Contents
Strikingly Different:
frontotemporal dementia.
Carers, patients, nursing and care
staff and even doctors often ask the question "What does that mean?"
when a diagnosis of frontotemporal dementia is mentioned.
The following are descriptions of two different patients who developed
frontotemporal dementia, each affected in quite different ways. Understanding
the very unusual presenting symptoms of this group of diseases can help them to
be recognised and diagnosed more effectively. The types of symptoms are quite
different to those seen in Alzheimer's disease, and types of support and
interventions that should be offered to both patients and carers will be
different.
Frank and Mary
Mary and her two daughters had struggled for seven years with her husband
Frank's changing behaviour before, as a family, they had decided that something
was medically wrong and had gone to seek advice from their family doctor. Frank
has worked for most of his life as an engineer, achieving managerial promotion
in his early fifties. When he was 57, Mary began to feel that something was
wrong at his work. Frank began to work very long hours, increasingly isolating
himself from the rest of the family; he appeared to be pre-occupied or under
stress. At weekends Frank would spend most of his time in his study and grew
much less inclined to take part in conversations.
The frontotemporal dementias
frequently have a very slow and insidious onset. The first symptoms are
sometimes a subtle change in personality and behaviour. The affected person may
withdraw socially, begin to neglect their personal appearance and behave out of
character. Mary began to worry that her husband was having some form of nervous
breakdown or mid-life crisis. However, if she tried to talk to him about the
changes that she could plainly see he would deny that there was anything wrong,
and said he felt fine. Other subtle changes continued to appear in his behaviour
over the next few months. Frank had always been a relatively tidy and punctual
person, but now this began to take the form of
an obsession; clothes would have to be put away in a certain order, meals
had to be served at particular times and Frank would often spend hours arranging
and ordering ornaments on the shelves in the sitting room. He also developed a
deepening interest in mystical religions; something he had always had a passing
interest in now also began to become a pre-occupation, with Mary increasingly
feeling that he was taking his beliefs much too seriously.
Obsessional symptoms are quite
common. People affected with these illnesses may become more rigid; they may
take to collecting odd items, and may become preoccupied with religious or
spiritual activities. This changing behaviour can be frightening and difficult
to understand for the carer. Along with these odd changes in behaviour, Frank
changed from being a kind and placid person to someone who appeared to be
constantly on the edge of irritability. He rarely became angry, but it seemed to
Mary and their daughters that whenever they spoke to him he seemed about to
become angry for no apparent reason.
One Sunday Mary's sister and
brother-in-law came for lunch. During the meal Frank commented on how fat Mary's
sister had become, yet seemed quite unaware of how insulting he had been. He
finished eating long before the rest of the family and got up from the table to
go to his study without any comment. At this point Mary realised that something
was seriously wrong, but did not know how to get help.
The sort of changes Frank was
displaying are typical of a frontal type of frontotemporal dementia. The frontal
lobes of the brain are relatively poorly understood in terms of the functions
they perform. Damage to the frontal lobes, either from brain tumours, trauma,
strokes or, as in Frank's case, dementia, change the affected person's
personality and behaviour.
Crisis point
The major crisis came a couple of weeks later. Frank came home early on a
Thursday and told Mary that he had finished work and didn't need to go back. He
didn't give any more details, and simply went to his study. Mary called Frank's
boss who told her that Frank had been dismissed, firstly for being apparently
inefficient and unable to do his job, but mainly because he had been
aggressively insulting to a senior secretary in the building.
Frank still insisted that there
was nothing wrong, but Mary managed to persuade him to go to the GP with her.
When she told the story, their GP suggested that Frank should see a
psychiatrist. Carers of people with this type of dementia often describe a
crisis as the turning point when they finally decide that something is medically
wrong, and seek the help of a doctor. This can be a very difficult decision to
make. Many patients with fronto-temporal dementia have absolutely no
awareness that anything is wrong, and may become very aggressive if
challenged about their symptoms. It was very sad that Frank's sacking from work
was the catalyst for seeing a doctor. If a medical problem had been recognised
earlier, Frank might have been able to have a period of sick leave ending in
retirement on medical grounds. This would have given him and Mary better pension
rights and wouldn't have ended his long career in such a sudden and unhappy way.
A few weeks later the
psychiatrist visited Frank at home. Talking with Mary after his assessment, he
felt that Frank's behaviour was very odd and didn't fit the pattern for
depression or any other psychiatric illness. He also felt that Frank's speech
and language was not normal, and suggested that as a first step he would arrange
a brain scan. The scan showed that the front part of Frank's brain had shrunk
and withered, while the rest appeared quite normal. It was then that the
psychiatrist mentioned fronto-temporal dementia.
Patients who present with
behavioural symptoms like Frank's are often referred initially to psychiatrists.
