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Are cognitive changes progressive in prediagnostic HD?

Julie C Stout1, Marjorie Weaver, Andrea C Solomon

  • 1Department of Psychological and Brain Sciences, Indiana University, Bloomington, IN, USA.

Cognitive and Behavioral Neurology : Official Journal of the Society for Behavioral and Cognitive Neurology
|December 20, 2007
PubMed
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Neurocognitive deficits in Huntington disease (HD) appear near diagnosis, not far from it. Genetic staging is more sensitive than motor staging for detecting early cognitive changes in HD.

Area of Science:

  • Neuroscience
  • Genetics
  • Neurology

Background:

  • Huntington disease (HD) is a progressive neurodegenerative disorder.
  • Early detection of neurocognitive changes is crucial for understanding disease progression.

Purpose of the Study:

  • Characterize neurocognitive signs of disease progression in prediagnosis and early Huntington disease (HD).
  • Compare the sensitivity of genetic vs. motor staging for detecting these signs.

Main Methods:

  • 306 at-risk or early HD individuals completed neurocognitive testing and staging.
  • Two staging schemes were used: genetic (CAG repeat length) and motor signs.
  • Effect sizes compared scheme sensitivity in detecting disease progression.

Main Results:

Related Experiment Videos

  • Individuals near estimated HD diagnosis showed cognitive impairment; those far from it did not.
  • Genetic staging yielded larger effect sizes for executive functions than motor staging.
  • Motor staging showed larger effect size for motor/psychomotor function.

Conclusions:

  • Neurocognitive function is not uniformly affected in early HD.
  • Individuals nearing diagnosis exhibit cognitive signs similar to HD.
  • Combined genetic and phenotypic staging may improve tracking of neurocognitive progression.