Dementia in People with Severe/Profound Intellectual (and Multiple) Disabilities: Practice-Based Observations of Symptoms

References

• Aller-Alvarez, J. S., Menéndez-González, M., Ribacoba-Montero, R., Salvado, M., Vega, V., Suárez-Moro, R., Sueiras, M., Toledo, M., Salas-Puig, J., & Álvarez-Sabin, J. (2017). Myoclonic epilepsy in Down syndrome and Alzheimer disease. Neurología (English Edition), 32(2), 69–73. https://doi.org/10.1016/j.nrleng.2014.12.019
   PubMed Web of Science ®Google Scholar
• Altuna, M., Giménez, S., & Fortea, J. (2021). Epilepsy in Down syndrome: A highly prevalent comorbidity. Journal of Clinical Medicine, 10(13), 1–17. https://doi.org/10.3390/jcm10132776
   Web of Science ®Google Scholar
• Alzheimer’s Association. (2021). 2021 Alzheimer’s disease facts and figures. Alzheimer’s and Dementia, 17(3), 327–406. https://doi.org/10.1002/alz.12328
   PubMed Web of Science ®Google Scholar
• American Psychiatric Association. (2013). Diagnostic and statistical manual of mental disorders (5th ed.) Washington, DC: Author.
   Google Scholar
• Ball, S. L., Holland, A. J., Huppert, F. A., Treppner, P., Watson, P., & Hon, J. (2004). The modified CAMDEX informant interview is a valid and reliable tool for use in the diagnosis of dementia in adults with Down’s syndrome. Journal of Intellectual Disability Research, 48(6), 611–620. https://.doi.org/10.1111/j.1365-2788.2004.00630.x.
   PubMedGoogle Scholar
• Benejam, B. (2009). Dementia symptoms in Down syndrome. SD-DS International Medical Journal on down Syndrome, 13(2), 18–21. https://doi.org/10.1016/s2171-9748(09)70018-2
   Google Scholar
• Bittles, A. H., & Glasson, E. J. (2004). Clinical, social, and ethical implications of changing life expectancy in Down syndrome. Developmental Medicine and Child Neurology, 46(4), 282–286. https://doi.org/10.1111/j.1469-8749.2004.tb00483.x
   PubMed Web of Science ®Google Scholar
• Boyce, C., & Neale, P. (2006). Conducting in-depth interviews: A guide for designing and conducting in-depth interviews. Pathfinder International Tool Series, Monitoring And Evaluation-2. https://doi.org/10.1080/14616730210154225
   Google Scholar
• Burt, D. B., Loveland, A. L., Primeaux-Hart, S., Chen, Y. W., Phillips, N. B., Cleveland, L. A., Lewis, K. R., Lesser, J., & Cummings, E. (1998). Dementia in adults with Down syndrome: Diagnostic challenges. American Journal on Mental Retardation, 103(2), 130–155. https://doi.org/10.1352/0895-8017(1998)103<0130:DIAWDS>2.0.CO;2
   PubMedGoogle Scholar
• Cleary, J., & Doody, O. (2017). Professional carers’ experiences of caring for individuals with intellectual disability and dementia. Journal of Intellectual Disabilities, 21(1), 68–86. https://doi.org/10.1177/1744629516638245
   PubMed Web of Science ®Google Scholar
• Cooper, S. A., & Smiley, E. (2007). The prevalence, incidence and factors predictive of mental ill-health in adults with profound intellectual disabilities. Prospective Study. Journal of Applied Research in Intellectual Disabilities, 20(6), 505–509 https://.doi.org/10.1111/j.1468-3148.2007.00403.x.
