Cortical Basal Ganglion Degeneration
What is Cortical Basal Ganglion Degeneration
Cortical Basal Ganglion 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.
Cortical Basal Ganglion 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.
Is there any treatment?
There is no treatment available to slow the course of Cortical Basal Ganglion 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 the disability.
What is the prognosis?
Cortical Basal Ganglion 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.
What research is being done?
The NINDS supports and conducts research studies on degenerative disorders such as cortico basal degeneration. The goals
of these studies are to increase scientific understanding of these disorders and to find ways to prevent, treat, and cure them.