{"orpha":641496,"name":"Childhood-onset schizophrenia","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["COS","VEOS","Very early-onset schizophrenia"],"definition":"A rare neurologic disease with psychiatric involvement characterized by prominent pre-psychotic developmental disabilities (cognitive, language, motor), socio-communicative disturbances, auditory hallucinations (visual and tactile hallucinations are rarer) preceding psychotic symptoms, presenting before 13 years of age. Co-occurrence of neurodevelopmental disorders (e.g. autism spectrum disorders, attention deficit hyperactivity disorder) is frequent. Disease course is more severe than adult-onset form of the disease, with major neurodevelopmental impact.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-641496/","orphanet_url":"https://www.orpha.net/en/disease/detail/641496","names":{"fr":{"name":"Schizophrénie à début infantile","synonyms":["SDTP"],"definition":"Maladie neurologique rare avec atteinte psychiatrique caractérisée par d'importants troubles du développement prépsychotiques (cognitifs, langagiers, moteurs), des troubles socio-communicatifs et des hallucinations auditives (les hallucinations visuelles et tactiles sont plus rares) précédant des symptômes psychotiques, se manifestant avant l'âge de 13 ans. La co-occurrence de troubles du développement neurologique (troubles du spectre de l'autisme, trouble déficitaire de l'attention/hyperactivité) est fréquente. L'évolution de la maladie est plus sévère que celle de la forme adulte de la maladie, avec un impact majeur sur le développement neurologique."},"de":{"name":"Schizophrenie im Kindesalter","synonyms":["Schizophrenie, früh beginnende"],"definition":"Eine seltene neurologische Erkrankung mit psychiatrischer Beteiligung, die durch ausgeprägte präpsychotische Entwicklungsstörungen (kognitiv, sprachlich, motorisch), sozio-kommunikative Störungen und auditive Halluzinationen (visuelle und taktile Halluzinationen sind seltener) gekennzeichnet ist, die psychotischen Symptomen vorausgehen und vor dem 13. Lebensjahr auftreten. Häufig treten gleichzeitig neurologische Entwicklungsstörungen (z. B. Autismus-Spektrum-Störungen, Aufmerksamkeitsdefizit-Hyperaktivitätsstörung) auf. Der Krankheitsverlauf ist schwerwiegender als bei der Form der Erkrankung, die im Erwachsenenalter auftritt, mit erheblichen Auswirkungen auf die neurologische Entwicklung."},"es":{"name":"Esquizofrenia de inicio en la infancia","definition":"Es una enfermedad neurológica poco frecuente con afectación psiquiátrica caracterizada por destacada discapacidad prepsicótica del desarrollo (cognitiva, del lenguaje, motora), alteraciones sociocomunicativas, alucinaciones auditivas (las alucinaciones visuales y táctiles son menos comunes) que preceden a los síntomas psicóticos y que se presentan antes de los 13 años de edad. Es frecuente la coexistencia de trastornos del desarrollo neurológico (p. ej., trastornos del espectro autista, trastorno por déficit de atención e hiperactividad). El curso de la enfermedad es más grave que en la forma de inicio de la enfermedad en la edad adulta, con un importante impacto en el desarrollo neurológico."},"it":{"name":"Schizofrenia ad esordio infantile","synonyms":["COS","Schizofrenia ad esordio molto precoce","VEOS"]},"nl":{"name":"Schizofrenie beginnend op kinderleeftijd","synonyms":["COS","VEOS","Zeer vroeg optredende schizofrenie"],"definition":"Een zeldzame neurologische aandoening met psychiatrische betrokkenheid, gekenmerkt door opmerkelijke prepsychotische ontwikkelingsstoornissen (cognitief, taal, motorisch), socio-communicatieve stoornissen, auditieve hallucinaties (visuele en tactiele hallucinaties zijn zeldzamer) die voorafgaan aan psychotische symptomen, en die zich presenteren voor de leeftijd van 13 jaar. Vaak treden gelijktijdig neurologische ontwikkelingsstoornissen op (bijvoorbeeld autismespectrumstoornis, aandachtstekortstoornis met hyperactiviteit). Het ziekteverloop is ernstiger dan bij volwassen vormen van de ziekte, met ernstige impact op de neurologische ontwikkeling."},"pt":{"name":"Esquizofrenia com início na infância","synonyms":["COS","Esquizofrenia de início muito precoce","VEOS"],"definition":"Doença neurológica rara com envolvimento psiquiátrico caracterizada por alterações do desenvolvimento pré-psicóticas proeminentes (cognitivas, de linguagem, motoras), distúrbios sociocomunicativos, alucinações auditivas (alucinações visuais e táteis são mais raras) precedendo sintomas psicóticos, apresentando-se antes dos 13 anos de idade. A co-ocorrência de transtornos do neurodesenvolvimento (por exemplo, transtornos do espectro autista, transtorno do défice de atenção e hiperatividade) é frequente. O curso da doença é mais grave do que a forma de início na idade adulta da doença, com grande impacto no neurodesenvolvimento."