20th July 2023

Corticobasal Degeneration (CBD)

A rare neurodegenerative disease involving the cerebral cortex and basal ganglia, manifesting as a gradual onset movement and cognitive disorder. It typically occurs in those aged 50-70 years, and it tends to progress over 6-8 years.

Patients find difficulties in cortical processing and basal ganglia dysfunction. Symptoms include bradykinesia, apraxia, aphasia, limb dystonia, parkinsonism, and myoclonus. These can present unilaterally initially, which then become bilateral. Another important feature is alien-limb phenomena. The pathophysiology behind this is an accumulation of tau proteins and astrocyte inclusions, which cause astrocytic plaques which appear as blurry outgrowths from the astrocyte. Unfortunately, there are no known treatments which slow the course of the disease. Parkinson’s drugs don’t show sustained improvement though clonazepam can be used for myoclonus. Otherwise an MDT approach, including speech therapy, is used to try to manage the symptoms of the disease. Patients typically die from complications such as pneumonia.