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While youths with intellectual disability (ID) have increased vulnerability for depressive disorders, cognitive problems and combined functional barriers make them less prone to receive adequate treatments. A systematic review of the literature was conducted (PROSPERO Registration number: CRD42022347703) based on several databases from 1980 to 2022 to examine the quality of tools for measuring depression in children and adolescents with ID. The COSMIN (COnsensus-based Standards for the selection of health status Measurement Instruments) checklist was used to assess several psychometric domains. Twelve studies evaluated the properties of six tools for measuring depression in youths with ID. The Center for Epidemiologic Studies Depression Scale-Intellectual Disability (CESD-ID) was the only scale with at least five domains of psychometric properties assessed to have strong or moderate evidence. Based on the reviewed findings, tools specifically developed for populations with developmental disabilities should be considered first in order to screen depression in youths with ID. Much work is required to confirm their validity in clinical samples with patients with a complex form of developmental disabilities. As a complement to self- and caregivers-report questionnaires, clinician rating scales were considered useful to catch the full picture of depression in youths with ID, in particular associated behavioral expressions. Their validity received little scrutiny and certainly deserve more attention to improve care practice of youths with ID.
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Individuals with neurodevelopmental disorders (NDDs) are more susceptible to developing severe or resistant mood disorders. However, they often face challenges in accessing specific treatments. To understand the barriers to treatment in this group, we conducted a secondary analysis of a multicenter observational study on 305 adolescents referred to specialized care (mean age 14.38 ± 0.13 years, 57% girls, 9% bipolar disorder, 91% depressive disorders). NDDs were categorized based on the number of co-occurring NDDs for each subject (none: 67%, one: 18%, two: 7%, three: 5%, four and more: 2%). We explored the predictive value of the number of NDDs on various clinical and therapeutic factors. The analysis revealed significant associations between the number of NDDs and the duration of mood symptoms, persistent irritability, associated disruptive behavioral disorders, lower scores on the Children's Global Assessment Scale (CGAS) at baseline, and polypharmacy. The number of NDDs did not correlate with the use of antidepressants, the Clinical Global Impression-Improvement scale (CGI-I) at discharge, or changes in the CGAS score during the stay. The challenges in distinguishing mood disorders from chronic emotional and behavioral difficulties may contribute to the difficulties in accessing specific care for mood disorders. These results suggest that treatments for mood disorders demonstrate comparable effectiveness when provided regardless of the associations with NDDs.
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Youths with severe and persistent irritability have a particularly high rate of school failures and learning difficulties. The aim of this study was to determine whether inpatient adolescents with Disruptive Mood Dysregulation Disorder (DMDD) have more motor and/or language impairments compared to patients with other psychiatric disorders. A retrospective chart review of all consecutive cases admitted in two adolescent inpatient units between January 2017 and December 2018 was conducted (N = 191). All patients received multi-disciplinary clinical and developmental assessments. For a subtest of subjects, additional standardized tests were used to document motor and language impairments. In this clinical chart 53 adolescents with a DMDD (mean age 13.6 ± 1.5, min 12, max 16, 70% males) were compared to patients with a major depressive disorder (MDD, n = 64, mean age 15.3 ± 1.6, 52% males) and patients with a non-mood disorder (NMD, n = 61, mean age 14.4 ± 1.55, 59% males). Among inpatients with DMDD, 71% had an associated motor and/or language disorder, with combined forms in around two-thirds of cases. Compared to youths with MDD, participants with DMDD were more likely to have an associated developmental coordination disorder (67% vs. 22%, OR = 4.7) and a written language disorder (35% vs. 10%, OR = 4.6). While 31% of inpatients with DMDD had an associated communication/oral language disorder, this rate was not statistically different from those observed in the MDD group (11%, OR = 3.2). The frequencies of motor and language impairments were not statistically different between participants in the DMDD group and in the NMD group. The high rate of motor and written language disorders found in DMDD patients may partly account for their academic difficulties. Such finding, if confirmed, supports systematic screening of motor and written language impairments in youths with chronic irritability and suggests remediation potential.
