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The Addenbrooke's Cognitive Examination-Revised accurately detects cognitive decline in Huntington's disease

Begeti, Faye; Tan, Adrian Y K; Cummins, Gemma A; Collins, Lucy M; Guzman, Natalie Valle; Mason, Sarah L and Barker, Roger A LU (2013) In Journal of Neurology 260(11). p.85-2777
Abstract

Cognitive features, which begin before manifestation of the motor features, are an integral part of Huntington's disease and profoundly affect quality of life. A number of neuropsychological batteries have been used to assess this aspect of the condition, many of which are difficult to administer and time consuming, especially in advanced disease. We, therefore, investigated a simple and practical way to monitor cognition using the Addenbrooke's Cognitive Examination-Revised (ACE-R) in 126 manifest Huntington's disease patients, 28 premanifest gene carriers and 21 controls. Using this test, we demonstrated a selective decrease in phonemic, but not semantic, fluency in premanifest participants Cognitive decline in manifest Huntington's... (More)

Cognitive features, which begin before manifestation of the motor features, are an integral part of Huntington's disease and profoundly affect quality of life. A number of neuropsychological batteries have been used to assess this aspect of the condition, many of which are difficult to administer and time consuming, especially in advanced disease. We, therefore, investigated a simple and practical way to monitor cognition using the Addenbrooke's Cognitive Examination-Revised (ACE-R) in 126 manifest Huntington's disease patients, 28 premanifest gene carriers and 21 controls. Using this test, we demonstrated a selective decrease in phonemic, but not semantic, fluency in premanifest participants Cognitive decline in manifest Huntington's disease varied according to disease severity with extensive cognitive decline observed in early-stage Huntington's disease patients, indicating that this would be an optimal stage for interventions designed to halt cognitive decline, and lesser changes in the advanced cases. We next examined cognitive performance in patients prescribed antidopaminergic drugs as these drugs are known to decrease cognition when administered to healthy volunteers. We paradoxically found that these drugs may be beneficial, as early-stage Huntington's disease participants in receipt of them had improved attention and Mini-Mental State Examination scores. In conclusion, this is the first study to test the usefulness of the ACE-R in a Huntington's disease population and demonstrates that this is a brief, inexpensive and practical way to measure global cognitive performance in clinical practice with potential use in clinical trials.

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author
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Contribution to journal
publication status
published
subject
keywords
Adult, Attention, Case-Control Studies, Cognition Disorders, Disability Evaluation, Female, Humans, Huntington Disease, Language, Male, Memory, Mental Status Schedule, Middle Aged, Neuropsychological Tests, Severity of Illness Index, Space Perception, Journal Article, Research Support, Non-U.S. Gov't
in
Journal of Neurology
volume
260
issue
11
pages
85 - 2777
publisher
Steinkopff
external identifiers
  • scopus:84889571015
ISSN
1432-1459
DOI
10.1007/s00415-013-7061-5
language
English
LU publication?
no
id
4d194a0d-e767-4f14-b159-44951aae61e5
date added to LUP
2016-11-24 15:17:31
date last changed
2018-01-07 11:36:58
@article{4d194a0d-e767-4f14-b159-44951aae61e5,
  abstract     = {<p>Cognitive features, which begin before manifestation of the motor features, are an integral part of Huntington's disease and profoundly affect quality of life. A number of neuropsychological batteries have been used to assess this aspect of the condition, many of which are difficult to administer and time consuming, especially in advanced disease. We, therefore, investigated a simple and practical way to monitor cognition using the Addenbrooke's Cognitive Examination-Revised (ACE-R) in 126 manifest Huntington's disease patients, 28 premanifest gene carriers and 21 controls. Using this test, we demonstrated a selective decrease in phonemic, but not semantic, fluency in premanifest participants Cognitive decline in manifest Huntington's disease varied according to disease severity with extensive cognitive decline observed in early-stage Huntington's disease patients, indicating that this would be an optimal stage for interventions designed to halt cognitive decline, and lesser changes in the advanced cases. We next examined cognitive performance in patients prescribed antidopaminergic drugs as these drugs are known to decrease cognition when administered to healthy volunteers. We paradoxically found that these drugs may be beneficial, as early-stage Huntington's disease participants in receipt of them had improved attention and Mini-Mental State Examination scores. In conclusion, this is the first study to test the usefulness of the ACE-R in a Huntington's disease population and demonstrates that this is a brief, inexpensive and practical way to measure global cognitive performance in clinical practice with potential use in clinical trials.</p>},
  author       = {Begeti, Faye and Tan, Adrian Y K and Cummins, Gemma A and Collins, Lucy M and Guzman, Natalie Valle and Mason, Sarah L and Barker, Roger A},
  issn         = {1432-1459},
  keyword      = {Adult,Attention,Case-Control Studies,Cognition Disorders,Disability Evaluation,Female,Humans,Huntington Disease,Language,Male,Memory,Mental Status Schedule,Middle Aged,Neuropsychological Tests,Severity of Illness Index,Space Perception,Journal Article,Research Support, Non-U.S. Gov't},
  language     = {eng},
  number       = {11},
  pages        = {85--2777},
  publisher    = {Steinkopff},
  series       = {Journal of Neurology},
  title        = {The Addenbrooke's Cognitive Examination-Revised accurately detects cognitive decline in Huntington's disease},
  url          = {http://dx.doi.org/10.1007/s00415-013-7061-5},
  volume       = {260},
  year         = {2013},
}