Progressive supranuclear palsy

Robert Fekete MD (Dr. Fekete of New York Medical College received consultation fees from Acadia, Impax, Lundbeck, Neurocrine, and Teva.)
Joseph Jankovic MD, editor. (Dr. Jankovic, Director of the Parkinson's Disease Center and Movement Disorders Clinic at Baylor College of Medicine, received research funding from Allergan, Allon, Ceregene, Chelsea, EMD Serono, Impax, Ipsen, Lundbeck, Medtronic, Merz, and Teva, and compensation for his services as a consultant or an advisory committee member by Allergan, Auspex, EMD Serono, Lundbeck, Merz, Neurocrine Biosciences, and Teva.)
Originally released April 10, 1993; last updated January 5, 2017; expires January 5, 2020

This article includes discussion of progressive supranuclear palsy, PSP, PSNP, and Steele-Richardson-Olszewski syndrome. The foregoing terms may include synonyms, similar disorders, variations in usage, and abbreviations.

Overview

Progressive supranuclear palsy remains an intractable and underrecognized neurodegenerative illness with a median survival of only 7 years. However, patients will benefit from accurate, early diagnosis that allows them to understand the prognosis, to avoid unnecessary tests and useless treatments, and to avail themselves of disease-specific support services. Advances in understanding the tau protein aggregation and mitochondrial dysfunction of progressive supranuclear palsy and the toxic and genetic insults underlying them are accruing swiftly. In this article, the author describes the subtypes of progressive supranuclear palsy as well as advances in pathology, imaging, and genetics of the disorder.

Key points

 

• Ophthalmoparesis, especially downgaze impairment, is a classic clinical feature of progressive supranuclear palsy that may appear later in the course of the disorder.

 

• Slowing of downward saccades appears earlier in the clinical course of progressive supranuclear palsy.

 

• Multiple subtypes of progressive supranuclear palsy have been identified, including the classic Richardson syndrome, progressive supranuclear palsy-parkinsonism (PSP-P), or pure-akinesia-with-gait-freezing (PAGF).

Historical note and terminology

The first complete clinicopathologic description of progressive supranuclear palsy was published in 1964 (Steele et al 1964). However, there were 13 earlier clinical cases in the literature, 6 with autopsy (Brusa and Peloso 1993). Some of these antedate the 1920s epidemic of postencephalitic parkinsonism, suggesting that progressive supranuclear palsy is not a variant of that condition despite their pathological similarity (Brusa et al 2004). The meticulously described motor disorder of a character in an 1857 Dickens novel is a convincing picture of progressive supranuclear palsy (Larner 2002). Since the early 1960s when many astute movement disorder specialists were unable to recall having seen progressive supranuclear palsy, this disorder has become commonly recognized at movement disorder referral centers, comprising 4% to 5% of their cases of parkinsonism (Golbe et al 1988).

Monographs (Litvan and Agid 1992; Brusa and Peloso 1993) and a layperson's guide (Golbe 2006) are available. Lay organizations were founded in the United States in 1990 (The Society for Progressive Supranuclear Palsy) and in Europe in 1994 (The Progressive Supranuclear Palsy (PSP-Europe) Association).

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