Corticobasal Degeneration

Synonyms

Corticobasal syndrome; Corticodentatonigral degeneration with neuronal achromasia

Short Description or Definition

First described as corticodentatonigral degeneration with neuronal achromasia by Rebeiz et al. in 1968, corticobasal degeneration (CBD) was long thought to be predominantly a motor disorder. Indeed, the original description of the disorder emphasized the relative preservation of mental faculties. More recently, emphasis has been placed on the neurobehavioral features of CBD, and the overlapping clinical and neuropathological features of CBD with frontotemporal lobar degenerations continue to generate debate about the classification and nosology of the disorder.

The motor presentation of CBD most often involves an asymmetric, progressive, akinetic-rigid parkinsonism of gradual onset that responds minimally if at all to levodopa and is sometimes accompanied by dystonia or myoclonus. Cortical signs that are common in CBD include asymmetric apraxia, cortical sensory...