Progressive Supranuclear Palsy

Abstract

Progressive supranuclear palsy (PSP) is the second most common cause of parkinsonism after Parkinson's disease (PD). The classic syndrome of PSP is widely recognized by neurologists as a combination of down gaze palsy with progressive rigidity and imbalance leading to falls. At the same time, few clinicians are proficient at treating PSP and recognizing the nonclassic presentations of this debilitating disorder often resulting in delays in diagnosis and misguided treatment. Over the last decade many lines of investigation have helped refine PSP at the clinical, neuroimaging, pharmacologic and molecular levels. It is the purpose of this literature review to help clinicians identify PSP earlier in its course, to better understand its pathophysiology, and to provide a more focused, symptom-based treatment approach. Eighty-two peer-reviewed articles on the topic of PSP and other neurodegenerative disorders have been reviewed. It is clear that PSP continues to be an under-recognized disorder with multilevel involvement of the neuraxis that helps differentiate it from other akinetic rigid syndromes such as PD. A greater appreciation of its atypical presentations, more attention to its neurobehavioral signs and better imaging techniques are some of the advances that will help facilitate earlier detection, which may reduce morbidity by helping anticipate early falls and minimizing unnecessary diagnostic procedures. Surgical approaches to PSP have been ineffective so far. Carefully targeted symptomatic treatment with drugs and other therapies is available and effective at reducing morbidity and improving quality of life.