Download Report of the Topic Group on Nomenclature

REPORT OF THE TOPIC GROUP ON NOMENCLATURE
Version 5.1 (10/31/16)
International Summit on Intellectual Disability and Dementia
Glasgow, Scotland – October 13-14, 23016
Report of the Topic Group on Nomenclature1
Co-leads: Phil McCallion & Matt Janicki
Members: Seth Keller Mike Splaine Flavia H dos Santos
Question: How might we harmonize and standardize the terminology used in research papers,
practice guidance and policy documents when referring to dementia and people with
intellectual disability/Down syndrome?
Premise
We propose that the issue of nomenclature with respect to work in intellectual disabilities has two focal
areas:
1. Agreement on a common terminology related to cognitive impairment related to dementia
among persons with intellectual disabilities.
2. Recommendations for standardization of terminology within studies and reports on dementia
and intellectual disabilities
Area #1 Rationale for Common Terminology
As noted in sourced definitions, nomenclature is a system of names or terms, or the rules for
forming these terms in a particular field of arts or sciences. It is recognized that the principles of
naming vary from the relatively informal conventions of everyday speech to the internationally agreed
principles, rules and recommendations that govern the formation and use of the specialist terms used in
scientific and other disciplines. To aid in communication, naming "things" become part of general
human communication using words and language: it is an aspect of everyday taxonomy as people
1
The contents of this report were developed under a grant from the United States Department of Health and
Human Services, Administration for Community Living (ACL), National Institute on Disability, Independent Living,
and Rehabilitation Research (NIDILRR) Grant # 90RT5020-03-00. However, those contents do not necessarily
represent the policy of the Department of Health and Human Services (DHHS), nor endorsement by the US Federal
Government.
REPORT OF THE TOPIC GROUP ON NOMENCLATURE
distinguish the objects of their experience, together with their similarities and differences, which
observers identify, name and classify.2 Further, the use of names, as the many different kinds of nouns
embedded in different languages, connects nomenclature to theoretical linguistics, while the way
humans mentally structure the world in relation to word meanings and experience relates to the
philosophy of language.3 This usage is finding a call for consistency in applications to expressions of
neuropathologies such as dementia. In this case, the most useful dementia nomenclature application is
in the consistency in terms with respect to clinical treatment and diagnostics.4 However, other relevant
reasons for improving consistency in taxonomy are to facilitate scientific replication, to raise crosscultural studies, and to refine prevalence studies, by adopting precise and equivalent criteria. Another is
choosing wording to avoiding stigma and misunderstanding when speaking with persons affected by
dementia, whether ‘patients’ or family members.
Reviews of publications (whether journal articles, book chapters, or reports and plans) involving
intellectual disability show mixed uses of terms that generally relate to dementia or the diseases
associated with it (see Appendix C). Terms in use (including dementia, Alzheimer’s, and other like
descriptors) lack precision or consistency when applied inappropriately. Some of this may be attributed
to a lack of understanding of the distinction in the terms, the nuances involved with neuropathologies,
or inconsistent use of language as well as absence of or inconsistency in an agreed upon core group of
methods used in diagnosis. However, in the intellectual disability field this lack of precision in language
in and of itself affects the understanding of the condition under discussion and confusion is further
increased by a lack of agreement on common terminology in the domain of ‘dementia’.
This lack of precision is not solely found in the intellectual disability field, as witnessed by recent
efforts in the mainstream Alzheimer’s and dementia field to address the same issue. The concern over
coherency and lack of agreement on terminology was recently discussed in a presentation on
nomenclature at the Alzheimer's Disease-Related Dementias 2016 Summit5, held under the auspice of
the National Institutes of Health in the United States. This presentation stimulated a dialogue on
dementia-related nomenclature addressing: (1) dementia in general, regardless of the etiology, (2)
specific clinical syndromes, and (3) underlying etiologies.
The identified problems with inconsistency in terminology included: (1) Alzheimer’s disease is
synonymous with dementia, (2) low public awareness of other forms of dementia, and (3) lack of
recognition that ‘Alzheimer’s disease’ services are also for those affected by non-Alzheimer’s dementias.
The presentation noted that diagnosing, treating, and educating persons affected by dementia is often
based on judgements of cognitive status (i.e. normal, MCI, dementia), clinical syndrome, and suspected
underlying pathology (with or without biomarkers) – factors whose need for precision is affected by
how the condition diagnosed is explained, understood, treated, and followed. The presentation at the
Summit called for greater sensitivity in differentiation between etiology and clinical syndrome and
differentiation between disease stages (i.e., preclinical, prodromal and symptomatic).
2
https://www.google.com/?gws_rd=ssl#q=nomenclature+definition
en.Wikipedia.org/wiki/nomenclature
4
Breitner J.C. Dementia--epidemiological considerations, nomenclature, and a tacit consensus definition. J Geriatr
Psychiatry Neurol. 2006 Sep;19(3):129-36.
5
http://www.fnih.org/what-we-do/current-lectures-awards-and-events/2016-alzheimers-disease-relateddementias
3
REPORT OF THE TOPIC GROUP ON NOMENCLATURE
Nomenclature has also been discussed at meetings of the National Advisory Council on
Alzheimer's Research, Care and Services, which under federal legislation (the National Alzheimer's
Project Act; NAPA)6 is responsible for the National Plan to Address Alzheimer’s Disease in the United
States. In a presentation by Dr. Ron Petersen, the chair of the Council, it was noted that additional
‘confusion’ was introduced by the new DSM-5 characterizations of ‘mild neurocognitive disorder’ (MCI)
and ‘major neurocognitive disorder’ (dementia).7,8 While this dichotomy has organized the syndrome
into a symptom-based manner, it has also introduced controversy as it has caused a loss of ‘condition
recognition’ in the eyes of the public and among practitioners – i.e., when a commonly recognizable
general term has been replaced by a diagnostic and clinical one. Another concern raised is the
possibility that when an adult is diagnosed with “minor neurocognitive disorder due to Alzheimer’s
disease”, he or she may assume it is a minor situation, not of concern, and not raise recognition of the
seriousness of the diagnosis in terms of disease progression. This lack of recognition might contribute to
negative effects, namely whether the adult complies with treatment; takes prescribed medications to
slow progression; begins involvement with constructing a will and assign a power of attorney, and
obtains an advance directive.9 This also contributes to employability and capacity implications for both
workers and employers. Given that the notion of major cognitive disorder could be attributed to many
conditions affecting thinking and function it has been argued that another element of confusion may
have been introduced – particularly for explaining the condition to persons with limited conceptual
development, such as intellectual disability.10
Given the concerns raised, recommendation #4 by the public members of the National Advisory
Council on Alzheimer's Research, Care and Services, in the 2016 US National Plan Update11, states that
“Emphasis should be given to the standardization of terminology in dealing with cognitive and
dementing disorders.” Further, the Update recommends that “an integrated conference should be
convened to develop consistent language for cognitive disorders among the scientists, care providers
and the public… [and there is a need to] engage all of the stakeholders around these issues to reach a
consensus for the benefit of persons with dementia, their family members and caregivers, and the
scientific and service communities.”12
Confusion as to the application of these terms is demonstrated in the U.S. Department of Health
and Human Services’ Healthy People 2020 Initiative document, where this wording is found:
6
https://aspe.hhs.gov/national-alzheimers-project-act#Council
American Psychiatric Association. (2013). Diagnostic and statistical manual of mental disorders: DSM-5. 5th ed.
Washington, D.C: American Psychiatric Association.
8
Issue 7: Differentiating dementias. In Brief for Healthcare Professionals. Alzheimer’s Association.
https://www.alz.org/health-care-professionals/documents/InBrief_Issue7dd_Final.pdf
9
Siberski, J. (2012). Dementia and DSM-5: Changes, cost, and confusion. Aging Well, 5(6), 12.
http://www.todaysgeriatricmedicine.com/archive/110612p12.shtml.
10
Guides for people with intellectual disability and their families involve proving facile explanations of ‘dementia’ -see Kerr, D. & Innes. M., (n.d.). What is dementia: A booklet about dementia for adults who have a learning
disability. Edinburgh, Scotland: Down’s Syndrome Scotland; Watchman, K. (2007). Living with dementia: Adapting
the home of a person who has Down’s syndrome and dementia – a guide for carers. Edinburgh, Scotland: Down’s
Syndrome Scotland; and Watchman, K. Tuffrey-Wijne, I. & Quinn, S. (2015). Jenny’s Diary: a resource to support
conversations about dementia with people who have a learning disability. London, Alzheimer’s Society.
11
https://aspe.hhs.gov/report/national-plan-address-alzheimers-disease-2016-update
12
https://aspe.hhs.gov/public-member-advisory-council-alzheimers-research-care-and-services-2016recommendations
7
REPORT OF THE TOPIC GROUP ON NOMENCLATURE
“Dementias, including Alzheimer’s disease and other dementias”.13 The Initiative also notes, “Reduce
the morbidity and costs associated with, and maintain or enhance the quality of life for, persons with
dementia, including Alzheimer’s disease.14 Although the intent of this phrasing was to infer that other
etiologies of dementia were included, the stated phrasings inadvertently imply that Alzheimer’s disease
is a dementia -- adding to confusion among workers and the general public. A more precise usage could
have been “… dementias, including those due to Alzheimer’s disease and other causes.” Alternatively,
the wording in the report could have been more precise and used ‘dementia of the Alzheimer’s type
(DAT)’ or ‘dementia due to Alzheimer’s disease’ – which shows that the dementia, in these cases, stems
from the presence or is a function of Alzheimer’s disease. It is important that workers understand that
Alzheimer’s disease is a brain neuropathology and not dementia and that dementia is an expression of
Alzheimer’s disease. The appropriateness of the phrasing used, however, may also be reflective of the
context – ‘dementia of the Alzheimer’s type’ might be more appropriate usage in social care, whereas
‘dementia due to Alzheimer’s disease’ might be more appropriate usage in medical treatment. Like in
the general population literature addressing dementia, there is also a dichotomy in the intellectual
disability literature – with articles dwelling on the medical treatment or biological aspects of the
neuropathologies and articles dwelling on social care aspects of dementia.
Another consideration with respect to nomenclature is that contextually there is more to be
learned from general population concerns, as it is evident that language can shape and form our
perceptions of a condition or situation. For example, one aspect is to present the condition of dementia
and people affected by in a manner that does not stigmatize. As noted by Alzheimer’s Europe, “Some
words and metaphors are used liberally and paint a very biased picture of dementia, whilst others are
avoided and considered demeaning, depersonalizing, and insulting. Even standard medical terms are
sometimes used with great caution due to an awareness of the possible impact on people’s lives and
wellbeing. Words clearly matter. They describe, communicate and reinforce our current perceptions of
dementia. With awareness, we can try to use them positively to challenge portrayals of dementia and
promote a more positive image of dementia and people with dementia.”15 Language usage and
terminology is germane to how people affected by dementia are viewed and to what extent the terms
used minimize stigma.16 Clearly, ‘people-first’ language [i.e., adults with dementia] creates positive
imagery and can go far to minimize negative perceptions.
Another aspect is the ease with which the general population grasps the concept behind the
disease or neuropathology – so avoiding ‘high science language’ in documents directed toward general
readership audiences can facilitate understanding. Using terms and definitions that are in common
usage can help to ensure the communication of messages. Conversely, creating definitions that
minimize the course and eventuality of brain changes leading to dementia – perhaps leading readers to
believe that dementia is no more impairing that a head cold and thus not worthy of concern – can leave
13
https://www.healthypeople.gov/2020/data-search/Search-the-Data#topic-area=3517
https://www.healthypeople.gov/2020/topics-objectives/topic/dementias-including-alzheimers-disease
15
Alzheimer Europe. The ethical issues linked to the perceptions and portrayal of dementia and people with
dementia. http://www.alzheimer-europe.org/Ethics/Ethical-issues-in-practice/The-ethical-issues-linked-to-theperceptions-and-portrayal-of-dementia-and-people-with-dementia
16
Dementia words matter: Guidelines on language about dementia. [Dementia Engagement and Empowerment
Project (DEEP)]. DEEP Guidelines. (2105) http://dementiavoices.org.uk/wp-content/uploads/2015/03/DEEPGuide-Language.pdf
14
REPORT OF THE TOPIC GROUP ON NOMENCLATURE
conditions untreated and eventualities devoid of planning. Additionally, communicating the definition
and process of dementia to people with intellectual disability creates another conundrum. To enable
understanding, language and phrasing needs to be at a word-level that persons communicating with
self-advocates or adults affected by dementia can effectively convey concepts associated with
dementia.17,18 Further, the concepts need to be communicated in a manner so as to convey the
seriousness of the condition, while not creating confusion or undue anxiety. To examine the degree of
concept conveyance through the level of language used, Appendix A also shows both the reading ease
and grade level of the manner of how the definitions are conveyed by different organizations. Of the 16
definitions extracted from organization websites and documents, the reading ease level ranged from
60.70 to 0.0 ( = 26.68) and the grade level ranged from 7.8 to 18.6 ( = 13.84).19 In general, the higher
the reading ease score, the more generally understandable wording is at lower levels of education; with
respect to the grade level, the lower scores correspond with lower grade level reading abilities. It
appears that most of the definitions in Appendix A encompass complex concepts, even when directed at
the general public. Those definitions directed toward professionals are clearly in the realm of technical
language and reflect more complexity.
However, the purpose of this paper is not to quibble with perspectives on wording usage in the
general dementia field, question the utility of the DSM-5 designations, or to be overly influenced by U.S.
or general dementia population deliberations. The focus of this paper is to further highlight the need to
examine how terms related to dementia are used in the intellectual disability field and propose how we
may introduce more precision with respect to dementia-related language. Thus, some review of the
terms in play is useful.
While it is not the function of the topic group to standardize a definition of ‘dementia’, we
recognize that its primary features are characterized by the progressive loss of brain function that occurs
with certain neuropathological diseases or trauma and is often associated with aging. The expression of
the resultant behavioral changes includes marked memory disorders, personality and behavioral
changes, and impaired reasoning.20 In addition, we recognize that dementia-like behavioral
dysfunctions generally are associated with adverse drug reactions, depression, psychological trauma,
and a range of other causes – but their defining feature is clearing/reduction of symptoms upon
17
See for example, ‘What is dementia’ [Edinburgh, Scotland: Down’s Syndrome Scotland; n.d.], a booklet designed
to explain dementia to persons with intellectual disability, where dementia is defined as “an illness of the brain; it
affects many things, but mostly the way people remember and do things.”
