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Psychopathology and treatment of Prader-Willi syndrome in adulthood: About a clinical case

Published online by Cambridge University Press:  13 August 2021

G.M. Ruiz Martinez ,
A. Alvarado Dafonte  and
L. Soldado Rodriguez
G.M. Ruiz Martinez*
Affiliation:
Mental Health Unit, Complejo Hospitalario de Jaen, Jaen, Spain
A. Alvarado Dafonte
Affiliation:
Mental Health Unit, Complejo Hospitalario de Jaen, Jaen, Spain
L. Soldado Rodriguez
Affiliation:
Mental Health Unit, Complejo Hospitalario de Jaen, Jaen, Spain
*
*Corresponding author.

Abstract

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Introduction

Prader-Willi syndrome is a congenital disease caused by a genetic alteration of chromosome 15, described by doctors Prader, Labhart and Willi in 1956. It’s a rare disease (prevalence 2.8/100,000) and it occurs equally in both sexes and in all races. This disorder produces changes in hypothalamic function that can lead to muscle hypotonia, short stature, a compulsion to eat, and a lack of satiety. At the psychopathological level, the clinic is very varied, being mainly important the psychomotor retardation in different degrees and behavioral problems;especially in the behavioral phenotype. Affective and psychotic symptoms are also frequent.

Objectives

Psychopathology and treatment analysis through a clinical case.

Methods

40-year-old patient undergoing mental health follow-up since adolescence with a diagnosis of paranoid personality disorder. No medical history of interest. He was admitted to the hospitalization unit for serious behavioral alterations in a context of probable paranoid ideation towards the neighborhood. In the psychopathological examination, marked cognitive rigidity, high impulsivity and very low tolerance to frustration stand out, showing a hostile and defiant attitude. Poorly structured paranoid ideation. Presents obsessive-compulsive behaviors (scratching). Hyperphagia and obesity.

Results

Psychometric assessment (Waiss-IV): total IQ 61 (mild mental retardation). Genetics:deletion 15 q11-q13 of chromosome 15), confirms Prader-Willi diagnosis. Remission of behavioral disorders, suspiciousness and heteroaggressive behaviors with treatment with monthly depot paliperidone (150 mg).

Conclusions

Knowledge of the clinical and morphological characteristics of this syndrome would allow an early diagnosis and treat its possible complications as soon as possible. Antipsychotic treatment is effective in the management of behavioral and psychotic symptoms.

Disclosure

No significant relationships.

Keywords

Treatmentgeneticsprader-williPSYCHOPATOLOGY
Type
Abstract
Information
European Psychiatry , Volume 64 , Special Issue S1: Abstracts of the 29th European Congress of Psychiatry , April 2021 , pp. S723
Creative Commons
Creative Common License - CCCreative Common License - BY
This is an Open Access article, distributed under the terms of the Creative Commons Attribution licence (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted re-use, distribution, and reproduction in any medium, provided the original work is properly cited.
Copyright
© The Author(s), 2021. Published by Cambridge University Press on behalf of the European Psychiatric Association
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