{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,15]],"date-time":"2026-05-15T10:53:59Z","timestamp":1778842439397,"version":"3.51.4"},"reference-count":36,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"13","content-domain":{"domain":["www.ahajournals.org"],"crossmark-restriction":true},"short-container-title":["JAHA"],"published-print":{"date-parts":[[2020,7,7]]},"abstract":"<jats:sec xml:lang=\"en\">\n            <jats:title>Background<\/jats:title>\n            <jats:p xml:lang=\"en\">\n              Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy, caused by\n              <jats:italic>\n                <jats:styled-content style=\"fixed-case\">NOTCH<\/jats:styled-content>\n                3\n              <\/jats:italic>\n              mutations, is characterized by recurrent ischemic strokes and progressive cognitive decline. It remains unclear whether cerebral microbleeds (\n              <jats:styled-content style=\"fixed-case\">CMB<\/jats:styled-content>\n              s) can serve as a surrogate marker for disease progression in cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy. We aimed to investigate the\n              <jats:styled-content style=\"fixed-case\">CMB<\/jats:styled-content>\n              burdens in\n              <jats:italic>\n                <jats:styled-content style=\"fixed-case\">NOTCH<\/jats:styled-content>\n                3\n              <\/jats:italic>\n              mutation carriers at different disease stages and test their associations with cognitive performance.\n            <\/jats:p>\n          <\/jats:sec>\n          <jats:sec xml:lang=\"en\">\n            <jats:title>Methods and Results<\/jats:title>\n            <jats:p xml:lang=\"en\">\n              Forty\u2010nine individuals carrying\n              <jats:italic>\n                <jats:styled-content style=\"fixed-case\">NOTCH<\/jats:styled-content>\n                3\n              <\/jats:italic>\n              cysteine\u2010altering mutations received brain magnetic resonance imaging with T1\u2010weighted and susceptibility\u2010weighted images. Whole brain images were segmented into 14 regions using Statistical Parametric Mapping and FreeSurfer software, and semiautomatic methods were used to locate and quantify the number and volume of\n              <jats:styled-content style=\"fixed-case\">CMB<\/jats:styled-content>\n              s. In our study participants, the median of\n              <jats:styled-content style=\"fixed-case\">CMB<\/jats:styled-content>\n              counts was 13, with a wide individual variation (range, 0\u2013286).\n              <jats:styled-content style=\"fixed-case\">CMB<\/jats:styled-content>\n              s were most frequently present in thalamus, followed by temporal lobe. In the whole brain, the\n              <jats:styled-content style=\"fixed-case\">CMB<\/jats:styled-content>\n              counts and\n              <jats:styled-content style=\"fixed-case\">CMB<\/jats:styled-content>\n              volume ratios (ie,\n              <jats:styled-content style=\"fixed-case\">CMB<\/jats:styled-content>\n              volume divided by the volume of corresponding brain region) gradually increased as the disease advanced.\n              <jats:styled-content style=\"fixed-case\">CMB<\/jats:styled-content>\n              counts in the thalamus and temporal and frontal lobes increased more rapidly than other brain regions as disease progressed. There were significant associations between Mini\u2010Mental State Examination scores and\n              <jats:styled-content style=\"fixed-case\">CMB<\/jats:styled-content>\n              counts in the frontal lobe, temporal lobe, and pons.\n            <\/jats:p>\n          <\/jats:sec>\n          <jats:sec xml:lang=\"en\">\n            <jats:title>Conclusions<\/jats:title>\n            <jats:p xml:lang=\"en\">\n              <jats:styled-content style=\"fixed-case\">CMB<\/jats:styled-content>\n              s may have an influential role in the clinical manifestations of cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy.\n              <jats:styled-content style=\"fixed-case\">CMB<\/jats:styled-content>\n              burdens and their distribution in different brain regions may be capable to serve as a disease marker for monitoring the disease severity of cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy.\n            <\/jats:p>\n          <\/jats:sec>","DOI":"10.1161\/jaha.120.016233","type":"journal-article","created":{"date-parts":[[2020,6,17]],"date-time":"2020-06-17T09:34:37Z","timestamp":1592386477000},"update-policy":"https:\/\/doi.org\/10.1161\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":27,"title":["Cerebral Microbleed Burdens in Specific Brain Regions Are Associated With Disease Severity of Cerebral Autosomal Dominant Arteriopathy With Subcortical Infarcts and Leukoencephalopathy"],"prefix":"10.1161","volume":"9","author":[{"given":"Chih\u2010Ping","family":"Chung","sequence":"first","affiliation":[{"name":"Department of Neurology Neurological Institute Taipei Veterans General Hospital Taipei Taiwan"},{"name":"School of Medicine National Yang\u2010Ming University Taipei Taiwan"},{"name":"Brain Research Center National Yang\u2010Ming University Taipei 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