Progressive supranuclear palsy

Handbook of Atypical Parkinsonism, eds. Carlo Colosimo, David E. Riley, and Gregor K. Wenning. Published by Cambridge University Press. © Cambridge University Press 2011. Historical review Although the earlier literature contained several accounts of progressive supranuclear palsy (PSP) [ 1 ], including the famous case reported by Charcot in the nineteenth century [ 2 ], the fi rst detailed description of this condition came from John Cliff ord Richardson, John Steele, and Jerzy Olszewski in the early 1960s [ 3 ]. Th e term PSP was introduced by the same Canadian investigators in 1964 [ 4 ]. Th ey described a pre-senile sporadic neurodegenerative disease characterized clinically by a combination of supranuclear vertical ophthalmoplegia, pseudobulbar palsy, nuchal dystonia, and dementia. In their report they described the clinical features of nine patients and reported the neuropathological changes in seven of them. Th e predominant pathological changes were cell loss and gliosis in the brainstem, basal ganglia, and cerebellum. In 1986, Pollock and colleagues fi rst reported that the fi lamentous aggregates found in the brains of patients with PSP shared antigenic determinants with microtubule-associated protein tau [ 5 ]. Histopathological studies then confi rmed that PSP is a tauopathy characterized by hyperphosphorylated tau protein deposition forming fi brillary aggregates (globose neurofi brillary tangles) in neurons and glia of numerous cerebral areas including the cerebral neocortex, pallidum, subthalamic nucleus, substantia nigra, periacqueductal gray matter, superior collicula, and dentate nucleus [ 6 ]. Th e abundant presence of these aggregates in all clinical PSP subtypes suggested that PSP should be considered a tauopathy along with corticobasal degeneration (CBD), frontotemporal dementia (FTD), and Alzheimer’s disease (AD) [ 7 ]. Th e introduction of clinical diagnostic criteria, fi rst proposed by Lees in 1987, made it easier to identify these patients clinically [ 8 ]. Others subsequently proposed diff erent sets of diagnostic criteria, mainly based on personal experience [ 9 – 11 ]. New clinical diagnostic criteria were developed in 1996 during a workshop held under the auspices of the National Institute for Neurological Disorders and Stroke and the Society for PSP (NINDS-SPSP) [ 12 ]. Th is time the criteria were also validated on a clinical data set from autopsy-confi rmed cases of PSP. Although the NINDS-SPSP criteria ( Table 4.1 ) have gained wide acceptance in the research community, in movement disorder clinics, they remain to be validated prospectively .