{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,13]],"date-time":"2026-10-13T22:01:44Z","timestamp":1791928904440,"version":"4.3.4"},"reference-count":88,"publisher":"Elsevier BV","issue":"1","license":[{"start":{"date-parts":[[2021,1,1]],"date-time":"2021-01-01T00:00:00Z","timestamp":1609459200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2021,1,1]],"date-time":"2021-01-01T00:00:00Z","timestamp":1609459200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/legal\/tdmrep-license"}],"funder":[{"DOI":"10.13039\/100006108","name":"National Center for Advancing Translational Sciences","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100006108","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100014751","name":"Tropical Resources Institute","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100014751","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100015317","name":"Translational Research Institute, University of Arkansas for Medical Sciences","doi-asserted-by":"publisher","award":["UL1 TR003107"],"award-info":[{"award-number":["UL1 TR003107"]}],"id":[{"id":"10.13039\/100015317","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["brainanddevelopment.com","clinicalkey.com","clinicalkey.com.au","clinicalkey.es","clinicalkey.fr","clinicalkey.jp","elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Brain and Development"],"published-print":{"date-parts":[[2021,1]]},"DOI":"10.1016\/j.braindev.2020.08.014","type":"journal-article","created":{"date-parts":[[2020,9,4]],"date-time":"2020-09-04T05:52:27Z","timestamp":1599198747000},"page":"32-44","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":82,"title":["Epilepsy in Angelman syndrome: A scoping review"],"prefix":"10.1016","volume":"43","author":[{"given":"Debopam","family":"Samanta","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"key":"10.1016\/j.braindev.2020.08.014_b0005","doi-asserted-by":"crossref","first-page":"211","DOI":"10.1016\/j.seizure.2007.08.004","article-title":"Epilepsy in Angelman syndrome","volume":"17","author":"Pelc","year":"2008","journal-title":"Seizure"},{"key":"10.1016\/j.braindev.2020.08.014_b0010","doi-asserted-by":"crossref","first-page":"171","DOI":"10.3345\/kjp.2012.55.5.171","article-title":"Epilepsy in Korean patients with Angelman syndrome","volume":"55","author":"Park","year":"2012","journal-title":"Korean J Pediatr"},{"key":"10.1016\/j.braindev.2020.08.014_b0015","doi-asserted-by":"crossref","first-page":"271","DOI":"10.1016\/j.pediatrneurol.2012.09.015","article-title":"Neurologic manifestations of Angelman syndrome","volume":"48","author":"Thibert","year":"2013","journal-title":"Pediatr Neurol"},{"key":"10.1016\/j.braindev.2020.08.014_b0020","doi-asserted-by":"crossref","first-page":"3001","DOI":"10.1093\/hmg\/dds130","article-title":"Ube3a-ATS is an atypical RNA polymerase II transcript that represses the paternal expression of Ube3a","volume":"21","author":"Meng","year":"2012","journal-title":"Hum Mol Genet"},{"key":"10.1016\/j.braindev.2020.08.014_b0025","doi-asserted-by":"crossref","first-page":"109","DOI":"10.3389\/fnmol.2019.00109","article-title":"The Autism and Angelman Syndrome Protein Ube3A\/E6AP: The Gene, E3 Ligase Ubiquitination Targets and Neurobiological Functions","volume":"12","author":"Khatri","year":"2019","journal-title":"Front Mol Neurosci"},{"key":"10.1016\/j.braindev.2020.08.014_b0030","doi-asserted-by":"crossref","first-page":"21","DOI":"10.1177\/08830738030180010801","article-title":"Peripheral markers of the gamma-aminobutyric acid (GABA)ergic system in Angelman's syndrome","volume":"18","author":"Borgatti","year":"2003","journal-title":"J Child Neurol"},{"key":"10.1016\/j.braindev.2020.08.014_b0035","doi-asserted-by":"crossref","first-page":"150","DOI":"10.1016\/j.devbrainres.2005.03.014","article-title":"GABAA