Clinical phenotype of FOXP1 syndrome: parent-reported medical signs and symptoms in 40 individuals.

BACKGROUND: The first studies on patients with forkhead-box protein P1 (FOXP1) syndrome reported associated global neurodevelopmental delay, autism symptomatology, dysmorphic features and cardiac and urogenital malformations. The aim of this study was to assess the prevalence of congenital abnormalities in an unbiased cohort of patients with FOXP1 syndrome and to document rare complications. METHODS: Patients with FOXP1 syndrome were included, mostly diagnosed via whole-exome sequencing for neurodevelopmental delay. A parent-report questionnaire was used to assess medical signs and symptoms, including questions about features rated as most burdensome by patients and their family. RESULTS: Forty individuals were included, 20 females and 20 males. The mean age at assessment was 13.2 years (median 8.5 years; range 2-54 years; ≥18 years n = 7). Seven adults were included. All patients had developmental problems, including cognitive, communication, social-emotional and motor delays. The most prevalent medical signs and symptoms include delayed bladder control, sleeping problems, hypermetropia, strabismus, sacral dimple, undescended testes, abnormal muscle tone and airway infections. The most burdensome complaints for patients with FOXP1 syndrome, as perceived by parents, include intellectual disability, impaired communication, behaviour problems, lack of age-appropriate self-reliance, attention problems and anxiety. According to parents, patients have quite similar reported symptoms, although incontinence, obsessions and a complex sensory profile have a higher ranking. CONCLUSION: The results of this study may be used to further guide medical management and identify patient priorities for future research targeted on those features of FOXP1 syndrome that most impair quality of life of patients and their families.

Mental healthcare for adults with mild intellectual disabilities: population-based database study in Dutch mental health services.

BACKGROUND: Adults with mild intellectual disability (MID) experience more mental health disorders than the general population. However, mental healthcare may be insufficiently tailored to match their needs. Detailed information is lacking regarding care provided to people with MID in mental health services. AIMS: To compare mental health disorders and care provided to patients with and without MID in Dutch mental health services, including patients with missing MID status in the service files. METHOD: In this population-based database study, we used a Statistics Netherlands mental health service database, containing health insurance claims of patients who utilised advanced mental health services in 2015-2017. Patients with MID were identified by linking this database with Statistic Netherlands' social services and long-term care databases. RESULTS: We identified 7596 patients with MID, of whom 60.6% had no intellectual disability registration in the service files. Compared with patients without intellectual disability (n = 329 864), they had different profiles of mental health disorders. They received fewer diagnostic (odds ratio 0.71, 95% CI 0.67-0.75) and treatment activities (odds ratio 0.56, 95% CI 0.53-0.59), and required more interprofessional consultations outside of the service (odds ratio 2.06, 95% CI 1.97-2.16), crisis interventions (odds ratio 2.00, 95% CI 1.90-2.10) and mental health-related hospital admissions (odds ratio 1.72, 95% CI 1.63-1.82). CONCLUSIONS: Patients with MID in mental health services have different profiles of mental health disorders and care than patients without intellectual disability. In particular, fewer diagnostics and treatments are provided, especially in those with MID with no intellectual disability registration, putting patients with MID at risk of undertreatment and poorer mental health outcomes.

The European guideline on the assessment and diagnosis of psychiatric disorders in adults with intellectual disabilities

Background and objectives. In most countries worldwide, general adult psychiatrists look after the mental health of adults with intellectual disabilities (ID) without appropriate specialist training in the field. It is, therefore, necessary to develop a practice guideline to help these clinicians to assess and diagnose psychiatric disorders in adults with ID. Methods. We have developed an evidence and consensus-based practice guideline for the assessment and diagnosis of major functional psychiatric disorders in adults with ID. Several senior psychiatrists from different European countries formed a guideline development group who assessed the evidence gathered from a systematic literature search to produce the guideline. Results. Adults with ID develop the same psychiatric disorders as their non-ID counterparts. The overall rate of major functional psychiatric disorders such as schizophrenia, depressive disorder, bipolar disorder, and anxiety disorders seems to be somewhere between 14.4-22.4%. However, if a wider definition of psychopathology and mental ill-health is considered including depressive and anxiety symptoms, behaviours of concern, and other neurodevelopmental disorders such as autism spectrum disorder the rate becomes much higher than that in the general population. The risk factors for psychiatric disorders seem similar in both the ID and the non-ID populations. However certain risk factors such as genetic burden, certain comorbidities, psychosocial adversities, and reduced coping capacity are more prevalent among adults with ID. Conclusion. This guideline describes how the main symptoms of major psychiatric disorders may manifest differently in adults with ID and provides recommendations as to how to address these issues in day-to-day clinical practice.