},"pl":{"name":"Schizofrenia o początku w wieku dziecięcym","synonyms":["COS","VEOS","Very early-onset schizophrenia"],"definition":"Rzadka choroba neurologiczna z objawami psychiatrycznymi, w której występują zaburzenia rozwojowe (poznawcze, językowe, ruchowe), społeczno-komunikacyjne, halucynacje słuchowe (halucynacje wzrokowe i dotykowe są rzadsze) oraz poprzedzające objawy psychotyczne, stwierdzane przed 13 rokiem życia. Często współwystępują zaburzenia neurorozwojowe (np. zaburzenia ze spektrum autyzmu, zespół nadpobudliwości psychoruchowej z deficytem uwagi). Przebieg choroby jest cięższy u osób młodych niż u dorosłych, z istotnym wpływem na funkcję i rozwój układu nerwowego."},"cs":{"name":"Schizofrenie s nástupem v dětství","synonyms":["COS","VEOS","Very early-onset schizophrenia"]},"uk":{"name":"Шизофренія з дебютом у дитинстві","synonyms":["COS","VEOS","Дуже рання шизофренія"],"definition":"A rare neurologic disease with psychiatric involvement characterized by prominent pre-psychotic developmental disabilities (cognitive, language, motor), socio-communicative disturbances, auditory hallucinations (visual and tactile hallucinations are rarer) preceding psychotic symptoms, presenting before 13 years of age. Co-occurrence of neurodevelopmental disorders (e.g. autism spectrum disorders, attention deficit hyperactivity disorder) is frequent. Disease course is more severe than adult-onset form of the disease, with major neurodevelopmental impact."}},"xrefs":[{"source":"ICD-10","code":"F20.0","qualifier":"BTNT","icd_relation":"attributed","validated":true},{"source":"ICD-10","code":"F20.1","qualifier":"BTNT","icd_relation":"attributed","validated":true},{"source":"ICD-10","code":"F20.2","qualifier":"BTNT","icd_relation":"attributed","validated":true},{"source":"ICD-10","code":"F20.3","qualifier":"BTNT","icd_relation":"attributed","validated":true},{"source":"ICD-10","code":"F20.4","qualifier":"BTNT","icd_relation":"attributed","validated":true},{"source":"ICD-10","code":"F20.5","qualifier":"BTNT","icd_relation":"attributed","validated":true},{"source":"ICD-10","code":"F20.6","qualifier":"BTNT","icd_relation":"attributed","validated":true},{"source":"ICD-10","code":"F20.8","qualifier":"BTNT","icd_relation":"attributed","validated":true},{"source":"MONDO","code":"0957430","qualifier":"E","icd_relation":null,"validated":true},{"source":"MeSH","code":"D012561","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0036346","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[{"type":"Referred to","orpha":96369,"name":"OBSOLETE: Early-onset schizophrenia","status":"inactive"}],"preferential_parent":{"orpha":98006,"name":"Rare neurologic disease"},"parents":[{"orpha":98033,"name":"Rare neurologic disease with psychiatric involvement","classifications":[181]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Multigenic/multifactorial"],"onset":["Adolescent","Childhood"],"prevalence":[{"type":"Annual incidence","qualification":"Class only","class":"1-5 / 10 000","value":0.0,"area":"Europe","status":"Validated","source":"22906158[PMID]_ORPHANET"},{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Europe","status":"Validated","source":"22906158[PMID]_ORPHANET"}],"phenotypes":{"validation_status":"y","online":"y","validation_date":"2026-02-06","source":"PNDS 2022[OTHER]_30969686[PMID]_38492329[PMID]_39867751[PMID]","terms":[{"hpo":"HP:0000709","label":"Psychosis","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0000745","label":"Abnormal diminished volition","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0007018","label":"Attention deficit hyperactivity disorder","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0000729","label":"Autistic behavior","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0100543","label":"Cognitive impairment","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0000746","label":"Delusion","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:5200310","label":"Diminishment of social interactions","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0025769","label":"Disordered formal thought process","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0000738","label":"Hallucinations","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0033051","label":"Impaired executive functioning","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0011999","label":"Paranoia","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0033511","label":"Addictive substance use","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000716","label":"Depression","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0006892","label":"Frontotemporal cerebral atrophy","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001263","label":"Global developmental delay","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0006956","label":"Lateral ventricle dilatation","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002300","label":"Mutism","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0031589","label":"Suicidal ideation","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:5200330","label":"Suicide behaviors","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0025784","label":"Word salad","frequency":"Occasional (29-5%)","criteria":null}]}}