Progressive Supranuclear Palsy

The first full description of PSP occurred in 1964. PSP begins during the late 50s to mid 60s (earliest reported age 43). Men are slightly more frequently affected than women. Median survival is under 10 years. PSP accounts for about 5% of parkinsonian patients in a typical movement disorders center. PSP is not a synucleinopathy like DLB or MSA, but a tauopathy, relating it more with CBGD.

40% to 50% of patients will have an initial mild, transient response to levodopa. The typical supranuclear eye movement abnormalities are often not seen until four years after the onset of the other symptoms. These begin as an impairment of down gaze. This can be picked up early by checking vertical optico-kinetic nystagmus. Associated ocular findings are horizontal square-wave jerks, slow and hypometric saccades, apraxia of eyelid opening, blepharospasm, or a severely reduced blink rate and a progressive paresis of gaze in all directions.

Expect an early disorder of gait with falls in over 60% to 90%. Axial rigidity particularly in the neck may be associated with hyperextension. Limb tone may be only mildly increased, increasing from distal to proximal. Dystonia of facial muscles create a recognizable pattern of a surprised, wide open eyes look. Tremor is uncommon (5 – 10%) and is evident more often with posturing and action. Personality and cognitive changes occur frequently and early. These changes include emotional incontinence, irritability, and social withdrawal. Severe dementia is rare.

By 5 years, 68% of patients have significant speech dysfunction. Some may progress to anarthria. 46% are affected by dysphagia with by 5 years. Sleep disturbance is common. Autonomic failure is less common than in Parkinson's disease with the exception of incontinence.

Mandatory Exclusion Criteria

Supportive Criteria

Recent history of encephalitis

Symmetric akinesia or rigidity, proximal greater than distal

Alien limb syndrome, cortical sensory loss, focal frontal or temporoparietal atrophy

Abnormal neck posture, especially torticollis

Hallucinations or delusions unrelated to dopaminergic therapy

Poor or absent response of parkinsonism to L-dopa therapy

Cortical dementia of Alzheimer's type

Early dysphagia or dysarthria

Prominent, early cerebellar symptoms or prominent, early unexplained dysautonomia

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

Severe, asymmetric parkinsonian signs

Neuroradiologic evidence of relevant structural abnormality

Whipple's disease confirmed by polymerase chain reaction

General References On Clinical Features of Parkinsonisms
*Handbook of Clinical Neurology Vol 49 Extrapyramidal disorders: Vinken, Bruyn, Klawans eds.

Movement Disorders a Comprehensive Survey;: Weiner, Lang A. eds. Futura publishing company 1989

Parkinson's Disease and Movement Disorders, Jankovic, Tolosa ,eds. Urban & Scharzenberg 1988

Rajesh Pahwa MD Parkinson's Disease and Parkinsonian Syndromes PROGRESSIVE SUPRANUCLEAR PALSY Medical Clinics of North America Volume 83 Number 2 March 1999