Mild cognitive impairment in prediagnosed Huntington disease(e–Pub ahead of print)
Identifieur interne : 000291 ( Ncbi/Curation ); précédent : 000290; suivant : 000292Mild cognitive impairment in prediagnosed Huntington disease(e–Pub ahead of print)
Auteurs : K Duff ; J Paulsen ; J Mills ; L Beglinger ; D Moser ; M Smith ; D Langbehn ; J Stout ; S Queller ; D HarringtonSource :
- Neurology [ 0028-3878 ] ; 2010.
Abstract
Cognitive decline has been reported in Huntington disease (HD), as well as in the period before diagnosis of motor symptoms (i.e., pre-HD). However, the severity, frequency, and characterization of cognitive difficulties have not been well-described. Applying similar cutoffs to those used in mild cognitive impairment (MCI) research, the current study examined the rates of subtle cognitive dysfunction (e.g., dysfunction that does not meet criteria for dementia) in pre-HD.
Using baseline data from 160 non–gene-expanded comparison participants, normative data were established for cognitive tests of episodic memory, processing speed, executive functioning, and visuospatial perception. Cutoff scores at 1.5 standard deviations below the mean of the comparison group were then applied to 575 gene-expanded pre-HD participants from the observational study, PREDICT-HD, who were stratified by motor signs and genetic risk for HD.
Nearly 40% of pre-HD individuals met criteria for MCI, and individuals closer to HD diagnosis had higher rates of MCI. Nonamnestic MCI was more common than amnestic MCI. Single-domain MCI was more common than multiple-domain MCI. Within the nonamnestic single-domain subtype, impairments in processing speed were most frequent.
Consistent with the Alzheimer disease literature, MCI as a prodromal period is a valid concept in pre-HD, with nearly 40% of individuals showing this level of impairment before diagnosis. Future studies should examine the utility of MCI as a marker of cognitive decline in pre-HD.
= Alzheimer disease;
= Benton Facial Recognition Test;
= Diagnostic Confidence Level;
= Huntington disease;
= Hopkins Verbal Learning Test–Revised;
= mild cognitive impairment;
= Parkinson disease;
= Stroop Color Word Test;
= Symbol Digit Modalities Test;
= Unified Huntington's Disease Rating Scale.
Url:
DOI: 10.1212/WNL.0b013e3181eccfa2
PubMed: 20610833
PubMed Central: 2918475
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PMC:2918475Le document en format XML
<record><TEI><teiHeader><fileDesc><titleStmt><title xml:lang="en">Mild cognitive impairment in prediagnosed Huntington disease<inline-graphic xlink:href="permznlpap0000n1.jpg"><alt-text>(e–Pub ahead of print)</alt-text>
</inline-graphic>
</title>
<author><name sortKey="Duff, K" sort="Duff, K" uniqKey="Duff K" first="K" last="Duff">K Duff</name>
</author>
<author><name sortKey="Paulsen, J" sort="Paulsen, J" uniqKey="Paulsen J" first="J" last="Paulsen">J Paulsen</name>
</author>
<author><name sortKey="Mills, J" sort="Mills, J" uniqKey="Mills J" first="J" last="Mills">J Mills</name>
</author>
<author><name sortKey="Beglinger, L J" sort="Beglinger, L J" uniqKey="Beglinger L" first="L" last="Beglinger">L Beglinger</name>
</author>
<author><name sortKey="Moser, D J" sort="Moser, D J" uniqKey="Moser D" first="D" last="Moser">D Moser</name>
</author>
<author><name sortKey="Smith, M M" sort="Smith, M M" uniqKey="Smith M" first="M" last="Smith">M Smith</name>
</author>
<author><name sortKey="Langbehn, D" sort="Langbehn, D" uniqKey="Langbehn D" first="D" last="Langbehn">D Langbehn</name>
</author>
<author><name sortKey="Stout, J" sort="Stout, J" uniqKey="Stout J" first="J" last="Stout">J Stout</name>
</author>
<author><name sortKey="Queller, S" sort="Queller, S" uniqKey="Queller S" first="S" last="Queller">S Queller</name>
</author>
<author><name sortKey="Harrington, D L" sort="Harrington, D L" uniqKey="Harrington D" first="D" last="Harrington">D Harrington</name>
</author>
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<author><name sortKey="Duff, K" sort="Duff, K" uniqKey="Duff K" first="K" last="Duff">K Duff</name>
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<author><name sortKey="Paulsen, J" sort="Paulsen, J" uniqKey="Paulsen J" first="J" last="Paulsen">J Paulsen</name>
