Symptoms are similar to those found in Parkinson’s disease, such as poor coordination, an absence of movements, rigidity, impaired balance and limb dystonia (abnormal muscle postures). A person with CBS eventually becomes unable to walk. CBS can lead to pneumonia or other complications of severe debility such as sepsis or pulmonary embolism. There can be overlap in symptoms at diagnosis with those of Parkinson’s, but the conditions are different and there can be frustration around lack of recognition and validation.    

Symptoms vary from person to person. Movement-related symptoms can include: 

• stiff muscles
• slow or awkward movement
• shaking
• clumsiness
• jerkiness
• loss of balance and coordination and
• numbness or loss of sensation in part of the body

In the early stages, people may experience the gradual loss of the use of one hand or leg. Some people with CBS experience an ‘alien limb’ or the feeling that they have no control over the affected limb or hand. Symptoms usually start on one side of the body (unilateral) and remain worse on that half as the condition progresses. It is also common for people with CBS to lose their sense of balance and coordination, which causes walking difficulties. Problems with visual-spatial awareness can occur where the brain misunderstands what the eyes see and people may experience difficulty in making simple purposeful movements such as picking up objects. 

Many people are initially diagnosed with Parkinson’s due to the similarity of symptoms. Treatments for Parkinson’s are generally ineffective in treating CBS. As the condition progresses, movement and balance problems become worse and limbs on both sides of the body may be affected. Speech can become slow and slurred due to reduced control of muscles of the mouth and throat. The muscles of the eyes can also be affected and people may have problems moving their eyes up and down or left to right. This can contribute to problems carrying out everyday tasks and the risk of falls. 

Many people with CBS also experience thinking problems, such as speech disturbance, word-finding difficulties, memory loss and problems with planning. Personality changes are also common and may include depression, anxiety, irritability and loss of inhibition. Some people may experience Obsessive-Compulsive Disorder (OCD) which can include repetitive actions or an obsession with cleanliness or with checking activities. 

We do not know what causes CBS. The damage and shrinkage to the brain are thought to be caused by an over-production of a protein called tau. Tau occurs naturally in the brain but people with CBS have excess levels. Excess tau forms into clumps and these are thought to kill nearby brain cells. The cause of tau overproduction is not known. Research is underway into factors such as infections and exposure to toxins, however there is no conclusive evidence of any link. Research is ongoing in Australia and overseas. Contact Fight Parkinson’s or your neurologist for further information about participating in CBS trials.

There is no evidence to suggest that CBS runs in families, however recent research indicates that genetic factors can make a person more likely to develop CBS. In other words, there may be some genetic susceptibility that makes some people more at risk than others, but CBS rarely affects more than one person in a family. 

CBS is a very rare condition. In Australia there are currently thought to be around 150 people with CBS. It is a difficult condition for doctors to diagnose, so actual numbers may be higher. People with CBS usually begin to get symptoms around the age of 60, however CBS has been found in people much younger, although this is rare. CBS can affect both men and women from all backgrounds and lifestyles.

Treatments and therapies are available to help manage symptoms of CBS, however there is no cure and we do not know of any way to prevent it from occurring or slow its progression. It is important that people with CBS see a neurologist, preferably one with expertise in movement disorders. Depending on symptoms, it is also important to seek the advice of other health professionals with expertise in the condition, such as a physiotherapist, occupational therapist, speech pathologist (speech therapist) or counsellor. Fight Parkinson’s can provide information about neurologists and other health professionals in your area. 

CBS cannot be cured, so everyone with CBS experiences deterioration prior to death. Initial symptoms become more severe over time and new symptoms may develop. As the condition progresses, people with CBS are at risk of developing serious complications such as pneumonia, bacterial infections or pulmonary embolism. People with CBS typically live for around eight years after the onset of symptoms. Research into CBS is currently underway giving us hope for the future, particularly in relation to more effective management of symptoms. 

CBS is difficult to diagnose. There are no blood tests or brain scans that can diagnose it, although tests and scans are commonly used to rule out other conditions that can cause similar symptoms. As CBS occurs in the brain, a diagnosis can only be confirmed at post mortem. It is common for people with early symptoms of CBS to be misdiagnosed with Parkinson’s or stroke. A limited response to Parkinson’s medication is a key indicator of the need to review a diagnosis of Parkinson’s. Because CBS is rare, many doctors are not even aware of the condition and do not know what symptoms to look for. It is important to see a neurologist (a doctor specialising in brain conditions). People with CBS should visit their neurologist regularly for ongoing treatment and advice. If possible see a neurologist with expertise in movement disorders as they are more likely to be up-to-date with the latest advances in treatment and management. 

