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January 22, 2013; 80 (4) Article

Functional impairment in progressive supranuclear palsy

Kevin Duff, Adam Gerstenecker, Irene Litvan, On behalf of the investigators and coordinators of the ENGENE-PSP Study Group
First published January 9, 2013, DOI: https://doi.org/10.1212/WNL.0b013e31827f0859
Kevin Duff
From the Department of Neurology (K.D.), University of Utah, Salt Lake City; Department of Psychological and Brain Sciences (A.G.), University of Louisville, Louisville, KY; and Department of Neurosciences (I.L.), University of California San Diego, La Jolla.
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Adam Gerstenecker
From the Department of Neurology (K.D.), University of Utah, Salt Lake City; Department of Psychological and Brain Sciences (A.G.), University of Louisville, Louisville, KY; and Department of Neurosciences (I.L.), University of California San Diego, La Jolla.
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Irene Litvan
From the Department of Neurology (K.D.), University of Utah, Salt Lake City; Department of Psychological and Brain Sciences (A.G.), University of Louisville, Louisville, KY; and Department of Neurosciences (I.L.), University of California San Diego, La Jolla.
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From the Department of Neurology (K.D.), University of Utah, Salt Lake City; Department of Psychological and Brain Sciences (A.G.), University of Louisville, Louisville, KY; and Department of Neurosciences (I.L.), University of California San Diego, La Jolla.
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Functional impairment in progressive supranuclear palsy
Kevin Duff, Adam Gerstenecker, Irene Litvan, On behalf of the investigators and coordinators of the ENGENE-PSP Study Group
Neurology Jan 2013, 80 (4) 380-384; DOI: 10.1212/WNL.0b013e31827f0859

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Abstract

Objective: The current study sought to describe the functional profiles of patients with early-stage progressive supranuclear palsy (PSP) in a large prospective, multisite study.

Methods: Using data from 202 individuals meeting criteria for clinically definite or probable PSP, 3 functional scales were examined. Functional scores were then compared to measures of motor, cognition, and psychiatric symptoms.

Results: Functional disability was high in early-stage PSP, with 100% of patients having less than perfect scores on all functional scales. Whereas functional scores tended not to be related to cognition or psychiatric symptoms, they were strongly related to motoric ratings.

Conclusions: Both clinically and in research settings, the definition of functional intactness/impairment has important implications. Future studies should examine if functional impairment is this high in PSP or if new scales of functional abilities need to be developed for this condition.

GLOSSARY

ADL=
activities of daily living;
DRS-2=
Dementia Rating Scale–2;
NPI=
Neuropsychiatric Inventory;
PSP=
progressive supranuclear palsy;
PSP-RS=
Progressive Supranuclear Palsy Rating Scale;
SE-ADL=
Schwab & England Activities of Daily Living Scale;
UPDRS=
Unified Parkinson's Disease Rating Scale

Footnotes

  • The Investigators and Coordinators of the ENGENE-PSP Study Group are listed on the Neurology® Web site at www.neurology.org.

  • Go to Neurology.org for full disclosures. Funding information and disclosures deemed relevant by the authors, if any, are provided at the end of the article.

  • Supplemental data at www.neurology.org

  • Received May 24, 2012.
  • Accepted September 6, 2012.
  • © 2013 American Academy of Neurology
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