receptor beta3 subunit gene-deficient heterozygous mice show parent-of-origin and gender-related differences in beta3 subunit levels, EEG, and behavior","volume":"157","author":"Liljelund","year":"2005","journal-title":"Brain Res Dev Brain Res"},{"key":"10.1016\/j.braindev.2020.08.014_b0040","doi-asserted-by":"crossref","first-page":"123","DOI":"10.1016\/S0920-1211(99)00046-7","article-title":"GABA and epileptogenesis: comparing gabrb3 gene-deficient mice with Angelman syndrome in man","volume":"36","author":"DeLorey","year":"1999","journal-title":"Epilepsy Res"},{"key":"10.1016\/j.braindev.2020.08.014_b0045","doi-asserted-by":"crossref","first-page":"8505","DOI":"10.1523\/JNEUROSCI.18-20-08505.1998","article-title":"Mice lacking the beta3 subunit of the GABAA receptor have the epilepsy phenotype and many of the behavioral characteristics of Angelman syndrome","volume":"18","author":"DeLorey","year":"1998","journal-title":"J Neurosci"},{"key":"10.1016\/j.braindev.2020.08.014_b0050","doi-asserted-by":"crossref","first-page":"99","DOI":"10.1016\/j.eplepsyres.2005.07.005","article-title":"Long-term effects of diazepam treatment of epileptic GABAA receptor beta3 subunit knockout mouse in early life","volume":"66","author":"Liljelund","year":"2005","journal-title":"Epilepsy Res"},{"key":"10.1016\/j.braindev.2020.08.014_b0055","doi-asserted-by":"crossref","first-page":"793","DOI":"10.1016\/j.neuron.2012.03.036","article-title":"Maternal loss of Ube3a produces an excitatory\/inhibitory imbalance through neuron type-specific synaptic defects","volume":"74","author":"Wallace","year":"2012","journal-title":"Neuron"},{"key":"10.1016\/j.braindev.2020.08.014_b0060","doi-asserted-by":"crossref","DOI":"10.1126\/scitranslmed.3004655","article-title":"Decreased tonic inhibition in cerebellar granule cells causes motor dysfunction in a mouse model of Angelman syndrome","volume":"4","author":"Egawa","year":"2012","journal-title":"Sci Transl Med"},{"key":"10.1016\/j.braindev.2020.08.014_b0065","doi-asserted-by":"crossref","first-page":"163","DOI":"10.1172\/JCI120816","article-title":"Ube3a reinstatement mitigates epileptogenesis in Angelman syndrome model mice","volume":"129","author":"Gu","year":"2019","journal-title":"J Clin Invest"},{"key":"10.1016\/j.braindev.2020.08.014_b0070","doi-asserted-by":"crossref","first-page":"56","DOI":"10.1016\/j.neuron.2016.02.040","article-title":"GABAergic Neuron-Specific Loss of Ube3a Causes Angelman Syndrome-Like EEG Abnormalities and Enhances Seizure Susceptibility","volume":"90","author":"Judson","year":"2016","journal-title":"Neuron"},{"key":"10.1016\/j.braindev.2020.08.014_b0075","doi-asserted-by":"crossref","DOI":"10.1186\/1824-7288-36-31","article-title":"Epilepsy in patients with Angelman syndrome","volume":"36","author":"Fiumara","year":"2010","journal-title":"Ital J Pediatr"},{"key":"10.1016\/j.braindev.2020.08.014_b0080","doi-asserted-by":"crossref","first-page":"1011","DOI":"10.1111\/j.1469-8749.1990.tb08125.x","article-title":"Angelman's syndrome in the first year of life","volume":"32","author":"Van Lierde","year":"1990","journal-title":"Dev Med Child Neurol"},{"key":"10.1016\/j.braindev.2020.08.014_b0085","doi-asserted-by":"crossref","unstructured":"Bakke KA, Howlin P, Retterst\u00f8l L, Kanavin \u00d8, Heiberg A, N\u00e6rland T. Effect of epilepsy on autism symptoms in Angelman syndrome.Mol Autism. 