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Transtorno Depressivo Maior , Transtornos do Desenvolvimento da Linguagem , Adolescente , Transtornos de Deficit da Atenção e do Comportamento Disruptivo , Criança , Feminino , Humanos , Pacientes Internados , Humor Irritável , Masculino , Transtornos do Humor/diagnóstico , Transtornos do Humor/epidemiologia , Estudos RetrospectivosRESUMO
BACKGROUND: Borderline personality disorder (BPD) and history of prior suicide attempt (SA) have been shown to be high predictors for subsequent suicide. However, no previous study has examined how both factors interact to modify clinical and suicide severity among adolescents. METHODS: This study presents a comprehensive assessment of 302 adolescents (265 girls, mean age = 14.7 years) hospitalized after a SA. To test clinical interactions between BPD and history of prior SA, the sample was divided into single attempters without BPD (non-BPD-SA, N = 80), single attempters with BPD (BPD-SA, N = 127) and multiple attempters with BPD (BPD-MA, N = 95). RESULTS: Univariate analyses revealed a severity gradient among the 3 groups with an additive effect of BPD on the clinical and suicide severity already conferred by a history of SA. This gradient encompassed categorical (anxiety and conduct disorders and non-suicidal-self-injury [NSSI]) and dimensional comorbidities (substance use and depression severity) and suicide characteristics (age at first SA). According to regression analyses, the BPD-MA group that was associated with the most severe clinical presentation also showed specific features: the first SA at a younger age and a higher prevalence of non-suicidal self-injury (NSSI) and anxiety disorders. The BPD-MA group was not associated with higher impulsivity or frequency of negative life events. CONCLUSIONS: Based on these findings and to improve youth suicide prevention, future studies should systematically consider BPD and the efficacy of reinforcing early interventions for anxiety disorders and NSSI.
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Adolescente Hospitalizado , Transtorno da Personalidade Borderline , Comportamento Autodestrutivo , Adolescente , Transtornos de Ansiedade/epidemiologia , Transtorno da Personalidade Borderline/epidemiologia , Feminino , Humanos , Comportamento Autodestrutivo/epidemiologia , Tentativa de SuicídioRESUMO
Suicide attempts (SAs) are a public health concern in adolescence. A brief hospitalization is recommended, but access to inpatient wards is often not available. In addition, numerous risk factors for SA recurrence have been identified, but few studies have explored protective factors. Here, we aimed to assess the role of both risk and protective factors on SA relapse in a context of free access to inpatient services. We performed a prospective follow-up study of 320 adolescents who were hospitalized for an SA between January 2011 and December 2014 in France. Assessments at baseline included socio-demographics, clinical characteristics, temperament, reasons for living, spirituality, and coping. Patients were re-evaluated at 6 months and 12 months for depression severity and SA relapse. A total of 135 and 91 patients (78 girls, 12 boys, aged 13-17) were followed up at 6 and 12 months, respectively. At the 12-month follow-up, 28 (30%) subjects had repeated an SA. Adolescents who either had a history of SA or were receiving psychotropic treatment at baseline were at higher risk of recurrence. Several variables had a protective effect: (1) productive coping skills, namely, working hard and achieving, physical recreation, and seeking relaxing diversions; (2) a particular temperament trait, namely, cooperativeness; and (3) having experienced more life events. We also found a significant interaction: the higher the depression score during follow-up, the lower the protective effect of productive coping. Our findings confirm that a history of SA and seeking psychiatric care with medication are risk factors for SA relapse. However, productive coping strategies and cooperativeness are protective factors, and the improvement of such strategies as well as treatment of persisting depression should be a goal of psychotherapy treatment offered to suicidal adolescents.
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While many psychiatric disorders are associated with an increased risk for suicidal behaviors (SB) in children and adolescents, a few studies have explored the role of clinical symptoms based on a dimensional approach. Irritability is seen as a marker, a general psychopathology, and a symptom of both externalizing and internalizing disorders. In this review, we are interested in determining whether and how irritability can predict SB in youth. First, we reviewed consistencies and variation in the literature linking irritability to suicidal ideation (SI) and suicide attempt (SA). Second, based on the available models, we proposed specific mechanistic pathways, whereby irritability may modulate the risk for SB. Irritability has been found associated with SB both in cross-sectional and in longitudinal studies. The relation is consistent in different settings (i.e., general population and clinical settings) and across psychiatric disorders. The association is reduced but persists after adjusting for psychiatric disorder, including depression. On one hand, irritability constitutes a risk factor for SI via the onset of internalized disorder. On the other hand, irritable youth may be more prone to attempt suicide when experiencing SI. The measures for irritability were heterogeneous. A limited number of studies were designed to explore the role of mediators and/or moderators. Recognizing irritability in children and adolescents is a key issue with regards to suicide prevention.
