title: Conventional magnetic resonance imaging in confirmed progressive supranuclear palsy and multiple system atrophy.
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.
authors.affiliation: Sara Koe PSP Research Centre, UCL Institute of Neurology, University College London, London, United Kingdom. l.massey@ion.ucl.ac.uk
authors.forename: Luke A
authors.initials: LA
authors.lastname: Massey
authors.forename: Caroline
authors.initials: C
authors.lastname: Micallef
authors.forename: Dominic C
authors.initials: DC
authors.lastname: Paviour
authors.forename: Sean S
authors.initials: SS
authors.lastname: O'Sullivan
authors.forename: Helen
authors.initials: H
authors.lastname: Ling
authors.forename: David R
authors.initials: DR
authors.lastname: Williams
authors.forename: Constantinos
authors.initials: C
authors.lastname: Kallis
authors.forename: Janice L
authors.initials: JL
authors.lastname: Holton
authors.forename: Tamas
authors.initials: T
authors.lastname: Revesz
authors.forename: David J
authors.initials: DJ
authors.lastname: Burn
authors.forename: Tarek
authors.initials: T
authors.lastname: Yousry
authors.forename: Andrew J
authors.initials: AJ
authors.lastname: Lees
authors.forename: Nick C
authors.initials: NC
authors.lastname: Fox
authors.forename: Hans R
authors.initials: HR
authors.lastname: Jäger
mesh_terms: D000368:Aged; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging / Q000379:methods; D008297:Male; D008875:Middle Aged; D019578:Multiple System Atrophy / Q000175:diagnosis / Q000473:pathology*; D012189:Retrospective Studies; D012680:Sensitivity and Specificity; D013494:Supranuclear Palsy, Progressive / Q000175:diagnosis / Q000473:pathology*