[Cancer in people with an intellectual disability in Germany: prevalence, genetics, and care situation]. / Krebserkrankungen bei Menschen mit einer Intelligenzminderung in Deutschland: Prävalenzen, Genetik und Versorgungslage.

Intellectual disability has a prevalence rate of approximately 1% of the population; in Germany, this is around 0.5-1 million people. The life expectancy of this group of people is reduced, with cancer being one of the most common causes of death (approx. 20%). Overall, the risk of cancer and mortality is increased compared to the general population.Certain genetic syndromes predispose to cancer in this vulnerable group, but associated comorbidities or lifestyle could also be risk factors for cancer. People with cognitive impairments are less likely to attend preventive check-ups, and challenges arise in medical care due to physical, communicative, and interactional characteristics. Optimized cooperation between clinical centers for people with disabilities and the respective cancer centers is required in order to tailor the processes to the individual.In Germany, there is a lack of data on the prevalence of cancer entities and the use and need for healthcare services. There is an urgent need to focus attention on cancer prevention, treatment, and research in the vulnerable and heterogeneous group of people with intellectual disabilities suffering from cancer in order to effectively counteract the increase in cancer-related deaths in this population group.The article summarizes specialist knowledge on cancer in people with an intellectual disability, identifies special features of treatment, presents care structures, and derives specific requirements for clinics and research.

[Psychotherapy in disorders of intellectual development-Current evidence and practical implementation]. / Psychotherapie bei Störungen der Intelligenzentwicklung ­ aktuelle Evidenz und praktische Umsetzung.

People with a disorder of intellectual development (German draft of the ICD-11, which came into force on 1 January 2022) suffer more frequently from mental illnesses. According to the international treatment guidelines multimodal approaches should include not only psychopharmacological treatment, but also disorder-specific psychotherapeutic methods. These psychotherapeutic interventions have to be adapted to the communicative and cognitive abilities (performance diagnostics with IQ tests) as well as the emotional developmental stage (developmental diagnostics, e.g., with the scale of emotional development, short version, SED-S 2; [1]). To ensure this, the rules of simple language should be observed and when appropriate relatives or caregivers should be involved in the therapeutic process. The effectiveness of cognitive behavioral therapy has received most scientific attention, especially for affective disorders. Posttraumatic stress disorders can be validly treated with eye movement desensitization and reprocessing (EMDR). There is also good evidence for exposure therapy with reinforcement in the treatment of anxiety disorders.

[Fetal Alcohol Spectrum Disorders in Adults - Results from a Diagnostic Outpatient Clinic]. / Fetale Alkoholspektrum-Störungen im Erwachsenenalter ­ Ergebnisse aus einer diagnostischen Sprechstunde.

AIM OF THE STUDY: Fetal alcohol spectrum disorders (FASD) are quite common and, due to the risk of psychiatric comorbidities, highly relevant until adulthood. Diagnostic clarification in adulthood is a prerequisite for targeted treatment and needs-based support. METHODS: In a German metropolitan region, 80 people with suspicion of FASD were assessed from May 2015 to July 2020. The results of this interdisciplinary diagnostic assessment were systematically evaluated and the clinical characteristics of the persons with or without FASD were analysed. RESULTS: Approximately 70% of the population accessing relevant health care was diagnosed with an entity from the FAS spectrum. People with FASD were more likely to have learning disabilities (50 vs. 33%) or intellectual disabilities (40 vs. 10%), while there were no group differences for age and gender. Psychiatric comorbidities, particularly depression (39%) and addiction disorders (31%), were common in both groups. CONCLUSION: As part of a multi-professional standardized diagnosis, FASD clarification is also possible and necessary in adulthood. The diagnostic criteria for FASD should be further evaluated and specified for adults.

Assessment of dementia in a clinical sample of persons with intellectual disability.

BACKGROUND: Assessment of age-associated disorders has become increasingly important. METHODS: In a clinical setting, people with intellectual disability with and without dementia were assessed retrospectively using the Neuropsychological Test Battery (NTB) and the Dementia Questionnaire for People with Learning Disabilities (DLD) at two different times to analyse neuropsychological changes and diagnostic validity. One group (n = 44) was assessed with both instruments, while the DLD was applied in 71 patients. RESULTS: In the NTB (n = 44), only patients with dementia (n = 26) showed a decline in the NTB total score and three subscales. Receiver operating characteristic analysis revealed a diagnostic sensitivity of .67, a specificity of .81, and an area under the curve (AUC) of .767. In the DLD group (n = 71), only those with dementia displayed a decrease in the cognitive and social scale; diagnostic sensitivity and specificity values were low (.61/.63) and the AUC was .704. CONCLUSIONS: Neuropsychological assessment was sensitive to detect cognitive changes over time. Sensitivity values of both instruments suggest a reassessment at a later time point.