18
Even among Down syndrome advocacy organizations, the definitions and explanations of dementia (or
Alzheimer’s disease) used tend toward complexity – see Appendix D. However, when materials are developed for
people with intellectual disability, more simple concepts are used. An example of a simple language definition is
found in the Down’s Syndrome Scotland publication, What is dementia? A booklet about dementia for adults who
have a learning disability. It reads “Dementia is an illness in the brain. It affects many things, but mostly the way
people remember and do things.” [http://aadmd.org/sites/default/files/whatisdementiabooklet.pdf].
19
The Flesch Reading Ease scores and the Flesch-Kincaid Grade Level scores, analytic instruments that are
embedded within Microsoft Word, were used to determine both the reading ease score and grade level score for
each definition cited in Appendix A.
20
National Task Group on Intellectual Disabilities and Dementia Practices. (2012). ‘My Thinker’s Not Working’: A
National Strategy for Enabling Adults with Intellectual Disabilities Affected by Dementia to Remain in Their
Community and Receive Quality Supports. http://aadmd.org/ntg/thinker
REPORT OF THE TOPIC GROUP ON NOMENCLATURE
treatment. Subordinate terms in the field tend to offer further uncertainty. Taylor21 in a presentation at
the Alzheimer's Disease-Related Dementias 2016 Summit noted a range of terms that are used within
the field:
Age-associated memory loss, mild cognitive impairment, minor neurocognitive impairment,
dementia, memory disorder, major neurocognitive impairment (DSM-V), Alzheimer’s disease,
dementia due to Alzheimer’s disease, Dementia with Lewy bodies, Lewy body dementias, Lewy
body disease, Lewy body disorders, frontotemporal dementia, degeneration, behavioral variant
FTD, Pick’s disease, primary progressive aphasia, progressive supranuclear palsy, corticobasal
syndrome, FTD/MND, FTD/ALS, vascular dementia, vascular contributions to cognitive,
impairment and dementia, mixed dementia, mixed etiology dementia.
Such a mélange of terms may offer imprecision and confound communication among
diagnosticians, explanations of diagnostic information to persons affected and their families, carers,
service workers or advocates, and subvert constructive treatment and support planning. Alternatively,
greater terminological precision in defining the nature and underlying cause of the dementia as well as
associated neuropathology (due also to neurodegenerative disorders or disease) can help with
understanding the course of functional changes and potential health factors, as well as duration and
potential sequelae.
Further, operational definitions of dementia-related functions and behaviors can aid in more
precise applications within related research and clinical treatment and interventions, such as the
ongoing work to define a pathway to standardization of language in wandering science. For example,
Algase et al.22 make the point that to empirically-based operational definitions of dementia assessment,
diagnostics, and care can improve clinical and research approaches to wandering and help with
improving dementia care.
While it is not the intent of this topic group to rectify inconsistencies in dementia-association
language usage across the general field of dementia, it is its intent to promote the understanding of the
distinctions among the terms in prevalent usage, and advance the application of precise usage and
agreement on dementia related terms that are commonly used in association with intellectual disability.
Such a result would advance clearer differentiation and appropriate usage among clinical terms
such as: dementia, Alzheimer’s disease, cognitive impairment, Alzheimer’s disease spectrum, pre-clinical
Alzheimer’s disease, dementia due to Alzheimer’s disease, mild cognitive impairment due to Alzheimer’s
disease, early versus late onset disease, and mild, moderate and advanced dementia (Alzheimer’s
disease), and others. We are not proposing a standard definition of dementia or the acceptance of
range of associated terms to apply to work in intellectual disability – no such consensus on terms exists
in the general dementia literature or documents (save for those that appear in the DSM-5 or ICD-10 for
diagnostic purposes) – but are suggesting that writers do provide an operational definition of their
choosing so as to offer readers a clear understanding of the variable and notions related to the
population being described.
21
Angela Taylor, Lewy Body Dementia Association. Dementia nomenclature. Presentation at the Alzheimer's
Disease-Related Dementias 2016 Summit. Bethesda, Maryland (USA), March 29-30, 2016.
22
Algase, D.L., Moore, D.H., Vanderweerd, C., & Gavin-Dreschnack. Mapping the maze of terms and definitions in
dementia-related wandering. Aging & Mental Health, 2007, 11(6), 686-698.
REPORT OF THE TOPIC GROUP ON NOMENCLATURE
A number of English-language international and national organization’s resources contain
attempts at defining dementia – See Appendix A. It is evident that these definitions vary in their
conceptualization, as these sources variously describe dementia as a collective name, condition, disease,
general term, illness, symptoms, and syndrome. These definitions are ones that are readily accessible to
the general public and workers in the field. It is clear that core themes of dysfunction run through
these, even if different means are used to describe them. General descriptors are prevalent among
websites that appeal to social care workers and caregivers. Much of focus in the general descriptions
note behavioral changes mostly associated with Alzheimer’s disease; some of the sites do offer nuanced
information referring to other forms of dementia. More precise descriptors are prevalent in sources
that address assessment and diagnostics, and sources devoted to specific neuropathologies resulting in
dementia (such as frontotemporal, vascular, Lewy body, Parkinsonian) offer more precision with
diagnosing the dementia covered.
Appendix B contains a listing of variations of the common dementia-related terms drawn from
the literature with relevance to intellectual disability, and provides a sampling of definitions (albeit not
definitive – as the general dementia field has itself not arrived at a consensus on common definitions).
This taxonomy is intended illustrate the variations used to describe similar phenomenon and to help
differentiate the meanings of the terms and aid writers with appropriate usage with the construction of
organic, functional and operational definitions.23 It also addresses the range of dementia-related social
care terminology prevalent in the field and used in varying degrees in the intellectual disability
literature.
Thus, it is our recommendation that the Summit report contain a taxonomy of terms typically
found in dementia reports and documents, so as to provide a resource for researchers, agency
personal, carers, and others who need to understand and use more precise terminology.
#2 Standardization in Reporting
Usage of terms can affect clarity in understanding which subject population is being referenced
and what conditions are being discussed. In a completely unscientific sampling of articles and reports
related to intellectual disability and dementia found in the literature, term usage was found to be
variable and at times less than clear (Appendix C). Authors in general used ‘dementia’ as a common
term defining the nature of the condition being discussed in their article or report. However, there was
often mixed reference to Alzheimer’s disease, dementia of the Alzheimer’s type, and generic ‘dementia’
without linkage to standard definitions (such as those in the ICD-10 and DSM-5). While the usage served
the intended purpose – of defining the general concern of the article – it left open interpretations of
how the condition was identified in the subjects, to what degree it was progressing, and what may have
been the underlying neuropathology. While in general most of these data may not be readily available
in social care research, it should be in medical research and in articles advancing or relying upon
standardized assessment.
23
Operational definitions define concepts and labels by the way they are measured and offer precision in
explaining a concept.
REPORT OF THE TOPIC GROUP ON NOMENCLATURE
Noted too is the high preponderance of articles in the intellectual disability and dementia
literature that focus predominantly on Down syndrome. Given the high risk for Alzheimer’s disease
among adults with Down this is not unexpected. However, it is important that authors are clear in
reporting results and generalizing effects that the information provided relates to dementia in adults
with Down syndrome. It is also useful to report the number or percentage of subjects with Down
syndrome within study populations of adults with intellectual disability, as this will alert readers to
possible skewing of the results. Additionally, as most studies involving adults with Down syndrome
relate to dementia of the Alzheimer’s type, this should be noted in the subject descriptions, and any
variations in forms of dementia noted. Further, concise definitions of dementia in subject recruitment
documents can benefit in gaining participants for studies.
In reviewing the central topic of articles in Appendix C related to intellectual disability and
dementia it appears that most articles reporting clinical or medical research are more apt to provide
some form a definition of dementia or related terms, while social care articles tend to only use the term,
but not define it or provide substantiation for ascribing the diagnoses to study subjects. We suspect
that clinical and medical work demands more precision in defining conditions. However, it would be
helpful in social care articles to also provide definitions and the basis for ascribing dementia to the
persons in the studies or when dementia is topic being discussed as this would offer more precision and
permit cross-article comparisons and contrasts.
Often also, many of the articles examined fail to define clearly the subject population with
respect to age, sex, level of intellectual disability, etiology, and other demographic variables, as well as a
basis for the designation of the presence of dementia. When these elements are absent, it leads to a
lack of precision with respect to application of the findings to other persons with suspected or
diagnosed dementia, and fails to provide the basis for follow-up or replications. Too often defining
subject populations in articles on dementia and intellectual disability is absent or loose and thus may
impede adding substance to the literature.
Standardization of language and terms can be affected by the issuance of a guidance document
on research, practice, and policy papers and how to accurately define and present information about
the individuals or group being referenced. The document should include both formal diagnostic criteria
and general definitions of the various dementia – preferably as used by groups representing these
conditions. Such a document should appear in a relevant publication (for archival purposes) and be
supported by a consortium of associated intellectual disability and gerontology groups.
As noted above, we are not proposing wording for a standard definition of dementia or the
adherence to the range of associated terms that apply to work in intellectual disability – no such
consensus terms exist in the general dementia literature or documents (with the exception of the DSM-5
or ICD-10 diagnostic definitions – as noted in Appendix A) – but are suggesting that writers provide an
operational definition of their choosing of the condition being considered, and ancillary demographic
and clinical data so as to offer readers an clear understanding of the population being described and
encourage replication and permit comparisons across studies on common factors. We also suggest that
reports stemming from studies untaken by the authors contain information about how diagnoses were
obtained as well as the protocols employed. This should include reporting the instruments used and the
criteria for the diagnosis of MCI or dementia.
REPORT OF THE TOPIC GROUP ON NOMENCLATURE
Thus, is it our recommendation that reports addressing neuropathologies or cognitive decline
or impairment add base data in the subject descriptions that identify the subjects’ ages, sex, level of
intellectual disability, residential situation, basis for dementia diagnosis, presence of Down syndrome,
years from diagnosis, and if available, an objective measure of changing function using a recognized
dementia scale.
-30-
REPORT OF THE TOPIC GROUP ON NOMENCLATURE
Appendix A: Sampling of definitions of ‘dementia’ by international and national organizations
Organization
Source
FRE/
F-KGL
Definition of Dementia
Alzheimer’s
Associations (USA)
Alzheimer’s Disease
International (INT)
http://www.alz.org/dementia/types-ofdementia.asp
https://www.alz.co.uk/about-dementia
52.0/
9.5
29.1/
14.1
Alzheimer’s Europe
(INT)
http://www.alzheimereurope.org/Glossary/dementia
29.1/
16.8
Alzheimer’s
Foundation of
America (USA)
Alzheimer Scotland
(SCT)
http://www.alzfdn.org/AboutDementia/
definition.html
32.0/
17.9
http://www.alzscot.org/information_an
d_resources/about_dementia
40.5/
14.0
Alzheimer’s Society
(CAN)
http://www.alzheimer.ca/en/Aboutdementia/What-is-dementia
28.5/
13.3
Alzheimer’s Society
(UK)
https://www.alzheimers.org.uk/site/scri
pts/documents.php?categoryID=200120
52.8/
10.0
American Psychiatric
Association (USA)
Diagnostic and Statistical Manual of
Mental Disorders-5 (DSM-5) .
Washington, DC: American Psychiatric
Association.
8.1/
18.2
Dementia is a general term for loss of memory and other mental abilities severe enough to interfere with daily
life. It is caused by physical changes in the brain.
Dementia is a collective name for progressive degenerative brain syndromes which affect memory, thinking,
behaviour and emotion. Symptoms may include: loss of memory, difficulty in finding the right words or
understanding what people are saying, difficulty in performing previously routine tasks, and personality and
mood changes.
Dementia knows no social, economic, ethnic or geographical boundaries. Although each person will experience
dementia in their own way, eventually those affected are unable to care for themselves and need help with all
aspects of daily life. There is currently no cure for most types of dementia, but treatments, advice, and support
are available.
“Dementia” is a syndrome (i.e. a pattern of symptoms) and typically involves loss of memory, mood changes and
problems with thinking, orientation, comprehension, calculation, learning capacity, language and judgement. It
is the umbrella term used to describe the symptoms that occur when the brain is damaged as a result of one or
more diseases or conditions, resulting in memory and intellectual impairments which are sufficiently severe as to
interfere with daily life. There are over 100 different diseases or conditions which can lead to dementia, the
most common being Alzheimer’s disease and a series of strokes. The underlying cause is often referred to when
describing the form of dementia, although a person may actually be affected by more than one type of
dementia.
Dementia is a general term that describes a group of symptoms-such as loss of memory, judgment, language,
complex motor skills, and other intellectual function-caused by the permanent damage or death of the brain's
nerve cells, or neurons.
Dementia is an illness that affects the brain, making it harder to remember things or think as clearly as before.
Dementia can affect every area of human thinking, feeling and behavior, but each person with dementia is
different - how the illness affects someone depends on which area of their brain is damaged.
Dementia is an overall term for a set of symptoms that are caused by disorders affecting the brain. Symptoms
may include memory loss and difficulties with thinking, problem-solving or language, severe enough to reduce a
person's ability to perform everyday activities. A person with dementia may also experience changes in mood or
behaviour.
Dementia is progressive, which means the symptoms will gradually get worse as more brain cells become
damaged and eventually die. Dementia is not a specific disease. Many diseases can cause dementia, including
Alzheimer's disease, vascular dementia (due to strokes), Lewy Body disease, head trauma, fronto-temporal
dementia, Creutzfeldt-Jakob disease, Parkinson’s disease, and Huntington’s disease. These conditions can have
similar and overlapping symptoms.
The word dementia describes a set of symptoms that may include memory loss and difficulties with thinking,
problem-solving or language. Dementia is caused when the brain is damaged by diseases, such as Alzheimer's
disease or a series of strokes. Dementia is progressive, which means the symptoms will gradually get worse.
The DSM-5 manual replaces the term “dementia” with major neurocognitive disorder and mild neurocognitive
disorder.
In DSM-5, a minor neurocognitive disorder is defined by the following:
• There is evidence of modest cognitive decline from a previous level of performance in one or more of the
domains outlined above based on the concerns of the individual, a knowledgeable informant, or the clinician;
and a decline in neurocognitive performance, typically involving test performance in the range of one and two
REPORT OF THE TOPIC GROUP ON NOMENCLATURE
standard deviations below appropriate norms (i.e., between the third and 16th percentiles) on formal testing or
equivalent clinical evaluation.
