Progressive supranuclear palsy: managing the disabilities and providing nursing support.

ABSTRACT

Progressive Supranuclear Palsy is an uncommon progressive neurodegenerative disorder which may initially present as Parkinson's disease. It is characterized by an abnormality of voluntary eye movement, pseudobulbar palsy, axial dystonia/rigidity, postural instability, mental changes and bradykinesia. The symptoms may be present for up to 4.5 years before diagnosis with an average survival rate after diagnosis of about two years. However, the diagnosis is often make much sooner when the individual is assessed in a specialty Movement Disorder Clinic. At the time the diagnosis is made the individual and family will have no awareness of the devastating impact this illness will have on their lives. Often they do not understand the mechanisms of deterioration, nevertheless, they must learn how to manage the severe disabilities over a short time period. This article will address the symptomatology of Progressive Supranuclear Palsy, how it differs from Parkinson's disease, the pathophysiology, treatment, recommendations for managing the disabilities, providing nursing support, care for the caregiver, future care planning, the legal options of Enduring Power of Attorney, Trusteeship, Guardianship, Advance Directives, and consideration for autopsy.