A Sense of Self
Hope in Lewy Body Disease
Sorel Coward
University of South Australia
A report prepared for the Honourable Kelly Vincent (MLC)
South Australian Parliamentary Internship
December, 2017.
PREFACE
T
his report was written for The Honourable Kelly Vincent, who, is a
member of the Legislative Council (MLC) in the South Australian
Parliament and leader of Dignity Party.
The author is a post-graduate student and was commissioned to prepare
this report as part of a Master’s of Social Work through the University of
South Australia.
THE HON. KELLY VINCENT, MLC
A SENSE OF SELF
i
ACKNOWLEDGEMENTS
I am grateful for the support and encouragement given to me by my
sponsoring MP, The Honourable Kelly Vincent MLC, and her staff in Dignity
Party.
I am grateful for the inspiration and leadership offered to me by the South
Australian Parliamentary Internship scholars, namely Professor Haydon
Manning (Flinders University), Dr. Wayne Errington (University of Adelaide),
and Dr. Cenz Lancione (University of South Australia).
I am grateful for the support of my fellow students, especially on the
occasion when I made the oral presentation of this report in Old Parliament
House.
Finally, I am grateful for my wife, Jane, who has been by my side for twentyfive years of marriage, and has supported me through three academic
degrees. All this achievement has been possible because of you.
I thank you all.
Sorel Coward
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ii
TABLE OF CONTENTS
PREFACE....................................................................................................... I
ACKNOWLEDGEMENTS .............................................................................. II
ABBREVIATIONS ......................................................................................... V
EXECUTIVE SUMMARY .............................................................................. VI
CASE STUDY: MALCOLM’S STORY ............................................................ 1
CHAPTER 1: AGEING, DISABILITY & DEMENTIA IN AUSTRALIA ................ 4
AGEING ...................................................................................................... 4
DISABILITY .................................................................................................... 5
DEMENTIA ................................................................................................... 6
DEMENTIA IN ABORIGINAL AUSTRALIANS ........................................................ 7
CHAPTER 2: LEWY BODY DISEASE ............................................................. 9
TERMINOLOGY ............................................................................................. 9
CORE FEATURES ......................................................................................... 10
KEY ISSUE #1 : DIAGNOSIS .......................................................................... 10
The problem of misdiagnosis ............................................................ 11
The problem caused by low awareness of DLB ............................. 13
New Clinical Diagnostic Criteria ...................................................... 14
REM Sleep Behaviour Disorder ......................................................... 15
Improving DLB Diagnosis: ALBA Screening Instrument (ASI) ........ 17
Improving DLB Diagnosis: The Lewy Body Composite Risk Score 19
KEY ISSUE #2 : CAREGIVING ....................................................................... 20
The burdens and consequences of DLB carer stress .................... 20
Measuring the burdens experienced by DLB carers ..................... 22
Nursing Home Admission .................................................................. 24
CHAPTER 3: A NEW MODEL OF CARE ..................................................... 25
Person-Centred Care ........................................................................ 26
Palliative Care.................................................................................... 26
Supportive Dementia Care .............................................................. 27
RECOMMENDATIONS ............................................................................... 29
ABORIGINAL AUSTRALIANS .......................................................................... 29
RESEARCH ................................................................................................. 29
EDUCATION AND AWARENESS ..................................................................... 29
DIAGNOSIS ................................................................................................ 29
CAREGIVING ............................................................................................. 30
REFERENCES .............................................................................................. 31
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APPENDIX A: REM SLEEP BEHAVIOUR DISORDER SCREENING
QUESTIONNAIRE ....................................................................................... 35
APPENDIX B: REM SLEEP BEHAVIOUR DISORDER QUESTIONNAIRE –
HONG KONG (RBDQ-HQ) ....................................................................... 36
APPENDIX C: ALBA SCREENING INSTRUMENT ....................................... 37
APPENDIX D: THE LEWY BODY COMPOSITE RISK SCORE (LBCRS) ........ 41
APPENDIX E: RELATIVE STRESS SCALE (RSS) ............................................ 45
APPENDIX F: SUPPORTIVE CARE ............................................................. 46
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ABBREVIATIONS
AD
ASI
DLB
LBD
LBCRS
MCI
NHA
PD
PDD
RBD
RBD1Q
RBD-HK
RSS
Alzheimer’s Disease
ALBA Screening Instrument
Dementia with Lewy Bodies
Lewy Body Disease / Lewy Body Dementia
Lewy Body Composite Risk Score
Mild Cognitive Impairment
Nursing Home Admission
Parkinson’s Disease
Parkinson’s Disease Dementia
REM Sleep Behaviour Disorder
RBD Single Question
RBD Hong Kong scale
Relative Stress Scale
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v
EXECUTIVE SUMMARY
T
hroughout his life, Robin Williams made us
feel so good to be alive. He brought to us a
reason to smile, to laugh, and to cry. Robin
Williams also, very privately, struggled with
depression for most of his life.
On August 11, 2014, Robin Williams committed
suicide.
After his death, an autopsy of his brain showed
that he had Lewy Body Disease (LDB).
Prior to his death he had been diagnosed with
Parkinson’s Disease, was known to experience
periods of deep depression, anxiety and
increasing episodes of psychosis, which are all
classic symptoms of this newly discovered
disease.
In Australia, Lewy Body Disease (LBD) is the second most commonly
diagnosed neurodegenerative dementia, after Alzheimer’s Disease (AD).
As the disease slowly progresses, it causes almost total disability and
eventual nursing home admission (NHA). For the person with LBD, and those
family members who care for them, the progression of this disease is often
totally overwhelming.
Surprisingly, very little is known about LBD. We know nothing of what causes
it or who might be at risk of developing it. At present, there is no known
cure for LBD. Significant challenges exist in diagnosing and managing
people with LBD.
A diagnosis of LBD brings with it frustration, misunderstanding, a lack of
appropriate and support along with social isolation.
This report will investigate LBD in Australia, discuss some of the key issues,
and recommend some important initiatives to better the lives of everyone
affected by Lewy Body Disease.
Sorel Coward
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vi
CASE STUDY: MALCOLM’S STORY
The following is a fictitious case study that can help us gain an appreciation
of how someone with LBD, and their carers, might experience the world.
The study has been drawn together from a number of different LBD stories
that have been published elsewhere. This is the story of Malcolm.
Malcolm is male, Caucasian, and seventy-five years of age. He and June
have been married for fifty years. June, who is seventy-three years of age,
lives on the outskirts of Perth, and has been caring for Malcolm who was
diagnosed with LBD six years ago. However, Malcolm had been living with
dementia symptoms for almost a decade before he received his diagnosis.
After seeing many specialists, he had originally had been diagnosed with
Alzheimer’s Disease.
Malcolm had been experiencing increasing amounts of anxiety,
hallucinations, paranoia, and aggression – all of which were not responding
to the treatments normally effective in managing Alzheimer’s Disease.
During one crisis, June was not able to cope with Malcolm’s behaviour and
an acute emergency admission to hospital was a consequence.
Unfortunately, the first treating hospital doctor was not experienced in the
management of LBD, and administered an anti-psychotic medication to
control Malcolm’s hallucinations, paranoia and aggressive behaviour.
Because of this lack of experience, the doctor was unaware of the severe
sensitivity associated with administering an anti-psychotic drug to someone
with LBD. Malcolm had an adverse reaction to this drug and it was some
time before his world return to normal again.
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1
During the hospital admission, it became clear to June that she did not feel
she could cope anymore. June felt that she was on her own, and felt
misunderstood, tired, and frustrated; there was no one else to help.
She just couldn’t go on any more.
It was with a feeling of great relief that she agreed with the hospital staff
that it would be best if Malcolm was admitted to a residential aged care
facility. June also felt overwhelmingly guilty and sad that she could not
keep her promise to Malcolm - a promise to keep him at home until he
died.
Malcolm spent a further ten months in the psychiatric ward of the hospital.
The reason it took so long was that most aged care facilities had refused
outright to admit Malcolm once they were told of his LBD diagnosis. In
every instance, they said they did not have the staff or the skills to cope
with the aggressive behaviour and violence that so often characterises
people with LBD, like Malcolm.
Finally, a place was found but it was a long way from where June lived. For
the first time in their fifty-year marriage, June and Malcolm began living
apart. Now, more than ever before, June felt alone and vulnerable.
Malcom is now living in an aged care facility specialising in psychiatric
disorders and behaviours, and while June feels the facility is “pretty
ordinary”, in terms of its decor and facilities, the staff can effectively
manage Malcolm’s complex behaviours.
June catches two buses to get from her home to the nursing home. The
journey takes an hour. June spends four hours with Malcolm before
returning home on public transport. She does this four days a week.
Although June’s quality of life has improved since Malcolm’s admission, as
has her health, she is not so sure that being in a nursing home is the best
option for Malcolm.
With the burden of 24/7 care of Malcolm now lifted, June has had the time
to reflect more about her experience of dementia. She feels that there is
inadequate access to respite for dementia carers, like herself, because
through the whole time she was caring for Malcolm, she had to carry this
burden almost entirely on her own. June now feels that had she had more
opportunities for having a break, along with some community based care
support, it probably would have been possible, and better, for her to carry
on caring for Malcolm at home, at least for a while longer.
To this end, June has made the suggestion that the government would do
well to fund a system of local respite homes, especially for those in the early
stages of dementia. Ideally, when Malcolm would have been in respite
care, June feels that changes could have been made to better support her
in her work of caring. Unfortunately, this is more of an ideal than a reality
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because, presently, only one of two options are available to people with
dementia: care by one’s spouse/children or care in a nursing home.
June had been in touch with a local carers group and there she discovered
that whilst palliative care support and community based health care was
provided to support people caring for those with many different illnesses,
June did attend an Alzheimer’s Disease support group but, because
Malcolm’s situation was so different from Alzheimer’s Disease, she felt they
did not really understand her or could offer her support.
June also contacted a local palliative care group but was unable to get
any assistance because dementia was regarded as a chronic health
condition, not as an end-of-life event.
A few weeks ago, June received news that Malcolm’s health was
deteriorating and he was only expected to live a few more weeks. It had
been seven years since his diagnosis. A social worker came to help June
with transport and to help with organising Malcolm’s funeral. June is very
grateful for this help, but, quietly in her heart, she feels that the help she so
desperately needed came too late.
