{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,25]],"date-time":"2026-03-25T06:55:07Z","timestamp":1774421707128,"version":"3.50.1"},"reference-count":40,"publisher":"Wiley","issue":"8","license":[{"start":{"date-parts":[[2023,5,23]],"date-time":"2023-05-23T00:00:00Z","timestamp":1684800000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100002347","name":"Bundesministerium f\u00fcr Bildung und Forschung","doi-asserted-by":"publisher","award":["01GM1906A"],"award-info":[{"award-number":["01GM1906A"]}],"id":[{"id":"10.13039\/501100002347","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100002347","name":"Bundesministerium f\u00fcr Bildung und Forschung","doi-asserted-by":"publisher","award":["01GM1920B"],"award-info":[{"award-number":["01GM1920B"]}],"id":[{"id":"10.13039\/501100002347","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100002347","name":"Bundesministerium f\u00fcr Bildung und Forschung","doi-asserted-by":"publisher","award":["01ZZ1603[A\u2010D]"],"award-info":[{"award-number":["01ZZ1603[A\u2010D]"]}],"id":[{"id":"10.13039\/501100002347","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100002347","name":"Bundesministerium f\u00fcr Bildung und Forschung","doi-asserted-by":"publisher","award":["01ZZ1804[A\u2010H]"],"award-info":[{"award-number":["01ZZ1804[A\u2010H]"]}],"id":[{"id":"10.13039\/501100002347","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Euro J of Neurology"],"published-print":{"date-parts":[[2023,8]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>The aim of this study was to investigate the neuroretinal structure of young patients with Leber hereditary optic neuropathy (LHON).<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>For this retrospective cross\u2010sectional analysis, the peripapillary retinal nerve fiber layer (pRNFL) thickness and the macular retinal layer volumes were measured by optical coherence tomography. Patients aged 12\u2009years or younger at disease onset were assigned to the childhood\u2010onset (ChO) group and those aged 13\u201316\u2009years to the early teenage\u2010onset (eTO) group. All patients received treatment with idebenone. The same measurements were repeated in age\u2010matched control groups with healthy subjects.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>The ChO group included 11 patients (21 eyes) and the eTO group 14 patients (27 eyes). Mean age at onset was 8.6\u2009\u00b1\u20092.7\u2009years in the ChO group and 14.8\u2009\u00b1\u20091.0\u2009years in the eTO group. Mean best\u2010corrected visual acuity was 0.65\u2009\u00b1\u20090.52 logMAR in the ChO group and 1.60\u2009\u00b1\u20090. 51 logMAR in the eTO group (<jats:italic>p<\/jats:italic>\u2009&lt;\u20090.001). Reduced pRNFL was evident in the eTO group compared to the ChO group (46.0\u2009\u00b1\u200912.7\u2009\u03bcm vs. 56.0\u2009\u00b1\u200914.5\u2009\u03bcm, <jats:italic>p<\/jats:italic>\u2009=\u20090.015). Additionally, a significantly lower combined ganglion cell and inner plexiform layer volume was found in the eTO compared to the ChO group (0.266\u2009\u00b1\u20090.0027\u2009mm<jats:sup>3<\/jats:sup> vs. 0.294\u2009\u00b1\u20090.033\u2009mm<jats:sup>3<\/jats:sup>, <jats:italic>p<\/jats:italic>\u2009=\u20090.003). No difference in these parameters was evident between the age\u2010matched control groups.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>Less neuroaxonal tissue degeneration was observed in ChO LHON than in eTO LHON, a finding that may explain the better functional outcome of ChO LHON.<\/jats:p><\/jats:sec>","DOI":"10.1111\/ene.15847","type":"journal-article","created":{"date-parts":[[2023,5,10]],"date-time":"2023-05-10T15:28:37Z","timestamp":1683732517000},"page":"2525-2533","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":2,"title":["Age\u2010dependent retinal neuroaxonal degeneration in children and adolescents with Leber hereditary optic neuropathy under idebenone therapy"],"prefix":"10.1111","volume":"30","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-0753-2408","authenticated-orcid":false,"given":"Benedikt","family":"Schworm","sequence":"first","affiliation":[{"name":"Department of Ophthalmology University Hospital, LMU Munich  Munich Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jakob","family":"Siedlecki","sequence":"additional","affiliation":[{"name":"Department of Ophthalmology University Hospital, LMU Munich  Munich Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Claudia","family":"Catarino","sequence":"additional","affiliation":[{"name":"Friedrich Baur Institute at the Department of Neurology University Hospital, LMU Munich  Munich Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Bettina","family":"von Livonius","sequence":"additional","affiliation":[{"name":"Department of Ophthalmology University Hospital, LMU Munich  Munich Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Daniel R.","family":"Muth","sequence":"additional","affiliation":[{"name":"Department of Ophthalmology University Hospital Zurich, University of Zurich  Zurich Switzerland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Guenther","family":"Rudolph","sequence":"additional","affiliation":[{"name":"Department of Ophthalmology University Hospital, LMU Munich  Munich Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Joachim","family":"Havla","sequence":"additional","affiliation":[{"name":"Institute of Clinical Neuroimmunology University Hospital, LMU Munich  Munich Germany"},{"name":"Data Integration for Future Medicine (DIFUTURE) Consortium University Hospital, LMU