Cognitive inhibition impairments in presymptomatic C9orf72 carriers - Archive ouverte HAL Access content directly
Journal Articles Journal of Neurology, Neurosurgery and Psychiatry Year : 2020

Cognitive inhibition impairments in presymptomatic C9orf72 carriers


bjective To investigate cognitive inhibition in presymptomatic C9orf72 mutation carriers (C9+) and its associated neuroanatomical correlates. Methods Thirty-eight presymptomatic C9orf72 mutation carriers (C9+, mean age 38.2±8.0 years) and 22 C9− controls from the PREV-DEMALS cohort were included in this study. They underwent a cognitive inhibition assessment with the Hayling Sentence Completion Test (HSCT; time to completion (part B−part A); error score in part B) as well as a 3D MRI. Results C9+ individuals younger than 40 years had higher error scores (part B) but equivalent HSCT time to completion (part B−part A) compared to C9− individuals. C9+ individuals older than 40 years had both higher error scores and longer time to completion. HSCT time to completion significantly predicted the proximity to estimated clinical conversion from presymptomatic to symptomatic phase in C9+ individuals (based on the average age at onset of affected relatives in the family). Anatomically, we found that HSCT time to completion was associated with the integrity of the cerebellum. Conclusion The HSCT represents a good marker of cognitive inhibition impairments in C9+ and of proximity to clinical conversion. This study also highlights the key role of the cerebellum in cognitive inhibition.
Fichier principal
Vignette du fichier
R1_Montembeault_Manuscript_NotMarked2.pdf (369.99 Ko) Télécharger le fichier

Dates and versions

hal-02484794 , version 1 (19-02-2020)



Maxime Montembeault, Sabrina Sayah, Daisy Rinaldi, Benjamin Le Toullec, Anne Bertrand, et al.. Cognitive inhibition impairments in presymptomatic C9orf72 carriers. Journal of Neurology, Neurosurgery and Psychiatry, 2020, ⟨10.1136/jnnp-2019-322242⟩. ⟨hal-02484794⟩
75 View
255 Download



Gmail Facebook Twitter LinkedIn More