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Sunjay Gupta noted the NTG's recommendations for assessing patients with intellectual disabilities which are intended to help provide healthcare professionals the information they need for the "detection of any cognitive impairment" -- a requirement that appears in the Medicare Annual Wellness Visit component of the Affordable Care Act.

Dementia in Mentally Disabled Hard to Identify

Resource document

A team of specialists who work extensively with adults with intellectual disability promulgated these Consensus Recommendations for the Evaluation and Management of Dementia in Adults With Intellectual Disabilities as a framework for the practicing physician who assesses adults with intellectual disability.

The National Task Group on Intellectual Disabilities and Dementia Practices consensus recommendations for the evaluation and management of dementia in adults with intellectual disabilities

Resource document

Educational presentation given at the Winter Meeting of ACCSES held in Phoenix, AZ, on January 16, 2023. The presentation covers the medical aspects of dementia in adults with intellectual disability, co-incident conditions, therapeutic pharmaceuticals, biomarkers, and general health and medical practices. This is Part 2 of a three-part presentation session (Given by Dr Seth M. Keller).

Importance in Overall Health in Adults with Intellectual Disability and Dementia

Educational Presentation

Educational presentation given at the Winter Meeting of ACCSES held in Phoenix, AZ, on January 16, 2023.  The presentation covers the US National Plan to Address Alzheimer's, some basics about dementia and particulars about dementia and intellectual disability, planning considerations for organizing dementia support service, and agency dementia services and administrative issues.  This is Part 1 of a three-part presentation session (Given by Dr. Matthew P. Janicki).

Intellectual Disability and Dementia Practice

Educational Presentation

Book chapter that provides an introduction to the topic of dementia in persons with developmental disabilities and covers the physiology of dementia, options for services, mechanisms for multidisciplinary management, and advances in advocacy, dementia prevention, and dementia research.

Implications of dementia for adults with developmental disabilities.

Book chapter

Read Publication
The F.D.A. included narrower and more cautionary language on the drug label than it initially had with Aduhelm. The Leqembi label says the drug should be used only for patients in early and mild stages of Alzheimer’s disease, matching the status of patients in the clinical trials of the drug. It instructs doctors not to treat patients without doing tests to confirm that they have one of the hallmarks of Alzheimer’s: a buildup of the protein amyloid, which Leqembi is designed to reduce.

Legembi - FDA Prescriptive Information

Drug label information

Food and Drug Administration on January 7, 2023 approved another Alzheimer’s drug that may modestly slow the pace of cognitive decline early in the disease, but also carries risks of swelling and bleeding in the brain.

F.D.A. Approves New Treatment for Early Alzheimer’s

Newspaper article

Congressional report on the regulatory review and approval, pricing, and marketing of biotechnology company Biogen Inc.’s Alzheimer’s disease drug, aducanumab, known more commonly by its trade name, Aduhelm.

The High Price of Aduhelm’s Approval: An Investigation into FDA’s Atypical Review Process and Biogen’s Aggressive Launch Plans


Jokinen, N., Janicki, M.P., Keller, S.M., McCallion, P., Force, F.T., and the National Task Group on
Intellectual Disabilities and Dementia Practices

Guidelines for Structuring Community Care and Supports for People With Intellectual Disabilities Affected by Dementia

Journal article

This bibliography is a working document that contains select general population publications related to Long-COVID with possible implications for intellectual disability.  Currently, there is a dearth of publications related specifically to Long-COVID.  As they appear, they will be added to future versions of this bibliography.

Long-COVID and Intellectual Disability Bibliography

Working bibliography

Concerns over the impact of Long-COVID upon adults with intellectual  disability and the omission of clear guidance on this issue as lead the NTG to prepare an "Advisory on Long-COVID and Impact on Cognitive Function in Adults with Intellectual Disability."  The document is now available for download and use via this website.

The Advisory contains the NTG's position on this condition and addresses the background and definition of the cognitive aspects of Long-COVID, its symptoms and effects, what to look for with respect to symptoms, and what agencies and others can do to help adults affected by Long-COVID.

The Advisory covers these main points:

- Long-COVID can include ‘brain fog’ (problems with thinking, motivation, and carrying out life activities).

- Adults with intellectual disability infected with the SARS-COV-2 variant virus may experience Long-COVID.

- Partners, families, and agencies need to be on the ‘look-out’ for changes in cognitive functions that may be due to Long-COVID.

- Screening and assessment should be undertaken to determine presence of Long-COVID.

- Accommodations and adaptations should be put in place to help mitigate effects of Long-COVID.

NTG issues advisory on Long-COVID and its cognitive effects on adults with intellectual disability

NTG Statement

NTG Long-COVID Application for Direct Support Professionals

Application of the NTG Statement on LONG-COVID, Intellectual Disability, and Dementia to Direct Support Professionals

Resource document

An extensive bibliography on a range of published studies, articles, videos, guides, etc., on care management and adults with intellectual disability associated with older age and dementia. Articles, media, books, book chapters and 'grey' materials are included along with abstracts and sourcing information.

