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Behavioural abnormalities contribute to functional decline in Huntington’s disease
  1. J M Hamilton1,
  2. D P Salmon1,
  3. J Corey-Bloom1,
  4. A Gamst2,
  5. J S Paulsen3,
  6. S Jerkins1,
  7. M W Jacobson1,
  8. G Peavy1
  1. 1Department of Neurosciences, University of California, San Diego, California, USA
  2. 2Department of Family and Preventative Medicine, University of California, San Diego, California
  3. 3Departments of Psychiatry and Neurology, The University of Iowa, Iowa City, Iowa, USA
  1. Correspondence to:
 Dr Joanne M Hamilton, Alzheimer’s Disease Research Center, 9500 Gilman Drive, MC 0948, La Jolla, California, USA;
 jmhamilton{at}ucsd.edu

Abstract

The independent and relative contributions of motor, cognitive, and behavioural deficits to functional decline in patients with Huntington’s disease are examined. Twenty two patients with Huntington’s disease were assessed with rating scales for motor dysfunction, cognitive measures of executive functions, and behavioural measures of apathy, executive dysfunction, and disinhibition. Their functional status was assessed with informant based and clinician based ratings of activities of daily living (ADL). A composite apathy/executive dysfunction behavioural index was strongly related to decline in ADL independently and after controlling for motor and cognitive deficits. These results suggest that behavioural dysfunction contributes to functional decline in patients with Huntington’s disease and may impede their ability to utilise motor or cognitive skills that remain available in the early stages of the disease.

  • Huntington’s disease
  • behavioural dysfunction
  • activities of daily living
  • ADL, activities of daily living
  • BDI, Beck depression inventory
  • CANTAB, Cambridge neuropsychological test automated battery
  • FLOPS, frontal lobe personality scale
  • HD-ADL, Huntington’s disease activities of daily living scale
  • TFC, total functional capacity scale
  • UHDRS, unified Huntington’s disease rating scale

https://doi.org/10.1136/jnnp.74.1.120

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    Footnotes

    • Competing interests: none declared