Corticobasal Degeneration is a rare type of parkinsonism that affects people from the age of 40, typically between the ages of 50 to 70. It tends to affect one side of the body more than the other initially, gradually spreading over a few years.
Corticobasal Degeneration has similarities with Progressive Supranuclear Palsy (PSP). Some people with Corticobasal Degeneration go on to develop PSP, and vice versa.
Corticobasal Degeneration is a very individual condition and the symptoms each person experiences vary. As Corticobasal Degeneration is a progressive neurodegenerative condition, symptoms gradually become worse over time.
The most common symptoms are outlined below, but many people have only a few symptoms.
- Difficulty controlling the limbs on one side of the body – often known as ‘alien limb’ syndrome – as arms or legs may seem to move independently
- Numbness and loss of coordinated movement in one hand (apraxia), making everyday tasks such as dressing, writing, and eating difficult
- Muscle stiffness (rigidity)
- Shaking (tremor)
- Jerky or awkward movement and spasms (dystonia)
- Balance and coordination problems.
Speech and communication problems: slow and slurred speech.
Swallowing difficulties: eating, drinking, and swallowing become progressively more difficult and food may ‘go down the wrong way’. This can lead to chest infections or pneumonia.
Cognitive and behavioural changes: thinking may become impaired, leading to memory problems and difficulty understanding and interpreting communication. It may also be difficult to carry out complex tasks that require planning ahead.
Changes in personality, such as apathy, irritability, and decreased interest in things previously enjoyed may be noticed by family and friends.
Diagnosis is usually based on the pattern of symptoms experienced and the exclusion of other conditions that may cause similar symptoms, such as Parkinson’s or stroke. Unfortunately, as with Parkinson’s, there is no single test or scan to diagnose Corticobasal Degeneration.
A diagnosis should be made by a specialist with experience in Corticobasal Degeneration, usually a neurologist. He or she may ask for a brain scan to rule out other causes, and they may also carry out tests to check memory, concentration, and understanding of verbal communication.
Corticobasal Degeneration occurs when cells in specific parts of the brain – the cortex and basal ganglia – are damaged as a protein called tau builds up and over time causes harmful clumps. In normal brains, tau is broken down to avoid a build-up, but in Corticobasal Degeneration this does not happen.
It is thought there may be some weak genetic link too but the risk of other family members developing Corticobasal Degeneration is very low.
There is currently no cure or treatment to stop Corticobasal Degeneration’s progression but medication and various therapies can relieve symptoms and improve quality of life.
- Medication can improve movement, cognitive and behavioural problems. However, the improvement is usually much less than in Parkinson’s.
- Physiotherapy can help with movement and balance problems.
- Speech and language therapy can help with swallowing and communication difficulties.
- An occupational therapist can advise on equipment and adaptations in the home, and can suggest strategies for carrying out daily tasks to retain as much independence as possible.
Content last reviewed: March 2020
We would like to thank Prof Carlo Colosimo (Università La Sapienza, Rome, Italy) for his help in reviewing this infomation.