Bibliography on Dementia Care Management and Intellectual Disability

Working bibliography

Janicki, M.P., Hendrix, J., & McCallion, P.
Project Principals         Report on Assessment and Neuroatypical Populations

The NTG and the Lumind IDSC Foundation have issued a comprehensive report on the barriers to determining MCI and dementia in adults with neuroatypical conditions, such as acquired brain injury, autism, cerebral palsy, Down syndrome, intellectual disability, serious mental illness, and significant sensory impairments. Developed by an Expert Panel, the report concludes that current federal guidelines for assessing for MCI or dementia do not provide sufficient information to assess such adults.
The Panel recommended broadening federal guidance to include adaptations of assessment practices; expanding training for clinicians about such conditions; and undertaking research to produce more evidence-based information on assessing neuroatypical adults.

Examining Adults with Neuroatypical Conditions for MCI/Dementia During Cognitive Impairment Assessments: Report of the Neuroatypical Conditions Expert Consultative Panel

Resource document

A closer look at the CMS decision on Aduhelm and anti-amyloid drugs - an explainer for the Down syndrome community.

CMS decision on Aduhelm: An Explainer by LuMind IDSC

Resource document

Recently, the CMS released a toolkit to combat the COVID-19 pandemic within nursing homes. The toolkit builds upon previous actions taken by CMS, which provide a wide range of tools and guidance to states, healthcare providers and others during the public health emergency. Additionally, CMS has contracted with 12 Quality Innovation Network-Quality Improvement Organizations (QIN-QIOs) to work with providers, community partners, beneficiaries, and caregivers on data-driven quality improvement initiatives designed to improve the quality of care for beneficiaries across the United States. The QIN-QIOs are reaching out to nursing homes across the country to provide virtual technical assistance for homes that have an opportunity for improvement based on an analysis of previous citations for infection control deficiencies using publicly available data found on Nursing Home Compare. This effort augments the CMS's five-part plan to ensure safety and quality in America’s nursing homes and recent agency efforts to combat the spread of COVID-19 within these facilities.

CMS Best Practices Toolkit for Nursing Homes to Combat COVID-19


An updated COVID-19 and Down Syndrome Resources Guide is now available. Covered is new information on the Delta variant, current safety recommendations, and important updates useful to families, organizations, and adults with Down Syndrome.

COVID-19 and Down Syndrome Resources Guide

Resource document

A comprehensive FAQ issued by the NTG's Medical Advisory Group on questions that were raised about Biogen's new medication for treating Alzheimer's disease still has relevance even though Biogen has withdrawn Aduhelm from production.  "We are glad to make this available to families and providers who have questions and concerns about the use of this new medication," said Dr. Seth Keller, the MAG leader.

NTG's FAQ on aducanumab and Down syndrome

Resource document

Holingue et al. looked at identifying obstacles associated with screening and assessment of dementia among older adults with ID in a crisis-prone population. The NTG-EDSD was administered to enrollees aged 50 years and older in the START program. The authors noted that the NTG-EDSD provided a helpful way to collect information and document changes in the enrollees' functioning and that the NTG-EDSD may be helpful for capturing potential dementia-associated changes over time in crisis-prone adults with ID, though obstacles remain to the access of further evaluation for dementia.

Screening for dementia among adults with intellectual disability: Outcomes from a pilot study

Journal article

Bibliography of a number of journal articles and other print and web resources specifically addressing COVID-19 and intellectual disabilities. These publications stem from 2020 to the present and represent the product of an extensive search via the web. The bibliography is being updated periodically, so check back for the latest version. The bibliography is the effort of a joint project between the NTG and the Health Matters Program's Healthy Brain Initiative (funded by a grant from the Centers for Disease Control and Prevention). 

COVID-19 and Intellectual Disabilities Bibliography

Working bibliography - Current v1'6'23

Virtual presentation on intellectual disability and possible application of aducanumab to
  the ACCSES organization on August 5, 2021

Aducanumab: Panacea or Risk for Adults with Down Syndrome

Conference presentation

Virtual presentation on aducanumab and implications for state I/DD agencies to the Medical & Clinical Director's Work Group of the National Association of State Directors of Developmental Disabilities Services on August 4, 2021

New Alzheimer’s Drug and Considerations for State I/DD Agencies: Aducanumab: Panacea or Risk for Adults with Down Syndrome

Conference presentation

NTG issued statement on use of Aduhelm by persons with Down syndrome. This statement, the result of a collaborative effort coordinated by the NTG Aduhelm and Down Syndrome Medical Advisory Group, was led by Seth M. Keller, MD, Co-President of the National Task Group on Intellectual Disabilities and Dementia Practices.

Aducanumab and Persons with Down Syndrome: What Do We Do Now?

Resource document

Published version of statement in Exceptional Parent (July 2021)

Aduhelm: Rush to Judgement?

