Quality Dementia Care Series: Younger Onset Dementia a practical guide

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The five-chapter, 38 page online booklet produced by the Prince of Wales Medical Research Institute (POWMRI), is based on 20 years of research and is designed to help those living with or exposed to people showing early onset dementia signs as well as those confirmed to already have symptoms.

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Quality Dementia Care Series: Younger Onset Dementia a practical guide

John R Hodges 1,2
Carol Gregory 1
Colleen McKinnon 2
Wendy Kelso 2
Eneida Mioshi 1
Olivier Piguet 1,2

1. FRONTIER. Frontotemporal Dementia Research Group
Prince of Wales Medical Research Institute, Barker St, Randwick, NSW 2031

2. Cognitive Disorders Clinic
Prince of Wales Hospital, Randwick, NSW 2031


© Alzheimer’s Australia 2009


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FOREWORD


“You’re too young for dementia” is often the reaction of health professionals, family and friends of people
with dementia because society associates dementia with older adults. Advocacy on the many issues that
impact on the lives of those with younger onset dementia has become an important part of the work of
Alzheimer’s Australia in recent years.

The objective of this publication is to draw together information on younger onset dementia and provide
practical information for those newly diagnosed as well as their families and carers.

Alzheimer’s Australia is grateful to Professor John Hodges and his team at FRONTIER, the Frontotemporal
Dementia Research Group at the Prince of Wales Medical Research Institute for writing this publication
and for doing such an excellent job.

The publication refers to many resources on the Alzheimer’s Australia website. I encourage the reader who
wants to access these resources and useful links to other websites and recommended further reading to
log on to www.alzheimers.org.au/youngeronsetdementia.

The development of this publication has been made possible by funding provided by the Australian
Government through the National Dementia Support Program administered by Alzheimer’s Australia.
This program is one of a number of initiatives of the Australian Government under the Dementia Initiative
- Making Dementia a National Health Priority. I should like to thank the Minister for Ageing and the
Department for the support they have given to Alzheimer’s Australia in promoting greater awareness of
younger onset dementia.

I should also like to acknowledge the unconditional grant received from Novartis to fund the printing and
distribution of the publication.

Finally, in acknowledging Professor John Hodges and his team for this publication, I would also like to
thank the members of the Alzheimer’s Australia National Consumer Committee, Patty Hodder, and Dr
Adrienne Withall who contributed comments.

Marc Budge
President
Alzheimer’s Australia


This publication was supported by funding from the Australian Government Department of Health and Ageing under the
National Dementia Support Program, and as an unconditional grant from Novartis. The opinions expressed in this document are those of the authors and not necessarily those of the Australian Government or Novartis.


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CONTENTS

Foreword

About this publication

A. About younger onset dementia
1. What is dementia?
2. How common is younger onset dementia?
3. Understanding symptoms of dementia
4. How is younger onset dementia diagnosed?
5. What causes dementia?

B. Practical approaches to management of dementia
1. Memory and other cognitive abilities: working with strengths
2. Challenging behaviours in dementia: tips on management
3. Activities of daily living: practical tips for improving everyday life
4. Sexuality and younger onset dementia
5. Employment issues
6. Driving and the law
7. Common drug treatments

C. The caring role
1. Changes in relationships
2. Caring and personality types
3. Coping styles
4. Carers’ well-being
5. Impact on other family members

D. Planning for the future
1. Advance care planning
2. Legal issues
3. Financial issues: where to go for advice and help
4. Payments for carers
5. Practical assistance in the home
6. Residential respite and permanent residential care

Research into younger onset dementia: brain donation, the ultimate gift

Further information

Books

Alzheimer’s Australia publications


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ABOUT THIS PUBLICATION

In many areas of dementia care, difficult judgements are involved about what might be
appropriate or what would not, given the unique characteristics of the person being
cared for. This publication contains much information that will assist those with younger
onset dementia to better understand, and those providing care to gain some practical
insights about possible approaches. However, professional advice should always be
sought if there are doubts about the care provided.

This booklet is evidence-based and draws on a very extensive research base. The
following key documents may be particularly useful to readers wishing to further
consider the evidence.

Burns A, O’Brien J and Ames D, Dementia, Third Edition 2005 Hodder & Arnold

Hodges, J.R. (Editor) 2001 Early-onset Dementia: A Multidisciplinary Approach, Oxford
University Press: Oxford

Hodges, J.R. (Editor) 2007 Frontotemporal dementia syndromes, Cambridge University
Press: Cambridge

Alzheimer’s Australia, Understanding Younger Onset Dementia Quality Dementia Care
Series #4, Alzheimer’s Australia 2008

Websites and further reading listed at the rear of the booklet are also sources of
practical information and support.


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A. ABOUT YOUNGER ONSET DEMENTIA


====================


A.1. What is dementia?

Dementia is not a single disorder. It is the term
used to describe the symptoms of a number
of disorders that usually cause a progressive
decline in a person’s intellectual functioning. It is
a broad term used to describe a loss of memory,
intellect, language skills, planning ability, social
skills and what would be considered normal
emotional reactions. Not all of these areas need
to be affected to make a diagnosis of dementia.
Different types, or causes, of dementia present in
different ways. For instance, Alzheimer’s disease
typically causes memory loss while frontotemporal
dementia presents with loss of communication
abilities or alterations in social behaviour. While
most of the diseases that cause dementia are not
currently reversible, a few rare conditions which
cause younger onset dementia are curable. For
this reason, it is important that every person with
suspected dementia is thoroughly investigated.


A. 2. How common is younger onset dementia?

Dementia becomes increasingly common with
age. It is a medical condition and not simply a
normal part of ageing. As such, it is important to
remember that not all older adults get dementia.
In the population of individuals aged 80 years and
over, up to a quarter of people have dementia.
By comparison, dementia in younger adults,
defined as an onset below the age of 65, is much
rarer. Less than one percent of people under 65
years of age have dementia. Access Economics
has estimated that in Australia around 10,000
individuals have younger onset dementia1. These
figures are based on surveys undertaken in Europe
and may not apply to other populations.
___
1 Access Economics, Dementia Estimates and Projections: Australian States
and Territories. Alzheimer’s Australia February 2005.
___


A.3. Understanding symptoms of dementia

The brain is made of two halves, called the
hemispheres, which are connected by a thick
bundle of nerve fibres. The hemispheres can
be divided into four main regions or lobes. The
diagram opposite shows the left hemisphere with
the lobes highlighted in different colours: frontal
(blue), temporal (green), parietal (yellow) and
occipital (pink). Other important brain structures,
such as the hippocampus (memory storage area)
and the basal ganglia (voluntary movement area),
are located deep within the brain and are not visible
in the diagram.

