Overview

Corticobasal degeneration, also called corticobasal syndrome, is a rare disease that causes areas of the brain to shrink. Over time, nerve cells break down and die.

Corticobasal degeneration affects the area of the brain that processes information and brain structures that control movement. People with this disease have trouble with movement on one or both sides of the body. Trouble with movement gets worse over time.

Symptoms also may include poor coordination, stiffness, trouble thinking, and trouble with speech or language.

Symptoms

Symptoms of corticobasal degeneration (corticobasal syndrome) include:

  • Trouble moving on one or both sides of the body, which gets worse over time.
  • Poor coordination.
  • Trouble with balance.
  • Stiffness.
  • Postures of the hands or feet that can't be controlled. For example, the hand may form a clenched fist.
  • Muscle jerks.
  • Trouble swallowing.
  • Changes in eye movements.
  • Trouble with thinking and language skills.
  • Speech changes, such as slow and halting speech.

Corticobasal degeneration gets worse over 6 to 8 years. Eventually, people with the disease lose the ability to walk.

Causes

Corticobasal degeneration (corticobasal syndrome) can have several causes. Most commonly, the disease results from a buildup of a protein called tau in brain cells. The buildup of tau may lead to the breakdown of the cells. This can cause symptoms of corticobasal degeneration.

Half of the people who have symptoms have corticobasal degeneration. But the second most common cause of corticobasal degeneration symptoms is Alzheimer's disease. Other causes of corticobasal degeneration include progressive supranuclear palsy, Pick's disease or Creutzfeldt-Jakob disease.

Risk factors

There are no known risk factors for corticobasal degeneration (corticobasal syndrome).

Complications

People with corticobasal degeneration (corticobasal syndrome) may develop serious complications. People with the disease may develop pneumonia, blood clots in the lungs or a dangerous response to an infection, known as sepsis. Complications often lead to death.

Dec. 22, 2023
  1. Factor SA, et al. Corticobasal degeneration. https://www.uptodate.com/contents/search. Accessed Oct. 25, 2023.
  2. Ropper AH, et al. Degenerative diseases of the nervous system. In: Adams and Victor's Principles of Neurology. 12th ed. McGraw Hill; 2023. https://accessmedicine.mhmedical.com. Accessed Oct. 25, 2023.
  3. Corticobasal degeneration. National Institute of Neurological Disorders and Stroke. https://www.ninds.nih.gov/Disorders/All-Disorders/Corticobasal-Degeneration-Information-Page. Accessed Oct. 25, 2023.
  4. Jankovic J, et al., eds. Alzheimer's disease and other dementias. In: Bradley and Daroff's Neurology in Clinical Practice. 8th ed. Elsevier; 2022. https://www.clinicalkey.com. Accessed Oct. 25, 2023.
  5. Budson AE, et al. Corticobasal degeneration and corticobasal syndrome. In: Memory Loss, Alzheimer's Disease, and Dementia. 3rd ed. Elsevier; 2022. https://www.clinicalkey.com. Accessed Oct. 25, 2023.
  6. Koga S, et al. Neuropathology and emerging biomarkers in corticobasal syndrome. Journal of Neurology, Neurosurgery and Psychiatry. 2022; doi:10.1136/jnnp-2021-328586.
  7. Alzheimer's disease research centers. National Institute on Aging. https://www.nia.nih.gov/health/alzheimers-disease-research-centers. Accessed Oct. 25, 2023.
  8. Constantinides VC, et al. Corticobasal degeneration and corticobasal syndrome: A review. Clinical Parkinsonism & Related Disorders. 2019; doi:10.1016/j.prdoa.2019.08.005.
  9. Mena AM, et al. Imaging pathological tau in atypical parkinsonisms: A review. Clinical Parkinsonism and Related Disorders. 2022; doi:10.1016/j.prdoa.2022.100155.
  10. Ami TR. Allscripts EPSi. Mayo Clinic. Sept. 4, 2023.
  11. Graff-Radford J, et al. New insights into atypical Alzheimer's disease in the era of biomarkers. Lancet Neurology. 2021; doi:10.1016/S1474-4422(20)30440-3.
  12. Shir D, et al. Clinicoradiologic and neuropathologic evaluation of corticobasal syndrome. Neurology. 2023; doi:10.1212/WNL.0000000000207397.
  13. Graff-Radford J (expert opinion). Mayo Clinic. Nov. 2, 2023.

Related

Products & Services

Corticobasal degeneration (corticobasal syndrome)