</author>
<author><name sortKey="Mills, J" sort="Mills, J" uniqKey="Mills J" first="J" last="Mills">J Mills</name>
</author>
<author><name sortKey="Beglinger, L J" sort="Beglinger, L J" uniqKey="Beglinger L" first="L" last="Beglinger">L Beglinger</name>
</author>
<author><name sortKey="Moser, D J" sort="Moser, D J" uniqKey="Moser D" first="D" last="Moser">D Moser</name>
</author>
<author><name sortKey="Smith, M M" sort="Smith, M M" uniqKey="Smith M" first="M" last="Smith">M Smith</name>
</author>
<author><name sortKey="Langbehn, D" sort="Langbehn, D" uniqKey="Langbehn D" first="D" last="Langbehn">D Langbehn</name>
</author>
<author><name sortKey="Stout, J" sort="Stout, J" uniqKey="Stout J" first="J" last="Stout">J Stout</name>
</author>
<author><name sortKey="Queller, S" sort="Queller, S" uniqKey="Queller S" first="S" last="Queller">S Queller</name>
</author>
<author><name sortKey="Harrington, D L" sort="Harrington, D L" uniqKey="Harrington D" first="D" last="Harrington">D Harrington</name>
</author>
</analytic>
<series><title level="j">Neurology</title>
<idno type="ISSN">0028-3878</idno>
<idno type="e-ISSN">1526-632X</idno>
<imprint><date when="2010">2010</date>
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<front><div type="abstract" xml:lang="en"><sec><title>Background:</title>
<p>Cognitive decline has been reported in Huntington disease (HD), as well as in the period before diagnosis of motor symptoms (i.e., pre-HD). However, the severity, frequency, and characterization of cognitive difficulties have not been well-described. Applying similar cutoffs to those used in mild cognitive impairment (MCI) research, the current study examined the rates of subtle cognitive dysfunction (e.g., dysfunction that does not meet criteria for dementia) in pre-HD.</p>
</sec>
<sec><title>Methods:</title>
<p>Using baseline data from 160 non–gene-expanded comparison participants, normative data were established for cognitive tests of episodic memory, processing speed, executive functioning, and visuospatial perception. Cutoff scores at 1.5 standard deviations below the mean of the comparison group were then applied to 575 gene-expanded pre-HD participants from the observational study, PREDICT-HD, who were stratified by motor signs and genetic risk for HD.</p>
</sec>
<sec><title>Results:</title>
<p>Nearly 40% of pre-HD individuals met criteria for MCI, and individuals closer to HD diagnosis had higher rates of MCI. Nonamnestic MCI was more common than amnestic MCI. Single-domain MCI was more common than multiple-domain MCI. Within the nonamnestic single-domain subtype, impairments in processing speed were most frequent.</p>
</sec>
<sec><title>Conclusions:</title>
<p>Consistent with the Alzheimer disease literature, MCI as a prodromal period is a valid concept in pre-HD, with nearly 40% of individuals showing this level of impairment before diagnosis. Future studies should examine the utility of MCI as a marker of cognitive decline in pre-HD.</p>
</sec>
<sec><title>GLOSSARY</title>
<def-list list-type="abr"><def-item><term><bold>AD</bold>
</term>
<def><p> = Alzheimer disease; </p>
</def>
</def-item>
<def-item><term><bold>BFRT</bold>
</term>
<def><p> = Benton Facial Recognition Test; </p>
</def>
</def-item>
<def-item><term><bold>DCL</bold>
</term>
<def><p> = Diagnostic Confidence Level; </p>
</def>
</def-item>
<def-item><term><bold>HD</bold>
</term>
<def><p> = Huntington disease; </p>
</def>
</def-item>
<def-item><term><bold>HVLT-R</bold>
</term>
<def><p> = Hopkins Verbal Learning Test–Revised; </p>
</def>
</def-item>
<def-item><term><bold>MCI</bold>
</term>
<def><p> = mild cognitive impairment; </p>
</def>
</def-item>
<def-item><term><bold>PD</bold>
</term>
<def><p> = Parkinson disease; </p>
</def>
</def-item>
<def-item><term><bold>SCWT</bold>
</term>
<def><p> = Stroop Color Word Test; </p>
</def>
</def-item>
<def-item><term><bold>SDMT</bold>
</term>
<def><p> = Symbol Digit Modalities Test; </p>
</def>
</def-item>
<def-item><term><bold>UHDRS</bold>
</term>
<def><p> = Unified Huntington's Disease Rating Scale.</p>
</def>
</def-item>
</def-list>
</sec>
</div>
</front>
</TEI>
</record>
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