Corticobasal degeneration (CBD), also known as corticobasal ganglionic degeneration, is a rare, progressive neurological disorder characterized by brain cell shrinkage and loss, as well as the buildup of tau protein deposits. CBD is often categorized as a type of frontotemporal dementia.
Symptoms of CBD
CBD shares several symptoms with Parkinson’s disease. Early symptoms include slow or clumsy movements, tremors or shakiness, muscle weakness, and stiffness. The individual with CBD often initially has symptoms only on one side of the body. Later, as the disease progresses, both sides are affected.CBD also affects the ability to speak and understand what others are saying, and can affect memory and the ability to do mathematical calculations. Behavioral components, such as compulsions, socially inappropriate behaviors, and repetitive actions, may also be present.
Who Gets CBD?
Researchers have not yet determined what causes CBD, although it does not appear to be an inherited disorder. The average age of onset is around 60. There are an estimated 2,000 to 3,000 Americans with this disorder, although it's possible that there are others who have an inaccurate diagnosis, since CBD is often initially misdiagnosed as Parkinson's or Alzheimer's disease.Treatment of CBD
As with the other disorders in the frontotemporal dementia group, treatment of CBD is limited. Medications that may help relieve some of the symptoms include cognitive enhancers usually prescribed for Alzheimer’s patients, such as cholinesterase inhibitors. Other people show some improvement in their physical abilities with medications like Sinemet, a drug often used in Parkinson's, Baclofen (to help reduce muscle rigidity or tightness), or Clonazepam, which may help decrease the muscle jerks.
Physical, occupational, and speech therapy may also be ordered by the physician to try to improve the motor functioning and speech, as well as prevent muscle contractures.
Prognosis
CBD is a progressive disease that often causes immobility after about five years; within 10 years, the person may succumb to complications such as a pulmonary embolism, pneumonia, or another infection.
Sources:
The Association for Frontotemporal Degeneration. Corticobasal Degeneration. Accessed January 27, 2012. http://www.theaftd.org/
National Center for Biotechnology Information. U.S. National Library of Medicine. Pub Med Health. Pick’s Disease. http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0001752/
National Institute of Neurological Disorders and Stroke. National Institutes of Health. Corticobasal Degeneration Information Page. Accessed December 11, 2011. http://www.ninds.nih.gov/disorders/corticobasal_degeneration/corticobasal_degeneration.htm
University of California, San Francisco. Forms of Frontotemporal Dementia. Accessed December 11, 2011. http://memory.ucsf.edu/ftd/overview/ftd/forms/multiple
University of California, San Francisco. Corticobasal Degeneration Signs and Symptoms. Accessed December 12, 2011. http://www.ucsfhealth.org/conditions/corticobasal_degeneration/signs_and_symptoms.html
University of California San Francisco. Corticobasal Degeneration Treatment. Accessed December 16, 2011. http://www.ucsfhealth.org/conditions/corticobasal_degeneration/treatment.html
U.S. National Institutes of Health. National Institute on Aging. Types of Frontotemporal Disorders. Accessed December 12, 2011. http://www.nia.nih.gov/Alzheimers/Publications/FTLD/02.htm
Worldwide Education and Awareness for Movement Disorders. Symptoms of Corticobasal Degeneration. Accessed January 27, 2012. http://www.wemove.org/cbd/cbd_sym.html
