Corticobasal degeneration: An update

Abstract

Corticobasal degeneration (CBD) is one of the primary tauopathies with a disease onset in the 5th to 7th decade. CBD is a progressive condition of unknown aetiology, which is characterised neuropathologically by neuronal loss, astrogliosis and deposition of filamentous tau inclusions, composed entirely of 4-repeat tau isoforms, in neurons and glial cells in cerebral cortical areas, basal ganglia, brainstem and cerebellar nuclei. The term CBD is now a neuropathological diagnostic one and for the canonical clinical syndrome associated with CBD neuropathological changes, the corticobasal syndrome (CBS) term is used. In addition to CBS, the clinical spectrum also includes a behavioural variant of frontotemporal dementia syndrome, speech disorders, Richardson’s syndrome and, rarely, posterior cortical syndrome. In addition to CBD, CBS can also be caused by other pathologies. A number of genetic risk factors of CBD have been identified. As specific biomarkers confirming CBD as the underlying pathology responsible for CBS or other clinical manifestations are still lacking, for a definitive diagnosis of CBD neuropathological investigation is required. Recent cryo-electron microscopic studies have proven that CBD is a distinct tauopathy associated with a unique molecular structure of the tau filaments, which firmly differentiates it from other primary tauopathies.