National Center for Advancing and Translational Sciences Genetic and Rare Diseases Information Center, a program of the National Center for Advancing and Translational Sciences

Corticobasal degeneration


Other Names:
Cortical-Basal Ganglionic degeneration; Cortico-Basal Ganglionic Degeneration (CBGD); CBGD; Cortical-Basal Ganglionic degeneration; Cortico-Basal Ganglionic Degeneration (CBGD); CBGD; Corticobasal syndrome See More
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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.[1] 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.[2]
Last updated: 3/13/2017

This table lists symptoms that people with this disease may have. For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed. This information comes from a database called the Human Phenotype Ontology (HPO) . The HPO collects information on symptoms that have been described in medical resources. The HPO is updated regularly. Use the HPO ID to access more in-depth information about a symptom.

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Medical Terms Other Names
Learn More:
HPO ID
80%-99% of people have these symptoms
Parkinsonism 0001300
Progressive extrapyramidal muscular rigidity 0007158
30%-79% of people have these symptoms
Akinesia 0002304
Bradykinesia
Slow movements
Slowness of movements
[ more ] 		0002067
Dementia
Dementia, progressive
Progressive dementia
[ more ] 		0000726
Frontal release signs 0000743
Gait disturbance
Abnormal gait
Abnormal walk
Impaired gait
[ more ] 		0001288
Limb apraxia 0030217
Limb dystonia 0002451
Limb myoclonus 0045084
Oromotor apraxia 0007301
Postural instability
Balance impairment
0002172
Sensory impairment 0003474
Tremor 0001337
5%-29% of people have these symptoms
Aphasia
Difficulty finding words
Losing words
Loss of words
[ more ] 		0002381
Dysphasia 0002357
Memory impairment
Forgetfulness
Memory loss
Memory problems
Poor memory
[ more ] 		0002354
Speech apraxia 0011098
1%-4% of people have these symptoms
Behavioral abnormality
Behavioral changes
Behavioral disorders
Behavioral disturbances
Behavioral problems
Behavioral/psychiatric abnormalities
Behavioural/Psychiatric abnormality
Psychiatric disorders
Psychiatric disturbances
[ more ] 		0000708
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Last updated: 7/1/2020
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.[3] Tau also appears to play a role in other neurodegenerative diseases such as Alzheimer disease, progressive supranuclear palsy, and frontotemporal dementia.[3][4] There is no evidence to suggest that environmental exposure to toxic or infectious agents plays a role in causing CBD.[4]
Last updated: 3/13/2017
Corticobasal degeneration (CBD) is almost always sporadic, developing by chance rather than being inherited.[4][5] Rare familial cases have been reported, leading to the possibility that there may be a genetic basis for at least a predisposition to CBD.[4] 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.[5]
Last updated: 3/13/2017

If you need medical advice, you can look for doctors or other healthcare professionals who have experience with this disease. You may find these specialists through advocacy organizations, clinical trials, or articles published in medical journals. You may also want to contact a university or tertiary medical center in your area, because these centers tend to see more complex cases and have the latest technology and treatments.

If you can’t find a specialist in your local area, try contacting national or international specialists. They may be able to refer you to someone they know through conferences or research efforts. Some specialists may be willing to consult with you or your local doctors over the phone or by email if you can't travel to them for care.

You can find more tips in our guide, How to Find a Disease Specialist. We also encourage you to explore the rest of this page to find resources that can help you find specialists.

Healthcare Resources

  • You can view a list of frontotemporal dementia research centers on the Frontotemporal Dementia Caregiver Support Center Web site by clicking on "research centers" above.

Research helps us better understand diseases and can lead to advances in diagnosis and treatment. This section provides resources to help you learn about medical research and ways to get involved.

Clinical Research Resources

  • ClinicalTrials.gov lists trials that are related to Corticobasal degeneration. Click on the link to go to ClinicalTrials.gov to read descriptions of these studies.

    Please note: Studies listed on the ClinicalTrials.gov website are listed for informational purposes only; being listed does not reflect an endorsement by GARD or the NIH. We strongly recommend that you talk with a trusted healthcare provider before choosing to participate in any clinical study.
  • Orphanet lists European clinical trials, research studies, and patient registries enrolling people with this condition. 
  • The Research Portfolio Online Reporting Tool (RePORT) provides access to reports, data, and analyses of research activities at the National Institutes of Health (NIH), including information on NIH expenditures and the results of NIH-supported research. Although these projects may not conduct studies on humans, you may want to contact the investigators to learn more. To search for studies, enter the disease name in the "Text Search" box. Then click "Submit Query".

Patient Registry

Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.

Organizations Supporting this Disease

Living with a genetic or rare disease can impact the daily lives of patients and families. These resources can help families navigate various aspects of living with a rare disease.

Financial Resources

These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

Where to Start

In-Depth Information

  • Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
  • The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
  • Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.
  • PubMed is a searchable database of medical literature and lists journal articles that discuss Corticobasal degeneration. Click on the link to view a sample search on this topic.

Questions sent to GARD may be posted here if the information could be helpful to others. We remove all identifying information when posting a question to protect your privacy. If you do not want your question posted, please let us know.

  1. NINDS Corticobasal Degeneration Information Page. National Institute of Neurological Disorders and Stroke. February 6, 2015; https://www.ninds.nih.gov/Disorders/All-Disorders/Corticobasal-Degeneration-Information-Page.
  2. A M Barrett. Cortical Basal Ganglionic Degeneration. Medscape Reference. June 3, 2014; http://emedicine.medscape.com/article/1150039-overview.
  3. Lee S. Corticobasal Degeneration. National Organization for Rare Disorders (NORD). 2012; http://www.rarediseases.org/rare-disease-information/rare-diseases/byID/1009/viewAbstract.
  4. Factor SA, Walls DG. Corticobasal degeneration. UpToDate. Waltham, MA: UpToDate; June 15, 2016;
  5. Corticobasal syndrome. The Association for Frontotemporal Degeneration. 2017; http://www.theaftd.org/understandingftd/disorders/corticobasal-degeneration.