2018;9:2,017-0185-1. eCollection 2018.","DOI":"10.1186\/s13229-017-0185-1"},{"key":"10.1016\/j.braindev.2020.08.014_b0090","doi-asserted-by":"crossref","first-page":"315","DOI":"10.1016\/j.ejmg.2016.05.003","article-title":"Angelman syndrome in Hong Kong Chinese: A 20 years' experience","volume":"59","author":"Luk","year":"2016","journal-title":"Eur J Med Genet"},{"key":"10.1016\/j.braindev.2020.08.014_b0095","doi-asserted-by":"crossref","first-page":"131","DOI":"10.1038\/sj.ejhg.5200258","article-title":"Phenotype\u2013genotype correlation in 20 deletion and 20 non-deletion Angelman syndrome patients","volume":"7","author":"Moncla","year":"1999","journal-title":"Eur J Hum Genet"},{"key":"10.1016\/j.braindev.2020.08.014_b0100","doi-asserted-by":"crossref","first-page":"1373","DOI":"10.1007\/s10072-016-2547-4","article-title":"Reflex seizures in a patient with Angelman syndrome and trisomy 21","volume":"37","author":"Ferlazzo","year":"2016","journal-title":"Neurol Sci"},{"key":"10.1016\/j.braindev.2020.08.014_b0105","doi-asserted-by":"crossref","first-page":"510","DOI":"10.1212\/CPJ.0000000000000692","article-title":"Reflex micturition defecation epilepsy in Angelman syndrome","volume":"9","author":"Pellinen","year":"2019","journal-title":"Neurol Clin Pract"},{"key":"10.1016\/j.braindev.2020.08.014_b0110","doi-asserted-by":"crossref","first-page":"122","DOI":"10.1001\/archneur.63.1.122","article-title":"Epilepsy in patients with angelman syndrome caused by deletion of the chromosome 15q11-13","volume":"63","author":"Valente","year":"2006","journal-title":"Arch Neurol"},{"key":"10.1016\/j.braindev.2020.08.014_b0115","doi-asserted-by":"crossref","first-page":"467","DOI":"10.1177\/088307389501000609","article-title":"Seizure and EEG patterns in Angelman's syndrome","volume":"10","author":"Viani","year":"1995","journal-title":"J Child Neurol"},{"key":"10.1016\/j.braindev.2020.08.014_b0120","doi-asserted-by":"crossref","first-page":"163","DOI":"10.1016\/j.eplepsyres.2005.09.003","article-title":"Angelman syndrome: uniparental paternal disomy 15 determines mild epilepsy, but has no influence on EEG patterns","volume":"67","author":"Valente","year":"2005","journal-title":"Epilepsy Res"},{"key":"10.1016\/j.braindev.2020.08.014_b0125","doi-asserted-by":"crossref","first-page":"195","DOI":"10.1111\/j.1528-1157.1997.tb01097.x","article-title":"Evolution of epilepsy and EEG findings in Angelman syndrome","volume":"38","author":"Laan","year":"1997","journal-title":"Epilepsia"},{"issue":"Suppl 5","key":"10.1016\/j.braindev.2020.08.014_b0130","doi-asserted-by":"crossref","first-page":"41","DOI":"10.1111\/j.1528-1167.2009.02119.x","article-title":"Myoclonic status in nonprogressive encephalopathies: an update","volume":"50","author":"Elia","year":"2009","journal-title":"Epilepsia"},{"key":"10.1016\/j.braindev.2020.08.014_b0135","doi-asserted-by":"crossref","first-page":"170","DOI":"10.1016\/j.yebeh.2018.02.006","article-title":"Myoclonus in Angelman syndrome","volume":"82","author":"Pollack","year":"2018","journal-title":"Epilepsy Behav"},{"key":"10.1016\/j.braindev.2020.08.014_b0140","doi-asserted-by":"crossref","first-page":"296","DOI":"10.1016\/S0387-7604(99)00007-8","article-title":"Diagnosis of Angelman syndrome: clinical and EEG criteria","volume":"21","author":"Buoni","year":"1999","journal-title":"Brain Dev"},{"key":"10.1016\/j.braindev.2020.08.014_b0145","doi-asserted-by":"crossref","first-page":"404","DOI":"10.1016\/S0013-4694(98)00007-8","article-title":"The diagnostic value of the EEG in Angelman and Rett syndrome at a young age","volume":"106","author":"Laan","year":"1998","journal-title":"Electroencephalogr Clin Neurophysiol"},{"key":"10.1016\/j.braindev.2020.08.014_b0150","first-page":"701","article-title":"Revisiting epilepsy and the electroencephalogram patterns in Angelman syndrome","volume":"35","author":"Leyser","year":"2014","journal-title":"Neurol Sci"},{"key":"10.1016\/j.braindev.2020.08.014_b0155","doi-asserted-by":"crossref","first-page":"58","DOI":"10.1177\/0883073814530498","article-title":"Electroencephalogram (EEG) duration needed to detect abnormalities in angelman syndrome: is 1 hour of overnight recording