• The cognitive deficits are insufficient to interfere with independence (e.g., instrumental activities of daily living,
like more complex tasks such as paying bills or managing medications, are preserved), but greater effort,
compensatory strategies, or accommodation may be required to maintain independence.
• The cognitive deficits do not occur exclusively in the context of a delirium.
• The cognitive deficits are not primarily attributable to another mental disorder (e.g., major depressive
disorder, schizophrenia).
Australian Institute
of Health and
Welfare (AUS)
http://www.aihw.gov.au/WorkArea/Do
wnloadAsset.aspx?id=10737422943
22.0/
15.8
National Task Group
on Intellectual
Disabilities and
Dementia Practices
(USA)
http://aadmd.org/ntg/thinker
16.4/
16.8
National Institute on
Aging (USA)
https://www.nia.nih.gov/alzheimers/top
ics/other-dementias
21.5/
18.3
New Zealand
Ministry of Health
(NZ)
http://www.health.govt.nz/system/files/
documents/publications/new-zealandframework-for-dementia-care-nov13.pdf
0.0/
18.2
National Health
Service (UK)
http://www.nhs.uk/conditions/dementi
a-guide/pages/about-dementia.aspx
33.8/
12.6
In DSM-5, a major neurocognitive disorder is defined by the following:
• There is evidence of substantial cognitive decline from a previous level of performance in one or more of the
domains outlined above based on the concerns of the individual, a knowledgeable informant, or the clinician;
and a decline in neurocognitive performance, typically involving test performance in the range of two or more
standard deviations below appropriate norms (i.e., below the third percentile) on formal testing or equivalent
clinical evaluation.
• The cognitive deficits are sufficient to interfere with independence (i.e., requiring minimal assistance with
instrumental activities of daily living).
• The cognitive deficits do not occur exclusively in the context of a delirium.
• The cognitive deficits are not primarily attributable to another mental disorder (e.g., major depressive
disorder, schizophrenia).
Dementia is not a single specific disease. It is an umbrella term describing a syndrome associated with more than
100 different diseases that are characterized by the impairment of brain functions, including language, memory,
perception, personality and cognitive skills. Although the type and severity of symptoms and their pattern of
development varies with the type of dementia, it is usually of gradual onset, progressive in nature and
irreversible.
Dementia is a term that characterizes the progressive loss of brain function that occurs with certain
neuropathological diseases or trauma and is often associated with aging. It is marked by memory disorders,
personality and behavioral changes, and impaired reasoning. Dementia is not a disease itself, but rather a group
of symptoms that are caused by various degenerative brain diseases or conditions, such as Alzheimer’s disease,
stroke, or other brain trauma. Alzheimer’s disease is the most prevalent cause, associated with some two-thirds
of the instances of dementia. There are different types of dementias, among them Alzheimer’s dementia,
vascular dementia, fronto-temporal dementia, Lewy body dementia are the most prevalent. Dementia related to
a brain disease, such as Alzheimer’s, generally has a progressive nature so that over time the individual affected
continually loses more cognitive and functional skills and eventually is totally unable to function independently.
Dementia is the loss of cognitive functioning—thinking, remembering, and reasoning—and behavioral abilities to
such an extent that it interferes with a person’s daily life and activities. Dementia ranges in severity from the
mildest stage, when it is just beginning to affect a person’s functioning, to the most severe stage, when the
person must depend completely on others for basic activities of daily living.
Dementia is a syndrome due to disease of the brain, usually of a chronic or progressive nature, that results in
cognitive function including memory, thinking, orientation, comprehension, calculation, learning capacity,
language, and judgement. Consciousness is not clouded. Impairments of cognitive function are commonly
accompanied, and occasionally preceded, by deterioration in emotional control, social behavior, or motivation.
Dementia occurs in Alzheimer’s disease, in cerebrovascular disease and in other conditions primarily or
secondarily affecting the brain.
Dementia is a common condition. Your risk of developing dementia increases as you get older, and the condition
usually occurs in people over the age of 65. Dementia is a syndrome (a group of related symptoms) associated
with an ongoing decline of the brain and its abilities. This includes problems with: memory loss, thinking speed,
REPORT OF THE TOPIC GROUP ON NOMENCLATURE
World Health
Organization (INT)
World Health
Organization (INT)
http://www.who.int/mediacentre/facts
heets/fs362/en/
[1]
http://apps.who.int/classifications/apps
/icd/icd10online2007/index.htm?gf00.ht
m+
0.4/
18.1
0.0/
18.6
[2]
http://www.icd10data.com/ICD10CM/C
odes/F01-F99/F01-F09/F03-/F03.90
60.7/
7.8
mental agility, language, understanding, and judgement. People with dementia can become apathetic or
uninterested in their usual activities, and have problems controlling their emotions. They may also find social
situations challenging, lose interest in socialising, and aspects of their personality may change. A person with
dementia may lose empathy (understanding and compassion), they may see or hear things that other people do
not (hallucinations), or they may make false claims or statements. As dementia affects a person's mental
abilities, they may find planning and organising difficult. Maintaining their independence may also become a
problem. A person with dementia will therefore usually need help from friends or relatives, including help with
decision making.
Dementia is a syndrome in which there is deterioration in memory, thinking, behavior and the ability to perform
everyday activities.
[1] Dementia (F00-F03) is a syndrome due to disease of the brain, usually of a chronic or progressive nature, in
which there is disturbance of multiple higher cortical functions, including memory, thinking, orientation,
comprehension, calculation, learning capacity, language, and judgement. Consciousness is not clouded. The
impairments of cognitive function are commonly accompanied, and occasionally preceded, by deterioration in
emotional control, social behaviour, or motivation. This syndrome occurs in Alzheimer's disease, in
cerebrovascular disease, and in other conditions primarily or secondarily affecting the brain.
[2] Dementia is the name for a group of symptoms caused by disorders that affect the brain. It is not a specific
disease. People with dementia may not be able to think well enough to do normal activities, such as getting
dressed or eating. They may lose their ability to solve problems or control their emotions. Their personalities
may change. They may become agitated or see things that are not there. Memory loss is a common symptom of
dementia. However, memory loss by itself does not mean you have dementia. People with dementia have
serious problems with two or more brain functions, such as memory and language. Although dementia is
common in very elderly people, it is not part of normal aging. Many different diseases can cause dementia,
including Alzheimer’s disease and stroke. Drugs are available to treat some of these diseases. While these drugs
cannot cure dementia or repair brain damage, they may improve symptoms or slow down the disease.
Note: FRES: Flesch Reading Ease score24; F-KGL: Flesch-Kincaid Grade Level score [Rates text on a U.S. grade-school level. For example, a score of 8.0 means that an eighth
grader can understand the document.]
24
https://en.wikipedia.org/wiki/Flesch%E2%80%93Kincaid_readability_tests
90.0–100.0 5th grade
Very easy to read. Easily understood by an average 11-year-old student.
80.0–90.0
6th grade
Easy to read. Conversational English for consumers.
70.0–80.0
7th grade
Fairly easy to read.
60.0–70.0
8th & 9th grade
Plain English. Easily understood by 13- to 15-year-old students.
50.0–60.0
10th to 12th grade Fairly difficult to read.
30.0–50.0
college
Difficult to read.
0.0–30.0
college graduate
Very difficult to read. Best understood by university graduates.
REPORT OF THE TOPIC GROUP ON NOMENCLATURE
Appendix B: Taxonomy of terms related to dementia and intellectual disability
Term
Definition
Usage/applications
Source
General descriptor
[1] The Later Stages of Dementia. Alzheimer’s Society
(GB). (2012).
https://www.alzheimers.org.uk/site/scripts/document
s_info.php?documentID=101
Diagnostic and condition-specific terms
Advanced dementia
Alzheimer’s disease
[1] The later stages of dementia are characterized by memory loss,
problems with communication, loss of mobility, eating and weight loss,
problems with continence, and unusual behavior.
[2] In adults with advanced dementia, the final year of life is characterized
by a trajectory of persistently severe disability. Stage 7 on the Global
Deterioration Scale (ranging from 1 to 7, with higher stages indicating
worse dementia) provides a useful description of the features of advanced
dementia, including profound memory deficits (e.g., inability to recognize
family members), minimal verbal abilities, inability to ambulate
independently, inability to perform any activities of daily living, and urinary
and fecal incontinence. The most common clinical complications are eating
problems, febrile episodes, and pneumonia.
[1] Alzheimer's disease (AD) is an age-related, non-reversible brain disorder
that develops over a period of years. Initially, people experience memory
loss and confusion, which may be mistaken for the kinds of memory
changes that are sometimes associated with normal aging. However, the
symptoms of AD gradually lead to behavior and personality changes, a
decline in cognitive abilities such as decision-making and language skills,
and problems recognizing family and friends. AD ultimately leads to a
severe loss of mental function. These losses are related to the worsening
breakdown of the connections between certain neurons in the brain and
their eventual death.
[2] Alzheimer's disease is a progressive, degenerative disorder that attacks
the brain's nerve cells, or neurons, resulting in loss of memory, thinking
and language skills, and behavioral changes. These neurons, which produce
the brain chemical, or neurotransmitter, acetylcholine, break connections
with other nerve cells and ultimately die. For example, short-term memory
fails when Alzheimer's disease first destroys nerve cells in the
hippocampus, and language skills and judgment decline when neurons die
in the cerebral cortex. Two types of abnormal lesions clog the brains of
individuals with Alzheimer's disease: Beta-amyloid plaques—sticky clumps
of protein fragments and cellular material that form outside and around
neurons; and neurofibrillary tangles—insoluble twisted fibers composed
largely of the protein tau that build up inside nerve cells. Although these
structures are hallmarks of the disease, scientists are unclear whether they
cause it or a byproduct of it. Alzheimer's disease is the most common cause
[2] Mitchell, S.L. Advanced Dementia. N Engl J Med
2015; 372:2533-2540 June 25, 2015 DOI:
10.1056/NEJMcp1412652
General diagnostic
descriptor
Specific diagnostic
descriptor
[1] What is Alzheimer's Disease? NINDS Alzheimer's
Disease Information Page. National Institute of
Neurological Disorders and Stroke.
http://www.ninds.nih.gov/disorders/alzheimersdiseas
e/alzheimersdisease.htm
[2] Defining Alzheimer’s Disease. About Alzheimer's
Disease. Alzheimer’s Foundation of America.
http://www.alzfdn.org/AboutAlzheimers/definition.ht
ml
REPORT OF THE TOPIC GROUP ON NOMENCLATURE
Alzheimer’s disease
spectrum
Behavioral and
psychological
symptoms of
dementia (BPSD
Cognitive disability
Cognitive impairment
of dementia, or loss of intellectual function, among people aged 65 and
older.
Term identifying the entire spectrum of Alzheimer’s disease (AD) including
preclinical AD, mild cognitive impairment due to AD, and dementia due to
AD; a major feature to distinguish between the clinical characteristics of
the disorder and the pathological features. The criteria include the use of
imaging and other biomarkers to aid in diagnosis.
Behavioral and psychological symptoms of dementia (BPSD), also known as
neuropsychiatric symptoms, represent a heterogeneous group of noncognitive symptoms and behaviors occurring in subjects with dementia.
BPSD constitute a major component of the dementia syndrome
irrespective of its subtype. They are as clinically relevant as cognitive
symptoms as they strongly correlate with the degree of functional and
cognitive impairment. BPSD include agitation, aberrant motor behavior,
anxiety, elation, irritability, depression, apathy, disinhibition, delusions,
hallucinations, and sleep or appetite changes. It is estimated that BPSD
affect up to 90% of all dementia subjects over the course of their illness,
and is independently associated with poor outcomes, including distress
among patients and caregivers, long-term hospitalization, misuse of
medication, and increased health care costs. Although these symptoms can
be present individually it is more common that various psychopathological
features co-occur simultaneously in the same patient. Thus, categorization
of BPSD in clusters taking into account their natural course, prognosis, and
treatment response may be useful in the clinical practice. The pathogenesis
of BPSD has not been clearly delineated but it is probably the result of a
complex interplay of psychological, social, and biological factors.
[1] The term cognitive disabilities encompasses various intellectual or
cognitive deficits, including intellectual disability, developmental delay,
developmental disability, learning disabilities, and conditions causing
cognitive impairment such as acquired brain injuries or neurodegenerative
diseases like dementia.
[2] A cognitive disability causes a person to have greater difficulty with one
or more types of mental tasks than the average person; cognitive
disabilities can be characterized as functional disability or by clinical
disability. Clinical diagnoses of cognitive disabilities include autism, Down
syndrome, traumatic brain injury (TBI), and even dementia. Less severe
cognitive conditions include attention deficit disorder (ADD), dyslexia
(difficulty reading), dyscalculia (difficulty with math), and learning
disabilities in general. In some jurisdictions, cognitive disability refers to
intellectual disability.
Cognitive impairment is when a person has trouble remembering, learning
new things, concentrating, or making decisions that affect their everyday
life. Cognitive impairment ranges from mild to severe. With mild
impairment, people may begin to notice changes in cognitive functions, but
still be able to do their everyday activities. Severe levels of impairment can
lead to losing the ability to understand the meaning or importance of
Theoretical medical
descriptor
Petersen, R.C. (2012). New clinical criteria for the
Alzheimer’s disease spectrum. Minn Med, Jan., 95(1),
42-45.
http://www.ncbi.nlm.nih.gov/pubmed/22355912
General descriptor
Cerejeira, J., Lagarto, L., & Mukaetova-Ladinska, E.B.,
(2012). Behavioral and psychological symptoms of
dementia. Frontiers in Neurology, 3(73), 1-21. Doi:
10.3389/fneur.2012.00074
General descriptor
[1] Cognitive disabilities. (n.d.). Children with Special
Needs.
http://www.childrenwithspecialneeds.com/disabilityinfo/cognitive-disabilities/
[2] Cognitive. (2016). WebAIM – web accessibility in
mind.
http://webaim.org/articles/cognitive/
General descriptor
Cognitive impairment: A call for Action, Now! Centers
for Disease Control and Prevention. (2011).
https://www.cdc.gov/aging/pdf/cognitive_impairment
/cogimp_poilicy_final.pdf
REPORT OF THE TOPIC GROUP ON NOMENCLATURE
Dementia
something and the ability to talk or write, resulting in the inability to live
independently.
[1] Dementia is a collective name for progressive degenerative brain
syndromes which affect memory, thinking, behavior and emotion.
Symptoms may include: loss of memory, difficulty in finding the right words
or understanding what people are saying, difficulty in performing
previously routine tasks, and personality and mood changes.