Behavioural, psychiatric and psychosocial factors associated with aggressive behaviour in adults with intellectual disabilities: A systematic review and narrative analysis.

BACKGROUND: Aggressive behaviour is prevalent in people with intellectual disabilities. To understand the aetiology, it is important to recognize factors associated with the behaviour. METHOD: A systematic review was conducted and included studies published between January 2002 and April 2017 on the association of behavioural, psychiatric and psychosocial factors with aggressive behaviour in adults with intellectual disabilities. RESULTS: Thirty-eight studies were included that presented associations with 11 behavioural, psychiatric and psychosocial factors. Conflicting evidence was found on the association of these factors with aggressive behaviour. CONCLUSIONS: The aetiology of aggressive behaviour is specific for a certain person in a certain context and may be multifactorial. Additional research is required to identify contributing factors, to understand causal relationships and to increase knowledge on possible interaction effects of different factors.

Association of Borderline Intellectual Functioning and Adverse Childhood Experience with adult psychiatric morbidity. Findings from a British birth cohort.

BACKGROUND: To examine whether Borderline Intellectual Functioning (BIF) and Adverse Childhood Experiences independently predict adult psychiatric morbidity. METHODS: We performed a secondary analysis of longitudinal data derived from the 1970 British Birth Cohort Study to examine whether BIF and Adverse Childhood Experiences independently predict adult mental distress as measured by the Malaise Inventory. Factor analysis was used to derive a proxy measure of IQ from cognitive testing at age 10 or 5. Variables that could be indicators of exposure to Adverse Childhood Experiences were identified and grouped into health related and socio-economic related adversity. RESULTS: Children with BIF were significantly more likely than their peers to have been exposed to Adverse Childhood Experiences (BIF mean 5.90, non-BIF mean 3.19; Mann-Whitney z = 31.74, p < 0.001). As adults, participants with BIF were significantly more likely to score above the cut-off on the Malaise Inventory. We found statistically significant relationships between the number of socio-economic Adverse Childhood Experiences and poorer adult psychiatric morbidity (r range 0.104-0.141, all p < 001). At all ages the indirect mediating effects of Adverse Childhood Experiences were significantly related to adult psychiatric morbidity. CONCLUSIONS: The relationship between BIF and adult psychiatric morbidity appears to be partially mediated by exposure to Adverse Childhood Experiences. Where possible, targeting Adverse Childhood Experiences through early detection, prevention and interventions may improve psychiatric morbidity in this population group.

It is time to bring borderline intellectual functioning back into the main fold of classification systems.

Borderline intellectual functioning is an important and frequently unrecognised comorbid condition relevant to the diagnosis and treatment of any and all psychiatric disorders. In the DSM-IV-TR, it is defined by IQ in the 71-84 range. In DSM-5, IQ boundaries are no longer part of the classification, leaving the concept without a clear definition. This modification is one of the least highlighted changes in DSM-5. In this article we describe the history of the classification of borderline intellectual functioning. We provide information about it and on the importance of placing it in the right context and in the right place in future DSM editions and other classification systems such as the International Classification of Diseases.

Brief Symptom Inventory symptom profiles of outpatients with borderline intellectual functioning and major depressive disorder or posttraumatic stress disorder: Comparison with patients from regular mental health care and patients with Mild Intellectual Disabilities.