sufficient?","volume":"30","author":"Robinson","year":"2015","journal-title":"J Child Neurol"},{"key":"10.1016\/j.braindev.2020.08.014_b0160","doi-asserted-by":"crossref","first-page":"261","DOI":"10.1016\/j.yebeh.2011.11.027","article-title":"Analysis of EEG patterns and genotypes in patients with Angelman syndrome","volume":"23","author":"Vendrame","year":"2012","journal-title":"Epilepsy Behav"},{"key":"10.1016\/j.braindev.2020.08.014_b0165","doi-asserted-by":"crossref","first-page":"752","DOI":"10.1016\/j.biopsych.2019.01.008","article-title":"Electrophysiological phenotype in Angelman syndrome differs between genotypes","volume":"85","author":"Frohlich","year":"2019","journal-title":"Biol Psychiatry"},{"key":"10.1016\/j.braindev.2020.08.014_b0170","doi-asserted-by":"crossref","first-page":"331","DOI":"10.1684\/epd.2011.0446","article-title":"Angelman syndrome and pseudo-hypsarrhythmia: a diagnostic pitfall","volume":"13","author":"Darteyre","year":"2011","journal-title":"Epileptic Disord"},{"key":"10.1016\/j.braindev.2020.08.014_b0175","doi-asserted-by":"crossref","first-page":"1590","DOI":"10.1046\/j.1528-1157.2002.30802.x","article-title":"A Rett patient with a typical Angelman EEG","volume":"43","author":"Laan","year":"2002","journal-title":"Epilepsia"},{"key":"10.1016\/j.braindev.2020.08.014_b0180","doi-asserted-by":"crossref","first-page":"1206","DOI":"10.1111\/j.1528-1157.1995.tb01064.x","article-title":"4p(-) syndrome: a chromosomal disorder associated with a particular EEG pattern","volume":"36","author":"Sgr\u00f2","year":"1995","journal-title":"Epilepsia"},{"key":"10.1016\/j.braindev.2020.08.014_b0185","doi-asserted-by":"crossref","first-page":"228","DOI":"10.4103\/0028-3886.177615","article-title":"Scrutinizing brain magnetic resonance imaging patterns in Angelman syndrome","volume":"64","author":"Leyser","year":"2016","journal-title":"Neurol India"},{"key":"10.1016\/j.braindev.2020.08.014_b0190","doi-asserted-by":"crossref","first-page":"271","DOI":"10.1016\/j.ejpn.2008.04.005","article-title":"Abnormal myelination in Angelman syndrome","volume":"13","author":"Harting","year":"2009","journal-title":"Eur J Paediatr Neurol"},{"key":"10.1016\/j.braindev.2020.08.014_b0195","doi-asserted-by":"crossref","first-page":"361","DOI":"10.1111\/j.1469-8749.2010.03838.x","article-title":"Alterations in white matter pathways in Angelman syndrome","volume":"53","author":"Peters","year":"2011","journal-title":"Dev Med Child Neurol"},{"key":"10.1016\/j.braindev.2020.08.014_b0200","doi-asserted-by":"crossref","first-page":"423","DOI":"10.1136\/adc.79.5.423","article-title":"Parental view of epilepsy in Angelman syndrome: a questionnaire study","volume":"79","author":"Ruggieri","year":"1998","journal-title":"Arch Dis Child"},{"key":"10.1016\/j.braindev.2020.08.014_b0205","first-page":"357","article-title":"Toxic hepatitis in a case of Angelman syndrome associated with Lennox-Gastaut syndrome","volume":"15","author":"Deda","year":"2004","journal-title":"Genet Couns"},{"key":"10.1016\/j.braindev.2020.08.014_b0210","doi-asserted-by":"crossref","first-page":"138","DOI":"10.1016\/j.yebeh.2016.04.030","article-title":"Seizure treatment in Angelman syndrome: A case series from the Angelman Syndrome Clinic at Massachusetts General Hospital","volume":"60","author":"Shaaya","year":"2016","journal-title":"Epilepsy Behav"},{"key":"10.1016\/j.braindev.2020.08.014_b0215","doi-asserted-by":"crossref","first-page":"2369","DOI":"10.1111\/j.1528-1167.2009.02108.x","article-title":"Epilepsy in Angelman syndrome: a questionnaire-based assessment of the natural history and current treatment options","volume":"50","author":"Thibert","year":"2009","journal-title":"Epilepsia"},{"key":"10.1016\/j.braindev.2020.08.014_b0220","doi-asserted-by":"crossref","first-page":"1185","DOI":"10.1212\/WNL.54.5.1185","article-title":"Topiramate