Corticobasal Degeneration( CBD)
• Rare neurodegenerative brain disease that affects the cortex( frontoparietal) and basal ganglia
• Originally described in 1968 by Rebeiz, Kolodny and Richardson of Harvard as“ corticodentatonigral degeneration with neuronal achromasia
• Pathologically classified as a tauopathy( accumulation of the tau protein in the brain)
• Onset of symptoms is markedly asymmetrical
• Diagnosis is difficult because clinical features often overlap with Parkinson’ s disease( PD), progressive supranuclear palsy( PSP), Alzheimer’ s, primary progressive aphasia and frontotemporal dementia
• Average age of onset is in the mid-60s
• Prevalence unknown; estimated to be less than one case per 100,000 people
• Slightly more common in women
• Life expectancy averages 7 years after symptom onset
• No known cure or medications to slow disease progression
Signs and Symptoms
• Asymmetrical presentation; symptoms begin on one side, which always remains worse
• Slowness and stiffness
• Shakiness
• Difficulty with fine control of hands and / or feet
• Dysphasia, dysarthria and dysphagia
• Changes in gait and balance
• Mild memory or behavior problems
• Mild to moderate parkinsonian symptoms- stiffness, slowness and balance problems
• Tremor
• Very fast, short involuntary movements called“ myoclonus”
• Dystonia
• Blepharospasm( involuntary eyelid spasm)
• Reduced perception of position of limbs in space
• Increasing speech and swallowing difficulty
• Mild to moderate cognitive impairments
• About half of persons with the“ corticobasal syndrome” actually have a brain condition other than corticobasal degeneration, usually PSP or a form of Alzheimer’ s disease
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