[Mental health in persons with intellectual disability]. / Psychische Gesundheit bei Personen mit Intelligenzminderung.

People with intellectual disability (ID) have a high vulnerability to develop mental health problems. The prevalence of mental disorders is higher than in the general population and, in addition, adults with ID often show behavioral problems that imply a need for psychiatric psychotherapeutic care. In view of the impairments of intellectual functioning, impaired adaptive behavioral skills and physical illnesses, the needs of this target group are usually complex and require particular expertise. A number of specific assessment instruments are available for target group-specific diagnostics to collate the cognitive performance and emotional development as well as for psychopathological symptoms and behavioral disorders. To improve the accuracy of diagnosis in the ID population, existing alternatives to DSM or ICD should be applied especially to adults with moderate or severe ID. Guidelines for psychopharmacotherapy and adapted user guides for psychotherapeutic treatment provide support in the context of target group-specific treatment.

[Fetal alcohol spectrum disorders in adults]. / Fetale Alkoholspektrumstörungen bei Erwachsenen.

Fetal alcohol spectrum disorders (FASD) are a common cause of a congenital developmental disability acquired in the womb due to alcohol consumption by the mother during pregnancy. The physical and mental consequences persist into adulthood. The 4­digit code is an evidence-based method for diagnosing the full spectrum of outcomes, i.e. the full picture of fetal alcohol syndrome (FAS), partial fetal alcohol syndrome (pFAS), alcohol-related neurodevelopmental disorder (ARND) and alcohol-related birth defects (ARBD). The four key diagnostic features are (1) growth disorder, (2) facial dysmorphia, (3) central nervous system (CNS) structural and functional abnormalities and (4) prenatal alcohol exposure. Even if the disorder cannot be cured, supportive therapeutic interventions can improve the quality of life and independence and psychiatric comorbidities can be treated.

Standardizing the assessment of emotional development in adults with intellectual and developmental disability.

OBJECTIVE: The Scale of Emotional Development-Short (SED-S) is an instrument to assess the level of emotional development (ED) in people with intellectual and developmental disability. Index cases are developed as a didactic tool to standardize the application of the scale. METHOD: In a stepwise process, a European working group from six countries developed five index cases, one for each level of ED. All cases were first scored by 20 raters using the SED-S and then rephrased to reduce inter-rater variations (SD > 0.5). RESULTS: All five index cases yielded overall ratings that matched the intended level of ED. Across the range of ED, Regulating Affect needed rephrasing most to ensure a distinct description within each level of ED. CONCLUSIONS: The tri-lingual, cross-cultural evolution of five index cases contributes to a standardized application of the SED-S and can serve as training material to improve the inter-rater reliability of the SED-S across different cultures and languages.

[Autism spectrum disorders in adults with intellectual disabilities : Frequencies and characteristics]. / Autismusspektrumstörungen bei Erwachsenen mit Intelligenzminderung : Häufigkeiten und Charakteristika.

OBJECTIVE: Collation of frequencies and clinical characteristics of autism spectrum disorders (ASD) in persons with intellectual disability (ID). METHODS: Cross-sectional analysis of a clinical psychiatric sample of 710 adults with ID and mental disorders. RESULTS: The frequency of ASD in an adult sample with ID was 19%. The occurrence of ASD was associated with a higher severity of ID, male gender (in mild to moderate ID), anticonvulsive therapy and reduced employment rates in workshops. CONCLUSION: The ASD are a frequent clinical diagnosis in adults with ID.

[Emotional development, aggression regulation and challenging behavior in individuals with intellectual disbility]. / Emotionale Entwicklung, Aggressionsregulation und herausforderndes Verhalten bei Menschen mit Intelligenzminderung.

OBJECTIVES: Analysis of the causes of challenging behavior in individuals with intellectual disability (ID). METHODS: The relatedness and the impact of cognitive and emotional functioning on challenging behavior was investigated by correlation and regression analyses in 262 individuals with ID and mental disorders / challenging behavior. RESULTS: Despite the high correlation between ID and emotional development, cognitive-emotional developmental discrepancies were found in every second patient. The severity of challenging behavior was associated with a lower level of emotional development, especially in the area of "aggression regulation". CONCLUSION: The level of emotional development should be ascertained in the context of an assessment of challenging behavior in individuals with ID.