Imagine living your whole life ….and not remembering it,
Imagine forgetting those you love the most…
Imagine becoming as vulnerable as a small child…
Imagine how terrifying it must be to have dementia.
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CHAPTER 1: AGEING, DISABILITY & DEMENTIA IN
AUSTRALIA
The people of Australia, as a society, are experiencing a quantum change
in their sense of self-identity. For a long time, we have focussed upon
growth, development and expansion and with that an emphasis on
supporting and promoting the survival of the strongest. Now, however, our
population has aged so well that Australians are amongst the oldest
peoples in the world.
The proportion of people in our midst who will be regarded as being ‘older’,
i.e. over the age sixty-five years, is dramatically increasing and is projected
to continue to keep growing in an exponential manner. With that ageincrease in our population, the incidence of dementia illnesses is also
steadily increasing.
Now we must change our self-identity from strengthening the fittest to one
where we start promoting the wellbeing of those who are the weakest and
most vulnerable in our society.
AGEING
A
ustralians now enjoy a life expectancy that is among the highest in
world. Men aged 65 years, in 2013-2015 can expect to live another
19.5 years with an expected age at death being 84.5 years, whilst
women aged 65 years in 2013-2015 can expect to live another 22.3
years, with an expected age at death being 87.3 years1. Over the last two
decades the median age of the Australian population has increased by
three years over the last two decades from 34 years in 1996 to 37 years in
2016. In June 2016, South Australia had the second highest median age
(after Tasmania) of 42 years2. Noting that the median age of the world’s
1
Australian Institute of Health and Welfare (AIHW), Deaths, AIHW Website,
viewed 10 October 2017, <https://www.aihw.gov.au/reports/lifeexpectancy-death/deaths-in-australia/contents/life-expectancy>.
2
Australian Demographic Statistics (ABS), June 2016, ABS website, viewed
10 September 2017,
<http://www.abs.gov.au/ausstats/abs@.nsf/featurearticlesbyCatalogue/38
4EDBC8C1EB7717CA2581470023DAD1?OpenDocument>.
A SENSE OF SELF
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human population is 30 years3, this means that South Australians represent a
community that has one of the highest median ages in world.
Between 1996 and 2016, the proportion of Australia’s population aged 65
years and over increased from 12% to 15%. The rapid growth of ageing is
projected to continue to increase over the next quarter of a century with
25% of all Australians predicted to be over the aged of 65 years by 20504.
Between 1996 and 2016, the proportion of Australians aged 85 years and
over doubled by 100% (1% to 2%)5 and by 2050 is expected to increase by
another 250% to 1.8 million people6.
Issues pertaining to ageing in Australia, and especially in South Australia, are
of critical importance to both current and future policy development,
particularly in regards to age-related health and disability.
DISABILITY
Along with an ageing Australia, the occurrence of age-related disability in
Australia is also expected to significantly increase. In fact, it has already
begun.
The 2015 Survey of Disability, Ageing, and Carers (SDAC)7 indicated that
some one in five Australians, over the age of eighteen years (18.3% or 4.3
million people), reported they were living with some form of disability or
3
United Nation 2017, World population prospects the 2017 revision: key
findings and advanced tables, UN Department of Economic and Social
Affairs: Population Division, New York.
4
Australian Human Rights Commission 2012, Age positive: promoting
positive and diverse portrayals of older Australians, viewed 29 October,
2017,
<https://www.humanrights.gov.au/sites/default/files/FTFOlderAustralians.pdf
>.
5 Australian
Demographic Statistics (ABS), June 2016, ABS website, viewed
10 September 2017,
<http://www.abs.gov.au/ausstats/abs@.nsf/featurearticlesbyCatalogue/38
4EDBC8C1EB7717CA2581470023DAD1?OpenDocument>.
6 Australian
Human Right Commission 2008, A human rights approach for
ageing and health – respect and choice: home based and residential care
for older people (2012), footnote 3: The Treasury, Australian Government,
Australia to 2050: future challenges (2010), p. 56.
7 Australian
Bureau of Statistics, Disability, ageing, and carers, Australia
(SDAC) 2015, Australian Bureau of Statistics Website, viewed 4 September
2017, <http://www.abs.gov.au/ausstats/abs@.nsf/mf/4430.0>.
A SENSE OF SELF
5
chronic health condition. The majority of people with a disability, nearly
79%, reported their impairment was a result of a physical health condition,
and 21% reported their disability as being caused by a behavioural or
mental disorder.
These statistics, however, do not describe the clearest of pictures because
disability is disproportionately experienced by people sixty-five years and
over, with health problems and illness among the greatest causes of
disability in this age group. This is clearly demonstrated in table 1.
TABLE 1: PSYCHOSOCIAL DISABILITY AND AGE
DEMENTIA
Dementia is a condition that mainly affects older people and describes a
syndrome, of a chronic and progressive nature, that causes cognitive
impairment including memory, thinking, behaviour and personality.
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6
Dementia results in increasing degrees of disability8. Dementia is most often
caused by, and is usually only one symptom, of a number
neurodegenerative illnesses including Alzheimer’s Disease, Lewy Body
Disease, Vascular Dementia, and Fronto-Temporal Lobal Degeneration
(FTLD), Huntington’s Disease, Parkinson’s Disease and Creutzfeldt-Jacob
Disease.
Secondary dementias are those caused by other (usually nonneurodegenerative illnesses) such as HIV, Korsakoff’s Syndrome (alcohol
abuse related dementia), and acquired brain injury)9.
While it is true some cognitive decline is normal with advancing age, it is not
true that dementia is a normal part of ageing, notwithstanding commonly
held myths in the general community to the contrary.
Noting the above discussion about Australia’s ageing population, dementia
is going to be one of the most significant sources of disability in this country.
DEMENTIA IN ABORIGINAL AUSTRALIANS
Like in most other regards about Aboriginal people in Australia, dementia
amongst our First Nation people is characterised by unique complexities
and social injustice.
Importantly, there are significant cultural distinctions between how
dementia is viewed amongst First Nation people and the wider Australian
community.
Firstly, in most Aboriginal cultures, dementia is often not recognised as a
medical condition10. Instead dementia is often viewed as becoming
“childlike”, “sickness” or “madness”. These are considered normal changes
in some ageing Aboriginal people. But it is not until these behaviours break
8
World Health Organisation and Alzheimer’s Disease International 2012,
Dementia: a public health priority, WHO, Geneva.
9
Alzheimer’s Australia 2017b, Types of dementia, Alzheimer’s Australia
National Website, viewed 2 October 2017,
<https://www.fightdementia.org.au/about-dementia/types-of-dementia>.
10
Arkles, RS, Jackson Pulver, LR, Robertson, H, Draper, B, Chalkley, S & Broe,
GA 2010, Ageing, cognition, and dementia in Australian Aboriginal and
Torres Strait Peoples, Neuroscience Research Australia and Muru Marri
Indigenous Health Unit, University of New South Wales, Sydney.
A SENSE OF SELF
7
Aboriginal cultural norms that assistance from outside of the community is
sought and a diagnosis is determined11.
It is an established fact that Aboriginal people experience one the poorest
health outcomes of any community of people in the world and their
experience of dementia follows a similarly tragic course. Aboriginal people
experience dementia at a younger age and at a higher prevalence than
the rest of the people of Australia. In fact, the prevalence rate amongst
rural Aboriginal Australians has been determined at a rate of 5.2 times
greater than the overall Australian rate of 2.4%12.
One explanation for this is because it an established fact that Aboriginal
people are exposed to significantly higher rates of known dementia risk
factors, which include:
•
•
•
•
•
•
•
Smoking
Physical inactivity
Poor nutrition
Being overweight or obese
Excess alcohol consumption
A low standard of education
Heart disease and diabetes13
11
Arkles, RS, Jackson Pulver, LR, Robertson, H, Draper, B, Chalkley, S & Broe,
GA 2010, Ageing, cognition, and dementia in Australian Aboriginal and
Torres Strait Peoples, Neuroscience Research Australia and Muru Marri
Indigenous Health Unit, University of New South Wales, Sydney, p. 2
12
Flicker, L, and Holdworth, K 2014, Aboriginal and Torres Strait Islander
people and dementia: a review of the research, viewed 1 December 2017,
<
https://www.dementia.org.au/files/Alzheimers_Australia_Numbered_Publica
tion_41.pdf>, p. 15.
13
Flicker, L, and Holdworth, K 2014, Aboriginal and Torres Strait Islander
people and dementia: a review of the research, viewed 1 December 2017,
<
https://www.dementia.org.au/files/Alzheimers_Australia_Numbered_Publica
tion_41.pdf>, pp. 13-14.
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CHAPTER 2: LEWY BODY DISEASE
Early in the twentieth century, Frederick Lewy (1885-1950) first discovered
abnormal deposits of the alpha-synuclein protein in the brains of some
patients who experienced dementia. LBD was first described by Japanese
psychiatrist and neuropathologist Kenji Kosaka in 1976. To this day, autopsy
remains the only definitive method of diagnosis of the Lewy Body Disease.
It has been only been clinically diagnosable, i.e. by observable symptoms,
since 2005. As such, LBD is a relatively new disease.
TERMINOLOGY
LBD is an umbrella term that encompasses Parkinson’s Disease Dementia
(PDD) and Dementia with Lewy Bodies (DLB). The essential difference
between each is the onset of timing. With the emergence of motor
symptoms first PDD is the diagnosis and the emergence of cognitive
symptoms first results in diagnosis of DLB.
For much of its short history LBD has been, and is still
commonly described, as Lewy Body Dementia.
Increasingly, however, the term Lewy Body Disease
is being used by peak bodies like Dementia
Australia (formerly Alzheimer’s Australia).
This report will principally focus on DLB.
When it is appropriate, the term LBD will be used to
describe both DLB and PDD. Essentially, there are only subtle differences
between the two disorders.
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CORE FEATURES
The central characteristic of DLB is progressive loss of cognitive function that
significantly interferes with everyday life (dementia). The disease can
include the following types of symptoms:14
Movement disorders
Parkinsonism) including tremors, rigidity,
slowness of movement, difficulty walking and
postural instability
Cognitive impairment
Confusion, visual reasoning, executive
decision making, short term memory loss
Neuro-psychiatric
Visual hallucinations, delusional thinking,
paranoia, sleep disturbances e.g. REM Sleep
Behaviour Disorder
Behavioural
Sudden fluctuations of attention and
alertness, sometime referred to a delirium
Mood
Depression, apathy, anxiety, agitation
Autonomic
Blood pressure control, temperature
regulation, swallowing, fatigue, bladder and
bowel function
There is no cure to arrest the progression of DLB. The drugs that are used to
manage symptoms have all been developed for other disorders, including
AD, PD and a number of psychiatric conditions.
