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

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 brain-stem 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.

Morning Glory Sign: A Particular MR Finding in Progressive Supranuclear Palsy

“…[23][24][25] These structures are mainly located in the cranial and dorsal part of the midbrain. Large numbers of neuroˆbrillary tangles, neuropil threads, and tufted astrocytes are also found within these brain regions.…”

Increased basal ganglia binding of 18F‐AV‐1451 in patients with progressive supranuclear palsy

“…Cortical ROIs from the AAL atlas were pooled into frontal, temporal, parietal, occipital, and cerebellar gray matter ROIs (see supplementary methods for details). Cerebellar gray matter, not including the dentate nucleus, was considered a suitable reference region as a result of the absence of tau pathology in this region in PSP patients without ataxia 15, 16, 17. Mean standardized uptake value ratios (SUVRs) were calculated for all patients for the 80‐ to 120‐minute time period.…”

Spatial patterns of the tau pathology in progressive supranuclear palsy

“…Patients had no family history of psychiatric or neurological disorders and were clinically diagnosed as 'definite' PSP using National Institute of Neurological Disorders and Stroke (NINDS) and the Society of PSP (SPSP) clinical criteria [27][28][29]. Two clinical phenotypes of PSP have been identified, viz., Richardson's syndrome (RS) and PSP-parkinsonism (PSP-P), the two subtypes varying in disease duration and in tau isoforms [30].…”