Each lobe is composed of an outer layer (the grey
matter) and an inner layer (the white matter). The
grey matter is where the nerve cells, or neurons,
reside. The neurons are the source of information
within the brain. They allow us to think, reason,
learn and feel. In contrast, the white matter
consists of connecting fibres. These fibres transmit
information between neurons within or across
different brain regions.

In dementia, such as Alzheimer’s disease or
frontotemporal dementia, pathological changes
take place within the brain. These changes include
the loss of neurons, abnormal accumulation of
protein in and around the cells, and changes in
the white matter. The type and location of these
changes within the brain depend on the type of
dementia. Progression of the pathology is often
slow and insidious and, in many instances, takes
years, even decades, before changes in behaviour
and cognition become apparent.

The brain is highly organised and each lobe
supports one or several thinking abilities, such
as memory, language or emotion processing as
described in the diagram. For that reason, the brain
pathology associated with each dementia type is
associated with predictable changes in thinking
and behaviour. For example, the pathological
changes associated with Alzheimer’s disease are
most pronounced in the hippocampus, which is
located in the temporal lobe. The hippocampus
is the brain structure that is crucial to the ability
to lay down new memories and keeping track of
day-to-day events. Clinically, change in memory
functioning is the most common deficit observed
on testing and the most common complaint
reported by individuals with Alzheimer’s disease
and their carers. The table on page 3 outlines
the major cognitive and behavioural deficits that
are commonly observed in each dementia type.
The colours refer to the diagram of the brain and
indicate the location of the function within the brain.


_______
Table. Effect of younger onset dementias on brain function

Alzheimer's disease

- Word understanding: + to ++
- Memory (new learning): +++
- Emotion: variable

- Spatial relations: ++
- Numbers: variable

- Vision: variable

- Problem solving: + to ++
- Word production: + to +++
- Behaviour control: variable
- Emotion: variable

- Movement Coordination: -


Behavioural variant FTD

- Word understanding: -
- Memory (new learning): variable
- Emotion: ++ to +++

- Spatial relations: -
- Numbers: -

- Vision: -

- Problem solving: ++ to +++
- Word production: variable
- Behaviour control: + to +++
- Emotion: + to +++

- Movement Coordination: -


Semantic dementia

- Word understanding: ++ to +++
- Memory (new learning): -
- Emotion: ++ to +++

- Spatial relations: -
- Numbers: -

- Vision: -

- Problem solving: -
- Word production: -
- Behaviour control: variable
- Emotion: + to +++

- Movement Coordination: -


Progressive nonfluent aphasia

- Word understanding: -
- Memory (new learning): -
- Emotion: -

- Spatial relations: -
- Numbers: -

- Vision: -

- Problem solving: -
- Word production: ++ to +++
- Behaviour control: -
- Emotion: -

- Movement Coordination: -


Vscular dementia

- Word understanding: -
- Memory (new learning): variable
- Emotion: variable

- Spatial relations: -
- Numbers: -

- Vision: -

- Problem solving: + to +++
- Word production: -
- Behaviour control: variable
- Emotion: variable

- Movement Coordination: -


Dementia with Lewy bodies

- Word understanding: -
- Memory (new learning): variable
- Emotion: -

- Spatial relations: + to ++
- Numbers: -

- Vision: + to +++

- Problem solving: + to +++
- Word production: -
- Behaviour control: variable
- Emotion: variable

- Movement Coordination: ++ to +++

___
For each function, deficits can be: - absent, + mild, ++ moderate, +++ severe
Variable indicates that a deficit is sometimes observed but not always.
Brain functions grouped by the location of the brain that controls them
________



A. 4. How is younger onset dementia diagnosed?

Consulting a doctor to obtain a diagnosis at
an early stage is critical. A complete medical
and psychological assessment may identify a
treatable condition. A number of conditions exist
that produce symptoms similar to dementia.
These include vitamin and hormone deficiencies,
depression, overmedication, infections and brain
tumours. If the symptoms are caused by dementia,
an early diagnosis will mean early access to
support, information, and medication.

It is important that anyone with suspected dementia
has a thorough assessment by a neurologist,
geriatrician or psychiatrist with a special interest
in dementia to establish the diagnosis. Another
option is to attend a memory clinic. A referral can
be obtained from a general practitioner. A typical
work-up is likely to include the following:

• Detailed medical history. If possible, this
history ought to be provided by the person with the
symptoms and a close relative or friend. Medical
history helps establish the onset (sudden or
insidious) and progression of the symptoms (fast
or slow). Often, behavioural changes are more
apparent to family and friends than to the person
with dementia.

• Thorough physical and neurological
examination. Usually this examination includes
tests of the senses and movements to rule out
other diseases and to identify medical conditions
which may worsen confusion associated with
dementia.

• Laboratory tests. These blood and urine
tests are sometimes called a ‘dementia screen’.
They test for a variety of possible illnesses which
could be responsible for the symptoms such
as an under-active thyroid gland or vitamin B12
deficiency.

• Brain imaging. Imaging of the brain is
mandatory in all younger adults with suspected
dementia to rule out reversible causes such as a
benign brain tumour or hydrocephalus (fluid on the
brain) and to look for patterns of brain changes
associated with the specific types of younger
onset dementia, such as local shrinkage (atrophy)
of the frontal lobes in frontotemporal dementia.
Computerised Tomography (CT) and Magnetic
Resonance Imaging (MRI) are forms of structural
imaging methods that are widely available. In
some specialist centres, structural imaging may
be supplemented by functional imaging (SPECT or
PET), which show how the brain is working.

• Psychiatric assessment. This investigation
helps identify treatable disorders such as
depression, which can mimic dementia. It also
helps to identify and manage symptoms such
as anxiety or delusions which may occur in
conjunction with dementia.

• Neuropsychological assessment. Tests of
cognition are administered to identify retained
abilities and specific problems in areas such as
memory, comprehension, visual abilities, problem
solving and numerical skills. These results can help
with determining the diagnosis.

