Carers' Questions

Answers to questions raised at the Carers' Support Group meetings

Can FTD or SD be brought on by a head injury?

No. FTD and SD cannot be caused by any environmental factors, such as a head injury, a virus or exposure to chemicals. However, because head injuries generally ‘shake up’ the brain, they may accelerate the development of emerging symptoms, and difficulties that were already there become more noticeable. This often gives relatives the impression that the head injury was the cause of the disease.

Do FTD and SD turn into Alzheimer’s disease as the disease progresses?

No. Alzheimer’s disease and FTD / SD are two separate diseases, which affect different parts of the brain and so lead to different patterns of symptoms. As described above, Alzheimer’s disease affects the back part of the brain and is associated with difficulties in memory, language, and visual and spatial function. As the disease progresses, the symptoms become worse but do not change in nature because the front parts of the brain remain spared. The same is true for FTD and SD. Because the back areas of the brain are not affected in these diseases, people do not develop the same problems Alzheimer’s sufferers and have no difficulty with their vision or finding their way around their environment.

Why don’t treatments for Alzheimer’s disease work in SD and FTD?

The brain produces a range of chemical substances (called “neurotransmitters”) to transport messages from one part of it to another. If the amount of these chemicals is reduced, then the areas of the brain that are dependent on them slow down and do not work as efficiently. Although this does not explain the whole problem, we know that people with Alzheimer’s disease have reduced levels of a neurotransmitter called ‘acetylcholine’, and that this contributes to their symptoms. Treatment for Alzheimer’s disease slows down the progression of symptoms by boosting the levels of acetylcholine in the brain. However, people with FTD and SD have normal levels of this substance in their brain and so do not benefit from treatment for Alzheimer’s disease. We now know that FTD and SD are associated with deficiency of a different neurotransmitter, called serotonin. There is some evidence that boosting levels of serotonin in some people can help with certain disruptive, repetitive behaviours, however the disease process appears to be more complicated than this and such treatments are by no means miracle cures.

Can sufferers develop both FTD and SD?

Although the two diseases are related, most people’s symptoms stay the same and remain well circumscribed. Occasionally, patients may develop certain features characteristic of the other disease. For example, FTD sufferers may develop mild difficulties in understanding word meaning or SD patients may become a little apathetic in their behaviour as the disease progresses. However, these changes are relatively mild and there is never a complete crossover between diseases. That is, FTD does not change into SD and vice versa.

Are there any factors which are known to trigger FTD or SD?

No, there are no known environmental triggers which can be avoided. The only cause that is currently recognised is familial inheritance, but this is only relevant in a small number of cases.

Are FTD and SD inherited diseases?

In the majority of cases, the disease occurs sporadically, that is, without any history of a previous similar illness in the family. However, occasionally, it may occur in more than one member of a family, suggesting a genetic predisposition in some people. Families who have a genetic predisposition towards FTD or SD are generally already aware that the illness runs in the family, because they know of other relatives who have developed similar symptoms. It should be emphasized that these cases are very rare and should not be regarded as the norm.

Are there any physical symptoms in FTD and SD?

People with FTD and SD are generally fit and healthy and do not have any physical symptoms. Many of our patients continue to exercise and pursue active hobbies for many years. In the late stages, people may slow down and show symptoms akin to a mild form of Parkinson’s disease (“parkinsonism”). They slow down physically and may develop problems in balance and slight trembling of the limbs.

Do the conditions shorten a person’s life span? Is dementia something you die with or die from?

Inevitably, a decline in brain functioning leads not only to mental, but also to physical decline. As the physical decline progresses towards the end of the disease course, patients become more immobile, and are increasingly prone to infection. It is these physical changes, rather than the mental decline per se which eventually cause the sufferer to die. The fact that these changes are inevitable means that a diagnosis of ‘dementia’ does indicate a shortening of lifespan. The progression of the disease varies widely between individuals, but the estimated prognosis is approximately 10 years.

Can language problems be helped?

In FTD, lack of communication is due to a person’s gradual loss of social awareness rather than to any true difficulty with language. Because of progressive damage to the frontal lobes, patients have difficulty seeing a situation from someone else’s point of view and so do not respond to other people’s conversation. In these cases, stimulating someone by talking to them a lot may not be of much help, because the sufferer will not understand the point of it. In SD, The sufferer’s difficulty is in understanding the meaning of individual words. However, it can often help understanding if words are put into a context: speaking in complete sentences is better than communicating in short phrases or by using single words. As we saw above, patients generally retain understanding of things that are personally relevant to them. Using words and expressions that the sufferer himself/herself uses in everyday life will help their understanding in conversation.