KEY ISSUE #1 : DIAGNOSIS
Health professionals, especially primary health physicians, struggle to
correctly diagnose DLB. For a disease that only recently was clinically
defined enough to permit a diagnosis being made this is not surprising. A
key issue in regards to the management of DLB is both elevated incidences
of misdiagnosis as well as under-diagnosis.
Delays in a timely and accurate diagnosis often result in lost opportunities
for earlier medical and social interventions for those with DLB. These lost
14
Lewy Body Dementia Association (LBDA), Lewy body dementia: the state
of the science, viewed online 22 August 2017,
<https://www.lbda.org/sites/default/files/2016_lbd_state_of_the_science_w
hite_paper.pdf>.
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10
opportunities of care and support quickly erode into the quality of life of
those living with DLB as well as carer experiencing burn-out.
As is the case with so many others aspects of DLB, and other similar
dementia related illnesses, the diagnostic issues are complex and multifaceted.
People with DLB will often report that they will have seen multiple specialists
before a correct diagnosis is determined on average some eighteen
months after the first visit to a doctor.
T HE PROBLEM OF MISDIAGNOSIS
Currently the only definitive method of diagnosing DLB is via a post-mortem
and the staining of brain tissue. Clearly, this is too late for clinicians, patients
and carers.
Prior to death, a differential diagnosis is obtained by asking the question
“What else could it be”? In the case of DLB there are two other likely
alternative answers to this question: Parkinson’s Disease and Alzheimer’s
Disease. These two illnesses share a significant overlap in symptoms,
particularly early in disease progression.
Consequently, people with DLB are frequently misdiagnosed with one of
these illnesses in up to 78% of instances15. As has previously been discussed,
a contributing factor lies in the order that various DLB symptoms may be
experienced, and reported.
Some people with early DLB will come to their doctor and report that they
15
Galvin, J, Duda, J, Kaufer, D, Lippa, C, Taylor, A & Zarit, S 2009, 'Lewy Body
Dementia: The Caregiver Experience of Clinical Care', Neurology, vol. 72,
no. 11, pp. A476-A476.
A SENSE OF SELF
11
have may be having a problem, predominantly, with their movements. This
group of DLB people may not experience cognitive problems until later, if at
all. In this instance, a clinician is likely to make a diagnosis of PD, and
understandably so. Just like with DLB, there is no objective test for PD: it too
is diagnosed by making an observation of symptoms. Studies have
frequently shown 15%-25% of people with a PD diagnosis are
misdiagnosed 1617.
Other people with DLB, however, present with problems being experienced
through cognitive impairment resulting in a diagnosis of AD, which is also
another illness that lacks any type of test to conclusively diagnosis it.
Consequently, people with AD are also frequently misdiagnosed in a similar
fashion18.
The situation is further complicated by post-mortem revelations that have
shown that a significant proportion of people with DLB pathology also had
AD pathology19, with some studies demonstrating rates as high as sixty
percent of people with DLB having mixed pathology with AD20.
The diagnostic situation is this: three similar illnesses, all with overlapping
symptoms, that follow a unique course with each person who is offered a
provisional diagnosis based on the observation of clinical symptoms prior to
death and a confirmed diagnosed only post-mortem.
The difficulty in making a diagnosis of DLB is further complicated by the fact
that there is a very high degree of subjective variability in what symptoms
are experience, in the order of their appearance and their degree of
impairment that they cause. Consequently, people with DLB are going to
experience the disease uniquely, making the task of objective diagnosis all
the more difficult.
16
Jellinger, KA 2003, 'How valid is the clinical diagnosis of Parkinson's disease
in the community?', Journal of Neurology, Neurosurgery and Psychiatry, vol.
74, no. 7, pp. 1005.
17
Joustra, J, Gardberg, M, Röyttä, M & Kassinen, V 2014, ‘Diagnostic
accuracy of parkinsonism syndromes by general neurologists’. Parkinsonism
& Related Disorders, vol. 20, no. 8, p. 840.
18
Qian, W, Schweizer, T, Munoz, D & Fischer, CE 2016, 'Misdiagnosis of
Alzheimer’s Disease: inconsistence between clinical diagnosis and
neuropathological confirmation’, Alzheimer's & Dementia: The Journal of
the Alzheimer's Association, vol. 12, no. 7, pp. 293.
19 Alzheimer’s Association 2017, Mixed Dementia, viewed on 8 November
2017, <https://www.alz.org/dementia/mixed-dementia-symptoms.asp>.
20
Barker, WW, Luis, AC, Kashuba, GA, Luis, GM, Harwood, GD, Loewenstein,
GD, et al 2002, 'Relative Frequencies of Alzheimer Disease, Lewy Body,
Vascular and Frontotemporal Dementia, and Hippocampal Sclerosis in the
State of Florida Brain Bank', Alzheimer Disease & Associated Disorders, vol.
16, no. 4, pp. 203-212.
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12
T HE PROBLEM CAUSED BY LOW AWARENESS OF DLB
A correct diagnosis is difficult to come by because doctors, patients, and
carers, together, often do not have even a basic awareness of DLB. Many
people associate dementia with the word ‘Alzheimer’s’. If the presenting
symptoms do not correlate with typical AD then ‘dementia, not further
described’, becomes the diagnosis and the common identifier. The
problem, of course, is that dementia is not an illness, per se, but a symptom
of some other pathology.
It is easy to imagine that a busy health professional is not going to be able
to easily diagnose a condition they are not familiar with, especially in a ten
to fifteen-minute consult.
Primary health physicians have reported they lack knowledge of support
services in the community, and that they have low confidence in their
diagnostic and management skills of conditions that include behavioural
problems as symptoms, such as dementia21.
In addition, patients and carers are not likely to mention, for example, sleep
disturbances or behavioural problems (key DLB symptoms) when discussing
problems with thinking or difficulties with movement. Furthermore, studies
have shown that few in the general community have an appreciation of
the distinction between normal age-related cognitive decline and
dementia.
The combination of little awareness by doctors and family/friends create a
situation where misdiagnosis becomes highly likely. This lack of awareness,
in fact, is sometimes described by some as being a ‘symptom’ of DLB, that
is, being misunderstood and a significant delay in receiving the correct
diagnosis.
The best way to appreciate why increasing DLB aware is so critical can be
best summarised can be found in the epithet of The Lewy Body Society (a
registered charity in England and Wales):
“
The more people who know, the fewer people who suffer
”
21
Galvin, J, Duda, J, Kaufer, D, Lippa, C, Taylor, A & Zarit, S 2009, 'Lewy Body
Dementia: The Caregiver Experience of Clinical Care', Neurology, vol. 72,
no. 11, p. A476.
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13
N EW C LINICAL D IAGNOSTIC C RITERIA
One of the inherent problems of DLB diagnosis, as has been discussed
above, is inherent inaccuracies associated with diagnosing
neurodegenerative diseases like DLB, AD and PD with clinical observations
alone.
Using the established DLB clinical guidelines has been demonstrated to
have high specificity (i.e. they can differentiate between diseases) but low
sensitivity (i.e. the illness has to be significantly advanced before it can be
detected)22. The detection of DLB sooner remains an elusive goal, as does
detection of the DLB in the prodromal (before the manifestation of
symptoms) stage.
Previously, the criteria were those used from DLB 2005 consortium23.
In the middle of 2017 a new set of clinical criteria for the diagnosis of DLB
were published by the same consortium of researchers24. These are as
follows:
Dementia is required. Cognitive impairment in attention, reasoning and
problem solving, visuospatial skills.
with
At least two of the following:
•
•
•
•
Delirium-like fluctuating cognition. Unpredictable changes in
thinking, attention and alertness
Repeated visual hallucinations. A common DLB symptom occurring
about 80% of the time)
REM Sleep Behaviour Disorder. Sometimes appearing up to a
decade or more before the onset of DLB.
Parkinsonism, specifically slowed movements, tremor when limbs at
rest, and muscle rigidity.
22
Nelson, P, Jicha, G, Kryscio, R, Abner, E, Schmitt, F, Cooper, G, et al 2010,
'Low sensitivity in clinical diagnoses of dementia with Lewy bodies', Journal
of Neurology, vol. 257, no. 3, pp. 359-366.
23
McKeith IG, Dickson DW, Lowe J, et al. 2005, ‘Dementia with Lewy bodies:
diagnosis and management: third report of the DLB Consortium,’
Neurology, vol. 65, no 1, pp. 1863–1872.
24
Mckeith, I, Boeve, B, Dickson, D, Halliday, G, Taylor, -P, Weintraub, D, et al
2017, 'Diagnosis and management of dementia with Lewy bodies Fourth
consensus report of the DLB Consortium', Neurology, vol. 89, no. 1, pp. 88100.
A SENSE OF SELF
14
Of note, is the fact that DLB patients, in particular, are extremely sensitive to
many antipsychotic medications that are used to treat the neuropsychiatric symptoms of the disease. The adverse reactions can actually
worsen the symptoms and in a few circumstances, they have caused
death. This makes managing the behavioural side of DLB particularly
challenging.
REM S LEEP B EHAVIOUR D ISORDER
In 2005, REM Sleep Behaviour Disorder (RBD) was noted as being highly
suggestive of the presence of DLB pathology.
In the 2017, DLB clinical criteria, RBD has now been recognised as a core
feature of DLB diagnoses, which is not surprising given how rare this disorder
is and how commonly it occurs in people with DLB (about 80% of DLB
diagnoses feature sleep disturbance caused by RBD)
First described in 1986, RBD, as its names describes, manifests itself as a
disturbance of sleep that occurs during REM sleep (stage 5 sleep). It is
characterised by two primary symptoms: unusually graphic and disturbingly
violent dreams and violent behaviours that are the acting out of those
dreams. Diagnosis occurs in an overnight sleep study where it is indicated
by the loss of muscle atonia that normally occurs during REM sleep thereby
allowing them to act their dreams25.