________
Figure: Brain MRI in the vertical plane
of a patient with frontotemporal
dementia (left) and a healthy
subject of similar age (right).
Compared to the healthy brain,
the brain on the left shows
marked tissue loss (atrophy)
of the frontal (A) and temporal
(B) lobes and enlargement
of the spaces in the brain
(ventricles)(C)
________



A.5. What causes dementia?

Younger onset dementia comes in many forms.
Each type of dementia has its own signature of
symptoms, signs and findings on investigation, and
is caused by a specific type of pathology in the
brain.

A breakdown of the main causes of younger onset
dementia is shown in the figure.


>> Alzheimer’s disease

Alzheimer’s disease remains the most common
form of dementia even in adults under the age
of 65 years and accounts for around 30% of all
cases of younger onset dementia. First described
by Alois Alzheimer in 1906, Alzheimer’s disease
is a progressive, degenerative illness, the basic
cause of which remains unclear (except in the
small proportion of individuals with a known genetic
mutation). The pathology of Alzheimer’s disease
involves two proteins that are usually present in
normal healthy brain cells. In Alzheimer’s disease,
these proteins accumulate in a toxic form and
cause cell death. The most important protein is
amyloid, which forms ‘plaques’ outside brain cells
(neurons) and induces an inflammatory response.
The second protein is tau which is the major
constituent of ‘tangles’ found within neurons.
Together, plaques and tangles are the hallmark of
Alzheimer’s disease. Research is currently focusing
on ways of removing the abnormal accumulations
or, at least, preventing their further build up.

In the early stages of the disease, the plaques and
tangles are confined to the hippocampus which is
part of the temporal lobe. Because of the pivotal
role of this brain region to memory, the presence
of pathology here causes problems with learning
and remembering new information and recent
events. As the disease progresses, the plaques
and tangles spread to other parts of the brain
causing more generalised deficits. In addition, the
part of the brain that produces a key chemical
called acetylcholine, which is involved with memory
and attention, is also affected early in Alzheimer’s
disease. Many of the currently available drugs
attempt to enhance levels of acetylcholine in the
brain.

Diagnosis in the very early stages can be difficult.
If the problems are confined to impaired memory
without the other signs of dementia then the label
mild cognitive impairment is applied. Research
has shown that the risk of converting to dementia
in individuals diagnosed with mild cognitive
impairment is around 10% per year.

As well as difficulty with new learning and recall of
recent events, other early symptoms are reduced
attention and difficulty with solving problems. Using
household appliances may become difficult and
tasks previously undertaken with ease (checking
the bank balance and completing tax returns)
become problematic. Many of these symptoms
are often attributed to stress and depression. A
common claim is that people with Alzheimer’s
disease are unaware of their problems but this is
incorrect. Early on, awareness can remain intact
leading to substantial mood disturbance, such
as depression or aggression. As the disease
progresses, language breakdown (particularly word
finding problems and difficulty understanding word
meaning) increases and disorientation in time and
place often develops.

Psychiatric symptoms are also frequently observed,
notably depression, irritability and anxiety. Apathy
(loss of motivation) is common and people with
dementia may withdraw from family and friends and
lose interest in pastimes. In some cases, delusions
(false beliefs) involving theft of possessions,
intruders, phantom lodgers or marital infidelity are
observed. In contrast, hallucinations (sensing or
experiencing things that do not exist) in the early
stages of Alzheimer’s disease are uncommon
and are much more suggestive of another type of
dementia: dementia with Lewy bodies.

No tests exist that can absolutely confirm a
diagnosis of Alzheimer’s disease in life. Currently,
investigations focus on ruling out other potential
causes of dementia, as well as looking for the
characteristic changes seen on neuropsychological
testing and on brain imaging. In the early stages,
routine structural brain imaging (CT or MRI) may be
normal. Functional imaging (SPECT or PET) will
often reveal changes in brain metabolism, but such
scans are not available in all centres and are quite
costly.

Compared to the situation in older adults, younger
onset Alzheimer’s disease is more often inherited,
especially when the onset is very early (40s or
50s). Up to a half of the people with younger onset
Alzheimer’s disease have another family member
with dementia, typically a single relative with late
onset dementia. Familial Alzheimer’s disease is
defined as younger onset disease if the affected
individual has two or more affected relatives with
younger-onset dementia in two generations. This
is much rarer (accounting for less than a fifth of
cases) but such people should be offered genetic
screening, after appropriate counselling, to look for
the presence of one of the known gene mutations
causing the disease. Together these mutations
account for about a half of cases with Familial
Alzheimer’s disease. It is assumed, therefore, that
there are important genes yet to be discovered.

Three different genes have been identified in which
mutations cause Familial Alzheimer’s disease. All
three gene mutations are inherited in an autosomal
dominant pattern: this mode of transmission means
that each child of a person with a known mutation
has a 50% risk of developing the disease. This is
because we all have two copies of each gene on
our chromosomes. We inherit one version of each
gene from our mothers and one version of the
same gene from our fathers. If a person inherits
the mutant gene that causes Familial Alzheimer’s
disease from one parent and a normal gene from
the other parent, the mutant gene will override
the normal gene and the person will develop the
disease.

The genes are called:
• Presenilin-1, located on chromosome 14 and is
by far the commonest cause of Familial Alzheimer’s
disease
• Presenilin-2, located on chromosome 1 and is
implicated in Familial Alzheimer’s disease in a small
group of families from an ethnic group known as
the Volga Germans, who mostly live in the USA and
Canada
• Amyloid precursor protein (APP) located on
chromosome 21 and implicated in Familial
Alzheimer’s disease in only 20 or so families in the
world.

Younger onset Alzheimer’s disease is also seen in
a high proportion of people with Down’s syndrome.
These individuals, who have an extra copy of
chromosome 21 and who experience premature
ageing, develop the same plaques and tangles in
their brains as other individuals with Alzheimer’s
disease.


>> Frontotemporal dementia (FTD)

Frontotemporal dementia, sometimes called
frontotemporal lobar degeneration, was first
described 100 years ago by Arnold Pick and was
previously referred to as Pick’s disease. It is
the second most common degenerative disease
causing dementia in younger adults. The age of
onset is typically in the 50s or 60s but can be as
young as 30.

