{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,16]],"date-time":"2026-10-16T12:42:42Z","timestamp":1792154562357,"version":"4.4.0"},"reference-count":32,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2023,11,15]],"date-time":"2023-11-15T00:00:00Z","timestamp":1700006400000},"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":["Epilepsia Open"],"published-print":{"date-parts":[[2024,2]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>We aim to assess the efficacy and tolerance of cannabidiol as adjunctive therapy for Rett syndrome (RTT) patients with epilepsy. We conducted a longitudinal observational study through a monocentric cohort of 46 patients with RTT. Patients were recruited from March 2020 to October 2022 and were treated with Epidyolex\u00ae (cannabidiol, CBD, 100\u2009mg\/mL oral solution). In our cohort, 26 patients had associated epilepsy (26\/46 [56%]), and 10\/26 (38%) were treated with CBD, in combination with clobazam in 50% of cases. The median dose at their last follow\u2010up was 15\u2009mg\/kg\/day. The median treatment duration was 13\u2009months (range: 1\u201332 months). CBD reduced the incidence of seizures in seven out of 10 patients (70%) with one seizure\u2010free patient, two patients with a reduction of seizures of more than 75%, and four patients with a decrease of more than 50%. No aggravation of symptoms or adverse effects were observed. Only one patient experienced a transitory drooling and somnolence episode at the CBD initiation. Half of the patients showed a reduction in agitation and\/or anxiety attacks, and an improvement in spasticity was reported in 4\/10 (40%) of patients. CBD appears to have potential therapeutic value for the treatment of drug\u2010resistant epilepsy in Rett syndrome. CBD is well tolerated and, when used in combination with clobazam, may increase the effectiveness of clobazam alone.<\/jats:p>","DOI":"10.1002\/epi4.12796","type":"journal-article","created":{"date-parts":[[2023,7,24]],"date-time":"2023-07-24T09:16:27Z","timestamp":1690190187000},"page":"397-403","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":18,"title":["Efficacy and tolerance of cannabidiol in the treatment of epilepsy in patients with Rett syndrome"],"prefix":"10.1002","volume":"9","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-2571-8564","authenticated-orcid":false,"given":"B\u00e9atrice","family":"Desnous","sequence":"first","affiliation":[{"name":"Pediatric Neurology Department Timone Enfant, APHM  Marseille France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Thibault","family":"Beretti","sequence":"additional","affiliation":[{"name":"Pediatric Neurology Department Timone Enfant, APHM  Marseille France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nathan","family":"Muller","sequence":"additional","affiliation":[{"name":"Pediatric Neurology Department Timone Enfant, APHM  Marseille France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Julien","family":"Neveu","sequence":"additional","affiliation":[{"name":"Pediatric Neurology Department Lenval  Nice France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nathalie","family":"Villeneuve","sequence":"additional","affiliation":[{"name":"Pediatric Neurology Department Timone Enfant, APHM  Marseille France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anne","family":"L\u00e9pine","sequence":"additional","affiliation":[{"name":"Pediatric Neurology Department Timone Enfant, APHM  Marseille France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"G\u00e9raldine","family":"Daquin","sequence":"additional","affiliation":[{"name":"Epileptology and Cerebral Rhythmology Department Timone Adulte, APHM  Marseille France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2454-8543","authenticated-orcid":false,"given":"Mathieu","family":"Milh","sequence":"additional","affiliation":[{"name":"Pediatric Neurology Department Timone Enfant, APHM  Marseille France"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2023,11,15]]},"reference":[{"key":"e_1_2_10_2_1","doi-asserted-by":"publisher","DOI":"10.1203\/PDR.0b013e3182242461"},{"key":"e_1_2_10_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jpeds.2005.10.037"},{"key":"e_1_2_10_4_1","doi-asserted-by":"publisher","DOI":"10.1038\/13810"},{"key":"e_1_2_10_5_1","first-page":"12","article-title":"Rett syndrome Spectrum in monogenic developmental\u2010epileptic encephalopathies and epilepsies: a review","volume":"28","author":"Spagnoli C","year":"2021","journal-title":"Genes (Basel)"},{"key":"e_1_2_10_6_1","doi-asserted-by":"publisher","DOI":"10.23736\/S0026-4946.19.05309-X"},{"key":"e_1_2_10_7_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1651-2227.2001.tb00252.x"},{"key":"e_1_2_10_8_1","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/aww302"},{"key":"e_1_2_10_9_1","doi-asserted-by":"publisher","DOI":"10.1111\/epi.12941"},{"key":"e_1_2_10_10_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.yebeh.2010.06.051"},{"key":"e_1_2_10_11_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.braindev.2007.07.002"},{"key":"e_1_2_10_12_1","doi-asserted-by":"publisher","DOI":"10.1177\/03000605211007714"},{"key":"e_1_2_10_13_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.0b013e3181d6b852"},{"key":"e_1_2_10_14_1","doi-asserted-by":"publisher","DOI":"10.1002\/brb3.1250"},{"key":"e_1_2_10_15_1","doi-asserted-by":"publisher","DOI":"10.2147\/TCRM.S55896"},{"key":"e_1_2_10_16_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajmg.a.34367"},{"key":"e_1_2_10_17_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.yebeh.2016.10.006"},{"key":"e_1_2_10_18_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.pediatrneurol.2012.11.001"},{"key":"e_1_2_10_19_1","doi-asserted-by":"publisher","DOI":"10.3389\/fneur.2020.00829"},{"key":"e_1_2_10_20_1","doi-asserted-by":"publisher","DOI":"10.1007\/s40263-021-00807-y"},{"key":"e_1_2_10_21_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.yebeh.2021.108152"},{"key":"e_1_2_10_22_1","doi-asserted-by":"publisher","DOI":"10.1007\/s13311-015-0377-3"},{"key":"e_1_2_10_23_1","doi-asserted-by":"publisher","DOI":"10.1111\/epi.17247"},{"key":"e_1_2_10_24_1","doi-asserted-by":"publisher","DOI":"10.1007\/s40263-018-0589-2"},{"key":"e_1_2_10_25_1","doi-asserted-by":"publisher","DOI":"10.1111\/1469-7610.00236"},{"key":"e_1_2_10_26_1","doi-asserted-by":"publisher","DOI":"10.1212\/01.wnl.0000291011.54508.aa"},{"key":"e_1_2_10_27_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1474-4422(15)00379-8"},{"key":"e_1_2_10_28_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.yebeh.2018.05.013"},{"key":"e_1_2_10_29_1","doi-asserted-by":"publisher","DOI":"10.1111\/epi.16546"},{"key":"e_1_2_10_30_1","doi-asserted-by":"publisher","DOI":"10.1111\/epi.13060"},{"key":"e_1_2_10_31_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.braindev.2009.02.004"},{"key":"e_1_2_10_32_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.neuropharm.2018.07.029"},{"key":"e_1_2_10_33_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.neuroscience.2020.09.041"}],"container-title":["Epilepsia Open"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/epi4.12796","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,2,7]],"date-time":"2024-02-07T06:49:24Z","timestamp":1707288564000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/epi4.12796"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,11,15]]},"references-count":32,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2024,2]]}},"alternative-id":["10.1002\/epi4.12796"],"URL":"https:\/\/doi.org\/10.1002\/epi4.12796","archive":["Portico"],"relation":{},"ISSN":["2470-9239","2470-9239"],"issn-type":[{"value":"2470-9239","type":"print"},{"value":"2470-9239","type":"electronic"}],"subject":[],"published":{"date-parts":[[2023,11,15]]},"assertion":[{"value":"2023-04-04","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2023-07-20","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2023-11-15","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}