Journal article

Abstract: Cerebral palsy (CP) neurologic care and research efforts typically focus on children. However, most people with CP are adults. Adults with CP are at increased risk of new neurologic conditions, such as stroke and myelopathy, that require ongoing neurologic surveillance to distinguish them from baseline motor impairments. Neurologic factors could also contribute to the motor function decline, chronic pain, and chronic fatigue that are commonly experienced by adults with CP. Based on a systematic literature review, we suggest (1) guidelines for neurologic surveillance and neurologist referral and (2) clinical research questions regarding the evolving neurologic risks for adults with CP.

Adults with cerebral palsy require ongoing neurologic care: a systematic review

Journal article

Ryan, M., Tuso, A., & Herge, A.. (2021). Usefulness of the NTG-EDSD: Evaluating the Validity of a Screening Tool for Dementia in Adults with Intellectual Disabilities. 

Doctorate of Occupational Therapy Program Capstone Presentations. Paper 22.

Usefulness of the NTG-EDSD: Evaluating the Validity of a Screening Tool for Dementia in Adults with Intellectual Disabilities


Ng,T.K.S., Tagawa, A., Ho, R.C., Larbi, A., Kua, E.H., Mahendran, R., Carollo, J.J., & Heyn, P.C., Aging (Albany NY), 2021, 13. [Epub ahead of print],/aging.202563

Abstract: Clinically, individuals with cerebral palsy (CP) experience symptoms of accelerated biological aging. Accumulative deficits in both molecular underpinnings and functions in young adults with CP can lead to premature aging, such as heart disease and mild cognitive impairment (MCI). MCI is an intermediate stage between healthy aging and dementia that normally develops at old age. Owing to their intriguingly parallel yet “inverted” disease trajectories, CP might share similar pathology and phenotypes with MCI, conferring increased risk for developing dementia at a much younger age. Thus, we examined this hypothesis by evaluating these two distinct populations (MCI= 55, CP = 72). A total of nine measures (e.g., blood biomarkers, neurocognition, Framingham Heart Study Score (FHSS) were compared between the groups. Compared to MCI, upon controlling for covariates, delta FHSS, brain-derived neurotrophic factor (BDNF) levels, and systolic blood pressure were significantly lower in CP. Intriguingly, high-sensitivity CRP, several metabolic outcomes, and neurocognitive function were similar between the two groups. This study supports a shared biological underpinning and key phenotypes between CP and MCI. Thus, we proposed a double-hit model for the development of premature aging outcomes in CP through shared biomarkers. Future longitudinal follow-up studies are warranted to examine accelerated biological aging.

Commonalities in biomarkers and phenotypes between mild cognitive impairment and cerebral palsy: a pilot exploratory study

Journal article

Smith, K.J.,Peterson, M.D.,Victor, C., & Ryan, J.M.
BMJ Open, 2021;11:e042652. doi:10.1136/ bmjopen-2020-042652Abstract: Determine the risk of incident dementia in adults with cerebral palsy (CP) compared with age, sex and general practice (GP) matched controls. A retrospective cohort study designed was used. The setting was the UK using GPs linked into the Clinical Practice Research Datalink (CPRD). CPRD data were used to identify adults aged 18 or older with a diagnosis of CP. Each adult with CP was matched to three controls who were matched for age, sex and GP. In total, 1703 adults with CP and 5109 matched controls were included in the analysis. The mean baseline age of participants was 33.30 years (SD: 15.48 years) and 46.8% of the sample were female. The primary outcome included a new diagnosis of dementia during the follow-up period (earliest date of 1987 to latest date of 2015).  The results showed that during the follow- up, 72 people were identified with a new diagnosis of dementia. The overall proportion of people with and without CP who developed dementia was similar (CP: n=19, 1.1%; matched controls n=54, 10.0%). The unadjusted HR suggested that people with CP had an increased hazard of being diagnosed with dementia when compared with matched controls (HR 2.69, 95% CI 1.44 to 5.00). This association was attenuated when CP comorbidities (sensory impairment, intellectual disability and epilepsy) were accounted for (HR 1.92, 95% CI 0.92 to 4.02).There was no difference in the proportion of people with CP and matched controls who were diagnosed with dementia during the follow-up. Furthermore, while there was evidence for an increased hazard of dementia among people with CP, the fact that this association was attenuated after controlling for comorbidities indicates that this association may be explained by comorbidities rather than being a direct result of CP. Findings should be interpreted with caution due to the low number of incident cases of dementia.

Risk of dementia in adults with cerebral palsy: a matched cohort study using general practice data

Journal article

The Irish COVID-19 Guidance and Practice Resource Pack (a practical support pack) was developed in Ireland for the national Disability Services in preparation for the roll out of the national COVID-19 vaccination program. The guidance was developed in accordance with national guidelines and legislation, and on the basis that those who provide health and social care services must work on the presumption that every person who uses our services has the capacity to make decisions about whether or not they wish to consent to the COVID-19 vaccination.

