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 include signs of parkinsonism such as poor coordination, akinesia (an absence of movements), rigidity (a resistance to imposed movement), and 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, and dysphagia (difficulty swallowing) may also occur. The patient is unable to walk. Symptoms vary among patients.

Is there any treatment?
There is no treatment available to slow the course of corticobasal degeneration, and the symptoms of the disease are generally resistant to therapy. Antiparkinsonian drugs do not produce any significant or sustained improvement. Clonazepam may help the myoclonus. Occupational, physical, and speech therapy may help in managing disability.

What is the prognosis?
The course of corticobasal degeneration is one of inexorable progression until death, usually 6 to 8 years after diagnosis. Death is generally caused by pneumonia or other complications of severe debility such as sepsis or pulmonary embolism.

Information provided by the
National Institute of Neurological Disorders and Stroke,
National Institutes of Health

Each year, Medical College of Wisconsin physicians care for more than 180,000 patients, representing nearly 500,000 patient visits. Medical College physicians practice at Children's Hospital of Wisconsin, Froedtert Memorial Lutheran Hospital, the Milwaukee VA Medical Center, and many other hospitals and clinics in Milwaukee and southeastern Wisconsin.