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Humor Irritável/fisiologia , Ideação Suicida , Adolescente , Adulto , Criança , Estudos Transversais , Feminino , Humanos , Masculino , Fatores de Risco , Adulto JovemRESUMO
AIM: To explore the heterogeneity of Tourette syndrome as part of a neurodevelopmental spectrum. METHOD: Using hierarchical ascendant clustering based on tic symptoms, developmental milestones, and neurodevelopmental comorbidities, we analyzed the heterogeneity of Tourette syndrome phenotypes in a sample of 174 children and adolescents with Tourette syndrome referred to a tertiary university clinic. RESULTS: The model yielded three distinct clusters characterized as follows. In cluster 1, we found many neurodevelopmental comorbidities (including intellectual disabilities, autism spectrum disorder, attention-deficit-hyperactivity disorder [ADHD], and learning disabilities) and academic impairments. In cluster 2, patients had no other neurodevelopmental comorbidities. In cluster 3, patients had higher intelligence, a high frequency of attentional impairment, school problems related to both ADHD and unspecific attention difficulties, and handwriting problems related to the tics themselves. Interestingly, clusters did not differ in terms of family history or anxious-depressive comorbidities. The only other differences that emerged were related to prenatal or perinatal risk factors (more represented in cluster 1) and treatment profiles (higher rates of stimulants in cluster 1). INTERPRETATION: We conclude that from a phenotypical perspective, Tourette syndrome is a heterogeneous syndrome with at least three main clusters that may help in addressing the etiological basis of Tourette syndrome and specific rehabilitative and therapeutic approaches. WHAT THIS PAPER ADDS: The clustering of Tourette syndrome based on comorbidity with other neurodevelopmental conditions reveals three clusters. A group of patients with Tourette syndrome show school difficulties related to non-specific attention and writing problems. Analysing only children and adolescents helps to distinguish between developmental comorbid conditions and coexistent disorders.
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Transtornos do Neurodesenvolvimento/epidemiologia , Síndrome de Tourette/classificação , Síndrome de Tourette/complicações , Adolescente , Criança , Análise por Conglomerados , Estudos de Coortes , Feminino , França , Humanos , Masculino , Transtornos do Neurodesenvolvimento/diagnóstico , Síndrome de Tourette/psicologiaRESUMO
Maternal depression symptoms (MDS) are a robust risk factor for internalising problems (IP) in the offspring. However, the relative importance of MDS and other factors associated with it (i.e. other types of maternal psychopathology, maternal parenting practices, family characteristics) is not well understood. To (a) identify a group of children with high levels of IP between 6 and 12 years using combined maternal and teacher assessments and (b) to quantify the associations between trajectories of MDS during early childhood and children's IP trajectories before and after controlling for family factors associated with MDS. MDS and family factors were assessed in a population-based sample in Canada (n = 1537) between 5 months and 5 years. The outcome variable was membership in trajectories of teacher- and mother-rated IP between ages 6 and 12 years. Family factors were included as covariates in a multinomial logistic regression model. There was a strong association between MDS and children's atypically high levels of IP in unadjusted analyses [OR 4.14 (95% CI 2.60; 6.61)]. The association was reduced, but remained strong [2.60 (1.55; 4.36)] when maternal psychopathology, maternal parenting, and family socioeconomic status were entered in the model. MDS, maternal anxiety, and low parental self-efficacy were associated with offspring's high IP trajectories. MDS is associated with high levels of children's IP independently of other maternal and family characteristics. Intervention targeting maternal psychopathology and parenting self-efficacy and testing the impact on children's IP would provide information on the putative causal pathways between maternal and offspring's symptomatology.
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Depressão/psicologia , Saúde da Família/tendências , Mães/psicologia , Psicopatologia/métodos , Criança , Feminino , Humanos , Estudos Longitudinais , Masculino , Fatores de RiscoRESUMO
The aim of this literature review was to examine the evidence for psychotherapeutic and pharmacological treatments in subjects with severely dysregulated mood and to identify potential areas for improvements in research designs. A literature search was conducted using several databases for published (PubMed, PsycINFO) and ongoing (clinical trial registries) studies conducted in youths who met NIMH's criteria for Severe Mood Dysregulation (SMD) or the DSM-5 diagnosis of Disruptive Mood Dysregulation Disorder (DMDD). Eight completed studies were identified: three randomized trials, four open pilot studies and one case report. Seven ongoing studies were found in trial registries. The available evidence suggests potential efficacy of psychotherapies which have previously been developed for internalizing and externalizing disorders. The two main pharmacological strategies tested are, first, a monotherapy of psychostimulant or atypical antipsychotic such as risperidone, already used in the treatment of severe irritability in youths with developmental disorders; and second, the use of a serotonergic antidepressant as an add-on therapy in youths treated with psychostimulant. Ongoing studies will further clarify the effectiveness of psychotherapeutic interventions for DMDD individuals and whether they should be given alone or in conjunction with other treatments. The short duration of the trials for a chronic disorder, the low number of studies, the lack of placebo or active comparator arm, and restrictive inclusion criteria in most of the controlled trials dramatically limit the interpretation of the results. Finally, future research should be conducted across multiple sites, with standardized procedures to measure DMDD symptoms reduction, and include a run-in period to limit placebo effect.
