{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,6]],"date-time":"2026-04-06T15:04:06Z","timestamp":1775487846501,"version":"3.50.1"},"reference-count":19,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2009,6,22]],"date-time":"2009-06-22T00:00:00Z","timestamp":1245628800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Develop Med Child Neuro"],"published-print":{"date-parts":[[2010,1]]},"abstract":"<jats:p><jats:bold>Aim\u2002<\/jats:bold> This study examines the overlap between children with bulbar cerebral palsy (Worster\u2013Drought syndrome [WDS]) and perisylvian polymicrogyria.<\/jats:p><jats:p><jats:bold>Method\u2002<\/jats:bold> A total of 121 children (81 males, 40 females; mean age 5y 5mo, SD 3y 6mo; age range 1mo\u201315y 4mo) were studied using retrospective clinical data and magnetic resonance imaging. In all, 70 children had WDS with normal perisylvian imaging, 31 had congenital bilateral perisylvian polymicrogyria (CBPP), and 20 had congenital unilateral perisylvian polymicrogyria (CUPP).<\/jats:p><jats:p><jats:bold>Results\u2002<\/jats:bold> All groups shared aetiological markers (male sex, congenital contractures, low familial incidence, excess antenatal events). There was a common phenotype of pseudobulbar palsy with mild limb pyramidal signs in all children with WDS, 90% of those with CBPP, and one\u2010third of those with CUPP, often also associated with learning disability<jats:ext-link xmlns:xlink=\"http:\/\/www.w3.org\/1999\/xlink\" xlink:href=\"#fn1\">*<\/jats:ext-link>, epilepsy, and behavioural difficulties. A further 15% of children with CUPP acquired this phenotype through an epileptic encephalopathy. Pseudobulbar palsy rather than polymicrogyria was more predictive of additional impairments other than epilepsy.<\/jats:p><jats:p><jats:bold>Interpretation\u2002<\/jats:bold> We propose that congenital perisylvian dysfunction is a spectrum encompassing the WDS phenotype and perisylvian polymicrogyria imaging abnormalities. As with other prenatal brain abnormalities, there is not necessarily concordance between imaging and clinical findings, although the phenotype is often more severe to manifest imaging abnormality. Clinical phenotype is the best indicator of prognosis. Epileptic encephalopathy can cause an acquired form of perisylvian dysfunction where there is.<\/jats:p>","DOI":"10.1111\/j.1469-8749.2009.03348.x","type":"journal-article","created":{"date-parts":[[2009,6,22]],"date-time":"2009-06-22T17:26:37Z","timestamp":1245691597000},"page":"33-39","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":17,"title":["Congenital perisylvian dysfunction \u2013 is it a spectrum?"],"prefix":"10.1111","volume":"52","author":[{"given":"MARIA","family":"CLARK","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"WUI K","family":"CHONG","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"TIM","family":"COX","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"BRIAN GR","family":"NEVILLE","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2009,12,18]]},"reference":[{"key":"e_1_2_8_2_2","doi-asserted-by":"publisher","DOI":"10.1017\/S0022215100053184"},{"key":"e_1_2_8_3_2","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1111\/j.1469-8749.1974.tb03419.x","article-title":"Congenital suprabulbar paresis","volume":"16","author":"Worster\u2010Drought C","year":"1974","journal-title":"Dev Med Child Neurol Suppl"},{"key":"e_1_2_8_4_2","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/123.10.2160"},{"key":"e_1_2_8_5_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1469-8749.2006.tb01233.x"},{"key":"e_1_2_8_6_2","doi-asserted-by":"publisher","DOI":"10.1002\/ajmg.a.32015"},{"key":"e_1_2_8_7_2","doi-asserted-by":"publisher","DOI":"10.1016\/0140-6736(93)90363-L"},{"key":"e_1_2_8_8_2","first-page":"788","article-title":"The changing MR imaging appearance of polymicrogyria: a consequence of myelination","volume":"24","author":"Takanashi J","year":"2003","journal-title":"Am J Neuroradiol"},{"key":"e_1_2_8_9_2","doi-asserted-by":"publisher","DOI":"10.1086\/339433"},{"key":"e_1_2_8_10_2","doi-asserted-by":"publisher","DOI":"10.1002\/ajmg.a.31443"},{"key":"e_1_2_8_11_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1528-1157.1999.tb01987.x"},{"key":"e_1_2_8_12_2","doi-asserted-by":"publisher","DOI":"10.1046\/j.1528-1157.2001.042007845.x"},{"key":"e_1_2_8_13_2","first-page":"185","volume-title":"Paediatric Epilepsy Syndromes and their Surgical Treatment","author":"Kuzniecky R","year":"1997"},{"key":"e_1_2_8_14_2","doi-asserted-by":"publisher","DOI":"10.1055\/s-2007-973700"},{"key":"e_1_2_8_15_2","first-page":"688","article-title":"Epileptic seizures, hemiplegia and hyperglycemia: late discovery of a localised cortical dysplasia","volume":"157","author":"Gastaut JL","year":"2001","journal-title":"Rev Neurol (Paris)"},{"key":"e_1_2_8_16_2","doi-asserted-by":"publisher","DOI":"10.1007\/s007870050128"},{"key":"e_1_2_8_17_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1528-1157.1991.tb04659.x"},{"key":"e_1_2_8_18_2","doi-asserted-by":"publisher","DOI":"10.1136\/jnnp.54.6.556"},{"key":"e_1_2_8_19_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.pediatrneurol.2005.11.003"},{"key":"e_1_2_8_20_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0387-7604(98)00097-7"}],"container-title":["Developmental Medicine &amp; Child Neurology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1111%2Fj.1469-8749.2009.03348.x","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/j.1469-8749.2009.03348.x","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,10,31]],"date-time":"2023-10-31T21:50:21Z","timestamp":1698789021000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/j.1469-8749.2009.03348.x"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2009,12,18]]},"references-count":19,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2010,1]]}},"alternative-id":["10.1111\/j.1469-8749.2009.03348.x"],"URL":"https:\/\/doi.org\/10.1111\/j.1469-8749.2009.03348.x","archive":["Portico"],"relation":{},"ISSN":["0012-1622","1469-8749"],"issn-type":[{"value":"0012-1622","type":"print"},{"value":"1469-8749","type":"electronic"}],"subject":[],"published":{"date-parts":[[2009,12,18]]},"assertion":[{"value":"2009-12-18","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}