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Which Ante Mortem Clinical Features Predict Progressive Supranuclear Palsy Pathology?

Respondek, G; Kurz, C; Arzberger, T; Compta, Y; Englund, E; Ferguson, LW; Gelpi, E; ... Hoeglinger, GU; + view all (2017) Which Ante Mortem Clinical Features Predict Progressive Supranuclear Palsy Pathology? Movement Disorders , 32 (7) pp. 995-1005. 10.1002/mds.27034.

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Abstract

BACKGROUND: Progressive supranuclear palsy (PSP) is a neuropathologically defined disease presenting with a broad spectrum of clinical phenotypes. OBJECTIVE: To identify clinical features and investigations that predict or exclude PSP pathology during life, aiming at an optimization of the clinical diagnostic criteria for PSP. METHODS: We performed a systematic review of the literature published since 1996 to identify clinical features and investigations that may predict or exclude PSP pathology. We then extracted standardized data from clinical charts of patients with pathologically diagnosed PSP and relevant disease controls and calculated the sensitivity, specificity, and positive predictive value of key clinical features for PSP in this cohort. RESULTS: Of 4166 articles identified by the database inquiry, 269 met predefined standards. The literature review identified clinical features predictive of PSP, including features of the following 4 functional domains: ocular motor dysfunction, postural instability, akinesia, and cognitive dysfunction. No biomarker or genetic feature was found reliably validated to predict definite PSP. High-quality original natural history data were available from 206 patients with pathologically diagnosed PSP and from 231 pathologically diagnosed disease controls (54 corticobasal degeneration, 51 multiple system atrophy with predominant parkinsonism, 53 Parkinson's disease, 73 behavioral variant frontotemporal dementia). We identified clinical features that predicted PSP pathology, including phenotypes other than Richardson's syndrome, with varying sensitivity and specificity. CONCLUSIONS: Our results highlight the clinical variability of PSP and the high prevalence of phenotypes other than Richardson's syndrome. The features of variant phenotypes with high specificity and sensitivity should serve to optimize clinical diagnosis of PSP.

Type: Article
Title: Which Ante Mortem Clinical Features Predict Progressive Supranuclear Palsy Pathology?
DOI: 10.1002/mds.27034
Publisher version: http://doi.org/10.1002/mds.27034
Language: English
Additional information: This version is the author accepted manuscript. For information on re-use, please refer to the publisher’s terms and conditions.
Keywords: Science & Technology, Life Sciences & Biomedicine, Clinical Neurology, Neurosciences & Neurology, Progressive supranuclear palsy, clinical features, diagnosis, clinico-pathological series, systematic review, RICHARDSON-OLSZEWSKI SYNDROME, FRONTOTEMPORAL LOBAR DEGENERATION, MULTIPLE SYSTEM ATROPHY, NINDS NEUROPATHOLOGIC CRITERIA, CORTICOBASAL DEGENERATION, DIAGNOSTIC-CRITERIA, NATURAL-HISTORY, FAMILIAL AGGREGATION, PARKINSONS-DISEASE, NONFLUENT APHASIA
UCL classification: UCL > Provost and Vice Provost Offices
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences > UCL Queen Square Institute of Neurology
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences > UCL Queen Square Institute of Neurology > Clinical and Movement Neurosciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences > UCL Queen Square Institute of Neurology > Department of Neuromuscular Diseases
URI: http://discovery.ucl.ac.uk/id/eprint/1556877
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