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Clinical characteristics–cognitive phenotype
J08 Different developmental trajectories in cognitive functions in pre-manifest HD—evidence from longitudinal neurophysiological data
  1. C Beste1,
  2. A-K Stock1,
  3. V Ness1,
  4. E Wascher2,
  5. HR Dinse3,
  6. C Saft4
  1. 1Institute for Cognitive Neuroscience, Biopsychology, Ruhr-Universität Bochum, Bochum, Germany
  2. 2Leibniz Research Centre Dortmund, Perceptual Cybernetics Unit, TU Dortmund, Dortmund, Germany
  3. 3Insitute for Neuroinformatics, Neural Plasticity Lab, Ruhr-Universität Bochum, Bochum, Germany
  4. 4Department of Neurology, Huntington Center, St. Josef Hospital, Ruhr-Universität Bochum, Bochum, Germany


Background There is a multitude of neurobiological processes leading to neurodegenerative effects in HD. One of these factors is excitotoxicity mediated via increased activation of NMDA receptors. However, NMDA receptors also play a crucial role in plasticity and learning. This dual role of effects leads to the counterintutive hypothesis that effects of LTP-like learning processes increase throughout HD pathogenesis.

Methods/techniques We tested this assumption in a perceptual learning task, where pre-HD subjects and controls have to report changes in the luminance of a stimulus in attentionally demanding task. We recorded EEG data to examine the processes on a neurophysiological level.

Results/Outcome The results show that the ability of perceptual learning to augment attentional processes is stronger in pre-HDs, compared to healthy controls. Neurophysiological data suggests that this increase is to a differential modulation of occipital brain areas. The degree of changes induced by perceptual learning depends on the strength of genetic disease load. Longitudinal data suggests that attentional processes undergo a decline over a 16-month period. Interestingly the ability to increase attentional control via LTP-like perceptual learning was not changed in its efficacy.

Conclusions The results suggest that while there is decline in cognitive processes, some functional domains maintain an even superior efficacy over a longer period in pre-manifest HD development.

  • Excitotoxicity
  • LTP
  • perceptual learning
  • attention

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