Abstract

BackgroundProgressive supranuclear palsy (PSP) is a neurodegenerative disorder characterized by neuronal loss in the extrapyramidal system with pathologic accumulation of tau in neurons and glia. The most common clinical presentation of PSP, referred to as Richardson syndrome, is that of atypical parkinsonism with vertical gaze palsy, axial rigidity, and frequent falls. Although cognitive deficits in PSP are often ascribed to subcortical dysfunction, a subset of patients has dementia with behavioral features similar to the behavioral variant of frontotemporal dementia. In this study we aimed to identify the clinical and pathological characteristics of PSP presenting with frontotemporal dementia.MethodsIn this study, we compared clinical and pathologic characteristics of 31 patients with PSP with Richardson syndrome with 15 patients with PSP with frontotemporal dementia. For pathological analysis, we used semiquantitative methods to assess neuronal and glial lesions with tau immunohistochemistry, as well image analysis of tau burden using digital microscopic methods.ResultsWe found greater frontal and temporal neocortical neuronal tau pathology in PSP with frontotemporal dementia compared with PSP with Richardson syndrome. White matter tau pathology was also greater in PSP with frontotemporal dementia than PSP with Richardson syndrome. Genetic and demographic factors were not associated with atypical distribution of tau pathology in PSP with frontotemporal dementia.ConclusionsThe results confirm the subset of cognitive‐predominant PSP mimicking frontotemporal dementia in PSP. PSP with frontotemporal dementia has distinct clinical features that differ from PSP with Richardson syndrome, as well as differences in distribution and density of tau pathology. © 2019 The Authors. Movement Disorders published by Wiley Periodicals, Inc. on behalf of International Parkinson and Movement Disorder Society.

Highlights

  • The 15 autopsy-proven Progressive supranuclear palsy (PSP) cases with antemortem clinical diagnosis of behavioral variant FTD (bvFTD) were compared with 31 cases of PSP with Richardson syndrome

  • Oligodendroglial coiled bodies were more numerous in the superior frontal gyrus and middle frontal gyrus of PSP and antemortem clinical features of bvFTD (PSP-FTD) compared with PSP-RS (Table 3). These results suggest that greater neuronal and glial tau pathology in both gray matter and white matter of the frontal and temporal lobes is associated with PSP-FTD

  • Clinical manifestations of PSP-RS are related to motor problems, with frequent falls and vertical gaze palsy[18]; many patients develop cognitive dysfunction that has characteristics of a subcortical dementia, characterized by cognitive dysfunction, bradyphrenia, slow responses, and poor recall.[19]

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Summary

Objectives

In this study we aimed to identify the clinical and pathological characteristics of PSP presenting with frontotemporal dementia. We aimed to better understand clinical and pathologic features of PSP-FTD

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