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H38 A new approach to digitized cognitive monitoring: validity of the selfcog in Huntington’s disease
  1. Marine Lunven1,2,3,4,
  2. Karen Hernandez Dominguez1,2,3,4,
  3. Katia Youssov1,2,3,4,
  4. Jennifer Hamet Bagnou1,2,3,4,
  5. Rafika Fliss1,2,3,4,
  6. Henri Vandendriessche1,2,3,4,
  7. Blanche Bapst5,6,
  8. Graça Morgado7,
  9. Robin Schubert8,9,
  10. Ralf Reilmann8,9,10,
  11. Monica Busse11,12,
  12. David Craufurd13,14,
  13. Renaud Massart1,2,3,4,
  14. Anne Rosser12,15,16,
  15. Anne-Catherine Bachoud-Lévi1,2,3,4
  1. 1Département d’Etudes Cognitives, École normale supérieure, PSL University, Paris, France
  2. 2University Paris Est Créteil, INSERM U955, Institut Mondor de Recherche Biomédicale, Equipe NeuroPsychologie Interventionnelle, Créteil, France
  3. 3AP-HP, Hôpital Henri Mondor-Albert Chenevier, Centre de référence Maladie de Huntington, Service de Neurologie, Créteil, France
  4. 4NeurATRIS, Créteil, France
  5. 5Department of Neuroradiology, AP-HP, Henri Mondor University Hospital, 51 Avenue du Mareéchal de Lattre de Tassigny, Créteil, France
  6. 6Faculty of Medicine, Université Paris Est Créteil, Créteil, France
  7. 7Inserm, Centre d’Investigation Clinique 1430, APHP, Hôpital Henri Mondor, Créteil, France
  8. 8George-Huntington-Institute, Technology-Park, Muenster, Germany
  9. 9Dept. of Neurodegeneration and Hertie Institute for Clinical Brain Research, University of Tuebingen, Tuebingen, Germany
  10. 10Department of Clinical Radiology University of Muenster, Muenster, Germany
  11. 11Centre for Trials Research, Cardiff University, UK
  12. 12Wales Brain Research And Intracranial Neurotherapeutics (BRAIN) Biomedical Research Unit, College of Biomedical and Life Sciences, Cardiff University, Cardiff, UK
  13. 13Manchester Centre for Genomic Medicine, St Mary’s Hospital, Manchester University NHS Foundation Trust, Manchester Academic Health Science Centre, Manchester, UK
  14. 14Division of Evolution and Genomic Sciences, School of Biological Sciences, Faculty of Biology, Medicine and Health, University of Manchester, Manchester Academic Health Science Centre, Manchester, UK
  15. 15Cardiff University Brain Repair Group, School of Biosciences, Life Sciences Building, Cardiff, UK
  16. 16Neuroscience and Mental Health Institute, Hadyn Ellis Building, Cardiff School of Medicine, UK


Background Cognitive deficits represent a hallmark of Huntington’s disease (HD) but evaluating their progression is complex. We developed the SelfCog, a digitized battery that tests motor, executive, visuospatial, language and memory functions in 15 minutes. Based on neuroscience principles, all cognitive functions are tested according to the same paradigm. A randomization algorithm provides a new test at each assessment.

Aims We assessed its validity, reliability, and sensitivity to detect decline in early-stage HD in a prospective and international multilingual study (France, United Kingdom, and Germany).

Methods Fifty-one out of 85 participants with HD and 40 of 52 healthy controls included at baseline were followed up for one year. We estimated associations between each of the classical clinical assessments and SelfCog using Pearson’s correlation and proneness to retest effects and sensitivity to decline through Linear Mixed Models. Longitudinal effect sizes were estimated for each cognitive score. Voxel-based morphometry and Tract-Based Spatial Statistics analyses were conducted to assess the consistency between performance and decline on the SelfCog and MRI 3D-T1 and diffusion weighted imaging.

Results The SelfCog detected the decline of patients with HD in a one-year follow-up period with satisfactory psychometric properties. The SelfCog showed larger effect sizes than the classical cognitive assessments. Its scores were associated with grey and white matter damage at baseline and over one year.

Conclusions The SelfCog should likely become a very useful tool for measuring cognition in HD in the future. It highlights the value of moving the field along the neuroscience principles.

  • digitized cognitive assessment
  • longitudinal follow-up
  • clinical trial

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