Rush Manual 1. What is Dementia?

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UNDERSTANDING DEMENTIA

Many people are afraid to discuss changes they may be noticing
in either their own or someone else's behavior. There can be
confusion about whether such changes are due to normal aging
or due to the onset of a disease. This chapter describes common
symptoms of dementia; the evaluation that determines the cause
and diagnosis; and explains different types of dementia.


What does dementia mean?

Dementia refers to a gradual decline in memory and other
cognitive functions. It is a broad term describing a widespread
problem in brain function. The symptoms of dementia interfere
with an individual's daily activities. These symptoms can
include, but are not limited to:
* Forgetting events, names, places.
* Repeating questions.
* Difficulty finding words or putting thoughts in conversation.
* Problems managing money or balancing a checkbook.
* Getting lost in familiar places.
* Trouble doing work or routine tasks.
* Personality changes.

There are many causes of dementia, Alzheimer's disease
being the most common. It is important to look for the cause of
dementia, so a diagnosis can be made and appropriate treatment
started.


Is dementia a normal part of aging?

Contrary to popular thinking, dementia is not a normal part
of aging. Most people over age 60 never show signs of cognitive
decline. Although the likelihood of dementia increases with age,
dementia affects only a minority of older people. It is true that as
a person gets older, it takes longer to learn something new.
However, once an older person has learned something new, it
should be remembered as well as when the person was young.
When there is a pattern of failing memory, it is considered
abnormal and should be evaluated as a medical problem


How is a diagnosis made?

Determining the cause of dementia requires a thorough
evaluation. The evaluation may include blood tests and a brain
scan. If some tests have been done previously, it may not be
necessary to repeat them. The various components of a compre-
hensive evaluation are described below.

- The history

Getting a complete medical history is the first step in the
evaluation. This entails an interview with the individual and
their spouse, relatives or close friends. Interview questions are
designed to determine the onset of any cognitive or behavioral
changes. It is important to know if there is a history of head
injury, loss of consciousness, seizure, stroke, diabetes, thyroid
disease or alcoholism. In addition, information about the
person's education, work, and family history will be collected.
Because medications can sometimes cause side effects such as
confusion, names and dosages of all prescription and over-the-
counter drugs will also be gathered.

- Neurological examination

The purpose of a thorough neurological examination is to
look for any physical findings that may account for cognitive
and/or behavioral changes. Illnesses such as stroke, tumor, or
Parkinson's disease may cause brain changes that mimic
Alzheimer's disease. Brain functions that are typically tested
include: memory, language, perception, orientation and motor
skills.

- Laboratory tests

At the discretion of the clinician, certain laboratory tests
and brain scans may be indicated. These may include blood work
such as a thyroid function test, vitamin B12 level, and RPR (a
test for syphilis). Scans of the brain, such as a CT, MRI or
SPECT may also be ordered.

- Neuropsychological testing

Mental status is typically evaluated by a battery of standard-
ized tests. A commonly used screening tool is the Mini Mental
State Exam (MMSE), which involves a brief set of questions
involving memory, orientation, perception, attention and
language. The MMSE is scored between 0-30. The following
categories provide a guideline for determining severity of cogni-
tive decline.

- MMSE: Score Stage

27-30: Normal
19-26: Early stage or mild impairment
11-18: Middle stage or moderate impairment
0-10: Late stage or severe impairment

Although the MMSE serves as a very useful screening tool
and helps to monitor progression of cognitive decline, it has
several limitations. For example, the MMSE score may be
affected by education, language or culture. In addition, the
MMSE is not sensitive to very early memory loss or very late
stage disease.

- Periodic evaluations

It is usually a good idea for someone with dementia to be re-
evaluated at least once a year or whenever significant changes
are noticed. These periodic evaluations will help to monitor the
effect of treatment and the progression of the disease.

- Genetic factors

Two types of Alzheimer's disease exist: early onset and late
onset. In early onset Alzheimer's disease, symptoms first appear
before age 60. Some early onset disease runs in families and often
involves inherited gene mutations that are believed to be the
cause of the disease. Early onset disease is rare, accounting for
only 5-10 percent of cases. In these rare instances of a strong
family history, genetic testing may be ordered.

