Clinical diagnosis of progressive supranuclear palsy: The movement disorder society criteria

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Abstract

PSP is a neuropathologically defined disease entity. Clinical diagnostic criteria, published in 1996 by the National Institute of Neurological Disorders and Stroke/Society for PSP, have excellent specificity, but their sensitivity is limited for variant PSP syndromes with presentations other than Richardson's syndrome.

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Movement Disorders Society Scientific Issues Committee report: SIC Task Force appraisal of clinical diagnostic criteria for Parkinsonian disorders.

Irene Litvan, Kailash P. Bhatia, David Burn … (2003)

As there are no biological markers for the antemortem diagnosis of degenerative parkinsonian disorders, diagnosis currently relies upon the presence and progression of clinical features and confirmation depends on neuropathology. Clinicopathologic studies have shown significant false-positive and false-negative rates for diagnosing these disorders, and misdiagnosis is especially common during the early stages of these diseases. It is important to establish a set of widely accepted diagnostic criteria for these disorders that may be applied and reproduced in a blinded fashion. This review summarizes the findings of the SIC Task Force for the study of diagnostic criteria for parkinsonian disorders in the areas of Parkinson's disease, dementia with Lewy bodies, progressive supranuclear palsy, multiple system atrophy, and corticobasal degeneration. In each of these areas, diagnosis continues to rest on clinical findings and the judicious use of ancillary studies. Copyright 2003 Movement Disorder Society

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Preliminary NINDS neuropathologic criteria for Steele-Richardson-Olszewski syndrome (progressive supranuclear palsy).

K Jellinger, J J Hauw, P L Lantos … (1994)

We present the preliminary neuropathologic criteria for progressive supranuclear palsy (PSP) as proposed at a workshop held at the National Institutes of Health, Bethesda, MD, April 24 and 25, 1993. The criteria distinguish typical, atypical, and combined PSP. A semiquantitative distribution of neurofibrillary tangles is the basis for the diagnosis of PSP. A high density of neurofibrillary tangles and neuropil threads in the basal ganglia and brain-stem is crucial for the diagnosis of typical PSP. Tau-positive astrocytes or their processes in areas of involvement help to confirm the diagnosis. Atypical cases of PSP are variants in which the severity or distribution of abnormalities deviates from the typical pattern. Criteria excluding the diagnosis of typical and atypical PSP are large or numerous infarcts, marked diffuse or focal atrophy, Lewy bodies, changes diagnostic of Alzheimer's disease, oligodendroglial argyrophilic inclusions, Pick bodies, diffuse spongiosis, and prion protein-positive amyloid plaques. The diagnosis of combined PSP is proposed when other neurologic disorders exist concomitantly with PSP.

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Characteristics of two distinct clinical phenotypes in pathologically proven progressive supranuclear palsy: Richardson's syndrome and PSP-parkinsonism.

Dominic Paviour, L Kilford, Alan Pittman … (2005)

The clinical diagnosis of progressive supranuclear palsy (PSP) relies on the identification of characteristic signs and symptoms. A proportion of pathologically diagnosed cases do not develop these classic features, prove difficult to diagnose during life and are considered as atypical PSP. The aim of this study was to examine the apparent clinical dichotomy between typical and atypical PSP, and to compare the biochemical and genetic characteristics of these groups. In 103 consecutive cases of pathologically confirmed PSP, we have identified two clinical phenotypes by factor analysis which we have named Richardson's syndrome (RS) and PSP-parkinsonism (PSP-P). Cases of RS syndrome made up 54% of all cases, and were characterized by the early onset of postural instability and falls, supranuclear vertical gaze palsy and cognitive dysfunction. A second group of 33 (32%) were characterized by asymmetric onset, tremor, a moderate initial therapeutic response to levodopa and were frequently confused with Parkinson's disease (PSP-P). Fourteen cases (14%) could not be separated according to these criteria. In RS, two-thirds of cases were men, whereas the sex distribution in PSP-P was even. Disease duration in RS was significantly shorter (5.9 versus 9.1 years, P < 0.001) and age at death earlier (72.1 versus 75.5 years, P = 0.01) than in PSP-P. The isoform composition of insoluble tangle-tau isolated from the basal pons also differed significantly. In RS, the mean four-repeat:three-repeat tau ratio was 2.84 and in PSP-P it was 1.63 (P < 0.003). The effect of the H1,H1 PSP susceptibility genotype appeared stronger in RS than in PSP-P (odds ratio 13.2 versus 4.5). The difference in genotype frequencies between the clinical subgroups was not significant. There were no differences in apolipoprotein E genotypes. The classic clinical description of PSP, which includes supranuclear gaze palsy, early falls and dementia, does not adequately describe one-third of cases in this series of pathologically confirmed cases. We propose that PSP-P represents a second discrete clinical phenotype that needs to be clinically distinguished from classical PSP (RS). The different tau isoform deposition in the basal pons suggests that this may ultimately prove to be a discrete nosological entity.

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Author and article information

Journal

Title: Movement Disorders

Abbreviated Title: Mov Disord.