[2] Dementia describes a set of symptoms that may include memory loss
and difficulties with thinking, problem-solving or language. Dementia is
caused when the brain is damaged by diseases, such as Alzheimer's disease
or a series of strokes. Dementia is progressive, which means the symptoms
will gradually get worse.
[3] Dementia is a term that characterizes the progressive loss of brain
function that occurs with certain neuropathological diseases or trauma and
is often associated with aging. It is marked by memory disorders,
personality and behavioral changes, and impaired reasoning. Dementia is
not a disease itself, but rather a group of symptoms that are caused by
various degenerative brain diseases or conditions, such as Alzheimer’s
disease, stroke, or other brain trauma. Alzheimer’s disease is the most
prevalent cause, associated with some two-thirds of the instances of
dementia. There are different types of dementias, among them Alzheimer’s
dementia, vascular dementia, fronto-temporal dementia, Lewy body
dementia are the most prevalent. Dementia related to a brain disease,
such as Alzheimer’s, generally has a progressive nature so that over time
the individual affected continually loses more cognitive and functional skills
and eventually is totally unable to function independently.
[4] Dementia is defined as ‘‘a progressive cognitive decline of sufficient
magnitude to interfere with normal social or occupational function.’’
Dementia assessment
(screening)
[5] Dementia is the loss of cognitive functioning—thinking, remembering,
and reasoning—to such an extent that it interferes with a person’s daily
life. Dementia is not a disease itself, but rather a set of symptoms. Memory
loss is a common symptom of dementia, although memory loss by itself
does not mean a person has dementia. Alzheimer’s disease is the most
common form of dementia, accounting for the majority of all diagnosed
cases.
Assessment (or screening) is undertaken to increase the precision of a
decision by reducing subjectivity and increasing objectivity. The properties
of an ideal assessment (screening) scale would be that it is valid, that is, it
has face validity (experts like clinicians, patients and carers would agree
that the questions are relevant and important), that it has construct
validity (it measures the construct it was designed to measure), concurrent
validity (when used alongside a gold standard assessment like a very well
validated scale or an expert clinical assessment, it performs well), that it
General descriptor
[1] About dementia. (n.d.)
Alzheimer’s Disease International
https://www.alz.co.uk/about-dementia
[2] About dementia. (2016). Alzheimer’s Society (GB).
https://www.alzheimers.org.uk/site/scripts/document
s.php?categoryID=200120
[3] National Task Group on Intellectual Disabilities and
Dementia Practice. (2012). ‘My Thinker’s Not
Working’: A National Strategy for Enabling Adults with
Intellectual Disabilities Affected by Dementia to
Remain in Their Community and Receive Quality
Supports.
www.aadmd.org/ntg/thinker
[4] McKeith, I., Taylor, J-P., Thomas, A., Donaghy, P., &
Kane, J. (2016).
Revisiting DLB Diagnosis: A Consideration of
Prodromal DLB and of the Diagnostic Overlap with
Alzheimer Disease. Journal of Geriatric Psychiatry and
Neurology 2016, Vol. 29(5) 249-253.
[5] Office of Disease Prevention and Health Promotion
(2014). Dementias, Including Alzheimer's Disease.
Overview. (Healthy People 2020).
https://www.healthypeople.gov/2020/topicsobjectives/topic/dementias-including-alzheimersdisease
Sheenhan, B. (2012). Assessment scales in dementia.
Ther Adv Neurol Disord (2012) 5(6) 349 –358.
REPORT OF THE TOPIC GROUP ON NOMENCLATURE
Dementia diagnosis –
mild cognitive
impairment
Dementia diagnosis –
probable dementia
Dementia diagnosis –
possible dementia
Dementia diagnosis –
people with ID
Dementia screening –
people with ID
shows reliability – typically inter-rater reliability (two or more raters using
the scale in the same subjects and conditions come up with the same
result) and test–retest reliability (the same rater using the scale on another
occasion in the same subject comes up with the same result). Importantly,
it should be practical to use – in practice, this often depends on it being
short (so it can be used in busy clinical practice … (and)… participants are
not overburdened by long interviews) and acceptable – so it does not
upset, exhaust or embarrass the patient or assessor.
Diagnostic criteria for mild cognitive impairment (MCI)
MCI is diagnosed when there is concern regarding a decline in cognition
reported by the patient, informant, or clinician, and there is objective
evidence of cognitive deficits in 1 or more domains (typically memory) and
most important there is preservation of independence in functional
abilities. The differentiation of dementia from MCI rests on whether there
is substantial interference in the ability to function at work or in usual daily
activities. This is a clinical judgment. Further evaluation examines the
pathogenesis of MCI, focusing on ruling out vascular, traumatic, and
medical causes, and consideration of AD genetic factors
Diagnostic criteria for probable Alzheimer’s dementia (AD) Probable AD is
diagnosed when the criteria for dementia are met, and symptoms have a
gradual onset over months to years, not suddenly over hours or days, and
there is clear worsening of cognition. Additionally, the initial and most
prominent cognitive deficits are usually amnestic (associated with
impairment in learning and recall of recently learned information) or less
commonly nonamnestic (when language deficits are most prominent, e.g.,
word-finding difficulties). Deficits should also occur in other domains such
as visuospatial abilities (face or object recognition) and executive function
(reasoning, judgment, problem solving). The diagnosis of probable AD
should not be applied when substantial concomitant cerebrovascular
disease is present.
Diagnostic criteria for possible Alzheimer’s dementia (AD) A diagnosis of
possible AD should be made when the criteria for AD are met (regarding
the nature of cognitive deficits) but the disease follows an atypical course
(eg, there is a sudden onset of cognitive impairment and cognitive decline
is not gradual), or when criteria for AD are met but there is evidence of a
mixed presentation, such as concomitant cerebrovascular disease, or the
patient has clinical features of dementia with Lewy bodies, has another
comorbidity (medical or neurologic), or is using medication that could have
a substantial effect on cognition.
Diagnosis in people with ID is generally on the basis of meeting
criteria ICD-10 (World Health Organization, 1992), DSM-IV-TR (American
Psychiatric Association, 2000).
Reactive screening is the assessment of functioning and exploring the
reasons for observed deterioration in any adult with intellectual disabilities
Guides the choice of
tests/assessments and
offers guidance for
consensus clinical diagnosis
conferencing
Moore, A., Patterson, C., Lee, L., Vedel, I., & Bergman,
H. (2014). Fourth Canadian Consensus Conference on
the Diagnosis and Treatment of Dementia:
Recommendations for Family Physicians. Canadian
Family Physician, 2014, 60(5), 433-438.
http://www.cfp.ca/content/60/5/433.full
Guides the choice of
tests/assessments and
offers guidance for
consensus clinical diagnosis
conferencing
Moore, A., Patterson, C., Lee, L., Vedel, I., & Bergman,
H. (2014). Fourth Canadian Consensus Conference on
the Diagnosis and Treatment of Dementia:
Recommendations for Family Physicians. Canadian
Family Physician, 2014, 60(5), 433-438.
http://www.cfp.ca/content/60/5/433.full
Guides the choice of
tests/assessments and
offers guidance for
consensus clinical diagnosis
conferencing
Moore, A., Patterson, C., Lee, L., Vedel, I., & Bergman,
H. (2014). Fourth Canadian Consensus Conference on
the Diagnosis and Treatment of Dementia:
Recommendations for Family Physicians. Canadian
Family Physician May 2014, 60(5), 433-438.
http://www.cfp.ca/content/60/5/433.full
Guides the choice of
tests/assessments and
offers guidance for
consensus clinical diagnosis
conferencing
Guides the choice of
tests/assessments and
Strydom A., Chan, T., King, M., Hassiotis, A., &
Livingston, G. (2013). Incidence of dementia in older
adults with intellectual disabilities. Res Dev Disabil.
2013 Jun;34(6):1881-5. doi:
10.1016/j.ridd.2013.02.021.
British Psychological Society. (2015). Dementia and
People with Intellectual Disabilities Guidance on the
REPORT OF THE TOPIC GROUP ON NOMENCLATURE
Dementia due to
Alzheimer’s disease
Dementia and
intellectual disability
after concerns have been raised; recommended to establish a baseline for
every adult with Down’s syndrome whilst they are healthy – ideally at age
30 – and then undertaking prospective screening for dementia for adults
with Down’s syndrome conducted at intervals from the age of 30 onwards.
Prospective screening (monitoring) entails checking for early signs of
dementia by repeating the baseline assessment at regular interval and the
frequency of prospective screening (monitoring) for dementia should be
matched to the rising risk with age.
Dementia when there are cognitive or behavioral (neuropsychiatric)
symptoms that:
1.Interfere with the ability to function at work or at usual activities; and
2.Represent a decline from previous levels of functioning and performing;
and
3.Are not explained by delirium or major psychiatric disorder;
4.Cognitive impairment is detected and diagnosed through a combination
of (1) history-taking from the patient and a knowledgeable informant and
(2) an objective cognitive assessment, either a “bedside” mental status
examination or neuropsychological testing. Neuropsychological testing
should be performed when the routine history and bedside mental status
examination cannot provide a confident diagnosis.
5.The cognitive or behavioral impairment involves a minimum of two of the
following domains:
• Impaired ability to acquire and remember new information—symptoms
include: repetitive questions or conversations, misplacing personal
belongings, forgetting events or appointments, getting lost on a familiar
route.
•Impaired reasoning and handling of complex tasks, poor judgment—
symptoms include: poor understanding of safety risks, inability to manage
finances, poor decision-making ability, inability to plan complex or
sequential activities.
•Impaired visuospatial abilities—symptoms include: inability to recognize
faces or common objects or to find objects in direct view despite good
acuity, inability to operate simple implements, or orient clothing to the
body.
•Impaired language functions (speaking, reading, writing)—symptoms
include: difficulty thinking of common words while speaking, hesitations;
speech, spelling, and writing errors.
•Changes in personality, behavior, or comportment—symptoms include:
uncharacteristic mood fluctuations such as agitation, impaired motivation,
initiative, apathy, loss of drive, social withdrawal, decreased interest in
previous activities, loss of empathy, compulsive or obsessive behaviors,
socially unacceptable behaviors.
Adults with an intellectual disability affected by dementia as those who:
● have intellectual limitations that significantly limit the person’s ability to
successfully participate in normal day-to-day activities such as self-care,
communication, work, or going to school, and
offers guidance for
consensus clinical diagnosis
conferencing
assessment, diagnosis, interventions and support of
people with intellectual disabilities who develop
dementia. Author: Leicester, United Kingdom.
Specific diagnostic
descriptor
McKhann et al. 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. Alzheimers Dement. 2011 May; 7(3): 263–
269.
General descriptor
National Task Group on Intellectual Disabilities and
Dementia Practice. (2012). ‘My Thinker’s Not
Working’: A National Strategy for Enabling Adults with
Intellectual Disabilities Affected by Dementia to
REPORT OF THE TOPIC GROUP ON NOMENCLATURE
Dementia stages
Dementia symptoms
– Down syndrome
● developed the intellectual limitation during the ‘developmental period’
(before approximately age 22), and
● the limitation is anticipated to result in long term adaptive or functional
support needs, and/or
● are eligible for State or Federal public support programs because they
have been diagnosed as having an intellectual disability; and
● are affected by dementia, and meet the criteria of having been
diagnosed with possible, probable, or definitive dementia, or have mild
cognitive impairment, as defined by the World Health Organization’s
International Classification of Diseases or meet the diagnostic criteria of
the American Psychiatric Association’s Diagnostic and Statistical Manual.
[1] Dementia can be divided in three stages:
• early stage – first year or two
• middle stage – second to fourth or fifth years
• late stage – fifth year and after
These periods are given as an approximate guideline and not all persons
with dementia will display the same symptoms.
[2] The middle and later stages of dementia in people with Down's
syndrome are similar to these stages in the general population. There is
some evidence that dementia in people with Down's syndrome progresses
more rapidly and that they may have earlier loss of basic skills such as
walking, becoming incontinent, and having swallowing difficulties.
[1] The symptoms of dementia in people with Down syndrome are broadly
similar to those seen in the general population, although there are some
differences. Changes in behavior and personality (e.g., becoming more
stubborn, irritable or withdrawn) or loss of daily living abilities are
common. Memory loss, the most common early symptom of Alzheimer's
disease among older people generally, is seen less often as an early
symptom in people with Down syndrome. This may be because most
people with Down syndrome will already have poor short-term memory.
[2] The symptoms of Alzheimer’s disease might be expressed differently
among adults with Down syndrome. For example, in the early stages of the
disease, memory loss is not always prominent. In addition, not all
symptoms ordinarily associated with Alzheimer’s disease will occur.
Generally, changes in activities of daily living skills occur, and the person
with Down syndrome might begin to have seizures when he or she had not
had them in the past. Changes in mental processes—such as thinking,
reasoning, and judgment—also might be present, but they often are not
readily noticeable because of limitation of the individual’s functioning in
general.
[3] The symptoms of Alzheimer’s disease show wide differences between
people with Down syndrome. In the early stage of the disease, memory
loss is not always seen, and not all the symptoms associated with
Alzheimer’s disease will be recognizable. Generally, changes in activities of
Remain in Their Community and Receive Quality
Supports.
www.aadmd.org/ntg/thinker
General descriptor
[1] B. Duthey. (2013). WHO Background Paper 6.11:
Alzheimer’s disease and other dementias.
http://www.who.int/medicines/areas/priority_medici
nes/BP6_11Alzheimer.pdf
[2] Learning Disabilities and Dementia. (2016).
Alzheimer’s Society (UK).
https://www.alzheimers.org.uk/site/scripts/document
s_info.php?documentID=103
General descriptor
[1] Learning Disabilities and Dementia. (2016).
Alzheimer’s Society (UK).
https://www.alzheimers.org.uk/site/scripts/document
s_info.php?documentID=103
[2] Alzheimer’s Disease and Down Syndrome. (2011).
Cleveland Clinic.
http://my.clevelandclinic.org/health/diseases_conditi
ons/hic_Alzheimers_and_Dementia_Overview/hic_Alz
heimers_Disease_and_Down_Syndrome
[3] Dementia and intellectual disabilities. Alzheimer’s
Disease International. (2003).
http://www.alz.co.uk/adi/pdf/intellectualdisabilities.p
df
[4] Down syndrome. Neurodegenerative diseases.
Alzheimer’s Europe. http://www.alzheimereurope.org/Dementia/Other-forms-ofdementia/Neurodegenerative-diseases/Downsyndrome
REPORT OF THE TOPIC GROUP ON NOMENCLATURE
daily living and work habits are noticed first. Epileptic seizures may occur
early or late in the course of the disease. Cognitive changes are frequently
present but they are difficult to evaluate because of limitations in the
individual’s language, communication and related intellectual abilities.