RBD is usually noticed either by a bed-partner or in a sleep study, often
while undertaking a diagnosis for something else. The person with the
disorder is usually unaware of the behaviour, save only when either their
partner or they themselves are injured by the violent dream enactment
behaviour itself.
The prevalence of RBD is not well understood. A recent (2015) population
based study estimated the prevalence of RBD to be 7%26. Initially, RBD was
thought to be idiopathic however a growing body evidence suggests that
a person with RBD has a very high risk of later developing
neurodegenerative illnesses. In a recent UK study, the risk of
neurodegenerative pathology was found to be
25
Mahowald, MW, Schenck, CH 2011, ‘REM sleep parasomnias’, in MH
Kryger, T Roth, C Dement (eds.), Principles and Practice of Sleep Medicine,
5th edn, Elsevier-Saunders, Philadelphia, 1083e1097.
26
Mahlknecht, P, Seppi, K, Frauscher, B, Kiechl, S, Willeit, J, Stockner, H, et al
2015, 'Probable RBD and association with neurodegenerative disease
markers: A population-based study', Movement Disorders, vol. 30, no. 10,
pp. 1417-1421.
A SENSE OF SELF
15
•
•
•
33% at five years post RBD diagnosis,
75% at ten years, and
90% at fourteen years 27.
The most frequent neurodegenerative illness that was diagnosed post RBD
diagnoses was DLB, then PD and MSA.
Essentially what this means is that nearly everyone who develops RBD will
going to develop a neurodegenerative disease and most likely the
diagnosis will be DLB.
Correspondingly, current estimates indicate that nearly 75-80% of DLB
patients will develop RBD post DLB diagnosis28.
It follows then, that in the presence of other diagnostic criteria, an RBD
diagnosis has the capacity to strongly confirm the presence of current DLB
pathology.
It is has become apparent, then, that RBD can also be, and should be, used
as a highly predictive DLB screening mechanism.
One of the inherent difficulties in the diagnosis of RBD is that a sleep study is
necessary to confirm it. This is expensive, time consuming and often not of
practical utility, noting that this procedure is available only in a specialised
sleep laboratories.
As interest in RBD has grown, particularly with its association to DLB and PD,
a number of screening instruments have been developed to make a
provisional RBD diagnosis and thereby identify the patients for whom an
overnight sleep study would be recommended. Two instruments are
commonly used for RBD screening: the thirteen question RBD Screening
Questionnaire (RBDSQ)29 and the RBD Hong Kong scale (RBD-HK)30. Their
27
Iranzo, A, Fernández-Arcos, A, Tolosa, E, Serradell, M, Molinuevo, JL,
Valldeoriola, F, et al. 2004, 'Neurodegenerative disorder risk in idiopathic
REM sleep behaviour disorder: study in 174 patients', PLoS ONE, vol. 9, no. 2,
pp. e89741.
28
Murray, EM, Ferman, JT, Boeve, FB, Przybelski, AS, Lesnick, GT, Liesinger,
MA, et al 2013, 'MRI and pathology of REM sleep behaviour disorder in
dementia with Lewy bodies', Neurology, vol. 81, no. 19, pp. 1681-1689.
29
Marelli, S, Rancoita, P, Giarrusso, F, Galbiati, A, Zucconi, M, Oldani, A, et
al 2016, 'National validation and proposed revision of REM sleep behavior
disorder screening questionnaire (RBDSQ)', Journal of Neurology, vol. 263,
no. 12, pp. 2470-2475.
30
Li, S, Wing, Y, Lam, S, Zhang, J, Yu, M, Ho, C, et al 2010, 'Validation of a
new REM sleep behavior disorder questionnaire (RBDQ-HK)', Sleep
Medicine, vol. 11, no. 1, pp. 43-48.
A SENSE OF SELF
16
length, however, make them unsuitable for inclusion in large scale
epidemiologic surveys. A one question adaption of these two instruments31
(RBD1Q) has recently demonstrated that it has equivalent, or better,
accuracy and sensitivity than RBDSQ and RBD-HK. The question that is
asked is
“Have you ever been told, or suspected yourself, that you seem to ‘act out
your dreams’ while asleep (for example, punching, flailing your arms in the
air, making running movements, etc.)?”
There are two limitations about these three RBD screening tools:
Firstly, screening tools cannot replace a video-polysomnogram that can
measure any loss of REM sleep muscle atonia, which is the only current
method of RBD diagnosis.
Secondly, the utility of these screening tools outside of the cohort of
patients in sleep clinics (who are likely to be knowledgeable of their
condition) remains unknown.
Notwithstanding this, the RBD1Q certainly possesses the potential utility for
inclusion in large scale epidemiological studies which would provide a
mechanism to more accurately determine the prevalence of RBD and in so
doing increase our knowledge of the risk of DLB in the general population.
Further studies along these lines will more be able to clarify the link between
RBD and DLB, with a view to assessing what environmental, lifestyle or
medical interventions might mitigate the onset of future DLB.
A copy of the REM Sleep Behaviour Screening Questionnaire has been
placed in Appendix A, at the end of this report.
IMPROVING DLB D IAGNOSIS: ALBA S CREENING INSTRUMENT (ASI)
Up until now, the only methods availability to suggest the presence of DLB
were general dementia screening tools.
However, earlier this year a new screening instrument was published with
high specificity and high sensitivity for DLB:32 the ALBA Screening Instrument
(ASI). The ASI has been proven useful in detecting DLB quite early on in the
31
Postuma, RB, Arnulf, I, Hogl, B, Iranzo, A, Miyamoto, T, Dauvilliers, Y, et al
2012, 'A single-question screen for rapid eye movement sleep behavior
disorder: A multicenter validation study', Movement Disorders, vol. 27, no. 7,
pp. 913-916.
32 Garcia, BM, Fernandez, M, Ojea, QM, Rojas, J, Garcia, BM, Bogliotti, E, et
al 2017, 'ALBA Screening Instrument (ASI): A brief screening tool for Lewy
Body Dementia', Archives of Gerontology and Geriatrics, vol. 70, pp. 67-75.
A SENSE OF SELF
17
progression of the disease. It also has the advantage of being able to be
easily administered by both medical and non-medical staff.
Published in mid-2017 by the Lewy Body Association of Argentina (ALBA),
little can be reported about the ASI, at this stage, except to say that in the
research domain it has demonstrated great promise in the earlier and more
accurate diagnosis of DLB than has hitherto been possible.
The ASI consists of seventeen questions divided into three sections:
screening for dream enactment behaviours, visual hallucinations, and
cognitive symptoms. The instrument can be administered by non-medically
trained people in about ten minutes.
The ASI was validated against the 2005 clinical criteria for DLB33.
Consequently, in the light of the new diagnostic criteria outlined above, the
ASI will need further validation against the 2017 clinical standards.
However, noting that a factor analysis of the ASI showed that its focus on
screening for REM sleep disorders was very strong it is highly likely that the
ASI will retain its validity in the new standard.
TABLE 2: ASI SCORES. (OTHER DEMENTIAS INCLUDE ALZHEIMER’S DISEASE AND VASCULAR
DEMENTIA)
These results34 demonstrate that the ASI has good specificity in
discriminating DLB from other forms of dementias as well as mild cognitive
impairment that is a normal age-related non-pathological condition.
33
McKeith, I, Dickson, D, Lowe, J, Emre, M, O’Brien, J, Feldman, H, et al.
2005, Diagnosis and management of dementia with Lewy Bodies: third
report of the DLB Consortium, Neurology, vol. 65, no. 12, pp. 1863–1872.
34
Garcia, BM, Fernandez, M, Ojea, QM, Rojas, J, Garcia, BM, Bogliotti, E, et
al 2017, 'ALBA Screening Instrument (ASI): A brief screening tool for Lewy
Body Dementia', Archives of Gerontology and Geriatrics, vol. 70, p. 72.
A SENSE OF SELF
18
A copy of the ASI has been placed in Appendix B at the end of this report.
IMPROVING DLB D IAGNOSIS: T HE L EWY B ODY C OMPOSITE R ISK S CORE
The Lewy Body Composite Risk Score (LBCRS), a screening instrument
designed by researchers at Florida Atlantic University, has also been
another recent development in the fight to improve DLB diagnosis35.
Published in 2015, the LBCRS consists of ten yes/no questions. Four of the
questions aim to detect motor symptoms characteristic of DLB. The
remaining six questions focus on DLB non-motor symptoms.
Impressively, the LBCRS was able to significantly discriminate between AD
(the most common misdiagnosis) and DLB with the following results:36
Visual hallucinations
Delusional misidentifications
REM Sleep Behaviour Disorder
Cognitive fluctuations
AD
8.4 %
1.1 %
5.3 %
43.9 %
DLB
47.8 %
34.8 %
41.5 %
92.2 %
A copy of the LBCRS can be located in Appendix C, at the end of this
report.
***
The two DLB screening tools discussed in this paper not only provide
mechanisms to more quickly and accurately diagnose DLB, but also
provide hope to those experiencing the disease that early diagnoses will
result in the more accurate interventions being able to be used and with
them significant improvements in quality of life, especially early on in the
disease progression. Furthermore, the high discrimination capacity of both
these instruments can mean that candidates for clinical trials and inclusion
on a DLB register will be more easily identified.
35
Galvin, JE 2015, 'Improving the clinical detection of Lewy body dementia
with the Lewy body composite risk score', Alzheimer's & Dementia:
Diagnosis, Assessment & Disease Monitoring, vol. 1, no. 3, pp. 316-324.
36
Galvin, JE 2015, 'Improving the clinical detection of Lewy body dementia
with the Lewy body composite risk score', Alzheimer's & Dementia:
Diagnosis, Assessment & Disease Monitoring, vol. 1, no. 3, p. 319.
A SENSE OF SELF
19
KEY ISSUE #2 : CAREGIVING
People who are aged, and those with dementia in particular, are one of
the most vulnerable groups of people in our community. When a person
starts to experience the symptoms of dementia it is nearly always a family
member, usually a spouse or the children, who take up the responsibility of
caregiving. As the disease progresses, the burden, correspondingly,
increases as well, and often intolerably so.
The rationale for placing someone in a nursing home is often made on the
basis that it is “for their own good”. However, it is often the case that it is not
so much the needs of the person with dementia that are of primary
concern in this decision-making process but instead the welfare of carer
and his or her’s wellbeing. The decision to place an individual in a nursing
home is usually made when the family-carer is no longer able to provide
adequate care.