   Web of Science ®Google Scholar
• Coppus, A. M. W. (2013). People with intellectual disability: What do we know about adulthood and life expectancy? Developmental Disabilities Research Reviews, 18(1), 6–16. https://doi.org/10.1002/ddrr.1123
   PubMed Web of Science ®Google Scholar
• Day, K. (1985). Psychiatric disorder in the middle-aged and elderly mentally handicapped. British Journal of Psychiatry, 147(6), 660–667. https://doi.org/10.1192/bjp.147.6.660
   PubMedGoogle Scholar
• Dekker, A. D., Strydom, A., Coppus, A. M. W., Nizetic, D., Vermeiren, Y., Naudé, P. J. W., Van Dam, D., Potier, M. C., Fortea, J., & De Deyn, P. P. (2015). Behavioural and psychological symptoms of dementia in Down syndrome: Early indicators of clinical Alzheimer’s disease? Cortex, 73, 36–61. https://doi.org/10.1016/j.cortex.2015.07.032
   PubMed Web of Science ®Google Scholar
• Dekker, A. D., Ulgiati, A. M., Groen, H., Boxelaar, V. A., Sacco, S., Falquero, S., Carfi, A., Paola, A. D., Benejam, B., Valldeneu, S., Fopma, R., Oosterik, M., Hermelink, M., Beugelsdijk, G., Schippers, M., Henstra, H., Scholten-Kuiper, M., Willink-Vos, J., Ruiter, L. D., & Deyn, P. P. D. (2021). The behavioral and psychological symptoms of dementia in down syndrome scale (BPSD-DS II): Optimization and further validation. Journal of Alzheimer’s Disease, 81(4), 1505–1527. https://doi.org/10.3233/JAD-201427
   PubMed Web of Science ®Google Scholar
• Dekker, A. D., Vermeiren, Y., Beugelsdijk, G., Schippers, M., Hassefras, L., Eleveld, J., Grefelman, S., Fopma, R., Bomer-Veenboer, M., Oosterling, G. D. E., Scholten, E., Tollenaere, M., Van Goethem, G., Zu Eulenburg, C., Coppus, A. M. W. W., De Deyn, P. P., Sacco, S., Carfi, A., Benejam, B., & De Deyn, P. P. (2018). The behavioral and psychological symptoms of dementia in down syndrome (BPSD-DS) scale: Comprehensive assessment of psychopathology in down syndrome. Journal of Alzheimer’s Disease, 49(5), 187–205. https://doi.org/10.3233/JAD-170920
   Google Scholar
• 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. (2021). 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 Disabilities, 34(1), 1–16. https://doi.org/10.1111/jar.12912
   PubMedGoogle Scholar
• Dierckx, E., Engelborghs, S., De Raedt, R., Van Buggenhout, M., De Deyn, P. P., Verleye, G., Verte, D., & Ponjaert-Kristoffersen, I. (2008). Differentiation between dementia and depression among older persons: Can the difference between actual and premorbid intelligence be useful? Journal of Geriatric Psychiatry and Neurology, 21(4), 242–249. https://doi.org/10.1177/0891988708324938
   PubMed Web of Science ®Google Scholar
• Duggan, L., Lewis, M., & Morgan, J. (1996). Behavioural changes in people with learning disability and dementia: A descriptive study. Journal of Intellectual Disability Research, 40(4), 311–321. https://doi.org/10.1111/j.1365-2788.1996.tb00636.x
   PubMedGoogle Scholar
• Elliott-King, J., Shaw, S., Bandelow, S., Devshi, R., Kassam, S., & Hogervorst, E. (2016). A critical literature review of the effectiveness of various instruments in the diagnosis of dementia in adults with intellectual disabilities. Alzheimer’s and Dementia: Diagnosis, Assessment and Disease Monitoring, 4, 126–148. https://doi.org/10.1016/j.dadm.2016.06.002
   PubMedGoogle Scholar
• Elo, S., & Kyngäs, H. (2008). The qualitative content analysis process. Journal of Advanced Nursing, 62(1), 107–115. https://doi.org/10.1111/j.1365-2648.2007.04569.x
   PubMed Web of Science ®Google Scholar
• Engelborghs, S., Maertens, K., Nagels, G., Vloeberghs, E., Mariën, P., Symons, A., Ketels, V., Estercam, S., Somers, N., & De Deyn, P. P. (2005). Neuropsychiatric symptoms of dementia: Cross-sectional analysis from a prospective, longitudinal Belgian study. International Journal of Geriatric Psychiatry, 20(11), 1028–1037. https://doi.org/10.1002/gps.1395
   PubMed Web of Science ®Google Scholar
• Esbensen, A. J., Hooper, S. R., Fidler, D., Hartley, S. L., Edgin, J., Liogier d’Ardhuy, X., Capone, G., Conners, F. A., Mervis, C. B., Abbeduto, L., Rafii, M. S., Krinsky-Mchale, S. J., Urv, T., Group, O. M. W., D’Ardhuy, X. L., Capone, G., Conners, F. A., Mervis, C. B., Abbeduto, L., & Weir, S. (2017). Outcome measures for clinical trials in Down syndrome. American Journal on Intellectual and Developmental Disabilities, 122(3), 247–281. https://.doi.org/10.1352/1944-7558-122.3.247.
