Frontotemporal dementia/degeneration

Frontotemporal degeneration (FTD – also known as frontotemporal dementia) has become more widely recognised and understood in recent years, particularly in its relationship to MND or ALS.
Researchers are recognising an important connection between FTD and MND/ALS.
I am going to make an attempt to explain this in everyday language, but as a medical condition there is all this medical jargon that surrounds it. Hopefully I can help you wade through it and come to some clearer understanding.
The way I’ve decided it may be best to approach this is to use coloured text (I’m a visual person so I like colours to distinguish things).

So I’m going to put medical descriptions in blue italics.

Then I’m going to put my everyday explanations in green. I have tried to keep most of the text green, but sometimes it helps to know some of the medical terms surrounding what is happening.

Finally I’m going to put examples of how these things happened in my own experience of caring for my husband in orange.

Is it dementia or degeneration?

Often it is now called Frontotemporal degeneration rather than dementia to distinguish it from being confused with Alzheimer’s disease (AD). Most people generally think of Alzheimer symptoms and behaviours as soon as they hear the word dementia. FTD and AD do have some similarities, but at the same time they are incredibly different.

FTD is a syndrome of progressive changes in behaviour and language due to loss of function of neurones in the frontal and temporal lobes of the brain. Many researchers now say that up to 50 per cent of people with MND/ALS will display some degree of FTD. This means some degree of change in behaviour and/or the ability to make complex decisions.
FTD has an insidious onset, typically presenting between fifty and sixty. There is relative preservation of memory, but impairment of behaviour, language and/or personality.

FTD starts in small ways, just little things you begin to notice and over time you start to realise that something is happening to the person you love. Usually it is changes in the behaviour or the personality of the person. Not everyone who experiences FTD have the same degree of changes, some are just slight and seem to happen over a long time, others are radical changes that happen fast.
A hallmark characteristic is that the person affected lacks insight into their problems. The immense importance of this statement is that, as far as the patient is concerned, they are not showing any change of behaviour, and their perception is completely valid so there is no problem. When a carer or health professional tries to talk to them about their behaviour, they are met with a brick wall, complete indifference or an escalation of paranoid anger.

Around one year before Chris began to have trouble speaking and swallowing, I began to notice personality changes. I thought he seemed mildly depressed but he asserted that he was not. He had always loved working with people and food. He would come home tired, but full of funny stories about his day. He was always pulling faces and talkig in an animated way, finding the good, funny and positive in everything. He began to come home from his cafe telling more stories of what had annoyed him in his day. He just wanted to sit and do very little once he was home and had his complaint session. He did still tell some funny stories, but over a period of months I realised that there was a real change happening in his attitude towards many things. As the months went by, if I said anything about it he would become very angry and accuse me of expecting to only share the good stuff. He did not realise that there was a change in him. I had always marvelled that he always saw the good and positive, and I saw this wonderful trait in him slowly dissolving.

Early symptoms

Most commonly the initial symptoms may include changes in behaviour, difficulty with expression of language and impaired comprehension.

These early symptoms can be subtle and it can take a little time before the partner or other close friends or family realise that something in the person has actually changed.
The people closest to the person affected begin to notice that they are just not behaving the same way and they notice ‘patterns of behaviour’ emerging with these changes.

Not every case is the same. There is a list of possible symptoms and they often occur in clusters.

This means that one person with FTD may display a group of typical symptoms and the next person may display a different group. It appears that one main reason for this lays in exactly which part of the frontal or temporal lobe is being affected.
It is very important to have known the person well before symptoms began in order to assess if there has been a change in the persons personality/behaviour. For example a person who has always been described as stubborn is not exhibiting FTD just because they are being stubborn now.
The real key is to know how the person would typically respond to situations and then notice that they are responding differently or ‘out of character’. This is not noticing they have a bad day and behave differently on the odd occasion, but that they progress to new patterns of behaviour. Because FTD is a progressive condition another key is to notice that the behavioural changes will become worse over time. For example irritation will progress to angry outbursts and on to rage. Another hallmark is that the person will rarely be able to reflect with you and admit they were in the wrong or see the need to apologise.

An example may be that the person was always far more concerned with the views and comfort of all other family members, but now they disregard everyone else and even berate them for their own views or needs.

Apathy is often the first symptom reported by caregivers and may be mistaken for depression. People experiencing these changes may become self-centered, emotionally distant, withdrawn, unaware of the emotions of others, avoid social contact or neglect previous hobbies and interests. They may develop a lack of concern for their personal appearance and become increasingly unkempt early in the course of disease.

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Common symptoms/signs of FTD

Poor judgment
Lack of insight
Loss of empathy
Paranoia
Socially inappropriate behaviour
Lack of inhibition
Repetitive compulsive behaviour
Inability to concentrate or plan
Frequent, abrupt mood changes
Easily frustrated
Quick to anger
Speech difficulties
Problems with balance or movement
Memory loss

Executive dysfunction

In what is known as ‘executive dysfunction’ problems arise with planning, organising, abstracting and prioritising, along with impaired verbal fluency. Most researchers agree that bulbar onset more often results in MND/ALS-FTD compared with those with MND/ALS alone. It can be difficult to assess the problems with verbal fluency as the person is also experiencing speech issues associated with the loss of the function of their tongue. It is interesting however, that FTD is more likely to occur in bulbar onset people.

