{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,17]],"date-time":"2026-06-17T16:13:59Z","timestamp":1781712839365,"version":"3.54.5"},"reference-count":15,"publisher":"Wiley","issue":"12","license":[{"start":{"date-parts":[[2020,10,5]],"date-time":"2020-10-05T00:00:00Z","timestamp":1601856000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Euro J of Neurology"],"published-print":{"date-parts":[[2020,12]]},"abstract":"<jats:sec><jats:title>Background and purpose<\/jats:title><jats:p>Investigating mutation carriers with Dutch\u2010type hereditary (D\u2010) cerebral amyloid angiopathy (CAA), offers the possibility to identify markers in pre\u2010 and symptomatic stages of CAA. Optical coherence tomography (OCT) has shown potential to detect retinal changes in several neurodegenerative diseases. The aim of the present exploratory study was to investigate thinning of retinal layers as a possible (early) biomarker in D\u2010CAA mutation carriers.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Twenty\u2010one D\u2010CAA mutation carriers (<jats:italic>n<\/jats:italic>\u00a0=\u00a08 presymptomatic, <jats:italic>n<\/jats:italic>\u00a0=\u00a013 symptomatic, median age 50\u00a0years) and nine controls (median age 53\u00a0years) were scanned using spectral\u2010domain OCT. Symptomatic mutation carriers were defined as having a history of \u22651 symptomatic intracerebral hemorrhage. D\u2010CAA mutation carriers and controls were recruited from our D\u2010CAA cohort and a healthy control cohort. Total peripapillary retinal nerve fiber layer (pRNFL) thickness, six regions of pRNFL, total macular volume (TMV), and individual macular region thickness were measured and analysed, adjusted for age.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>The overall median (interquartile range) thickness of pRNFL was lower in symptomatic, but not presymptomatic D\u2010CAA mutation carriers compared with controls [91\u00a0(86\u201395) \u00b5m vs. 99\u00a0(87\u2013108) \u00b5m; <jats:italic>P<\/jats:italic>\u00a0=\u00a00.006]. Both presymptomatic [111 (93\u2013122) \u00b5m vs. 131 (123\u2013143) \u00b5m; <jats:italic>P<\/jats:italic>\u00a0&lt;\u00a00.001] and symptomatic carriers [119\u00a0(95\u2013128) \u00b5m vs. 131\u00a0(123\u2013143) \u00b5m; <jats:italic>P<\/jats:italic>\u00a0=\u00a00.034] had a thinner temporal\u2010superior quadrant of the pRNFL versus controls. TMV or individual macular layer thickness did not differ between carriers and controls.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>Thinning of the retinal nerve fiber layer may be a candidate marker of disease in hereditary CAA. Further studies are needed to determine whether retinal thinning is present in sporadic CAA and estimate its value as a marker for disease progression.<\/jats:p><\/jats:sec>","DOI":"10.1111\/ene.14507","type":"journal-article","created":{"date-parts":[[2020,9,14]],"date-time":"2020-09-14T08:05:49Z","timestamp":1600070749000},"page":"2635-2640","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":8,"title":["Optical coherence tomography detects retinal changes in hereditary cerebral amyloid angiopathy"],"prefix":"10.1111","volume":"27","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-8301-6846","authenticated-orcid":false,"given":"E. S.","family":"van Etten","sequence":"first","affiliation":[{"name":"Department of Neurology Leiden University Medical Center Leiden The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"I.","family":"de Boer","sequence":"additional","affiliation":[{"name":"Department of Neurology Leiden University Medical Center Leiden The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"S.R.","family":"Steenmeijer","sequence":"additional","affiliation":[{"name":"Department of Ophthalmology Leiden University Medical Center Leiden The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M.","family":"Al\u2010Nofal","sequence":"additional","affiliation":[{"name":"Department of Ophthalmology Leiden University Medical Center Leiden The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M. J. H.","family":"Wermer","sequence":"additional","affiliation":[{"name":"Department of Neurology Leiden University Medical Center Leiden The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"I. C.","family":"Notting","sequence":"additional","affiliation":[{"name":"Department of Ophthalmology Leiden University Medical Center Leiden The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3140-6882","authenticated-orcid":false,"given":"G. M.","family":"Terwindt","sequence":"additional","affiliation":[{"name":"Department of Neurology Leiden University Medical Center Leiden The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2020,10,5]]},"reference":[{"key":"e_1_2_8_2_1","doi-asserted-by":"publisher","DOI":"10.1097\/WCO.0000000000000510"},{"key":"e_1_2_8_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.schres.2019.03.022"},{"key":"e_1_2_8_4_1","doi-asserted-by":"publisher","DOI":"10.1001\/jamaneurol.2018.1563"},{"key":"e_1_2_8_5_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1468-1331.2007.01795.x"},{"key":"e_1_2_8_6_1","doi-asserted-by":"publisher","DOI":"10.1002\/ana.24548"},{"issue":"1","key":"e_1_2_8_7_1","first-page":"277","article-title":"An investigation of the retinal nerve fibre layer in progressive multiple sclerosis using optical coherence tomography","volume":"131","author":"Henderson AP","year":"2008","journal-title":"Brain"},{"key":"e_1_2_8_8_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.0000000000002774"},{"key":"e_1_2_8_9_1","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0023024"},{"key":"e_1_2_8_10_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0197-0186(01)00129-2"},{"key":"e_1_2_8_11_1","doi-asserted-by":"publisher","DOI":"10.1161\/01.STR.18.2.311"},{"key":"e_1_2_8_12_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1440-1789.2005.00631.x"},{"key":"e_1_2_8_13_1","doi-asserted-by":"publisher","DOI":"10.1002\/ana.22005"},{"key":"e_1_2_8_14_1","doi-asserted-by":"publisher","DOI":"10.1097\/01.wno.0000204645.56873.26"},{"key":"e_1_2_8_15_1","doi-asserted-by":"publisher","DOI":"10.1159\/000251173"},{"key":"e_1_2_8_16_1","doi-asserted-by":"publisher","DOI":"10.1111\/aos.13681"}],"container-title":["European Journal of Neurology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1111%2Fene.14507","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/ene.14507","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1111\/ene.14507","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/ene.14507","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,8,25]],"date-time":"2023-08-25T22:12:16Z","timestamp":1693001536000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/ene.14507"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2020,10,5]]},"references-count":15,"journal-issue":{"issue":"12","published-print":{"date-parts":[[2020,12]]}},"alternative-id":["10.1111\/ene.14507"],"URL":"https:\/\/doi.org\/10.1111\/ene.14507","archive":["Portico"],"relation":{},"ISSN":["1351-5101","1468-1331"],"issn-type":[{"value":"1351-5101","type":"print"},{"value":"1468-1331","type":"electronic"}],"subject":[],"published":{"date-parts":[[2020,10,5]]},"assertion":[{"value":"2020-06-30","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2020-08-31","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2020-10-05","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}