La dégénérescence cortico-basale : une tauopathie qui est bien plus qu'une pathologie du mouvement

Cortico-basal degeneration (CBD) is classified among focal atrophies. Pathologically, it is precisely defined by the presence of neuronal and glial cortico-striatal lesions, immunostained by antibodies specific for tau protein. While the disease was initially described in patients presenting with a predominantly motor syndrome (asymmetric rigidity or dystonia, focal myoclonus) and most often parietal cortical symptoms (unilateral apraxia, sensory disorders, alien hand syndrome), it appears to be very proteiform. There are many sources of anatomo-clinical discrepancies. Thus, it is wiser to coin the name 'corticobasal syndrome' to design the initially described clinical syndrome, and to reserve the term CBD for neuropathologically confirmed cases. The new diagnostic criteria for probable or possible CBD are presented.