Vincent Van Gogh Voor Geestelijke Gezondheidszorg

Birk-Barel syndrome (BIBARS), also known as KCNK9 imprinting syndrome, is an extremely rare genetic condition caused by pathogenic variants in the KCNK9 gene located on the long arm of chromosome 8 (8q24). This gene is paternally imprinted and therefore only expressed from the maternal allele. KCNK9 encodes for a member of the two-pore domain potassium channel (K2P) subfamily. BIBARS syndrome is characterized by congenital hypotonia and weakness of proximal muscles, intellectual disability, behavioural problems, dysmorphic features, feeding problems and epilepsy. The syndrome is implied in neurodevelopment, plays a role in various regulatory systems (eg, blood pressure maintenance, respiratory function, sleep, and cognitive function), and is associated with absence epilepsy. An institutionalized adult female patient with intellectual disability and challenging behaviours was presented whose history was carefully described and in whom extensive somatic, neurological, psychiatric and psychological investigation was performed, in addition to whole exome sequencing. Intellectual disability, developmental delay with severe psychotrauma from early age on, as well as severe aggressive and self-injurious behaviours were established. Whole exome sequencing finally disclosed a pathogenic variant in the KCNK9 gene and an absence epilepsy in association with BIBARS was suspected. After treatment with valproic acid epileptic phenomena no longer occurred and behaviour and emotion regulation improved significantly. A pathogenic heterozygous missense variant in the KCNK9 gene corresponding with a diagnosis of Birk-Barel syndrome was considered to be largely responsible for the severely disinhibited behaviours and causative for the absence epilepsy. For adequate diagnosis and treatment of neurodevelopmental disorders, this case clearly demonstrates the importance of adherence to clinical standards, in particular periodically renewed genetic analysis by means of WES/WGS.

Although there is more evidence in neuropsychological rehabilitation for transfer of treatment effects to daily life when using compensatory strategy training, computerized programs for patients with acquired brain injury still focus on function training. Therefore, we developed Karman Line Plan, a digitalized version of an evidence-based compensatory Goal Management Training intervention for individuals with brain injury and executive function problems. Karman Line Plan consists of a digital environment (Plan Game) and a mobile application (Plan Tool). This study aims to describe the design process and evaluate the usability and acceptance of Karman Line Plan. The program was developed using a three-phase standardized user-centred design approach. Results indicate that Karman Line Plan is perceived as user-friendly and valuable by both patients and therapists. The findings emphasize the importance of involving the target patient population early in the development process to allow for early adjustments and meet end-user needs.

Harmonization of neuropsychological assessment for vascular cognitive impairment and dementia (VCID) is important for ensuring the highest standards and consistency of diagnosis. A battery proposed in 2006 by the National Institute for Neurological Disorders and Stroke and the Canadian Stroke Network (NINDS-CSN) has received much international support. Considering significant advances in the field, including the rise of computerized and remote assessment methods, a revision is needed. To develop an updated harmonized battery and associated assessment guidelines for VCID. NINDS-CSN and other relevant published harmonized neuropsychological batteries, aided by literature review of recent developments in VCID, were used as reference points for an online Delphi survey (≥3 rounds, ≥75% threshold for agreement), including questions on a core test battery based on key cognitive domains that should be assessed for VCID, consideration of computerized and remote assessment methods, and assessment of diverse populations. International experts in neuropsychological assessment from diverse international regions were invited to participate in 2023. Data were analyzed from October 11, 2023, to June 20, 2024. A total of 44 experts participated in 3 survey rounds, with 28 to 31 participants each. Consensus was reached on a core assessment battery of neuropsychological tests based on key cognitive domains, and additional guidelines for a more comprehensive test battery, cognitive screening, telehealth and computerized assessment methods, principles for normative standardization, and the assessment of diverse populations. The key cognitive domains were harmonized with the International Society for Vascular Behavioural and Cognitive Disorders version 2 World Stroke Organization (VasCog-2-WSO) diagnostic criteria for VCID. This consensus statement describes the development of a harmonized neuropsychological assessment battery and guidelines for VCID (VasCog-NP) that expands on the NINDS-CSN battery with more comprehensive and flexible assessment of VCID. Harmonized with the VasCog-2-WSO diagnostic criteria, VasCog-NP could be adopted internationally to further help more consistent neuropsychological evaluations related to VCID, facilitating global comparisons for clinicians and researchers.

To examine the safety and efficacy of a brief cognitive dual-task (using eye movements) add-on module to treatment as usual (TAU) in reducing the severity and frequency of intrusive suicidal mental images and suicidal ideation. We conducted a single-blind, parallel multicenter randomized trial (No. NTR7563) among adult psychiatric outpatients (N = 91; Mage = 34.4, SD = 13.54; 68% female) with elevated depressive symptoms and experiencing distressing suicidal intrusions in the Netherlands. Primary outcome was the severity (Suicidal Intrusions Attributes Scale) and frequency (Clinical Interview for Suicidal Intrusions) of suicidal mental imagery intrusions at 1-week posttreatment and 3-month follow-up. Primary analysis was intention-to-treat. Between November 27, 2018 and September 13, 2021, 91 patients were included and randomly assigned to intervention group (Cognitive Dual Task Add-on + TAU) (n = 46) or TAU-only (n = 45). Cognitive Dual Task Add-on + TAU had greater reductions in severity (mean difference, -15.50, 95% CI [23.81, -7.19]; p < .001, d = 0.60), and frequency (geometric mean difference, 0.47, 95% CI [0.29, 0.79]; p = .004) of suicidal intrusions over time than TAU-alone. Cognitive Dual Task Add-on + TAU patients also showed lower suicidal ideation over time (p = .008, d = 0.42). There were no significant group differences in reductions in depressive symptoms, rumination, or hopelessness. Four serious adverse events occurred (three Cognitive Dual Task Add-on + TAU; one TAU-only); all unlikely attributable to intervention/trial. Findings provide support for the effectiveness of adding a cognitive dual-task module to the treatment of psychiatric outpatients with elevated depressive symptoms in reducing suicidal intrusions and ideation and can be executed safely. (PsycInfo Database Record (c) 2024 APA, all rights reserved).