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Medicina Baseada em Evidências/normas , Transtornos do Humor/psicologia , Transtornos do Humor/terapia , Adolescente , Antipsicóticos/uso terapêutico , Doença Crônica , Medicina Baseada em Evidências/métodos , Feminino , Humanos , Humor Irritável , Masculino , Transtornos do Humor/diagnóstico , Projetos Piloto , Psicoterapia/métodos , Psicoterapia/normas , Ensaios Clínicos Controlados Aleatórios como Assunto/métodos , Ensaios Clínicos Controlados Aleatórios como Assunto/normas , Risperidona/uso terapêuticoRESUMO
This study aimed to determine the prevalence and the clinical correlates of Adverse Childhood Experiences (ACEs) among 158 inpatient youths with two types of severe psychiatric disorders. ACEs were retrospectively collected with the ACEs scale and the List of Threatening Experiences Questionnaire in 77 patients hospitalized for a catatonic syndrome (average age 15.2 years) and 81 for a manic or mixed episode (average age 15.7 years). ACEs were frequent in youths suffering from bipolar disorder type I (BD-I) (58 %) and from catatonia (57 %), with around one quarter exposed to severe abuse (i.e., physical/sexual/emotional abuse or physical/emotional neglect). Youths with BD-I were more likely to be exposed to family violence compared to those with catatonia. Youths who had been exposed to ACEs did not exhibit a more severe presentation or a poorer response to treatment compared to others, either in the bipolar group or in the catatonic group.
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Transtorno Bipolar , Catatonia , Maus-Tratos Infantis , Violência Doméstica/psicologia , Exposição à Violência , Acontecimentos que Mudam a Vida , Adolescente , Adulto , Idade de Início , Transtorno Bipolar/diagnóstico , Transtorno Bipolar/epidemiologia , Transtorno Bipolar/psicologia , Catatonia/diagnóstico , Catatonia/epidemiologia , Catatonia/psicologia , Criança , Maus-Tratos Infantis/psicologia , Maus-Tratos Infantis/estatística & dados numéricos , Exposição à Violência/psicologia , Exposição à Violência/estatística & dados numéricos , Feminino , França/epidemiologia , Humanos , Pacientes Internados/psicologia , Pacientes Internados/estatística & dados numéricos , Masculino , Prevalência , Psicopatologia , Estudos Retrospectivos , Estatística como Assunto , Inquéritos e QuestionáriosRESUMO
In recent decades, ongoing research programmes on primary prevention and early identification of bipolar disorder (BD) have been developed. The aim of this article is to review the principal forms of evidence that support preventive interventions for BD in children and adolescents and the main challenges associated with these programmes. We performed a literature review of the main computerised databases (MEDLINE, PUBMED) and a manual search of the literature relevant to prospective and retrospective studies of prodromal symptoms, premorbid stages, risk factors, and early intervention programmes for BD. Genetic and environmental risk factors of BD were identified. Most of the algorithms used to measure the risk of developing BD and the early interventions programmes focused on the familial risk. The prodromal signs varied greatly and were age dependent. During adolescence, depressive episodes associated with genetic or environmental risk factors predicted the onset of hypomanic/manic episodes over subsequent years. In prepubertal children, the lack of specificity of clinical markers and difficulties in mood assessment were seen as impeding preventive interventions at these ages. Despite encouraging results, biomarkers have not thus far been sufficiently validated in youth samples to serve as screening tools for prevention. Additional longitudinal studies in youths at high risk of developing BD should include repeated measures of putative biomarkers. Staging models have been developed as an integrative approach to specify the individual level of risk based on clinical (e.g. prodromal symptoms and familial history of BD) and non-clinical (e.g. biomarkers and neuroimaging) data. However, there is still a lack of empirically validated studies that measure the benefits of using these models to design preventive intervention programmes.