Bulbar onset is when MND/ALS begins with speech and swallowing issues as the nerve to the tongue begins to fail. Most people with MND/ALS go on to develop bulbar problems as the disease progresses. Most people with bulbar onset go on to lose the use of their arms and legs as the disease progresses.

Organising is a key word here. This is talking about how the person is able to pay attention to detail and then plan something. Can the person still look at a problem and work out what order things should be done in and what the priorities are? Once they work this out can they still put this plan into action and stick to it? Can they make good decisions based on the details they have been given?

My Chris had run café’s for many years and his life revolved around food and making people happy. He just loved planning meals and interacting with people, taking a real interest in them and seeing them leave feeling good about themselves.
He constantly said that he was going to ‘live with the disease, rather than die from it’, and showed everyone a very positive attitude.
Maintaining weight is one of the key factors in living well and living longer with MND/ALS and has been medically proven.
He totally lost the ability to organize, plan and execute a good diet for himself from the very early stages. He wanted to be in control of this, but he could not actually choose good foods and take in 3 meals a day. He would talk about what he was going to do, the kinds of meals and smoothies he was going to make and how he was going to eat all these calories. Dietiticians would discuss the need for a very high calorie diet and he knew of many ways he could add calories to meals he already makes. Yet he could no longer put a meal plan together, let alone actually create the food, nor take the food in. This was happening right from when he could still swallow and could still use his hands and arms and was doing most of the nightly cooking.

No matter who tried to talk with him about his steady weight loss and the impact of this, he was adamant that his weight loss was to be expected and nothing could be done to change it.

This has to be compared to the person that he was before the disease started. He believed solidly in the importance of eating well, and enjoying to eat. His life revolved around constantly thinking about, planning, preparing and serving food. As time went on and he lost the ability to eat more than a little pureed food and was dependent on being fed through a PEG tube, he would simply refuse feeds. This was despite doctors and dieticians weighing him regularly and talking to him about the connection between his poor eating and weight loss. In the end he began to blame his weight loss on me, saying I wasn’t giving him enough. In reality he would refuse to allow me to give him anywhere near enough.

Survival in patients with MND/ALS-FTD is generally shorter compared with those with MND/ALS alone, especially when the executive functions are highly impaired. One of the main reasons for this is the lack of insight and poor judgment. This often results in the person making very poor decisions on how to maintain supportive therapies such as nutrition, hydration and safe swallowing or early PEG placement. Another very common and distressing result of this can be a total lack of insight into the need for safety. People with FTD involvement frequently have a high rate of falls that result in serious injury and at times death as a result.

When safety was discussed with Chris he would state that he was going to die anyway so it made no difference whether he died of the disease or by a head injury.
He did in fact have two serious falls resulting in ruptured tendons in the shoulder on one occasion and a head injury and broken bones on another occasion. He continued to insist he could remain mobile unassisted and that safety was not an issue because it was up to him how he died. He showed no sign of understanding the impact the injuries had on the rest of his life, both in hastening the loss of limbs after injury, and the amount of pain he suffered as a result of injuires that did not completely heal, nor the impact they had on me as his wife who watched him suffer.

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Diagnosing FTD

No single diagnostic test exists to confirm or rule out a diagnosis of FTD. This often results in a long and frustrating process of testing for other disorders with symptoms similar to FTD to rule them out.
In recent years, advances in diagnostic technology have led to some tools that physicians can use to determine a diagnosis of FTD with more confidence. Neuropsychological testing and neuroimaging (MRI, SPECT and PET scans) have shown that there are specific features or clinical findings consistent with FTD that can be identified and helpful in the diagnostic work-up.

This means that testing can be complicated and if we are dealing with someone with MND/ALS, they already have a terminal illness to come to terms with. When a lot of anger and paranoia are involved testing can become extremely traumatic and cause many problems between the person and those that love them.
On top of this, the person does not realise their behaviour and personality have changed and so it becomes very difficult for them to undergo a psychological examination as they find the questions confronting. Add to this the physical limitations they are experiencing as the MND/ALS progresses and the entire process becomes muddied.
A caregiver questionnaire avoids many of these limitations posed by the patient’s physical disabilities and lack of insight. Caregivers can better identify behavioural changes. To ask a patient a question like ‘Do you find x more difficult now than you used to?’ is fairly meaningless. The patient will either answer no or answer yes but state it is because of a physical limitation of the disease. Caregivers can answer a variety of questions about change in behaviour, mood and attitude that can quantify the level of behavioural change.