therapy of epilepsy associated with Angelman's syndrome","volume":"54","author":"Franz","year":"2000","journal-title":"Neurology"},{"key":"10.1016\/j.braindev.2020.08.014_b0225","doi-asserted-by":"crossref","first-page":"593","DOI":"10.1111\/j.1528-1167.2006.00969.x","article-title":"Lamotrigine therapy of epilepsy with Angelman's syndrome","volume":"48","author":"Dion","year":"2007","journal-title":"Epilepsia"},{"key":"10.1016\/j.braindev.2020.08.014_b0230","doi-asserted-by":"crossref","first-page":"368","DOI":"10.1212\/WNL.58.3.368","article-title":"Modulation of cerebral GABA by topiramate, lamotrigine, and gabapentin in healthy adults","volume":"58","author":"Kuzniecky","year":"2002","journal-title":"Neurology"},{"key":"10.1016\/j.braindev.2020.08.014_b0235","doi-asserted-by":"crossref","first-page":"415","DOI":"10.1016\/S0893-133X(01)00385-2","article-title":"Lamotrigine increases gene expression of GABA-A receptor \u00ce23 subunit in primary cultured rat hippocampus cells","volume":"26","author":"Wang","year":"2002","journal-title":"Neuropsychopharmacology"},{"key":"10.1016\/j.braindev.2020.08.014_b0240","doi-asserted-by":"crossref","first-page":"1518","DOI":"10.1212\/WNL.57.8.1518","article-title":"High-dose ethosuximide for epilepsy in Angelman syndrome: implication of GABA(A) receptor subunit","volume":"57","author":"Sugiura","year":"2001","journal-title":"Neurology"},{"key":"10.1016\/j.braindev.2020.08.014_b0245","doi-asserted-by":"crossref","first-page":"5462","DOI":"10.1172\/JCI130419","article-title":"Cannabidiol attenuates seizures and EEG abnormalities in Angelman syndrome model mice","volume":"129","author":"Gu","year":"2019","journal-title":"J Clin Invest"},{"key":"10.1016\/j.braindev.2020.08.014_b0250","doi-asserted-by":"crossref","first-page":"24","DOI":"10.1016\/j.pediatrneurol.2019.03.014","article-title":"Cannabidiol: A Review of Clinical Efficacy and Safety in Epilepsy","volume":"96","author":"Samanta","year":"2019","journal-title":"Pediatr Neurol"},{"key":"10.1016\/j.braindev.2020.08.014_b0255","doi-asserted-by":"crossref","first-page":"135","DOI":"10.1055\/s-0040-1701694","article-title":"Changing Landscape of Dravet Syndrome Management: An Overview","volume":"51","author":"Samanta","year":"2020","journal-title":"Neuropediatrics"},{"key":"10.1016\/j.braindev.2020.08.014_b0260","doi-asserted-by":"crossref","first-page":"389","DOI":"10.1016\/j.braindev.2020.02.007","article-title":"Perampanel for nonepileptic myoclonus in Angelman syndrome","volume":"42","author":"Kawano","year":"2020","journal-title":"Brain Dev"},{"key":"10.1016\/j.braindev.2020.08.014_b0265","doi-asserted-by":"crossref","first-page":"35","DOI":"10.1016\/j.spen.2010.02.002","article-title":"A \u201chappy\u201d toddler presenting with sudden, life-threatening seizures","volume":"17","author":"Stein","year":"2010","journal-title":"Semin Pediatr Neurol"},{"key":"10.1016\/j.braindev.2020.08.014_b0270","doi-asserted-by":"crossref","first-page":"45","DOI":"10.1016\/j.yebeh.2016.12.018","article-title":"Low glycemic index treatment for seizure control in Angelman syndrome: A case series from the Center for Dietary Therapy of Epilepsy at the Massachusetts General Hospital","volume":"68","author":"Grocott","year":"2017","journal-title":"Epilepsy Behav"},{"key":"10.1016\/j.braindev.2020.08.014_b0275","doi-asserted-by":"crossref","first-page":"1498","DOI":"10.1111\/j.1528-1167.2012.03537.x","article-title":"Low glycemic index treatment for seizures in Angelman syndrome","volume":"53","author":"Thibert","year":"2012","journal-title":"Epilepsia"},{"key":"10.1016\/j.braindev.2020.08.014_b0280","doi-asserted-by":"crossref","first-page":"395","DOI":"10.1007\/s00381-018-3723-z","article-title":"Vagal nerve stimulation