RT Journal Article
SR Electronic
T1 Clinical research criteria for the diagnosis of progressive supranuclear palsy (Steele-Richardson-Olszewski syndrome)
JF Neurology
JO Neurology
FD Lippincott Williams & Wilkins
SP 1
OP 9
DO 10.1212/WNL.47.1.1
VO 47
IS 1
A1 I. Litvan
A1 Y. Agid
A1 D. Calne
A1 G. Campbell
A1 B. Dubois
A1 R. C. Duvoisin
A1 C. G. Goetz
A1 L. I. Golbe
A1 J. Grafman
A1 J. H. Growdon
A1 M. Hallett
A1 J. Jankovic
A1 N. P. Quinn
A1 E. Tolosa
A1 D. S. Zee
YR 1996
UL http://n.neurology.org/content/47/1/1.abstract
AB To improve the specificity and sensitivity of the clinical diagnosis of progressive supranuclear palsy (PSP, Steele-Richardson-Olszewski syndrome), the National Institute of Neurological Disorders and Stroke (NINDS) and the Society for PSP, Inc. (SPSP) sponsored an international workshop to develop an accurate and universally accepted set of criteria for this disorder. The NINDS-SPSP criteria, which were formulated from an extensive review of the literature, comparison with other previously published sets of criteria, and the consensus of experts, were validated on a clinical data set from autopsy-confirmed cases of PSP. The criteria specify three degrees of diagnostic certainty: possible PSP, probable PSP, and definite PSP. Possible PSP requires the presence of a gradually progressive disorder with onset at age 40 or later, either vertical supranuclear gaze palsy or both slowing of vertical saccades and prominent postural instability with falls in the first year of onset, as well as no evidence of other diseases that could explain these features. Probable PSP requires vertical supranuclear gaze palsy, prominent postural instability, and falls in the first year of onset, as well as the other features of possible PSP. Definite PSP requires a history of probable or possible PSP and histopathologic evidence of typical PSP. Criteria that support the diagnosis of PSP, and that exclude diseases often confused with PSP, are presented. The criteria for probable PSP are highly specific, making them suitable for therapeutic, analytic epidemiologic, and biologic studies, but not very sensitive. The criteria for possible PSP are substantially sensitive, making them suitable for descriptive epidemiologic studies, but less specific. An appendix provides guidelines for diagnosing and monitoring clinical disability in PSP. NEUROLOGY 1996;47: 1-9