The clinical features and natural history of the Steele‐Richardson‐Olszewski syndrome (progressive supranuclear palsy)

Abstract

We analyzed the clinical features and natural history of 52 consecutive patients with the Steele-Richardson-Olszewski syndrome. The most common symptoms at onset were unsteady gait, backward falls because of poor balance, visual disturbances, slurred speech, and forgetfulness. The median duration from onset to death was 5.9 years, with a median survival after diagnosis of only 1.8 years. Bronchopneumonia was the usual cause of death recorded on death certificates.

The Clinical Features of 67 Patients with Clinically Definite Steele-Richardson-Olszewski Syndrome

“…Subsequently, numerous cases have been reported in the literature (Brusa et at., 1980;Kristensen, 1985;Maher and Lees, 1986), confirming that this entity is a disease of world-wide distribution, occurring in the presenium, relentlessly progressive and leading to death usually within 5-9 years of diagnosis (David et at., 1968;Golbe et at., 1988). Following the original complete anatorno-clinical description, as predicted by Richardson, the clinical and pathological spectrum has been broadened.…”

“…The diagnostic criteria used were those defined by Maher and Lees (1986). Twenty-one patients in this series were already included in a previous study (Maher and Lees, 1986).…”

“…The diagnostic criteria used were those defined by Maher and Lees (1986). Twenty-one patients in this series were already included in a previous study (Maher and Lees, 1986). All patients had supranuclear palsy of vertical gaze and at least one of the following features: pseudobulbar palsy, dementia, axial or limb rigidity, bradykinesia, pyramidal signs, or gait disorders.…”

Diffusion Tensor Imaging Shows Different Topographic Involvement of the Thalamus in Progressive Supranuclear Palsy and Corticobasal Degeneration

“…3 Although diagnostic criteria are based on motor impairment, the clinical hallmarks of these conditions, 4,5 in both disorders cognitive decline and behavioral abnormalities are always present and dementia is frequent. 6,7 In PSP, anterior frontal lobe dysfunction is the most disabling cognitive deficit, partly due to marked deafferentation of the prefrontal areas, resulting from degeneration of striatothalamocortical pathways. 8,9 The circuits implicated in behavioral control involve the thalamus, mainly the medial thalamus connected to the fronto-orbital cortex and the anterior thalamus connected to the cingulate gyrus.…”

An uncommon presentation of Progressive Supranuclear Palsy diagnosed on DaT scan

“…In PSP, the response to levodopa is typically poor, but about 20% of patients have some improvement initially or in the early stages of the disease [1]. The overall prognosis of PSP is poor because of its progressive nature, with death occurring at a median of six years after onset of disease [5].…”