Dementia symptoms
– intellectual
disability
Diagnosing
Alzheimer’s disease in
Down syndrome
[4] Clinical symptoms at first are increasing depression, indifference and a
decline in social communication. Later symptoms are: seizures in previously
unaffected persons, changes in personality, loss of memory and general
functions, long periods of inactivity or apathy, hyperactive reflexes, loss of
activity of daily skills, visual retention deficits, loss of speech,
disorientation, increase in stereotyped behavior and abnormal neurological
signs. Average age of onset is 54 years and average interval from diagnosis
to death is less than 5 years.
[1] The symptoms of dementia in people with an intellectual disability
(other than Down syndrome) can vary widely. For those with mild
intellectual disability and dementia seems to appear and progress similarly
to dementia in the general population. For those with more severe
intellectual disability, the initial symptoms of dementia are often less
typical, possibly involving changes in personality or behavior, which can
make diagnosing dementia more difficult.
[2] Warning signs of Alzheimer’s disease for adults with intellectual and
developmental disabilities include loss of activity of daily living skills;
difficulty with well-learned abilities, changes in personality; more
withdrawn, frustration; periods of inactivity or apathy, disinterest in
activities the individual previously enjoyed; development of seizures not
previously seen; disorientation to time and place; increase in stereotyped
behaviors; hyperactive reflexes; visual retention deficits; speech
difficulties, not able to use words or speech that is not clear
[1] The process of making a diagnosis of Alzheimer’s disease in someone
with Down syndrome is the same as making a diagnosis of Alzheimer’s
disease in anyone else. Diagnosis is based on a detailed history of
progressive change over time in thinking, memory and daily living skills,
physical examination, tests of thinking and memory, and investigations
(blood tests and brain scans) to rule out other causes of decline in
functioning.
[2] Making a diagnosis of Alzheimer’s disease can be difficult when the
person has Down syndrome for a number of reasons:
• People with Down syndrome are susceptible to a number of reversible
conditions that can be mistaken for Alzheimer’s disease. These include
hypothyroidism and depression. The side effects of some medications can
also mimic Alzheimer’s disease
• The usual skill tests used for diagnosis do not take into account the
existing problems of a person with Down syndrome
• The limited communication skills of some people with Down syndrome
may affect the assessment.
[1] Learning Disabilities and Dementia. (2016).
Alzheimer’s Society (UK).
https://www.alzheimers.org.uk/site/scripts/document
s_info.php?documentID=103
[2] Berry, M., Alzheimer's and Individuals with
Intellectual and Developmental Disabilities. In Heller,
T., Stafford, P., Davis, L.A., Sedlezky, L., & Gaylord, V.
(Eds.). (Winter 2010). Impact: Feature Issue on Aging
and People with Intellectual and Developmental
Disabilities, 23(1). [Minneapolis: University of
Minnesota, Institute on Community Integration].
https://ici.umn.edu/products/impact/231/21.html
[1] Down syndrome and Alzheimer’s disease. (n.d.).
Alzheimer’s Australia and Down Syndrome Victoria.
http://www.cddh.monash.org/assets/dsad-bookletfinal.pdf
[2] Down Syndrome and Alzheimer's Disease. (n.d.).
Alzheimer’s Australia.
https://www.fightdementia.org.au/aboutdementia/types-of-dementia/down-syndrome-andalzheimers-disease
REPORT OF THE TOPIC GROUP ON NOMENCLATURE
Early onset
Alzheimer’s disease
[1] Younger-onset (also known as early-onset) Alzheimer's disease affects
people younger than age 65.
General descriptor
[2] The early-onset form of Alzheimer’s disease most often shows up in the
40s and 50s; in some persons it may show in the 30s.
Early onset Dementia
– people with Down
syndrome
[2] What Is Early-Onset Alzheimer's Disease?
Alzheimer’s Disease Health Center. (2016).
http://www.webmd.com/alzheimers/guide/earlyonset-alzheimers
[1] Stanton, L.R., & Coetzee, R.H. (2003). Down’s
syndrome and dementia. Advances in Psychiatric
Treatment, Dec 2003, 10(1), 50-58; DOI:
10.1192/apt.10.1.50
[1] Age-related cognitive decline and frontal lobe dementia appear to be
more prevalent in the younger age groups (30–49 years of age) of adults
with Down syndrome, and frontal lobe dementia appears to be more
prevalent in those adults with more severe intellectual disability. Earlyonset presents with more frontal symptoms and this may represent a
different clinical subtype of Alzheimer’s disease in Down’s syndrome.
[2] Down Syndrome and Alzheimer's Disease. (2016).
Alzheimer’s Association.
http://www.alz.org/dementia/down-syndromealzheimers-symptoms.asp
[2] As adults with Down syndrome live, on average, 55 to 60 years, they are
more likely to develop younger-onset Alzheimer’s (Alzheimer’s occurring
before age 65) than older-onset Alzheimer’s (Alzheimer’s occurring at age
65 or older). Autopsy studies show that by age 40, the brains of almost all
individuals with Down syndrome have significant levels of plaques and
tangles, abnormal protein deposits considered Alzheimer's hallmarks. But
despite the presence of these brain changes, not everyone with the
syndrome develops Alzheimer's symptoms.
Frontotemporal
dementia
Lewy-body dementia
[3] Studies have also shown that by the age of about 40, almost all people
with Down's syndrome develop changes in the brain associated with
Alzheimer's disease. However, not all go on to develop clinical symptoms of
dementia. The reason for this increased risk has not been fully identified,
however, it is thought to be linked to the extra copy of chromosome 21
which most people with Down's syndrome have. This chromosome carries
the amyloid gene thought to play a role in Alzheimer's disease.
[1] Frontotemporal dementia (FTD) or frontotemporal degenerations refers
to a group of disorders caused by progressive nerve cell loss in the brain's
frontal lobes (the areas behind the forehead) or its temporal lobes (the
regions behind the ears).
[2] Frontotemporal degeneration (FTD) is a disease process that results in
progressive damage to the temporal and/or frontal lobes of the brain. It
causes a group of brain disorders that share many clinical features. FTD is
also commonly referred to as frontotemporal dementia, fronto-temporal
lobar degeneration (FTLD), or Picks disease.
[1] Lewy body dementia, also known as dementia with Lewy bodies, is the
second most common type of progressive dementia after Alzheimer's
disease dementia. Protein deposits, called Lewy bodies, develop in nerve
cells in the brain regions involved in thinking, memory and movement
(motor control). Lewy body dementia causes a progressive decline in
[1] Younger/early onset Alzheimer’s & dementia.
Alzheimer’s Association. (2016).
http://www.alz.org/alzheimers_disease_early_onset.a
sp
[3] Learning Disabilities and Dementia. (2016).
Alzheimer’s Society (UK).
https://www.alzheimers.org.uk/site/scripts/document
s_info.php?documentID=103
Specific diagnostic
descriptor
[1] Frontotemporal Dementia (FTD). Alzheimer’s &
dementia. (2016).
http://www.alz.org/dementia/fronto-temporaldementia-ftd-symptoms.asp
[2] Disease overview. The Association for
Frontotemporal Degeneration. (2016).
http://www.theaftd.org/understandingftd/ftdoverview
Specific diagnostic
descriptor
[1] Lewy body dementia. Patient care & health
information. (2016).
http://www.mayoclinic.org/diseases-conditions/lewybody-dementia/home/ovc-20200344
REPORT OF THE TOPIC GROUP ON NOMENCLATURE
mental abilities. People with Lewy body dementia may experience visual
hallucinations, and changes in alertness and attention. Other effects
include Parkinson's disease-like symptoms such as rigid muscles, slow
movement and tremors.
Mild cognitive
impairment
Mild cognitive
impairment due to
Alzheimer’s disease
[2] A new category of features ‘‘suggestive’’ of DLB includes rapid eye
movement sleep behavior disorder (RBD), severe neuroleptic sensitivity,
and abnormal dopamine transporter neuroimaging imaging…. if one or
more of these suggestive features are present, in addition to one or more
core features (i.e., fluctuating cognition with pronounced variations in
attention and alertness; recurrent visual hallucinations that are typically
well formed and detailed; and spontaneous motor features of
parkinsonism), a diagnosis of probable DLB could be made. Possible DLB
was to be diagnosed if one or more suggestive features were present in a
patient with dementia even in the absence of any core features.
Mild cognitive impairment (MCI) causes a slight but noticeable and
measurable decline in cognitive abilities, including memory and thinking
skills. A person with MCI is at an increased risk of developing dementia due
to Alzheimer's disease or dementia due to other neuropathology.
MCI—Criteria for the clinical and cognitive syndrome
•Concern regarding a change in cognition
There should be evidence of concern about a change in
cognition, in comparison with the person’s previous level.
This concern can be obtained from the patient, from an informant who
knows the patient well, or from a skilled clinician observing the patient.
•Impairment in one or more cognitive domains
There should be evidence of lower performance in one or
more cognitive domains that is greater than would be expected for the
patient’s age and educational background. If repeated assessments are
available, then a decline in performance should be evident over time. This
change can occur in a variety of cognitive domains, including memory,
executive function, attention, language, and visuospatial skills. An
impairment in episodic memory (i.e., the ability to learn and retain new
information) is seen most commonly in MCI patients who subsequently
progress to a diagnosis of AD dementia.
•Preservation of independence in functional abilities
Persons with MCI commonly have mild problems performing complex
functional tasks which they used to perform previously, such as paying
bills, preparing a meal, or shopping. They may take more time, be less
efficient, and make more errors at performing such activities than in the
past. Nevertheless, they generally maintain their independence of function
in daily life, with minimal aids or assistance. It is recognized that the
application of this criterion is challenging, as it requires knowledge about
an individual’s level of function at the current phase of their life. However,
it is noteworthy that this type of information is also necessary for the
determination of whether a person is demented.
[2] McKeith, I., Taylor, J-P., Thomas, A., Donaghy, P., &
Kane, J. (2016).
Revisiting DLB diagnosis: A consideration of prodromal
DLB and of the diagnostic overlap with Alzheimer
disease. Journal of Geriatric Psychiatry and Neurology
2016, Vol. 29(5) 249-253.
General descriptor
Specific diagnostic
descriptor
Alzheimer’s & Dementia: Mild cognitive impairment.
Alzheimer’s Association. (2016).
http://www.alz.org/dementia/mild-cognitiveimpairment-mci.asp
Albert, M.S., DeKosky, S.T., Dickson, D., Dubois, B.,
Feldman, H.H., Fox, N.C., Gamst, A., Holtzman, D.M.,
Jagust, W.J., Petersen, R.C. et al. (2011). The diagnosis
of mild cognitive impairment due to Alzheimer’s
disease: Recommendations from the National
Institute on Aging and Alzheimer’s Association
workgroup. Alzheimer’s Dement. 2011 May; 7(3):2709.
REPORT OF THE TOPIC GROUP ON NOMENCLATURE
Neurodegenerative
disorders
Neurodevelopmental
disorders
Parkinson disease
dementia
Rapidly progressive
dementia
Vascular dementia
[1] Disorders involving diseases in which cells of the central nervous system
stop working or die. Neurodegenerative disorders usually get worse over
time and have no cure. They may be genetic or be caused by a tumor or
stroke. Neurodegenerative disorders also occur in people who drink large
amounts of alcohol or are exposed to certain viruses or toxins. Examples of
neurodegenerative disorders include Alzheimer's disease and Parkinson's
disease.
[2] Neurodegenerative disease is an umbrella term for a range of
conditions which primarily affect the neurons in the human brain. Neurons
are the building blocks of the nervous system which includes the brain and
spinal cord. Neurons normally don’t reproduce or replace themselves, so
when they become damaged or die they cannot be replaced by the body.
Examples of neurodegenerative diseases include Parkinson’s, Alzheimer’s,
and Huntington’s disease. Neurodegenerative diseases are incurable and
debilitating conditions that result in progressive degeneration and / or
death of nerve cells. This causes problems with movement (called ataxias),
or mental functioning (called dementias).
Neurodevelopmental disorders are a group of disorders in which the
development of the central nervous system is disturbed. This can include
developmental brain dysfunction, which can manifest as neuropsychiatric
problems or impaired motor function, learning, language or non-verbal
communication.
Parkinson disease dementia (PDD) is defined as cognitive impairment that
has reached the stage of dementia and which appears in the context of
preexisting PD. Visual hallucinations, autonomic, and sleep disturbances
are common. A number of authors consider dementia with Lewy bodies
(DLB) and PDD as two syndromes within the same disease spectrum
Rapidly progressive dementia is a rare condition with a large number of
possible causes. Rapidly progressive dementia has been poorly defined in
the literature. Although Creutzfeldt-Jakob disease is a prominent cause,
care must be taken to identify potentially treatable conditions, such as
unsuspected vascular disease, reversible structural pathogenesis (26% of
RPDs), infections, and immunologically mediated disorders. RPD is a
condition that develops within 12 months after the appearance of the first
cognitive symptoms.
[1] Vascular dementia occurs when the brain is damaged because of
problems with the supply of blood to the brain, and results in symptoms
such as memory loss and difficulties with thinking, problem-solving or
language. When the vascular system within the brain becomes damaged so that the blood vessels leak or become blocked - then blood cannot reach
the brain cells and they will eventually die and this can cause problems
with memory, thinking or reasoning.
[2] Vascular dementia is a syndrome comprising ischemic and hemorrhagic
etiologies. The dominant feature of the cognitive syndrome is an abnormal
executive function that interferes with social or occupational functioning.
The term vascular cognitive impairment (VCI) was devised to incorporate
General descriptors
[1] NCI Dictionary of Cancer Terms. (n.d.).
Neurodegenerative disorders.
https://www.cancer.gov/publications/dictionaries/can
cer-terms?cdrid=687218
[2] EU Joint Programme – Neurogenerative Disease
Research. (2014). What is Neurodegenerative
Disease?
http://www.neurodegenerationresearch.eu/about/wh
at/
General descriptor
Nature.com. (2016). Neurodevelopmental disorders.
http://www.nature.com/subjects/neurodevelopment
al-disorders
Specific diagnostic
descriptor
Garcia-Ptacek, S. & Kramberger, M.G. (2016).
Parkinson disease and dementia. Journal of Geriatric
Psychiatry and Neurology 2016, Vol. 29(5) 261-270.
