What is corticobasal degeneration (CBD)?
Patients with Corticobasal Degeneration display a combination of cortical (pyramidal signs, myoclonus, progressive aphasia, and apraxia) and subcortical findings (rigidity and dystonia) as well as a distinctive alien limb sign.
Corticobasal Degeneration is virtually the only disease that causes this constellation of symptoms and signs, although there appears to be an overlap of CBD, progressive aphasia, and frontotemporal dementia.
Until late stages of the disease, patients do not experience cognitive decline or dysautonomia.
Convergence disturbances and oculomotor apraxia are common neuro-ophthalmic signs.
The neuropathologic hallmarks are swollen achromatic neurons, neuronal loss, gliosis in the cerebral cortex, SN, lateral nuclei of the thalamus, striatum, locus ceruleus, and Purkinje layer of the cerebellum.
The cause is entirely obscure. No familial forms have been reported.
The disease progresses relentlessly until death, usually within 10 years after onset. Response to dopaminergic therapy is usually poor.