Brain Matters

The PSP diagnostic criteria is from:

Litvan I, Agid Y, Calne D, et al. Clinical research criteria for the diagnosis of progressive supranuclear palsy (Steele-Richardson-Olszewski syndrome): report of the NINDS-SPSP international workshop. Neurology 1996;47:1-9.

NINDS = National Institute of Neurological Disorders and Stroke
SPSP = Society for Progressive Supranuclear Palsy

Core features (mandatory inclusion criteria)

Possible progressive supranuclear palsy
* Gradually progressive disorder
* Onset at age 40 or later
* Either vertical supranuclear palsy or both slowing of vertical saccades and prominent postural instability with falls in the first year of disease onset
* No evidence of other diseases that could explain the foregoing features, as indicated by mandatory exclusion criteria

Probable progressive supranuclear palsy
* Gradually progressive disorder
* Onset at age 40 or later
* Vertical supranuclear palsy and prominent postural instability with falls in the first year of disease onset
* No evidence of other diseases that could explain the foregoing features, as indicated by mandatory exclusion criteria

Definite progressive supranuclear palsy
* Clinically probable or possible PSP and histopathologic evidence of typical PSP

Supportive features
* Symmetric akinesia or rigidity, proximal more than distal
* Abnormal neck posture, especially retrocollis
* Poor or absence of response of parkinsonism to levodopa therapy
* Early dysphagia and dysarthria
* Early onset of cognitive impairment including at least two of the following: apathy, impairment in abstract thought, decreased verbal fluency, use or imitation behavior, or frontal release signs

Exclusion criteria
* Recent history of encephalitis
* Alien limb syndrome, cortical sensory deficits, focal frontal or temporoparietal atrophy
* Hallucinations or delusions unrelated to dopaminergic therapy
* Cortical dementia of Alzheimer type
* Prominent early cerebellar symptoms or prominent early unexplained dysautonomia
* Severe, asymmetric parkinsonian signs
* Neuroradiologic evidence of relevant structural abnormalities
* Whipple's disease, confirmed by polymerase chain reaction