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1.  Measuring Executive Dysfunction Longitudinally and in Relation to Genetic Burden, Brain Volumetrics, and Depression in Prodromal Huntington Disease 
Executive dysfunction (ED) is a characteristic of Huntington disease (HD), but its severity and progression is less understood in the prodromal phase, e.g., before gross motor abnormalities. We examined planning and problem-solving abilities using the Towers Task in HD mutation-positive individuals without motor symptoms (n = 781) and controls (n = 212). Participants with greater disease progression (determined using mutation size and current age) performed more slowly and with less accuracy on the Towers Task. Performance accuracy was negatively related to striatal volume while both accuracy and working memory were negatively related to frontal white matter volume. Disease progression at baseline was not associated with longitudinal performance over 4 years. Whereas the baseline findings indicate that ED becomes more prevalent with greater disease progression in prodromal HD and can be quantified using the Towers task, the absence of notable longitudinal findings indicates that the Towers Task exhibits limited sensitivity to cognitive decline in this population.
doi:10.1093/arclin/acs105
PMCID: PMC3569950  PMID: 23246934
Huntington's disease; Genetic disorders; Executive functions; Neuroimaging (structural); Norms/normative studies; Practice effects/reliable change; longitudinal change
2.  The Neuropsychological Course of Acute Delirium in Adult Hematopoietic Stem Cell Transplantation Patients 
Although delirium is a common medical comorbidity with altered cognition as its defining feature, few publications have addressed the neuropsychological prodrome, profile, and recovery of patients tested during delirium. We characterize neuropsychological performance in 54 hemapoietic stem cell/bone marrow transplantation (BMT) patients shortly before, during, and after delirium and in BMT patients without delirium and 10 healthy adults. Patients were assessed prospectively before and after transplantation using a brief battery. BMT patients with delirium performed more poorly than comparisons and those without delirium on cross-sectional and trend analyses. Deficits were in expected areas of attention and memory, but also in psychomotor speed and learning. The patients with delirium did not return to normative “average” on any test during observation. Most tests showed a mild decline in the visit before delirium, a sharp decline with delirium onset, and variable performance in the following days. This study adds to the few investigations of neuropsychological performance surrounding delirium and provides targets for monitoring and early detection; Trails A and B, RBANS Coding, and List Recall may be useful for delirium assessment.
doi:10.1093/arclin/acq103
PMCID: PMC3104596  PMID: 21183605
Bone marrow transplantation; Cognition; Cancer; Attention; Delirium
3.  Estimating Premorbid Functioning in Huntington's Disease: The Relationship between Disease Progression and the Wide Range Achievement Test Reading Subtest 
The estimation of premorbid abilities is an essential part of a neuropsychological evaluation, especially in neurodegenerative conditions. Although word pronunciation tests are one standard method for estimating the premorbid level, research suggests that these tests may not be valid in neurodegenerative diseases. Therefore, the current study sought to examine two estimates of premorbid intellect, the Wide Range Achievement Test (WRAT) Reading subtest and the Barona formula, in 93 patients with mild to moderate Huntington's disease (HD) to determine their utility and to investigate how these measures relate to signs and symptoms of disease progression. In 89% of participants, WRAT estimates were below the Barona estimates. WRAT estimates were related to worsening memory and motor functioning, whereas the Barona estimates had weaker relationships. Neither estimate was related to depression or functional capacity. Irregular word reading tests appear to decline with HD progression, whereas estimation methods based on demographic factors may be more robust but overestimate premorbid functioning.
doi:10.1093/arclin/acq088
PMCID: PMC3021970  PMID: 21147861
Huntington's disease; movement disorders; basal ganglia; assessment; dementia
4.  Predicting Cognitive Change in Older Adults: The Relative Contribution of Practice Effects 
Assessing cognitive change in older adults is a common use of neuropsychological services, and neuropsychologists have utilized several strategies to determine if a change is “real,” “reliable,” and “meaningful.” Although standardized regression-based (SRB) prediction formulas may be useful in determining change, SRBs have not been widely applied to older adults. The current study sought to develop SRB formulas on a group of 127 community-dwelling older adults for several widely used neuropsychological measures. In addition to baseline test scores and demographic information, the current study also examined the role of short-term practice effects in predicting test scores after 1 year. Consistent with prior research on younger adults, baseline test performances were the strongest predictors of future test performances, accounting for 25%–58% of the variance. Short-term practice effects significantly added to the predictability of all nine of the cognitive tests examined (3%–22%). Future studies should continue extending SRB methodology for older adults, and the inclusion of practice effects appears to add to the prediction of future cognition.
doi:10.1093/arclin/acp105
PMCID: PMC2819829  PMID: 20064816
Predicting cognition; Practice effects
5.  Practice effects in the prediction of long-term cognitive outcome in three patient samples: A novel prognostic index 
Practice effects, defined as improvements in cognitive test performance due to repeated exposure to the test materials, have traditionally been viewed as sources of error. However, they might provide useful information for predicting cognitive outcome. The current study used three separate patient samples (older adults with mild cognitive impairments, individuals who were HIV +, individuals with Huntington’s disease) to examine the relationship between practice effects and cognitive functioning at a later point. Across all three samples, practice effects accounted for as much as 31 to 83% of the variance in the follow-up cognitive scores, after controlling for baseline cognitive functioning. If these findings can be replicated in other patients with neurodegenerative disorders, clinicians and researchers may be able to develop predictive models to identify the individuals who are most likely to demonstrate continued cognitive decline across time. The ability to utilize practice effects data would add a simple, convenient, and non-invasive marker for monitoring an individual patient’s cognitive status. Additionally, this prognostic index could be used to offer interventions to patients who are in the earliest stages of progressive neurodegenerative disorders.
doi:10.1016/j.acn.2006.08.013
PMCID: PMC1847360  PMID: 17142007
practice effects; cognitive outcome; Mild Cognitive Impairment; HIV; Huntington’s disease

Results 1-5 (5)