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Mov Disord. 2017 Jul;32(7):995-1005. doi: 10.1002/mds.27034. Epub 2017 May 13.

Which ante mortem clinical features predict progressive supranuclear palsy pathology?

Author information

1
Department of Neurology, Technische Universität München, Munich, Germany.
2
German Center for Neurodegenerative Diseases, Munich, Germany.
3
Department of Psychiatry, Ludwig-Maximilians-Universität, Munich, Germany.
4
Center for Neuropathology and Prion Research, Ludwig-Maximilians-University, Munich, Germany.
5
Parkinson's Disease & Movement Disorders Unit, Neurology Service, Hospital Clinic/IDIBAPS/University of Barcelona/CIBERNED, Barcelona, Catalonia, Spain.
6
Department of Clinical Sciences, Division of Oncology and Pathology, Lund University, Lund, Sweden.
7
Division of Neurology, Royal University Hospital, University of Saskatchewan, Saskatchewan, Canada.
8
Neurological Tissue Bank and Neurology Department, Hospital Clínic de Barcelona, Universitat de Barcelona, IDIBAPS, CERCA, Barcelona, Catalonia, Spain.
9
Frontotemporal Degeneration Center, Department of Neurology, University of Pennsylvania, Pennsylvania, USA.
10
University of Bordeaux, Institut des Maladies Neurodégénératives, UMR 5293, Bordeaux, France.
11
Centre national de la recherche scientifique (CNRS), Institut des Maladies Neurodégénératives, UMR 5293, Bordeaux, France.
12
Service de Neurologie, Hôpital Pellegrin, CHU de Bordeaux, Bordeaux, France.
13
Department of Clinical Sciences, Division of Neurology, Lund University, Lund, Sweden.
14
Department of Neurology, Johns Hopkins University, Baltimore, Maryland, USA.
15
Department of Neurology, Erasmus Medical Centre, Rotterdam, The Netherlands.
16
London Neurodegenerative Diseases Brain Bank, Institute of Psychiatry, Psychology and Neuroscience, Kings College London, UK.
17
Department of Neurology, Mayo Clinic, Rochester, Minnesota, USA.
18
Morton and Gloria Shulman Movement Disorders Clinic and the Edmond J Safra Program in Parkinson's Disease, Toronto Western Hospital, Toronto, Canada.
19
Paracelsus-Elena Klinik Kassel and University Medical Center Goettingen, Institute of Neuropathology, Goettingen, Germany.
20
Institute of Human Genetics, Giessen, Germany.
21
Department of Radiology, Mayo Clinic, Rochester, Minnesota, USA.
22
Parkinson and Movement Disorders Unit, Istituto di ricovero e cura a carattere scientifico (IRCCS) Hospital San Camillo and Department of Neurosciences (DNS), Padova University, Padova, Italy.
23
Sobell Department of Motor Neuroscience and Movement Disorders, UCL Institute of Neurology, London, UK.
24
Department of Neurology, University of California, Los Angeles, California, USA.
25
Sorbonne Universités, Université Pierre et Marie Curie (UPMC Univ) Paris 06; and INSERM UMRS_1127, CIC_1422; and CNRS UMR_7225; and Assistance publique - Hôpitaux de Paris (AP-HP); and Institut du Cerveau et de la Moelle Epinière (ICM), Hôpital Pitié-Salpêtrière, Département des maladies du système nerveux, F-75013, Paris, France.
26
Department of Neurology, Santa Maria University Hospital of Terni, Terni, Italy.
27
Department of Geriatric Medicine, University Hospital Essen, Essen, Germany.
28
Department of Neurology, University of Ulm, Ulm, Germany.
29
Department of Neurology, Medical University Innsbruck, Innsbruck, Austria.
30
Department of Neurology, Ludwig-Maximilians-Universität, Munich, Germany.
31
Department of Palliative Medicine, Munich University Hospital, Ludwig-Maximilians-Universität (LMU), Munich, Munich, Germany.
32
Institute of Nursing Science and Practice, Paracelsus Medical University, Salzburg, Austria.
33
Department of Neurology, Hospital Agatharied, Agatharied, Germany.
34
Department of Clinical Neuroscience, UCL Institute of Neurology, London, UK.
35
German Center for Neurodegenerative Diseases, Magdeburg, Germany.
36
Department of Neurology, Philipps Universität, Marburg, Germany.
37
Memory and Aging Center, Department of Neurology, University of California, San Francisco, California, USA.
38
Department of Clinical Neurosciences, Cambridge University, Cambridge, UK.
39
Department of Nuclear Medicine, University Hospital Cologne, Cologne, Germany.
40
Department of Neurology, Rutgers Robert Wood Johnson Medical School, New Brunswick, New Jersey, USA.
41
Department of Neurology, University of California, San Diego, California, USA.
42
Second Department of Neurology, Attikon University Hospital, University of Athens, Greece.
43
HYGEIA Hospital, Athens, Greece.

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. © 2017 International Parkinson and Movement Disorder Society.

KEYWORDS:

Progressive supranuclear palsy; clinical features; clinico-pathological series; diagnosis; systematic review

PMID:
28500752
PMCID:
PMC5543934
DOI:
10.1002/mds.27034
[Indexed for MEDLINE]
Free PMC Article

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