Clinical Approach to Progressive Supranuclear Palsy

Abstract

Sixty years ago, Steele, Richardson and Olszewski designated progressive supranuclear palsy (PSP) as a new clinicopathological entity in their seminal paper. Since then, in addition to the classic Richardson's syndrome (RS), different clinical phenotypic presentations have been linked with this four-repeat tauopathy. The clinical heterogeneity is associated with variability of regional distribution and severity of abnormal tau accumulation and neuronal loss. In PSP subtypes, the presence of certain clinical pointers may be useful for antemortem prediction of the underlying PSP-tau pathology. Midbrain atrophy on conventional MRI correlates with the clinical phenotype of RS but is not predictive of PSP pathology. Cerebrospinal fluid biomarkers and tau ligand positron emission tomography are promising biomarkers of PSP. A multidisciplinary approach to meet the patients' complex needs is the current core treatment strategy for this devastating disorder.

Keywords: Atypical parkinsonism; Corticobasal syndrome; Progressive supranuclear palsy; Richardson’s syndrome; Tauopathy.

Figure 1.

Tau immunohistochemistry using anti-tau (AT8) antibody shows tufted astrocytes in the frontal cortex of a case with pathologically confirmed progressive supranuclear palsy (× 20 magnification).