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December 22, 2003

Profile of cognitive progression in early Huntington’s disease

December 23, 2003 issue
61 (12) 1702-1706

Abstract

Objective: To examine the pattern of cognitive decline in early Huntington’s disease (HD).
Methods: The authors studied 61 patients with mild to moderate HD who had at least three annual neuropsychological assessments using the Core Assessment Program for Intracerebral Transplantation in Huntington’s Disease short battery. A subset of 34 patients had additional neuropsychological tests, and another subset of 21 patients was assessed annually on the Cambridge Neuropsychological Test Automated Battery. Neuropsychological measures that changed significantly over time were submitted to a multiple analysis of covariance to explore associations with demographic and neurologic indices.
Results: Patients showed a progressive impairment in attention, executive function, and immediate memory, with timed tests of psychomotor skill being particularly sensitive to decline. In contrast, general cognition, semantic memory, and delayed recall memory were relatively unaffected.
Conclusion: The profile of cognitive performance shows selective and progressive dysfunction of attention and executive function in patients with mild to moderate HD, consistent with frontostriatal pathology at this stage of disease.

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Information & Authors

Information

Published In

Neurology®
Volume 61Number 12December 23, 2003
Pages: 1702-1706
PubMed: 14694033

Publication History

Received: March 27, 2002
Accepted: August 19, 2003
Published online: December 22, 2003
Published in print: December 23, 2003