Specific diagnostic
descriptor
Moore, A., Patterson, C., Lee, L., Vedel, I., & Bergman,
H. (2014). Fourth Canadian Consensus Conference on
the Diagnosis and Treatment of Dementia:
Recommendations for Family Physicians. Canadian
Family Physician May 2014, 60(5), 433-438.
http://www.cfp.ca/content/60/5/433.full
Specific diagnostic
descriptor
[1] What is vascular dementia? About dementia.
(2016). Alzheimer’s Society.
https://www.alzheimers.org.uk/site/scripts/document
s_info.php?documentID=161
[2] Khan, A. Kalaria, R.N., Corbett, A.., & Ballard, C.
(2161). Update on vascular dementia. Journal of
Geriatric Psychiatry and Neurology 2016, Vol. 29(5)
281-301
REPORT OF THE TOPIC GROUP ON NOMENCLATURE
conditions in any cognitive domain with a vascular origin or impaired brain
perfusion and encompasses a slow, progressive, clinical course and the
contributions of vascular disease to cognitive impairment (CI) as does
vascular cognitive disorder (VCD).
General social care terms
Advanced care
planning
Dementia capable
Advance care planning (ACP) is a process of discussing and sharing planning
for future health care. For the purposes of this framework, ACP focuses on
the person with dementia and involves both that person and the health
care professionals responsible for their care. ACP may also involve the
person with dementia’s family if that is the person’s wish. ACP helps the
person with dementia identify their personal beliefs and values and
incorporate these beliefs and values into plans for their future health care.
It provides people with the opportunity to develop and express their
preferences for care, informed not only by their personal beliefs and values
but also by an understanding of their current and anticipated future health
status and the treatment and care options available. ACP may result in the
person with dementia choosing to write an advance care plan (see above)
and/or advance directive and/or to appoint an enduring power of attorney
(see below). If the person has strong views or preferences about medical
treatments and procedures, they should be advised to consider completing
an advance directive.
Dementia capable encompasses an ability (a combination of staff
knowledge, skills, and competency as well as available programs and
services) to fulfill the needs of adults with dementia and their caregivers.
The concept applies to workforces, programs, services, and systems. Both
the “dementia-friendly” approach led by the European
Union countries and the dementia-capable approach carried
out in the United States apply to similar ends. The dementia-friendly
approach focuses on the living
experience/user experience, whereas the dementia-capable
approach focuses on special needs. With a focus on the
living experience/user experience, the dementia-friendly
normalization process requires modifications of the living
environment (involving people, resources, places, activities,
cultures, technologies, and so on) to ensure that adults with dementia and
their families can enjoy a normal life in their communities for as long as
possible. With a focus on special needs, the dementia-capable approach
adapts the U.S. language and philosophy of care for persons with
disabilities to serve adults with dementia and their families and ensure
their needs are met. Both approaches encourage inclusion and acceptance,
but the difference is that the dementia friendly approach attempts to
include adults with dementia and their families in the community directly,
whereas the dementia capable approach uses a two-step approach (where
Step 1 involves inclusion into the disability community and Step 2
promotes inclusion into the society at large through being a member of the
disability community).
Guidance for care planning
and the design of services
New Zealand Framework for Dementia Care.
http://www.health.govt.nz/system/files/documents/p
ublications/new-zealand-framework-for-dementiacare-nov13.pdf
Guidance for care planning
and design of services
Shih-Yin Line, M.M., & Marcus Lewis, F. (2015).
Dementia friendly, dementia capable, and dementia
positive: Concepts to prepare for the future. The
Gerontologist, 2015, 55(2), 237-244.
REPORT OF THE TOPIC GROUP ON NOMENCLATURE
Dementia capability
Dementia friendly
community
Dementia friendly
environment
A model system:
1. Educates the public about brain health. This would include information
about the risk factors associated with developing dementia, first signs of
cognitive problems, management of symptoms if individuals have
dementia, support programs, and opportunities to participate in research.
2. Identifies people with possible dementia and recommend that they see a
physician for a timely, accurate diagnosis and to rule out reversible causes
of dementia or conditions that resemble it.
3. Ensures that program eligibility and resource allocation take into
account the impact of cognitive disabilities.
4. Ensures that staff communicate effectively with people with dementia
and their caregivers and provide services that: a) are person- and familycentered, b) offer self-direction of services, and c) are culturally
appropriate.
5. Educates workers to identify possible dementia, and understand the
symptoms of dementia and appropriate services.
6. Implements quality assurance systems that measure how effectively
providers serve people with dementia and their caregivers.
7. Encourages development of dementia-friendly communities, which
include key parts of dementia-capability
Dementia-capable service systems recognize that
1) people with dementia use more and different services than people with
physical disabilities
2) rely on caregivers to remain in their communities
In dementia-friendly communities, residents, agencies, businesses, and
service providers are learning about dementia through education and
awareness efforts, and providing assistance to people with the condition
and their caregivers as they go about their daily lives. These efforts go
beyond developing dementia-capable health and long-term services and
supports. Dementia friendly communities involve improved customer
service at participating agencies and businesses, supportive faith or
spiritual communities, emergency services that understand dementia, and
suitable transportation and public spaces.
[1] To maintain as much independence as possible, a person with
Alzheimer's dementia needs to live in as familiar an environment as
possible, with people who are as familiar to them as possible. This is not a
time to introduce drastic changes into a person's life; it is a time to start
thinking about ways to enable them to keep their independence as long as
possible.
The environment should be as stress-free and calm as possible, and
designed with a person's sensory and other problems in mind.
* Daily routines should be maintained.
* Flooring should be one color throughout, as changes in color and texture
can be seen as steps or obstacles. Flooring should not be shiny, as this can
look like water. Patterned flooring can appear 'frightening' in dim light (to
Guidance for care planning
and the design of services
Tilly, J., Wiener, J.M., & Gould, E. (2014). Dementia
Capable States and Communities: The Basics.
http://www.aoa.acl.gov/AoA_Programs/HPW/Alz_Gra
nts/docs/BH-Brief-Dementia-Capable-Basics.pdf
Guidance for care planning
and the design of services
Tilly, J., Wiener, J.M., & Gould, E. (2014). Dementia
Capable States and Communities: The Basics.
http://www.aoa.acl.gov/AoA_Programs/HPW/Alz_Gra
nts/docs/BH-Brief-Dementia-Capable-Basics.pdf
Guidance for care planning
and the design of services
[1] Holland. A. (2007). Alzheimer's Dementia: What
You Need to Know, What You Need to Do. University
of Hertfordshire, Intellectual Disability and Health.
http://www.intellectualdisability.info/mentalhealth/articles/alzheimers-dementia-what-you-needto-know,-what-you-need-to-do
[2] Ministry of Health. 2013. New Zealand Framework
for Dementia Care. Wellington: Ministry of Health.
http://www.health.govt.nz/system/files/documents/p
ublications/new-zealand-framework-for-dementiacare-nov13.pdf
REPORT OF THE TOPIC GROUP ON NOMENCLATURE
someone with dementia, it can look as if there are moving insects/objects
on the floor at night-time).
* Pictures and signs can be used to help a person find their way around the
house, the toilet door could be painted a bright color to make it easy to
find.
* Mirrors can be removed or covered, as a person may not recognize their
own reflection, lighting should not glare.
* People with whom the person spends time, including other people with
intellectual disabilities, should be helped to understand the condition and
how they can be involved in the person's support.
Dementia ready residential
[2] Dementia-friendly environments are created using a flexible approach
that maximizes the person with dementia’s individual requirements for
freedom and involvement and minimizes regimentation
Living situations that may include living with family, living on one’s own or
with a partner/friend, living in a small supervised setting, living in a large
supervised setting or living in a dementia specific setting.
Modifications/changes may be needed to support continuation of the living
situation (see Table 6 from Jokinen et al., 2013))
Guidance for care planning
and the design of services
Jokinen, N., Janicki, M.P., Keller, S.M., McCallion, P.,
Force, L.T., and the National Task Group on
Intellectual Disabilities and Dementia Practices (2013).
Guidelines for Structuring Community Care and
Supports for People with Intellectual Disabilities
Affected by Dementia. Albany NY: NTGIDDP & Center
for Excellence in Aging & Community Wellness.
https://aadmd.org/sites/default/files/NTGcommunitycareguidelines-Final.pdf
REPORT OF THE TOPIC GROUP ON NOMENCLATURE
Dementia-ready – day
programs
[1] Dementia-ready day programs evolve when dementia becomes more
prominent and there is a need to reorganize to better support small group
activities and flexibility in hours of involvement for the individual with
Guidance for care planning
and the design of services
[1] Nickle, T., & McCallion, P. (2005). Redesigning Day
Programmes. The Frontline of Learning Disability, 64
(Winter), 27-28.
REPORT OF THE TOPIC GROUP ON NOMENCLATURE
dementia (e.g. partial versus full days) (Table 8). Such day programs work
more closely with aging and Alzheimer/dementia specific service providers
and begin to focus on maintaining the individual’s previously learned skills
and enjoyable activities rather than the addition of new skills and
experiences. Staffing becomes dementia-capable and training is offered
with attention to address wandering management, health related
concerns, and incontinence to reduce the likelihood that people dementia
will be discharged and no longer able to attend the program. Greater
interaction with social and medical day care programs and with dementia
specific programs and senior centers in the community likely increases the
value of day programs in an overall intellectual disabilities and dementia
strategy and create opportunities for cross-programming and shared
resources.
[2] Jokinen, N., Janicki, M.P., Keller, S.M., McCallion,
P., Force, L.T., and the National Task Group on
Intellectual Disabilities and Dementia Practices (2013).
Guidelines for Structuring Community Care and
Supports for People with Intellectual Disabilities
Affected by Dementia. Albany NY: NTGIDDP & Center
for Excellence in Aging & Community Wellness.
https://aadmd.org/sites/default/files/NTGcommunitycareguidelines-Final.pdf
Day program descriptors
Dementia trained
staff
[1] Staff who are trained to understand how to communicate with people
with dementia and respond to BPSD, the complexity of the condition which
combines features of chronic neurological disease, mental illness and
physical frailty, and function within integrated care. Staff can spot the early
symptoms of dementia, understand how to interact with people with
dementia and signpost to the most appropriate care and support.
[2] Staff who are trained in the following areas: Alzheimer’s & dementia
disease awareness; strategies for caring for the person with dementia;
communication and understanding behavior; social needs and activities;
[1] Alzheimer’s Society. (2016). Formal care of people
with dementia.
https://www.alzheimers.org.uk/site/scripts/document
s_info.php?documentID=402
[2} Alzheimer’s Association. (2016). Dementia care
training and certification.
http://www.alz.org/professionals_and_researchers_1
1176.asp
REPORT OF THE TOPIC GROUP ON NOMENCLATURE
eating well; reducing pain; falls; wandering. restraint-free care, and end-oflife care.
Note: AD: Alzheimer’s disease; ID: intellectual disability
REPORT OF THE TOPIC GROUP ON NOMENCLATURE
Appendix C: Examples of terms used in reference to dementia and ID within a convenience sample of articles and documents
Citation
Kirk, Hick, & Laraway (2006)
Assessing dementia in people with
learning disabilities: the relationship
between two screening measures.
[J Intellect Disabil. 2006 Dec;10(4):35764.]
Silverman, Schupf, Zigman, Devenny,
Miezejeski, Schubert, & Ryan (2004)
Dementia in Adults with Mental
Retardation: Assessment at a Single
Point in Time
[Am J Ment Retard, 2004, 109(2), 111125]
Paper focus
Assessment
Subject pool
“Learning disability”
Terminology used/cited
Dementia, early stages of dementia
Defined/undefined?
No specific definitions provided
Assessment
Down syndrome,
intellectual disability
Dementia, severe dementia,
Alzheimer’s disease, dementia
associated with Alzheimer’s disease
Adams, Oliver, Kalsy, Peters, Broquard,
Basra, Konstandinidi, & McQuillan
(2008)
Behavioural characteristics associated
with dementia assessment referrals in
adults with Down syndrome
[JIDR, 2008, 52(4), 358-368]
Assessment
Down syndrome
Alzheimer’s disease, dementia, early
stages of dementia, dementia of the
Alzheimer’s type
O’Caoimh, Clune & Molloy (2013)
Screening for Alzheimer's Disease in
Downs Syndrome.
[Journal of Alzheimer’s Disease &
Parkinsonism, S7:001. doi:
10.4172/2161-0460.S7-001]
Assessment Screening
Down syndrome,
“learning disability”
Alzheimer’s disease, dementia
Dementia associated with
Alzheimer’s disease (DAT) is
defined in the Diagnostic and
Statistical Manual of the
American Psychiatric Association
DSM-IV (1994) as
the development of multiple
cognitive deficits that include
memory impairment and at least
one of the following: aphasia,
apraxia, agnosia, or a disturbance
in executive functioning. The
cognitive deficits must be
sufficiently severe to cause
impairment in occupational or
social functioning. (1994, p. 134)
“… diagnosis of ‘dementia of the
Alzheimer’s type’ can be made on
the basis of a thorough diagnostic
process which includes medical
examinations,
neuropsychological tests and
interviews.”
No specific definitions provided
“A selective pattern of cognitive
changes is usually seen,
characterized
by impairment in long-term
memory and visuospatial
construction with relative
REPORT OF THE TOPIC GROUP ON NOMENCLATURE
Temple & Konstantareas (2005)
A comparison of the behavioural and
emotional characteristics of Alzheimer's
dementia in individuals with and
without Down syndrome.
[Can J Aging. 2005 Summer; 24(2):17989]
Millichap, Oliver, McQuillan, Kalsy,
Lloyd & Hall (2003)
Descriptive functional analysis of
behavioral excesses shown by adults
with Down syndrome and dementia.
[Int J Geriatr Psychiatry. 2003
Sep;18(9):844-54]
Yachnis, Roth, & Heilman (2010).
Fragile X dementia Parkinsonism
Syndrome (FXDPS). [Cogn Behav
Neurol. 2010 Mar;23(1):39-43]
Margallo-Lana, Moore, Kay, Perry, Ried,
Berney, & Tyrer (2007)
Fifteen-year follow-up of 92
hospitalized adults with Down's
syndrome: incidence of cognitive
decline, its relationship to age and
neuropathology.
