The neural substrates of script knowledge deficits as revealed by a PET study in Huntington's disease

• Published in: Neuropsychologia Vol. 49; no. 9; pp. 2673 - 2684
• Main Authors: Allain, Philippe, Gaura, Véronique, Fasotti, Luciano, Chauviré, Valérie, Prundean, Adriana, Sherer-Gagou, Clarisse, Bonneau, Dominique, Bachoud-Levi, Anne-Catherine, Dubas, Frédéric, Remy, Philippe, Le Gall, Didier, Verny, Christophe
• Format: Journal Article
• Language: English
• Published: Kidlington Elsevier Ltd 01.07.2011; Elsevier
• Subjects: Adult; Adult and adolescent clinical studies; Basal Ganglia - metabolism; Basal Ganglia - physiopathology; Biological and medical sciences; Blood Glucose - metabolism; Brain - metabolism; Brain Mapping; Case-Control Studies; Cognition Disorders - complications; Cognition Disorders - physiopathology; Degenerative and inherited degenerative diseases of the nervous system. Leukodystrophies. Prion diseases; Female; Fluorodeoxyglucose F18; Frontal Lobe - metabolism; Frontal Lobe - physiopathology; Human Activities; Humans; Huntington Disease - complications; Huntington Disease - physiopathology; Huntington's disease; Hypometabolism; Medical sciences; Mental Processes - physiology; Middle Aged; Neostriatum - metabolism; Neostriatum - physiopathology; Nervous system (semeiology, syndromes); Nervous system as a whole; Neural Pathways - metabolism; Neural Pathways - physiopathology; Neurology; Organic mental disorders. Neuropsychology; PET; Positron-Emission Tomography; Psychology. Psychoanalysis. Psychiatry; Psychopathology. Psychiatry; Reference Values; Script knowledge; Serial Learning - physiology; Huntington's disease; Hypometabolism; Script knowledge; PET; Human; Script; Nervous system diseases; Huntington disease; Experimental study; Knowledge; Genetic disease; Cerebral disorder; Central nervous system disease; Degenerative disease; Extrapyramidal syndrome; Emission tomography
• ISSN: 0028-3932; 1873-3514
• DOI: 10.1016/j.neuropsychologia.2011.05.015

► The correlation between resting-state brain glucose utilization and performance on script generation and sorting tasks was examined in Huntington's disease. ► A selectively greater impairment for the organizational aspects of scripts was observed. ► Significant negative correlations between script errors and the metabolism of several cortical frontal and posterior regions were found. ► The cortical frontal regions are more crucial in script retrieval and script sequencing than the basal ganglia. Previous neuropsychological investigations have suggested that both the prefrontal cortex and the basal ganglia are involved in the management of script event knowledge required in planning behavior. This study was designated to map, the correlations between resting-state brain glucose utilization as measured by FDG-PET (positron emission tomography) and scores obtained by means of a series of script generation and script sorting tasks in 8 patients with early Huntington's disease. These patients exhibited a selectively greater impairment for the organizational aspects of scripts compared to the semantic aspects of scripts. We showed significant negative correlations between the number of sequencing, boundary, perseverative and intrusion errors and the metabolism of several cortical regions, not only including frontal, but also posterior regions. Our findings suggest that, within the fronto-striatal system, the cortical frontal regions are more crucial in script retrieval and script sequencing than the basal ganglia.