Severe/Profound
Intellectual Disability
Assessment and Diagnosis of Dementia
Adults with severe intellectual disability generally show major delays in development, often have the ability to understand speech but otherwise have limited communication skills, can learn simple daily routines and engage in simple self-care, but need supports in a supervised setting. Adults with profound intellectual disability often have congenital syndromes, cannot live independently, have very limited ability to communicate, and often have physical limitations and co-incident medical conditions. They require close supervision and help with self-care activities.
Source: Boat, T.F. & Wu, J.T. (2015). Clinical Characteristics of Intellectual Disabilities. National Library of Medicine. https://www.ncbi.nlm.nih.gov/books/NBK332877/
Determining the presence of dementia among adults with severe or profound intellectual disability
It is difficult to diagnose dementia in adults with severe or profound intellectual disability. Differentiating dementia from baseline level of functioning is challenging in adults with severe or profound intellectual disabilities. Moreover, informative studies on assessment methods and observable dementia symptoms are scarce. However, timely identification and diagnosis of dementia is helpful to permit a timely response to changing wishes and needs of affected adults. Clinicians have noted that diagnosing dementia in people with severe or profound intellectual disabilities is complicated. Most note that existing dementia screening instruments are generally unsuitable for this population, but studies have shown that select individual items of common instruments used with other adults with intellectual disability may apply (usually items associated with mobility and general functioning). Limited studies that examined symptoms and assessment techniques for discerning the presence of dementia have found that these areas were key: observed decline in activities of daily living (ADL) and functioning, behavioral, and psychological changes. Specifically, changes show as increased irritability, anxiety, apathy, and decreased eating/drinking abilities and diminution in verbal communication and/or mobility. Medical comorbidities are not necessarily key markers, except for the presence of epilepsy. Overall, the difference in decline features in adults with severe or profound intellectual disability, and potentially indicators of the presence of dementia, stem from behavioral changes rather than cognitive decline.
"...the difference in decline features in adults with severe or profound intellectual disability, and potentially indicators of the presence of dementia, stem from behavioral changes rather than cognitive decline."
Dementia in people with severe or profound intellectual (and multiple) disabilities: Focus group research into relevance, symptoms and training needs.
Differentiating dementia from baseline level of functioning is difficult among people with severe/profound intellectual (and multiple) disabilities. Moreover, studies on observable dementia symptoms are scarce. This study examined (a) the relevance of dementia diagnosis, (b) observable symptoms and (c) training/ information needs. Four explorative focus groups were held with care professionals and family members who have experience with people with severe/profound intellectual (and multiple) disabilities (≥40 years) and decline/dementia. Thematic analysis showed that participants wanted to know about a dementia diagnosis for a better understanding and to be able to make informed choices (question 1). Using a categorization matrix, cognitive and behavioral changes were shown to be most prominent (question 2). Participants indicated that they needed enhanced training, more knowledge development and translation, and supportive organizational choices/policies (question 3). Timely identifying/diagnosing dementia allows for a timely response to changing needs. This requires a better understanding of symptoms.
Source: Dekker, A.D., Wissing, M.B.G., Ulgiati, A.M., Bijl, B., van Gool, G., Groen, M.R., Grootendorst, E.S., van der Wal, I.A., Hobbelen, J.S.M., De Deyn, P.P., & Waninge, A. Dementia in people with severe or profound intellectual (and multiple) disabilities: Focus group research into relevance, symptoms and training needs. Journal of Applied Research in Intellectual Disability, 2021 Nov, 34(6), 1602-1617. https://doi.org/10.1111/jar.12912
Dementia in people with severe/profound intellectual (and multiple) disabilities: applicability of items in dementia screening instruments for people with intellectual disabilities
Diagnosing dementia in people with severe/profound intellectual (and multiple) disabilities (SPI(M)D) is complex. Whereas existing dementia screening instruments as a whole are unsuitable for this population, a number of individual items may apply. Therefore, this study aimed to identify applicable items in existing dementia screening instruments. Informant interviews about 40 people with SPI(M)D were conducted to identify applicable items in the Dementia Scale for Down Syndrome, Behavioral and Psychological Symptoms of Dementia in Down Syndrome II scale, Dementia Questionnaire for persons with Mental Retardation and Social competence Rating scale for people with Intellectual Disabilities. Among 193 items, 101 items were found applicable, categorized in β5 domains: behavioral and psychological functioning (60 items), cognitive functioning (25), motor functioning (6), activities of daily living (5) and medical comorbidities (5). Identifying applicable items for people with SPI(M)D is an essential step in developing a dedicated dementia screening instrument for this population.
Source: Wissing, M.B.G., Dijkstra, R., van der Wal, I.A., Grootendorst, E.S., Hobbelen, J.S.M., van der Putten, A.A.J., De Deyn, P.P., Waninge, A., Dekker, A.D. Dementia in people with severe/profound intellectual (and multiple) disabilities: applicability of items in dementia screening instruments for people with intellectual disabilities. Journal of Mental Health Research in Intellectual Disabilities, 2022, 15(4), 322-363. https://doi.org/10.1080/19315864.2022.2111737
Dementia in people with severe/profound intellectual (and multiple) disabilities: practice-based observations of symptoms
Observable dementia symptoms are hardly studied in people with severe/profound intellectual (and multiple) disabilities (SPI(M)D). Insight in symptomatology is needed for timely signaling/diagnosis. This study aimed to identify practice-based observations of dementia symptoms in this population.