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Authors

Affiliations & Disclosures

A.K. Ho, PhD
From the University of Cambridge (Drs. Ho, Sahakian, and Barker), UK; Institute of Psychiatry (Dr. Brown), London, UK; MRC Cognition and Brain Sciences Unit (Dr. Hodges), Cambridge, UK; Leiden University (M.-N. Ané), the Netherlands; Greater Manchester Neuroscience Centre (Dr. Snowden and J. Thompson), Manchester, UK; Belfast City Hospital (Dr. Esmonde), UK; University of Wales School of Medicine (R. Gentry), Cardiff, UK; Dumfries and Galloway Royal Infirmary (Dr. Moore), Dumfries, UK; and University of Innsbruck (Dr. Bodner), Austria.
B.J. Sahakian, PhD
From the University of Cambridge (Drs. Ho, Sahakian, and Barker), UK; Institute of Psychiatry (Dr. Brown), London, UK; MRC Cognition and Brain Sciences Unit (Dr. Hodges), Cambridge, UK; Leiden University (M.-N. Ané), the Netherlands; Greater Manchester Neuroscience Centre (Dr. Snowden and J. Thompson), Manchester, UK; Belfast City Hospital (Dr. Esmonde), UK; University of Wales School of Medicine (R. Gentry), Cardiff, UK; Dumfries and Galloway Royal Infirmary (Dr. Moore), Dumfries, UK; and University of Innsbruck (Dr. Bodner), Austria.
R.G. Brown, PhD
From the University of Cambridge (Drs. Ho, Sahakian, and Barker), UK; Institute of Psychiatry (Dr. Brown), London, UK; MRC Cognition and Brain Sciences Unit (Dr. Hodges), Cambridge, UK; Leiden University (M.-N. Ané), the Netherlands; Greater Manchester Neuroscience Centre (Dr. Snowden and J. Thompson), Manchester, UK; Belfast City Hospital (Dr. Esmonde), UK; University of Wales School of Medicine (R. Gentry), Cardiff, UK; Dumfries and Galloway Royal Infirmary (Dr. Moore), Dumfries, UK; and University of Innsbruck (Dr. Bodner), Austria.
R.A. Barker, MRCP PhD
From the University of Cambridge (Drs. Ho, Sahakian, and Barker), UK; Institute of Psychiatry (Dr. Brown), London, UK; MRC Cognition and Brain Sciences Unit (Dr. Hodges), Cambridge, UK; Leiden University (M.-N. Ané), the Netherlands; Greater Manchester Neuroscience Centre (Dr. Snowden and J. Thompson), Manchester, UK; Belfast City Hospital (Dr. Esmonde), UK; University of Wales School of Medicine (R. Gentry), Cardiff, UK; Dumfries and Galloway Royal Infirmary (Dr. Moore), Dumfries, UK; and University of Innsbruck (Dr. Bodner), Austria.
J.R. Hodges, MD FRCP
From the University of Cambridge (Drs. Ho, Sahakian, and Barker), UK; Institute of Psychiatry (Dr. Brown), London, UK; MRC Cognition and Brain Sciences Unit (Dr. Hodges), Cambridge, UK; Leiden University (M.-N. Ané), the Netherlands; Greater Manchester Neuroscience Centre (Dr. Snowden and J. Thompson), Manchester, UK; Belfast City Hospital (Dr. Esmonde), UK; University of Wales School of Medicine (R. Gentry), Cardiff, UK; Dumfries and Galloway Royal Infirmary (Dr. Moore), Dumfries, UK; and University of Innsbruck (Dr. Bodner), Austria.
M.-N. Ané, MSc
From the University of Cambridge (Drs. Ho, Sahakian, and Barker), UK; Institute of Psychiatry (Dr. Brown), London, UK; MRC Cognition and Brain Sciences Unit (Dr. Hodges), Cambridge, UK; Leiden University (M.-N. Ané), the Netherlands; Greater Manchester Neuroscience Centre (Dr. Snowden and J. Thompson), Manchester, UK; Belfast City Hospital (Dr. Esmonde), UK; University of Wales School of Medicine (R. Gentry), Cardiff, UK; Dumfries and Galloway Royal Infirmary (Dr. Moore), Dumfries, UK; and University of Innsbruck (Dr. Bodner), Austria.
J. Snowden, PhD
From the University of Cambridge (Drs. Ho, Sahakian, and Barker), UK; Institute of Psychiatry (Dr. Brown), London, UK; MRC Cognition and Brain Sciences Unit (Dr. Hodges), Cambridge, UK; Leiden University (M.-N. Ané), the Netherlands; Greater Manchester Neuroscience Centre (Dr. Snowden and J. Thompson), Manchester, UK; Belfast City Hospital (Dr. Esmonde), UK; University of Wales School of Medicine (R. Gentry), Cardiff, UK; Dumfries and Galloway Royal Infirmary (Dr. Moore), Dumfries, UK; and University of Innsbruck (Dr. Bodner), Austria.
J. Thompson, BSc
From the University of Cambridge (Drs. Ho, Sahakian, and Barker), UK; Institute of Psychiatry (Dr. Brown), London, UK; MRC Cognition and Brain Sciences Unit (Dr. Hodges), Cambridge, UK; Leiden University (M.-N. Ané), the Netherlands; Greater Manchester Neuroscience Centre (Dr. Snowden and J. Thompson), Manchester, UK; Belfast City Hospital (Dr. Esmonde), UK; University of Wales School of Medicine (R. Gentry), Cardiff, UK; Dumfries and Galloway Royal Infirmary (Dr. Moore), Dumfries, UK; and University of Innsbruck (Dr. Bodner), Austria.
T. Esmonde, MD
From the University of Cambridge (Drs. Ho, Sahakian, and Barker), UK; Institute of Psychiatry (Dr. Brown), London, UK; MRC Cognition and Brain Sciences Unit (Dr. Hodges), Cambridge, UK; Leiden University (M.-N. Ané), the Netherlands; Greater Manchester Neuroscience Centre (Dr. Snowden and J. Thompson), Manchester, UK; Belfast City Hospital (Dr. Esmonde), UK; University of Wales School of Medicine (R. Gentry), Cardiff, UK; Dumfries and Galloway Royal Infirmary (Dr. Moore), Dumfries, UK; and University of Innsbruck (Dr. Bodner), Austria.
R. Gentry, MPhil
From the University of Cambridge (Drs. Ho, Sahakian, and Barker), UK; Institute of Psychiatry (Dr. Brown), London, UK; MRC Cognition and Brain Sciences Unit (Dr. Hodges), Cambridge, UK; Leiden University (M.-N. Ané), the Netherlands; Greater Manchester Neuroscience Centre (Dr. Snowden and J. Thompson), Manchester, UK; Belfast City Hospital (Dr. Esmonde), UK; University of Wales School of Medicine (R. Gentry), Cardiff, UK; Dumfries and Galloway Royal Infirmary (Dr. Moore), Dumfries, UK; and University of Innsbruck (Dr. Bodner), Austria.
J.W. Moore, D Psychol
From the University of Cambridge (Drs. Ho, Sahakian, and Barker), UK; Institute of Psychiatry (Dr. Brown), London, UK; MRC Cognition and Brain Sciences Unit (Dr. Hodges), Cambridge, UK; Leiden University (M.-N. Ané), the Netherlands; Greater Manchester Neuroscience Centre (Dr. Snowden and J. Thompson), Manchester, UK; Belfast City Hospital (Dr. Esmonde), UK; University of Wales School of Medicine (R. Gentry), Cardiff, UK; Dumfries and Galloway Royal Infirmary (Dr. Moore), Dumfries, UK; and University of Innsbruck (Dr. Bodner), Austria.
T. Bodner, PhD for
From the University of Cambridge (Drs. Ho, Sahakian, and Barker), UK; Institute of Psychiatry (Dr. Brown), London, UK; MRC Cognition and Brain Sciences Unit (Dr. Hodges), Cambridge, UK; Leiden University (M.-N. Ané), the Netherlands; Greater Manchester Neuroscience Centre (Dr. Snowden and J. Thompson), Manchester, UK; Belfast City Hospital (Dr. Esmonde), UK; University of Wales School of Medicine (R. Gentry), Cardiff, UK; Dumfries and Galloway Royal Infirmary (Dr. Moore), Dumfries, UK; and University of Innsbruck (Dr. Bodner), Austria.
for The NEST-HD Consortium
From the University of Cambridge (Drs. Ho, Sahakian, and Barker), UK; Institute of Psychiatry (Dr. Brown), London, UK; MRC Cognition and Brain Sciences Unit (Dr. Hodges), Cambridge, UK; Leiden University (M.-N. Ané), the Netherlands; Greater Manchester Neuroscience Centre (Dr. Snowden and J. Thompson), Manchester, UK; Belfast City Hospital (Dr. Esmonde), UK; University of Wales School of Medicine (R. Gentry), Cardiff, UK; Dumfries and Galloway Royal Infirmary (Dr. Moore), Dumfries, UK; and University of Innsbruck (Dr. Bodner), Austria.

Notes

Address correspondence and reprint requests to Dr. Barbara Sahakian, University of Cambridge School of Clinical Medicine, Addenbrooke’s Hospital (Box 189), Cambridge CB2 2QQ UK

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