Unfortunately, this is a common scenario.
An important social question here becomes apparent: is it just to place a
person with dementia in nursing care, simply out of concern for the carer’s
wellbeing? It is unfortunately true that often there are no other alternatives.
In our society, the two primary forms of care for people with dementia is
either family-based care or institutional care. Particularly in the instance of
Dementia with Lewy Bodies, it is also true that, with the exception of where
the disease progression is very advanced, often neither of these alternatives
are the most optimal.
Numerous studies have found that neuropsychiatric symptoms are the most
predictive of caregiver burden. The most problematic neuropsychiatric
symptoms were disruptive behaviours (e.g. agitation and aggression),
delusions, hallucinations, and mood disturbances. These findings have
been found regardless of particular dementia diagnoses37. Consequently,
in regards to Dementia with Lewy Bodies, the prominence of
neuropsychiatric symptoms would predict a higher carer burden than many
other forms of dementia. This is, in fact, the case38.
T HE BURDENS AND CONSEQUENCES OF DLB CARER STRESS
37
Cheng, S 2017, 'Dementia Caregiver Burden: a Research Update and
Critical Analysis', Current Psychiatry Reports, vol. 19, no. 9, pp. 1-8.
38
Leggett, AN, Zarit, S, Taylor, A & Galvin, JE 2011, 'Stress and Burden
among Caregivers of Patients with Lewy Body Dementia', Gerontologist, vol.
51, no. 1, pp. 76-85.
A SENSE OF SELF
20
A 2010 Lewy Body Dementia Association survey of caregivers found that
90% of people with DLB were unable to attend to even the most basic of
daily needs such as shopping and cooking, and over 60% needed
assistance with activities of daily living such as personal hygiene. The most
common burdens reported were fears about the future of their loved one,
balancing caregiving with other personal responsibilities, social isolation,
and high levels of uncertainty.
It is also of interest to note. that in relation to AD, the commonest form of
dementia, DLB caregivers are often younger with a mean age of 55.9 years
and were predominantly female.39 This places DLB spousal caregivers in a
unique situation within the dementia world in that being younger, and
especially female, caregivers may also have the additional responsibilities
of providing care and support to either their children and/or their parents40.
DLB Family caregivers frequently report experiencing chronic stress with
adverse outcomes on their physical, emotional, and mental health.
Frequently, over-stressed carers will report decreased well-being,
depression, anger, anxiety, and a variety of health and medical problems
that are normally exacerbated by experiencing abnormally high levels of
stress41.
Pearlin, and his colleagues, proposed a highly influential model of care,
namely, The Stress Process Model of Caregiving42. In this model, and others
that are similar to it, caregiving is understood to be, in itself, a significant
stressor. If this stressor is then appraised as a burden then there is a high
likelihood of psychological distress developing in the carer. In a 2010 DLB
caregiver study43, Leggett and his colleagues found ample support that the
Stress Process Model of Caregiving explained the significant burdens of DLB
39
Lewy Body Dementia Association 2010, Caregiver burden in Lewy Body
dementias: challenges in obtaining diagnosis and providing daily care,
viewed 2 December 2017,
<https://www.lbda.org/sites/default/files/caregiver-burden-in-lbd--released-7-12-2010.pdf>
40
Zweig, Y & Galvin, J 2014, 'Lewy body dementia: the impact on patients
and caregivers', Alzheimer’s Research & Therapy, vol. 6, no. 2, pp. 21.
41
Leggett, AN, Zarit, S, Taylor, A & Galvin, JE 2011, 'Stress and Burden
among Caregivers of Patients with Lewy Body Dementia', Gerontologist, vol.
51, no. 1, pp. 76-85.
42
Pearlin, L, Mullan, J, Semple, S & Skaff, M 1990, 'Caregiving and the Stress
Process: An Overview of Concepts and Their Measures', The Gerontologist,
vol. 30, no. 5, p. 583.
43
Leggett, AN, Zarit, S, Taylor, A & Galvin, JE 2011, 'Stress and Burden
among Caregivers of Patients with Lewy Body Dementia', Gerontologist, vol.
51, no. 1, pp. 76-85.
A SENSE OF SELF
21
carers. They further elaborated this approach to asserting that DLB
caregiving burden should not be understood as linear form of caring (e.g.
accommodating a gradual but systemic loss of memory as in AD). Instead
DLB caring should be appreciated multidimensional and contextually
driven.
Because of the multidimensional nature of DLB symptomology, rather than
these stressors being additive it would be the author’s hypothesis that they
are instead exponential in their nature. Further research would be required
to determine whether or not this was true.
So, the emerging picture is that the greatest burden experienced by those
caring for people with DLB stems from the behavioural and emotional
problems associated with the neuropsychiatric symptoms of this particular
disease. This, in turn, compounds the stresses experienced by having to
provide support for activities of daily living that are common amongst all
carers of people with dementia in general.
Another important, but often over-looked, source of stress and burden
amongst carers of people with DLB is that of isolation. People who have
DLB, and those who care for them, find themselves socially isolated
because there is little public awareness about DLB and consequently they
often feel misunderstood, alone, and feeling poorly supported.44
The majority of community dementia support groups are often focussed to
address support for those caring for AD or PD and consequently are of
limited benefit to DLB carers and their unique challenges. Clearly, the
caring for a person with DLB is a situation with unique complexities. It is vital
that DLB carers receive not only adequate, but also appropriately targeted,
support.
M EASURING THE BURDENS EXPERIENCED BY DLB CARERS
A 2016 study by Svendsboe and colleagues45 examined directly the
question of burden of care experienced by people caring for those with
DLB. They compared carers of people with AD with carers of those
experiencing DLB and found that 55.4% of caregivers generally were
44
Leggett, AN, Zarit, S, Taylor, A & Galvin, JE 2011, 'Stress and Burden
among Caregivers of Patients with Lewy Body Dementia', Gerontologist, vol.
51, no. 1, pp. 76-85.
45
Svendsboe, E, Terum, T, Testad, I, Aarsland, D, Ulstein, I, Corbett, A et al
2016, 'Caregiver burden in family carers of people with dementia with Lewy
bodies and Alzheimer's disease', International Journal of Geriatric Psychiatry,
vol. 31, no. 9, pp. 1075-1083.
A SENSE OF SELF
22
spouses, and 44.6% were children. In other words, the only non-institutional
caring that was supporting people with DLB was family based.
What happens, then, to those people who are single, widowed or divorced,
and who have no children, remains a serious social justice question for
which there are, at present, no just answers. Even with mild impairment, this
group of people are going to end up with a premature nursing home
admission, and sometimes that is going to be involuntarily so.
Using the Relative Stress Scale (RSS)46, the Svendsboe study they found that
carers of people with DLB experienced significantly higher levels of stress
when compared with AD and other dementia carers, and represented a
particularly vulnerable group of people. Consequently, people who care
for those experiencing DLB are at significantly higher risk of burn-out.
In an earlier 2007 study, Ulstein and his colleagues demonstrated that the
RSS was a very useful tool in measuring dementia carer burden.47 The
outcomes of this study recommended the following responses when
measuring dementia care burden: those scoring
•
≤ 23
(low risk)
Should be referred to community based
education
•
≥23 - ≤30
(intermediate risk)
Should be closely monitored for the presence,
or onset of, depression and other stressrelated psychiatric disorders. Individual
counselling and support group participation is
recommended.
•
>30
(high risk)
Referral for psychiatric assessment and
treatment
The Svendsboe study48 found that 31% of DLB carers were in the
intermediate or high-risk group. It is clearly demonstrated, then, that the
need for more effective support for DLB carers is pivotal.
46
Greene, JG, Smith, R, Gardiner, M & Timbury, GC 1982, 'Measuring
behavioural disturbance of elderly demented patients in the community
and its effects on relatives: a factor analytic study', Age and ageing, vol. 11,
no. 2, pp. 121.
47
Ulstein, I, Bruun, WT & Engedal, K 2007, 'The relative stress scale, a useful
instrument to identify various aspects of carer burden in
dementia?', International Journal of Geriatric Psychiatry, vol. 22, no. 1, pp.
61-67.
48
Svendsboe, E, Terum, T, Testad, I, Aarsland, D, Ulstein, I, Corbett, A et al
2016, 'Caregiver burden in family carers of people with dementia with Lewy
bodies and Alzheimer's disease', International Journal of Geriatric Psychiatry,
vol. 31, no. 9, pp. 1075-1083.
A SENSE OF SELF
23
A copy of the RSS has been placed in Appendix E.
N URSING H OME A DMISSION
One of the greatest stressors that a carer has to deal with is the difficult
decision of deciding when nursing home admission become impossible to
avoid. People with DLB, and their carers, have already experienced the
frustration, isolation, and the consequences that have resulted from the
significant lack of professional knowledge about the disease and how to
‘treat’ it. There are constant concerns about overmedication or being
inappropriately medicated.
A second stressor, in regards to nursing home admission, arises from the
testimonies of numerous carers who have reported that many facilities are
reluctant to admit a person with an DLB diagnosis. This is because of the
behavioural and emotional symptoms that people with DLB exhibit, the
disruption it causes to others, and the lack of staff training to manage these
complex behavioural and emotional symptoms.49 This is especially so given
that many of these behavioural and emotional symptoms cannot be safely
managed pharmacologically in people who have DLB.
49
Zweig, Y & Galvin, J 2014, 'Lewy body dementia: the impact on patients
and caregivers', Alzheimers Research & Therapy, vol. 6, no. 2, pp. 21.
A SENSE OF SELF
24
CHAPTER 3: A NEW MODEL OF CARE
This report began by asserting that the majority of older people do not
develop dementia. The risks of developing dementia, nevertheless,
exponentially increase with age. Noting that Australia has one of the oldest
populations in the world, Professor Julian Hughes, in a 2013 seminal
discussion paper for Alzheimer’s Australia50, has proposed two important
conceptual questions in regards to the future of dementia care in this
country.
The first of these issues is that if the prevalence of dementia rises with age
should it be now be regarded as normal part of aging?
Secondly, if the prevalence of dementia increases as we approach death,
should we then begin to understand dementia as a terminal condition, as
opposed to a chronic health condition as we currently do.
