Corticobasal degeneration (CBD) is a rare progressive neurological disorder characterized by cell loss and deterioration of specific areas of the brain. Affected individuals often experience movement disorders initially in one limb that might spread to both the arms and legs.
Symptoms include muscle rigidity and the inability to perform purposeful or voluntary movements. Affected individuals may have the sufficient muscle power for manual tasks but struggle to direct their movements appropriately. Although CBD was historically described as a motor disease, it is now recognized that cognitive and behavioural symptoms are also associated with CBD and often come before the motor symptoms. Initial signs typically appear in people ages 60-70, and may include language difficulties, poor coordination and issues with memory. The exact cause of CBD is unknown; however, it may be linked to the accumulation of the tau protein in the brain.
What causes CBD?
The cause of CBD is unknown. Scientists know that in some people with CBD, there is a large build-up of a protein called tau. Tau occurs normally in the brain, but we do not yet understand what causes it to build up in large amounts.
What are the first symptoms of corticobasal degeneration (CBD)?
CBD typically affects the limb at first. This is usually a hand or arm, but sometimes a leg. Problems affecting the limb can include:
- a clumsy or “useless” hand
- muscle stiffness
- shaking and spasms
- loss of feeling
- feeling like the limb doesn’t belong to you (an “alien” limb)
Some people also develop problems with walking and coordination.
How is CBD diagnosed?
The absolute diagnosis of these is made based on a brain autopsy performed by a neuropathologist. Autopsy-confirmed cases of CBD are almost always sporadic, which means they randomly occur in the population at large without a specific genetic mutation.
CBD Frequently Asked Questions
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 the time.
- Difficulty moving on one or both sides of the body, which gets worse over time
- Poor coordination
- Trouble with balance
- Abnormal postures of the hands or feet, such as a hand forming a clenched fist
- Muscle jerks
- Difficulty swallowing
- Abnormal eye movements
- Trouble with thinking, speech and language
Is CBD disease hereditary?
CBD never runs in families. However, a variant in the gene on chromosome 17 that encodes the tau protein is a little more common in CBD than in the rest of the population. Called the “H1 haplotype,” it occurs in 92 percent of people with CBD and 77 percent of the rest of the population.
Is corticobasal degeneration a form of dementia?
Researchers currently think corticobasal degeneration is a form of frontotemporal degeneration and distinct from Alzheimer’s disease. The breakdown of cells here affects your brain’s frontal and temporal lobes. The condition occurs when a tau, a protein normally found in brain cells, abnormally clumps together.
How quickly does corticobasal degeneration progress?
Corticobasal degeneration usually progresses slowly over 6 to 8 years. Death is caused by pneumonia or other complications of severe debilities, such as sepsis or pulmonary embolism.
Is corticobasal syndrome fatal?
The symptoms of corticobasal degeneration (corticobasal syndrome) progress to serious complications, such as pneumonia, blood clots in the lungs, or sepsis, a life-threatening response to an infection. Corticobasal degeneration complications ultimately lead to death.
What is the treatment for corticobasal degeneration?
There are no treatments that help slow the progression of corticobasal degeneration (corticobasal syndrome). Your doctor may recommend medications to manage your symptoms. Getting occupational and physical therapy may help you manage the disabilities caused by corticobasal degeneration.
Is corticobasal degeneration a form of Parkinson’s?
Corticobasal syndrome (CBS) is a form of atypical parkinsonism (a parkinsonism-plus syndrome), so it shares some features with Parkinson’s disease such as stiffness (rigidity), and tremor at rest, slowness of movement (bradykinesia) and postural instability (balance difficulties).
How many people in the world have a corticobasal syndrome?
The disorder is estimated to affect 5 people per 100,000 in the general population, with approximately 1 new case per year per 100,000 people. CBD also represents 4–6% of patients with parkinsonism.
How do you test for corticobasal degeneration?
Magnetic resonance imaging (MRI) scan, where a strong magnetic field and radio waves are used to produce detailed images of the inside of the brain. Positive emission tomography (PET) scan is a scan that detects the activity of the brain.
Can corticobasal degeneration be reversed?
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.
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