Information for patients and carers
What is Frontotemporal lobar degeneration?
Frontotemporal lobar degeneration (FTLD) refers to a group of disorders, affecting thinking and behaviour, which result from progressive damage to the front (frontal) and side (temporal) parts of the brain.
Why haven’t I heard of ‘FTLD’ before?
FTLD is one of the conditions which can cause dementia. Often when people think of dementia, they think of the most common cause, Alzheimer’s disease; but there are many different causes of dementia. FTLD is rarer than other kinds of dementia so people are less likely to have come across it. Despite this it is the fourth most common cause of dementia overall, and the second most common cause of dementia in people under the age of 65.
Sometimes people will not have heard of FTLD because it is called by other names. Other terms for FTLD, or syndromes which are caused by FTLD include “Pick’s Disease”, “Frontotemporal Dementia”, Semantic Dementia” and “Primary Progressive Aphasia”. If you would like to know more about which of these labels apply to you or your loved one, please ask in clinic.
What are the symptoms?
Different people have different symptoms depending on precisely which parts of brain are affected. These may include changes in personality and behaviour, thinking, language, face and object recognition and memory.
Personality and behaviour
The changes in the brain mean that sometimes people with FTLD may become more irritable, or appear selfish or inconsiderate. They may behave in ways that others find socially embarrassing or they may lose interest in hobbies and appear content to sit doing nothing. Sometimes people adopt a rigid daily routine, doing things at the same time each day, and may be resistant to change. They may show less interest in their appearance or personal hygiene. Some people with FTLD develop an increase in appetite, food fads or a sweet tooth.
People with FTLD may find it difficult to think through different options or make judgements about things. This might mean that they need extra help in making decisions. The thinking skills which can often be affected in FTLD are the ones which help with planning, organising, keeping track of what we are doing, stopping ourselves from becoming distracted and stopping ourselves from following automatic impulses. You may hear these thinking skills referred to as “Executive Functions”.
People may have difficulty finding the correct word. They might use the wrong word or pronounce things incorrectly. Some people may develop a slight stutter. Some people may repeat the same thing over and over again, or copy what others say. Some people simply speak less and less until they do not speak at all. Understanding what others say may also be affected to some extent.
Recognition of people and objects
Some people have difficulty in recognising faces and may use objects in unusual ways 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 FTLD may appear forgetful. Difficulties in attention, concentration and taking in what is being said may contribute to the forgetfulness.
Can the person alter his/her behaviour?
No. The changes in the person’s behaviour are a direct result of damage to brain cells and are not under their conscious control. Apparent selfishness or awkwardness is not deliberate.
Is the person aware of his/her symptoms?
The degree of awareness varies depending of the sufferer’s precise symptoms. In general, it is often the friends and family who are most aware and on whom the greatest burden falls.
Are there any physical changes?
People with FTLD generally remain physically well, although they are likely to ‘slow up’ as the disease progresses. Some people who have a lot of speech and communication problems may develop stiffness and poor use of their right arm. A minority of people with FTLD may also develop symptoms of Motor Neurone Disease. If you or your family notice any physical changes you should make sure your doctor is aware of these.
Will the symptoms become worse?
The disease is progressive, so symptoms will get worse over time. However, there is wide variation in the speed of progression. Some people show little decline over the course of a year whereas others change more quickly. Usually, monitoring a person’s progress over a year or so will give a good guide as to the likely future rate of progression.
At what age are people affected?
FTLD most commonly affects people between 50 and 65, 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. This means that cells in certain parts of the brain gradually stop working correctly and can die off, which is why symptoms develop. In this condition, this mostly affects the frontal and temporal lobes of the brain. There are a few different changes in the brain which can cause this condition; these are associated with different proteins in the brain. It is very difficult to be sure which underlying cause a person might be experiencing, but this is the subject of a lot of research. 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 FTLD inherited
Most cases of FTLD occur ‘out of the blue’, with no history of similar problems in other family members. However, in a small number of cases, the condition does run in the family and is passed on through the genes. Your doctor may discuss with you the possibility of genetic testing. Families with a high genetic risk are generally already aware of this because they know of other relatives who have developed similar symptoms. If you have particular concerns or questions please let us know. Our colleagues in the Genetics Department are able to provide individual advice.
Is there any treatment?
Unfortunately, current treatments do not provide a cure for the disease. However, there are drugs available for other conditions, such as anxiety and depression, that may help some FTLD symptoms. Your doctor will discuss appropriate treatment options with you and your family in clinic.
Is there any research into FTLD?
Yes, the interest in this area of 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 FTLD and other degenerative disorders. If you would like to find out more about the research carried out in our unit, please visit our website or ask when you attend the clinic.