Comparison of the Neuropsychological Deficits Associated With Early and Advanced Huntington's Disease

Butters, Nelson; Sax, Daniel S.; Montgomery, Kathleen; Tarlow, Susan

doi:10.1001/archneur.1978.00500330033006

Cited by 237 publications

(88 citation statements)

References 11 publications

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“…Cognitive dysfunction is considered to be intrinsic to HD, and there is abundant neuropsychological evidence for WM and executive deficits in HD patients both during early and late stages of the disorder [Butters et al, 1978;Lange et al, 1995;Paulsen et al, 1995]. In accordance with previous studies on cognitive dysfunction in HD [Zakzanis, 1998], the behavioral data provided by this study is indicative of demonstrated a widespread deficit in a variety of cognitive domains including alertness, divided attention, verbal and spatial WM, and executive function.…”

Section: Discussionsupporting

confidence: 88%

“…Apart from its role in the central executive subcomponent of WM [Baddeley, 2003], the DLPFC has been implicated in mediating cognitive and behavioral control as well as in sustaining supraspan memory capacity [Rypma et al, 2002]. Thus, hypoactivation of the left DLPFC in HD patients might reflect a fundamental functional deficit associated with cognitive and behavioral deficits found in early stages of HD [Butters et al, 1978].…”

Section: Discussionmentioning

confidence: 99%

“…Cognitive dysfunction is considered to be an intrinsic feature of HD, varying within the affected cognitive domains over time [Brandt and Butters, 1986;Rosas et al, 2004]. While an impairment of attention, concentration, visuospatial processing, and mnemonic function can be observed during early stages of HD [Butters et al, 1978], HD patients exhibit deficits in executive function on tests requiring planning, problem solving, cognitive flexibility, and the maintenance of a cognitive set at later stages of the disorder [Butters et al, 1978;Lange et al, 1995;Paulsen et al, 1995]. Over time, memory deficits become more evident, including impairments in working memory (WM), as well as declarative and procedural memory.…”

Section: Introductionmentioning

confidence: 99%

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Cortical dysfunction in patients with Huntington's disease during working memory performance

Wolf

Vasić

Schönfeldt‐Lecuona

et al. 2008

Human Brain Mapping

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Previous functional neuroimaging studies on executive function suggested multiple functionally aberrant cortical regions in patients with Huntington's disease (HD). However, little is known about the neural mechanisms of working memory (WM) function in this patient population. The objective of this study was to investigate the functional neuroanatomy of WM in HD patients. We used event-related functional magnetic resonance imaging and a parametric verbal WM task to investigate cerebral function during WM performance in 16 healthy control subjects and 12 mild to moderate stage HD patients. We excluded incorrectly performed trials to control for potential accuracy-related activation confounds. Voxel-based morphometry (VBM) was used to control for confounding cortical and subcortical atrophy. We found that HD patients were slower and less accurate than healthy controls across all WM load levels. In addition, HD patients showed lower activation in the left dorso-and ventrolateral prefrontal cortex, the left inferior parietal cortex, the left putamen, and the right cerebellum at high WM load levels only. VBM revealed gray matter differences in the bilateral caudate nucleus and the thalamus, as well as in inferior parietal and right lateral prefrontal regions. However, volumetric abnormalities in the patient group did not affect the activation differences obtained during WM task performance. These findings demonstrate that WM-related functional abnormalities in HD patients involve distinct WM network nodes associated with cognitive control and subvocal rehearsal. Moreover, aberrant cortical function in HD patients may occur in brain regions, which are relatively well preserved in terms of brain atrophy.

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Section: Discussionsupporting

confidence: 88%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Cortical dysfunction in patients with Huntington's disease during working memory performance

Wolf

Vasić

Schönfeldt‐Lecuona

et al. 2008

Human Brain Mapping

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“…These deficits are consistent with extensive damage to the medial temporal lobe structures important for memory (e.g., hippocampus, entorhinal cortex) and to the association cortices of the frontal, temporal, and parietal lobes (Braak & Braak, 1998;Brun & England, 1981;Terry & Katzman, 1983). HD, in contrast, is characterized by marked motor disturbance (i.e., choreiform movements), bradyphrenia, prominent executive dysfunction, moderate concentration and memory deficits, and visuospatial impairments (Brandt & Butters, 1986;Butters et al, 1978;Salmon et al, 2001a). The cognitive and motor deficits of HD are mediated by the extensive basal ganglia damage, particularly to the caudate nucleus and putamen, that occurs in the disease (Alexander et al, 1986;Bruyn et al, 1979;Vonsattel et al, 1985) and the attendant interruption of several anatomically and functionally distinct circuits that link these subcortical structures with the frontal cortex (for review, see Middleton & Strick, 2001).…”

Section: Introductionmentioning

confidence: 74%

Differential effects of Alzheimer's disease and Huntington's disease on the performance of mental rotation