Munich  Munich Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2805-4652","authenticated-orcid":false,"given":"Thomas","family":"Klopstock","sequence":"additional","affiliation":[{"name":"Friedrich Baur Institute at the Department of Neurology University Hospital, LMU Munich  Munich Germany"},{"name":"Munich Cluster for Systems Neurology (SyNergy)  Munich Germany"},{"name":"German Center for Neurodegenerative Diseases (DZNE)  Munich Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Claudia","family":"Priglinger","sequence":"additional","affiliation":[{"name":"Department of Ophthalmology University Hospital, LMU Munich  Munich Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2023,5,23]]},"reference":[{"key":"e_1_2_10_2_1","doi-asserted-by":"publisher","DOI":"10.1136\/jmg.2003.011247"},{"key":"e_1_2_10_3_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00417-020-04993-1"},{"key":"e_1_2_10_4_1","doi-asserted-by":"publisher","DOI":"10.1172\/JCI138267"},{"key":"e_1_2_10_5_1","doi-asserted-by":"publisher","DOI":"10.1136\/jmedgenet-2016-104212"},{"key":"e_1_2_10_6_1","doi-asserted-by":"publisher","DOI":"10.3390\/genes12040521"},{"key":"e_1_2_10_7_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.mito.2017.07.004"},{"key":"e_1_2_10_8_1","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/awp158"},{"key":"e_1_2_10_9_1","doi-asserted-by":"publisher","DOI":"10.1038\/cddis.2015.364"},{"key":"e_1_2_10_10_1","doi-asserted-by":"publisher","DOI":"10.1097\/WNO.0000000000000570"},{"key":"e_1_2_10_11_1","doi-asserted-by":"publisher","DOI":"10.1167\/iovs.06-0520"},{"key":"e_1_2_10_12_1","doi-asserted-by":"publisher","DOI":"10.1136\/bjophthalmol-2016-310072"},{"issue":"11","key":"e_1_2_10_13_1","first-page":"1271","article-title":"Leber's hereditary optic neuropathy","volume":"236","author":"Priglinger C","year":"2019","journal-title":"Klin Monatsblatter fur Augenheilkd"},{"key":"e_1_2_10_14_1","doi-asserted-by":"publisher","DOI":"10.1136\/bjophthalmol-2021-320580"},{"key":"e_1_2_10_15_1","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/awr170"},{"key":"e_1_2_10_16_1","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/aws279"},{"key":"e_1_2_10_17_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ajo.2018.05.029"},{"key":"e_1_2_10_18_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ophtha.2004.06.034"},{"key":"e_1_2_10_19_1","doi-asserted-by":"publisher","DOI":"10.1111\/ceo.13326"},{"key":"e_1_2_10_20_1","doi-asserted-by":"publisher","DOI":"10.1038\/s41433-019-0695-5"},{"key":"e_1_2_10_21_1","doi-asserted-by":"publisher","DOI":"10.1080\/02713683.2019.1567792"},{"key":"e_1_2_10_22_1","doi-asserted-by":"publisher","DOI":"10.1136\/bjophthalmol-2015-307326"},{"key":"e_1_2_10_23_1","doi-asserted-by":"publisher","DOI":"10.1167\/iovs.05-0981"},{"key":"e_1_2_10_24_1","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0034823"},{"key":"e_1_2_10_25_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.0000000000012125"},{"key":"e_1_2_10_26_1","doi-asserted-by":"publisher","DOI":"10.1167\/tvst.9.3.24"},{"key":"e_1_2_10_27_1","doi-asserted-by":"publisher","DOI":"10.1097\/WNO.0000000000001045"},{"key":"e_1_2_10_28_1","doi-asserted-by":"publisher","DOI":"10.1038\/s41433-021-01535-9"},{"key":"e_1_2_10_29_1","doi-asserted-by":"publisher","DOI":"10.1038\/s41433-020-01177-3"},{"key":"e_1_2_10_30_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ophtha.2018.03.026"},{"key":"e_1_2_10_31_1","doi-asserted-by":"publisher","DOI":"10.1007\/s10384-012-0142-7"},{"key":"e_1_2_10_32_1","doi-asserted-by":"publisher","DOI":"10.1038\/eye.2008.87"},{"key":"e_1_2_10_33_1","doi-asserted-by":"publisher","DOI":"10.1001\/jamaneurol.2018.1563"},{"key":"e_1_2_10_34_1","doi-asserted-by":"publisher","DOI":"10.1097\/WNO.0000000000000203"},{"key":"e_1_2_10_35_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00415-020-09796-2"},{"key":"e_1_2_10_36_1","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/awn269"},{"key":"e_1_2_10_37_1","doi-asserted-by":"publisher","DOI":"10.3109\/01658107.2011.580898"},{"key":"e_1_2_10_38_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1474-4422(17)30278-8"},{"key":"e_1_2_10_39_1","doi-asserted-by":"publisher","DOI":"10.1097\/OPX.0000000000000991"},{"key":"e_1_2_10_40_1","doi-asserted-by":"publisher","DOI":"10.1186\/s12974-021-02160-9"},{"issue":"24","key":"e_1_2_10_41_1","first-page":"6921","article-title":"Targeting estrogen receptor \u03b2 as preventive therapeutic strategy for Leber's hereditary optic neuropathy","volume":"24","author":"Pisano A","year":"2015","journal-title":"Hum Mol Genet"}],"container-title":["European Journal of Neurology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/ene.15847","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,8,22]],"date-time":"2023-08-22T05:34:04Z","timestamp":1692682444000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/ene.15847"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,5,23]]},"references-count":40,"journal-issue":{"issue":"8","published-print":{"date-parts":[[2023,8]]}},"alternative-id":["10.1111\/ene.15847"],"URL":"https:\/\/doi.org\/10.1111\/ene.15847","archive":["Portico"],"relation":{},"ISSN":["1351-5101","1468-1331"],"issn-type":[{"value":"1351-5101","type":"print"},{"value":"1468-1331","type":"electronic"}],"subject":[],"published":{"date-parts":[[2023,5,23]]},"assertion":[{"value":"2022-06-21","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2023-05-05","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2023-05-23","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}