Damage to brain cells is more localised than
in Alzheimer’s disease, and begins in the
frontal and/or temporal lobe. In FTD the clinical
presentation varies, depending on whether the
frontal or temporal lobe is affected first. When
the initial pathology affects the frontal lobes, the
main changes are in personality and behaviour.
Individuals with predominant temporal lobe
involvement present with loss of language skills
known as progressive aphasia (aphasia is the loss
of the ability to produce or understand language).

The pathology of FTD is much more complex and
variable than the pathology of Alzheimer’s disease.
Instead of the ‘plaques and tangles’ which
characterise Alzheimer’s disease, the brains of
people with FTD brains show a severe loss of brain
cells (neurons). In some individuals, the tau protein,
which is also involved in Alzheimer’s disease,
collects in neurons known as ‘Pick bodies’. A
small proportion of people with tau accumulations
have a mutation of the tau gene on chromosome
17. More commonly, the brains of people with
FTD shows an accumulation of another cell protein
– ubiquitin. Ubiquitin is involved in clearing waste
products from brain cells but for reasons that
are currently unknown, this protein builds up in
some people with FTD. Very recent research has
suggested that the accumulation of ubiquitin is
attached to another protein (called TDP-43) which
has a fundamental role in cell nuclei. Again, a small
proportion of people with ubiquitin accumulation
have a genetic mutation, this time of the progranulin
gene which is also located on chromosome 17.

In the frontal or behavioural variant of
frontotemporal dementia, the person’s mood
and behaviour may become fixed and difficult to
change, making individuals appear selfish and
unfeeling. A loss of empathy and emotional
warmth is very common. In contrast to Alzheimer’s
disease, recent memory is typically preserved.
Apathy or lack of motivation is very common,
leading people with FTD to abandon hobbies
and avoid social contact. Others lose normal
inhibitions and start talking to strangers or exhibiting
embarrassing behaviour in public. Difficulty in
reasoning, judgement, organisation and planning
is frequent, along with a reduction in spontaneous
conversation. Changes in eating patterns are
very common often with a craving for sweet food,
a tendency to overeat and a restriction in food
preferences. A decline in self-care and a reduction
in the ability to perform activities of daily living is
another early feature. As the disease progresses,
the person may become ‘obsessional’, repeating
patterns of movement and behaviours like hand-
wringing or echoing back whatever is said.

In the progressive aphasia version of the
disease, the initial symptom is usually a decline
in language abilities. This can take two different
forms. In the first form, the problem is a loss of
memory for words and impaired comprehension
of word meaning. The left temporal lobe is critical
for the fluent production of words and especially
for assigning meaning to words. Because the
language disorder reflects a breakdown in the
meaning (or semantic) system underlying language,
the term semantic dementia is used to describe
this form of FTD. Reading and spelling are also
typically affected, although numerical abilities can
be remarkably well preserved. Everyday memory
and skills associated with posterior brain regions
such as navigation, route finding and eye-hand
coordination are spared, at least until very late in
the disease. Subtle personality changes of the type
seen in the behavioural form are also common.

In the other form of progressive aphasia, known
as progressive nonfluent aphasia, the main
symptom is a difficulty communicating due to
slow and laboured production of words often with
distortion of speech and a tendency to produce the
wrong words and make grammatical errors. Using
the telephone and communicating with groups of
people is particularly difficult. Changes in behaviour
are uncommon in the early stages but do occur
later. Some people develop clumsiness of hand-
use, known as apraxia. In later stages, the disease
spreads to the frontal lobes, so that many of the
features described above, especially the changes
in organisational abilities, emotional responses
and empathy occur. There is considerable overlap
between progressive nonfluent aphasia and
corticobasal degeneration.

As with Alzheimer’s disease the diagnosis depends
on careful clinical evaluation supplemented by
neuropsychological testing and brain imaging
to look for the characteristic pattern of atrophy
(shrinkage).

Most cases of FTD are not inherited but there are
a number of families, perhaps accounting for 20%
to 30% of cases, where the disease is passed
on in the genes. In these cases there is a strong
family history of a dementia (or sometimes of Motor
Neurone Disease) usually with an early onset of
the disease. Abnormal (mutated) genes that are
found on chromosome 17 and are related to tau
protein and progranulin (which is normally involved
in growth and repair) have been found in families
around the world. It is possible to screen for these
mutations but screening is usually only offered only
to people with a clear family history of younger
onset dementia.

More detailed information on frontotemporal
dementia can be found on the website of
FRONTIER, the Frontotemporal Dementia Research
Group (www.ftdrg.org)


Motor Neurone Disease with dementia

Motor Neurone Disease (MND) - also referred to as
Amyotrophic Lateral Sclerosis (ALS) – was thought
to spare mental abilities and that FTD, in turn, did
not cause significant muscle problems. This has
been found to be incorrect. In fact, a proportion of
people with frontotemporal dementia do develop
features of MND, typically slurring of speech,
problems swallowing or weakness and wasting of
the muscles of the limbs. Conversely, a proportion
of people with MND go on to develop behavioural
and/or language problems. The degree of overlap
between MND and FTD is not yet known. A
proportion of people with the FTD-MND overlap
condition have delusions (abnormal and sometimes
bizarre beliefs that are not true) or hallucinations
(seeing or hearing things that are not there) that are
otherwise rare in FTD.


>> Parkinsonian disorders associated with dementia

Parkinson’s disease

Parkinson’s disease is a degenerative disorder
characterised by impairments in motor skills,
speech, and in some cases, cognitive functions.
The condition is named after the English physician
James Parkinson, who made a detailed description
of the disease in his historic publication: An Essay
on the Shaking Palsy (1817). Most cases have an
onset in later life but one in 20 cases are reported
to occur in people under the age of 40 years and
one in five under 65.

The typical features of Parkinson’s disease are
muscle rigidity, tremor and a slowing of physical
movement (bradykinesia). The primary symptoms
are caused by a deficiency of dopamine, which
is produced in the dopaminergic neurons of the
substantia nigra, a structure located deep within
the brain. Dopaminergic neurons are the main
source of dopamine, a neurotransmitter that has
many roles and is involved in behaviour, cognition,
movement, mood, sleep and attention. Except in a
very small proportion of inherited cases, the cause
of Parkinson’s disease remains unknown.