for medically refractory epilepsy in Angelman syndrome: a series of three cases","volume":"34","author":"Tomei","year":"2018","journal-title":"Childs Nerv Syst"},{"key":"10.1016\/j.braindev.2020.08.014_b0285","doi-asserted-by":"crossref","DOI":"10.5812\/aapm.57826","article-title":"Anesthetic Considerations for Angelman Syndrome: Case Series and Review of the Literature","volume":"7","author":"Warner","year":"2017","journal-title":"Anesth Pain Med"},{"key":"10.1016\/j.braindev.2020.08.014_b0290","doi-asserted-by":"crossref","first-page":"952","DOI":"10.1177\/0883073808331344","article-title":"Benefit of corticosteroid therapy in Angelman syndrome","volume":"24","author":"Forrest","year":"2009","journal-title":"J Child Neurol"},{"key":"10.1016\/j.braindev.2020.08.014_b0295","doi-asserted-by":"crossref","first-page":"74","DOI":"10.1016\/j.yebeh.2018.02.027","article-title":"Diazepam for outpatient treatment of nonconvulsive status epilepticus in pediatric patients with Angelman syndrome","volume":"82","author":"Worden","year":"2018","journal-title":"Epilepsy Behav"},{"key":"10.1016\/j.braindev.2020.08.014_b0300","doi-asserted-by":"crossref","first-page":"1213","DOI":"10.1111\/j.1528-1157.1998.tb01314.x","article-title":"Adverse effects of vigabatrin in Angelman syndrome","volume":"39","author":"Kuenzle","year":"1998","journal-title":"Epilepsia"},{"key":"10.1016\/j.braindev.2020.08.014_b0305","doi-asserted-by":"crossref","first-page":"119","DOI":"10.1159\/000369454","article-title":"Angelman syndrome: a case series assessing neurological issues in adulthood","volume":"73","author":"Giroud","year":"2015","journal-title":"Eur Neurol"},{"key":"10.1016\/j.braindev.2020.08.014_b0310","doi-asserted-by":"crossref","first-page":"356","DOI":"10.1002\/(SICI)1096-8628(19961218)66:3<356::AID-AJMG21>3.0.CO;2-K","article-title":"Angelman syndrome in adulthood","volume":"66","author":"Laan","year":"1996","journal-title":"Am J Med Genet"},{"key":"10.1016\/j.braindev.2020.08.014_b0315","doi-asserted-by":"crossref","first-page":"225","DOI":"10.1016\/j.yebeh.2017.07.041","article-title":"Epilepsy and sleep disorders improve in adolescents and adults with Angelman syndrome: A multicenter study on 46 patients","volume":"75","author":"Sueri","year":"2017","journal-title":"Epilepsy Behav"},{"key":"10.1016\/j.braindev.2020.08.014_b0320","doi-asserted-by":"crossref","first-page":"331","DOI":"10.1002\/ajmg.a.36864","article-title":"Angelman syndrome in adulthood","volume":"167A","author":"Larson","year":"2015","journal-title":"Am J Med Genet A"},{"key":"10.1016\/j.braindev.2020.08.014_b0325","doi-asserted-by":"crossref","first-page":"227","DOI":"10.1684\/j.1950-6945.2005.tb00126.x","article-title":"Atypical absences and recurrent absence status in an adult with Angelman syndrome due to the UBE3A mutation","volume":"7","author":"Espay","year":"2005","journal-title":"Epileptic Disord"},{"key":"10.1016\/j.braindev.2020.08.014_b0330","doi-asserted-by":"crossref","first-page":"601","DOI":"10.1517\/14728222.2016.1115837","article-title":"Potential therapeutic approaches for Angelman syndrome","volume":"20","author":"Bi","year":"2016","journal-title":"Expert Opin Ther Targets"},{"key":"10.1016\/j.braindev.2020.08.014_b0335","doi-asserted-by":"crossref","DOI":"10.1016\/j.neuroscience.2020.02.017","article-title":"Angelman syndrome: from mouse models to therapy","author":"Rotaru","year":"2020","journal-title":"Neuroscience"},{"key":"10.1016\/j.braindev.2020.08.014_b0340","doi-asserted-by":"crossref","first-page":"280","DOI":"10.1038\/nn1845","article-title":"Rescue of neurological deficits in a mouse model for Angelman syndrome by reduction of alphaCaMKII inhibitory phosphorylation","volume":"10","author":"van Woerden","year":"2007","journal-title":"Nat