Progressive supranuclear palsy: cognitive and behavioral changes

Linda A Hershey MD PhD (Dr. Hershey of the University of Oklahoma Health Sciences Center received research grants from Forum.)
David G Lichter MD ChB (Dr. Lichter of SUNY University at Buffalo received honorariums from Teva for speaking engagements.)
Martin R Farlow MD, editor. (Dr. Farlow of Indiana University received research grant support from AbbVie, Accera, Biogen, Eisai,  Eli Lilly, Genentech, Roche, Lundbeck, Novartis, Suven Life Sciences Ltd, and Boehringer Ingelheim; fees from Accera, Allergan, AstraZeneca, Avanir, Axovant, AZ Therapies, Eli Lilly and Company, FORUM Pharmaceuticals,  INC Research, KCRN Research, Kyowa Kirin Pharma, Longeveron, Medavante, Merck, Medtronic, Proclara, Neurotrope Biosciences, Novartis, Sanofi-Aventis, Stemedica Cell Technologies Inc., Takeda, United Neuroscience Inc., and vTv Therapeutics for consultancy, or advisory board/DSMB membership; and licensing fees from Elan.)
Originally released August 7, 1997; last updated July 25, 2016; expires July 25, 2019

This article includes discussion of progressive supranuclear palsy: cognitive and behavioral changes and Richardson syndrome. The foregoing terms may include synonyms, similar disorders, variations in usage, and abbreviations.

Overview

Many patients with progressive supranuclear palsy present to neurologists with unsteady gait, postural instability, and falls, but others come with complaints of cognitive slowing, apathy, loss of verbal fluency, and loss of ability to recognize emotion in others. The brains of patients with classic supranuclear palsy, or “Richardson syndrome” show frontal atrophy and excessive amounts of abnormally aggregated tau protein. The apathy of supranuclear palsy is associated with atrophy of the ventromedial frontal cortex. The cognitive slowing is correlated with fronto-cerebellar gray matter atrophy and widespread changes in white matter tracts. Patients with the clinical variant of “PSP-parkinsonism” present with dysarthria, ataxia, rigidity and slowness that responds for a year or 2 to levodopa (they do not have early gaze palsy and their brains show less severe tau pathology and less severe cortical atrophy). In this update, the authors describe how the cognitive and behavioral changes in patients with progressive supranuclear palsy can be used to distinguish it from other common neurodegenerative syndromes, such as Parkinson disease, dementia with Lewy bodies, Alzheimer disease, frontotemporal dementia, multiple system atrophy, and corticobasal degeneration. New data about the use of various neuroimaging tools in the diagnosis of supranuclear palsy are described.

Key points

 

• Patients with progressive supranuclear palsy are more likely to progress faster if they have more severe depression at onset and poorer baseline performance on cognitive tests.

 

• Patients with the classic form of progressive supranuclear palsy (Richardson syndrome) are more likely to show early cognitive and behavioral changes than those with the milder variant of supranuclear palsy (PSP-parkinsonism). The disease progresses more rapidly among those with Richardson syndrome.

 

• Apathy is the most common behavioral symptom seen in both of the main subtypes of supranuclear palsy. Apathy has significant negative predictive value for health-related quality of life in prospective studies.

 

• Sleep disturbances can be a marker for early death among supranuclear palsy patients. Although REM sleep disorder is more commonly observed in Parkinson disease, dementia with Lewy bodies, and multiple system atrophy, it is still seen in 37% of those with supranuclear palsy.

Historical note and terminology

In 1877, Dr. Charcot described a 40-year-old woman who had rigid-akinetic parkinsonism, neck dystonia, dysarthria, and eye-movement problems (Charcot 1877). Chavany and others reported the clinical and pathologic features of a 50-year-old man with a rigid and akinetic form of parkinsonism with postural instability, neck dystonia, dysarthria, and staring gaze (Chavany et al 1951). Richardson, Steele, and Olszewski recognized the same clinical syndrome in 8 patients and described the autopsy findings in 6 of them (Richardson et al 1963). Progressive supranuclear palsy was not a “new” disease in 1963, as 22 well-documented case reports had been identified in the neurologic literature between 1877 and 1963 (Brusa et al 2004). The unique frontal lobe cognitive changes of progressive supranuclear palsy (apathy, loss of spontaneity, slowing of thought processes, and loss of executive functions) were first described by Albert and colleagues (Albert et al 1974).

The content you are trying to view is available only to logged in, current MedLink Neurology subscribers.

If you are a subscriber, please log in.

If you are a former subscriber or have registered before, please log in first and then click select a Service Plan or contact Subscriber Services. Site license users, click the Site License Acces link on the Homepage at an authorized computer.

If you have never registered before, click Learn More about MedLink Neurology  or view available Service Plans.