Not everyone with parkinsonism, a term which
refers to the syndrome of tremor, rigidity and
bradykinesia, has Parkinson’s disease. Other
causes of parkinsonism are drug side effects
(most notably the drugs used to treat psychosis)
and the so-called Parkinson-plus diseases:
progressive supranuclear palsy (PSP), corticobasal
degeneration (CBD) and dementia with Lewy
bodies (DLB). The Parkinson-plus diseases are
important because they progress more quickly than
the more common idiopathic Parkinson’s disease
and are typically associated with more marked
cognitive dysfunction and respond less well to
the dopaminergic drugs used to treat Parkinson’s
disease. Dopaminergic drugs affect the levels of
dopamine in the brain.

The pathological hallmark of Parkinson’s disease is
the presence of Lewy bodies (clumps of the protein
alpha-synuclein) found in the dying dopamine-
producing nerve cells within the substantia nigra
region of the brain stem.

In the early stages of Parkinson’s disease, cognitive
changes are mild and variable. The most common
symptoms are mental slowness (bradyphrenia),
problems with executive functions such as mental
flexibility, prioritising and goal setting; and the
correct interpretation of social cues. Dementia is a
late development occurring in approximately 20%
to 40% of people, typically starting with slowing of
thought and progressing to difficulties with abstract
thought, memory, and behavioural regulation
accompanied by visual hallucinations.

Psychiatric symptoms are common in Parkinson’s
disease. Estimates of the prevalence of
depression range from as low as 20% to as
high as 80%. A proportion of people develop
‘punding’, a behaviour in which there is an intense
fascination with repetitive handling and examining
of mechanical objects, such as taking apart
watches and radios or arranging common objects
(lining up pebbles, rocks, or other small objects).
Punding is thought to be related to dopaminergic
stimulation secondary to the commonly
prescribed medications, which help relieve
physical symptoms. Behaviours associated with
impulse control - including compulsive shopping,
hypersexuality, binge eating and pathological
gambling have also been associated with the
dopamine agonist medications used to treat
Parkinson’s disease.

Sleep disturbance is also common in Parkinson’s
disease particularly Rapid Eye Movement (REM)
sleep behaviour disorder that causes abnormal
motor activity during the REM sleep phase
characterised by rapid eye movements. There
is a loss of the muscle paralysis that is normally
present during intact REM sleep when most
dreaming occurs. People with Parkinson’s disease
may simply have limb twitches or appear to be
unconsciously acting out their dreams causing
violent movements which can result in injury to
either the person or his or her partner.


Dementia with Lewy bodies

Dementia with Lewy bodies is associated with the
build-up of a protein called alpha-synuclein inside
dying nerve cells that are distributed throughout the
cerebral cortex (the layer of grey matter covering
the brain). This build-up takes the form of abnormal
spherical structures that are called Lewy bodies.
Often, this pathology is combined with the plaques
and tangles in the brain that are also seen in
Alzheimer’s disease.

In many ways, dementia with Lewy bodies
represents a cross between Parkinson’s disease
and Alzheimer’s disease. People with this disorder
show the cognitive features of Alzheimer’s
disease combined with the movement disorder
of Parkinson’s disease. In dementia with Lewy
bodies, people tend to have particularly real or
vivid visual hallucinations, such as seeing animals
or faces, experience stiffness or shakiness of
their limbs (parkinsonism), and are prone to falls.
A particular feature of dementia with Lewy bodies
is the tendency for the condition to fluctuate
quite rapidly, often from hour to hour, or day to
day, with periods of confusion and hallucinations
alternating with periods of clear thinking. People
are often described as having distinctive good and
bad days. The degree of memory impairment is
generally less severe than in Alzheimer’s disease
but people have difficulty with attention. They also
typically perform very poorly on tests involving the
copying of shapes or understanding visual material,
and this is due to changes in their visual area of the
brain.

People with Lewy body dementia are also very
susceptible to the side effects of antipsychotic
(neuroleptic) drugs which should be avoided as
they tend to aggravate difficulties with movement.
Furthermore, the dopamine replacement therapies
used to treat Parkinson’s disease typically do
not help and usually worsen hallucinations and
confusion. There may be a good response to the
cholinergic-based drugs which are used to treat
Alzheimer’s disease.

As with Parkinson’s disease, sleep disturbance is
common with many people experiencing rapid eye
movement sleep behaviour disorder.

The distinction between dementia with Lewy
bodies and Parkinson’s disease can be difficult
to make. Some individuals who have Parkinson’s
disease do indeed develop a dementia similar to
that seen in dementia with Lewy bodies but this
typically occurs after a period of several years
during which time they have shown response to
dopaminergic treatment.


Progressive supranuclear palsy

Progressive supranuclear palsy (PSP) is a rare,
but increasingly recognised, disorder that was first
described by Steele, Richardson and Olzsewski
in 1964. It is sometimes named after them. The
pathological features resemble those found in
Alzheimer’s disease (presence of tangles) but the
distribution of pathology in the brain is different and
involves structures deep in the brain and brainstem.
The main neurotransmitter deficit is dopamine.

Clinically, PSP is similar to Parkinson’s disease,
except that the motor deficits are symmetrical in
onset, and severe rigidity is more prominent in the
neck and body rather than the limbs. Tremor is
rare. Another early feature is a marked tendency
to falls. The characteristic feature, which gives it
the name progressive supranuclear palsy, is an
inability to voluntarily move the eyes in an up-and-
down direction. This causes difficulty reading and
walking downstairs. Eventually, eye movements are
affected in all directions.

Problems with speech and swallowing are
common. People with this disorder typically
converse much less than normal and may have
slurring of speech. Cognitive impairment is present
in the majority and reflects dysfunction of the frontal
areas of the brain. Apathy is a frequent feature.


Corticobasal degeneration (CBD)

Corticobasal degeneration is a progressive
neurodegenerative disease related to
frontotemporal dementia. It is associated with
atrophy (shrinkage) of the frontal lobes, the parietal
lobes and the basal ganglia.

The disease usually begins with motor symptoms,
such as parkinsonism (rigidity, slow movements
and postural instability) or apraxia. Apraxia is an
inability to perform complex purposeful motor tasks
even though there is no weakness or sensory loss.
This is because the brain cannot send the correct
messages to the limb. In some people, the hand
may behave as if it has a will of its own (so called
‘alien limb syndrome’). Typically one side of the
body is affected more than the other. The affected
side may become increasingly stiff and useless and
may even develop involuntary jerks in response to
a variety of stimuli, such as noise and surprise.

As the disease progresses many people develop
features of the behavioural variant of frontotemporal
dementia such as apathy, loss of empathy,
disinhibition and poor decision-making. Others with
CBD may develop features of progressive nonfluent
aphasia.