[JIDR, 2007, 51(6), 463-477]
Behavior - Behavioral
characteristics
Down syndrome,
intellectual disability
Alzheimer’s Disease, Alzheimer’s
dementia
Behavior - Behavioral
excesses
Down syndrome
Alzheimer’s disease, dementia, late
stage dementia
Behavior - Clinical
report
Fragile X
Dementia
Behavior - Cognitive
decline
Down syndrome
Clinical dementia; Dementia of
Alzheimer’s type; Alzheimer-type
dementia
Wilkinson, H., Kerr, D., & Cunningham,
C. (2005). Equipping staff to support
people with an intellectual disability
and dementia in care home settings.
Dementia, August, 2005, 4(3), 387-400.
Care practices
Intellectual disability
Alzheimer’s disease, dementia
preservation of immediate
memory (recall) and language.
When dementia develops in
adults with DS, it is characterized
by global neuropsychological
deficits, albeit with retention of
some
basic language skills, and often
presents atypically.”
“diagnosis of Alzheimer’s
dementia given by a physician”
“… a clinical diagnosis of
AD outlined by Holland (1999),
which included the
presence of gradual decline and
cognitive impairment
in the absence of significant
health or sensory problems.”
“… rapidly progressive dementia,
characterized by getting lost,
poor judgment, and hygiene.
“…diagnosis of dementia requires
a decline from a previous level of
cognitive functioning sufficient to
impair personal activities of daily
living that is not due to other
non-organic causes such as
physical illness or depression.”
“Alzheimer-type dementia, as
described in ICD-10”
Terms not defined
REPORT OF THE TOPIC GROUP ON NOMENCLATURE
Forbat, L., & Service, K.P. (2005). Who
cares? Contextual layers in end-of-life
care for people with intellectual
disability and dementia. Dementia,
August, 2005, 4(3), 413-431
Rosewarne, M. (2001)
Learning disabilities and dementia: a
pilot therapy group.
[Journal of Dementia Care, 2001,
July/August, 9(4), 18-20]
Courtenay, K., Jokinen, N.S., & Strydom,
A. (2010). Caregiving and adults with
intellectual disabilities affected by
dementia. Journal of Policy and Practice
in Intellectual Disabilities, 2010, 7(1), 26
- 33.
Care practices
Intellectual disability
Dementia
Term not defined
Care practices
Down syndrome,
“learning disabilities”
Dementia, Alzheimer’s disease
Alzheimer’s disease undefined;
dementia undefined, but author
cites Prasher & Krishman’s (1993)
list of behavioral changes.
Care practices
Intellectual disability
Dementia, dementia-related caregiving,
early-state dementia, dementia care
mapping
Janicki, M.P., Dalton, A.J., McCallion, P.,
Baxley, D.D., & Zendell, A. (2005).
Group home care for adults with
intellectual disabilities and Alzheimer’s
disease. Dementia, August, 2005, 4(3),
361-385.
Carling-Jenkins, R., Torr, J., Iacono, T., &
Bigby, C. (2012).
Experiences of supporting people with
Down syndrome and Alzheimer’ s
disease in aged care and family
environments. Journal of Intellectual &
Developmental Disability, 37(1), 54-60.
Care practices
Intellectual disability
Dementia, dementia care, dementia
care services, dementia-capable
services
“… dementia care mapping is
used to observe and evaluate the
quality of life and care of people
living with dementia. It consists
of three phases where trained
“mappers” carry out detailed
observations of behavior and
care provision and assign “wellbeing” or “ill-being”
ratings.”
“…dementia is a progressive
condition associated with
slowly increasing care needs and
carer burden…”
Other terms not defined
No terms defined
Care practices
Down syndrome
Alzheimer’s disease, early onset
Alzheimer’s disease,
Donaldson (2002).
Work stress and people with Down
syndrome and dementia. [Down
Care practices - Work
stress
Down syndrome,
“learning disabilities”
Dementia
“… early onset Alzheimer’s
disease, “a progressive
degenerative condition of the
brain that results in a gradual
change, over years, in a person’s
ability to think and remember
and to perform tasks of everyday
living ”
Term not defined
REPORT OF THE TOPIC GROUP ON NOMENCLATURE
Syndrome Research and Practice, 2002,
8(2), 74-78]
Jaycock, Persaud & Johnson (2006).
The effectiveness of dementia care
mapping in intellectual disability
residential services: A follow-up study
[Journal of Intellectual Disabilities,
2006, 10(4), 365-375]
Care practices –
Dementia care
mapping
Intellectual disability
Dementia care mapping, dementia,
dementia services
Nagdee (2011)
Dementia in intellectual disability: A
review of diagnostic challenges.
Afr J Psychiatry (Johannesbg). 2011
Jul;14(3):194-9. doi:
http://dx.doi.org/10.4314/ajpsy.v14i3.1
.
De Vreese, L.P., Uberti, M., Mantesso,
U., De Bastiani, E., Weger, E.,
Marangoni, A.C., Weiner, M.F. &
Gomiero, T. (2012). Measuring quality
of life in intellectually disabled persons
with dementia with the Italian version
of the quality of life in late-stage
dementia (QUALID) scale. Journal of
Alzheimer's Disease and Parkinsonism,
2012, 2:104e. doi:10.4172/21610460.1000104
Diagnostics
Intellectual disability
Dementia
Diagnostics
Intellectual disability
Advanced dementia, dementia,
dementia due to Alzheimer’s disease
Van Buggenhout, Trommelen,
Shoenmaker, De Bal, Verbeek, Smeets,
Ropers, Devriendt, Hamel & Fryns
(1999)
Down syndrome in a population of
elderly mentally retarded patients:
genetic-diagnostic survey and
implications for medical care. [Am J
Med Genet. 1999 Aug 6;85(4):376-84]
Wiseman, F.K., Al-Janabi, T., Hardy, J.,
John Hardy, Karmiloff-Smith, A., Nizetic,
D., Tybulewicz, V.L.J. Fisher, E.M.C. &
Diagnostics
Down syndrome
Dementia
Genetics
Down syndrome
Alzheimer disease, behavioral and
psychological symptoms of dementia
“…dementia care mapping (DCM,
7th edition: Bradford Dementia
Group, 1997.” “DCM (Kitwood
and Bredin, 1992) is a
developmental evaluation tool
measuring the quality of care
from the perspective of the
recipient.”
Term not defined explicitly;
references to IASSID-AAMR
criteria and processes and by
behavioral description
“The progressive deterioration of
cognitive functions and verbal
communication, the inability to
perform previously rewarding
activities along with changes in
perception, content of thought,
mood and behavior, render
troublesome the use of reliable
and comparable criteria for
evaluating QoL relative to the
person and his social context
during the course of dementia.”
Others not defined
Dementia not defined
No explicit definitions, but some
allusions to Alzheimer’s
neuropathology and behavioral
REPORT OF THE TOPIC GROUP ON NOMENCLATURE
Strydom, A. ( 2015). A genetic cause of
Alzheimer disease: mechanistic insights
from Down syndrome. Nature Reviews:
Neuroscience, 16 (Sept), 564-574.
(BPSD), dementia, early-onset
Alzheimer disease (EOAD),
Carr, J., & Collins, S. (2014). Ageing and
dementia in a longitudinal study of
a cohort with Down Syndrome. Journal
of Applied Research in Intellectual
Disabilities 2014, 27, 555–563
Prasher & Krishman (1993)
Age of onset and duration of dementia
in people with Down’s syndrome.
[International Journal of Geriatric
Psychiatry, 1993, 8(11), 915-922]
Epidemiology
Down syndrome
Dementia
Epidemiology
Down syndrome
Alzheimer disease, dementia
Coppus, A., Evenhuis, H., Verberne, GJ., Visser, F., Van Gool, P., Eikelenboom,
P., & Van Duijin, C. (2006).
Dementia and mortality in persons with
Down’s syndrome. Journal of
Intellectual Disability Research, 2006,
50(10), 768-777.
Epidemiology
Down syndrome
Alzheimer’s disease, dementia
descriptors of dementia in Down
syndrome (e.g., “…development
of amyloid plaques and
neurofibrillary tangles (NFTs),
which are typical of AD brain
neuropathology…”; “… decline in
memory and language skills that
may be present…”. BPSD is
described as ‘non-cognitive’
changes *** are associated with
deficits in executive functioning
and with frontal antrophy…”.
EOAD is defined as “Occurrence
of Alzheimer disease before the
age of 65 years.”
Dementia not defined
Dementia not defined; however,
dx statement was provided: “The
diagnosis of clinical dementia was
based on the authors of each
report but included changes in
personality, loss of intellectual
and social skills, presence of
neurological signs (e.g., seizures),
and resulting death not due to
some other medical disorder.”
Terms not defined.
Dx criteria alluded to: “The
diagnosis is based on the ICD-10
Symptom Checklist for Mental
Disorders, in particular, dementia
in Alzheimer’s disease (World
Health Organization 1992) and
the guidelines produced by an
international consensus panel
established under the auspices of
the Ageing Special Interest Group
of the International Association
REPORT OF THE TOPIC GROUP ON NOMENCLATURE
Zigman WB, Schupf N, Devenny DA,
Miezejeski C, Ryan R, Urv TK, Schubert
R, Silverman W. (2004). Incidence and
prevalence of dementia in elderly
adults with mental retardation without
down syndrome.
[Am J Ment Retard. 2004
Mar;109(2):126-41]
Strydom, Livingston, King & Hassiotis
(2007)
Prevalence of dementia in intellectual
disability using different diagnostic
criteria
[British Journal of Psychiatry Jul 2007,
191 (2) 150-157]
Epidemiology
Intellectual disability
Alzheimer’s disease; Dementia of the
Alzheimer’s type;
Epidemiology
Intellectual disability
Dementia
for the Scientific Study of
Intellectual Disabilities (IASSID)
(Aylward et al., 1997; Burt &
Aylward, 2000).
“Dementia of the Alzheimer’s
type (DAT) is characterized by a
mid to late-life onset of a
progressive deterioration of
cognitive and functional abilities,
with considerable variability in
behavioral manifestation.”
“… criteria for dementia: ICD–10
Research Diagnostic Criteria
(World Health Organization,
1993), DSM–IV–TR (American
Psychiatric Association, 2000)
and DC–LD criteria (Royal College
of Psychiatrists, 2001; ICD–10
(World Health Organization,
1993), DSM–IV (American
Psychiatric Association, 2000)
and the National Institute of
Neurological and Communicative
Disorders and Stroke–Alzheimer’s
Disease and Related Disorders
Association (NINCDS–ADRDA;
McKhann et al, 1984) criteria for
Alzheimer’s disease; ICD–10,
DSM–IV and National Institute of
Neurological Disorders and
Stroke–Association Internationale
pour la Recherche et
l’Enseignement en Neurosciences
NINDS–AIREN (Roman et al,
1993) criteria for vascular
dementia; the Consortium on
Dementia with Lewy Bodies (DLB)
criteria (McKeith et al, 1996); and
the Work Group on
Frontotemporal Dementia and
Pick’s Disease criteria for
REPORT OF THE TOPIC GROUP ON NOMENCLATURE
frontotemporal dementia (FTD;
McKhann et al, 2001).
Terms not defined
Jozsvai, E. (2006). Behavioural and
psychological symptoms of dementia in
individuals with Down syndrome.
[Journal of Developmental Disabilities,
2006, 12(1) 31-40]
Janicki & Dalton (2000)
Prevalence of dementia and impact on
intellectual disability services,
[Mental Retardation, 2000, 38(3), 276–
288]
Epidemiology
Down syndrome
Dementia of the Alzheimer’s type,
dementia
Epidemiology
Intellectual disability
Dementia, Alzheimer-type dementia
Janicki & Keller (2015)
Why do we need national guidelines for
adults with intellectual disability and
dementia?
[Alzheimers Dement (Amst). 2015 Sep;
1(3): 325–327]
British Psychological Society (2009)
Dementia and People with Learning
Disabilities: Guidance on the
assessment, diagnosis, treatment and
support of people with learning
disabilities who develop dementia.
Leicester, UK: British Psychological
Society.
Watchman, Kerr & Wilkinson (2010)
Supporting Derek: A practice
development guide to support staff
working with people who have a
learning difficulty and dementia. York:
UK: Roundtree Foundation.
Guidance document
Intellectual disability
Alzheimer’s disease, dementia
Guidance document
‘Learning disability”
Dementia
Dementia defined via ICD-10
definition
Guidance document
“learning difficulty”
Dementia
Shooshtari, Martens, Burchill, Dik, &
Naghipur (2011)
Health (Depression)
Developmental
disabilities
Dementia
Dementia is an umbrella term
that describes a number of
conditions on the brain; there is
not just one condition called
dementia. Dementia is a
progressive and terminal
condition. Early indicators may be
changes om memory, behaviour
or personality, difficulty learning
new things and struggling to find
the right word to use.
“Dementia is a mental disorder
characterized by a loss of
intellectual abilities of sufficient
Dementia was defined as “the
deterioration of mental abilities,
memory, judgement, work and
activities of daily living skills and
orientation. It involves marked
changes in a person’s behavior
and has a variety of causes.”
Alzheimer’s disease and
dementia not defined
REPORT OF THE TOPIC GROUP ON NOMENCLATURE
[Int J Family Med. 2011;2011:319574.
doi: 10.1155/2011/319574. Epub 2011
Aug 8.]
Prevalence of Depression and Dementia
among Adults with Developmental
Disabilities in Manitoba, Canada.
Henderson, Lynch, Wilkinson & Hunter
(2007)
Adults with Down's syndrome: the
prevalence of complications and health
care in the community.
[Br J Gen Pract. 2007, Jan; 57(534), 505.]
McCarron, Gill, McCallion & Begley
(2005)
Health co-morbidities in ageing persons
with Down syndrome and Alzheimer's
dementia.
[J Intellect Disabil Res. 2005 Jul;49(Pt
7):560-6.]
Strydom, Hassiotis, & Livingston (2005)
Mental Health and Social Care Needs of
Older People with Intellectual
Disabilities.
[JARID, 2005, 18(3), 229–235]
Prasher, V.P., Metseagharun, T., &
Hague, S. (2004)
Weight loss in adults with Down
syndrome and with dementia in
Alzheimer's disease.
[Research in Developmental
Disabilities, v25 n1 p1-7 Jan-Feb 2004]
McQuillan, Kalsy, Oyebode, Millichap,
Oliver, & Hall (2003)
Adults with Down's Syndrome and
Alzheimer's Disease.
[Tizard Learning Disability Review,
8(4),.4–13]
Simard & van Reekum (2001)
Dementia with Lewy bodies in Down's
syndrome.
severity to interfere with social or
occupational performance.”
