Progressive supranuclear palsy: cognitive and behavioral changes

Linda A Hershey MD PhD (Dr. Hershey of the University of Oklahoma Health Sciences Center has no relevant financial relationships to disclose.)
David G Lichter MD ChB (Dr. Lichter of SUNY University at Buffalo received honorariums from Teva for speaking engagements and from US World Meds for consulting.)
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 March 22, 2018; expires March 22, 2021

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. There are now at least 7 well-defined syndromes that are subtypes of progressive supranuclear palsy. 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, asymmetrical tremors, rigidity, and slowness that respond for 1 or 2 years 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 poorer baseline performance on cognitive tests. This is particularly true of the PSP-Richardson syndrome variant and the PSP-behavioral variant frontotemporal dementia, where mean survival is about 7 years, compared to 11 years for the milder variants of supranuclear palsy (PSP-parkinsonism and PSP-pure akinesia and gait freezing).

 

• The most common cognitive impairment in PSP-Richardson syndrome is the frontal dysexecutive syndrome, which is manifested by difficulty with planning and organization. These patients also have problems with impulsivity, rigid thinking, and lack of emotional recognition in others.

 

• Apathy is the most common behavioral symptom seen in several of the main subtypes of supranuclear palsy. Apathy has significant negative predictive value for health-related quality of life and appears to be correlated with damage to subcortical white matter tracts.

 

• There are 2 subtypes of supranuclear palsy that can imitate other neurodegenerative diseases. PSP-behavioral variant frontotemporal dementia patients present with changes in personality and socially inappropriate behaviors years before they show signs of gaze palsy, falls, or axial rigidity. Those with PSP-cortical basal syndrome manifest with signs of dementia and motor apraxia, alien limb phenomenon, or cortical sensory loss before they fall or show other signs of 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).

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