J Mov Disord.  2018 May;11(2):72-77. 10.14802/jmd.17067.

Prospective Characterization of Cognitive Function in Typical and ‘Brainstem Predominant'Progressive Supranuclear Palsy Phenotypes

• Lee YE
• Williams D
• Anderson J

• KMID: 2413377
• DOI: http://doi.org/10.14802/jmd.17067

Abstract

OBJECTIVE
Clinicopathological studies over the last decade have broadened the clinical spectrum of progressive supranuclear palsy (PSP) to include several distinct clinical syndromes. We examined the cognitive profiles of patients with PSP-Richardson's syndrome (PSP-RS) and two atypical "˜brainstem predominant' PSP phenotypes (PSP-parkinsonism, PSP-P; and PSP-pure akinesia with gait freezing, PSP-PAGF) using a comprehensive neuropsychological battery.
METHODS
Fourteen patients diagnosed as PSP-RS, three patients with PSP-P and four patients with PSP-PAGF were assessed using a comprehensive battery of neuropsychological tests.
RESULTS
The typical PSP-RS subgroup demonstrated greater impairments in processing speed [t(19) = -4.10, p = 0.001 (d =1.66)] and executive function [t(19) = -2.63, p = 0.02 (d = 1.20)] compared to the "˜brainstem predominant' PSP phenotype.
CONCLUSION
This is the first prospective study to demonstrate that PSP-RS and "˜brainstem predominant' PSP phenotypes can be differentiated on cognitive grounds. These differences correspond with variations in pathological profiles reported in the literature.

Keyword

Progressive supranuclear palsy; phenotypes; cognition; neuropsychology

MeSH Terms

Cognition*
Executive Function
Freezing
Gait
Humans
Neuropsychological Tests
Neuropsychology
Paralysis*
Phenotype*
Prospective Studies*
Supranuclear Palsy, Progressive