Neurosci"},{"key":"10.1016\/j.braindev.2020.08.014_b0345","doi-asserted-by":"crossref","first-page":"2634","DOI":"10.1523\/JNEUROSCI.23-07-02634.2003","article-title":"Derangements of hippocampal calcium\/calmodulin-dependent protein kinase II in a mouse model for Angelman mental retardation syndrome","volume":"23","author":"Weeber","year":"2003","journal-title":"J Neurosci"},{"key":"10.1016\/j.braindev.2020.08.014_b0350","doi-asserted-by":"crossref","first-page":"548","DOI":"10.1038\/mt.2015.236","article-title":"Protein Delivery of an Artificial Transcription Factor Restores Widespread Ube3a Expression in an Angelman Syndrome Mouse Brain","volume":"24","author":"Bailus","year":"2016","journal-title":"Mol Ther"},{"key":"10.1016\/j.braindev.2020.08.014_b0355","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pone.0027221","article-title":"Adeno-associated virus-mediated rescue of the cognitive defects in a mouse model for Angelman syndrome","volume":"6","author":"Daily","year":"2011","journal-title":"PLoS One"},{"key":"10.1016\/j.braindev.2020.08.014_b0360","doi-asserted-by":"crossref","first-page":"185","DOI":"10.1038\/nature10726","article-title":"Topoisomerase inhibitors unsilence the dormant allele of Ube3a in neurons","volume":"481","author":"Huang","year":"2011","journal-title":"Nature"},{"key":"10.1016\/j.braindev.2020.08.014_b0365","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pgen.1004039","article-title":"Truncation of Ube3a-ATS unsilences paternal Ube3a and ameliorates ehavioral defects in the Angelman syndrome mouse model","volume":"9","author":"Meng","year":"2013","journal-title":"PLoS Genet"},{"key":"10.1016\/j.braindev.2020.08.014_b0370","doi-asserted-by":"crossref","first-page":"409","DOI":"10.1038\/nature13975","article-title":"Towards a therapy for Angelman syndrome by targeting a long non-coding RNA","volume":"518","author":"Meng","year":"2015","journal-title":"Nature"},{"key":"10.1016\/j.braindev.2020.08.014_b0375","doi-asserted-by":"crossref","first-page":"993","DOI":"10.3390\/cells9040993","article-title":"CRISPR\/Cas9 epigenome editing potential for rare imprinting diseases: a review","volume":"9","author":"Syding","year":"2020","journal-title":"Cells"},{"key":"10.1016\/j.braindev.2020.08.014_b0380","doi-asserted-by":"crossref","DOI":"10.1186\/1471-2202-15-76","article-title":"The prospect of molecular therapy for Angelman syndrome and other monogenic neurologic disorders","volume":"15","author":"Bailus","year":"2014","journal-title":"BMC Neurosci."},{"key":"10.1016\/j.braindev.2020.08.014_b0385","doi-asserted-by":"crossref","first-page":"2069","DOI":"10.1172\/JCI80554","article-title":"Ube3a reinstatement identifies distinct developmental windows in a murine Angelman syndrome model","volume":"125","author":"Silva-Santos","year":"2015","journal-title":"J Clin Invest"},{"key":"10.1016\/j.braindev.2020.08.014_b0390","unstructured":"STARS: Results from a safety and efficacy study of OV101 (gaboxadol) in adults and adolescents with Angelman syndrome. American Academy of Neurology 2019 Annual Meeting, Philadelphia, PA; 2019."},{"key":"10.1016\/j.braindev.2020.08.014_b0395","doi-asserted-by":"crossref","first-page":"142","DOI":"10.1016\/j.neuropharm.2016.12.009","article-title":"Effects of the synthetic neurosteroid ganaxolone on seizure activity and behavioral deficits in an Angelman syndrome mouse model","volume":"116","author":"Ciarlone","year":"2017","journal-title":"Neuropharmacology"},{"key":"10.1016\/j.braindev.2020.08.014_b0400","doi-asserted-by":"crossref","first-page":"1994","DOI":"10.1002\/ajmg.a.33509","article-title":"Double-blind therapeutic trial in Angelman syndrome using betaine and folic acid","volume":"152A","author":"Peters","year":"2010","journal-title":"Am J Med Genet