What is the definition of Corticobasal Degeneration?
Corticobasal degeneration 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.
What are the alternative names for Corticobasal Degeneration?
- Cortical-Basal Ganglionic degeneration
- Cortico-Basal Ganglionic Degeneration (CBGD)
- Corticobasal syndrome
What are the causes for Corticobasal Degeneration?
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.
Is Corticobasal Degeneration an inherited disorder?
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.