TABLE 3: PROJECTED INCREASE OF DEMENTIA IN AUSTRALIA (HUGHES, 2003)
Table 3, above, presents some extraordinary data: by 2050 the number of
older people with dementia is project to increase from between 90% to
592%. It represents the single most acute crisis in health that our society has
ever had to face.
In his paper, Hughes explores three philosophies of dementia care:
1. Person-centred care
2. Palliative Care
3. Supportive Care
50
Hughes, J 2013, Models of dementia care: person-centred, palliative and
supportive, viewed 29 November 2017, <
https://www.dementia.org.au/files/NATIONAL/documents/AlzheimersAustralia-Numbered-Publication-35.pdf>.
A SENSE OF SELF
25
P ERSON -C ENTRED C ARE
Person-Centred Dementia Care51 has been, especially in recent years, the
predominant model of dementia care in this country. In this philosophy
four fundamental tenets are affirmed. People with dementia should be:
•
•
•
•
Valued
treated as Individuals
Their Perspective must inform our understanding and our responses
And their Social life and their relationships must be promoted as the
very basis of personhood.
(VIPS)
A criticism of this approach, however, is that this philosophy does not
consider dementia as an end-of-life issue, and, consequently, it provides
little guidance in relation to the very difficult decisions regarding nursing
home admissions, medical treatments and the withdrawal of medical
interventions.
P ALLIATIVE C ARE
At present, the resources and the responses of our society to the dying are
qualitatively more life promoting than for those with a diagnosis of
dementia. Slowly, there is an increasing movement to promote to the
palliative care model as a response to dementia. In palliative care:
•
•
•
The focus is not on dying but on living and living as well as possible
Distressing symptoms are actively treated
Care is holistic and is a synthesis of physiological, psychological, social
and spiritual interventions.
A number barriers, however, present themselves in regards to palliative
dementia care. Principal among these is that, conceptually, this philosophy
was developed largely in response to cancer care. Inherent in this model is
the concept that people with cancer often die within months, however
with dementia that process can span many years.
51
Brooker, D & Netlibrary, I 2007, Person-centred dementia care making
services better, Jessica Kingsley Publishers, London ; Philadelphia.
A SENSE OF SELF
26
S UPPORTIVE D EMENTIA C ARE
A new and innovative philosophy, Supportive Dementia Care, has been
proposed by Professor Hughes as a new and innovative philosophy of
dementia care that synthesises both person-centred care and palliative
care. Professor Hughes describes Supportive Care as…
“… a full mixture of biomedical dementia care, with good quality,
person-centred care, psychosocial and spiritual care under the
umbrella of holistic palliative care throughout the course of a
person’s whole experience of dementia, from diagnosis until death,
and for families and close carers, beyond.”52
In its essence, it is a form of multidimensional, biopsychosocial and spiritual
dementia care that extends across time, and across the complete course
of diseases like DLB.
At the end of this report, Appendix F display a table of the proposed
components of Supportive Dementia Care. Given the very high degree of
subjective differences in dementia care, particularly amongst people with
DLB, this model cannot, of course, be a complete description of the facets
and possibilities of this new model.
At the very least Supportive Dementia Care provides a philosophy of care
for people with dementia, particularly those with DLB. It represents a
quantum change in our attitude to caring for with dementia; it represents
52
Hughes, J 2010, ‘End-of-life decisions and dementia’, in D Ames, D Burns
& J O’Brien (eds), Dementia, 4th edn, Arnold Health Sciences, London,
p.301.
A SENSE OF SELF
27
an innovative change that is imperative for our health system and finally,
Supportive Dementia Care engenders hope, dignity, value and respect.
A Daughter’s Decree to
Lewy Body
A SENSE OF SELF
28
RECOMMENDATIONS
ABORIGINAL AUSTRALIANS
•
Dementia should be incorporated into pre-existing preventative
health strategies and programmes aimed at lowering the exposure
of Aboriginal people to known dementia risk factors.
•
A focus on awareness, education and support services for
Aboriginal people with dementia and their carers needs to be
radically improved, particularly for those residing in remote areas.
RESEARCH
•
It is recommended that future epidemiological studies include the
RBD1Q to determine more accurate prevalence data for REM Sleep
Behaviour Disorder.
•
With the use of the new DLB and RBD screening tools, it is
recommended that a national LBD register be established. This
register can then provide a pool of participants with probable DLB,
PDD, and RBD for inclusion in clinical trials.
EDUCATION AND AWARENESS
•
Public awareness campaigns and education programmes to raise
the profile of dementia generally and Lewy Body Disease in
particular is a critical need.
•
It is imperative that medical education about and training in DLB
diagnosis amongst health professionals, particularly for primary
health physicians, be prioritised.
DIAGNOSIS
•
The DLB diagnostic guidelines should be circulated widely amongst
South Australia’s Local Health Networks and the various Divisions of
General Practice.
•
Training of primary health care physicians needs to emphasise the
clinical relationship between REM Sleep Behaviour Disorder and the
later development of neurodegenerative diseases in general and
DLB in particular.
A SENSE OF SELF
29
CAREGIVING
•
The majority of dementia related support is addressed toward AD or
PD. The report illustrated that the majority of this support network
does not address the unique burdens LBD caregivers experience,
especially in regards to the neuro-psychiatric symptoms which are a
particularly demanding source of stress. It is recommended that
specialised support for LBD carers be developed.
•
In this report, the underlying assumption about caregiving is that this
will be provided by either a spouse or by children, especially in the
early to moderate levels of dementia progression. Single, widowed
and or divorced people without children, and who also have a
diagnosis of dementia, represent one of the most vulnerable groups
of people in our society today. This group of people face the unjust
situation of being admitted to an aged care facility, not because
their mental health or quality of life warrant it, but because there are
at present no options. Alternatives to nursing home admissions need
to be investigated and developed that this social injustice may be
addressed.
•
This reported has also alluded to the fact that when the time of
nursing home admission becomes impossible to avoid that the
opportunities of professional care are often limited for those with a
LBD diagnosis. It is recommended that training in the aged care
sector include specialist LBD education and training for its workforce.
As our society grows the number of people with LBD is going to
increase sufficiently that dedicated LBD facilities will need to be
developed in response to this growing need.
•
In particular the adoption of the model of Supportive Dementia
Care is recommended and governments are encouraged to
explore this option.
•
In particular, noting the significant increase we are facing in this
country now, it crucial that provisions be made for the construction
of more nursing homes that are specifically set up to handle
behaviourally challenging clients such as those with DLB
•
It is strongly recommended that “Supportive Care” become the
preferred conceptual model for supporting dementia patients in
general, people with DLB and dementia carers in particular.
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30
REFERENCES
Alzheimer’s Association 2017, Mixed Dementia, viewed on 8 November
2017, <https://www.alz.org/dementia/mixed-dementia-symptoms.asp>.
Alzheimer’s Australia 2017, Types of dementia, Alzheimer’s Australia National
Website, viewed 2 October 2017,
<https://www.fightdementia.org.au/about-dementia/types-of-dementia>.
Arkles, RS, Jackson Pulver, LR, Robertson, H, Draper, B, Chalkley, S & Broe,
GA 2010, Ageing, cognition, and dementia in Australian Aboriginal and
Torres Strait Peoples, Neuroscience Research Australia and Muru Marri
Indigenous Health Unit, University of New South Wales, Sydney.
Australian Bureau of Statistics, Disability, ageing, and carers, Australia, 2015,
Australian Bureau of Statistics Website, viewed 4 September 2017,
<http://www.abs.gov.au/ausstats/abs@.nsf/mf/4430.0>.
Australian Bureau of Statistics 2016, Australian Demographic Statistics, June
2016, ABS website, viewed 10 September 2017,
<http://www.abs.gov.au/ausstats/abs@.nsf/featurearticlesbyCatalogue/38
4EDBC8C1EB7717CA2581470023DAD1?OpenDocument>.
Australian Human Right Commission 2008, A human rights approach for
ageing and health – respect and choice: home based and residential care
for older people (2012), footnote 3: The Treasury, Australian Government,
Australia to 2050: future challenges (2010), p. 56.
Australian Human Rights Commission 2012, Age positive: promoting positive
and diverse portrayals of older Australians, viewed 29 October, 2017,
<https://www.humanrights.gov.au/sites/default/files/FTFOlderAustralians.pdf
>.
Australian Institute of Health and Welfare (AIHW), Deaths, AIHW Website,
viewed 10 October 2017, <https://www.aihw.gov.au/reports/lifeexpectancy-death/deaths-in-australia/contents/life-expectancy>.
Barker, WW, Luis, AC, Kashuba, GA, Luis, GM, Harwood, GD, Loewenstein,
GD, et al 2002, 'Relative Frequencies of Alzheimer Disease, Lewy Body,
Vascular and Frontotemporal Dementia, and Hippocampal Sclerosis in the
State of Florida Brain Bank', Alzheimer Disease & Associated Disorders, vol.
16, no. 4, pp. 203-212.
Brooker, D & Netlibrary, I 2007, Person-centred dementia care making
services better, Jessica Kingsley Publishers, London.
Cheng, S 2017, 'Dementia Caregiver Burden: a Research Update and
Critical Analysis', Current Psychiatry Reports, vol. 19, no. 9, pp. 1-8.
Flicker, L, and Holdworth, K 2014, Aboriginal and Torres Strait Islander people
and dementia: a review of the research, viewed 1 December 2017, <
A SENSE OF SELF
31
https://www.dementia.org.au/files/Alzheimers_Australia_Numbered_Publica
tion_41.pdf>.
Galvin, JE 2015, 'Improving the clinical detection of Lewy body dementia
with the Lewy body composite risk score', Alzheimer's & Dementia:
Diagnosis, Assessment & Disease Monitoring, vol. 1, no. 3, pp. 316-324.
Galvin, JE 2017, The Lewy Body Composite Risk Score (LBCRS), viewed 17
November 2017,
<http://med.fau.edu/research/Lewy%20Body%20Composite%20Risk%20Scor
e%20Form%20and%20Instructions.pdf>.
Galvin, J, Duda, J, Kaufer, D, Lippa, C, Taylor, A & Zarit, S 2009, 'Lewy Body
Dementia: The Caregiver Experience of Clinical Care', Neurology, vol. 72,
no. 11, pp. A476-A476.
Garcia, BM, Fernandez, M, Ojea, QM, Rojas, J, Garcia, BM, Bogliotti, E, et al
2017, 'ALBA Screening Instrument (ASI): A brief screening tool for Lewy Body
Dementia', Archives of Gerontology and Geriatrics, vol. 70, pp. 67-75.