Fortunately Frank's psychiatrist was aware of the possibility of a dementia that
could cause these symptoms and arranged the right investigations. In some cases
patients can be treated for long periods with antidepressants or other
medications when an
incorrect psychiatric diagnosis is made. This can seriously add to the
stress levels in the family, particularly as medication is unlikely to improve
the symptoms.
Increasing disability
Over the next few years Frank became increasingly disabled. Initially his
obsessional behaviour became worse, and if meals were not served on time, or his
daily routine was broken, he would become anxious and agitated. At the same
time, he spoke less and less, until five or six years later he was entirely
mute. Throughout this time, despite these striking changes he remained
independent, taking the dog for a walk every day without getting lost, washing,
dressing and feeding himself, and sitting in his study apparently reading his
books. Up to this point Mary had needed little practical help, though she
received a great deal of moral support by being in contact with other members of
the Pick's Disease Support Group. These types of disease tend to progress quite
slowly. Carers are often desperate for accurate and useful information about
these rare diseases.
Carers, friends and relatives
often find the illness hard to understand; how someone can be so changed in
their behaviour, or have a diagnosis of dementia, and yet be able to follow a
relatively normal pattern of everyday life. In particular, fronto-temporal
dementia rarely results in disorientation, and affected people can often go out
for long walks without ever getting lost; quite unlike someone in even the early
stages of Alzheimer's disease. The Pick's Disease Support Group offers specific
advice on focal and unusual dementias, and can also put carers in touch with one
another to share experience and gain mutual support.
More support needed
Over the next three or four years Frank and Mary needed increasing practical
support, including day centre care and respite admission to give Mary a break.
Frank began to choke on his food at mealtimes and was less and less mobile. His
appetite was also reduced, and he began to suffer increasingly frequent chest
infections. At the end he passed away in his sleep from pneumonia.
Through her contact with the
Pick's Disease Support Group, Mary had agreed that Frank's brain tissue could be
used for research after his death. A few months later her GP got in touch with
her to tell her that analysis of the brain tissue had shown that Frank had been
suffering from a frontal lobe degeneration. As time goes on these types of
disease become more global, and towards the end the symptoms may resemble other
dementias. As with all forms of dementia, the definitive diagnosis can only be
made after death.
Frontotemporal dementia is a
diagnosis that describes that pattern in which the brain is affected by the
disease; causing atrophy of the frontal and/or temporal lobes. The other lobes
of the brain, the parietal and occipital lobes, are usually unaffected early in
the disease. A number of different pathological changes in the brain can result
in these strikingly focal
causes of atrophy; the two main causes are non-specific frontal lobe
degeneration, where nerve cells appear simply to die, and Pick's disease where
swollen nerve cells (Pick cells) and accumulations of protein inside cells (Pick
bodies) can be seen. The causes of these diseases are still poorly understood,
which is why it was so helpful that Mary offered Frank's brain for research
after he had died.
John and Brenda
Brenda was a seamstress before she married John. Once married she settled
down to raising a family, and latterly became involved in many activities in her
local community. One of her great joys was singing in the choir, and as well as
being a member of her local church choir she had joined the county choral
society. She was also a keen gardener, and it was while she was out in her
garden that she realised something was not right. As she walked around the
garden with a friend she found that she was unable to recall the Latin names of
the flowers she had previously known well. Brenda was quite aware of her
difficulty with words, but initially said nothing, as she felt it might just be
a sign of getting older.
This is a very different picture
from that presented by Frank. There was no change in Brenda's personality; in
her case it was her language that was affected. The other difference was that
Brenda was immediately aware that she was having problems. Unfortunately the
problems began to spread, and recalling the names of more everyday and mundane
objects began to become a struggle for Brenda. She had no problems with everyday
conversation, but whenever the need to name an object came up she would stumble
and become stuck. Brenda became more and more frustrated and decided to see her
doctor.
People affected with this type of
the disease see their doctors much earlier on average; they recognise that they
have problems and want to seek help. When Brenda and John described the problem
to their GP, her first suspicion was that Brenda might have had a stroke, and
referred her to the local neurologist. A sudden loss of speech or language can
be the sign of a stroke, but the fact that Brenda's language problems developed
slowly made it very unlikely that a stroke was to blame. However, the decision
to refer to a neurologist was the right one.
The neurologist arranged
for Brenda to have neuropsychological testing and an MRI brain scan. The
psychology tests confirmed a focal language impairment, suggesting a problem
with the left side of the brain. The scan showed that the left temporal lobe
part of Brenda's brain was severely shrunken. With the results of the tests in
front of him, the neurologist
told Brenda that she probably had a frontotemporal dementia, and that it
could be Pick's disease. As with Frank, the pattern of Brenda's problems and the
pattern of brain atrophy seen on the MRI scan provided the diagnosis of
frontotemporal dementia. In Brenda's case the pattern was that the disease was
affecting only the left temporal lobe. This is quite commonly seen in Pick's
disease. Brenda was acutely aware of her difficulty with speaking,
and this made her begin to avoid social situations. She also became
increasingly depressed and would often say that she wished she were dead.