Guidance and Practical Resource Pack in Preparation for Covid-19 Vaccination Program in Disability Services

Resource document

Dr. Michael Rafii, the chair of the ISTAART Down Syndrome and Alzheimer's Disease PIA, and M. Florencia Iulita issued this useful 4-page report which highlights the progress that was made during 2020 as part of an extensive effort to look back at the landscape of the field of Down syndrome and dementia research, and recognize the efforts of various researchers look at hints of where the field is headed in the future. 

ISTAART Down Syndrome PIA Report on Research in 2020


Silverman, W., Krinsky-McHale, S.J., Lai, F., Rosas, H.D., Hom, C., Doran, E., Pulsifer, M., Lott. I., Schupf, N, and Alzheimer’s Disease in Down Syndrome (ADDS) Consortium
Journal of Applied Research in Intellectual Disabilities, 2020, Dec 13. doi:10.1111/jar.12849.

Abstract: The accuracy of the National Task Group-Early Detection Screen for Dementia (NTG-EDSD) was evaluated in a sample of 185 adults with Down syndrome (DS), emphasizing 'mild cognitive impairment (MCI-DS)'. Knowledgeable informants were interviewed with the NTG-EDSD, and findings were compared to an independent dementia status rating based on consensus review of detailed assessments of cognition, functional abilities and health status (including physician examination). Results indicated that sections of the NTG-EDSD were sensitive to MCI-DS, with one or more concerns within the 'Memory' or 'Language and Communication' domains being most informative.  The NTG-EDSD is a useful tool for evaluating dementia status, including MCI-DS. However, estimates of sensitivity and specificity, even for detecting frank dementia, indicated that NTG-EDSD findings need to be supplemented by additional sources of relevant information to achieve an acceptable level of diagnostic/screening accuracy.

Evaluation of the National Task Group-Early Detection Screen for Dementia: Sensitivity to 'Mild Cognitive Impairment' in Adults with Down Syndrome

Journal article

This NTG statement, issued December 11, 2020, provides the rationale for placing adults with intellectual disability affected by Alzheimer’s disease and other causes for dementia on priority lists for receiving special consideration in mitigation strategies for managing the spread of COVID-19 and for receiving inoculation by vaccines designed to inhibit infections by the COVID-19 virus.

NTG Statement on COVID-19, Intellectual Disability, and Dementia

NTG Statement

Walaszek et al. trained direct care workers, case managers, health-care providers, and other social services personnel on using the NTG-EDSD. After training, attendees reported a marked increase in confidence in their ability to track various health circumstances and detect functional decline in their clients. Authors noted that social and health-care professionals can learn to document signs of cognitive decline in adults with ID using the NTG-EDSD. The study participants were highly satisfied with the training, experienced an increase in confidence in their care of persons with ID, and found the NTG- EDSD feasible to use.

Effectively training professional caregivers to screen and refer persons with dementia and intellectual/developmental disability

Journal article

AAIDD Research Brief: This brief provides an overview of what the research to date reveals about people with intellectual and developmental disabilities (IDD), COVID-19, and vaccination. The research to date indicates that people with IDD (a) are more likely to be exposed to COVID-19 and have poorer COVID-19-related health outcomes than the general public; (b) are as likely as the general population to engage in preventive health strategies; and (c) have experienced massive disruption in health, home, and community services, exacerbating existing inequities in health care, and putting additional pressure on already over-taxed formal and informal support networks.

State of the Science on COVID-19 and People with IDD

Resource document

NTG Evaluation Committee 2019 Summary Report - 7/2020    Consistent with the mission of the NTG to enhance the knowledge and support of care providers for older individuals with intellectual and developmental disabilities, the NTG Evaluation Committee examined the impact of 11 education and training workshops provided by both NTG's Master Trainers and various Lead Trainers and held between January and December 2019.

Using the ADKS-ID pre-post test data as well as post training evaluation data from 2-day training workshops, the Evaluation Committee's summary report covered trainee satisfaction, knowledge acquisition, perceived value of content presented, and intent to apply information included in the training delivered. The training locations from which data were collected and analyzed covered 10 states across the United States.

Key Findings

The NTG Evaluation Committee determined that the ADKS-ID 2.0 tool met required criteria for further use by the NTG as a pre-posttest training measure. In February 2020, the authors of the original ADKS instrument granted permission to the NTG to continue to use the adapted tool, the ADKS-ID, for NTG training purposes.  It is currently in use for NTG purposes only.

The Committee also found that evaluation responses affirmed that participant learning outcomes aligned with the stated training objectives. Responses indicated that the objectives of the 2-day training workshop were met. More than 90% of the training attendees would recommend the training to others.

