Progressive supranuclear palsy.

Abstract

Progressive supranuclear palsy has been recognized as a distinct nosological entity for about three decades now. Typically, this progressive neurological disease manifests itself late in the sixth decade with a terminal course of approximately four to six years. Well over one hundred cases have been described in the literature and the heterogeneous nature of progressive supranuclear palsy includes the characteristic vertical ophthalmoplegia, frequent falling and a profound nuchal rigidity. Other features are similar in many respects to those found in Parkinson's disease. The present article reviews the literature on progressive supranuclear palsy with particular reference to its clinical manifestations including the ophthalmoplegia, characteristic sleep disturbances and unique dementia. Also addressed, are neuropathological and epidemiological findings. Finally, conclusions and recommendations for further investigation are offered especially with regard to the neuropsychological nature of this neurological disorder.