Changes in the medical professions attitude to MND/ALS and behaviour

Interestingly, in the past, before FTD was widely recognised, the following list was commonly reported in MND/ALS patients:

withdrawn due to depression
stubbornness
seeking control in some area of life
anger outbursts due to frustration
denial
language problems due to dysarthria

The symptoms on this list were considered to be understandable and normal, rather than symptoms of further involvement of the brain in the disease process and degeneration.
For carers, the disinterest by medical practitioners and dismissal of FTD symptoms has been a major issue. They were left unsupported and not understanding why they were being subjected to so many behaviours that had never been a part of the person they knew before the disease.

For myself, even though I came to understand FTD and what was happening to Chris, it was extremely difficult not to take the lack of empathy, paranoia, anger and lack of insight into the whole situation to heart and feel like a total failure as his wife and carer. How anyone caring for a person with MND/ALS- FTD, without understanding this aspect of the disease, comes out of the situation without severe scarring is beyond me.

When executive functions are affected, the person begins to think more slowly than they used to. So health professionals find themselves constantly saying to the person at each visit, ‘We are worried about you falling’, ‘We are worried about you choking’, and the patient has a lot of difficulty understanding the impact of this. When the family is brought in on this they say, ‘My loved one just doesn’t seem to be the same person any more and he doesn’t seem to understand. He does not want to listen to me and does not want to do anything that is being suggested as the best for his health and quality of life.’

For me as a carer, the very worst part was that I could not talk to friends, most health professionals or family about the FTD, as my view was immediately discounted as me just not understanding what an awful thing Chris was going through. Time and again the items on that list were thrown at me until I stopped talking about this side of the horror at all.

Many carers find it incredibly difficult to even discuss the behavioural changes they are witnessing and experiencing in their loved one as they feel guilty that they are speaking ill of a person who has a terminal illness. They find themselves explaining away the behaviours. Worse can be that others who are not so close to the situation can add more guilt on top by explaining the behaviours as a normal response to such a terrible situation. Often a person with FTD has enough control of themselves that they can appear to others as still quite highly functioning for the duration of a visit, and can present none of the behaviours the partner or primary carer is experiencing. This often makes it hard for a carer to stand up and say anything. One of the responses from health professionals or other people that distressed me the most was to be told “Oh when someone is ill they always take it out on the person closest to them, it’s perfectly normal”.

Chris was the most amazing man. I loved him completely, and all the emotional horror we lived through was because this disease was degenerating the frontotemporal area of his brain.
Memory is not always affected by FTD. I never had the blessing of him forgetting any of the terrible situations that developed, or forgetting his attitude towards any of them. He always held to his paranoia and each time he went into a rage about some perceived action I was taking against him only convinced him further that his paranoia was justified. For this reason, many of his symptoms increased as he built on them and convinced himself I did not care about him as a person, that I wanted him to die, and that my problems in trying to cope were insignificant.

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Language variants

Generally it is understood that people who start with behavioural variant progress to develop language problems. Some people begin with the language problems but progress to displaying the behavioural and executive function symptoms over time.
Language issues usually begin with slow hesitant speech and difficulty with grammar. They may begin to leave out joining words in sentences like ‘an, to, the, from’.
They show difficulty in following the flow of a longer complex sentence spoken to them.
As the disease progresses they begin to forget words for things that were common to them.
They will find it difficult to give an appropriate response and so may say yes when they mean no.

When this is combined with bulbar speech issues of MND/ALS communication becomes incredibly difficult as using speech technology or even simple charts becomes very difficult as they lose the ability to spell, remember words, and put sentence structure together. The person often becomes very withdrawn as their ability to use language to communicate lessens. Eventually, even the ability to communicate simply on their most basic needs becomes difficult for them to articulate using just a few simple words on a chart.

Chris progressed into the language issues during his last few months of life. Communication was only possible using charts with words and/or pictures. He would often nod his head whilst saying no, or shake his head whilst saying yes. He could not speak well enough to make a noise that could be distinguished as 'yes' or 'no' so the head movement became critical. He could not grasp the concept of using one sound for yes and a double sound for no. Much of the time I was left guessing his answer even to a closed question. He would say no to a range of questions I would use to follow through a path to what he wanted. When all questions had been answered no, I would have to start again as the problem was that he could not follow the conversation well enough to give the appropriate answer. He also did not realise it was happening - he would accuse that I was the one confused.

References used to create this page and recommended further reading:

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If you suspect FTD involvement I would recommend you start taking note of behaviour changes and record it in a diary fashion. This will help you to work out if there are patterns to the behaviour and give you something you can use to discuss with your health care team. Usually it is best to discuss this without the affected person being present as it is very distressing for them to think you even suspect there is something wrong with the way their brain is functioning. This way you can decide with your doctor what approach you are comfortable with. This approach may involve a formal diagnosis which will mean the affected person will be aware of what is happening.

Go on to caring for someone with FTD

This website has been designed to support CALS - people who are caring for someone with MND/ ALS.
Katrina Jeffery cared for her husband Chris through MND/ALS with FTD and provides these supports from first hand experience.
Copyright 2015. All resources that have been used or quoted provide direct links to the source.