   PubMedGoogle Scholar
• Evans, E., Bhardwaj, A., Brodaty, H., Sachdev, P., Draper, B., & Trollor, J. N. (2013). Dementia in people with intellectual disability: Insights and challenges in epidemiological research with an at-risk population. International Review of Psychiatry, 25(6), 755–763. https://doi.org/10.3109/09540261.2013.866938
   PubMed Web of Science ®Google Scholar
• Evans, K. M., Cotton, M. M., Einfeld, S. L., & Florio, T. (1999). Assessment of depression in adults with severe or profound intellectual disability. Journal of Intellectual and Developmental Disability, 24(2), 147–160. https://doi.org/10.1080/13668259900033941
   Web of Science ®Google Scholar
• Evenhuis, H. M. (1990). The natural history of Dementia in Down’s syndrome. Archives of Neurology, 47(3), 263–267. https://doi.org/10.1001/archneur.1990.00530030029011
   PubMedGoogle Scholar
• Finkel, S. I. (2000). Introduction to behavioural and psychological symptoms of dementia (BPSD). International Journal of Geriatric Psychiatry, 15(1), 2–4. https://.doi.org/10.1002/(SICI)1099-1166(200004)15:1+<S2::AID-GPS159>3.0.CO;2-3.
   Google Scholar
• Fletcher, R. J., Barnhill, J., McCarthy, J., & Strydom, A. (2016). From DSM to DM-ID. Journal of Mental Health Research in Intellectual Disabilities, 9(3), 189–204. https://doi.org/10.1080/19315864.2016.1185324
   Web of Science ®Google Scholar
• Gisev, N., Bell, J. S., & Chen, T. F. (2013). Interrater agreement and interrater reliability: Key concepts, approaches, and applications. Research in Social and Administrative Pharmacy, 9(3), 330–338. https://doi.org/10.1016/j.sapharm.2012.04.004
   PubMed Web of Science ®Google Scholar
• Harris, P. A., Taylor, R., Thielke, R., Payne, J., Gonzalez, N., & Conde, J. G. (2009). Research electronic data capture (REDCap) - A metadata-driven methodology and workflow process for providing translational research informatics support. Journal of Biomedical Informatics, 42(2), 377–381 https://.doi.org/10.1016/j.jbi.2008.08.010.
   PubMed Web of Science ®Google Scholar
• Hon, J., Huppert, F. A., Holland, A. J., & Watson, P. (1999). Neuropsychological assessment of older adults with Down’s syndrome: An epidemiological study using the Cambridge cognitive examination (CAMCOG). British Journal of Clinical Psychology, 38(2), 155–165. https://.doi.org/10.1348/014466599162719.
   PubMedGoogle Scholar
• Jamieson-Craig, R., Scior, K., Chan, T., Fenton, C., & Strydom, A. (2010). Reliance on carer reports of early symptoms of dementia among adults with intellectual disabilities. Journal of Policy and Practice in Intellectual Disabilities, 7(1), 34–41. https://doi.org/10.1111/j.1741-1130.2010.00245.x
   Web of Science ®Google Scholar
• Janicki, M. P. (2011). Quality outcomes in group home dementia care for adults with intellectual disabilities. Journal of Intellectual Disability Research, 55(8), 763–776. https://doi.org/10.1111/j.1365-2788.2011.01424.x
   PubMed Web of Science ®Google Scholar
• Johnson, K., & Traustadóttir, R. (2005). Deinstitutionalization and people with intellectual disabilities: In and out of institutions. Jessica Kingsley Publishers.
   Google Scholar
• Keller, S. M., Janicki, M. P., & Esralew, L. (2016). Dementia: Screening, evaluation, diagnosis and management. In I. L. Rubin, J. Merrick, D. E. Greydanus, & D. R. Patel (Eds.), Health care for people with intellectual and developmental disabilities across the lifespan (pp. 1449–1463). Springer International Publishing. https://doi.org/10.1007/978-3-319-18096-0_116
   Google Scholar
• Kurrle, S., Hogarth, R., & Brodaty, H. (2012). Physical comorbidities of Dementia. Cambridge University Press. https://doi.org/10.1007/978-981-10-0370-7_14-1
   Google Scholar
• Llewellyn, P. (2011). The needs of people with learning disabilities who develop dementia: A literature review. Dementia, 10(2), 235–247. https://doi.org/10.1177/1471301211403457
   Google Scholar
• Lott, I. T., & Head, E. (2019). Dementia in Down syndrome: Unique insights for Alzheimer disease research. Nature Reviews Neurology, 15(3), 135–147. https://.doi.org/10.1038/s41582-018-0132-6.
