Preliminary NINDS neuropathologic criteria for steele-richardson-olszewski syndrome(progressive supranuclear palsy)

J. J. Hauw, S. E. Daniel, D. Dickson, D. S. Horoupian, K. Jellinger, P. L. Lantos, A. Mc Kee, M. Tabaton, I. Litvan

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Abstract

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 brainstem 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 whichthe severity or distribution of abnormalities deviates from the typical pattern. Criteria excluding the diagnosis of typical and atypical PSP are large or numerousinfarcts, 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.

Original languageEnglish (US)
Pages (from-to)2015-2019
Number of pages5
JournalNeurology
Volume44
Issue number11
StatePublished - Nov 1994

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ASJC Scopus subject areas

  • Clinical Neurology

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Hauw, J. J., Daniel, S. E., Dickson, D., Horoupian, D. S., Jellinger, K., Lantos, P. L., Mc Kee, A., Tabaton, M., & Litvan, I. (1994). Preliminary NINDS neuropathologic criteria for steele-richardson-olszewski syndrome(progressive supranuclear palsy). Neurology, 44(11), 2015-2019.