Cognitive inhibition impairments in presymptomatic C9orf72 carriers. Issue 4 (13th February 2020)

• Record Type: Journal Article
• Authors: Montembeault, Maxime; Sayah, Sabrina; Rinaldi, Daisy; Le Toullec, Benjamin; Bertrand, Anne; Funkiewiez, Aurélie; Saracino, Dario; Camuzat, Agnès; Couratier, Philippe; Chouly, Marianne; Hannequin, Didier; Aubier-Girard, Carole; Pasquier, Florence; Delbeuck, Xavier; Colliot, Olivier; Batrancourt, Bénédicte; Azuar, Carole; Lévy, Richard; Dubois, Bruno; Le Ber, Isabelle; Migliaccio, Raffaella
• Other Names: author non-byline.; Girard Carole Aubier author non-byline.; Benchetrit Eve author non-byline.; Bertin Hugo author non-byline.; Bertrand Anne author non-byline.; Bioux Sandrine author non-byline.; Bissery Anne author non-byline.; Bliaux Évangéline author non-byline.; Bombois Stéphanie author non-byline.; Boncoeur Marie-Paule author non-byline.; Cassagnaud Pascaline author non-byline.; Chastan Mathieu author non-byline.; Chen Yaohua author non-byline.; Chupin Marie author non-byline.; Colliot Olivier author non-byline.; Couratier Philippe author non-byline.; Delbeuck Xavier author non-byline.; Deramecourt Vincent author non-byline.; Delmaire Christine author non-byline.; Gerardin Emmanuel author non-byline.; Hossein-Foucher Claude author non-byline.; Dubois Bruno author non-byline.; Habert Marie-Odile author non-byline.; Hannequin Didier author non-byline.; Lautrette Géraldine author non-byline.; Lebouvier Thibaud author non-byline.; Ber Isabelle Le author non-byline.; Lehéricy Stéphane author non-byline.; Toullec Benjamin Le author non-byline.; Levy Richard author non-byline.; Martinaud Olivier author non-byline.; Martineau Kelly author non-byline.; Mackowiak Marie-Anne author non-byline.; Monteil Jacques author non-byline.; Pasquier Florence author non-byline.; Petyt Grégory author non-byline.; Pradat Pierre-François author non-byline.; Pouliquen Dorothée author non-byline.; Oya Assi-Hervé author non-byline.; Rinaldi Daisy author non-byline.; Rollin-Sillaire Adeline author non-byline.; Salachas François author non-byline.; Sayah Sabrina author non-byline.; Wallon David author non-byline.;
• Is Part Of: Journal of neurology, neurosurgery and psychiatry. Volume 91:Issue 4(2020)
• Page Start: 366
• Page End: 372
• Publication Date: 2020-02-13
• Subjects: C9orf72 mutation -- cognitive inhibition -- hayling sentence completion test -- voxel-based morphometry -- cerebellum; Neurology -- Periodicals; Nervous system -- Surgery -- Periodicals; Psychiatry -- Periodicals; 616.8
• DOI: 10.1136/jnnp-2019-322242
• Languages: English
• ISSNs: 0022-3050

Abstract

Abstract : Objective: 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.