   PubMed Web of Science ®Google Scholar
• Määttä, T., Tervo-Määttä, T., Taanila, A., Kaski, M., & Livanainen, M. (2006). Mental health, behaviour and intellectual abilities of people with Down syndrome. Down’s Syndrome, Research and Practice, 11(1), 37–43. https://.doi.org/10.3104/reports.313.
   PubMedGoogle Scholar
• Margallo-Lana, M. L., Moore, P. B., Kay, D. W. K., Perry, R. H., Reid, B. E., Berney, T. P., & Tyrer, S. P. (2007). Fifteen-year follow-up of 92 hospitalized adults with Down’s syndrome: Incidence of cognitive decline, its relationship to age and neuropathology. Journal of Intellectual Disability Research, 51(6), 463–477. https://.doi.org/10.1111/j.1365-2788.2006.00902.x.
   PubMedGoogle Scholar
• McKenzie, K., Metcalfe, D., & Murray, G. (2018). A review of measures used in the screening, assessment and diagnosis of dementia in people with an intellectual disability. Journal of Applied Research in Intellectual Disabilities, 31(5), 725–742. https://doi.org/10.1111/jar.12441
   PubMed Web of Science ®Google Scholar
• McKhann, G. M., Knopman, D. S., Chertkow, H., Hyman, B. T., Jack, C. R., Kawas, C. H., Klunk, W. E., Koroshetz, W. J., Manly, J. J., Mayeux, R., Mohs, R. C., Morris, J. C., Rossor, M. N., Scheltens, P., Carrillo, M. C., Thies, B., Weintraub, S., & Phelps, C. H. (2011). The diagnosis of dementia due to Alzheimer’s disease: Recommendations from the National Institute on aging-Alzheimer’s Association workgroups on diagnostic guidelines for Alzheimer’s disease. Alzheimer’s & Dementia, 7(3), 263–269. https://.doi.org/10.1016/j.jalz.2011.03.005.
   PubMed Web of Science ®Google Scholar
• Menéndez, M. (2005). Down syndrome, Alzheimer’s disease and seizures. Brain & Development, 27(4), 246–252. https://doi.org/10.1016/j.braindev.2004.07.008
   PubMed Web of Science ®Google Scholar
• Moriconi, C., Schlamb, C., & Harrison, B. (2015). Down syndrome and Dementia: Guide to identification, screening, and management. Journal for Nurse Practitioners, 11(8), 812–818. https://.doi.org/10.1016/j.nurpra.2015.05.015.
   Google Scholar
• O’Cathain, A., Murphy, E., & Nicholl, J. (2008). The quality of mixed methods studies in health services research. Journal of Health Services Research and Policy, 13(2), 92–98. https://doi.org/10.1258/jhsrp.2007.007074
   PubMed Web of Science ®Google Scholar
• Palinkas, L. A., Horwitz, S. M., Green, C. A., Wisdom, J. P., Duan, N., & Hoagwood, K. (2015). Purposeful sampling for qualitative data collection and analysis in mixed method implementation research. Administration and Policy in Mental Health, 42(5), 533–544 doi:10.1007/s10488-013-0528-y.Purposeful.
   PubMed Web of Science ®Google Scholar
• Passmore, C., Dobbie, A. E., Parchman, M., & Tysinger, J. (2002). Guidelines for constructing a survey. Family Medicine, 34(4), 281–286.
   PubMed Web of Science ®Google Scholar
• Prasher, V. P. (2009). . Neuropsychological Assessments of Dementia in down Syndrome and Intellectual Disabilities (Switzerland: Springer International Publishing) . https://.doi.org/10.1007/978-3-319-61720-6
   Google Scholar
• Reid, A. H., & Aungle, P. G. (1974). Dementia in ageing mental defectives: A clinical psychiatric study. Journal of Mental Deficiency Research, 18(1), 15–23. https://.doi.org/10.1111/j.1365-2788.1974.tb01214.x.
