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NEUROLOGY 1994;44:2015
© 1994 American Academy of Neurology

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

J.-J. Hauw, MD, S. E. Daniel, MD, D. Dickson, MD, D. S. Horoupian, MD, K. Jellinger, MD, P. L. Lantos, MD, A. McKee, MD, M. Tabaton, MD and I. Litvan, MD

Raymond Escourolle Neuropathology Laboratory (Dr. Hauw), INSERM U 360, Hôpital de la Salpêtrière, Paris, France; the Parkinson's Disease Society Brain Tissue Bank and Department of Neuropathology (Dr. Daniel), Institute of Neurology, London, UK; the Department of Neuropathology (Dr. Dickson), Albert Einstein College of Medicine, Bronx, NY; the Department of Pathology (Neuropathology) (Dr. Horoupian), Stanford School of Medicine, San Francisco, CA; the Ludwig Boltzmann Institute of Clinical Neurobiology (Dr. Jellinger), Vienna, Austria; the Department of Neuropathology (Dr. Lantos), Institute of Psychiatry, London, UK; the Department of Neuropathology (Dr. McKee), Massachusetts General Hospital, Boston, MA; the Division of Neuropathology (Dr. Tabaton), Case Western Reserve University, Cleveland, OH; and the Neuroepidemiology Branch (Dr. Litvan), National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD.

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 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.

Address correspondence and reprint requests to Prof. J.-J. Hauw, Laboratoire de Neuropathologie Raymond Escourolle, Hôpital de la Salpêtrière, 47 Bd de l' Hôpital, 75651 Paris, Cedex 13, France, or Dr. Irene Litvan, Federal Building, Room 714, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD 20892.

The workshop was funded by the National Institute of Neurological Disorders and Stroke. Dr. S.E. Daniel is supported by a grant from the Parkinson's Disease Society of the United Kingdom. Support from the Medical Research Council to Prof. P.L. Lantos is acknowledged.

Received April 26,1994. Accepted in final form May 23,1994




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Arch Neurol, July 1, 1998; 55(7): 969 - 978.
[Abstract] [Full Text] [PDF]

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 Arch NeurolHome page
I. Litvan and A. M. Saunders
Apolipoprotein E{epsilon}4 (Epsilon) Allele Does Not Affect the Onset or Symptom Severity in Progressive Supranuclear Palsy
Arch Neurol, May 1, 1998; 55(5): 752 - 754.
[Full Text] [PDF]

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 J. Neurol. Neurosurg. PsychiatryHome page
G K Wenning, I Litvan, J Jankovic, R Granata, C A Mangone, A McKee, W Poewe, K Jellinger, K R. Chaudhuri, L D'Olhaberriague, et al.
Natural history and survival of 14 patients with corticobasal degeneration confirmed at postmortem examination
J. Neurol. Neurosurg. Psychiatry, February 1, 1998; 64(2): 184 - 189.
[Abstract] [Full Text] [PDF]

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 Arch NeurolHome page
J. Ghika and J. Bogousslavsky
Presymptomatic Hypertension Is a Major Feature in the Diagnosis of Progressive Supranuclear Palsy
Arch Neurol, September 1, 1997; 54(9): 1104 - 1108.
[Abstract] [PDF]

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 Arch NeurolHome page
I. Litvan, C. G. Goetz, J. Jankovic, G. K. Wenning, V. Booth, J. J. Bartko, A. McKee, K. Jellinger, E. C. Lai, J.-P. Brandel, et al.
What Is the Accuracy of the Clinical Diagnosis of Multiple System Atrophy? A Clinicopathologic Study
Arch Neurol, August 1, 1997; 54(8): 937 - 944.
[Abstract] [PDF]


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