People may experience a range of feelings after being diagnosed with CBS. Most people experience a feeling of grief, which may include fear, sadness, denial, anger or concern for the future. Some people may experience a feeling of relief at having a name for the difficulties they have been experiencing after searching so long for answers. It is important to have a safe space in which to work through these feelings of grief and be aware that those around you will be experiencing the same range of emotions. Speaking to a counsellor can help you to come to terms with your feelings and make meaning of the situation. Getting information and support can help you to adjust to the diagnosis and begin to make changes that will help with the daily management of the condition. Remember, you are not alone and help is available. The best place to start is by contacting Fight Parkinson’s. 

CBS is a complex and challenging condition. It is important to get help and to communicate your needs. Fight Parkinson’s provides information and support for people with CBS and their families and carers. We also provide a free telephone interpreter service for people who speak a language other than English.

There are a range of services available to assist people with daily living and to help members of the community to make the most of living with an illness or disability, including:

• information and emotional support – either over the phone or in person
• therapies and resources to maintain independence
• financial support and assistance
• support groups where you can share your experiences and learn from others
• help with personal care, such as bathing, dressing, and eating
• home delivered meals and other food services
• home help for assistance with housework, laundry, and shopping
• help for carers, including information, counselling, advice and respite to help them take a break from caring.

For further information about services that can help, contact Fight Parkinson’s or your local council. The Commonwealth Respite and Carelink Centre on 1800 052 222 can also provide information about services in your local area.

Fight Parkinson’s holds a bi-monthly online support group for people living with CBS. If you are interested in attending an online meeting or would like to know more, please call us on 03 8809 0400 or email us at info@fightparkinsons.org.au.

No-one has to face CBS alone.

Our Health Team offers a national specialist support  for individuals and families and can help with information on symptom management, services, health-related benefits and entitlements and everyday living. As families and people affected by CBS often come into contact with health professionals with no or limited knowledge of their condition, we can also provide specific information for, and advocate on your behalf, to medical and healthcare professionals. This service is available by phoning 03 8809 0400 from 9am – 5pm on weekdays 

Fight Parkinson’s provides a range of resources and publications that can assist people living with CBS, their families and carers to feel more informed about the condition. We are also committed to funding research that will provide life quality outcomes for people living with CBS and ultimately, find a cure.

People living with CBS – and their carers – need to continue to take precautions to prevent exposure to the Coronavirus. This is because COVID-19 can infect the entire respiratory system and is likely to cause pneumonia, especially in people living with pre-existing conditions.

This can cause complications for people with CBS, particularly those with more advanced symptoms, which may include difficulty swallowing or coughing up secretions, which can collect in the throat causing a gurgly voice or drip into the lungs causing chest infection and sometimes pneumonia.

In addition, a person with CBS may have some difficulties coughing up the lung’s secretions, allowing the secretions to fill spaces in the lungs where air should be flowing, reducing the ability of oxygen to reach the tissues.

Keep track of all the latest Victorian updates via the Department of Health and Human Services.

People with CBS – as well as their carers – were eligible for COVID-19 vaccination in Stage 1B, which commenced roll-out in early 2021. Fight Parkinson’s recommends you discuss the COVID-19 vaccination with your GP or Neurologist, including when to receive your booster shot. In line with recommendations of the International Movement Disorder Society and Australian Government advice, we encourage people living with CBS to receive the COVID-19 vaccination.

This recommendation is given because of the overwhelming benefits, with the risks being the same for people living with CBS as with the aged matched general population.

Read Fight Parkinson’s’ COVID-19 vaccination statement.

Fight Parkinson’s is one of 12 associations working as a single international body to deliver an increased focus into PSP|CBD research.

PROMOTUS, the PSP|CBD Global Alliance was formed in June 2019 to:

• represent the views of national PSP|CBD associations, providing a single point of contact for pharmaceutical companies and researchers
• provide best practice care and support for people affected by the conditions
• encourage research into PSP|CBD and ensure that research outcomes are disseminated around the world
• partner with pharmaceutical companies and biotechs to facilitate easier access to patients for clinical trials
• grow the network of PSP|CBD associations around the world to reach and support more people affected by the conditions.