Health (Health care)
Down syndrome
Alzheimer’s disease
Alzheimer’s disease – Not defined
Health (Health comorbidities)
Down syndrome
Alzheimer’s dementia
Alzheimer’s dementia not
defined
Health (Mental health)
Intellectual disability
Dementia
Dementia not defined
Health (Weight loss)
Down syndrome
Alzheimer’s disease, dementia,
dementia in Alzheimer’s disease
“… diagnosed as having
“Dementia in Alzheimer’s
disease” according to DCR-10
criteria (WHO, 1993)…”
Review (DS and
Alzheimer’s disease)
Down syndrome
Alzheimer’s disease, dementia
Dementia defined
Alzheimer’s defined
Review (DS and DLB)
Down syndrome
Alzheimer’s disease, dementia,
dementia with Lewy bodies (DLB)
No specific definitions provided
REPORT OF THE TOPIC GROUP ON NOMENCLATURE
[Int J Geriatr Psychiatry. 2001
Mar;16(3):311-20]
Puri, Ho, & Singh (2001)
Age of seizure onset in adults with
Down's syndrome.
[Int J Clin Pract. 2001 Sep;55(7):442444]
Menendez (2005)
Down syndrome, Alzheimer's disease
and seizures.
[Brain Dev. 2005 Jun;27(4):246-52.]
Seizures (Age of onset)
Down syndrome
Alzheimer’s disease
Cited DSM-III-R dx criteria
Seizures
Down syndrome
Alzheimer-type abnormalities,
dementia, Alzheimer’s disease,
Advanced Alzheimer’s disease
No terms defined
d’Orsi & Specchio (2014)
Seizures (myoclonus
Down syndrome
Alzheimer’s disease (undefined);
“Diagnosis of dementia was
[Progressive myoclonus epilepsy in
epilepsy)
dementia;
generated using the modified
Down syndrome patients with
ICD-10 criteria for adults with
dementia. [J Neurol, 2014, 261(8),
intellectual disabilities based on
1584-1597]
DSM-IV-TR.”
Lott, Doran, Walsh & Hill (2006)
Telemedicine
Down syndrome
Alzheimer’s disease, dementia of the
No terms defined
Telemedicine, dementia and Down
Alzheimer’s type (DAT)
syndrome: implications for Alzheimer
disease.
[Alzheimers Dement. 2006 Jul;2(3):17984]
Note: This table represents a completely unscientific and unsystematic sampling of articles and documents dealing with ‘dementia’ and ID. Conceivable they can serve as a
reasonable sample of papers found in the field and reflect the terms used.
References (for Appendix C)
Adams, D., Oliver, C., Kalsy, S., Peters, S., Broquard, M., Basra, T., Konstandinidi, E., & McQuillan, S. (2008). Behavioural characteristics associated with dementia assessment
referrals in adults with Down syndrome. Journal of Intellectual Disability Research, 52(4), 358-368.
British Psychological Society (2009). Dementia and people with learning disabilities: Guidance on the assessment, diagnosis, treatment and support of people with learning
disabilities who develop dementia. Leicester, UK: British Psychological Society.
Carling-Jenkins, R., Torr, J., Iacono, T., & Bigby, C. (2012). Experiences of supporting people with Down syndrome and Alzheimer’ s disease in aged care and family environments.
Journal of Intellectual & Developmental Disability, 37(1), 54-60.
Carr, J., & Collins, S. (2014). Ageing and dementia in a longitudinal study of a cohort with Down Syndrome. Journal of Applied Research in Intellectual Disabilities 2014, 27, 555–
563.
Coppus, A., Evenhuis, H., Verberne, G-J., Visser, F., Van Gool, P., Eikelenboom, P., & Van Duijin, C. (2006). Dementia and mortality in persons with Down’s syndrome. Journal of
Intellectual Disability Research, 50(10), 768-777.
REPORT OF THE TOPIC GROUP ON NOMENCLATURE
Courtenay, K., Jokinen, N.S., & Strydom, A. (2010). Caregiving and adults with intellectual disabilities affected by dementia. Journal of Policy and Practice in Intellectual
Disabilities, 2010, 7(1), 26 - 33.
De Vreese, L.P., Uberti, M., Mantesso, U., De Bastiani, E., Weger, E., Marangoni, A.C., Weiner, M.F. & Gomiero, T. (2012). Measuring quality of life in intellectually disabled
persons with dementia with the Italian version of the quality of life in late-stage dementia (QUALID) scale. Journal of Alzheimer's Disease and Parkinsonism, 2:104e.
doi:10.4172/2161-0460.1000104
d’Orsi, G. & Specchio, L.M. (2014). Progressive myoclonus epilepsy in Down syndrome patients with dementia. Journal of Neurology, 261(8), 1584-1597.
Donaldson, S. (2002). Work stress and people with Down syndrome and dementia. Down Syndrome Research and Practice, 8(2), 74-78.
Forbat, L., & Service, K.P. (2005). Who cares? Contextual layers in end-of-life care for people with intellectual disability and dementia. Dementia, 4(3), 413-431.
Henderson, A., Lynch, S.A., Wilkinson, S., & Hunter, M. (2007). Adults with Down's syndrome: the prevalence of complications and health care in the community. British Journal
of General Practice, 57(534), 50-55.
Janicki, M.P. & Dalton, A.J. (2000). Prevalence of dementia and impact on intellectual disability services. Mental Retardation, 38(3), 276–288.
Janicki, M.P. & Keller, S.M. (2015). Why do we need national guidelines for adults with intellectual disability and dementia? Alzheimer's & Dementia (Amsterdam, Netherlands),
1(3), 325–327.
Janicki, M.P., Dalton, A.J., McCallion, P., Baxley, D.D., & Zendell, A. (2005). Group home care for adults with intellectual disabilities and Alzheimer’s disease. Dementia, 4(3), 361385.
Jaycock, S., Persaud, M., & Johnson, R. (2006). The effectiveness of dementia care mapping in intellectual disability residential services: A follow-up study. Journal of Intellectual
Disabilities, 2006, 10(4), 365-375.
Jozsvai, E. (2006). Behavioural and psychological symptoms of dementia in individuals with Down syndrome. Journal of Developmental Disabilities, 2006, 12(1) 31-40.
Kirk, L.J., Hick, R., & Laraway, A. (2006). Assessing dementia in people with learning disabilities: the relationship between two screening measures. Journal of Intellectual
Disabilities, 10(4):357-364.
Lott, I.T., Doran, E., Walsh, D.M., & Hill, M.A. (2006). Telemedicine, dementia and Down syndrome: implications for Alzheimer disease. Alzheimer’s & Dementia, 2(3), 179-184.
Margallo-Lana, M.L., Moore, P.B., Kay, D.W., 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
McCarron, M., Gill, M., McCallion, P., & Begley, C. (2005). Health co-morbidities in ageing persons with Down syndrome and Alzheimer's dementia. Journal of Intellectual
Disability Research, 49(7), 560-566.
McQuillan, S., Kalsy, S., Oyebode, J., Millichap, D., Oliver, C., & Hall, S. (2003). Adults with Down's syndrome and Alzheimer's disease. Tizard Learning Disability Review, 8(4), 4–13
Menendez, M. (2005). Down syndrome, Alzheimer's disease and seizures. Brain Development, 27(4), 246-252.
REPORT OF THE TOPIC GROUP ON NOMENCLATURE
Millichap, D., Oliver, C., McQuillan, S., Kalsy, S., Lloyd, V., & Hall, S. (2003). Descriptive functional analysis of behavioral excesses shown by adults with Down syndrome and
dementia. International Journal of Geriatric Psychiatry, 18(9), 844-854.
Nagdee, M. (2011). Dementia in intellectual disability: A review of diagnostic challenges. African Journal of Psychiatry (Johannesberg), 14(3), 194-199. doi:
http://dx.doi.org/10.4314/ajpsy.v14i3.1.
O’Caoimh, R., Clune, Y., & Molloy, D.W. (2013). Screening for Alzheimer's disease in Downs syndrome. Journal of Alzheimer’s Disease & Parkinsonism, S7:001. doi:
10.4172/2161-0460.S7-001.
Prasher, V.P. & Krishnan, H.R. (1993). Age of onset and duration of dementia in people with Down’s syndrome. International Journal of Geriatric Psychiatry, 8(11), 915-922.
Prasher, V.P., Metseagharun, T., & Hague, S. (2004). Weight loss in adults with Down syndrome and with dementia in Alzheimer's disease. Research in Developmental
Disabilities, 25(1), 1-7.
Puri, B.K., Ho, K.W., & Singh, I. (2001). Age of seizure onset in adults with Down's syndrome. International Journal of Clinical Practice, 55(7), 442-444.
Rosewarne, M. (2001). Learning disabilities and dementia: a pilot therapy group. Journal of Dementia Care, 9(4), 18-20.
Shooshtari, S., Martens, P.J., Burchill, C.A., Dik, N., & Naghipur, S. (2011). Prevalence of depression and dementia among adults with developmental disabilities in Manitoba,
Canada. International Journal of Family Medicine, 2011(319574). doi: 10.1155/2011/319574.
Silverman, W., Schupf, N., Zigman, W., Devenny, D., Miezejeski, C., Schubert, R., & Ryan, R. (2004). Dementia in adults with mental retardation: assessment at a single point in
time. American Journal of Mental Retardation, 109(2), 111-125.
Simard, M. & van Reekum, R. (2001). Dementia with Lewy bodies in Down's syndrome. International Journal of Geriatric Psychiatry, 16(3), 311-320.
Strydom, A., Hassiotis, A., & Livingston, G. (2005). Mental health and social care needs of older people with intellectual disabilities. Journal of Applied Research in Intellectual
Disabilities, 18(3), 229–235.
Strydom, A., Livingston, G., King, M., & Hassiotis, A. (2007). Prevalence of dementia in intellectual disability using different diagnostic criteria. British Journal of Psychiatry,
191(2), 150-157.
Temple V. & Konstantareas, M.M. (2005). A comparison of the behavioural and emotional characteristics of Alzheimer's dementia in individuals with and without Down
syndrome. Canadian Journal on Aging, 24(2), 179-189.
Van Buggenhout, G.J., Trommelen, J.C., Shoenmaker, A., De Bal, C., Verbeek, J.J., Smeets, D.F., Ropers, H.H., Devriendt, K., Hamel, B.C., & Fryns, J.P. (1999). Down syndrome in a
population of elderly mentally retarded patients: genetic-diagnostic survey and implications for medical care. American Journal of Medical Genetics, 85(4), 376-384.
Watchman, K., Kerr,D., & Wilkinson, H. (2010). Supporting Derek: A practice development guide to support staff working with people who have a learning difficulty and
dementia. York: UK: Roundtree Foundation.
Wilkinson, H., Kerr, D., & Cunningham, C. (2005). Equipping staff to support people with an intellectual disability and dementia in care home settings. Dementia, August, 2005,
4(3), 387-400.
REPORT OF THE TOPIC GROUP ON NOMENCLATURE
Wiseman, F.K., Al-Janabi, T., Hardy, J., John Hardy, 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 (Sept), 564-574.
Yachnis, A.T., Roth, H.L., & Heilman, K.M. (2010). Fragile X dementia Parkinsonism Syndrome (FXDPS). Cognitive and Behavioral Neurology, 23(1), 39-43.
Zigman, W.B., Schupf, N., Devenny, D.A., Miezejeski, C., Ryan, R., Urv, T.K., Schubert, R., & Silverman, W. (2004). Incidence and prevalence of dementia in elderly adults with
mental retardation without down syndrome. American Journal on Mental Retardation, 109(2), 126-141.
REPORT OF THE TOPIC GROUP ON NOMENCLATURE
Appendix D: Statements related to dementia/Alzheimer’s disease by select Down syndrome organizations
Source
Down
Syndrome
International
(INT)
Down’s
Syndrome
Association
(UK)
National Down
Syndrome
Society (USA)
website
https://ds-int.org/resource/adults-18ageing
Statement
“Dementia is a frequently voiced concern about people with Down syndrome.
There is a tendency for people with Down syndrome to develop dementia at a
younger age than in the general population. However, research indicates that the
overall percentage of adults with Down syndrome who develop dementia is
similar to that of the population who do not have Down syndrome. It is not
inevitable that those with Down syndrome will develop dementia.”
http://www.downs“Alzheimer’s disease is a type of dementia that gradually destroys brain cells,
syndrome.org.uk/for-families-andaffecting a person’s memory and their ability to learn, make judgments,
carers/health-and-well-being/getting- communicate and carry out basic daily activities. Alzheimer’s disease is
older/alzheimers-disease/
characterized by a gradual decline that generally progresses through three stages:
early, middle and late stage disease.” “Dementia is not a disease in its own right.
Dementia is an umbrella term covering more than 100 different conditions
associated with a cognitive decline. One of these conditions is Alzheimer’s
disease.”
http://www.ndss.org/Resources/Aging “Alzheimer’s disease is a type of dementia that gradually destroys brain cells,
-Matters/Alzheimers-Disease/Anaffecting a person’s memory and their ability to learn, make judgments,
Introduction-to-Alzheimers-Disease/
communicate and carry out basic daily activities. Alzheimer’s disease is
characterized by a gradual decline that generally progresses through three stages:
early, middle and late stage disease. These three stages are distinguished by their
general features, which tend to progress gradually throughout the course of the
disease. Alzheimer’s disease is not inevitable in people with Down syndrome.
While all people with Down syndrome are at risk, many adults with Down
syndrome will not manifest the changes of Alzheimer’s disease in their lifetime.
Although risk increases with each decade of life, at no point does it come close to
reaching 100%. This is why it is especially important to be careful and thoughtful
about assigning this diagnosis before looking at all other possible causes for why
changes are taking place with aging. Estimates show that Alzheimer’s disease
affects about 30% of people with Down syndrome in their 50s. By their 60s, this
number comes closer to 50%.”
REPORT OF THE TOPIC GROUP ON NOMENCLATURE
Down
Syndrome
Australia (AUS)
http://www.downsyndrome.org.au/d
ocuments/Ageing_Resource_Health_C
heck.pdf
“Alzheimer’s disease is a progressive degenerative condition of the brain that
results in a gradual change, over years, in a person’s ability to think, remember
and perform tasks of daily living. In ways that scientists don’t yet understand, the
extra copies of genes present in people with Down syndrome cause
developmental problems and health issues. Scientists think that the increased risk
of dementia in individuals with Down syndrome may also result from the extra
gene present on chromosome 21. Some studies suggest that 30 per cent of
people with Down syndrome aged in their 50’s have Alzheimer’s disease and
more than 75 per cent aged 65 and older have Alzheimer’s disease.”