[Cortico-basal degeneration: a new entity]

Abstract

Cortico-basal degeneration is a progressive disease comprisong characteristic features concerning both cortical and basal ganglionic dysfunction. Manifestations include akineto-rigid syndrome and apraxia strictly limited at disease onset to one hemibody, especially the left. Myoclonus, chorea a alien limb syndrome can begin within the clinicalcome. Functional exploration of the brain shows an asymmetric hypometabolism affecting both cortical and basal structures. Neuropathologic studies show pathological features including neuronal loss, gliosis and neuronal achromasia affecting the cortex (frontal and parietal), basal ganglia and locus niger. Further evaluation of this disease is needed to understand its links with the other neurological degenerative diseases especially progressive aphasia and Pick's disease.