Overview

Corticobasal degeneration (CBD) is a rare condition that can cause gradually worsening problems with movement, speech, memory and swallowing.

It's often also called corticobasal syndrome (CBS).

CBD is caused by increasing numbers of brain cells becoming damaged or dying over time.

Most cases of CBD develop in adults aged between 50 and 70.

CBD symptoms

The symptoms of CBD get gradually worse over time. They are very variable and many people only have a few of them.

Symptoms can include:

• difficulty controlling your limb on one side of the body (a "useless" hand)
• shaking (tremors), jerky movements and spasms (dystonia)
• problems with balance and co-ordination
• slow and slurred speech
• symptoms of dementia, such as memory and visual problems
• difficulty swallowing
• slow, effortful speech

One limb is usually affected at first, before spreading to the rest of the body. The rate at which the symptoms progress varies widely from person to person.

What causes CBD?

CBD occurs when brain cells in certain parts of the brain are damaged as a result of a build-up of a protein called tau.

The surface of the brain (cortex) is affected, as well as a deep part of the brain called the basal ganglia.

Tau occurs naturally in the brain and is usually broken down before it reaches high levels. In people with CBD, it isn't broken down properly and forms harmful clumps in brain cells.

CBD has been linked to changes in certain genes, but these genetic links are weak and the risk to other family members is very low.

Diagnosing CBD

There's no single test CBD. Instead, the diagnosis is based on the pattern of your symptoms. Your doctor will try to rule out other conditions that can cause similar symptoms, such as a stroke or Parkinson’s disease.

You may need to have a brain scan to look for other possible causes of your symptoms, as well as tests of your memory, concentration and ability to understand language.

You will usually see a neurologist (a specialist in conditions affecting the brain and nerves).

Treatments for CBD

As someone with CBD can be affected in many different ways, treatment and care is best provided by a team of health and social care professionals working together. Treatments may include:

• medication - to improve stiffness and muscle spasms, sleep and mood, pain or memory
• physiotherapy - to help with movement and balance difficulties
• speech and language therapy - to help with communication and swallowing problems
• occupational therapy - to improve the skills and abilities needed for daily activities at home
• palliative care and advanced care planning

Outlook

There is currently no treatment that has been shown to stop CBD getting gradually worse, although treatments can reduce many of the symptoms.

Good care and assistance can help someone with CBD be more independent and enjoy a better quality of life, but the condition will eventually put them at risk of serious complications.

CBD usually changes very slowly. Many people find it helpful to plan ahead with their doctors (GP and specialist) to make decisions about what to do in later stages of the illness.

Difficulty swallowing can cause chocking, or inhaling food or liquid into the airways. This can lead to pneumonia, which can be life-threatening.

As a result of these complications, the average life expectancy for someone with CBD is around 6 to 8 years from when their symptoms start. However, this is only an average and CBD is very variable.

Information about you

If you have CBD, your clinical team will pass information about you on to the National Congenital Anomaly and Rare Diseases Registration Service (NCARDRS).

This helps scientists look for better ways to prevent and treat this condition. You can opt out of the register at any time.

Find out more about the register on the National Disease Registration Service website.

Symptoms

Diagnosis

It can be difficult to diagnose corticobasal degeneration (CBD), as there's no single test for it, and the condition can have similar symptoms to a number of others.

A diagnosis of CBD will be based on the pattern of your symptoms. Your doctor will also try to rule out other conditions that can cause similar symptoms, such as a stroke, Parkinson’s disease, and Alzheimer's disease.

Your doctor will need to carry out assessments of your symptoms, as well as other tests and scans.

The diagnosis will usualy be made by a neurologist (specialist in conditions affecting the brain and nerves).

Brain scans

If you have symptoms of CBD that suggest there is something wrong with your brain, it is likely that you will be referred for a brain scan.

Types of scan that you may have include:

• MRI scan – where a strong magnetic field and radio waves are used to produce detailed images of the inside of the brain
• PET scan – a scan that detects the activity of the brain
• a DaTscan – to measure the amount of a chemical called dopamine that your brain is making

These scans can be useful in ruling out other possible conditions, such as brain tumours or strokes.

MRI scans can also detect abnormal changes to the brain that are consistent with a diagnosis of CBD, such as shrinkage of certain areas.

Brain scans are also being developed to show the build-up of a protein called tau. This is associated with CBD.

Ruling out Parkinson’s disease

A person's symptoms usually help distinguish CBD from Parkinson's disease, but sometimes tests may be used to support the diagnosis and rule out other possible conditions.

You may be prescribed a short course of a medication called levodopa. This typically works well in Parkinson's disease, but not so well in CBD.

If it doesn't lead to a significant improvement in your symptoms, it can help your doctor distinguish CBD from Parkinson's disease.

Neuropsychological testing

It is also likely that you will be referred to a neurologist and possibly also a psychiatrist for neuropsychological testing.

This involves having a series of "memory tests" with words and pictures. They're designed to evaluate the full extent of your symptoms and their impact on your mental abilities.

The tests will look at abilities such as:

• memory
• concentration
• understanding language
• the processing of visual information, such as words and pictures
• numbers and counting

Most people with CBD have a distinct pattern of difficulties with these tests.

The memory of previously learned facts and the person's own life story is generally maintained.

Coping with a diagnosis

Being told that you have CBD can be an devastating and difficult to take in.