Late onset Alzheimer's disease, the most common form of
the disease, is thought to be less likely to run in families. At the
present time, genetic testing is not a routine part of a dementia
work-up. However, the role of genetics in the occurrence of late
onset disease continues to be an area of study for many
researchers.

- Putting it all together

Although there is no definitive test for Alzheimer's disease,
it is estimated that the diagnosis is 90 percent accurate by ruling
out treatable or reversible causes of cognitive decline. Important
illnesses to rule out include: depression, infection, thyroid
disorder, stroke and hydrocephalus. The only definitive test for
Alzheimer's disease is microscopic examination of brain tissue
at autopsy.

___
There are many possible
causes of dementia and
many illnesses that can
mimic Alzheimer's disease.
Therefore, the physician's
main task is to conduct a
thorough examination and
sort out the medical facts.
___


Types of Dementia

- Alzheimer's disease

Alzheimer's disease (pronounced Alts'-hi-merz), was first
described by Dr. Alois Alzheimer in 1906. This disease accounts
for approximately 70% of all dementia. Despite ongoing research
efforts, the cause of Alzheimer's disease is still unknown.
Suspected causes include a genetic predisposition, a slow virus,
environmental toxins and inflammatory or immunologic
changes. At the present time, there is no effective cure for
Alzheimer's disease, although existing treatments aim to slow the
progression of cognitive decline.

Typically, a person with the disease will show gradual and
progressive decline in intellectual functions. Symptoms include
short-term memory loss and impairment of language, judgment,
orientation and other intellectual capacities. These symptoms
usually worsen over a period of many years, causing a person to
forget recent events or familiar tasks. Communication becomes
difficult as the affected person struggles to find words, finish
thoughts or follow directions. The disease may last 10 to 20 years
with an average length of illness lasting approximately eight
years from the time of diagnosis. However, the rate of progression
can vary greatly from person to person. The disease eventually
causes confusion, personality and behavior changes, and
impaired judgment. Ultimately, the person with Alzheimer's
disease becomes dependent on others for care.

The prevalence of dementia increases with age. One in 10
persons over age 65 and nearly half of those over age 85 have
Alzheimer's disease. Most people with Alzheimer's disease are
over age 65 but some individuals are diagnosed in their 40s and
50s. About 4.5 million people in the United States are now
believed to have this disease. It is estimated that 14 million
Americans will develop Alzheimer's disease by the year 2050.
Caregivers are affected by this disease too. In a national survey,
19 million Americans said they have a family member with
Alzheimer's disease, and 37 million said they knew someone with
the disease. In the meantime, research efforts continue to focus
on ways to delay the onset and slow the progression of this
illness.


Related dementias

Other less common causes of dementia include: Dementia
with Lewy Bodies, vascular dementia, Parkinson's disease, fronto-
temporal dementias, and Creutzfeldt-Jakob disease. Dementia
with Lewy Bodies accounts for approximately 20% of dementia
and presents clinically as a combination of dementia and
Parkinsonian symptoms. Parkinsonian symptoms include slowed
movements and muscle rigidity. Other symptoms of this disease
may include hallucinations, difficulty walking, falls, sleep distur-
bances and fluctuating alertness. Visual-perceptual problems are
also common and may lead to difficulty driving. Due to the
complexity of symptoms, this disease may be challenging to diag-
nose and can be confused with Parkinson's disease. In fact,
medications to treat Parkinson's disease may worsen the
symptoms of Dementia with Lewy Bodies.

Vascular dementia accounts for approximately 10% of
dementia. This diagnosis is reserved for cases where cognitive
decline occurs subsequent to a stroke. Symptoms of stroke may
include brief episodes of confusion, slurred speech, weakness and
visual changes. These symptoms usually appear suddenly. Unlike
Alzheimer's disease, vascular dementia may not cause progressive
cognitive decline unless further strokes occur. Therefore, stroke
prevention efforts such as controlling high blood pressure,
cholesterol management and smoking cessation are very impor-
tant. People may be instructed to take anti-clotting medications
such as aspirin and to follow a heart healthy diet.

Parkinson's disease can also cause dementia. Approximately
20-30% of people with a primary diagnosis of Parkinson's disease
develop dementia as the disease progresses. Drugs used to treat
the motor difficulties associated with Parkinson's disease will not
improve the memory symptoms.