The majority of people with CBD present with motor
problems. But, existing research indicates that
cognitive, emotional or language problems may
be the first signs, followed by (sometimes several
years later) the onset of parkinsonism or apraxia.
CBD progresses slowly over a number of years.
Unfortunately, no treatment currently exists for this
disease. Management requires the expertise of
speech and language and occupational therapists.


>> Vascular dementia

Vascular dementia is the broad term for dementia
associated with problems of circulation of blood
to the brain and is a relatively common form of
dementia even in younger adults. Two of the most
common forms of vascular dementia are Multi-
infarct dementia and Binswanger’s disease.

Multi-infarct dementia is caused by an
accumulation of damage from small strokes,
called mini-strokes or transient ischaemic attacks
(TIA), in the grey and white matter of the brain.
Binswanger’s disease (also known as subcortical
vascular dementia) is associated with changes to
the deep white matter of the brain due to blockage
of very small blood vessels in the brain but without
discrete stroke-like events. Both of these diseases
reflect underlying disease of the blood vessels and
usually occur in association with cardiovascular
risk factors such as smoking, hypertension, high
cholesterol or diabetes.

In younger adults, the presence of vascular
dementia may be due to a genetic disorder.
Cerebral autosomal dominant arteriopathy with
subcortical infarcts and leukoencephalopathy,
usually called CADASIL, is an inherited condition
that causes stroke and dementia. The underlying
abnormality involves the muscle cells surrounding
these blood vessels (vascular smooth muscle
cells). The resulting blood vessel damage can
cause migraines and, later in life, recurrent strokes
that progressively damage the brain, causing
dementia. Mutations in the NOTCH3 gene on
chromosome 19 cause CADASIL. The NOTCH3
gene provides instructions for producing a receptor
protein, which is important for the normal function
of vascular smooth muscle cells.

A range of unusual inflammatory and autoimmune
disorders (such as the lupus anticoagulant
syndrome) can also affect the blood vessels in
the brain, or clotting function, and lead to mini-
strokes. Autoimmune disorders are ones in which a
person’s body tissues are attacked by that person’s
own immune system. Some of these disorders
are treatable. For this reason, any younger adult
showing signs of vascular dementia should be
investigated to exclude these rare causes of stroke.

In vascular dementia, memory is usually less
affected than in Alzheimer’s disease. Problems
with organisation, motivation (apathy) and planning
are common in the early stages. Language and
communication difficulties are also frequent as
are motor problems such as difficulty walking and
controlling the bladder. Behavioural problems
can also occur, particularly if the strokes occur in
the frontal areas of the brain. Vascular dementia
typically progresses in a stepwise fashion;
deterioration in memory and reasoning abilities are
followed by periods of stability, only to give way
to further decline. Because the site of damage in
vascular dementia varies from person to person
the symptoms and signs of the dementia are also
variable.

The management of vascular dementia depends
upon the underlying cause and may involve
control of vascular risk factors such as high blood
pressure, diabetes and raised cholesterol.


>> Huntington’s disease

Huntington’s disease is an inherited brain disease.
The disorder is named after George Huntington, the
American physician who first described it in 1872.
It is caused by a mutation in a specific gene, the
Huntingtin gene, located on chromosome 4. The
diagnosis of Huntington’s disease is established by
testing for the gene abnormality. It is inherited as
an autosomal dominant disorder which means that
each child of a person with Huntington’s disease
has a 50% chance of inheriting the disease.

Physical symptoms are usually the first to be
noticed and typically appear between the ages
of 30 and 50. The most characteristic are jerky,
random, and uncontrollable movements called
chorea. People with Huntington’s disease also
show a general lack of coordination causing
an unsteady gait and slurring of speech. Eating
difficulties commonly cause weight loss and
may lead to malnutrition in the late stages of the
disease.

The cognitive features of Huntington’s disease
reflect involvement of the frontal lobes of the brain,
giving rise to problems with planning, motivation,
flexibility and abstract thinking. As the disease
progresses, memory problems tend to appear.

Psychiatric symptoms are also common but vary
far more across individuals than the cognitive and
physical symptoms. These symptoms may include
anxiety, depression, reduced display of emotions
(blunted affect), irritability and aggression, as
well as compulsive behaviour, which can cause,
or worsen addictions, including alcoholism and
gambling.

No treatment currently exists to stop the
progression of Huntington’s disease, but
medications are available that can control
movement disorders and psychiatric symptoms.


>> Alcohol related dementia and Korsakoff’s syndrome

Too much alcohol, particularly if associated with a
diet deficient in thiamine (Vitamin B1) can lead to
irreversible brain damage. Prisoners of war who
have a very poor and restricted diet, have been
observed to have similar brain damage to people
who chronically abuse alcohol.

The National Health & Medical Research Council
of Australia recommends that men and women
should drink no more than two standard drinks
daily. Development of alcohol related dementia and
Korsakoff’s syndrome has not been reported in
people drinking regularly at or below these levels.

Korsakoff’s syndrome is characterised by an
abrupt onset of severe loss of recent memory
(amnesia) accompanied by confusion and apathy,
as well as physical problems such as loss of
balance, painful or weak limbs, and disordered eye
movements. If not treated urgently, this can be fatal
or leave the person with permanent and severe
memory loss.

Whether chronic high alcohol intake can produce
a gradual and progressive dementia remains
controversial. Accumulating evidence indicates that
high alcohol consumption (over six standard drinks
per day) can cause shrinkage of the frontal lobes
producing poor initiation, planning, organisation,
motivation and problem solving. People with
alcohol-related brain damage also have a memory
disorder called ‘confabulation’ (false memories).
These people can seem very convincing when
reciting personal anecdotes and recent events.
Such people are not deliberately making things
up but rather subconsciously filling in gaps in their
memory. These changes may be partially reversible
if the person stops drinking and, as such, alcohol-
related dementia is often described as static.

For more information on the different types of
dementia, see the Alzheimer’s Australia website at
alzheimers.org.au, then select About Dementia &
Memory Loss > Types of dementia


====================


B. PRACTICAL APPROACHES TO MANAGEMENT OF DEMENTIA


====================


The following sections cover aspects of the
management of dementia emphasising aspects
that are particularly relevant to younger people.
The literature on this topic is quite extensive and
the following is meant to be an outline only with
key points and signposts to further reading. In
particular the Alzheimer’s Australia website has
many excellent help sheets (see www.alzheimers.
org.au/helpsheets) or you can call the National
Dementia Helpline on 1800 100 500.

