Hostname: page-component-586b7cd67f-dlnhk Total loading time: 0 Render date: 2024-11-25T01:34:30.645Z Has data issue: false hasContentIssue false
  • English
  • Français

Psychiatric disorders in a cohort of individuals with Prader–Willi syndrome

Published online by Cambridge University Press:  23 March 2020

L. Shriki-Tal ,
H. Avrahamy ,
Y. Pollak ,
V. Gross-Tsur ,
L. Genstil ,
H.J. Hirsch  and
F. Benarroch
L. Shriki-Tal
Affiliation:
PWS Multidisciplinary Clinic, Child Neurology Unit, Shaare Zedek Medical Center, POB 3235, 9103102Jerusalem, Israel Beer-Yaacov Nes-Ziona Mental Health Center, POB 1, 74100Nes-Ziona, Israel
H. Avrahamy
Affiliation:
PWS Multidisciplinary Clinic, Child Neurology Unit, Shaare Zedek Medical Center, POB 3235, 9103102Jerusalem, Israel
Y. Pollak
Affiliation:
PWS Multidisciplinary Clinic, Child Neurology Unit, Shaare Zedek Medical Center, POB 3235, 9103102Jerusalem, Israel School of Education, The Hebrew University of Jerusalem, Mount Scopus, 91905Jerusalem, Israel
V. Gross-Tsur
Affiliation:
PWS Multidisciplinary Clinic, Child Neurology Unit, Shaare Zedek Medical Center, POB 3235, 9103102Jerusalem, Israel School of Education, The Hebrew University of Jerusalem, Mount Scopus, 91905Jerusalem, Israel
L. Genstil
Affiliation:
PWS Multidisciplinary Clinic, Child Neurology Unit, Shaare Zedek Medical Center, POB 3235, 9103102Jerusalem, Israel
H.J. Hirsch
Affiliation:
PWS Multidisciplinary Clinic, Child Neurology Unit, Shaare Zedek Medical Center, POB 3235, 9103102Jerusalem, Israel
F. Benarroch*
Affiliation:
PWS Multidisciplinary Clinic, Child Neurology Unit, Shaare Zedek Medical Center, POB 3235, 9103102Jerusalem, Israel Herman Dana Division of Child and Adolescent Psychiatry, Hadassah-Hebrew University Medical Center, POB 24035, Mount Scopus, 91240Jerusalem, Israel
*
* Corresponding author. Dana Division of Child and Adolescent Psychiatry, Hadassah-Hebrew University Medical Center, Mount Scopus POB 24035, 91240 Jerusalem, Israel. E-mail address:[email protected] (F. Benarroch).
Get access

Abstract

Background:

Psychiatric manifestations in Prader–Willi Syndrome (PWS) are common and often are the most debilitating problem in these individuals. We present an epidemiological nation-wide survey of psychiatric diagnoses in the PWS population, based on full-range psychiatric interviews.

Methods:

We studied the distribution of psychiatric diagnoses (as opposed to a symptom-based approach) in the Israel national cohort of adolescents and adults with PWS. There was a total of 53 (32 males) ages 12 years and older. All individuals and their caretakers were interviewed using standardized psychiatric questionnaires. Demographic and clinical variables, Clinical Global Impression (CGI) score, IQ, severity of hyperphagia and quality of life (QOL) were also assessed and correlations with NPD (number of psychiatric diagnoses) calculated.

Results:

An overwhelming majority (89%) of the study participants had at least one psychiatric diagnosis. The most common were disruptive behavior disorders (DBD) (68%), obsessive compulsive disorder (OCD) (45%) and skin picking (35%). Individuals with DBD were at increased risk for OCD and skin picking. Psychotic disorders were found in 11%. NPD had a significant negative influence on QOL. There was no correlation between NPD and BMI, IQ, hyperphagia severity, hormonal profile or genetic subtypes.

