Conventional magnetic resonance imaging in confirmed progressive supranuclear palsy and multiple system atrophy

Authors

  • Luke A. Massey MRCP,

    Corresponding author
    1. Sara Koe PSP Research Centre, UCL Institute of Neurology, University College London, London, United Kingdom
    2. Queen Square Brain Bank for Neurological Disorders, UCL Institute of Neurology, University College London, London, United Kingdom
    3. Reta Lila Weston Institute of Neurological Studies, UCL Institute of Neurology, University College London, London, United Kingdom
    • Sara Koe PSP Research Centre, 1 Wakefield Street, London WC1N 1PJ, UK

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  • Caroline Micallef MD, FRCR,

    1. Lysholm Department of Neuroradiology, National Hospital for Neurology and Neurosurgery, London, United Kingdom
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  • Dominic C. Paviour PhD,

    1. Sara Koe PSP Research Centre, UCL Institute of Neurology, University College London, London, United Kingdom
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  • Sean S. O'Sullivan PhD, MRCPI,

    1. Queen Square Brain Bank for Neurological Disorders, UCL Institute of Neurology, University College London, London, United Kingdom
    2. Reta Lila Weston Institute of Neurological Studies, UCL Institute of Neurology, University College London, London, United Kingdom
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  • Helen Ling BScMed, BMBS, MSc,

    1. Sara Koe PSP Research Centre, UCL Institute of Neurology, University College London, London, United Kingdom
    2. Queen Square Brain Bank for Neurological Disorders, UCL Institute of Neurology, University College London, London, United Kingdom
    3. Reta Lila Weston Institute of Neurological Studies, UCL Institute of Neurology, University College London, London, United Kingdom
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  • David R. Williams PhD,

    1. Van Cleef Roet Centre for Nervous Diseases, Monash University, Melbourne, Australia
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  • Constantinos Kallis PhD,

    1. Forensic Psychiatry Research Unit, Queen Mary, University of London, London, United Kingdom
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  • Janice L. Holton PhD, FRCPath,

    1. Sara Koe PSP Research Centre, UCL Institute of Neurology, University College London, London, United Kingdom
    2. Queen Square Brain Bank for Neurological Disorders, UCL Institute of Neurology, University College London, London, United Kingdom
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  • Tamas Revesz MD, FRCPath,

    1. Sara Koe PSP Research Centre, UCL Institute of Neurology, University College London, London, United Kingdom
    2. Queen Square Brain Bank for Neurological Disorders, UCL Institute of Neurology, University College London, London, United Kingdom
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  • David J. Burn MD, FRCP,

    1. Institute of Ageing, University of Newcastle, Newcastle upon Tyne, United Kingdom
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  • Tarek Yousry Dr med Habil, FRCR,

    1. Lysholm Department of Neuroradiology, National Hospital for Neurology and Neurosurgery, London, United Kingdom
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  • Andrew J. Lees MD, FRCP,

    1. Sara Koe PSP Research Centre, UCL Institute of Neurology, University College London, London, United Kingdom
    2. Queen Square Brain Bank for Neurological Disorders, UCL Institute of Neurology, University College London, London, United Kingdom
    3. Reta Lila Weston Institute of Neurological Studies, UCL Institute of Neurology, University College London, London, United Kingdom
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  • Nick C. Fox PhD, FRCP,

    1. Dementia Research Centre, UCL Institute of Neurology, University College London, London, United Kingdom
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  • Hans R. Jäger MD, FRCR

    1. Lysholm Department of Neuroradiology, National Hospital for Neurology and Neurosurgery, London, United Kingdom
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  • Funding agencies: This work has been supported by grants from the PSP (Europe) Association, the Rita Lila Weston Institute for Neurological Studies at UCL Institute of Neurology, and a proportion of funding from the UK Department of Health's National Institute for Health Research Biomedical Research Centres funding scheme (UCLH/UCL Comprehensive Biomedical Research Trust).

  • Relevant conflicts of interest/financial disclosures: LAM, DCP, and HL have been supported by grants from the PSP (Europe) Association. NF has been supported by the Medical Research Council, Alzheimer Research UK and the National Institute for Health Research. This work was undertaken at UCLH/UCL, which received a proportion of funding from the UK Department of Health's National Institute for Health Research Biomedical Research Centres funding scheme (UCLH/UCL Comprehensive Biomedical Research Trust).

Abstract

Conventional magnetic resonance imaging (cMRI) is often used to aid the diagnosis of progressive supranuclear palsy (PSP) and multiple system atrophy (MSA), but its ability to predict the histopathological diagnosis has not been systematically studied. cMRI from 48 neuropathologically confirmed cases, including PSP (n = 22), MSA (n = 13), Parkinson's disease (PD) (n = 7), and corticobasal degeneration (n = 6), and controls (n = 9) were assessed blinded to clinical details and systematically rated for reported abnormalities. Clinical diagnosis and macroscopic postmortem findings were retrospectively assessed. Radiological assessment of MRI was correct in 16 of 22 (72.7%) PSP cases and 10 of 13 (76.9%) MSA cases with substantial interrater agreement (Cohen's kappa 0.708; P < .001); no PSP case was misclassified as MSA or vice versa. MRI was less sensitive but more specific than clinical diagnosis in PSP and both more sensitive and specific than clinical diagnosis in MSA. The “hummingbird” and “morning glory” signs were highly specific for PSP, and “the middle cerebellar peduncle sign” and “hot cross bun” for MSA, but sensitivity was lower (up to 68.4%) and characteristic findings may not be present even at autopsy. cMRI, clinical diagnosis, and macroscopic examination at postmortem have similar sensitivity and specificity in predicting a neuropathological diagnosis. We have validated specific radiological signs in pathologically confirmed PSP and MSA. However, the low sensitivity of these and macroscopic findings at autopsy suggest a need for imaging techniques sensitive to microstructural abnormalities without regional atrophy. © 2012 Movement Disorder Society

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