[Dementia in Adults with Down Syndrome: A Frequent Reason for Admission to Psychiatry]. / Demenz bei Down-Syndrom: Ein häufiger psychiatrischer Vorstellungsgrund.

INTRODUCTION: This study aimed to assess the prevalence and clinical characteristics of dementia in a clinical sample of adults with Down syndrome. Consequences for clinical practice were deduced. METHODS: Patient characteristics and prevalence rates of dementia were evaluated in adults with Down syndrome who were admitted to psychiatry from 2005 to 2012 (N = 75). RESULTS: In every third patient with Down syndrome, dementia was diagnosed in a second assessment 6 to 12 months after initial hospital admission. Patients with dementia were older and more often female, while no association was found with the level of intellectual disability. Thyroid function and calcium values were often abnormal in those with and without dementia. DISCUSSION: In persons with Down syndrome, dementia is a prevalent cause for admission to psychiatry, especially in females and those of advanced age. Services should be adapted to the increased demands.

An improvisational theatre intervention in people with intellectual disabilities and mental health problems.

People with intellectual disability (ID) generally tend to have difficulties expressing their thoughts and feelings verbally. Art-based therapies rely less on cognitive ability and verbal skills and aim to promote mental health. Drama therapy provides a playful framework to communicate problematic issues and to foster social skills. Improvisational theatre (improv) methods applied in therapy settings were reported to show positive effects on social competences and self-esteem. This pilot study investigates the feasibility (N = 24) and appropriateness (n = 12) of an intervention using improv methods in people with mild to moderate ID. Feasibility was measured on the basis of the average participation period, while appropriateness was assessed through a standardised patient satisfaction questionnaire (CSQ-8) and a self-developed questionnaire. The frequency of the applied methods was compared in two subgroups with participants requiring different support. The average participation rate of 19 months indicated a good feasibility. High CSQ-8 scores (M = 27.6/max. 32) and positive feedback on the self-developed questionnaire indicated the overall appropriateness. The frequency analysis of the applied methods reflected the adaptive nature of the intervention. Further research on the efficacy of improv methods contributing to mental health in people with ID is recommended.

Validation of the German version of the DSQIID in adults with intellectual disabilities.

BACKGROUND: An observer-rated screening questionnaire for dementia for people with intellectual disabilities (ID), DSQIID, was developed in the UK. So far, the German version has not yet been validated in adults with ID. AIMS/METHODS: We validated a German version of DSQIID (DSQIID-G) among adults with ID attending a German clinic. PROCEDURES/OUTCOMES: DSQIID-G was completed by the caregivers of 104 adults with ID at baseline (T1), 94 at six months (T2) and 83 at 12 months (T3). A Receiver Operating Curve (ROC) was used to determine the total DSQIID-G cutoff score for the best fit between sensitivity and specificity. RESULTS: Sixteen of the 104 participants at T1 (15%) received a diagnosis of dementia. At T1, the scores among the non-dementia group ranged from 0 to 33 (mean: 6.7; SD: 7.65), and the dementia group ranged from 3 to 43 (mean: 22.12; SD: 11.6). The intergroup difference was statistically significant (W: 158; p < .001) (AUC:.89). A total score of 9 provided the best fit between sensitivity (.94) and specificity (.72). CONCLUSIONS AND IMPLICATIONS: DSQIID-G total score can discriminate between dementia and non-dementia cases in adults with ID. A lower cutoff score with a higher sensitivity is desirable for a screening instrument.

[Autism Spectrum Disorders in Adulthood: Empirical Findings on Comorbidities, Differential Diagnoses, and the Clinical Utility of the AQ]. / Autismus-Spektrum-Störungen im Erwachsenenalter: Empirische Befunde zu Komorbiditäten, Differenzialdiagnosen und dem klinischen Nutzen des AQ.

AUTISM: spectrum disorders (ASD) often remain undiagnosed until adulthood. The aim of this study is to report differential diagnoses (DDX) and comorbidities of adults without intellectual impairment suspected of having an ASD and to test the self-assessment questionnaire Autism Quotient (AQ) for its suitability for screening. METHODS: DDX and comorbidities were recorded with standardized scales in an autism outpatient clinic in 106 individuals. The AQ was tested against the expert judgment of an interdisciplinary case conference using ROC analysis. RESULTS: Affective disorders were common in both groups (48%); other DDX were phobias (33%) and personality disorders (22%). The AQ showed an AUC of 0,527 with sensitivity/specificity of 70%/35%. CONCLUSIONS: Adults suspected of having autism are highly burdened by DDX and comorbidities. An interdisciplinary diagnostic procedure based on standardized scales is useful, whereas the AQ hardly differentiates between persons with and without ASD.