The aims of the current study was to investigate the relation between the narcissistic personality syndrome (NPS) and schema modes, which refer to the predominant emotional state, schemas, and coping responses that are active for an individual at a particular time. Participants consisted of 25 clients who filled out the Schema Mode Inventory and 25 clinicians who provided assessment of NPS using the Shedler-Westen Assessment Procedure. This study showed a significant correlation between NPS and a lack of frustration tolerance (i.e., undisciplined child mode). There were no significant relations with self-aggrandizement (i.e., self-aggrandizer mode), feeling intensely angry (i.e., angry child and enraged child modes), and trying to suppress painful emotions by compulsively and excessively committing to distracting and soothing activities, such as abusing drugs (i.e., detached self-soother mode). This preliminary study contributes to our understanding of pathological narcissism in Dutch outpatient clients. We point to the importance of a self-reported lack of frustration tolerance as a potentially valuable diagnostic characteristic of pathological narcissism. We additionally emphasize the importance of diagnostic approaches based on clinical judgement when it comes to pathological narcissism.

Germline pathogenic variants in the RAS/mitogen-activated protein kinase (MAPK) signaling pathway are the molecular cause of RASopathies, a group of clinically overlapping genetic syndromes. RASopathies constitute a wide clinical spectrum characterized by distinct facial features, short stature, predisposition to cancer, and variable anomalies in nearly all the major body systems. With increasing global recognition of these conditions, the 8th International RASopathies Symposium spotlighted global perspectives on clinical care and research, including strategies for building international collaborations and developing diverse patient cohorts in anticipation of interventional trials. This biannual meeting, organized by RASopathies Network, was held in a hybrid virtual/in-person format. The agenda featured emerging discoveries and case findings as well as progress in preclinical and therapeutic pipelines. Stakeholders including basic scientists, clinician-scientists, practitioners, industry representatives, patients, and family advocates gathered to discuss cutting edge science, recognize current gaps in knowledge, and hear from people with RASopathies about the experience of daily living. Presentations by RASopathy self-advocates and early-stage investigators were featured throughout the program to encourage a sustainable, diverse, long-term research and advocacy partnership focused on improving health and bringing treatments to people with RASopathies.

Objectives One of the main reasons for people with dementia to move to a dementia special care unit of a nursing home is challenging behavior. This behavior is often difficult to manage, and in the Netherlands, residents are sometimes relocated to a severe challenging behavior specialized unit. However, relocation often comes with trauma and should be prevented if possible. This study aimed to investigate the patient- and context-related reasons for these relocations. Methods Qualitative multiple case study using individual (n = 15) and focus group interviews (n = 4 with n = 20 participants) were held with elderly care physicians, physician assistants, psychologists, nursing staff members, and relatives involved with people with dementia and severe challenging behavior who had been transferred to a severe challenging behavior specialized unit. Audio recordings were transcribed and analyzed with thematic analysis, including directed content analysis. Results After five cases, data-saturation occurred. The thematic analysis identified three main processes: increasing severity of challenging behavior, increasing realization that the clients’ needs cannot be met, and an increasing burden of nursing staff. The interaction between these processes, triggered mainly by a life-threatening situation, led to nursing staff reaching their limits, resulting in relocation of the client. Conclusion Our study resulted in a conceptual framework providing insight into reasons for relocation in cases of severe challenging behavior. To prevent relocation, the increasing severity of challenging behavior, increasing burden on nursing staff, and increasing realization that the clients’ needs cannot be met need attention.

In a visual environment, objects are encoded within a spatial and temporal context. The present study investigated whether incidental learning of spatial and temporal associations in hybrid visual and memory search enable observers to predict targets in space and time. In three experiments, observers looked for four, previously memorized, target items across many trials. We examined effects of learning target item sequences (e.g., the butterfly always follows the paint box), target item-location associations (the butterfly is always in the right corner), and target item-location sequences (the butterfly in the right corner always follows the paint box in the lower middle-left). We found only weak incidental learning for the sequences of target items alone. By contrast, we found good learning of target item-location associations. Furthermore, we did find a reliable effect of sequence learning for target item-location associations. These findings suggest that spatiotemporal learning in hybrid search is hierarchical: Only if spatial and non-spatial target features are bound, temporal associations can bias attention dynamically to the task-relevant features expected to occur next.

ABSTRACT Over the last decades, numerous memory interventions have been developed to mitigate memory decline in normal ageing. However, there is a large variability in the success of memory interventions, and it remains poorly understood which memory intervention programs are most effective and for whom. This is partially explained by the heterogeneity of memory intervention protocols across studies as well as often poor reporting of the study design. To facilitate a reporting framework that enables researchers to systemize the content and design of memory intervention paradigms, we developed the Classification Of MeMory InTerventions (COMMIT) tool using a 3-stage developmental process. Briefly, COMMIT was based on qualitative content analysis of already existing memory intervention studies published between April 1983 and July 2020, and iteratively validated by both internal and external expert panels. COMMIT provides an easily-applicable interactive tool that enables systematic description of memory intervention studies, together with instructions on how to use this classification tool. Our main goal is to provide a tool that enables the reporting and classification of memory interventions in a transparent, comprehensible, and complete manner, to ensure a better comparability between memory interventions, and, to ultimately contribute to the question which memory intervention shows the greatest benefits.