Conclusions:

Psychiatric diagnoses are very frequent in PWS and strongly influence QOL. Furthermore, characterizing the profile of psychiatric comorbidity in PWS is crucial for planning effective interventions. Precise behavioral phenotyping in PWS in combination with a well-defined genetic etiology may aid biological research linking biological correlates to behavior.

Keywords

Prader–Willi syndromePsychiatryBehavior
Type
Original article
Information
European Psychiatry , Volume 44 , July 2017 , pp. 47 - 52
Copyright
Copyright © European Psychiatric Association 2017

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

References

Whittington, JEHolland, AJWebb, TButler, JClarke, DBoer, HPopulation prevalence and estimated birth incidence and mortality rate for people with Prader–Willi syndrome in one UK Health Region. J Med Genet 2001; 38(11): 792–798.CrossRefGoogle ScholarPubMed
Beardsmore, ADorman, TCooper, SAWebb, TAffective psychosis and Prader–Willi syndrome. J Intellect Disabil Res 1998; 42(Pt 6): 463–471.10.1046/j.1365-2788.1998.4260463.xCrossRefGoogle ScholarPubMed
Verhoeven, WMCurfs, LMTuinier, SPrader–Willi syndrome and cycloid psychoses. J Intellect Disabil Res 1998; 42(Pt 6): 455–462.CrossRefGoogle ScholarPubMed
Lanfranchi, SVianello, RLocus of control and family cohesion and adaptability in parents of children with Down, Williams Fragile X, Prader–Willi syndromes. Am J Intellect Dev Disabil 2012; 117(3): 207–224.CrossRefGoogle ScholarPubMed
Whitman, BYAccardo, PEmotional symptoms in Prader–Willi syndrome adolescents. Am J Med Genet 1987; 28(4): 897–905.CrossRefGoogle ScholarPubMed
Dykens, EMLeckman, JFCassidy, SBObsessions and compulsions in Prader–Willi syndrome. J Child Psychol Psychiatry 1996; 37(8): 995–1002.CrossRefGoogle ScholarPubMed
Clarke, DJBoer, HChung, MCSturmey, PWebb, TMaladaptive behaviour in Prader–Willi syndrome in adult life. J Intellect Disabil Res 1996; 40(Pt 2): 159–165.CrossRefGoogle ScholarPubMed
Clarke, DJBoer, HWhittington, JHolland, AButler, JWebb, TPrader–Willi syndrome, compulsive and ritualistic behaviours: the first population-based survey. Br J Psychiatry 2002; 180: 358–362.CrossRefGoogle ScholarPubMed
Skokauskas, NSweeny, EMeehan, JGallagher, LMental health problems in children with Prader–Willi syndrome. J Can Acad Child AdolescPsychiatry 2012; 21(3): 194–203.Google ScholarPubMed
Version 1.0, K-SADS-PL version 1.0, at National Institute of Neurological Disorders and Stroke; 1996.Google Scholar
Shalev, AAbramovitz, MKaplan De-Nour, KStructured clinical interview for axis DSM-IV-patient edition (Hebrew Version). Jerusalem, Israel Hadassah University Hospital, Center for Traumatic Stress, Department of Psychiatry; 1996.Google Scholar
Avrahamy, HPollak, YShriki-Tal, LGenstil, LHirsch, HJGross-Tsur, V, et al.A disease specific questionnaire for assessing behavior in individuals with Prader–Willi syndrome. Compr Psychiatry 2015; 58: 189–197.CrossRefGoogle ScholarPubMed
Kay, SRFiszbein, AOpler, LAThe positive and negative syndrome scale (PANSS) for schizophrenia. Schizophr Bull 1987; 13(2): 261–276.CrossRefGoogle Scholar
