Table 3.

PSP Syndromes

Variant PSP	Clinical Features	Regional Pathology*
PSP-RS (Richardson Syndrome)	Early gait instability, falls, supranuclear gaze palsy, axial rigidity, dysarthria, dysphagia, progressive dementia	Dentate, globus pallidus, striatum, midbrain, and superior cerebellar (CBL) peduncle
PSP-Parkinsonism	Tremor, rigid-bradykinesia, Levodopa responsive** Late cognitive decline Longer life expectancy (9+ yrs)	Substantia nigra, subthalamic nucleus
PSP-PAGF (Pure akinesia-gait failure)	Early gait difficulty, freezing of gait/motor block, micrographia, speech impairment, hypophonia Disease duration (11–15 yr)	Motor cortex, pons, cerebellum
PSP-CBS	Dystonia, dyspraxia, cortical sensory loss, apraxia speech	Frontal and parietal cortex
PSP-bvFTD (behavioral variant FTD)	Predominant cognitive, personality change, late Parkinsonism	Frontotemporal cortex
PSP-PLS (primary lateral sclerosis)	Bulbar, limb weakness, upper motor neuron signs/spasticity	Frontal predominant, corticospinal tract
PSP-cerebellar	Cerebellar ataxia	Deep cerebellar nuclei

^*Levodopa response for PSP-Parkinsonism diminishes later in disease.

(Reprinted with permission from McFarland NR. Diagnostic Approach to Atypical Parkinsonian Syndromes. Continuum 2016;22(4):1123. doi: 10.1212/CON.0000000000000348)