Arithmetic Word-Problem Solving as Cognitive Marker of Progression in Pre-Manifest and Manifest Huntington’s Disease

• Published in: Journal of Huntington's disease Vol. 10; no. 4; pp. 459 - 468
• Main Authors: Horta-Barba, Andrea, Martinez-Horta, Saul, Perez-Perez, Jesús, Sampedro, Frederic, de Lucia, Natascia, De Michele, Giuseppe, Salvatore, Elena, Kehrer, Stefanie, Priller, Josef, Migliore, Simone, Squitieri, Ferdinando, Castaldo, Anna, Mariotti, Caterina, Mañanes, Veronica, Lopez-Sendon, Jose Luis, Rodriguez, Noelia, Martinez-Descals, Asunción, Júlio, Filipa, Janurio, Cristina, Delussi, Marianna, de Tommaso, Marina, Noguera, Sandra, Ruiz-Idiago, Jesus, Sitek, Emilia J., Wallner, Renata, Nuzzi, Angela, Pagonabarraga, Javier, Kulisevsky, Jaime
• Format: Journal Article
• Language: English
• Published: London, England SAGE Publications 01.01.2021; Sage Publications Ltd
• Subjects: Attention; Biomarkers; Cognition; Cognitive ability; Diagnosis; Disease Progression; Executive Function; Humans; Huntingtin; Huntington Disease - genetics; Huntington's disease; Huntingtons disease; Mathematics; Memory; Mental task performance; Mutation; Neuropsychological Tests; Neuropsychology; Patients; Problem Solving; Short term memory; Visual perception; neuropsychology; acalculia; arithmetic; word-problem solving; Huntington’s disease; cognition
• ISSN: 1879-6397; 1879-6397; 1879-6400
• DOI: 10.3233/JHD-210480

Background: Arithmetic word-problem solving depends on the interaction of several cognitive processes that may be affected early in the disease in gene-mutation carriers for Huntington’s disease (HD). Objective: Our goal was to examine the pattern of performance of arithmetic tasks in premanifest and manifest HD, and to examine correlations between arithmetic task performance and other neuropsychological tasks. Methods: We collected data from a multicenter cohort of 165 HD gene-mutation carriers. The sample consisted of 31 premanifest participants: 16 far-from (>12 years estimated time to diagnosis; preHD-A) and 15 close-to (≤12 years estimated time to diagnosis; preHD-B), 134 symptomatic patients (early-mild HD), and 37 healthy controls (HC). We compared performance between groups and explored the associations between arithmetic word-problem solving and neuropsychological and clinical variables. Results: Total arithmetic word-problem solving scores were lower in preHD-B patients than in preHD-A (p < 0.05) patients and HC (p < 0.01). Early-mild HD patients had lower scores than preHD patients (p < 0.001) and HC (p < 0.001). Compared to HC, preHD and early-mild HD participants made more errors as trial complexity increased. Moreover, arithmetic word-problem solving scores were significantly associated with measures of global cognition (p < 0.001), frontal-executive functions (p < 0.001), attention (p < 0.001) visual working memory (p < 0.001), mental rotation (p < 0.001), and confrontation naming (p < 0.05). Conclusion: Arithmetic word-problem solving is affected early in the course of HD and is related to deficient processes in frontal-executive and mentalizing-related processes.