More than a Physical Problem: The Effects of Physical and Sensory Impairments on the Emotional Development of Adults with Intellectual Disabilities.

With the introduction of the ICD-11 and DSM-5, indicators of adaptive behavior, including social-emotional skills, are in focus for a more comprehensive understanding of neurodevelopmental disorders. Emotional skills can be assessed with the Scale of Emotional Development-Short (SED-S). To date, little is known about the effects of physical disorders and sensory impairments on a person's developmental trajectory. The SED-S was applied in 724 adults with intellectual disabilities, of whom 246 persons had an additional physical and/or sensory impairment. Ordinal regression analyses revealed an association of movement disorders with more severe intellectual disability and lower levels of emotional development (ED) on the overall and domain levels (Others, Body, Material, and Communication). Visual impairments predicted lower levels of ED in the SED-S domains Material and Body, but not the overall level of ED. Hearing impairments were not associated with intellectual disability or ED. Epilepsy correlated only with the severity of intellectual disability. Multiple impairments predicted more severe intellectual disabilities and lower levels of overall ED. In conclusion, physical and sensory impairments may not only affect physical development but may also compromise intellectual and emotional development, which should be addressed in early interventions.

Developmental Delays in Socio-Emotional Brain Functions in Persons with an Intellectual Disability: Impact on Treatment and Support.

Intellectual disability is a neurodevelopmental disorder with a related co-occurrence of mental health issues and challenging behaviors. In addition to purely cognitive functions, socio-emotional competencies may also be affected. In this paper, the lens of developmental social neuroscience is used to better understand the origins of mental disorders and challenging behaviors in people with an intellectual disability. The current concept of intelligence is broadened by socio-emotional brain functions. The emergence of these socio-emotional brain functions is linked to the formation of the respective neuronal networks located within the different parts of the limbic system. Thus, high order networks build on circuits that process more basic information. The socio-emotional skills can be assessed and complement the results of a standardized IQ-test. Disturbances of the brain cytoarchitecture and function that occur at a certain developmental period may increase the susceptibility to certain mental disorders. Insights into the current mental and socio-emotional functioning of a person may support clinicians in the calibration of treatment and support. Acknowledging the trajectories of the socio-emotional brain development may result in a more comprehensive understanding of behaviors and mental health in people with developmental delays and thus underpin supports for promotion of good mental health in this highly vulnerable population.

International clinician perspectives on pandemic-associated stress in supporting people with intellectual and developmental disabilities.

BACKGROUND: People living with intellectual and developmental disabilities (IDD) have suffered disproportionately in health outcomes and general well-being during the COVID-19 pandemic. There is emerging evidence of increased psychological distress. Increased strain has also fallen on clinicians managing the psychological needs of people with IDD, in the context of learning new technologies, staff shortages, reduced services and paused training opportunities. AIMS: To examine clinicians' experiences of patient care, clinical management and the impact of care delivery. METHOD: A mixed fixed-response and free-text survey comprising 28 questions covering four areas (responder demographics, clinical practice, changes to local services and clinician experiences) was developed, using the STROBE guidance. It was disseminated through an exponential snowballing technique to clinicians in seven high-income countries. Quantitative data were analysed and presented with Microsoft Excel. Qualitative data were coded and thematically analysed, and presented with in-text quotations. RESULTS: There were 139 respondents, mostly senior physicians (71%). Two-thirds reported over 10 years working in the field. Quantitative findings include increased clinician stress (77%), referrals (53%), patient distress presentations (>70%), patient isolation (73%) and carer burden (89%), and reduced patient participation in daily activities (86%). A third reported increased psychotropic prescribing. Qualitative analysis outlined changes to clinical practice, particularly the emergence and impact of telehealth. CONCLUSIONS: In the countries surveyed, the pandemic has not only had a significant impact on people with IDD, but also their carers and clinicians. A proactive, holistic international response is needed in preparedness for future public health emergencies.

The Autism-Competency-Group (AutCom). A promising approach to promote social skills in adults with autism spectrum disorder and intellectual disability.