Fronto-Temporal dementias (FTD) such as Pick's disease
and Primary Progressive Aphasia are rare and are believed to
account for only a small percentage of all dementia cases.
Fronto-Temporal dementia presents with personality and behav-
ioral changes before the onset of memory loss. This form of
dementia typically occurs in mid-life rather than at an older age.
Fronto-Temporal symptoms may include impulsive and/or disin-
hibited behaviors, loss of social awareness, poor judgement, loss
of interest in grooming and language problems. Depression is also
common in Fronto-Temporal dementia. Occasionally, this
disease may be misdiagnosed as a psychiatric illness.

Primary Progressive Aphasia affects speech and language
and begins with word-finding problems rather than memory loss.
This form of dementia is extremely rare and can begin as early as
age 40. People with Primary Progressive Aphasia are very aware
of their deficits and can usually continue to live independently
for many years. Although memory remains relatively intact, help
with paying bills, writing checks and following directions may be
needed as as a result of language deficits. Memory, personality
and visual processing are not affected until late in the illness.

Creutzfeldt-Jakob disease is a rare form of dementia that has
a sudden onset and is rapidly progressive. It is marked by rapid
cognitive decline and involuntary muscle jerking that may look
like a seizure. Unlike Alzheimer's disease, which can progress
over a period of 10-20 years, the duration of Creutzfeldt-Jakob
disease is one year or less. This disease typically affects individu-
als age 50-75.

Of note, it is common for Alzheimer's disease to coexist
with any of the above mentioned dementias.


___
As answers are found to the
mysteries of Alzheimer's
disease, progress also will
be made in clarifying the
causes and potential treat-
ments of these related
disorders.
___


THE STAGES OF ALZHEIMER'S DISEASE

Families often ask which stage of Alzheimer's disease their rela-
tive is in. Although we can identify and describe stages of the
disease, it is important to remember that the disease develops
differently in each person. Some people may appear to change
very little over a period of many years, while others show a rapid
decline within just a few years. The reasons for these differences
are not yet understood.

Although each person progresses at a different rate, a
description of general patterns of dementia may help determine
suitable activities and resources.


___
Symptoms get worse over
time, but they vary greatly
from person to person.
Some people may appear
to change very little over a
period of many years,
while others show a rapid
decline within just a few
years. The reasons for
these differences are not
yet understood.
___


Early stage/mild impairment

* Impairment of recent memory is usually the hallmark of the
disease in this stage. People with early-stage dementia often
write down reminders as a means of maintaining their inde-
pendence as much as possible.
* The person may have difficulty performing calculations, inte-
grating a lot of information, following multiple steps or
directions and making complex decisions.
* The person may be unable to perform certain household
tasks or job responsibilities. Decision-making, handling
multiple duties and operating machinery may be too difficult
to do well or safely.
* Problems develop in handling finances such as preparation of
taxes, payment of bills or maintaining a checkbook.
* Although driving skills may become impaired, each case of
driving safety needs to be assessed on an individual basis.
* The person with dementia may feel more comfortable in
smaller groups and with well-known family and friends in
familiar surroundings. The individual may be aware of their
impaired memory and fearful of embarrassing themselves in
social situations.

Although dementia imposes limitations, the person with
early-stage symptoms is still able to enjoy many normal
activities. Family members should emphasize the person's
remaining abilities instead of focusing on the impairments.


Middle stage/moderate impairment

* The person often repeats questions.
* Difficulty with written and spoken language becomes more
prominent. For example, trouble finding the right word in
conversation may increase.
* Understanding concepts such as time and other abstractions
may become more difficult.
* The person becomes unable to manage personal grooming
and hygiene. At this point, you may need to take a more
active role in assisting the individual to initiate and follow
through on certain tasks such as bathing and brushing teeth.
* Getting lost or disoriented in familiar surroundings may
occur.
* Losing things or hiding things may become problematic.
* In this stage, frustration or agitation may arise around certain
situations. For example, when the person misplaces items
such as keys, others may be blamed for "taking" the objects.
Poor memory and misperceptions about the environment
may lead to agitated behavior.


