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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.
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.
Major Research on Disease:
There are few national and international research organizations conducting 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. 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.
There were 151 patients with "probable CBD", comprising 121 patients with classical form, 17 with quasi-classical form and 13 with non-classical from, while patients with "definite CBD" were 13. The number of patients with progressive supranuclear palsy (PSP) was also examined. The ratio of the number of patients CBD/PSP was 1/2.6 in clinical cases and 1/2.5 in autopsy cases. Nakashima et al. performed population survey of PSP patients in Yonago city in 1999, demonstrating prevalence of PSP 4.36/100,000. Considering this rate, the prevalence of CBD is assumed as 1.7 and the presumed number of PSP and CBD patients in 1999 in Japan could be 5,500 and 2,100, respectively.