Mild cognitive impairment in prediagnosed Huntington disease(e–Pub ahead of print)
Identifieur interne : 000291 ( Ncbi/Merge ); 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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</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>
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<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>
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<author><name sortKey="Harrington, D L" sort="Harrington, D L" uniqKey="Harrington D" first="D" last="Harrington">D Harrington</name>
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<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>
</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>
</imprint>
</series>
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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>
<pmc article-type="research-article"><pmc-comment>The publisher of this article does not allow downloading of the full text in XML form.</pmc-comment>
<front><journal-meta><journal-id journal-id-type="nlm-ta">Neurology</journal-id>
<journal-title>Neurology</journal-title>
<issn pub-type="ppub">0028-3878</issn>
<issn pub-type="epub">1526-632X</issn>
<publisher><publisher-name>American Academy of Neurology</publisher-name>
</publisher>
</journal-meta>
<article-meta><article-id pub-id-type="pmid">20610833</article-id>
<article-id pub-id-type="pmc">2918475</article-id>
<article-id pub-id-type="publisher-id">znl03010000500</article-id>
<article-id pub-id-type="doi">10.1212/WNL.0b013e3181eccfa2</article-id>
<article-categories><subj-group subj-group-type="heading"><subject>Articles</subject>
</subj-group>
</article-categories>
<title-group><article-title>Mild cognitive impairment in prediagnosed Huntington disease<inline-graphic xlink:href="permznlpap0000n1.jpg"><alt-text>(e–Pub ahead of print)</alt-text>
</inline-graphic>
</article-title>
</title-group>
<contrib-group><contrib contrib-type="author"><name><surname>Duff</surname>
<given-names>K.</given-names>
</name>
<degrees>PhD</degrees>
</contrib>
<contrib contrib-type="author"><name><surname>Paulsen</surname>
<given-names>J.</given-names>
</name>
<degrees>PhD</degrees>
</contrib>
<contrib contrib-type="author"><name><surname>Mills</surname>
<given-names>J.</given-names>
</name>
<degrees>MS</degrees>
</contrib>
<contrib contrib-type="author"><name><surname>Beglinger</surname>
<given-names>L.J.</given-names>
</name>
<degrees>PhD</degrees>
</contrib>
<contrib contrib-type="author"><name><surname>Moser</surname>
<given-names>D.J.</given-names>
</name>
<degrees>PhD</degrees>
</contrib>
<contrib contrib-type="author"><name><surname>Smith</surname>
<given-names>M.M.</given-names>
</name>
<degrees>PhD</degrees>
</contrib>
<contrib contrib-type="author"><name><surname>Langbehn</surname>
<given-names>D.</given-names>
</name>
<degrees>MD, PhD</degrees>
</contrib>
<contrib contrib-type="author"><name><surname>Stout</surname>
<given-names>J.</given-names>
</name>
<degrees>PhD</degrees>
</contrib>
<contrib contrib-type="author"><name><surname>Queller</surname>
<given-names>S.</given-names>
</name>
<degrees>PhD</degrees>
</contrib>
<contrib contrib-type="author"><name><surname>Harrington</surname>
<given-names>D.L.</given-names>
</name>
<degrees>PhD</degrees>
</contrib>
<contrib contrib-type="author"><collab>On behalf of the PREDICT-HD Investigators and Coordinators of the Huntington Study Group</collab>
</contrib>
</contrib-group>
<aff id="N0x2e246e0N0x1f26ac0">From the University of Utah (K.D.), Salt Lake City; The University of Iowa (J.P., J.M., L.J.B., D.J.M., M.M.S., D.L.), Carver College of Medicine, Iowa City; Monash University (J.S.), Victoria, Australia; Indiana University (S.Q.), Bloomington; and the University of California (D.L.H.), San Diego.<break></break>
</aff>
<pub-date pub-type="ppub"><day>10</day>
<month>8</month>
<year>2010</year>
</pub-date>
<volume>75</volume>
<issue>6</issue>
<fpage>500</fpage>
<lpage>507</lpage>
<copyright-statement>Copyright © 2010 by AAN Enterprises, Inc.</copyright-statement>
<abstract><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>
</abstract>
</article-meta>
<notes><p>Address correspondence and reprint requests to Dr. Jane Paulsen, The University of Iowa, 1-305 MEB, Iowa City, IA 52242 <email>jane-paulsen@uiowa.edu</email>
</p>
<p>Editorial, page 490</p>
<p><italic>e-Pub ahead of print on July 7, 2010, at</italic>
<ext-link ext-link-type="uri" xlink:href="www.neurology.org">www.neurology.org</ext-link>
.</p>
<p><italic>Study funding:</italic>
Supported by the NIH (NINDS 40068 [J.P.], NIMH 01579, and NIA 1K23AG028417 [K.D.]); the Roy J. and Lucille Carver Trust; the Howard Hughes Medical Institute; the Huntington Disease Society of America; and CHDI Foundation, Inc. (formerly High Q Foundation).</p>
<p><italic>Disclosure:</italic>
Author disclosures are provided at the end of the article.</p>
<p>Received December 30, 2009. Accepted in final form March 29, 2010.</p>
</notes>
</front>
</pmc>
<affiliations><list></list>
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<name sortKey="Duff, K" sort="Duff, K" uniqKey="Duff K" first="K" last="Duff">K Duff</name>
<name sortKey="Harrington, D L" sort="Harrington, D L" uniqKey="Harrington D" first="D" last="Harrington">D Harrington</name>
<name sortKey="Langbehn, D" sort="Langbehn, D" uniqKey="Langbehn D" first="D" last="Langbehn">D Langbehn</name>
<name sortKey="Mills, J" sort="Mills, J" uniqKey="Mills J" first="J" last="Mills">J Mills</name>
<name sortKey="Moser, D J" sort="Moser, D J" uniqKey="Moser D" first="D" last="Moser">D Moser</name>
<name sortKey="Paulsen, J" sort="Paulsen, J" uniqKey="Paulsen J" first="J" last="Paulsen">J Paulsen</name>
<name sortKey="Queller, S" sort="Queller, S" uniqKey="Queller S" first="S" last="Queller">S Queller</name>
<name sortKey="Smith, M M" sort="Smith, M M" uniqKey="Smith M" first="M" last="Smith">M Smith</name>
<name sortKey="Stout, J" sort="Stout, J" uniqKey="Stout J" first="J" last="Stout">J Stout</name>
</noCountry>
</tree>
</affiliations>
</record>
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