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From the National Hospital for Neurology and Neurosurgery (Dr. Osaki), London; the Department of Social Medicine University of Bristol (Dr. Ben-Shlomo), UK; the Department of Neurology (Dr. Wenning), University Hospital, Innsbruck, Austria; Queen Square Brain Bank for Neurological Disorders (Drs. Daniel and Lees), London, UK; Austin and Repatriation Medical Centre (Dr. Hughes), Heidelberg West, Victoria, Australia; Reta Lila Weston Institute for Neurological Studies (Dr. Lees), Royal Free and UCL Medical School, London; Neurovascular Medicine Unit (Dr. Mathias), Imperial College of Science, Technology & Medicine at St. Marys Hospital, London; and Sobell Department of Motor Neuroscience and Movement Disorders (Dr. Quinn), Institute of Neurology, London, UK.
Address correspondence and reprint requests to Prof. N. Quinn, Sobell Department of Motor Neuroscience and Movement Disorders, Institute of Neurology, Queen Square, London WC1N 3BG, UK; e-mail: n.quinn{at}ion.ucl.ac.uk
Objective: To assess the accuracy of a clinical diagnosis of multiple system atrophy (MSA) and compare it to the Quinn and Consensus criteria for MSA using neuropathologically examined cases from the Queen Square Brain Bank for Neurological Disorders.
Methods: Fifty-nine cases with a neurologic diagnosis of MSA when last assessed prior to death were studied.
Results: In 51 (86%) of these cases, the diagnosis of MSA was confirmed pathologically. False positive diagnoses included PD (n = 6), progressive supranuclear palsy (n = 1), and cerebrovascular disease (n = 1). When applying either set of diagnostic criteria, a diagnosis of probable MSA gave lower sensitivity but higher positive predictive value than one of possible MSA. Application of either set of diagnostic criteria was superior to actual clinical diagnosis made early in the disease, but there was little difference by the last clinic visit.
Conclusions: This study shows a high diagnostic accuracy for the clinical diagnosis of MSA by neurologists, with PD accounting for most of the false positive diagnoses. Application of either Quinn or Consensus criteria was superior to actual clinical diagnosis made early in the disease, but there was little difference by last clinic visit.
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