You may feel numb, overwhelmed, angry, distressed, scared or in denial. Some people are relieved that a cause for their symptoms has finally been found. There's no right or wrong way to feel - everybody is different and copes in their own way.

Support from your family and care team can help you come to terms with the diagnosis.

The PSP Association (PSPA) can give you information and practical advice about living with CBD, as well as providing support to help you cope with the emotional impact of the condition.

You can get in touch with the PSP Association by calling their helpline on 0300 0110 122, or by emailing: helpline@pspassocation.org.uk.

You may find that many people - even the doctors you meet - have not heard of CBD. The PSP Association has online and printed information for patients, their families and professionals.

Treatment

There's currently no cure for corticobasal degeneration (CBD) and no treatment to slow it down, but there are lots of things that can be done to help manage the symptoms.

Care will be provided by a team of health and social care professionals working together. This is known as a multidisciplinary team.

Members of your multidisciplinary team may include:

• a neurologist – a specialist in conditions that affect the brain and nerves
• a physiotherapist – who can help with movement and balance difficulties
• a speech and language therapist - who can help with speech or swallowing problems
• an occupational therapist – who can help you improve the skills you need for daily activities at home, such as washing, dressing or getting around
• a social worker – who can advise you about the support available from social services
• a specialist neurology nurse – who may act as your point of contact with the rest of the team
• special care advisors from the PSP Association

A care plan will be drawn up in discussion with your team. This will outline the treatments you need to help the symptoms of CBD, as well as support and advice you require to make your life easier.

Medicine

There are currently no medicines that treat CBD specifically. Depending on the person's symptoms or complications, there are medicines that may help with:

• muscle stiffness of contractions
• jerky movements 
• memory and related mental abilities - the medicines used to treat Alzheimer's disease may also be used in CBD, such as donepezil or memantine.
• irritability or depression
• sleep problems .
• bladder problems and incontinence 
• pain and anxiety - simple painkillers like ibuprofen, as well as more specialist medicines 
• bone strength - if people are prone to regular falling, osteoporosis (weak bones) and vitamin D problems should be ruled out or treated.

In general, people with CBD are sensitive to medicine side effects. Doses may start low and be increased gradually.

Cognitive stimulation

Cognitive stimulation is a type of therapy used to treat dementia, and it may be helpful if a person with CBD has dementia symptoms.

It involves taking part in activities and exercises designed to improve your memory, problem-solving skills and language ability.

Read more about treatments for dementia.

Physiotherapy

A physiotherapist can give you advice about how to remain safely mobile. Regular exercise can help strengthen your muscles, improve your posture and prevent stiffening of your joints.

They can teach you breathing exercises to use when you eat, to reduce your risk of developing aspiration pneumonia (a chest infection caused by food particles falling into your lungs).

Occupational therapy

An occupational therapist (OT) can give you advice about the best ways to increase your safety and prevent trips and falls during your day-to-day activities.

For example, a person with CBD may benefit from having bars placed along the sides of their bath to make it easier to get in and out.

The occupational therapist can also arrange access to mobility equipment such as walking frames and wheelchairs. They can also arrange equipment to help the person or their carer manage other everyday activities for washing, dressing, eating and using the bathroom safely.

Speech and language therapy

A speech and language therapist (SLT) can help assess and treat your speech and swallowing problems.

They can teach techniques to help make the voice as clear as possible and can advise you about suitable communication aids or devices that the person may need as CBD progresses.

A therapist can also advise you about different swallowing techniques and, working together with a dietitian, they may suggest altering the consistency of your food to make swallowing easier.

Diet and severe swallowing problems

You may be referred to a dietitian, who will advise you about making changes to your diet, such as including food and liquids that are easier to swallow while ensuring that you eat a healthy, balanced diet.

Feeding tubes may be recommended for severe swallowing problems, where the risk of malnutrition, weight loss, fatigue and dehydration is increased. You should discuss the pros and cons of feeding tubes with your family and care team.

The decision about whether and when to consider a feeding tube depends on the individual and should be discussed with a specialist.

Read more about treatment for swallowing problems.

Palliative care

Palliative care aims to relieve pain and other distressing symptoms while providing psychological, social and spiritual support. It can be offered at any stage of CBD, alongside other treatments.

Palliative care can be received:

• in a hospice
• at home or in a residential home
• on a day patient basis in a hospice
• in a hospital

Advanced care planning

Many people with CBD make plans for the future that outline their wishes about medical care and other decisions. They share these plans with both their family and the health professionals involved in their care.

This can be useful in case you're unable to communicate your decisions later on because you're too ill. However, you don't have to do it if you don't want to.

Issues that you may want to cover include:

• if you want to be treated at home, in a hospice or in a hospital when you reach the final stages of CBD
• if you would be willing to use a feeding tube if you were no longer able to swallow food and liquid
• if you'd be willing to be resuscitated if your heart were to stop

If you decide to discuss these issues, they can be written down in a number of ways:

• Advance decision to refuse treatment (living will)
• Advance statement about your wishes
• Emergency healthcare plan
• Preferred place of care
• Lasting power of attorney

Your care team can provide you with more information and advice about these decisions and how best to record them.

Care and support

If someone you know develops CBD, you may need information and advice about caring for them.

You can contact the PSP Association (PSPA), who provide help and advice to people living with CBD. Their email address is: helpline@pspassociation.org.uk and you can call their helpline on 0300 011 0122.

The Parkinson's nurse at your local hospital may also be able to provide you with useful information and support.