Late stage/severe impairment

* This stage is characterized by the inability to remember even
the most basic things. Short-term and long-term memory are
profoundly impaired.
* Language may be reduced to a few basic words.
* Incontinence (first of urine and then of bowel) usually
occurs.
* The person ultimately becomes totally dependent and must
be assisted with all tasks such as bathing, dressing and eating.
* Communication with the person must be carried out in very
simple terms using short sentences, commands and gestures.
* Mobility may become unsteady or may be lost altogether.
Even in this late stage, a person with dementia is able to
respond to emotions of happiness, anger and love. Although the
words you use to express these emotions may not always be
understood, you can communicate how you feel by nonverbal
means such as touch and gestures.



TREATMENT OF ALZHEIMER'S DISEASE

Until recently, nothing seemed to slow the cognitive decline of
people with Alzheimer's disease. However, as researchers have
learned more about the development of the disease, several
promising treatments have been identified. Five drugs for the
treament of Alzheimer's disease have been approved by the U.S.
Food and Drug Administration (FDA) and many more are in
development. Researchers are now optimistic that new
treatments will be found to delay the onset of the disease and
slow the mental deterioration it causes. Until new treatments are
developed, learning how to cope with the effects of the disease
will remain a major challenge for family and professional
caregivers.


___
...as researchers have
learned more about the
development of the
disease, several promising
treatments have been
identified...

...researchers are now
cautiously optimistic that
new treatments will be
found to delay the onset
of the disease and slow
the mental deterioration
it causes.
___


Acetylcholinesterase inhibitors

Cognitive impairment (memory loss, difficulty concentrat-
ing or difficulty using the correct words when speaking) is the
main feature of dementia and the target of current drugs specifi-
cally designed for Alzheimer's disease. We know that low levels
of a brain chemical known as acetylcholine is associated with the
disease. Since acetylcholine is involved in memory and
attention, drugs that increase the availability of this chemical in
the brain are the focus of this type of treatment.

Thus far, four drugs in this class have received approval by
the FDA for the treatment of Alzheimer's disease: tacrine
(Cognex) and donepezil (Aricept), rivastigmine (Exelon) and
galantamine (Reminyl). All of these drugs are known as acetyl-
cholinesterase inhibitors. In other words, they inhibit the
breakdown of acetylcholine so more of this chemical remains in
the brain. Cognex was approved first but is no longer used
because of its side-effects. Donepezil (Aricept) was approved in
1997 and once-a-day dosing makes it convenient to administer.
Rivastigmine (Exelon) was approved in 2000 and galantamine
(Reminyl) in 2001. Both of these drugs require twice-a-day
dosing. In general, these medications are well tolerated but can
cause side-effects such as diarrhea, nausea and weight loss. The
potential benefits of these medications include a perceived
improvement in memory, an increased ability to perform activi-
ties of daily living and reduced behavioral problems.

Although this type of medication may help stabilize cogni-
tive impairment for an unknown period of time, they do nothing
to alter the pathological progression of the disease. Further
research is needed to determine the long-term effects of these
medications.


NMDA - Receptor Antagonists

A new class of drug that prevents the over accumulation of
glutamate in the brain was approved by the FDA in October
2003. Memantine (Namenda) is the first drug in this new class of
medication to become available. It is indicated for moderate to
severe Alzheimer's disease. It may be used alone or in combina-
tion with a cholinesterase inhibitor. This medication requires
twice a day dosing and is generally well tolerated with very few
reported side-effects. Possible side-effects to watch for include
dizziness, headache and constipation.


Antioxidant therapy

Several lines of evidence indicate that oxidative stress plays
a role in aging and, in particular, in neurodegenerative diseases
such as Alzheimer's disease. Vitamin E belongs to a class of
chemicals called antioxidants. These chemicals seem to protect
cells from damage caused by oxygen and oxygen-breakdown
products. A study of vitamin E given to people with Alzheimer's
disease showed some benefits. The study participants who
received a daily dose of 2000 IU of vitamin E fared better than a
comparison group on several measures.

Ginkgo biloba has long been thought to enhance cognition
and improve memory but lacks any evidence-based research to
support its use. Although the mechanism of action remains
uncertain, there is some evidence to support an antioxidant
effect. Like all other medications, the decision to take ginkgo
biloba should be discussed openly with one's health care
provider. Ginkgo biloba can potentially interfere with other
medicines and cause bleeding.


