title: Problem solving, impulse control and planning in patients with early- and late-stage Huntington's disease.
abstract: Sub-domains of executive functions, including problems with planning, accuracy, impulsivity, and inhibition, are core features of Huntington's disease. It is known that the decline of cognitive function in Huntington's disease is related to the anatomical progression of pathology in the basal ganglia. However, it remains to be determined whether the severity of executive dysfunction depends on the stage of the disease. To examine the severity of sub-domains of executive dysfunction in early- and late-stage Huntington's disease, we studied performance in the Tower of London task of two groups of Huntington's disease patients (Group 1: early, n = 23, and Group 2: late stage, n = 29), as well as a third group of age, education, and IQ matched healthy controls (n = 34). During the task, we measured the total number of problems solved, total planning time, and total number of breaks taken. One aspect of executive function indexed by the number of solved problems seems to progress in the course of the disease. Late-stage Huntington's disease patients scored significantly worse than early-stage patients and controls, and early-stage patients scored significantly worse than controls on this measure of accuracy. In contrast, late- and early-stage HD patients did not differ in terms of planning time and number of breaks. Early- and late-stage HD pathology has a different impact on executive sub-domains. While accuracy differs between early- and late-stage HD patients, other domains like planning time and number of breaks do not. Striatal degeneration, which is a characteristic feature of the disease, might not affect all aspects of executive function in HD.
authors.affiliation: Department of Psychiatry, Medical University of Graz, Auenbruggerplatz 31/1, 8036, Graz, Austria.
authors.forename: Sabrina
authors.initials: S
authors.lastname: Mörkl
authors.affiliation: Department of Psychiatry, Medical University of Graz, Auenbruggerplatz 31/1, 8036, Graz, Austria.
authors.forename: Nicole J
authors.initials: NJ
authors.lastname: Müller
authors.affiliation: Department of Psychiatry, Medical University of Graz, Auenbruggerplatz 31/1, 8036, Graz, Austria. claudia.blesl@medunigraz.at.
authors.forename: Claudia
authors.initials: C
authors.lastname: Blesl
authors.affiliation: Behavioral Neurology Unit, National Institute of Neurological Disorders and Stroke, National Institutes of Health, 10 Center Dr., MSC 1440, Bethesda, MD, 20892-1440, USA.
authors.forename: Leonora
authors.initials: L
authors.lastname: Wilkinson
authors.affiliation: Department of Psychiatry, Medical University of Graz, Auenbruggerplatz 31/1, 8036, Graz, Austria.
authors.forename: Adelina
authors.initials: A
authors.lastname: Tmava
authors.affiliation: Department of Psychiatry, Medical University of Graz, Auenbruggerplatz 31/1, 8036, Graz, Austria.
authors.forename: Walter
authors.initials: W
authors.lastname: Wurm
authors.affiliation: Department of Psychiatry, Medical University of Graz, Auenbruggerplatz 31/1, 8036, Graz, Austria.
authors.forename: Anna K
authors.initials: AK
authors.lastname: Holl
authors.affiliation: Department of Psychiatry, Medical University of Graz, Auenbruggerplatz 31/1, 8036, Graz, Austria.
authors.forename: Annamaria
authors.initials: A
authors.lastname: Painold
mesh_terms: D000328:Adult; D000368:Aged; D060825:Cognitive Dysfunction / Q000209:etiology / Q000503:physiopathology*; D056344:Executive Function / Q000502:physiology*; D005260:Female; D006801:Humans; D006816:Huntington Disease / Q000150:complications / Q000503:physiopathology*; D007175:Impulsive Behavior / Q000502:physiology*; D007266:Inhibition, Psychological; D008297:Male; D008875:Middle Aged; D011340:Problem Solving / Q000502:physiology*; D012720:Severity of Illness Index
keywords: Disease severity; Executive function; Huntington’s disease; Neuropsychology; Tower of London