Autism spectrum disorders (ASD) are often associated with intellectual disability (ID). ASD-specific group concepts usually focus on people on a high functioning level. The Autism-Competence-Group (AutCom) combines a psycho-educative approach with music and dance/movement interventions in adults with ASD and ID. AutCom includes 16 structured 90-minute sessions to foster social and emotional competencies. This study investigates the acceptability and effectiveness of AutCom. Practicability and acceptability were measured based on participation frequency and patient satisfaction (CSQ-8). Efficacy was assessed in a pre-post design (N = 12) based on self- and third-party assessment with a control group matched by gender and level of ID. Primary outcome variables were social and emotional competence, and secondary outcomes were challenging behavior and quality of life. A participation rate of 86% indicated practicability; high CSQ-8 scores (M = 30 of max. 32) indicated acceptability. Significant improvement was found in social competence compared to the control group and emotional competence in the pre-post self-assessment on the AutCom questionnaire. No significant improvement was found in challenging behavior and quality of life. AutCom is shown to be a promising and highly accepted group concept in fostering social and emotional skills in adults with ASD and ID.

[Validity of the Scale of Pervasive Developmental Disorder in a Clinical Sample of Adults with Intellectual Disability]. / Validierung der Skala zur Erfassung von Autismusspektrumstörung bei Minderbegabten (SEAS-M) an einer klinisch-psychiatrischen Stichprobe Erwachsener.

OBJECTIVES: The study assesses the diagnostic validity of the Scale of Pervasive Developmental Disorder in Mentally Retarded Persons (PDD-MRS). METHODS: Unblinded conduct of PDD-MRS in a clinical psychiatric sample of adults with intellectual disability (ID; N = 109) and suspect of autism. RESULTS: For a cut-off-score of 10, sensitivity was 89 %, specificity 62 % and the Area Under the Curve (AUC) 0.83. The total score was independent of age, gender and level of ID. However the diagnostic validity was better balanced in adults with mild to moderate ID (sensitivity/specificity: 85 %/73 %) compared to those with severe to profound ID (93 %/42 %). The SEAS-M total score was correlated with various ASD screening measures (r = .3-.6), while no correlation was found for scales assessing challenging behavior. CONCLUSION: To conclude, the PDD-MRS is a screening instrument for diagnosing autism in adults with mild to moderate ID. Knowledge of the PDD-MRS results within the consensus conference may have led to an overestimation of its diagnostic validity.

The Medical Care of People With Intellectual Disability.

BACKGROUND: An estimated 1.5 million persons in Germany are intellectually disabled. Persons with intellectual disability (ID) are especially vulnerable to somatic and mental illnesses. METHODS: This review is based on pertinent literature retrieved by selective searches in PubMed and the Cochrane Library. RESULTS: Genetic abnormalities are a frequent cause of diseases that affect multiple organs and need interdisciplinary treatment. A number of somatic diseases are more common in persons with ID than in the general population, including epilepsy (30-50% in persons with severe or very severe ID, vs. 0.5% in the general popu- lation) and dementia (five times more common than in the general population). Patients with Down syndrome are 20 times more likely than the general population to develop acute lymphoblastic leukemia. Some mental illnesses, too, are more common in persons with ID, e.g., autism spectrum disorders (7.5-15% vs. 1% in the general population). The history and the findings of the physical and psychiatric examination are assessed in accordance with the biopsychosocial model of disease, and in the light of the patient's mental developmental age. Structured instruments for behavioral evaluation and diagnosis are an important additional component of the diagnostic assessment. A holistic approach is required that takes multiple life areas into account and involves the patient's familial and social environment, while obeying the rules of simple language. Psychotherapeutic and psychosocial measures must be adapted to the patient's cognitive abilities and mental developmental age. CONCLUSION: Intellectually disabled persons can be treated in a multimodal, multiprofessional approach. As of early 2019, there were 38 medical centers for adults with intellectual disability or severe multiple disabilities in Germany (Medizinische Behandlungszentren für Erwachsene mit geistiger Behinderung oder schweren Mehrfachbehinderungen, MZEB), where they can be cared for with due attention to their special needs.

How is your mind-set? Proof of concept for the measurement of the level of emotional development.

BACKGROUND: In persons with intellectual and developmental disabilities, not only cognitive brain functions, but also socio-emotional processing networks may be impaired. This study aims to validate the Scale of Emotional Development-Short (SED-S) to provide an instrument for the assessment of socio-emotional brain functions. METHOD: The SED-S was applied in 160 children aged 0-12 years. Criterion validity was investigated at item and scale level in terms of the agreement between the scale classification and the child's chronological age. Additionally, interrater reliability and internal consistency were assessed. RESULTS: For the majority of items, the expected response pattern emerged, showing the highest response probabilities in the respective target age groups. Agreement between the classification of the different SED-S domains and chronological age was high (κw = 0.95; exact agreement = 80.6%). Interrater reliability at domain level ranged from κw = .98 to 1.00 and internal consistency was high (α = .99). CONCLUSION: The study normed the SED-S in a sample of typically developing children and provides evidence for criterion validity on item, domain and scale level.