Lineweaver

Salmon

Bondi

et al. 2005

J Int Neuropsychol Soc

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The ability to spatially rotate a mental image was compared in patients with Alzheimer's disease (AD; n = 18) and patients with Huntington's disease (HD; n = 18). Compared to their respective age-matched normal control (NC) group, the speed, but not the accuracy, of mental rotation abnormally decreased with increasing angle of orientation for patients with HD. In contrast, the accuracy, but not the speed, of rotation abnormally decreased with increasing angle of orientation for patients with AD. Additional analyses showed that these unique patterns of performance were not attributable to different speed/accuracy trade-off sensitivities. This double dissociation suggests that the distinct brain regions affected in the two diseases differentially contribute to speed and accuracy of mental rotation. Specifically, the slowing exhibited by HD patients may be mediated by damage to the basal ganglia, whereas the spatial manipulation deficit of AD patients may reflect pathology in parietal and temporal lobe association cortices important for visuospatial processing. (JINS, 2005, 11, 30-39.) 2

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“…This was measured using the paragraph recall test (Wechsler 1945;Wechsler 1987), a validated (Squire 1987) and sensitive measure (Butters et al 1978;Storandt et al 1984) of verbal declarative memory performance. Subjects hear two recorded narratives, each with 44 pieces of information, followed by immediate and delayed (30 min) verbatim recall.…”

Section: Declarative Memory Measuresmentioning

confidence: 99%

Ketamine-Induced NMDA Receptor Hypofunction as a Model of Memory Impairment and Psychosis

Newcomer

Farber

Jevtović-Todorović

et al. 1999

Neuropsychopharmacology

541

350

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Considerable research interest has recently been focused on the role of glutamate and related neural circuitry in the neurobiology of schizophrenia. The results of these investigations have emphasized hypofunction of glutamatergic neurons and/or the N-methyl-D-aspartate (NMDA) glutamate receptor (Tsai et al. 1995;Kim et al. 1980aKim et al. , 1980bSherman et al. 1991;Deutsch et al. 1989;Javitt and Zukin 1991;Olney 1988a;Olney 1988b;Olney and Farber 1995). An important element of several of these theoretical positions is that NMDA receptor hypofunction (NRH) produced by any mechanism can be psychotogenic. This has renewed interest in the clinical effects of NMDA glutamate receptor antagonists.Ketamine and phencyclidine (PCP) are non-competitive NMDA glutamate receptor antagonists (Zukin and Zukin 1979;Vincent et al. 1979;Lodge and Anis 1982;Lodge et al. 1987) which can produce a transient state of NRH in the brain. Early investigators characterized a PCP-induced clinical syndrome of schizophrenia-like Received March 18, 1998; revised June 19, 1998; accepted June 29, 1998. N EUROPSYCHOPHARMACOLOGY 1999 -VOL . 20 , NO . 2 NMDA Receptor Hypofunction Induced Memory Decrease 107 symptoms, including hallucinations, delusions, idiosyncratic and illogical thinking, poverty of speech and thought, agitation, disturbances of emotion, affect, withdrawal, decreased motivation, and dissociation (Johnstone et al. 1959;Luby et al. 1959;Rosenbaum et al. 1959;Luby et al. 1962;Corssen and Domino 1966;Bakker and Amini 1961;Davies and Beech 1960;Domino and Luby 1981). This PCP-induced syndrome can be indistinguishable from acute presentations of schizophrenia (Yesavage and Freeman 1978;Erard et al. 1980). Ketamine, a PCP analog still used in human anesthesia, has been reported to cause reactions similar to but not as severe as those caused by PCP, including brief, reversible "positive" and "negative" schizophrenia-like symptoms (Krystal et al. 1994;Malhotra et al. 1996). Both PCP and ketamine can exacerbate psychosis in schizophrenia Luby et al. 1962;Lahti et al. 1995a;Lahti et al. 1995b;Malhotra et al. 1997).Declarative, explicit or secondary memory and learning deficits in patients with schizophrenia occur early in the course of the illness and are quantitatively large compared with deficits in other differentiated elements of cognitive performance, showing stability "on" versus "off" antipsychotic medication and over repeated testing (Gruzelier et al. 1988;Saykin et al. 1991Saykin et al. , 1994Cannon et al. 1994). Clinical and preclinical investigations suggest the hypothesis that changes in NMDA glutamate receptor activity in patients with schizophrenia may be causally related to memory impairments found in this disorder. Relevant to this hypothesis, the activation of post-synaptic NMDA receptors is important for the induction of the activity-dependent synaptic modification called long-term potentiation (LTP) (Bliss and Collingridge 1993;Collingridge and Bliss 1995), and hippocampal LTP has been postulated to underlie cert...

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Comparison of the Neuropsychological Deficits Associated With Early and Advanced Huntington's Disease

Cited by 237 publications

References 11 publications

Cortical dysfunction in patients with Huntington's disease during working memory performance

Cortical dysfunction in patients with Huntington's disease during working memory performance

Differential effects of Alzheimer's disease and Huntington's disease on the performance of mental rotation

Ketamine-Induced NMDA Receptor Hypofunction as a Model of Memory Impairment and Psychosis

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