David Lichter1, Ahmed Koriesh2, Nidhi Kapoor2 and Linda Hershey2*
1Department of Neurology, SUNY University at Buffalo, USA
2Department of Neurology, University of Oklahoma Health Sciences Center, USA
Progressive Supranuclear Palsy (PSP) is a Parkinson-plus syndrome associated with a variety of different clinical presentations. We describe the clinical and pathological features of the 7 major phenotypes of PSP in addition to new information about the genetic causes of PSP. We also discuss useful imaging tools, and review various management strategies. The classic form of PSP (PSPRichardson syndrome) is more likely to be associated with postural instability, vertical gaze palsy, akinesia and cognitive changes, compared to the milder variants of PSP. There are 6 other variants of PSP besides PSP-RS, including PSP-parkinsonism, which can imitate Parkinson disease and can respond to levodopa for the first 2-3 years. The Microtubular Associated Protein Tau (MAPT) gene has a larger influence in PSP-RS than in PSP-parkinsonism. The rarer cortical variants of PSP (PSPprogressive non-fluent aphasia, PSP-frontal temporal dementia and PSP-cortical basal syndrome) can be mistaken for other neurodegenerative diseases, since the classic PSP signs may not appear for months to years after the cortical signs present themselves. MRI and Diffusion Tensor Imaging (DTI) scans are useful tools in assessing patients with signs of PSP. There is currently no disease modifying therapy available for PSP, but many signs of PSP can be managed with symptomatic treatments and various non-pharmacologic approaches
Lichter D, Koriesh A, Kapoor N, Hershey L. Progressive Supranuclear Palsy: New Diagnostic and Therapeutic Strategies. Am J Gerentol Geriatr. 2018; 1(2): 1007.