Greene, JG, Smith, R, Gardiner, M & Timbury, GC 1982, 'Measuring
behavioural disturbance of elderly demented patients in the community
and its effects on relatives: a factor analytic study', Age and ageing, vol. 11,
no. 2, pp. 121.
Hughes, J 2010, ‘End-of-life decisions and dementia’, in D Ames, D Burns & J
O’Brien (eds), Dementia, 4th edn, Arnold Health Sciences, London, p.301.
Hughes, J 2013, Models of dementia care: person-centred, palliative and
supportive, viewed 29 November 2017, <
https://www.dementia.org.au/files/NATIONAL/documents/AlzheimersAustralia-Numbered-Publication-35.pdf>.
Iranzo, A, Fernández-Arcos, A, Tolosa, E, Serradell, M, Molinuevo, JL,
Valldeoriola, F, et al. 2004, 'Neurodegenerative disorder risk in idiopathic
REM sleep behaviour disorder: study in 174 patients', PLoS ONE, vol. 9, no. 2,
pp. e89741.
Jellinger, KA 2003, 'How valid is the clinical diagnosis of Parkinson's disease in
the community?', Journal of Neurology, Neurosurgery and Psychiatry, vol.
74, no. 7, pp. 1005.
Joustra, J, Gardberg, M, Röyttä, M & Kassinen, V 2014, ‘Diagnostic accuracy
of parkinsonism syndromes by general neurologists’, Parkinsonism & Related
Disorders, vol. 20, no. 8, p. 840.
Leggett, AN, Zarit, S, Taylor, A & Galvin, JE 2011, 'Stress and Burden among
Caregivers of Patients with Lewy Body Dementia', Gerontologist, vol. 51, no.
1, pp. 76-85.
Lewy Body Dementia Association 2010, Caregiver burden in Lewy Body
dementias: challenges in obtaining diagnosis and providing daily care,
viewed 2 December 2017,
A SENSE OF SELF
32
<https://www.lbda.org/sites/default/files/caregiver-burden-in-lbd--released-7-12-2010.pdf>.
Lewy Body Dementia Association 2017, Lewy body dementia: the state of
the science, viewed online 22 August 2017,
<https://www.lbda.org/sites/default/files/2016_lbd_state_of_the_science_w
hite_paper.pdf>.
Li, S, Wing, Y, Lam, S, Zhang, J, Yu, M, Ho, C, et al 2010, 'Validation of a new
REM sleep behaviour disorder questionnaire (RBDQ-HK)', Sleep Medicine,
vol. 11, no. 1, pp. 43-48.
Mahlknecht, P, Seppi, K, Frauscher, B, Kiechl, S, Willeit, J, Stockner, H, et al
2015, 'Probable RBD and association with neurodegenerative disease
markers: A population-based study', Movement Disorders, vol. 30, no. 10,
pp. 1417-1421.
Mahowald, MW & Schenck, CH 2011, ‘REM sleep parasomnias’, in: MH
Kryger, T Roth & C Dement (eds.), Principles and Practice of Sleep Medicine,
5th edn, Elsevier-Saunders, Philadelphia, pp. 1097-1083.
Marelli, S, Rancoita, P, Giarrusso, F, Galbiati, A, Zucconi, M, Oldani, A, et al
2016, 'National validation and proposed revision of REM sleep behaviour
disorder screening questionnaire (RBDSQ)', Journal of Neurology, vol. 263,
no. 12, pp. 2470-2475.
Mckeith, I, Boeve, B, Dickson, D, Halliday, G, Taylor, -P, Weintraub, D, et al
2017, 'Diagnosis and management of dementia with Lewy bodies Fourth
consensus report of the DLB Consortium', Neurology, vol. 89, no. 1, pp. 88100.
McKeith IG, Dickson DW, Lowe J, et al. 2005, ‘Dementia with Lewy bodies:
diagnosis and management: third report of the DLB Consortium,’
Neurology, vol. 65, no 1, pp. 1863–1872.
McKeith, I, Dickson, D, Lowe, J, Emre, M, O’Brien, J, Feldman, H, et al. 2005,
Diagnosis and management of dementia with Lewy Bodies: third report of
the DLB Consortium, Neurology, vol. 65, no. 12, pp. 1863–1872.
Murray, EM, Ferman, JT, Boeve, FB, Przybelski, AS, Lesnick, GT, Liesinger, MA,
et al 2013, 'MRI and pathology of REM sleep behaviour disorder in dementia
with Lewy bodies', Neurology, vol. 81, no. 19, pp. 1681-1689.
Nelson, P, Jicha, G, Kryscio, R, Abner, E, Schmitt, F, Cooper, G, et al 2010,
'Low sensitivity in clinical diagnoses of dementia with Lewy bodies', Journal
of Neurology, vol. 257, no. 3, pp. 359-366.
Pearlin, L, Mullan, J, Semple, S & Skaff, M 1990, 'Caregiving and the Stress
Process: An Overview of Concepts and Their Measures', The Gerontologist,
vol. 30, no. 5, pp. 583-594.
Postuma, RB, Arnulf, I, Hogl, B, Iranzo, A, Miyamoto, T, Dauvilliers, Y, et al
2012, 'A single-question screen for rapid eye movement sleep behavior
A SENSE OF SELF
33
disorder: A multicenter validation study', Movement Disorders, vol. 27, no. 7,
pp. 913-916.
Qian, W, Schweizer, T, Munoz, D & Fischer, CE 2016, 'Misdiagnosis of
Alzheimer’s Disease: inconsistence between clinical diagnosis and
neuropathological confirmation’, Alzheimer's & Dementia: The Journal of
the Alzheimer's Association, vol. 12, no. 7, pp. 293.
Svendsboe, E, Terum, T, Testad, I, Aarsland, D, Ulstein, I, Corbett, A et al 2016,
'Caregiver burden in family carers of people with dementia with Lewy
bodies and Alzheimer's disease', International Journal of Geriatric Psychiatry,
vol. 31, no. 9, pp. 1075-1083.
United Nation 2017, World population prospects the 2017 revision: key
findings and advanced tables, UN Department of Economic and Social
Affairs: Population Division, New York.
World Health Organisation and Alzheimer’s Disease International 2012,
Dementia: a public health priority, WHO, Geneva.
Ulstein, I, Bruun, WT & Engedal, K 2007, 'The relative stress scale, a useful
instrument to identify various aspects of carer burden in dementia?',
International Journal of Geriatric Psychiatry, vol. 22, no. 1, pp. 61-67.
Zweig, Y & Galvin, J 2014, 'Lewy body dementia: the impact on patients
and caregivers', Alzheimer’s Research & Therapy, vol. 6, no. 2, pp. 21.
A SENSE OF SELF
34
APPENDIX A: REM SLEEP BEHAVIOUR DISORDER
SCREENING QUESTIONNAIRE
REM Sleep Behavior Disorder Screening Questionnaire
Please answer each question by checking the approriate box
NO
I sometimes have very vivid dreams
2.
My dreams often have aggressive or action-packed content
3.
The dream content mostly matches my nocturnal behavior
4.
I know that I move my arms or my legs in my sleep
5.
When this has happened, I have sometimes (almost) hurt my sleeping partner or myself
6.
I experience or have experienced the following phenomena during my dreams:
iss
io
1.
n
YES
6.1 speaking, shouting, swearing, laughing loudly
6.2 sudden limb movements, “fights”
6.3 gestures, sequences of movements that are pointless during sleep,
e.g. waving, saluting, shooing away a fly, falling out of bed
6.4 things that have fallen down around the bed, e.g. bedside lamp, book, glasses
7.
At times I'm woken up by my own movements
8.
On waking up, I can usually remember the content of my dreams well
9.
My sleep is often disturbed
10.
I have/have had a disease of the nervous system (e.g. stroke, head trauma,
Parkinson's Disease, RLS, narcolepsy, depression, epilepsy,
inflammatory disease of the brain). If so, which one?
t U Re
se vie
W wC
ith o
ou py
tP
er
m
Do
No
Copyright © 2007 International Parkinson and Movement Disorder Society (MDS). All Rights Reserved.
RBDSQ - United States/English
RBDSQ_AU1.0_eng-US.doc
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APPENDIX B: REM SLEEP BEHAVIOUR DISORDER
QUESTIONNAIRE – HONG KONG (RBDQ-HQ)
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36
APPENDIX C: ALBA SCREENING INSTRUMENT
(This document has been translated from the original Spanish)
Neurology Department
Cognitive and Behavioral
Neurology Section
Asociación Lewy Body Argentina
ALBA SCREENING INSTRUMENT (ASI)
Instructions for administration and evaluation
Clinical Questionnaire
Ask the questions of the questionnaire as they are written.
Ideally, the questionnaire must be conducted in the presence of a family caregiver.
Questions 4, 6, 7 and 8 are scored two points if the answer is yes, as long as the
information comes from the caregiver. However, if affirmative answers to these
questions are from the patients, the score must be “1”.
Physical Examination
For the assessment of both Items 12 and 13, the presence of tremor must be clear
and evident at first sight. If the patient argues that the tremor occurs due to
medication or another factor, nevertheless please assign a score of “1”.
Cognitive Examination
Every cognitive item must be scored “1” or “0” depending on an incorrect or correct
performance, respectively, for each item.
Item 14 (Category fluency): Score “1” if the patient names less than 12 animals
(e.g.11).
Item 15 (Inverse Digit Span). Score “0” points for the sequence correctly repeated.
(The correct response for this trial is 7-2-8-5).
Item 16 (Executive and visuoconstructional Skill: Clock-test). Score: One point is
allocated for each of the following three criteria:
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37
Circle (0 points): the clock face must be a circle with only minor distortion
acceptable (e.g., slight imperfection on closing the circle);
Numbers (0 points): all clock numbers must be present with no additional numbers;
numbers must be in the correct order and placed in the approximate quadrants on
the clock face; Roman numerals are acceptable but numbers cannot be placed
outside the circle contour;
Hands (0 points): The patient must draw two hands jointly, indicating the correct
time; the hour hand must be clearly shorter than the minute hand; hands must be
centred within the clock face with their junction close to the clock centre.
A point is assigned for a given element if any of the above-criteria are not met.
