Corticobasal Degeneration
Corticobasal degeneration is a progressive neurological disorder characterized by nerve cell loss and atrophy (shrinkage) of multiple areas of the brain including the cerebral cortex and the basal ganglia. Corticobasal degeneration progresses gradually. Initial symptoms, which typically begin at or around age 60, may first appear on one side of the body (unilateral), but eventually affect both sides as the disease progresses. Symptoms are similar to those found in Parkinson disease, such as poor coordination, akinesia (an absence of movements), rigidity (a resistance to imposed movement), disequilibrium (impaired balance); and limb dystonia (abnormal muscle postures). Other symptoms such as cognitive and visual-spatial impairments, apraxia (loss of the ability to make familiar, purposeful movements), hesitant and halting speech, myoclonus (muscular jerks), and dysphagia (difficulty swallowing) may also occur. An individual with corticobasal degeneration eventually becomes unable to walk.
Treatment
There is no treatment available to slow the course of corticobasal degeneration, and the symptoms of the disease are generally resistant to therapy. Drugs used to treat Parkinson disease-type symptoms do not produce any significant or sustained improvement. Clonazepam may help the myoclonus. Occupational, physical, and speech therapy can help in managing disability.
Prognosis
Corticobasal degeneration usually progresses slowly over the course of 6 to 8 years. Death is generally caused by pneumonia or other complications of severe debility such as sepsis or pulmonary embolism.
Research
The NINDS supports and conducts research studies on degenerative disorders such as corticobasal degeneration. The goals of these studies are to increase scientific understanding of these disorders and to find ways to prevent, treat, and cure them.
Organizations
National Organization for Rare Disorders (NORD)
Federation of voluntary health organizations dedicated to helping people with rare "orphan" diseases and assisting the organizations that serve them. Committed to the identification, treatment, and cure of rare disorders through programs of education, advocacy, research, and service.
55 Kenosia Avenue
Danbury, CT 06810
orphan@rarediseases.org
http://www.rarediseases.org
Tel: Danbury
Fax: 203-798-2291
CUREPSP (Foundation for PSP|CBD and Related Brain Diseases)
A nonprofit voluntary health organization whose mission is to increase awareness of progressive supranuclear palsy, corticobasal degeneration and related brain diseases (including atypical parkinsonisms); fund research toward cure and prevention; educate healthcare professionals; and provide support, information and hope for affected persons and their families.
30 E. Padonia Road, Ste. 201
Timonium, MD 21093
info@curepsp.org
http://www.curepsp.org
Tel: Timonium
Fax: 410-785-7009
Association for Frontotemporal Degeneration (AFTD)
Nonprofit organization that promotes and funds research into finding the cause and cure for frontotemporal degeneration, also called frontotemporal dementia (FTD); provides information, education, and support to those affected by FTD and their caregivers; and sponsors professional health education programs related to FTD.
Radnor Station Building #2
Suite 320
290 King of Prussia Road
Radnor, PA 19087
info@theaftd.org
http://www.theaftd.org
Tel: Radnor
Content Provided By
NINDS Disorders is an index of neurological conditions provided by the National Institute of Neurological Disorders and Stroke. This valuable tool offers detailed descriptions, facts on treatment and prognosis, and patient organization contact information for over 500 identified neurological disorders.
