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Progressive Apraxia

Information for patients and carers

What is progressive apraxia?
Progressive apraxia is a disorder of skilled movement, which results from selective degeneration of parts of the brain associated with the control of movement. Patients are not paralysed or physically weak.  Moreover, they may know what actions they want to perform. However, they have difficulty converting their intention (e.g. to greet someone) into the correct action (e.g. shaking hands).

What are the symptoms?
The initial symptom is often a difficulty carrying out actions of the upper limbs (e.g. writing, drawing, using tools). Coordinating actions with the two hands, such as using a knife and fork or striking a match, may be particularly difficult. Some people find that the actions of one hand interfere with those of the other. The affected limb may seem to have a “will of its own”, so may drift in the air or touch the person’s face. Involuntary actions of the limb are referred to as “alien limb”. The disorder may affect both sides of the body, although one side may be more impaired than the other.

Some people experience problems in use of legs, resulting in a difficulty in walking.  Again, the problem is not a physical one of weakness or paralysis.  The problem is in sending “messages” from the brain to the legs telling them what to do. Occasionally such problems in mobility are the earliest symptom. 

Some people may develop problems in speaking.  The person knows what he/she wants to say and has the correct words available but has difficulty converting the sounds of the words into the correct movements of the mouth, lips and tongue. The result is that speech becomes unclear and pronunciation difficult.  Occasionally speech problems are the earliest symptom.

How do symptoms progress?
The problems with movement tend to become gradually worse over time. Moreover, the difficulties, which may initially be confined to one part of the body (e.g. arms) may spread to other parts (e.g. legs and face).  A person may have difficulty carrying out movements involving the whole body, such as sitting down or getting out of a car.  There may be a gradual stiffening of the limbs.

The disorder is predominantly one of production of movement. Thus, the person has difficulty using gestures him/herself but can generally understand gestures and actions carried out by other people. However, in the late stages, understanding of other people’s gestures may also be compromised to some degree.

How quickly do symptoms progress?
There is wide variation in the speed with which symptoms progress.  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.

Are people with progressive apraxia aware of their difficulties?
Initially people are aware and, understandably, may be frustrated by their difficulties.  Generally, awareness lessens as the disease progresses.  One reason is that the brain stores motor representations of the correct movement, which gradually erode as brain cells die.  Thus, the person no longer has a correct internal representation or ‘model’ of the movement against which to check the accuracy of his/her own actions. As awareness diminishes so too do any feelings of frustration or distress.

At what age are people affected?
The condition most commonly affects people in their 50s and 60s, although can develop in younger or 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 cease to function properly, which is why symptoms develop. The cause of this degeneration is at present not fully understood and is the key question for 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 progressive apraxia inherited?
Most cases of progressive apraxia occur ‘out of the blue’, with no other family members being affected. However, in a very small number of cases, the condition runs in the family and is passed on through the genes. Families with a genetic predisposition are generally already aware of this as they know of other relatives who have developed similar symptoms. If you have queries or concerns please let us know. Our colleagues in the Genetics Department are happy to provide individual advice.

Is there any treatment?
Current treatments do not provide a cure for the disease. However, there are medical treatments available that may alleviate some of the symptoms, such as tablets for reducing stiffness in the limbs. Many of the symptoms can also be helped by physiotherapy. Regular exercise can help to maintain mobility of the limbs, thereby reducing possible stiffness and pain.  Simple remedies such as holding and squeezing a sponge ball or beanbag can help to maintain movement of the hands and prevent contraction of the fingers.

Why haven’t I heard of this condition before?
The condition has only recently been recognised.  In the past progressive apraxia was typically thought to be a form of Parkinson’s disease.

Is there research into progressive apraxia?
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 progressive apraxia 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.