INTRODUCTION: In most countries, people with borderline intellectual functioning (BIF) are not considered a separate group in mental health care. There is little to no research on the impact of BIF on the presentation, nature and severity of mental health problems. The aim of the present exploratory study was to compare, in a naturalistic setting of patients referred to secondary care, symptom profiles of patients with BIF diagnosed with either major depressive disorder (MDD) or posttraumatic stress disorder (PTSD) to patients from regular mental health care (RMHC) and patients with Mild ID diagnosed with the same disorders. METHODS: We used a cohort of adolescent and adult outpatients (aged 16-88) with or without BIF diagnosed with a primary diagnosis MDD or PTSD. Primary outcome was the nature and severity of psychopathological symptoms assessed at baseline using the Brief Symptom Inventory. All outcomes were adjusted for gender and age. RESULTS: Results showed that BIF patients with a primary diagnosis MDD reported less severe symptoms on BSI Total and the subscales Depression, Obsession-Compulsion and Psychoticism than patients from regular mental health care (RMHC). There were no statistically significant differences in reported symptom severity on BSI Total and the different BSI subscales between BIF patients with PTSD and either patients from RMHC or patients with Mild ID. Patients Mild ID, did report significantly less severe symptoms on the subscale Depression and on the subscale Psychoticism than patients from RMHC. DISCUSSION: Since there were no other published studies into symptom profiles in patients with BIF compared to either patients with higher or lower levels of cognitive functioning, the study was mainly exploratory in nature, providing direction for future research. Results indicate that symptom profiles did not widely differ, but that there might be some characteristics unique to patients BIF separating them as a group from both patients from RMHC and patients with Mild ID.

The prevalence of personality disorders in psychiatric outpatients with borderline intellectual functioning: Comparison with outpatients from regular mental health care and outpatients with mild intellectual disabilities.

BACKGROUND: There is little research on the subject of personality disorder (PD) in individuals with borderline intellectual functioning (BIF). Unlike in most countries, in the Netherlands, patients with BIF are eligible for specialized mental health care. This offers the unique possibility of examining the rates of PDs in patients, who in other countries are treated relatively invisibly in regular mental health care. AIM: To compare, in a naturalistic setting, the frequency of PD diagnoses in outpatients with BIF with outpatients from regular mental health care and outpatients with mild ID. METHODS: We compared the rates of all DSM-IV-TR axis II PDs in outpatients with BIF (BIF group; n = 235) with rates of the same disorders in outpatients from regular mental health care (RMHC group; n = 1026) and outpatients with mild intellectual disability (ID) (mild ID group; n = 152) in a naturalistic cross-sectional anonymized medical chart review. RESULTS: Over half of the patients with BIF (52.8%) were diagnosed with a PD, compared with one in five in the RMHC group (19.3%) and one in three of the mild ID group (33.6%). All PD diagnoses, except for cluster A PDs and histrionic PDs, were most frequently diagnosed in the BIF group. PD NOS and borderline PD were the most frequently diagnosed PDs in BIF. The majority of PD patients had one or more comorbid axis I disorder. CONCLUSION: There is a high frequency of PD diagnoses in BIF outpatients in daily clinical practice. In anticipation of further scientific research, results suggest that PDs should not be overlooked in patients with BIF.

Psychiatric disorders in outpatients with borderline intellectual functioning: comparison with both outpatients from regular mental health care and outpatients with mild intellectual disabilities.

OBJECTIVE: In the Netherlands, patients with borderline intellectual functioning are eligible for specialized mental health care. This offers the unique possibility to examine the mix of psychiatric disorders in patients who, in other countries, are treated in regular outpatient mental health care clinics. Our study sought to examine the rates of all main Diagnostic and Statistical Manual of Mental Disorders, Fourth Edition, Text Revision, Axis I psychiatric diagnoses in outpatients with borderline intellectual functioning of 2 specialized regional psychiatric outpatient departments and to compare these with rates of the same disorders in outpatients from regular mental health care (RMHC) and outpatients with mild intellectual disabilities (IDs). METHOD: Our study was a cross-sectional, anonymized medical chart review. All participants were patients from the Dutch regional mental health care provider Rivierduinen. Diagnoses of patients with borderline intellectual functioning (borderline intellectual functioning group; n = 235) were compared with diagnoses of patients from RMHC (RMHC group; n = 1026) and patients with mild ID (mild ID group; n = 152). RESULTS: Compared with the RMHC group, psychotic and major depressive disorders were less common in the borderline intellectual functioning group, while posttraumatic stress disorder and V codes were more common. Compared with the mild ID group, psychotic disorders were significantly less common. CONCLUSION: Mental health problems in people with borderline intellectual functioning may not be well addressed in general psychiatry, or by standard psychiatry for patients with ID. Specific attention to this group in clinical practice and research may be warranted lest they fall between 2 stools.