Corticobasal degeneration (CBD), also known as corticobasal syndrome (CBS), is a rare neurological disorder considered an Atypical Parkinson's condition.
By building a strong support system and implementing adaptive treatment strategies, individuals with Parkinson's corticobasal degeneration (CBD) can enhance their resilience and overall wellbeing.
Understanding the symptoms and cognitive impacts
Corticobasal degeneration(CBD) is a type of dementia where the loss of ability to think, remember or reason interferes with a person's daily life and activities. Typically, progressive damage to specific areas of the brain, including the cerebral cortex and the basal ganglia impacts various functions such as movement, memory, and cognition.
The condition typically leads to symptoms that may initially affect one side of the body but eventually progress to both sides. Typically indicative of CBD is an alien arm, one-sided presentation and other symptoms may include:
- difficulty moving, which worsens over time
- poor coordination and balance issues
- stiffness and muscle jerks
- abnormal postures of the hands or feet
- difficulty swallowing
- abnormal eye movements
- trouble with thinking and language skills
- speech problems, such as slow and halting speech.
There have been no recorded cases in people under the age of 40 years, with an average age of onset 60-70. There are no known risk factors, and it can affect both men and women, however slightly more women than men.
CBD has no known genetic links. It is less common than other atypical Parkinsonian syndromes like Multiple System Atrophy (MSA) or Progressive Supranuclear Palsy (PSP).
The disease progresses over six to eight years, and as it advances, people living with CBD may lose the ability to walk.
The underlying cause of CBD is often associated with the buildup of the protein tau in brain cells, leading to their deterioration. This is like other neurodegenerative diseases including Alzheimer's and PSP.
There is currently no cure for CBD, and treatment focuses on managing symptoms. This may include medications, physiotherapy, speech pathology, and dietary changes to help improve quality of life. If you or someone you know is experiencing symptoms that could be related to CBD, it is important to consult a healthcare provider for a proper diagnosis and assemble a care team to form a comprehensive management plan.
Causes
Currently, the cause of Corticobasal Degeneration is not known, but several different factors are thought to be involved including age, genetic and environmental influences. It is thought to be linked to abnormal cellular Tau (a naturally occurring brain protein) changes.
Symptoms
CBD often presents like Parkinson's at first, with one side of the body. Early symptoms are:
- rigidity and slow movement
- an inability to perform purposeful tasks
- postural and action tremor in some cases
- trouble with speech or language
- clumsiness, jerking and sensory changes in one limb.
Later symptoms include:
- loss of ability to carry out skilled movement and gestures, despite having the physical ability and desire to perform them (visual apraxia)
- eating difficulties due to apraxia
- self-care difficulties
- progressive communication changes
- dementia.
However, you may need an imaging test, such as a CT scan or MRI, to rule out other conditions. Sometimes, these tests are performed every few months to look for abnormal changes in the brain.
Diagnosis
A diagnosis is based on your signs and symptoms and a history because no classical signs are seen on routine imaging. A strong feature of 'alien limb' phenomenon, where the hand seems to have a mind of its own. CBD also responds poorly to levodopa. These traits help with the diagnosis.
Your care
There are currently no treatments that are shown to help slow the progression of CBD. Your GP or neurologist may recommend medications to manage your symptoms.
Getting a care team together with occupational and physiotherapy (OT and PT respectively) can also help manage symptoms. An OT can recommend walking devices that may help with mobility and prevent falls. PTs can help with symptom management. A Speech Pathologist can help with communication and swallowing problems. A dietitian may be helpful in ensuring proper nutrition and reducing the risk of inhaling food into the lungs (aspiration). Support and allied health input is recommended.
Swallowing assessment and dietary modifications may help, and modifying the environment will help with safety. Parkinson's nurse specialists are familiar with this condition and can be a valuable resource and support for the person and family.
Advance Care Planning Australia (ACPA) is a national program, enabling Australians choice and control of their life and future health care. Find more information about advance care planning in your state or territory or for advice and support freecall 1300 208 582 or visit advancecareplanning.org.au.
Further information can be found in the CBD Information Kit available from Fight Parkinson's Victoria
Support for carers
Given the CBD journey and the challenges care partners face, we also wanted to share a few additional resources that may be helpful. Palliative Care Australia offer compassionate support and information for people living with progressive neurological conditions like CBD. For carers, you might find valuable support through Carer Gateway and Carers Australia, which provide counselling, respite options, and practical advice tailored to your needs.