In the first section common cognitive problems are
identified and some suggestions made on the way
to help individuals overcome them. This is followed
by sections on challenging behaviours, activities of
daily living, and sexuality. The final section raises a
number of employment issues.


B.1. Memory and other cognitive abilities: working with strengths

As part of the diagnostic process, many people
with younger onset dementia have been asked
by their medical specialist to have an assessment
by a neuropsychologist. The assessment looks
at a person’s cognitive abilities (e.g. attention,
memory, thinking, problem solving), particularly
their strengths and weaknesses. This information
is important for both carers and the medical team
as it can be used to develop strategies to assist
with cognitive and behavioural interventions which
will vary from individual to individual. It is, therefore,
important to have information about what the
person can still do well, and in what areas they
may need extra assistance. Once you know the
person’s strengths, you can use these to try to
overcome some of their weaknesses.

At the current time there is no way of restoring
lost memory and thinking capabilities. The most
effective way to manage cognitive difficulties
is by making changes to the way information
is presented to the person with younger onset
dementia and by adapting the surroundings. By
adapting the surroundings the individual may have
less need to rely on memory and thinking skills.


>> Attention and concentration

Attention refers to the ability to focus and to sustain
concentration over prolonged periods of time.
Dual-tracking, another component of attention, is
the ability to attend to more than one thing at once.
Impairments in these areas cause the following
problems:

Common problems:
• Easily distracted
• Difficulty paying attention
• Inability to ‘follow through’ with a task or
instruction.
• Inability to do more than one thing at a time
(multi-task)

Strategies:
• Remove distractions whenever possible (turn off
the TV and radio during conversations)
• Talk one-on-one rather than in a large group
• Concentrate on relevant material
• Simplify information and written instructions
• Reduce the amount of information that is
presented
• Present information in small chunks in both a
verbal and written format
• Get the person to repeat back information to
ensure he or she has understood
• Get the person to focus on one task at a time,
one step at a time
• Keep the person focused by breaking down
tasks into manageable parts
• Allow enough time – it will take longer to process
information


>> Memory

Problems with what is called ‘episodic memory’
(the ability to recall personally experienced events
from the recent or more distant past) are very
common in all the dementias but are the hallmark
of Alzheimer’s disease. Impairments in this domain
cause the following:

Common Problems:
• Forgetting phone messages/conversations
• Misplacing commonly used items
• Repeating stories
• Poor recall of events
• Forgetting appointments/meetings/medications

Strategies:
• Keep a notepad by the phone for messages
• Use an answering machine for phone messages
• Decide on a consistent place to keep handbags,
keys and wallets
• Attach important items so they cannot be mislaid
(using a neck cord for glasses, tying keys to a
belt)
• Label desk drawers and cupboards
• Avoid reminding the person that he or she has
asked the question already
• Use distraction by introducing other enjoyable
activities or conversations if the person continues
to repeat him or herself
• Try giving a different response or turning the
questions into a discussion
• When a person is trying to recall an event, assist
them by providing a meaningful context (who
was there, what the event was for, how long ago
it occurred); providing cues and prompts can aid
recall
• Try to understand why the person is repeating
the question – is it due to increasing anxiety
about an upcoming event/issue?
• Write down all appointments in a clear and
simple diary or wall-calendar. Make sure the
person checks the diary or calendar at the same
time each day (e.g. after breakfast and after
dinner)
• Only use one diary or wall-calendar; use it
consistently and encourage the person to refer
to it every day
• Set an alarm to remind the individual of things
that he or she needs to complete - if the person
is used to using a mobile phone or electronic
daily planner these are ideal tools to assist with
remembering
• Ask your local pharmacist about having tablets
placed in a Webster-pak which can help in
remembering to take tablets
• Make sure the person has his or her name,
address and contact in his or her wallet/purse
and consider an identity bracelet if disorientation
is becoming a problem.


>> Speech and Language

Language is a complex human ability which
depends upon a network of structures in the
dominant (usually left) hemisphere of the brain.

Common Problems:
• Difficulty finding words or constructing sentences
• Difficulty expressing oneself and in being
understood
• Difficulty understanding others
• Difficulty understanding the meaning of words
• Frustration because of difficulties communicating
• Reduced ability to express needs and therefore
needs not met

Strategies:
• Allow more time to communicate
• If the person is struggling to find a word in
conversation, offer assistance after allowing a
reasonable amount of time (do not jump in too
soon)
• Stand in front of the person and minimise
distractions
• Place yourself at eye level
• Avoid talking over the person with dementia
• Speak gently and clearly – do not shout
• Use statements rather than questions
• Wait for a response from the person with
dementia before continuing
• Smile and use humour
If the ability to communicate through spoken
word or written language is lost, then other
forms of communication can be used. Non-
verbal communication refers to all other forms of
communication and includes:
• Facial expressions (such as smiling and
frowning)
• Gestures (such as pointing, touching or waving)
• Eye contact (such as looking at or away from
others)
• Behaviour (such as walking away or crying)
• Volume (such as speaking loudly or softly)
• Tone of voice (such as high or low pitch)
An assessment by a speech pathologist can
be very helpful in devising strategies to enable
more effective communication for the person with
dementia. Talk to your doctor about organising an
assessment.


>> Executive functioning

The term ‘executive functioning’ refers to higher-
level thinking abilities that enable a person to
successfully engage in independent goal-directed
behaviour. These abilities are most commonly
linked to the frontal cortex and they guide complex
behaviour over time through planning, decision-
making and self-monitoring of judgments and
impulses.