2020 Report of the Evaluation of the NTG Workshops


The aim of this internet-based survey was to obtain information from community-based organizations (CBOs) affiliated with ACCSES, a national provider association based in Washington DC, as to what types of difficulties provider agencies were encountering during a transitional phase of the coronavirus COVID-19 pandemic. The study was conducted in collaboration with the University of Illinois at Chicago in the summer of 2020.

Key Findings

- CBOs providing direct services noted experiencing contagion, lockdowns, loss of staff, challenges in obtaining PPEs, and lack of state or federal guidance.

- Maintaining safe living environments and coping with infected staff and clientele were urgent concerns.

- CBOs reported financial and clinical support challenges, as well as staffing problems.

- Testing availability, lack of sufficient PPEs, dealing with clientele and staff boredom during lockdowns, and increased costs for equipment and staff (with no commensurate increases in fees) were noted as problems.

- Re-opening challenges were reported, including funding, staffing, PPEs, liability, and lack of guidance. CBOs found ways to work-around some programmatic issues, by using telehealth, drive-by visits, instituting rigorous safety measures, and offering virtual services, when possible

NTG Collaborative Study of COVID-19 Impact on Provider Agencies

Research report

Fortea J, et al.  Further understanding the connection between Alzheimer’s disease and Down syndrome., Vol 395, June 27, 2020, 1988-1997.Abstract: Alzheimer’s disease and its complications are the leading cause of death in adults with Down syndrome. Studies have assessed Alzheimer’s disease in individuals with Down syndrome, but the natural history of biomarker changes in Down syndrome has not been established. The study characterized the order and timing of changes in biomarkers of Alzheimer’s disease in a population of adults with Down syndrome.  Authors did a dual-centre cross-sectional study of adults with Down syndrome recruited through a population based health plan in Barcelona (Spain) and through services for people with intellectual disabilities in Cambridge (UK). Cognitive impairment in participants with Down syndrome was classified with the Cambridge Cognitive Examination for Older Adults with Down Syndrome (CAMCOG-DS). Only participants with mild or moderate disability were included who had at least one of the following Alzheimer’s disease measures: apolipoprotein E allele carrier status; plasma concentrations of amyloid β peptides 1–42 and 1–40 and their ratio (Aβ1–42/1–40), total tau protein, and neurofilament light chain (NFL); tau phosphorylated at threonine 181 (p-tau), and NFL in cerebrospinal fluid (CSF); and one or more of PET with ¹⁸F-fluorodeoxyglucose, PET with amyloid tracers, and MRI. Cognitively healthy euploid controls aged up to 75 years who had no biomarker abnormalities were recruited from the Sant Pau Initiative on Neurodegeneration. The authors studied 388 participants with Down syndrome (257 [66%] asymptomatic, 48 [12%] with prodromal Alzheimer’s disease, and 83 [21%] with Alzheimer’s disease dementia) and 242 euploid controls. CSF Aβ1–42/1–40 and plasma NFL values changed in individuals with Down syndrome as early as the third decade of life, and amyloid PET uptake changed in the fourth decade. ¹⁸F-fluorodeoxyglucose PET and CSF p-tau changes occurred later in the fourth decade of life, followed by hippocampal atrophy and changes in cognition in the fifth decade of life. Prodromal Alzheimer’s disease was diagnosed at a median age of 50·2 years (IQR 47·5–54·1), and Alzheimer’s disease dementia at 53·7 years (49·5–57·2). Symptomatic Alzheimer’s disease prevalence increased with age in individuals with Down syndrome, reaching 90–100% in the seventh decade of life.  The concluded that Alzheimer’s disease in individuals with Down syndrome has a long preclinical phase in which biomarkers follow a predictable order of changes over more than two decades. The similarities with sporadic and autosomal dominant Alzheimer’s disease and the prevalence of Down syndrome make this population a suitable target for Alzheimer’s disease preventive treatments.

Clinical and biomarker changes of Alzheimer’s disease in adults with Down syndrome: a cross-sectional study

Journal article

Snyder HM, Bain LJ, Brickman AM, et al. Further understanding the connection between Alzheimer’s disease and Down syndrome. Alzheimer’s Dement. 2020;16:1065–1077.        Abstract: Improved medical care of individuals with Down syndrome (DS) has led to an increase in
life expectancy to over the age of 60 years. In conjunction, there has been an increase in age-related co-occurring conditions including Alzheimer’s disease (AD). Understanding the factors that underlie symptom and age of clinical presentation of dementia in people with DS may provide insights into the mechanisms of sporadic and DS-associated AD (DS-AD). In March 2019, the Alzheimer’s Association, Global Down Syndrome Foundation and the LuMind IDSC Foundation partnered to convene a workshop to explore the state of the research on the intersection of AD and DS research; to identify research gaps and unmet needs; and to consider how best to advance the field. This article provides a summary of discussions, including noting areas of emerging science and discovery, considerations for future studies, and identifying open gaps in our understanding for future focus.

