Information for patients and carers
What is Frontotemporal dementia with motor neurone disease (FTD/MND)?
FTD-MND is a disorder of behaviour, cognition and physical ability. It results from loss of function of the front parts of the brain, which control behaviour and judgement, and the nerve cells that control movement. FTD and MND both occur on their own; however a minority of people will start with symptoms of one of these conditions and also develop symptoms of the other. This is because the underlying damage is caused by the same mechanism and it is only the location of the damage which dictates which symptoms a person will suffer.
Why haven’t I heard of FTD/MND before?
In the past all “dementia” was typically thought to be Alzheimer’s disease. It is only recently that FTD has been distinguished as a distinct form of dementia. It is even more recently that the association between the physical changes of motor neurone disease (MND) and the cognitive and behavioural changes of FTD has been recognised.
What are the symptoms?
Personality and behaviour
Changes in a person’s character and behaviour are common. Often people will have difficulties with motivation (apathy) and will seem happy to sit and do little. They may show less interest in personal appearance and hygiene. People with FTD-MND may also have difficulty being considerate of others. They may behave in ways that other people find embarrassing. Some people adopt a rigid daily routine, doing things at the same time each day, and may be resistant to change. People with FTD-MND often show a preference for sweet foods. They may also eat more or develop food fads. They may choke and splutter when eating because of the physical problems of MND.
People with FTD-MND often have difficulty with complex planning or problem solving. Their reasoning and judgement may be impaired. For some people the thinking difficulties they have mean they are no longer able to make important decisions without the input of their loved ones.
People also may have difficulty in understanding and recognising the thoughts and emotions of others. This can lead to some of the changes in behaviour mentioned above.
People with FTD-MND may experience changes in their language. They may speak less and eventually become totally silent. They might repeat the same thing over and over again, or copy what others say, or else have difficulty finding the word they want and use wrong words. People with FTD-MND tend to be better with understanding what is said to them than expressing themselves. However, people may have difficulty taking in everything that is said. Physical difficulties in controlling the muscles used for speech may contribute to speech problems.
Recognition of people and objects
Occasionally people develop difficulty recognising faces and may use objects inappropriately because they no longer remember what they are for. These recognition problems are not caused by poor eyesight, but because the part of the brain that helps us to recognise and understand what we see is not working properly.
Memory and concentration
People with FTD-MND may appear forgetful. Difficulties in attention and taking in what is being said contribute to the forgetfulness.
Can they alter their behaviour?
No. The changes in behaviour are a direct result of damage to brain cells and are not under a person’s conscious control.
What are the physical changes?
People with FTD-MND develop weakness and wasting of the muscles. This can lead to problems in doing things with their hands, walking, speaking, eating and swallowing. In some people the mental changes (FTD) come first, whereas in others it is the physical changes (MND).
Are people with FTD/MND aware of their symptoms?
People with FTD-MND typically don’t have a full awareness of the mental and physical changes to themselves. It is often those close to them who are most aware and this can be very difficult.
Will the symptoms become worse?
The disease is progressive, so symptoms do get worse over time. People vary in terms of the speed of progression, so it is only by following people up in the clinic that we can gauge rate of progression in individual patients. Their mental and physical changes might progress at different speeds.
At what age are people affected?
The condition most commonly affects people between 50 and 70, but can occur in both younger and older people. The greater prevalence in middle age is one factor that distinguishes this from other forms of dementia, such as Alzheimer’s disease, which are more common in the elderly.
What is the cause?
The disorder is a degenerative condition of the brain and nervous system. This means that cells gradually cease to function properly, which is why symptoms develop. This is associated with the build-up of certain proteins the most common of which is called TDP-43. The cognitive and behavioural changes of FTD are caused by the build-up of these proteins within the frontal and temporal lobes of the brain. This can cause these parts of the brain to shrink. The physical changes of MND are caused by the build-up of these proteins in the nerve cells which carry information from the brain, down the spinal cord and to the muscles.
The underlying cause of this protein build-up is not fully understood and is the key area of current research. However we know that this condition is not caused by external environmental or lifestyle factors, such as diet, occupational exposure or head injury. It can affect people from all walks of life and is not simply the result of ‘over-use’ or ‘under-use’ of the brain.
Is FTD/MND inherited?
It can be. In many cases FTD-MND is sporadic, which means it occurs “out of the blue”, with no history of similar problems in other family members. However, it can also be caused by specific genetic problems which may be passed on through the family. Affected individuals within a family may show both behavioural and physical changes (FTD/MND) or the behavioural changes (FTD) or physical changes (MND) alone. Generally the risk of FTD-MND being inherited increases if multiple members of the family have FTD, MND, or FTD-MND. Your doctor may discuss with you the possibility of genetic testing and our colleagues in the Genetics Department are also happy to provide individual advice. If you or your family have particular concerns or questions please let us know.
Is there any treatment?
Current treatments do not provide a cure for the disease. However, there are drugs available that are designed to alleviate symptoms. Treatment options will be discussed with you in clinic.
Is there any research into FTD/MND?
Yes, certainly. There is now worldwide scientific interest in FTD/MND and research increases each year. The aim is to understand the mechanisms that underlie this disorder, so that better treatments can be developed. Our department is an active contributor to research in FTD/MND and other neurodegenerative disorders. If you would like to know more about research into FTD/MND please visit our website or ask when you attend the clinic.