Anti-inflammatory agents

There is evidence to support that people who use non-
steroidal anti-inflammatory agents (NSAIDs) for many years
have a lower risk of developing Alzheimer's disease. However,
preliminary findings from NSAID studies suggest that NSAID
therapy does not significantly benefit a person once dementia
has occurred. Rather, further studies aim to define the role of
NSAIDS in the prevention of disease.


Hormone-replacement

There is controversey regarding whether or not there is a
reduced risk for Alzheimer's in postmenopausal women treated
with estrogen-replacement therapy compared with those who do
not receive hormone therapy. Presently, postmenopausal women
should not take estrogen solely on the basis that it may reduce
Alzheimer Risk. No studies support the use of estrogen solely for
the treatment of dementia once the person has the disease.
Ongoing studies continue to explore the possible role of estrogen
in the prevention of dementia.


Other potential treatments

At the present time, researchers are actively studying the
role of cholesterol lowering drugs, high dose B complex vitamins
and other alternative treatments for dementia. In addition, the
role of a potential vaccine and the role of nerve growth factor is
also being examined.


PARTICIPATING IN RESEARCH

Since Dr. Alzheimer first described the characteristics of the
disease that today bears his name, we've struggled with its
mysteries. Despite many scientific advances over the past decade,
the cause and cure of the disease continues to elude us. The key
to solving the puzzle lies in the research activities now being
conducted throughout the world. The U.S. government, largely
due to advocacy from family members, has greatly increased
funding for Alzheimer's research. The federal government now
funds 29 Alzheimer's disease centers, including the Rush
Alzheimer's Disease Center.


___
Despite many scientific
advances over the past
decade, the cause and
cure of the disease
continues to elude us.
The key to solving the
puzzle lies in the research
activities now being
conducted throughout
the world.
___


Research supports the building of knowledge and enables
more and more pieces of the puzzle to be gathered. Once enough
pieces are in place, it becomes easier to complete the picture.
Alzheimer's research includes a variety of areas:
* Studying older adults who do not have dementia to compare
normal aging with disease-related changes.
* Identifying risk factors, including the role of genetics.
* Detecting means of early diagnosis.
* Testing experimental medications that might prevent or slow
the disease process or alleviate symptoms.
* Identifying why there is such great variability among people
in regard to disease progression.
* Gathering information about the behavioral changes that
may occur in the course of the disease.
* Exploring techniques that reduce the stress of caregiving.
* Examining the brain at autopsy for insights into the origins
and progression of the disease.

Research studies offering the potential of direct, personal
benefits are very appealing. For example, those taking part in
trials of experimental drugs hope to be among the first to use an
effective drug. Also, studies designed to help caregivers improve
their coping skills may have immediate practical value. However,
most research is focused on more basic questions concerning the
nature of the disease. These studies may not offer any immediate
benefits but may eventually lead to knowledge that has applica-
tions for others in the future.

Individuals agree to participate in research for several
reasons. The key reason is that they want to help find the miss-
ing pieces of the puzzle. Many participants realize the picture will
not be complete in their lifetime but perhaps future generations
will benefit. Often participants say, "This may not help me
personally, but it may prevent my children and grandchildren
from facing this disease."

Without human participation in research, it will take longer
to understand this disease. The hope for the future lies in
people's generous agreement to participate in research studies.
Most people are eager to accept this challenge in one way or
another.

Participation in research may vary from an interview on just
one occasion to a long-range commitment over a period of many
years. All studies are strictly controlled and only people meeting
certain criteria may be eligible to participate. There are usually
numerous opportunities to participate in research so you will
need to determine how much time and energy you can devote to
the effort.

All research follows strict ethical guidelines. Individuals
must be informed of each study's goals, procedures and potential
risks. Participation is always voluntary and an individual can
withdraw at any time. Information obtained during the study and
the participant's name are always kept confidential.

The ultimate goal of Alzheimer's research is to provide
benefits to those with the disease and their families. It may take
a long time to finally discover effective treatments or a cure. All
of us involved in research, evaluation and treatment are
interested in working with you to help solve this puzzle. Your
participation in this combined effort is welcomed and greatly
appreciated.