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Corticobasal Degeneration

Information for patients and carers

What is corticobasal degeneration?
Corticobasal degeneration (CBD) is a progressive neurological disorder that affects both the outer (cortical) parts of the brain involved in thinking, and inner (basal ganglia) parts important for control of movement.

What are the symptoms?
Initial symptoms may include stiffness of the limbs, shakiness, jerkiness, and physical slowing.  Symptoms tend to be more pronounced on one side of the body (they are asymmetric). 

Typically, sufferers will have particular difficulty using one hand/arm.   This symptom is termed ‘apraxia’.   Often, sufferers report that the affected hand/arm is not under their control and does not seem to do what they want it to.  Some people with CBD have the feeling that this limb does not ‘belong’ to them, and moves of its own accord.

Patients may find that they become clumsy and lack co-ordination.  There may also be problems in walking and balance.

As the illness progresses, both sides of the body become disturbed.  Both the upper limbs (arms) and lower limbs (legs) may be affected.

Problems in speech may also occur in CBD.  Sufferers’ speech may become slurred (dysarthria), and they can find it difficult to generate speech.  Swallowing problems may also occur.

In addition to these physical symptoms, there may be problems in mental ability.  However, these are usually mild, and may include difficulties in memory and planning ability. 

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.

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.

At what age are people affected?
The disorder most commonly occurs after the age of 60, although it can occur in younger people. 

Is this condition inherited?
Most cases of corticobasal degeneration appear to occur ‘out of the blue’, with no history of similar problems in other family members. In these cases, the condition is not inherited and cannot be passed on to children.  However, in a very small number of cases, the condition appears to run in the family and is passed on through the genes. Families with a genetic predisposition to corticobasal degeneration are generally already aware that this condition is inherited, as they know of other relatives who have developed similar symptoms. If you have particular 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 drugs available, which are designed to alleviate symptoms. The most effective way to manage the disorder is through occupational, physical, and speech therapy.  Although the disease will continue to progress, therapies such as these may help to improve aspects of day-to-day living.  Physical therapy may help to maintain a range of motion in stiff joints, in turn helping to prevent pain and sustain mobility.  Occupational therapy may be useful in providing and developing helpful adaptive equipment.  Speech therapy may improve articulation and volume.

Why haven’t I heard of this condition before?
Corticobasal degeneration is rare.  Because of patients’ physical difficulties and stiffness of the limbs patients are often assumed to be suffering from Parkinson’s disease or ‘atypical Parkinsonism’.

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 corticobasal degeneration 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.