[Dementia in progressive supranuclear paralysis patients]

Progressive supranuclear paralysis is difficult to detect in its initial stages. Its symptoms are not specific and often the patients are mistaken as Parkinson s disease patients, or even Alzheimer s ones.

Initial features in progressive supranuclear paralysis are typically a gait disorder with frequent falls, behavioral disorder and often dysarthria. The gaze disorder, showing voluntary conjugate gase paralysis is characteristic, but it can appear late in the course of disease. Other motor signs are axial rigidity, bradykinesia, stiffness, and rarely chorea, ataxia, muscle weakness or myoclonus. Pseudobulbar paralysis is the more remarkable clinical finding. These motor symptoms are accompanied by cognitive disturbances, the typical ones of subcortical dementia, with mind slowness, apathy, depression, and, more often that in other primary dementias, psychotic features and relatively early behavioral disorders. A useful feature to distinguish the mental changes of progressive supranuclear paralysis with other primary subcortical dementias is a more remarkable impairment in visuospatial function, because of the need of a good gaze function to accomplish it. Progressive supranuclear paralysis patients work better in verbal tests that in visuospatial test. We try to describe clinical data and evolutive profile of this entity, whose cognitive disturbances as described by Albert in 1974 strongly stabilized the concept of subcortical dementia.