Further understanding the connection between Alzheimer’s disease and Down syndrome

Journal article

Mattia Rosso, Ellen Fremion, Stephanie L. Santoro, Nicolas M. Oreskovic, Tanuja Chitnis, Brian G. Skotko and Jonathan D. Santoro
Pediatrics, June 2020, 145 (6) e20192939;   Abstract: Down syndrome disintegrative disorder (DSDD), a developmental regression in persons with Down syndrome (DS), is a clinical entity that is characterized by a loss of previously acquired adaptive, cognitive, and social functioning in persons with DS usually in adolescence to early adulthood. Initially reported in 1946 as “catatonic psychosis,” there has been an increasing interest among the DS community, primary care, and subspecialty providers in this clinical area over the past decade. This condition has a subacute onset and can include symptoms of mood lability, decreased participation in activities of daily living, new-onset insomnia, social withdrawal, autistic-like regression, mutism, and catatonia. The acute phase is followed by a chronic phase in which baseline functioning may not return. No strict criteria or definitive testing is currently available to diagnose DSDD, although a comprehensive psychosocial and medical evaluation is warranted for individuals presenting with such symptoms. The etiology of DSDD is unknown, but in several hypotheses for regression in this population, psychological stress, primary psychiatric disease, and autoimmunity are proposed as potential causes of DSDD. Both psychiatric therapy and immunotherapies have been described as DSDD treatments, with both revealing potential benefit in limited cohorts. In this article, the authors review the current data regarding clinical phenotypes, differential diagnosis, neurodiagnostic workup, and potential therapeutic options for this unique, most disturbing, and infrequently reported disorder.

Down syndrome disintegrative disorder: A clinical regression syndrome of increasing importance

Journal article

Effectively training professional caregivers to screen and refer persons with dementia and intellectual disabilities.

AAIC 2020 Poster on NTG-EDSD

Conference poster

'COVID-19 and Pneumonia: Increased Risk for Individuals with Intellectual and Developmental Disabilities during the Pandemic'
A report from the Lerner Center for Public Health Promotion at Syracuse University

Prevalence of Underlying Health Conditions in COVID-19 Infections - United States


Brian Chicoine, MD, Adult Down Syndrome Center, Advocate Lutheran General Hospital, Park Ridge, Illinois     This video is of a recording of the Center's medical director, Brian Chicoine, MD, presentation on "Regression and Loss of Skills in Adolescents and Adults with Down Syndrome." He defines regression and loss of skills as well as describes possible causes and potential treatments. He also discusses strategies that can be used when supporting an individual with Down syndrome who is experiencing regression or loss of skills. This was a presentation at part of the Center's 2020 Health Education Series, held on February 17, 2020, in Park Ridge, IL.

Regression and loss of skills in adolescents and adults with Down syndrome

Resource video

NCI Data Brief   Report on Characteristics and Outcomes of Older Adult with Intellectual and Developmental Disabilities (March 2020). Report also covers information on adults with ID and dementia. Report notes that of 24,453 adults, 1.1% are adults with dementia under age 55, 6.4% are 55-64, 8.5% are 65-74, and 19.0% are age 75+

National Core Indicators Data Base

Research report

American Journal of Medical Genetics, 12 February 2020, 182A(3), 421-422.    Abstract unavailable - select text extracted

To address the challenges of diagnosing and managing URDS, a working group was created within the Down Syndrome Medical Interest Group (DSMIG-USA), which, in turn, generated a definition that includes 28 core and common clinical features of URDS. Researchers created a database that included both patients with unexplained regression and matched controls. Standardized data on clinical symptoms and tiered medical evaluations were collected, and a total of 35 patients with DS and unexplained regression were identified, with a mean age at regression of 17.5 years. Cases of URDS were compared with age- and sex-matched controls for clinical features, depression and stressor screens, and medical evaluations. Study results showed that diagnostic features between the 2 cohorts differed substantially. One of the key takeaways of this work is that patients with URDS have many more stressors, depressive symptoms, and mental health concerns than those without. “On the clinical side, I am [now] more attuned to stressors for my patients, and more apt to refer to psychiatry, psychology, or neuropsychology when there appears to be a co-occurring mental health condition,” says Dr. Santoro. “Although we can't be certain without knowing the interplay/mechanism for URDS, it seems prudent to take steps to minimize and treat these factors for all of our patients with Down syndrome. One of the key takeaways of this work is that patients with URDS have many more stressors, depressive symptoms, and mental health concerns than those without. “On the clinical side, I am [now] more attuned to stressors for my patients, and more apt to refer to psychiatry, psychology, or neuropsychology when there appears to be a co-occurring mental health condition,” says Dr. Santoro. “Although we can't be certain without knowing the interplay/mechanism for URDS, it seems prudent to take steps to minimize and treat these factors for all of our patients with Down syndrome.


