Clinical Short CommunicationNatural history and predictors of survival in progressive supranuclear palsy
Introduction
Progressive supranuclear palsy (PSP) is a neurodegenerative disorder characterized by parkinsonism, supranuclear gaze palsy and postural instability associated with frequent falls [1].
Clinical progression is associated with high functional disability leading to dependency in activities of daily living. Few available studies have estimated the average survival to range from 5 to 10 years. However, several studies have been hindered by small samples sizes and by the lack of a comprehensive neuropsychological evaluation as possible predictors of survival probability. Older age at symptom onset, male sex, clinical phenotype, presence of falls at the early disease stages, bulbar symptoms or sleep disturbances have been variably associated with a shorter survival [2], [3], [4], [5], [6], [7]. Few studies considered also the presence of cognitive deficits as possible predictors of reduced survival, providing contrasting results, possibly due to the lack of extensive neuropsychological assessments [2], [3], [5], [7], [8]. Indeed, cognitive decline frequently occurs in PSP, so that the recently revised criteria have included cognitive assessment in the diagnostic work-up [9].
The aim of the present study was to evaluate the natural history and predictors of survival in a large cohort of PSP patients.
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Subjects
Patients fulfilling clinical criteria for probable PSP-Richardson Syndrome [1] were consecutively recruited from the Centre for Ageing Brain and Neurodegenerative Disorders, Department of Clinical and Experimental Sciences, University of Brescia, Italy, from December 2001 to July 2016. All patients underwent a neurological, neuropsychological and behavioral evaluation, and routine laboratory examination.
The neuropsychological assessment included the Mini-Mental State Examination (MMSE) [10]
Results
The analysis was carried out on 100 PSP patients. The average age at symptom onset was 69.6 ± 6.3 years, the age at diagnosis was 72.8 ± 6.1 years and 51% of patients were male. At the time of enrollment, PSP patients had an average MMSE score of 23.19 ± 6.39, mainly affected by visuospatial deficits, as documented by the Rey copy figure and Clock drawing test, and attentive/executive disturbances, as documented by the Trail Making A and B tests (TMT A and B). 39% patients were classified as having
Discussion
We analyzed the natural course and prognostic factors associated with survival in a large group of PSP patients. The demographic features were similar to those reported in previous studies. Notably, diagnosis occurred late in the course of the disease, almost three and half years from symptom onset [3]. The mean survival time from the onset of symptoms was comparable to previous reports [3], [4], [6]. The only significant risk factor associated with survival probability, in our cohort, was the
Author disclosures
All authors report no disclosures.
Acknowledgements
The authors are indebted to Dr. Michele Magoni, from the Local Health Agency of Brescia Province, for providing survival data.
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