{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,3]],"date-time":"2026-10-03T20:43:58Z","timestamp":1791060238654,"version":"4.1.0"},"reference-count":15,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2005,1,11]],"date-time":"2005-01-11T00:00:00Z","timestamp":1105401600000},"content-version":"vor","delay-in-days":10,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Epilepsia"],"published-print":{"date-parts":[[2005,1]]},"abstract":"<jats:p><jats:bold>Summary:\u2002<\/jats:bold><jats:italic>Purpose:<\/jats:italic>We investigated the evolution of epilepsy, seizure types, and effective drugs in Wolf\u2010Hirschhorn syndrome, which is a malformation syndrome often with refractory seizures and status epilepticus.<\/jats:p><jats:p><jats:italic>Methods:<\/jats:italic>We reviewed 11 cases of Wolf\u2010Hirschhorn syndrome (age range, 2\u201325 years; SD, 7.2 years) and who were treated in Osaka University or Osaka Medical Center of Research Institute for Maternal and Child Health.<\/jats:p><jats:p><jats:italic>Results:<\/jats:italic>In all patients, febrile or afebrile convulsions had developed. Epileptic seizures included alternative hemiconvulsions, generalized tonic\u2013clonic seizures, focal clonic seizures, tonic seizures, and epileptic spasms. Seizures were often induced by a high fever or a hot bath. Status epilepticus occurred in all patients, including one patient who died at the first status epilepticus. In some cases, intratracheal intubation was needed because of respiratory insufficiency. The effective antiepileptic drugs for long\u2010term use were sodium bromide (four of four), followed by clorazepate (CLP; one of two), and nitrazepam (NZP; two of four). Sodium bromide was particularly effective for preventing status epilepticus. The mean age of last status epilepticus in patients receiving sodium bromide (1 year 8 months) was significantly younger than that in those not treated with sodium bromide (3 year 4 months).<\/jats:p><jats:p><jats:italic>Conclusions:<\/jats:italic>We identified that, in most patients of Wolf\u2010Hirschhorn syndrome, the frequency of both seizures and status epilepticus decreased gradually after age 5 years. However, during infancy, status epilepticus sometimes resulted in permanent disability or even death. We propose that sodium bromide should be used as the initial treatment for the prevention of the development of status epilepticus associated with Wolf\u2010Hirschhorn syndrome.<\/jats:p>","DOI":"10.1111\/j.0013-9580.2005.02804.x","type":"journal-article","created":{"date-parts":[[2005,1,11]],"date-time":"2005-01-11T10:11:23Z","timestamp":1105438283000},"page":"150-155","source":"Crossref","is-referenced-by-count":30,"title":["Epilepsy in Wolf\u2010Hirschhorn Syndrome (4p\u2010)"],"prefix":"10.1111","volume":"46","author":[{"given":"Kuriko","family":"Kagitani\u2010Shimono","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Katsumi","family":"Imai","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kazumasa","family":"Otani","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Noriko","family":"Kamio","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Takeshi","family":"Okinaga","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yasuhisa","family":"Toribe","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yasuhiro","family":"Suzuki","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Keiichi","family":"Ozono","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2005,1,11]]},"reference":[{"key":"e_1_2_5_2_2","first-page":"479","article-title":"Deletion of short arms of chromosome 4\u20105 in a child with defects of midline fusion","volume":"1","author":"Hirschhorn K","year":"1965","journal-title":"Humangenetik"},{"key":"e_1_2_5_3_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1399-0004.1980.tb00167.x"},{"key":"e_1_2_5_4_2","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/6.2.317"},{"key":"e_1_2_5_5_2","first-page":"363","article-title":"4p\u2010 phenotype in an infant with t (4p\u2010; 19p or q+) mat translocation","volume":"129","author":"Neu RL","year":"1975","journal-title":"Am J Dis Child"},{"key":"e_1_2_5_6_2","first-page":"653","article-title":"Epileptic seizures in the 4p\u2010 syndrome: Report of two cases","volume":"45","author":"Kanazawa O","year":"1991","journal-title":"Jpn J Psychiatry Neurol"},{"key":"e_1_2_5_7_2","doi-asserted-by":"publisher","DOI":"10.1046\/j.1528-1157.2003.63502.x"},{"key":"e_1_2_5_8_2","doi-asserted-by":"publisher","DOI":"10.1542\/peds.103.4.830"},{"key":"e_1_2_5_9_2","doi-asserted-by":"crossref","first-page":"75","DOI":"10.1016\/S0065-3101(23)00074-9","article-title":"Wolf\u2010Hirschhorn (4p\u2010) syndrome","volume":"48","author":"Battaglia A","year":"2001","journal-title":"Adv Pediatr"},{"key":"e_1_2_5_10_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1528-1157.1995.tb01064.x"},{"key":"e_1_2_5_11_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0387-7604(88)80098-6"},{"key":"e_1_2_5_12_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1528-1157.1994.tb01780.x"},{"key":"e_1_2_5_13_2","first-page":"1634","volume-title":"Epilepsia: a comprehensive textbook","author":"Browne TR","year":"1998"},{"key":"e_1_2_5_14_2","doi-asserted-by":"publisher","DOI":"10.1086\/320609"},{"key":"e_1_2_5_15_2","doi-asserted-by":"publisher","DOI":"10.1006\/geno.1999.5881"},{"key":"e_1_2_5_16_2","doi-asserted-by":"publisher","DOI":"10.1007\/s002109900162"}],"container-title":["Epilepsia"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1111%2Fj.0013-9580.2005.02804.x","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/j.0013-9580.2005.02804.x","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,1,19]],"date-time":"2024-01-19T18:55:00Z","timestamp":1705690500000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/j.0013-9580.2005.02804.x"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2005,1]]},"references-count":15,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2005,1]]}},"alternative-id":["10.1111\/j.0013-9580.2005.02804.x"],"URL":"https:\/\/doi.org\/10.1111\/j.0013-9580.2005.02804.x","archive":["Portico"],"relation":{},"ISSN":["0013-9580","1528-1167"],"issn-type":[{"value":"0013-9580","type":"print"},{"value":"1528-1167","type":"electronic"}],"subject":[],"published":{"date-parts":[[2005,1]]}}}