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Profile of cognitive progression in early Huntington’s disease

A.K. Ho, PhD^*, B.J. Sahakian, PhD^*, R.G. Brown, PhD^*, R.A. Barker, MRCP PhD^*, J.R. Hodges, MD FRCP^*, M.-N. Ané, MSc, J. Snowden, PhD, J. Thompson, BSc, T. Esmonde, MD, R. Gentry, MPhil, J.W. Moore, D Psychol and T. Bodner, PhD for 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.

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

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.

Received March 27, 2002. Accepted in final form August 19, 2003.

Additional material related to this article can be found on the Neurology Web site. Go to www.neurology.org and scroll down the Table of Contents for the December 23 issue to find the title link for this article.

*Coordinators of the CAPIT-HD Neuropsychology Working Group and writing committee.

Members of the CAPIT-HD Neuropsychology Working Group and writing committee.

Members of the NEST-HD Consortium are listed in the Appendix on page 1705.

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