Patients with dementia who are
aware of their problems are much more likely to become depressed. It is vitally
important to recognise these type of symptoms, as treatment with antidepressants
is usually very effective. Patients with frontotemporal dementia seem to be
particularly predisposed to depression, and may sometimes carry through their
thoughts of suicide. Fortunately Brenda's GP recognised her depression and
started her on an antidepressant which worked within a few weeks.
Language deteriorating
As time passed Brenda's language deteriorated further so that she was unable
to name any objects and was often unable to understand what words meant any
longer. John might ask her if she would like something, such as a cup of coffee,
to which Brenda would reply "Coffee? What do you mean by
coffee?"
Brenda was able to read and
repeat words, but her ability to know what words meant, and what the names of
objects were, was severely damaged. She was suffering from what a psychologist
would describe as a semantic dementia (semantics refers to word meanings). The
dementia had damaged the connections in Brenda's brain that allowed her to know
what the things she was seeing were called, and the meanings of the words she
was hearing and reading.
Even when Brenda's language was
severely impaired she was still able to sing in the choir; her ability to read
and sing were not affected at all. Similarly she continued to cook and shop. She
would know what she wanted to make, and could choose the items she needed to buy
in the supermarket. However, she would not be able to say what she was trying to
make, nor give the names of the items she was buying. With only isolated
language symptoms, Brenda was able to continue a relatively normal, though more
limited life. Encouraging her to continue with activities that she was still
able to do maintained her self esteem and independence.
Over the next seven to eight
years other changes began to occur. Brenda became more impulsive and impatient.
John had to become more involved in everyday tasks as Brenda's frustrations
would often end up with temper tantrums.
Even in cases like this where damage is focused in one part of the brain,
the disease eventually spreads more widely. It often spreads first to to the
frontal lobes, and some of the personality and behaviour changes that Frank
experienced in his illness, begin to appear.
Different presentations
These two case histories have illustrated two strikingly different
presentations of the same disease. The reason for the differences in these
patterns of symptoms is that the disease started in a different area of the
brain in each case. Being alert to personality, language and behaviour changes
can help to identify these diseases.
References
The Lund and Manchester Groups (1994) Clinical and neuropathological
criteria for frontotemporal dementia. Journal of Neurology, Neurosurgery &
Psychiatry 57 416-418.
Dr. Richard Harvey MD MRCP Psych.
Reproduced with the permission of the Journal of
Dementia Care Vol 6 October 1998. (Tel. 0171 498 3023).
The
Young Person's Day
On the 24th August, my family and I
met with Penelope, Carol and another family to discuss the difficulties of
living with a father with dementia. This was very helpful as Penelope
explained what was happening to my father's brain and we all discussed the
things he did and many were identical.
We then had a barbecue which was
great fun. After the other family had left, Michelle (my sister) and I,
had a mini session with Carol, whilst my mother spoke to Penelope.
All in all it helped a lot
especially speaking to other children who actually understood. Only the people
who are suffering, or who have suffered, fully understand. My father has
been ill for a long time even though he was only recently diagnosed. It
leaves a great effect on a child and this certainly helped.
Thank you Penelope and Carol.
John Ramsey
New Web Site.
The University College of London has
been very generous in allowing us to publish the PDSG information and
Newsletters on their Web Site but they are reorganising and it is time for us to
have our own web site on the World Wide Web. For the moment our
newsletters will appear on both sites.
http://www.pdsg.org.uk/
and
http://dementia.ion.ucl.ac.uk/candid/pdsg/
We are deeply indebted to John
Jennings who designed our web pages and who looks after it for us.
Penelope’s Gossip
Corner.
Many carers have told me about the “doesn’t
he look well” syndrome as well as the “he looks all right to me, there can’t
be anything wrong” syndrome. Both these cause a lot of worry and
embarrassment for carers. Pick’s sufferers sometimes look very much
better than when they were well because they often have much less worry, after
all many of them can’t see any problems. When asked they invariably say
they are “fine thank you” which doesn’t help. The problems which arise for
carers include trying to explain why their spouse isn’t working when
he/she look so well, trying to explain to close friends and family what
the problems are, as well as trying to explain to irate neighbours why the
annoyances caused are due to the illness. This is not helped by the very
good social front which sufferers can put on and indeed keep going for days at a
time. This facade often delays getting a diagnosis or referral as the problems
may be so well hidden. Family and friends often say that they can see nothing
wrong. The fact that many would rather deny there is a problem which they cannot
face is an additional factor. It also means that help and support from those who
could give it to those who most need it is not forthcoming. I have met the
“doesn’t she look well syndrome” slightly myself. Sometimes
people I haven’t seen recently ask me what I am doing and say how well I
look. I feel rather an idiot saying I have retired on health grounds: pain
is another hidden problem.
