What are the other Names for this Condition? (Also known as/Synonyms)

• CBD (Corticobasal Degeneration)
• Cortico-Basal Ganglionic Degeneration (CBGD)
• Corticobasal Syndrome

What is Corticobasal Degeneration? (Definition/Background Information)

• Corticobasal Degeneration (CBD) is characterized by the break down (degeneration) of parts of the brain, including the cerebral cortex and basal ganglia
• The cerebral cortex is responsible for most of the brain's processing of information, and the basal ganglia are deep brain structures that help start and control movement
• Signs and symptoms of Corticobasal Degeneration include poor coordination, loss of movement, rigidity, poor balance, unnatural posturing of the muscles, intellectual (cognitive) impairment, speech impairment, muscular jerks, and difficulty swallowing. These symptoms develop and worsen over time
• Currently the cause of Corticobasal Degeneration is not known
• Treatment depends on the symptoms in each person
• People with Corticobasal Degeneration usually do not survive beyond an average of 7 years after symptoms begin. Aspiration pneumonia or other complications are usually the cause of death

(Source: Corticobasal Degeneration; Genetic and Rare Diseases Information Center (GARD) of National Center for Advancing Translational Sciences (NCATS), USA.)

Who gets Corticobasal Degeneration? (Age and Sex Distribution)

• Corticobasal Degeneration is a rare congenital disorder. The presentation of symptoms may occur at birth
• Both males and females may be affected
• Worldwide, individuals of all racial and ethnic groups may be affected

What are the Risk Factors for Corticobasal Degeneration? (Predisposing Factors)

• A positive family history may be an important risk factor, since Corticobasal Degeneration can be inherited

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases one’s chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Corticobasal Degeneration? (Etiology)

The underlying cause of Corticobasal Degeneration (CBD) is poorly understood.

• However, researchers have found that a protein called tau plays a role in the development of CBD. Tau is a specific type of protein that is normally found in the brain
• In CBD, abnormal levels of tau accumulate in the brain cells, eventually leading to their deterioration and causing symptoms of the condition. Exactly why this happens is unknown
• Tau also appears to play a role in other neurodegenerative diseases such as Alzheimer disease, progressive supranuclear palsy, and frontotemporal dementia

There is no evidence to suggest that environmental exposure to toxic or infectious agents plays a role in causing CBD.

• Corticobasal Degeneration (CBD) is almost always sporadic, developing by chance rather than being inherited
• Rare familial cases have been reported, leading to the possibility that there may be a genetic basis for at least a predisposition to CBD
• Some research has found associations with CBD and a specific form (variant) of the tau gene
• However, not all people with CBD have the tau gene variant, and not all people with the gene variant develop CBD

(Source: Corticobasal Degeneration; Genetic and Rare Diseases Information Center (GARD) of National Center for Advancing Translational Sciences (NCATS), USA.)

What are the Signs and Symptoms of Corticobasal Degeneration?

The signs and symptoms of Corticobasal Degeneration include:

• Poor coordination
• Loss of movement, rigidity
• Poor balance
• Unnatural posturing of the muscles
• Intellectual (cognitive) impairment
• Speech impairment
• Muscular jerks
• Difficulty swallowing

These symptoms develop and worsen over time.

(Source: Corticobasal Degeneration; Genetic and Rare Diseases Information Center (GARD) of National Center for Advancing Translational Sciences (NCATS), USA.)

How is Corticobasal Degeneration Diagnosed?

Corticobasal Degeneration is diagnosed on the basis of the following information:

• Complete physical examination
• Thorough medical history evaluation
• Assessment of signs and symptoms
• Laboratory tests
• Imaging studies
• Biopsy studies, if necessary

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Corticobasal Degeneration?

The complications of Corticobasal Degeneration may include:

• Aspiration pneumonia
• Intellectual disability
• Poor feeding
• Walking difficulties

Complications may occur with or without treatment, and in some cases, due to treatment also.

How is Corticobasal Degeneration Treated?

There is no cure for Corticobasal Degeneration, since it may be a genetic condition. The treatment is usually given to manage the signs and symptoms and any complications that develops.

How can Corticobasal Degeneration be Prevented?

Currently, Corticobasal Degeneration is not preventable.

• If there is a family history of the condition, then genetic counseling will help assess risks, before planning for a child
• Active research is currently being performed to explore the possibilities for treatment and prevention of inherited and acquired genetic disorders

Regular medical screening at periodic intervals with tests and physical examinations are recommended.

What is the Prognosis of Corticobasal Degeneration? (Outcomes/Resolutions)

• People with Corticobasal Degeneration usually do not survive beyond an average of 7 years after symptoms begin
• Aspiration pneumonia or other complications are usually the cause of death

(Source: Corticobasal Degeneration; Genetic and Rare Diseases Information Center (GARD) of National Center for Advancing Translational Sciences (NCATS), USA.)

Additional and Relevant Useful Information for Corticobasal Degeneration:

The following DoveMed website link is a useful resource for additional information:

https://www.dovemed.com/diseases-conditions/rare-disorders/