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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).
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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

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