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Consensus recommendations on mental health issues in Phelan-McDermid syndrome.
van Balkom, Ingrid D C; Burdeus-Olavarrieta, Monica; Cooke, Jennifer; de Cuba, A Graciela; Turner, Alison; Vogels, Annick; Maruani, Anna.
Affiliation
  • van Balkom IDC; Jonx, Department of (Youth) Mental Health and Autism, Lentis Psychiatric Institute, Groningen, the Netherlands; Rob Giel Research Centre, Department of Psychiatry, University Medical Center Groningen, Groningen, the Netherlands. Electronic address: i.d.c.van.balkom@umcg.nl.
  • Burdeus-Olavarrieta M; Department of Child and Adolescent Psychiatry, Institute of Psychiatry and Mental Health, Hospital General Universitario Gregorio Marañón, IiSGM, Instituto de Investigación Sanitaria Gregorio Marañón, Madrid, Spain; School of Psychology, Universidad Autónoma, Madrid, Spain.
  • Cooke J; Forensic and Neurodevelopmental Sciences Department, Institute of Psychiatry, Psychology, and Neuroscience, King's College London, United Kingdom.
  • de Cuba AG; Jonx, Department of (Youth) Mental Health and Autism, Lentis Psychiatric Institute, Groningen, the Netherlands.
  • Turner A; Phelan-McDermid Syndrome Foundation UK, 99 Highgate W Hill, London, N6 6NR, United Kingdom.
  • Vogels A; Centre for Human Genetics, University Hospital of Leuven, Leuven, Belgium.
  • Maruani A; Excellence Center for Autism Spectrum & Neurodevelopmental Disorders, Inovand, Child and Adolescent Psychiatry Department, Hôpital Robert Debre, Aphp, Paris, France; CRMR DICR, Rare Disease Center for Intellectual Disabilities, Defiscience, France.
Eur J Med Genet ; 66(6): 104770, 2023 Jun.
Article in En | MEDLINE | ID: mdl-37085014
Phelan-McDermid syndrome is a rare genetic condition caused by a deletion encompassing the 22q13.3 region or a pathogenic variant of the gene SHANK3. The clinical presentation is variable, but main characteristics include global developmental delay/intellectual disability (ID), marked speech impairment or delay, along with other features like hypotonia and somatic or psychiatric comorbidities. This publication delineates mental health, developmental and behavioural themes across the lifetime of individuals with PMS as informed by parents/caregivers, experts, and other key professionals involved in PMS care. We put forward several recommendations based on the available literature concerning mental health and behaviour in PMS. Additionally, this article aims to improve our awareness of the importance of considering developmental level of the individual with PMS when assessing mental health and behavioural issues. Understanding how the discrepancy between developmental level and chronological age may impact concerning behaviours offers insight into the meaning of those behaviours and informs care for individuals with PMS, enabling clinicians to address unmet (mental health) care needs and improve quality of life.
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Full text: 1 Collection: 01-internacional Database: MEDLINE Main subject: Mental Health / Chromosome Disorders Type of study: Guideline Aspects: Patient_preference Limits: Humans Language: En Journal: Eur J Med Genet Journal subject: GENETICA MEDICA Year: 2023 Document type: Article Country of publication: Netherlands

Full text: 1 Collection: 01-internacional Database: MEDLINE Main subject: Mental Health / Chromosome Disorders Type of study: Guideline Aspects: Patient_preference Limits: Humans Language: En Journal: Eur J Med Genet Journal subject: GENETICA MEDICA Year: 2023 Document type: Article Country of publication: Netherlands