1097/JCMA.0000000000001206>  \nHsu et al. J Chin MedAssoc  \n1. INTRODUCTION  \nCerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is the most prevalent hereditary cerebral small vessel disease (CSVD) .1,2 It is usually caused by cysteine-altering variants in the NOTCH3 gene, which lead to the accumulation of granular osmiophilic material in the basement membrane of vascular smooth muscle cells and pericytes, contributing to the progressive degeneration of small vessels in the brain.3,4 This vascular pathology manifests as a variety of neurological symptoms, including recurrent ischemic strokes, cognitive declines of varying severity, psychiatric disturbances, and migraine with aura.5 Magnetic resonance imaging (MRI) studies typically reveal diffuse white matter hyperintensities (WMHs), lacunes, cerebral microbleeds (CMBs), and brain atrophy, which reflect the underlying small vessel pathology associated wit","cbCaif0ey7iohKAC","https://ap.wps.com/l/cbCaif0ey7iohKAC","pdf",887584,"English","# Introduction\n# The prevalence of ICH in CADASIL","[{\"question\":\"What disease does CADASIL describe, and what genetic cause is most common?\",\"answer\":\"CADASIL is a hereditary cerebral small vessel disease caused by mutations in the NOTCH3 gene, typically cysteine-altering variants. These mutations contribute to progressive degeneration of small brain vessels.\"},{\"question\":\"How common is intracerebral hemorrhage (ICH) among CADASIL patients, and why does it vary?\",\"answer\":\"Reported ICH prevalence in CADASIL ranges from 0.5% to 33.3%. The variability is mainly influenced by ethnicity.\"},{\"question\":\"Which factors in CADASIL are associated with higher ICH risk and worse outcomes?\",\"answer\":\"Hypertension, cerebral microbleeds (especially high CMB load), and ICH localization in the thalamus and basal ganglia are associated with increased ICH risk. Patients with ICH experience greater morbidity, higher mortality, and increased annual stroke recurrence risk.\"}]","Intracerebral hemorrhage in CADASIL - Review Article | PDF",1790676855,18]