Primary Progressive Aphasia

Information for patients and carers

What is Primary Progressive Aphasia?

Primary progressive aphasia (PPA) refers to a progressive problems in language resulting from degeneration of the left side of the brain.

What are the symptoms?

The main symptoms are difficulty in communication. People may have difficulty speaking, and their speech might be more hesitant or stuttering.  People with PPA may have difficulty formulating what they want to say and finding the correct word.  Word sounds may be produced incorrectly, or in the wrong order, e.g. “aminal” for “animal”; “par carp” for “car park”. People may also say the wrong word, and it is quite common for people to mix up “Yes” and “No”. People also often have difficulty in grammar and might miss out small grammatical words like “a” “the” “to” “from” etc., or they might get tenses mixed up.

Gradually people speak less and less and eventually may not speak at all. Understanding is often better than speech production, although may be affected to some degree.

Is it possible to get around the communication problem by writing?

Some people with PPA find it easier to write than to speak, so using a notebook can help.  However, for many people the difficulties are equally present in writing as speaking.  It is worthwhile experimenting to find out which method of communication works best for you. A Speech and Language therapist might be able to help you work out the best ways to communicate.

Can communication be improved by non-verbal means?

Some people with PPA are very good at communicating their needs by gesture and miming actions.  We recommend that loved ones encourage people to use gestures to help with communication and relieve some of the frustration they may feel when unable get points across verbally. Equally, use of gestures by loved ones and formal carers may help people’s understanding.  However, it is important to recognise that PPA is a disorder of communication, and some people find it just as difficult to communicate by non-verbal means as by speech.  Experimenting to find the way that works best for each individual is often the best way forward.

Are there other ways of helping?

Some carers wonder whether they should ‘fill in’ words that their relative cannot find. There is no right or wrong answer. We all find it more difficult to get speech out when we are anxious or under pressure. It is important to give people with PPA time as they often find it even more difficult when they are rushed.  At the same time, if they are struggling to find a word, offering suggestions can be a great help.  In conversation it is better to use short, simple sentences rather than long, complicated ones.   People with PPA will also find it easier to understand one person, than follow the discussion of a whole group.

Are there other symptoms apart from the difficulties in communication?

Some people with PPA also develop difficulties in carrying out manual tasks (apraxia), such as dressing and using cutlery.  People may also develop changes in behaviour. They may show less interest, or become more self-centred or inflexible. These changes are a direct result of changes in the brain and are not under the control of the person.

Does the disorder affect memory?

It may seem like people with PPA ‘forget’ telephone messages, the names of people they have seen, or information they have just been told.  This difficulty is part of the problem in language; they find it more difficult to make memories for the verbal information.  People may seem ‘overloaded’ by too much information.  This is an important reason for communicating using short simple sentences.  Memory for past events often remains good.

Are there physical changes?

People with PPA are often initially physically well.  They may slow up as the disease progresses and develop stiffness in the limbs. This may be more apparent in the right arm (because the left side of the brain controls the right side of the body). Weakness due to wasting of the muscles can occur, although this is very rare.

Are sufferers aware of what is happening to them?

People with PPA are frequently aware of difficulty speaking, and are often very frustrated. People often tell us that they feel anxious using the phone and in social situations so they avoid them. Their awareness may diminish as the disease progresses, and feelings of frustration and anxiety can improve. However, this reduction in the awareness of the person affected can be very difficult for their loved ones.

Will the symptoms become worse?

The disease is progressive, so symptoms do worsen 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 will give a good guide as to the likely future rate of progression. 

There are also differences in the way symptoms progress.  For some people the problem remains a relatively selective problem of communication. Other people develop more obvious physical problems (manual difficulties, stiffness of one limb – sometimes giving the impression that the person has had a stroke). Others show altered behaviour. Others may develop difficulties in spatial orientation and memory.  

At what age are people affected?

The condition most commonly begins in the 50s or 60s, but can occur in both younger and older people.

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 properly, which is why symptoms develop.  There are a few different causes of the brain changes seen in PPA, associated with changes in different proteins in the brain. It is difficult to be sure which of these is underlying a person’s PPA and there is a lot of research into understanding this better. How and why the brain changes develop and cause the symptoms is the key question for current research. However, we know that it 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 progressive aphasia inherited?

In most cases, PPA is sporadic, which means it occurs ‘out of the blue’, with no history of similar problems in other family members. However, in a small number of cases, PPA appears to run in families, suggesting a genetic component.  Generally the risk of PPA being inherited is low unless other family members have the same condition. Your doctor may discuss with you the possibility of genetic testing. If you or your family have any concerns or queries please let us know. 

Is there any treatment?

Current treatments do not provide a cure for the disease. However, there are some drugs that can have a beneficial effect on some of the symptoms of the condition (e.g. stiffening of the limbs). In some cases, speech and language therapy can help people to manage their communication difficulties. Any treatment options will be discussed with you in the clinic.

Is there research into the condition?

Yes, certainly. 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 primary progressive aphasia 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.