La paralysie supranucléaire progressive : nouveaux concepts cliniques et neuropathologiques

Progressive supranuclear palsy (PSP) is a neurodegenerative disease, clinically characterized by association of early postural instability, supranuclear palsy and cognitive dysfunction with frontal syndrome. This disease is neuropathologically defined by the accumulation of phosphorylated tau protein in basal ganglia and brainstem. Over the last decade, many studies contributed to redefine PSP clinically and neuropathologically, leading to the description of new phenotypes. These new insights emphasize that abnormal accumulation of tau protein is rather a dynamic process that can develop at variable degree of severity. Despite the existence of a set of diagnosis criteria, the diagnosis is sometimes difficult, particularly in the early stages of the disease. Morphologic and functional imaging can be helpful, while biomarkers because of their low specificity should be carefully interpreted. Treatment is often minimally beneficial, and new therapeutic approaches based on the pathophysiology of the disease are currently ongoing.