A"},{"key":"10.1016\/j.braindev.2020.08.014_b0405","doi-asserted-by":"crossref","first-page":"38","DOI":"10.1016\/j.nbd.2016.08.002","article-title":"Ketone ester supplementation attenuates seizure activity, and improves behavior and hippocampal synaptic plasticity in an Angelman syndrome mouse model","volume":"96","author":"Ciarlone","year":"2016","journal-title":"Neurobiol Dis"},{"key":"10.1016\/j.braindev.2020.08.014_b0410","doi-asserted-by":"crossref","first-page":"12","DOI":"10.1016\/j.nbd.2017.10.016","article-title":"Lovastatin suppresses hyperexcitability and seizure in Angelman syndrome model","volume":"110","author":"Chung","year":"2018","journal-title":"Neurobiol Dis"},{"key":"10.1016\/j.braindev.2020.08.014_b0415","doi-asserted-by":"crossref","first-page":"1088","DOI":"10.3390\/ijms19041088","article-title":"Taurine Administration Recovers Motor and Learning Deficits in an Angelman Syndrome Mouse Model","volume":"19","author":"Guzzetti","year":"2018","journal-title":"Int J Mol Sci"},{"key":"10.1016\/j.braindev.2020.08.014_b0420","doi-asserted-by":"crossref","DOI":"10.1016\/j.redox.2019.101223","article-title":"Taurine and its analogs in neurological disorders: focus on therapeutic potential and molecular mechanisms","volume":"24","author":"Jakaria","year":"2019","journal-title":"Redox Biol"},{"key":"10.1016\/j.braindev.2020.08.014_b0425","doi-asserted-by":"crossref","DOI":"10.1186\/s12883-014-0232-x","article-title":"An open-label pilot trial of minocycline in children as a treatment for Angelman syndrome","volume":"14","author":"Grieco","year":"2014","journal-title":"BMC Neurol."},{"key":"10.1016\/j.braindev.2020.08.014_b0430","doi-asserted-by":"crossref","first-page":"144","DOI":"10.1186\/s13023-018-0891-6","article-title":"A randomized placebo controlled clinical trial to evaluate the efficacy and safety of minocycline in patients with Angelman syndrome (A-MANECE study)","volume":"13","author":"Ruiz-Antoran","year":"2018","journal-title":"Orphanet J Rare Dis"},{"key":"10.1016\/j.braindev.2020.08.014_b0435","doi-asserted-by":"crossref","first-page":"134","DOI":"10.1186\/s13023-017-0686-1","article-title":"A web-based, patient driven registry for Angelman syndrome: the global Angelman syndrome registry","volume":"12","author":"Napier","year":"2017","journal-title":"Orphanet J Rare Dis"},{"key":"10.1016\/j.braindev.2020.08.014_b0440","doi-asserted-by":"crossref","first-page":"431","DOI":"10.1111\/jir.12482","article-title":"Research protocol: The initiation, design and establishment of the Global Angelman Syndrome Registry","volume":"62","author":"Tones","year":"2018","journal-title":"J Intellect Disabil Res"}],"container-title":["Brain and Development"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0387760420302400?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0387760420302400?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2025,10,8]],"date-time":"2025-10-08T19:49:33Z","timestamp":1759952973000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S0387760420302400"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2021,1]]},"references-count":88,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2021,1]]}},"alternative-id":["S0387760420302400"],"URL":"https:\/\/doi.org\/10.1016\/j.braindev.2020.08.014","relation":{},"ISSN":["0387-7604"],"issn-type":[{"value":"0387-7604","type":"print"}],"subject":[],"published":{"date-parts":[[2021,1]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"Epilepsy in Angelman syndrome: A scoping review","name":"articletitle","label":"Article Title"},{"value":"Brain and Development","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.braindev.2020.08.014","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"\u00a9 2020 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.","name":"copyright","label":"Copyright"}]}}