Publisher: Wiley

ISSN: 08853185

Publication date Created: June 2017

Publication date (Print): June 2017

Publication date (Electronic): May 03 2017

Volume: 32

Issue: 6

Pages: 853-864

Affiliations

[1 ]Department of Neurology; Technische Universität München; Munich Germany

[2 ]German Center for Neurodegenerative Diseases (DZNE); Munich Germany

[3 ]Second Department of Neurology; Attikon University Hospital, University of Athens; Athens Greece

[4 ]Department of Psychiatry; Ludwig-Maximilians-Universität; Munich Germany

[5 ]Department of Neurology; Mayo Clinic; Rochester Minnesota USA

[6 ]Morton and Gloria Shulman Movement Disorders Clinic and the Edmond J. Safra Program in Parkinson's Disease; Toronto Western Hospital; Toronto Canada

[7 ]Paracelsus-Elena Klinik, Kassel, Germany, and University Medical Center Göttingen, Institute of Neuropathology; Göttingen Germany

[8 ]Institute of Human Genetics; Giessen Germany

[9 ]Department of Clinical Sciences, Division of Neurology; Lund University; Lund Sweden

[10 ]Department of Radiology; Mayo Clinic; Rochester Minnesoya USA

[11 ]Center for Neuropathology and Prion Research; Ludwig-Maximilians-Universität; Munich Germany

[12 ]Department of Clinical Sciences; Division of Oncology and Pathology, Lund University; Lund Sweden

[13 ]Neurological Tissue Bank of the Biobank - Hospital Clínic de Barcelona; Universitat de Barcelona, IDIBAPS; Barcelona Spain

[14 ]Frontotemporal Degeneration Center; Department of Neurology, University of Pennsylvania; Philadelphia Pennsylvania USA

[15 ]Université de Bordeaux, Institut des Maladies Neurodégénératives; UMR 5293 Bordeaux France

[16 ]CNRS, Institut des Maladies Neurodégénératives; UMR 5293 Bordeaux France

[17 ]Service de Neurologie, Hôpital Pellegrin, CHU de Bordeaux; Bordeaux France

[18 ]Department of Neurology; Johns Hopkins University; Baltimore Maryland USA

[19 ]Division of Neurology; Royal University Hospital, University of Saskatchewan; Saskatoon SK Canada

[20 ]Department of Neurology; Erasmus Medical Centre; Rotterdam The Netherlands

[21 ]London Neurodegenerative Diseases Brain Bank; Institute of Psychiatry, Psychology and Neuroscience, Kings College London; London United Kingdom

[22 ]Parkinson and Movement Disorders Unit; IRCCS Hospital San Camillo, Venice, and Department of Neurosciences, Padova University; Padova Italy

[23 ]Sobell Department of Motor Neuroscience and Movement Disorders; UCL Institute of Neurology; Queen Square London United Kingdom

[24 ]Department of Neurology; University of California; Los Angeles California USA

[25 ]Parkinson's Disease & Movement Disorders Unit; Neurology Service, Hospital Clinic/IDIBAPS/University of Barcelona; Barcelona, Catalonia Spain

[26 ]Sorbonne Universités, UPMC Univ Paris 06; and INSERM UMRS_1127, CIC_1422; and CNRS UMR_7225; and AP-HP; and ICM, Hôpital Pitié-Salpêtrière, Département des maladies du système nerveux; Paris France

[27 ]Department of Neurology; Santa Maria University Hospital of Terni; Terni Italy

[28 ]Mayo Clinic; Jacksonville Florida USA

[29 ]Department of Geriatric Medicine; University Hospital Essen; Essen Germany

[30 ]Department of Neurology; University of Ulm; Ulm Germany

[31 ]Department of Neurology; Medical University Innsbruck; Innsbruck Austria

[32 ]Department of Neurology; Ludwig-Maximilians-Universität; Munich Germany

[33 ]Institute of Nursing Science and Practice; Paracelsus Medical University; Salzburg Austria

[34 ]Department of Neurology; Hospital Agatharied; Agatharied Germany

[35 ]Department of Palliative Medicine; Munich University Hospital; LMU Munich Munich Germany

[36 ]Department of Clinical Neuroscience; UCL Institute of Neurology; London United Kingdom

[37 ]German Center for Neurodegenerative Diseases (DZNE); Magdeburg Germany

[38 ]Department of Neurology; Philipps Universität; Marburg Germany

[39 ]Memory and Aging Center; Department of Neurology, University of California; San Francisco California USA

[40 ]Department of Clinical Neurosciences; Cambridge University; Cambridge United Kingdom

[41 ]Department of Pathology and Laboratory Medicine; Perelman School of Medicine, University of Pennsylvania; Philadelphia Pennsylvania USA

[42 ]Departments of Nuclear Medicine and Neurology; University of Cologne; Cologne Germany

[43 ]Department of Neurology; Rutgers Robert Wood Johnson Medical School; New Brunswick New Jersey USA

[44 ]Department of Neurology; University of California; San Diego California USA

Article

DOI: 10.1002/mds.26987

SO-VID: ada4eb24-e8b7-4696-984f-f0ccbcde0106

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Clinical diagnosis of progressive supranuclear palsy: The movement disorder society criteria : MDS Clinical Diagnostic Criteria for PSP

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Abstract

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Most cited references 36

Movement Disorders Society Scientific Issues Committee report: SIC Task Force appraisal of clinical diagnostic criteria for Parkinsonian disorders.

Preliminary NINDS neuropathologic criteria for Steele-Richardson-Olszewski syndrome (progressive supranuclear palsy).

Characteristics of two distinct clinical phenotypes in pathologically proven progressive supranuclear palsy: Richardson's syndrome and PSP-parkinsonism.

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Affiliations

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History

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