Progressive Supranuclear Palsy

First described by Steele, Richardson, and Olzweski in 1964, progressive supranuclear palsy (PSP) is also known by its acronym. The original descriptions focused on the parkinsonian movement disorder with progressive eye movement abnormality and frequent falls. Dementia also occurs as part of the clinical expression of PSP. PSP is almost never familial in nature. More recent investigations have tended to widen the scope of the clinical spectrum, and clinical overlaps occur, particularly with corti-cobasal degeneration. PSP may be misdiagnosed as PD or vascular dementia. False-negative diagnoses confirmed pathologically may include not only corticobasal degeneration but also multiple-system atrophy, central nervous system Whipple's disease, diffuse Lewy body disease, subcortical gliosis, and prion diseases.

Of some difficulty in diagnosis is that the characteristic eye movements may not be present initially, although there is good agreement that slowed vertical saccades are the most consistent early eye movement abnormality.

There have been a number of sets of diagnostic criteria proposed, mostly on clinical grounds. These are shown in Table 19. The validity and reliability of these criteria have been investigated in several studies, and the results are summarized in Table 20.

The latest set of diagnostic criteria is the National Institute of Neurological Disorders and Stroke-PSP clinical criteria and is included in Table 21.

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