New definition of unexplained regression in Down syndrome proposed

Journal article

NTG Board members participated in the Alzheimer's Association International Conference - AAIC 2020 - with two 'Developing Topics' poster presentations.  These presentations are cited in a Fall 2020 issue of Alzheimer's & Dementia: The Journal of the Alzheimer's Association. Nancy Jokinen, Kathryn Service, Christine Marsack-Topolewski, and Matthew Janicki addressed a "Support-staging model for caregivers of adults with intellectual disability affected by dementia". (#47273)

ABSTRACT:  Adults with intellectual disability (ID) and dementia are a sub-population of persons who are often un- or underserved. Most adults with ID are integrated within the general community (living autonomously, or in apartments/group residences); but significant numbers also reside with their families, particularly adults with Down syndrome. Family help/counseling approaches, such as the New York University-Caregiver Intervention (NYUCI), might benefit from a support-staging model assessment focus on what specific aid a family requires to meet their needs. Patterns of such needs have been identified that can help with providing dementia-capable care.  Objective needs include: (a) information on signs and symptoms, (b) diagnostic advice, (c) understanding behavioral changes and managing dementia-related behaviors, (d) adapting homes, (e) determining daily routines most conducive to calming, (f) planning for the future, (g) finding and navigating resources, and (h) responding to end-of-life needs.  Subjective needs include: (a) being informed at time of diagnosis and throughout the course of dementia, (b) coping with a profound sense of loss from knowing the diagnosis, (c) fearing the future [including financial concerns], (d) formulating long-term plans, (e) accessing community-based coordinated care, (f) facing difficulties from the medical community, (g) feeling overwhelmed by caregiving demands, (h) feeling a sense of isolation and abandonment, and (i) facing end-of-life issues.  A working group emanating from the 2016 Glasgow Summit on Intellectual Disability and Dementia organized a schema encapsulating these concerns into a support-staging model. The schema suggested four fluid stages: Diagnostic (seeking cause of changes in function, abilities, personality), Explorative (exploring dementia capable interventions), Adaptive (coping with and managing the symptoms/changes), and Closure (resolving / relief from responsibilities). Using this schema, a process (utilizing the NYUCI) is underway to operationalize a support-staging assessment instrument which would enable counseling staff to frame the state of a family’s concerns, build relationships through this knowledge of the caregiver and provide tailored services to them.  The outcome will enable systematic coding and organizing both objective and subjective data so that specific interventions and counseling can be adapted to meet both intermittent and continuous caregiver needs. 

Support-Staging Model for Caregivers of Adults with Intellectual Disability Affected by Dementia (#47273)

Conference poster

NTG Board members participated in the Alzheimer's Association International Conference - AAIC 2020 - with two 'Developing Topics' poster presentations.    Matthew Janicki covered "Community-based housing and NPI-care practices for adults with intellectual disability and dementia". (#47061) 

ABSTRACT: Aging persons with intellectual disability (ID) represent a vulnerable population with respect to cumulative neuropathological conditions, including dementia. Adults with Down syndrome (DS), a subset, have a recognized high risk for Alzheimer’s disease. With dementia present, how to provide post-diagnostic supports is a challenge. Dementia care group homes (GHs) along with NPIs are emerging as a mode for providing out-of-home community supports. Data from a longitudinal study provide insights on what care organizations need to consider when organizing specialty group home care. The study, begun in 2011, followed three co-located homes providing NPIs to 15 adults with dementia. Findings revealed trajectories of changes over time, housing need/function level patterning, and health status outcomes. Key findings noted 3 age-of-admission clusters (ꭕ=50.5; ꭕ=57.1; χ=66.8); overall mortality (ꭕage-death=65.4; ID=69.3; DS=56.3) – half of original entrants died within 7 years; age at entry (ꭕ= 59.1); years from entry to death (ꭕ= 5.4 yrs); LOS (ꭕ=49.4 months/4.12 yrs); morbidities (number of co-morbidities decreased among survivors). In same period, 8/15 deaths in GHs vs 3/15 deaths in Controls. NPI-related practices included day program activities (adults in mid- to later stages were engaged in regular off-site day activities that agency provided; adults with advanced dementia remained in homes), staffing patterns differed based on level of care – more staff assigned to homes with residents with advanced dementia, and staff training included dementia capable communications, engagement, and managing daily routines. Trends showed adults with Down syndrome were admitted to homes earlier but had more life-years in the GHs than older adults admitted at later age but who succumbed earlier to disease complications. Dementia care GHs should expect varied trajectories of decline; mortality linked to complexity of pre-existing conditions and progression of dementia; changes in the focus of care needs over time (including advanced dementia and end-of-life care). Dementia care GHs can enable provision of in-community group housing and quality care in accord with stage-defined functional changes and needs if structured in a planful way (factoring in dementia-stage, type of dementia, mortality expectations, health status, patterns of care needs, dementia-related behaviors, aging-related issues, and probable trajectories of decline of the residents).