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Transtorno Bipolar/diagnóstico , Transtorno Bipolar/terapia , Intervenção Médica Precoce/estatística & dados numéricos , Sintomas Prodrômicos , Adolescente , Transtorno Bipolar/psicologia , Criança , Diagnóstico Precoce , Humanos , Masculino , Estudos Prospectivos , Psicologia do Adolescente , Psicologia da Criança , Estudos RetrospectivosAssuntos
Inibidores da Captação Adrenérgica/uso terapêutico , Cloridrato de Atomoxetina/uso terapêutico , Transtorno da Conduta/tratamento farmacológico , Adolescente , Inibidores da Captação Adrenérgica/administração & dosagem , Inibidores da Captação Adrenérgica/efeitos adversos , Agressão , Cloridrato de Atomoxetina/administração & dosagem , Cloridrato de Atomoxetina/efeitos adversos , Atenção , Criança , Tolerância a Medicamentos , Feminino , Humanos , MasculinoRESUMO
We aimed to (1) describe the treatment used in a large sample of young inpatients with catatonia, (2) determine which factors were associated with improvement and (3) benzodiazepine (BZD) efficacy. From 1993 to 2011, 66 patients between the ages of 9 and 19 years were consecutively hospitalized for a catatonic syndrome. We prospectively collected sociodemographic, clinical and treatment data. In total, 51 (77%) patients underwent a BZD trial. BZDs were effective in 33 (65%) patients, who were associated with significantly fewer severe adverse events (p = 0.013) and resulted in fewer referrals for electroconvulsive therapy (ECT) (p = 0.037). Other treatments included ECT (N = 12, 18%); antipsychotic medications, mostly in combination; and treatment of an underlying medical condition, when possible. For 10 patients, four different trials were needed to achieve clinical improvement. When all treatments were combined, there was a better clinical response in acute-onset catatonia (p = 0.032). In contrast, the response was lower in boys (p = 0.044) and when posturing (p = 0.04) and mannerisms (p = 0.008) were present as catatonic symptoms. The treatment response was independent of the underlying psychiatric or systemic medical condition. As in adults, BZDs should be the first-line symptomatic treatment for catatonia in young patients, and ECT should be a second option. Additionally, the absence of an association between the response to treatment and the underlying psychiatric condition suggests that catatonia should be considered as a syndrome.
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Benzodiazepinas/uso terapêutico , Catatonia/diagnóstico , Catatonia/terapia , Eletroconvulsoterapia/métodos , Adolescente , Criança , Feminino , Hospitalização , Humanos , Pacientes Internados , Masculino , Estudos Prospectivos , Fatores Socioeconômicos , Síndrome , Resultado do Tratamento , Adulto JovemRESUMO
Juvenile mania is a concept widely developed but also highly debated since the 1990s. In the heart of this debate, Severe Mood Dysregulation (SMD) and "Temper Dysregulation disorder with Dysphoria" (recently integrated in DSM-5) showed their interest. Actually, the objective is to distinguish two clinical phenotypes in order to avoid confusion between (1) what would raise more of mood dysregulation with chronic manic like symptoms, and (2) bipolar disorder type I with episodic and acute manic episodes. Therapeutic stakes are major. In adolescents, even if DSM adult diagnostic criteria can be used and bipolar disorder type I clearly established, differential diagnostic at onset between acute manic episode and schizophrenia onset remain sometimes difficult to assess. Furthermore, it is crucial to better assess outcome of these adolescents, in terms of morbidity and potential prognosis factors, knowing that a younger age at onset is associated with a poorer outcome according to several adult studies. Therapeutic implications could then be drawn.
