PSP

Mandatory inclusion criteria

Mandatory exclusion criteria

Supportive criteria

Possible

- Gradually progressive disorder

- Onset at age 40 or later

- Either vertical (upward or downward gaze) 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

- 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’s type (severe amnesia and aphasia or agnosia, according to NINCDS-ADRA criteria)

- Prominent, early cerebellar symptoms or prominent, early unexplained dysautonomia (marked hypotension and urinary disturbances)

- Symmetric akinesia or rigidity, proximal more than distal

- Abnormal neck posture, especially retrocollis

- Poor or absent 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, utilization or imitation behavior, or frontal release signs

Probable

- Gradually progressive disorder

- Onset at age 40 or later

- Vertical (upward or downward gaze) 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

- Severe, asymmetric parkinsonian signs (i.e., bradykinesia)

- Neuroradiologic evidence of relevant structural abnormality (i.e. basal ganglia or brainstem infarcts, lobar atrophy)

- Whipple’s disease, confirmed by polymerase chain reaction, if indicated

Definite

- Clinically probable or possible PSP and histopathologic evidence of typical PSP