Guy, W editors. ECDEU assessment manual for psychopharmacology. Rockville, MD, U.S: Department of Health, Education and Welfare; 1976.Google Scholar
Wechsler, D3rd Ed, Wechsler Adult intelligence scale: administration and scoring manual, San Antonio: TX: The Psychological Corporation; 1997.Google Scholar
Wechsler, DFourth Ed, Wechsler Intelligence Scale for Children, San Antonio, TX: The Psychological Corporation; 2003.Google Scholar
Dykens, EMMaxwell, MAPantino, EKossler, RRoof, EAssessment of hyperphagia in Prader–Willi syndrome. Obesity (Silver Spring) 2007; 15(7): 1816–1826.10.1038/oby.2007.216CrossRefGoogle ScholarPubMed
Ware, JE Jr.Kosinski, MBjorner, JBTurner-Bowker, DMGandek, BMaruish, MEUser's manual for the SF-36v2TM health survey. In: Lincoln, RI editor. 2nd Ed, QualityMetric Incorporated; 2007.Google Scholar
Clarke, DPrader–Willi syndrome and psychotic symptoms: 2. A preliminary study of prevalence using the psychopathology assessment schedule for adults with developmental disability checklist. J Intellect Disabil Res 1998; 42(Pt 6): 451–454.CrossRefGoogle ScholarPubMed
Boer, HHolland, AWhittington, JButler, JWebb, TClarke, DPsychotic illness in people with Prader–Willi syndrome due to chromosome 15 maternal uniparental disomy. Lancet 2002; 359(9301): 135–136.CrossRefGoogle ScholarPubMed
Verhoeven, WMTuinier, SCurfs, LMPrader–Willi syndrome: the psychopathological phenotype in uniparental disomy. J Med Genet 2003;40(10):e112.CrossRefGoogle ScholarPubMed
Vogels, AMatthijs, GLegius, EDevriendt, KFryns, JPChromosome 15 maternal uniparental disomy and psychosis in Prader–Willi syndrome. J Med Genet 2003; 40(1): 72–73.CrossRefGoogle ScholarPubMed
Hiraiwa, RMaegaki, YOka, AOhno, KBehavioral and psychiatric disorders in Prader–Willi syndrome: a population study in Japan. Brain Dev 2007; 29(9): 535–542 [Epub 2007 Feb 20].CrossRefGoogle Scholar
Descheemaeker, MJGovers, VVermeulen, PFryns, JPPervasive developmental disorders in Prader–Willi syndrome: the Leuven experience in 59 subjects and controls. Am J Med Genet A 2006; 140(11): 1136–1142.CrossRefGoogle Scholar
Soni, SWhittington, JHolland, AJWebb, TMaina, EBoer, H, et al.The course and outcome of psychiatric illness in people with Prader–Willi syndrome: implications for management and treatment. J Intellect Disabil Res 2007; 51(Pt 1): 32–42.CrossRefGoogle ScholarPubMed
Kim, JWYoo, HJCho, SCHong, KEKim, BNBehavioral characteristics of Prader–Willi syndrome in Korea: comparison with children with mental retardation and normal controls. J Child Neurol 2005; 20(2): 134–138.CrossRefGoogle ScholarPubMed
Reddy, LAPfeiffer, SIBehavioral and emotional symptoms of children and adolescents with Prader–Willi Syndrome. J Autism Dev Disord 2007; 37(5): 830–839.10.1007/s10803-006-0210-2CrossRefGoogle ScholarPubMed
Lo, STCollin, PJHokken-Koelega, ACPsychiatric disorders in children with Prader–Willi syndrome-Results of a 2-year longitudinal study. Am J Med Genet A 2015; 167A(5): 983–991.CrossRefGoogle ScholarPubMed
de Ruiter, KFive-year development of psychopathology in young people with intellectual disabilities Amsterdam: Vrije Universiteit Amsterdam; 2013. p. 77–98.Google Scholar