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Transtorno Bipolar/diagnóstico , Transtorno Bipolar/terapia , Adolescente , Antipsicóticos/uso terapêutico , Transtorno Bipolar/epidemiologia , Humanos , PsicoterapiaRESUMO
BACKGROUND: Previous pandemics have had negative effects on mental health, but there are few data on children and adolescents who were receiving ongoing psychiatric treatment. AIMS: To study changes in emotions and clinical state, and their predictors, during the COVID-19 pandemic in France. METHOD: We administered (by interview) the baseline Youth Self-Report version of the CoRonavIruS Health Impact Survey v0.3 (CRISIS, French translation) to 123 adolescent patients and the Parent/Caregiver version to evaluate 99 child patients before and during the first 'lockdown'. For 139 of these patients who received ongoing treatment in our centre, treating physicians retrospectively completed longitudinal global ratings for five time periods, masked to CRISIS ratings. RESULTS: The main outcome measure was the sum of eight mood state items, which formed a single factor in each age group. Overall, this score improved for each age group during the first lockdown. Clinician ratings modestly supported this result in patients without intellectual disability or autism spectrum disorder. Improvement of mood states was significantly associated with perceived improvement in family relationships in both age groups. CONCLUSIONS: Consistent with previous studies of clinical cohorts, our patients had diverse responses during the pandemic. Several factors may have contributed to the finding of improvement in some individuals during the first lockdown, including the degree of family support or conflict, stress reduction owing to isolation, limitations of the outcome measures and/or possible selection bias. Ongoing treatment may have had a protective effect. Clinically, during crises additional support may be needed by families who experience increased conflict or who care for children with intellectual disability.
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Efficacious intervention for severe, treatment-refractory self-injurious behavior and aggression (SIB/AGG) in children and adolescents with intellectual disability and concomitant psychiatric disorders remains a complex and urgent issue. The aim of this study is to assess the efficacy of electroconvulsive therapy (ECT) on severe and treatment-resistant SIB/AGG in young people with intellectual disability and current psychiatric disorder. We reviewed the charts of all patients (N = 4) who received ECT in the context of SIB/AGG with resistance to behavioral interventions, milieu therapy and pharmacotherapy from 2007 to 2011. We scored the daily rate of SIB/AGG per patient for each hospital day. Inter rater reliability was good (intraclass correlations = 0.91). We used a mixed generalized linear model to assess whether the following explanatory variables (time, ECT) influenced the course of SIB/AGG over time, the dependant variable. The sample included two girls and two boys. The mean age at admission was 13.8 years old [range 12-14]. The patients had on average 19 ECT sessions [range 16-26] and one patient received maintenance ECT. There was no effect of time before and after ECT start. ECT was associated with a significant decrease in SIB/AGG scores (p < 0.001): mean aggression score post-ECT was half the pre-ECT value. ECT appears beneficial in severe, treatment-resistant SHBA in adolescents with intellectual disability.
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Agressão/psicologia , Eletroconvulsoterapia/métodos , Deficiência Intelectual/complicações , Comportamento Autodestrutivo/complicações , Comportamento Autodestrutivo/terapia , Adolescente , Criança , Feminino , Humanos , Deficiência Intelectual/psicologia , Masculino , Reprodutibilidade dos Testes , Estudos Retrospectivos , Comportamento Autodestrutivo/psicologia , Resultado do TratamentoRESUMO
Introduction: Prader-Willi Syndrome (PWS) is a rare genetic condition, which affects one in 25,000 births and results in various phenotypes. It leads to a wide range of metabolic and endocrine disorders including growth delay, hypogonadism, narcolepsy, lack of satiety and compulsive eating, associated with mild to moderate cognitive impairment. Prognosis is especially determined by the complications of obesity (diabetes, cardiorespiratory diseases) and by severe behavioral disorders marked by impulsivity and compulsion. This heterogeneous clinical picture may lead to mis- or delayed diagnosis of comorbidities. Moreover, when diagnosis is made, treatment remains limited, with high interindividual differences in drug response. This may be due to the underlying genetic variability of the syndrome, which can involve several different genetic mutations, notably deletion or uniparental disomy (UPD) in a region of chromosome 15. Here, we propose to determine whether subjects with PWS differ for clinical phenotype and treatment response depending on the underlying genetic anomaly. Methods: We retrospectively included all 24 PWS patients who were referred to the Reference Center for Rare Psychiatric Disorders (GHU Paris Psychiatrie and Neurosciences) between November 2018 and July 2022, with either deletion (N = 8) or disomy (N = 16). The following socio-demographic and clinical characteristics were recorded: age, sex, psychiatric and non-psychiatric symptoms, the type of genetic defect, medication and treatment response to topiramate, which was evaluated in terms of eating compulsions and impulsive behaviors. We compared topiramate treatment doses and responses between PWS with deletion and those with disomy. Non-parametric tests were used with random permutations for p-value and bootstrap 95% confidence interval computations. Results: First, we found that disomy was associated with a more severe clinical phenotype than deletion. Second, we observed that topiramate was less effective and less tolerated in disomy, compared to deletion. Discussion: These results suggest that a pharmacogenomic-based approach may be relevant for the treatment of compulsions in PWS, thus highlighting the importance of personalized medicine for such complex heterogeneous disorders.