Care professionals and family members were invited to complete a survey about symptoms. Quantitatively analyzed survey data were further deepened through semi-structured interviews with care professionals having vast experience in signaling/diagnosing dementia in this population. Symptoms were categorized using a symptom matrix. Survey respondents and interviewees frequently observed a decline in activities of daily living (ADL) functioning and behavioral and psychological changes, like increased irritability, anxiety, apathy, and decreased eating/drinking behavior. Cognitive symptoms were particularly recognized in persons with verbal communication and/or walking skills. To a lesser extent motor changes and medical comorbidities were reported. Increased insight in dementia symptoms contributes to developing a dedicated screening instrument for dementia in people with SPI(M)D.
Source: Wissing, M.B.G., Fokkens, A.S., Dijkstra, R., Hobbelen, J.S. M., van der Putten, A.A.J., De Deyn, P.P., Waninge, A., & Dekker, A.D. Dementia in people with severe/profound intellectual (and multiple) disabilities: practice-based observations of symptoms. Journal of Mental Health Research in Intellectual Disabilities, 2022, 15(4), 364-393. https://doi.org/10.1080/19315864.2022.2061092
Dementia in people with severe/profound intellectual disabilities
In people with severe or profound intellectual disabilities, it is difficult to diagnose dementia. As timely identification and diagnosis of dementia allows for a timely response to changing client wishes and needs, this study examined symptoms, and diagnosis of dementia in practice. Family members and professionals were invited to fill out survey about symptoms and diagnosis of dementia in people with severe or profound intellectual disabilities. Results of the survey were further explored within semi-structured interviews with professionals having experience with signaling and diagnosing dementia in these people. Symptoms found in the survey and transcripts of the interviews were qualitatively analyzed, using thematic analyses based on a developed symptom-matrix. The survey was filled out completely by 14 family members and 90 professionals with different backgrounds. Results showed that behavioral changes were recognized more frequently than cognitive decline. Compared to those without dementia, epilepsy and motor decline were more present in case of dementia. Fifteen interviews (until saturation) with professionals provided an in-depth view into the symptoms, and how to identify them, again stressing behavioral alterations and to a lesser extent cognitive symptoms.
Source: Waninge, A., Wissing, M., Hobbelen, H., Fokkens, A., Dekker, A., & De Deyn, P. Dementia in people with severe/profound intellectual disabilities. Journal of Applied Research in Intellectual Disabilities, 2021, 34(5), 1214-1215. https://doi.org/10.1111/jar.12917
Dementia symptoms in persons with severe/profound intellectual disability: expertise of practice.
Life expectancy of people with severe or profound intellectual disability (SPID) increases, which contributes to the risk of developing dementia. However, early detection and diagnosing dementia is complex, because of their low-level baseline functioning. Therefore, the aim is to identify observable dementia symptoms in adults with SPID in available literature. A systematic literature search, in line with PRISMA guidelines, was conducted in PubMed, PsycINFO and Web of Science using a combination of search terms for SPID, dementia/aging and aged population. In total, fifteen studies met inclusion criteria. Cognitive, behavioral and psychological symptoms (BPSD) and a decline in the ability to perform activities of daily living as well as neurological and physical changes were found. This presentation gives an overview of reported symptoms of (possible) dementia-related symptoms in SPID. Despite growing attention for dementia in people with ID in literature, only very few studies have studied dementia symptoms in SPID. Given the complexity of signaling and diagnosing dementia in SPID, dedicated studies are required to unravel the natural history of dementia in SPID, specifically focusing on observable symptoms for caregivers of (early) dementia in this population.
Source: Waninge, A., Wissing, M., Hobbelen, J., Fokkens, A., Dekker, A., & De Deyn, P. (2021). Dementia symptoms in persons with severe/profound intellectual disability: expertise of practice. 1214-1215. Abstract from 6th IASSIDD virtual Europe Congress, Amsterdam, Netherlands.
The neglected puzzle of dementia in people with severe/profound intellectual disabilities: A systematic literature review of observable symptoms
Dementia is increasingly prevalent in people with severe/profound intellectual disabilities. However, early detection and diagnosis of dementia is complex in this population. This study aimed to identify observable dementia symptoms in adults with severe/profound intellectual disabilities in available literature.
A systematic literature search was conducted in PubMed, PsycINFO and Web of Science with an exhaustive search string using a combination of search terms for severe/profound intellectual disabilities and dementia/ageing. Eleven studies met inclusion criteria. Cognitive decline, behavioral and psychological alterations, decline in activities of daily living as well as neurological and physical changes were found. Only a very limited number of studies reported symptoms ascribed to dementia in adults with severe/profound intellectual disabilities. Given the complexity of signalling and diagnosing dementia, dedicated studies are required to unravel the natural history of dementia in this population.
Source: Wissing MBG, Ulgiati AM, Hobbelen JSM, De Deyn PP, Waninge A, Dekker AD. The neglected puzzle of dementia in people with severe/profound intellectual disabilities: A systematic literature review of observable symptoms. J Appl Res Intellect Disabil. 2022 Jan;35(1):24-45. doi: 10.1111/jar.12920. Epub 2021 Jul 4. PMID: 34219327; PMCID: PMC9292142.