Is sense of smell affected in SD?

Yes. It’s not only the meaning of words and objects that is affected in SD. Sufferers also have difficulty recognising everyday sounds, tastes and smells. Although their sensory abilities are well preserved (i.e. they can distinguish between two different sounds or smells), they have difficulty ascribing a meaningful identity to what they perceive and so may be unable to recognise familiar sounds, such as the ringing of the telephone or doorbell, or identify the smell of cooking as a sign that food is being prepared.

Are sufferers aware of pain and temperature?

An altered reaction to pain and touch is frequently observed in FTD and SD. Certain sufferers react in an exaggerated manner to non-threatening stimuli, such as a light touch on the shoulder, and yet may show a reduced response to pain and not even flinch when they have visibly injured themselves. Similarly, they often seem to be less aware of extremes of temperature and may step into a scalding bath without realising they have burned themselves.

Can the sufferer alter his/her behaviour?

Although it may sometimes feel like sufferers are acting in a deliberately awkward or selfish way, their behaviour is the result of their brain disease, and is not under their control. Patients are no longer able to see things from someone else’s point of view, so are unaware of the effect of their behaviour on others.

Do all FTD sufferers ultimately become disinhibited?

No. Although there are two main patterns of behaviour in FTD, apathetic behaviour and disinhibited behaviour, all patients eventually become more apathetic as the disease progresses. People with apathetic behaviour do not become disinhibited.

My husband has frontotemporal dementia and seems to have a rather ‘selective’ memory. Is this part of the illness?

It is understandably frustrating to live with and care for someone who seems only to remember things that are important to them, and to forget things which are important to you. This type of ‘selective memory’ does occur as a result of the illness, and is not so much a problem in memory as it is a problem in behaviour and attention. As you will know, one of the symptoms of frontotemporal dementia in particular is that people can become more self-centred, and less interested in matters that do not specifically relate to them. They lose insight into others’ wishes and feelings. Also, there can be problems in concentration and attention to information. All of these things can affect the way that information is ‘encoded’ and processed. You will know from personal experience that if you are not interested in something, you are less likely to take it in and remember it. This is exactly what is happening here, but on a much broader scale.

Will routines improve motivation?

People with frontotemporal dementia and semantic dementia do typically respond well to routines. There is a tendency for reduced flexibility in thinking processes, and as part of this, patients seem to prefer rigid and structured schedules.

My wife has semantic dementia, and has great problems in understanding simple words. Despite this, she can type letters to copy without any difficulty. Why?

This type of observation is common. Semantic dementia results in a very specific problem in understanding, with relative preservation of other linguistic skills. Patients can often repeat, write, and read words, without understanding what they mean. Furthermore, spatial and motor abilities remain intact, so there is no reason why a person should be unable to continue to perform basic skills of typing, particularly if this was a skill that was well practised before the onset of the illness.

My husband, who has semantic dementia, has started to swear on a regular basis. He has always been a mild mannered man, and never used to use bad language. Is there any reason for this change?

There is no ‘scientific’ explanation for this, but interestingly, it is a common phenomenon in other neurological disorders, for example in patients who have had a stroke. In semantic dementia, there are a number of possible explanations:

  • A loss of understanding of the significance of the words
  • Social disinhibition
  • Reduced vocabulary – using the first word that comes to mind

My husband complains that he is tired all of the time, and would stay in bed all day if he could. Is this normal?

This is quite common in these types of dementias. It is likely that reports of ‘tiredness’ actually reflect apathy and lack of motivation. Unfortunately, this is something that is rather difficult to overcome. A loss of interest in familiar hobbies is a common feature, and relatives often find that suggesting activities that were previously enjoyable is unsuccessful. Carers use different methods to try and motivate, depending on the wants and needs of the individual. For example: * Visiting relatives – sometimes interaction with others (e.g. grandchildren) can help * Being involved in different activities. When people come to the clinic to be tested, they often engage in neuropsychological testing without question. Relatives can be surprised by this, as it is a stark contrast to what their behaviour is like at home. If possible, try to become involved in some simple, novel activities outside of the home – there may be a difference in motivation in different environments.