The term ‘executive functioning’ originated in the
business world, where the executive monitors
all the departments so that the company runs
efficiently and is well-organised. Impairments in this
domain may cause the following:

Common Problems:
• Difficulties with planning and organisation
• Difficulty making decisions
• Poor judgment
• Impulsive behaviour (e.g. spending recklessly,
dangerous driving)
• Disinhibition (e.g. talking inappropriately to a work
colleague)
• Rigid or concrete thinking
• Inability to stop or start an action
• Inability to monitor behaviour or pick up on others
responses
• Frequent changes in mood (emotional
rollercoaster)
• Lack of remorse toward people
• Reduced drive or motivation
• Unawareness or denial that the behaviour is a
problem

Strategies:
Executive difficulties are the hardest of all cognitive
problems to manage. You may need to try a
number of approaches and review these regularly.
• Establish a consistent daily routine
• Use a diary, mobile phone and/or electronic daily
planner
• Break down tasks into manageable components
• Minimise external distractions and stressful
situations
• Allow time for decision making and ensure that
the person has access to the relevant
information and support
• If initiation is a problem, start the activity with the
person and show him or her what to do
• Guide the person through the steps of making a
decision
• If the person is still working, make sure that
there are checks in place to monitor work
performance; supervision is crucial, especially if
the person is making important decisions that
have ramifications for others
• Socially inappropriate behaviour can be
challenging and needs expert assistance.
Ask your doctor to assist you in finding help.
A good place to start is the Dementia Behaviour
Management Advisory Service (DBMAS)* or the
National Dementia Helpline 1800 100 500.
• Ask for a driving assessment for the person with
dementia to ensure that he or she is safe on
the road.

Cognitive problems can have a significant impact
on the well-being of affected individuals and their
families. Try to remember that it is the disease that
is causing these changes in thinking, personality
and behaviour. The person with dementia is not
intentionally trying to be difficult. It is very important
that both you and the person with younger onset
dementia continue to enjoy pleasurable and
relaxing activities and to maintain social contacts. It
might be helpful to take up a pursuit together or to
involve friends and family members.


For more information on changes in memory see
the Alzheimer’s Australia website at alzheimers.
org.au, then select About Dementia & Memory
Loss>Memory Loss

* The Dementia Behaviour Advisory Service is
available to care-workers, services and carers
providing care to people with dementia who are
seeking or accessing Australian Government aged
care services, where the behaviour of the person
is impacting on their care, including staff and
volunteers of Australian Government funded aged
care services, other clinicians (eg GPs, staff of
mental health services for older people), and family
carers.


B.2. Challenging behaviours in dementia : tips on management

People with dementia may develop behavioural
symptoms that may be a consequence of the
person no longer being able to meet his or her own
needs, or be a direct symptom of changed brain
function. Behavioural symptoms vary between
individuals and change over time as the illness
progresses.

If behaviour is having an impact on the person
with dementia or those around them, consider the
following:


Know what functions of the brain have been
affected:

To manage behavioural symptoms the family carer
needs information.

This includes finding out from the medical specialist
or GP what functions of the brain have been
affected by the illness. If the person with dementia
has seen a neuropsychologist he or she will also
be able to give this information.


Know the person:

It is important to know the person well. This
includes his or her personality, past experiences,
likes and dislikes, and the things which are
important to him or her. The family carer is
best placed to know this information and use
this knowledge when developing behavioural
management plans.


Ensure the person with dementia is as
physically well as possible:

When people with dementia are unwell they will
be less able to use the skills they still have. Having
check ups with the GP, providing good nutrition,
encouraging exercise and managing medications
will assist with this. Check for visual and hearing
problems and make sure the person has glasses
or hearing aids if needed. If behaviour deteriorates
rapidly see your GP as there may be a medical
cause for this (such as urinary tract infection).


Manage/examine your own behaviour:

Behavioural symptoms in people with dementia are
made worse when their family carers are stressed.
A high proportion of carers develop significant
depression which further lowers the ability to deal
with the person with dementia.


Modify the environment:

The environment has an impact on the ability
of the person with dementia to use his or her
skills. An environment which has cues for
people with memory loss and disorientation will
assist in meeting their needs more effectively
and can reduce frustration or fear. A noisy and
over-stimulating environment may interfere with
concentration and comprehension leading to more
agitation.


Reflect on incidents:

Reflecting on what is happening can be a useful
learning tool. Think about possible triggers that
have promoted the well-being of the person and
triggers that contribute to the person’s behavioural
symptoms.


Be aware of your own limitations:

Acknowledge when you respond appropriately
and when you need assistance from others. We all
have our strengths and weaknesses and you may
be able to manage some behaviours better than
others.

If you are having difficulty coping with behavioural
symptoms, especially those relating to safety, you
may find contacting a health professional useful.
Alzheimer’s Australia’s Help Sheets provide general
information on various behaviours. They can be
found on the website alzheimers.org.au


For more information on changed behaviours and
dementia see the Alzheimer’s Australia website
Help Sheets section at alzheimers.org.au, then
select Publications & Resources>Help Sheets
& Update Sheets>Changed behaviours and
dementia.

For 24 hour assistance contact the Dementia
Behaviour Management Advisory Service (DBMAS)
1800 699 799


B.3. Activities of daily living: Practical tips for improving everyday life

People with dementia typically encounter difficulties
performing activities that were once very easy.
These difficulties can be caused by lack of
motivation, planning difficulties, memory decline,
or because of language problems. It is possible,
however, to help the person to continue performing
their everyday activities. Independent of the
reasons for the decline in activity, there are three
important points to remember when trying to keep
the person active:
• Keep the person with dementia and the carers
safe. This is crucial.
• Focus on strengths
• Avoid conflict between you and the person with
dementia

Try to keep the individual involved in everyday
activities as much as possible, but if this creates
too much distress or conflict consider taking a
break, ask other family members to participate, or
as a last resort, to take over the activity.
The importance of early assessment by the local
Age Care Assessment Team (ACAT) cannot
be overemphasised; this is typically the key to
accessing local services, including occupational
therapy.


>> Using telephones and mobile phones

There are usually three distinct problems with
using the telephone: memory problems, language
difficulties, and problems caused by behavioural
change.

If there are memory problems:
• The individual does not pass on messages ask
your friends to contact you on your mobile, or
to call again later. Answering machines can also
be an alternative. Some carer partners prefer to
turn the volume down so that the calls reach
the answering machine directly.
• The person with dementia forgets how to use
the telephone: help by taking the individual
through each step of the task.
• Write important phone numbers on a large sign
placed next to the phone or store them as quick-
dial numbers.

If there are language difficulties:
• Motivate or help the person with dementia to
prepare a script to be used when making a call.
• If the person has some computer skills, using
email is usually better because it can be re-read
a few times. There is also more time to reply and
deal with any issues.

If the problems are caused by behavioural
changes:
• The telephone is constantly used for
unnecessary calls: contact your telephone
provider to set up a control system for outgoing
calls e.g. using a pin number before mobile
cal