Community-Based Housing and NPI-Care Practices for Adults with Intellectual Disability and Dementia" (#47061)

Conference poster

Project led by Janet Miller of the University of Nebraska's Medical Center   The NTG aided the Nebraska Council on Developmental Disabilities and the Munroe-Meyer Institute in Omaha with the development of the Intellectual Disability and Dementia: A Caregiver's Resource Guide for Nebraskans, a project led by Janet Miller of the University of Nebraska's Medical Center.

Intellectual Disability and Dementia: A Caregiver's Resource Guide for Nebraskans

Caregiver resource guide

Santoro, S.L., Cannon, S., Capone, G., Franklin, C., Hart, S.J., et al. Genetics in Medicine, 2019, Apr;22(4):767-776.  oi: 10.1038/s41436-019-0706-8. Epub 2019 Nov 26.   ​Abstract: An entity of regression in Down syndrome (DS) exists that affects adolescents and young adults and differs from autism spectrum disorder and Alzheimer disease. Since 2017, an international consortium of DS clinics assembled a database of patients with unexplained regression and age- and sex-matched controls. Standardized data on clinical symptoms and tiered medical evaluations were collected. Elements of the proposed definition of unexplained regression in DS were analyzed by paired comparisons between regression cases and matched controls. We identified 35 patients with DS and unexplained regression, with a mean age at regression of 17.5 years. Diagnostic features differed substantially between regression cases and matched controls (p < 0.001 for all but externalizing behaviors). Patients with regression had four times as many mental health concerns (p < 0.001), six times as many stressors (p < 0.001), and seven times as many depressive symptoms (p < 0.001). Tiered medical evaluation most often identified abnormalities in vitamin D 25-OH levels, polysomnograms, thyroid peroxidase antibodies, and celiac screens. Analysis of the subset of patients with nondiagnostic medical evaluations reinforced the proposed definition. Our case-control evidence supports a proposed definition of unexplained regression in Down syndrome. Establishing this clinical definition supports future research and investigation of an underlying mechanism.

Unexplained regression in Down syndrome: 35 cases from an international Down syndrome database

Journal article

Judith H Miles, Nicole Takahashi, Julie Muckerman, Kerri P Nowell, Muaid Ithman
Neuropsychiatric Disease and Treatment, 2019, Sep 20, 15, 2723-2741.
doi:10.2147/NDT.S210613. eCollection 2019.  Abstract: The authors sought to expand their knowledge of catatonia occurring in adolescents and young adults with Down syndrome (DS) by describing the first prospective, consecutive, well-characterized cohort of seven young people with DS diagnosed with catatonia and treated between 2013 and 2018, and to assess each patient's treatment responses. Longitudinal assessment of each patient's response to treatment is intended to provide clinicians and psychiatrists a firm foundation from which assess treatment efficacy. Young adults with Down syndrome were consecutively enrolled in the study as they were diagnosed with catatonia. A comprehensive data set included medical, laboratory, developmental, demographic, family, social and genetic data, including query into disorders for which individuals with DS are at risk. Catatonia was diagnosed based on an unequivocal history of regression, positive Bush-Francis Catatonia Rating Scale and positive response to intravenous lorazepam. Patients' longitudinal progress was monitored using the Catatonia Impact Scale (CIS) developed for this purpose. Seven consecutive DS patients, who presented with unequivocal regression were diagnosed with catatonia and treated for 2.7-6 years using standard-of-care therapies; primarily GABA agonist, lorazepam, electroconvulsive therapy (ECT) and glutamate antagonists (dextromethorphan/ quinidine, memantine, minocycline). Responses to each treatment modality were assessed at clinic visits and through weekly electronic CIS reports. Seven young adults with DS were diagnosed with catatonia; all responded to Lorazepam and/or ECT therapy with good to very good results. Though ECT most dramatically returned patients to baseline, symptoms often returned requiring additional ECT. Dextromethorphan/quinidine, not used until mid-2017, appeared to reduce the reoccurrence of symptoms following ECT. Though all seven patients improved significantly, each continues to require some form of treatment to maintain a good level of functioning. Findings of a significant number of autoimmune disorders and laboratory markers of immune activation in this population may guide new diagnostic and treatment opportunities.

Catatonia in Down syndrome: Systematic approach to diagnosis, treatment and outcome assessment based on a case series of seven patients

Journal article

McCallion, P., Knowles, M., & Gould, E. (2019). Intellectual and developmental disabilities and dementia: Practical strategies for professionals. National Alzheimer’s and Dementia Resource Center for the U.S. Administration on Aging.  This guide provides background and practical strategies that can be used by professionals—from both the intellectual and developmental disability and aging communities—when working with individuals living with intellectual and developmental disabilities (IDD) and dementia. The guide is organized into 12 sections that address different aspects of identifying and providing services to individuals living with IDD and dementia or who are at risk of developing dementia. Included also are tools professionals can share with family caregivers or other caregivers who work with individuals living with IDD and dementia.

Link to web source page:

Intellectual and Developmental Disabilities and Dementia: Practical Strategies for Professionals

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