   PubMedGoogle Scholar
• Ries, J. D. (2018). Rehabilitation for Individuals with Dementia: Facilitating success. Current Geriatrics Reports, 7(1), 59–70. https://doi.org/10.1007/s13670-018-0237-1
   Web of Science ®Google Scholar
• Sabbagh, M., & Edgin, J. (2015). Clinical assessment of cognitive decline in adults with Down syndrome. Current Alzheimer Research, 13(1), 30–34. https://doi.org/10.2174/1567205012666150921095724
   Web of Science ®Google Scholar
• Sauna-Aho, O., Bjelogrlic-Laakso, N., Siren, A., & Arvio, M. (2018). Signs indicating dementia in Down, Williams and Fragile X syndromes. Molecular Genetics and Genomic Medicine, 6(5), 855–860. https://doi.org/10.1002/mgg3.430
   PubMed Web of Science ®Google Scholar
• Scott, K. R., & Barrett, A. M. (2007). Dementia syndromes: Evaluation and treatment. Expert Review of Neurotherapeutics, 7(4), 407–422. https://doi.org/10.1586/14737175.7.4.407
   PubMedGoogle Scholar
• Sheehan, R., Sinai, A., Bass, N., Blatchford, P., Bohnen, I., Bonell, S., Courtenay, K., Hassiotis, A., Markar, T., McCarthy, J., Mukherji, K., Naeem, A., Paschos, D., Perez-Achiaga, N., Sharma, V., Thomas, D., Walker, Z., & Strydom, A. (2015). Dementia diagnostic criteria in Down syndrome. International Journal of Geriatric Psychiatry, 30(8), 857–863. https://doi.org/10.1002/gps.4228
   PubMed Web of Science ®Google Scholar
• Smiley, E., & Cooper, S.-A. (2003). Intellectual disabilities, depressive episode, diagnostic criteria and diagnostic criteria for psychiatric disorders for use with adults with learning disabilities/Mental retardation (DC-LD). Journal of Intellectual Disability Research, 47(Suppl.1), 62–71. https://.doi.org/10.1046/j.1365-2788.47.s1.7.x.
   PubMedGoogle Scholar
• Strydom, A., Shooshtari, S., Lee, L., Raykar, V., Torr, J., Tsiouris, J., Jokinen, N., Courtenay, K., Bass, N., Sinnema, M., & Maaskant, M. (2010). Dementia in older adults with intellectual disabilities - epidemiology, presentation, and diagnosis. Journal of Policy and Practice in Intellectual Disabilities, 7(2), 96–110. https://.doi.org/10.1111/j.1741-1130.2010.00253.x.
   Web of Science ®Google Scholar
• Tong, A., Sainsbury, P., & Craig, J. (2018). Consolidated criteria for reporting qualitative research: A 32-item checklist for interviews and focus groups. International Journal for Quality in Health Care, 19(6), 349–357. https://doi.org/10.1093/intqhc/mzm042
   Google Scholar
• van Timmeren, E. A., van der Schans, C. P., van der Putten, A. A. J., Krijnen, W. P., Steenbergen, H. A., van Schrojenstein Lantman-de Valk, H. M. J., & Waninge, A. (2017). Physical health issues in adults with severe or profound intellectual and motor disabilities: A systematic review of cross-sectional studies. Journal of Intellectual Disability Research, 61(1), 30–49. https://doi.org/10.1111/jir.12296
   PubMed Web of Science ®Google Scholar
• Wiseman, F. K., Al-Janabi, T., Hardy, J., Karmiloff-Smith, A., Nizetic, D., Tybulewicz, V. L. J., Fisher, E. M. C., & Strydom, A. (2015). A genetic cause of Alzheimer disease: Mechanistic insights from Down syndrome. Nature Reviews. Neuroscience, 16(9), 564–574. https://doi.org/10.1038/nrn.3983.A.
   PubMed Web of Science ®Google Scholar
• Wissing, M. B. G., Hobbelen, J. S. M., Dekker, A. D., Ulgiati, A. M., & Deyn, P. P. D. (2021). The neglected puzzle of dementia in people with severe/profound intellectual disabilities : A systematic literature review of observable symptoms. Journal of Applied Research in Intellectual Disabilities, 34(1), 1–22. https://doi.org/10.1111/jar.12920
   PubMedGoogle Scholar
• World Health Organization. (2018). International Statistical Classification of Diseases and Related Health Problems, 11th revision (ICD-11). https://icd.who.int/browse11/l-m/en
   Google Scholar
• Zigman, W. B. (2013). Atypical aging in down syndrome. Developmental Disabilities Research Reviews, 18(1), 51–67. https://.doi.org/10.1002/ddrr.1128.
   PubMedGoogle Scholar
• Zigman, W. B., Devenny, D. A., Krinsky-mchale, S. J., Jenkins, E. C., Urv, T. K., Wegiel, J., Schupf, N., & Silverman, W. (2008). Alzheimer’s Disease in adults with Down Syndrome. International Review of Research in Mental Retardation doi:. https://doi.org/10.1016/S0074-7750(08)00004-9
   Google Scholar