Holland, AJWhittington, JEButler, JWebb, TBoer, HClarke, DBehavioural phenotypes associated with specific genetic disorders: evidence from a population-based study of people with Prader–Willi syndrome. Psychol Med 2003; 33(1): 141–153.CrossRefGoogle ScholarPubMed
Hodapp, RMDykens, EMMasino, LLFamilies of children with Prader–Willi syndrome: stress-support and relations to child characteristics. J Autism Dev Disord 1997; 27(1): 11–24.CrossRefGoogle ScholarPubMed
Dykens, EMHodapp, RMWalsh, KNash, LJAdaptive and maladaptive behavior in Prader–Willi syndrome. J Am Acad Child Adolesc Psychiatry 1992; 31(6): 1131–1136.CrossRefGoogle ScholarPubMed
Sinnema, MEinfeld, SLSchrander-Stumpel, C.T.R.M.Maaskant, MABoer, HCurfs, LMGBehavioral phenotype in adults with Prader–Willi syndrome. Res Dev Disabil 2011; 32: 604–612.CrossRefGoogle ScholarPubMed
Dykens, EMMaladaptive and compulsive behavior in Prader–Willi syndrome: new insights from older adults. Am J Ment Retard 2004; 109(2): 142–153.10.1352/0895-8017(2004)109<142:MACBIP>2.0.CO;22.0.CO;2>CrossRefGoogle ScholarPubMed
American Psychiatric Association. 4th ed, Diagnostic and statistical manual of mental disorders 2000 [Text rev].Google Scholar
Greaves, NPrince, EEvans, DWCharman, TRepetitive and ritualistic behaviour in children with Prader–Willi syndrome and children with autism. J Intellect Disabil Res 2006;50(Pt 2):92–100.10.1111/j.1365-2788.2005.00726.xCrossRefGoogle ScholarPubMed
Soni, SWhittington, JHolland, AJWebb, TMaina, ENBoer, H, et al.The phenomenology and diagnosis of psychiatric illness in people with Prader–Willi syndrome. Psychol Med 2008; 38(10): 1505–1514.CrossRefGoogle ScholarPubMed
Dykens, EMCassidy, SBKing, BHMaladaptive behavior differences in Prader–Willi syndrome due to paternal deletion versus maternal uniparental disomy. Am J Ment Retard 1999; 104(1): 67–77.2.0.CO;2>CrossRefGoogle ScholarPubMed
Veltman, MWThompson, RJRoberts, SEThomas, NSWhittington, JBolton, PFPrade–Willi syndrome – a study comparing deletion and uniparental disomy cases with reference to autism spectrum disorders. Eur Child Adolesc Psychiatry 2004; 13(1): 42–50.CrossRefGoogle Scholar
Pride, NAPayne, JMNorth, KNThe impact of ADHD on the cognitive and academic functioning of children with NF1. Dev Neuropsychol 2012; 37(7): 590–600.CrossRefGoogle ScholarPubMed
Schneider, MDebbané, MBassett, ASChow, EWFung, WLvan den Bree, M, et al.Psychiatric disorders from childhood to adulthood in 22q11.2 deletion syndrome: results from the International Consortium on Brain and Behavior in 22q11.2 Deletion Syndrome. Am J Psychiatry 2014; 171(6): 627–639.10.1176/appi.ajp.2013.13070864CrossRefGoogle Scholar
Morgan, JRStorch, EAWoods, DWBodzin, DLewin, ABMurphy, TKA preliminary analysis of the phenomenology of skin-picking in Prader–Willi syndrome. Child Psychiatry Hum Dev 2010; 41(4): 448–463.CrossRefGoogle ScholarPubMed
Gross-Tsur, VHirsch, HJBenarroch, FEldar-Geva, TThe FSH-inhibin axis in Prader–Willi syndrome: heterogeneity of gonadal dysfunction. Reprod Biol Endocrinol 2012;10:39.CrossRefGoogle ScholarPubMed
Submit a response

Comments

No Comments have been published for this article.