{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,15]],"date-time":"2026-10-15T03:37:12Z","timestamp":1792035432905,"version":"4.4.0"},"reference-count":45,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2019,3,3]],"date-time":"2019-03-03T00:00:00Z","timestamp":1551571200000},"content-version":"vor","delay-in-days":2,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Epilepsia"],"published-print":{"date-parts":[[2019,3]]},"abstract":"<jats:title>Summary<\/jats:title><jats:sec><jats:title>Objective<\/jats:title><jats:p>To investigate the occurrence of psychosis and serious behavioral problems in females with protocadherin 19 gene (<jats:italic>PCDH19<\/jats:italic>) pathogenic variants.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>We evaluated whether psychosis and serious behavioral problems had occurred in 60 females (age 2\u201075\u00a0years) with<jats:italic><jats:styled-content style=\"fixed-case\">PCDH<\/jats:styled-content>19<\/jats:italic>pathogenic variants belonging to 35 families. Patients were identified from epilepsy genetics databases in Australia, New Zealand, the<jats:styled-content style=\"fixed-case\">United States,<\/jats:styled-content>and Canada. Neurologic and psychiatric disorders were diagnosed using standard methods.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Eight of 60 females (13%) from 7 families developed a psychotic disorder: schizophrenia (6), schizoaffective disorder (1), or an unspecified psychotic disorder (1). Median age at onset of psychotic symptoms was 21\u00a0years (range 11\u201028\u00a0years). In our cohort of 39 females aged 11\u00a0years or older, 8 (21%) developed a psychotic disorder. Seven had ongoing seizures at onset of psychosis, with 2 continuing to have seizures when psychosis recurred. Psychotic disorders occurred in the setting of mild (4), moderate (2), or severe (1) intellectual disability, or normal intellect (1). Preexisting behavioral problems occurred in 4 patients, and autism spectrum disorder in 3. Two additional females (3%) had psychotic features with other conditions: an adolescent had recurrent episodes of postictal psychosis, and a 75\u2010year\u2010old woman had major depression with psychotic features. A further 3 adolescents (5%) with moderate to severe intellectual disability had onset of severe behavioral disturbance, or significant worsening.<\/jats:p><\/jats:sec><jats:sec><jats:title>Significance<\/jats:title><jats:p>We identify that psychotic disorders, including schizophrenia, are a later\u2010onset manifestation of<jats:italic><jats:styled-content style=\"fixed-case\">PCDH<\/jats:styled-content>19<\/jats:italic>Girls Clustering Epilepsy. Affected girls and women should be carefully monitored for later\u2010onset psychiatric disorders.<\/jats:p><\/jats:sec>","DOI":"10.1111\/epi.14678","type":"journal-article","created":{"date-parts":[[2019,3,4]],"date-time":"2019-03-04T01:14:57Z","timestamp":1551662097000},"page":"429-440","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":39,"title":["Schizophrenia is a later\u2010onset feature of<i><scp>PCDH<\/scp>19<\/i>Girls Clustering Epilepsy"],"prefix":"10.1111","volume":"60","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-6820-8268","authenticated-orcid":false,"given":"Danique R. 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