The booklets are now available from
Carol Jennings and we would be grateful if carers could send an A5 envelope
(same size used for the PDSG newsletter) with a 39p stamp. The booklets
contains material taken from earlier newsletters. We are sending them to
new members and hope that they find them useful. We charge £1 per copy
when more than one booklet is requested. We are very grateful to the British
Lions in Leicester who sponsored this and to Peter Davis at Phoenix Photo Litho
plc also in Leicester who produced and printed the booklet at cost price.
I hope that Christmas is a
pleasurable time for all of you and do remember to take lots of photographs.
Penelope Roques
PDSG 1999
Plans for the PDSG include a
travelling study day, in the first instance to be run mostly by Carol Jennings
and myself (Advert). The proceeds after expenses to be given to the PDSG.
We have already got our first booking! Talking of fund raising I have been
asked if we accept donations and we certainly do. Cheques should be made
payable to the Pick’s Disease Support Group and sent either to myself, David
Hunter or Carol Jennings. However I do ask you to try and get support from
others rather than sending money yourselves. We are aware how very
expensive caring is.
Meetings 1999
Meetings in London:
The Old Boardroom, National Hospital for Neurology and Neurosurgery, Queen
Square London WC1N 3BG. From 11.30 - 1.30 invited speaker, 1.30 - 2.30
lunch in the Swan Pub, 2.30 - 4.30 Your own experiences.
January 21st Genetics
Explained: Dr. John Janssen & Penelope Roques
June 17th Carers Needs:
Carol Jennings
September 9th Providing Day Care
Facilities for FLD: Hazel Templeton
December 9th The Experience
of Dementia: Clare Morris
Meetings in Liverpool
will be held in the Lounge of
the Glaxo Neurological Centre, Norton Street, Liverpool starting at 2.30pm.
January 14th Carers Needs
Carol Jennings
June 10th Genetics Explained
Penelope Roques
September 9th Local Support and
Services Fiona Johnson
December 9th Research
TBA
AGM March 10th in
Nottingham
The seminar is free and a lunch
will be provided for which there will be charge.
Please contact Carol Jennings for further information and reservations.
9.30-10.30 AGM
10.30-11.00 Coffee
11.00-12.00 Unusual Dementias - Penelope Roques
12.00-1300 Carers’ Needs - Carol Jennings
13.00-14.00 Lunch
14.00-1500 Recent Research - Professor Jim Lowe
15.00-15.30 Organising Residential Care - Ann Johnson
15.30-16.00 The Experience of a Carer - Wendy Wells
16.00 Adjourn to bar!
Lille 16th April, 1999.
We will be having a day trip to Lille to meet with the Frontotemporal Group
and to explore Lille. We will be travelling by Eurostar. Anyone and
everyone welcome. We plan to have a ratio of two carers to every sufferer.
Some people have expressed an interest in staying over in Lille for the
weekend. If you would like to know more please contact Mrs. Lynn
Goodfellow on 0181 788 7861.
Kew Picknick 19th May 1999
Kew Gardens
We will be meeting by the Main Gates at 12.00. Come prepared with
umbrellas, raincoats, lots of food and drink. For late comers we will be
picnicking as close to the lake as possible so come and find us.
Young Persons Day 26th August,
1999.
Carol and I were very pleased to introduce two families to each other this
year so they could exchange thoughts on the problems of coping with a parent who
has a young onset dementia and how it was affecting their lives. (See article by
John Ramsay BBQ chef to the PDSG). If any families would like to exchange
experiences this year could they please let me or Carol Jennings know and we
would be very pleased to organise another day.
Penelope Roques
Contact Details
Carol Jennings
8 Brooksby Close
Oadby
Leicester
LE2 5AB
0116 211 0416
carol@pdsg.org.uk |
Penelope Roques
71 Myrtleside Close
Northwood
Middlesex
HA6 2XH
01923 822 700
frontotemp@AOL.com |
David Hunter
16 Grove Road
Upholland
Lancashire
WN8 0LM
01695 624 781
113234.2572@CompuServe.com |
Clare Morris
The Dementia Research Group
The National Hospital for Neurology and Neurosurgery
8-11 Queen Square
London
WC1N 3BG
Telephone: 0171 829 8772
Facsmile: 0171 209 0182
candid@dementia.ion.ucl.ac.uk |
PLEASE NOTE if you are receiving the newsletter by snail mail and
can access it through the WWW please could you let us know your e-mail
address which would save us postage.
If you have written an article, case study, or anecdote on any aspect of
caring, or have any comments on the news letter, please send it to Penelope at
the above address.
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