Progressive Supranuclear Palsy

Abstract

Progressive supranuclear palsy (PSP) has been reported as a disorder mainly characterized by a tendency to fall, vertical supranuclear gaze palsy, and constriction of the body axis, akinesia, and cognitive impairment. Patients presenting with these typical clinical features are diagnosed as having the PSP-Richardson syndrome (PSP-RS). Electronystagmography (ENG) is useful for the detection of ocular motility disorder, which is the main feature of PSP. In cases of PSP, ocular motility disorder in the vertical direction occurs from the initial stage of the disease, and is characterized by prolonged latency and reduced velocity of the saccadic eye movement in the vertical direction. Horizontal saccadic eye movement disorder also occurs after the intermediate stage. Square wave jerks (SWJ) are often recognized from the initial stage of the disease. In terms of nystagmus, the quick phase is impaired due to impaired saccadic eye movements, and impairment of optokinetic nystagmus also starts from the quick phase movements. Pursuit (smooth) eye movement may also be impaired as the disease progresses. On the other hand, the vestibulo-ocular reflex is maintained even after disease progression to a late stage.