Item 17 (Visuoconstructional Skill: Pentagon copy) Score: To be acceptable, there
must be two figures that appear to be intersecting. At least one of the figures must
have five angles. Tremor, rotation, relative size, and symmetry are ignored.
Consider only lines that the patient has drawn (ignore any lines from the original
model that the patient may have included as part of the copy). If the patient has
drawn more than one copy, grade the best copy.
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38
ALBA SCREENING INSTRUMENT (ASI)
Assessment Protocol
Clinical Questionnaire
1. Do you have, or have you had, any nightmares in recent
times that have called your attention?
Yes - No
/1
2. Do dream images seem vivid to you? (Do they seem very
real)?
Yes - No
/1
3. Are the vivid dreams frequent?
Yes - No
/1
4. Have you ever woken up and tried searching for a dream
character or some object that you were dreaming of?
Yes - No
/2
5. Have you felt that a person close to you, or a family member
that has passed away, or somebody from your childhood is
currently in your home?
Yes - No
/1
6. Have you seen an image of any of these people, even
fleetingly, although they are not really there?
Yes - No
/2
7. Have you ever heard the patient talking or screaming during
sleep? (Care giver)
Yes - No
/2
Yes - No
/2
9. Have you experienced forgetfulness in recent times?
Yes - No
/1
10. Have you had any episodes of confusion, even during a
hospital stay?
Yes - No
/2
7(a) Do you know if you have ever spoken or screamed
while sleeping? (Patient alone)
8. Have you ever seen the patient acting out his/her dreams
while sleeping? (Punching, flailing of arms, kicking)? (Care
giver).
8(a) Do you know if you have ever acted out dreams
(Punching, flailing of arms, kicking)? (Patient alone)
/1
11. Could you say that your mental status changes from time to
time or on different days, some better and some worse?
Physical Examination
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39
12. Check for postural tremor, asking patient to stretch hands
before him/her and spread the fingers.
Yes - No
/1
13. Check for rest tremor, asking patient to put hands at rest on
lap or table.
Yes - No
/1
Cognitive Examination
Yes - No
/1
15. Inverse Digit Span. Ask the patient to repeat the following
series in reverse order: 5-8-2-7. Read at a speed of one
digit per second.
Yes - No
/1
16. Executive and Visuoconstructional Skill (Clock-test). “Draw
a circular clock. Put in all the numbers and set the time to
10 past 11.”
Circle/
Numbers/
Hands
/3
Yes - No
/1
14. Category Fluency. Say: “I want you to name me as many
animals as you can. You will have 60 seconds before I tell
you to stop. Do you have any questions?”
17. Visuoconstructional Skill (Pentagon copy). Ask the patient
to copy the interlocking pentagons:
Total Score:
/24
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40
APPENDIX D: THE LEWY BODY COMPOSITE RISK
SCORE (LBCRS)
53
Purpose of Use
The Lewy body dementias, composed of two related disorders: Dementia
with Lewy bodies (DLB) and Parkinson’s disease dementia (PDD) are a
challenge to diagnose, particularly outside of expert centers. One of the
great challenges in differential diagnosis of neurodegenerative disorders is
attributing clinical symptoms to specific pathologies to guide treatment
choices and discuss prognosis and clinical course. While PDD provides a
potentially easier route to diagnosis because the cognitive disorder begins
in face of an established movement disorder and criteria have defined a
mild cognitive impairment (MCI) state, DLB is a more difficult entity to
diagnose with delays in diagnosis approaching 18 months leading to
significant burden to patients and caregivers. Patients with DLB are often
misdiagnosed. While consensus criteria for DLB have excellent specificity
(79-100%), there is no standardized way to assess or operationalize many of
the cognitive and behavioural symptoms which markedly decreases
sensitivity in clinical practice (range 12-88%). We developed the Lewy Body
Composite Risk Score (LBCRS) to improve the ability to detect DLB and PDD
in clinic and research populations and increase the likelihood of
determining whether Lewy bodies are contributing pathology to the
cognitive diagnosis. The LBCRS was derived from clinical features in
autopsy-verified cases of healthy controls, Alzheimer’s disease (AD), DLB,
and PD with and without dementia. The LBCRS was tested in a consecutive
series of 256 patients compared with the Clinical Dementia Rating and gold
standard measures of cognition, motor symptoms, function, and behaviour.
The LBCRS increases diagnostic probability that Lewy body pathology is
contributing to the dementia syndrome and should improve clinical
detection and enrolment for clinical trials.
Administration and Scoring Guidelines
The questions are completed by a clinician after interview with patient and
caregiver and a complete physical and neurological exam. The
operationalization of physical findings as being present for at least 6 months
or symptoms occurring at least 3 times over the past 6 months permitted the
scoring of the LBCRS by totalling the sum of signs and symptoms rated as
present. In the context of a patient with cognitive impairment, the LBCRS
can increase the probability that Lewy bodies are a significant contributor
to the clinical diagnosis. The LBCRS has 10 Yes/No questions; 4 questions
Galvin, JE 2017, The Lewy Body Composite Risk Score (LBCRS), viewed 17
November 2017,
<http://med.fau.edu/research/Lewy%20Body%20Composite%20Risk%20Scor
e%20Form%20and%20Instructions.pdf>.
53
A SENSE OF SELF
41
cover motor symptoms while 6 questions cover non-motor symptoms. The
clinician rates the presence or absence of physical signs and can elicit
information from either the patient or caregiver regarding the presence or
absence of symptoms.
Interpretation of the QDRS
A screening test in itself is insufficient to diagnose a dementing disorder. The
LBC RS is, however, quite sensitive to suggesting that there is a high
probability that Lewy bodies are a contributing pathology to the underlying
cognitive decline either as a single pathology or as a mixed dementia. The
LBCRS discriminates DLB, PDD and MCI due to Lewy body disease from
other forms of cognitive impairment.
The LBCRS is scored on a continuous scale with a range of 0 - 10. Based on
receiver operator characteristic curves from 265 individuals included in the
development and validation samples, LBCRS scores differentiate with the
following cut - points:
Non-Lewy Body Case
Probable Lewy Body Case
0- 2
3 - 10
Using the cut-off of 3 or greater, the LBCRS was able to discriminate:
DLB vs. AD
Area under the
curve
Sensitivity
Specificity
Positive
Likelihood ratio
Negative
Likelihood ratio
0.94 (0.90 – 0.97)
DLB vs. any
dementia
0.94 (0.91 – 0.98)
MCI DLB vs MCI
AD
0.96 (0.91 – 1.0)
94.2
78.2
4.1
97.9
86.1
7.0
100
72.9
3.2
0.08
0.02
0.0
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42
LBCRS Permission Policy
James E. Galvin, MD, MPH grants permission to use and reproduce The Lewy
Body Composite Risk Score, also referred to as the “LBCRS”, without
modification or editing of any kind solely for (1) clinical care purposes,
defined as a physician’s use of the LBCRS for non-research patient care
services, and (2) non-commercial research, defined as investigator-initiated
clinical research that is not funded or supported, in whole or in part, by any
for-profit entity (collectively, the “Purpose”). The Purpose specifically
excludes any use, reproduction, publication, and/or distribution of the
LBCRS for any other reason or purpose, including without limitation (a) the
sale, distribution, publication, or transfer of the LBCRS for any consideration
or commercial value; (b) the creation of any derivative works of the LBCRS,
including translations thereof; (c) the use of the LBCRS as a marketing tool
for the promotion or sale of any drug; (d) incorporation of the LBCRS in an
electronic medical record application software; and/or (e) any use of the
LBCRS in connection with research or clinical trials that are supported, in
whole or in part, by any for-profit entity.
All copies of the Lewy Body Composite Risk Score (LBCRS) should include
the following notice: "Reprinted with permission. Copyright 2015. Lewy Body
Composite Risk Score: A Rapid Method to Improve the Clinical Detection of
Lewy Body Dementia is a copyrighted instrument of James E. Galvin. All
Rights Reserved." Individuals or corporations intending to use the LBCRS for
any use other than the Purpose stated above, including clinical trial or
commercial purposes, must obtain Dr. Galvin’s prior written permission. Dr.
Galvin is employed by the Charles E. Schmidt College of Medicine, Florida
Atlantic University. Please contact James E. Galvin, MD, MPH
(galvinj@health.fau.edu) for more information.
Copyright 2015: The Lewy Body Composite Risk Score is a copyrighted
instrument of James E. Galvin. All Rights Reserved.
A SENSE OF SELF
43
LEWY BODY COMPOSITE RISK SCORE
Please rate the following physical findings being present or
absent for the past 6 months and symptoms as being present
or absent for at least 3 times over the past 6 months. Does the
patient…
YES
NO
Have slowness in initiating and maintaining movement or
have frequent hesitations or pauses during movement?
Have rigidity (with or without cog-wheeling) on passive range
of motion in any of the four extremities?
Have a loss of postural stability (balance) with or without
frequent falls?
Have a tremor at rest in any of the four extremities or head?
Have excessive daytime sleepiness and/or seem drowsy and
lethargic when awake?
Have episodes of illogical thinking or incoherent, random
thoughts?
Have frequent staring spells or periods of blank looks?
Have visual hallucinations (seeing things not really there)?
Appear to act out his/her dreams (kick, punch, thrash, shout
or scream)?
Have orthostatic hypotension or other signs of autonomic
insufficiency?
TOTAL SCORE
Copyright 2015 The Lewy Body Composite Risk Score, James E. Galvin
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44
APPENDIX E: RELATIVE STRESS SCALE (RSS)
Never
Seldom
Sometimes
1. Do you ever feel you can no longer cope with the
situation
2. Do you ever feel you need a break?
3. Do you ever get depressed by the situation?
4. Has your own health suffered at all?
5. Do you worry about accidents happening too ..….?
6. Do you ever feel that there will be no end to the
problem?
7. Do you find it difficult to get away on holiday?
8. How much has your social life suffered?
9. How much has the household routine been upset?
10. Is your sleep interrupted by……?
11. Has your standard of living been reduced?
12. Do you ever feel embarrassed by……?
13. Are you at all prevented from having visitors?
14. Do you ever get cross or angry with……?
15. Do you ever feel frustrated at times with……?
Greene, JG, 1982
A SENSE OF SELF
45
Often
Always
APPENDIX F: SUPPORTIVE CARE
Hughes, J 2013
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46