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Background: The Dandy–Walker Malformation (DWM) is a congenital birth malformation that is characterized by a triad of features: cerebellar dysgenesis, cystic dilation of the fourth ventricle, and an enlarged posterior fossa that displaces the dural sinuses and the tentorium. Despite this defining triad, clinical presentation can be highly heterogeneous in part due to severity of structural changes. To date, there been limited consideration of cognitive-behavioral symptoms of DWM in relation to nonmotor functions of the cerebellum, specifically cerebellar cognitive affective syndrome (CCAS). Method: In this case study, we describe the neuropsychological and behavioral profile of a 48-year-old man with DWM who was seen due to concerns, expressed solely by the patient's father, about his son's atypical housing, employment and social skills. Results: Neuropsychological test findings revealed high average intellect on standard intellectual measures (WAIS-IV), with stronger verbal (superior) than perceptual reasoning (average) skills. Across all cognitive domains, performance was generally within expectations, although bilateral fine motor skills were impaired. In contrast, he exhibited weaknesses on nontraditional neuropsychological measures assessing orbitofrontal-limbic circuitry, including reward sensitivity decision making and indices of threat-related emotional physiology. Conclusions: Through the use of traditional and nontraditional neuropsychological measures, subtle cognitive weaknesses in fronto-executive and affective regulation were illuminated and likely explain the patient's functional difficulties. Etiologically, these findings are consistent with the nonmotor functions of the cerebellum as described by CCAS. [ABSTRACT FROM AUTHOR]

Objective: Many patients require repeat neuropsychological evaluations to determine change over time. Repeat evaluations lead to practice effects, which can impact the validity of the assessment. The current study assessed, in older adults, the validity of an alternative set of verbal memory stories created by Newcomer and colleagues. Method: A total 154 of non-demented adults, ages 60–92, completed the WMS-III logical memory (LM) stories and two Newcomer stories (Carson–Jones) as part of a larger battery of neurocognitive tests. The Carson–Jones stories were scored for: (1) verbatim (traditional) and (2) thematic (developed for this study) accuracy. Story memory variables were compared to each other and additional neurocognitive measures using bivariate correlations. A subset of participants (n = 133) completed magnetic resonance imaging (MRI) and various structural regions (e.g. thickness and volume of medial temporal lobe structures) were used to assess external validity of Carson–Jones stories with hierarchical multiple regression analyses. Results: There was a strong positive correlation between WMS-III LM and Carson–Jones stories for both verbatim and thematic scoring. Both scoring types showed convergent validity with other verbal memory measures (e.g. WMS-III LM and HVLT-R Delay/Learning) and divergent validity with Stroop Word Reading and JOLO. Regarding neuroimaging correlates, Carson–Jones verbatim scoring was significantly associated with left subiculum and left whole hippocampal volume whereas thematic scoring was significantly associated only with left subiculum. Conclusions: Newcomer stories appear to be a valid alternative to WMS-III LM stories in terms of assessing verbal memory in healthy older adults. [ABSTRACT FROM AUTHOR]

Exercise “stress tests” are widely used to assess cardiovascular function and to detect abnormalities. In line with the view of exercise as a stressor, the present study examined the relationship between cognitive function and cardiovascular activity before and after light physical exercise in a sample of 84 non-demented community-dwelling older adults. Based on known relationships between hypertension, executive function and cerebral white matter changes, we hypothesized that greater post-exercise reactivity, as indexed by higher pulse pressure, would be more related to worse performance on frontal-executive tasks than pre-exercise physiologic measures. All participants were administered a comprehensive neuropsychological battery and underwent a Six Minute Walk Test (6MWT), with blood pressure (BP) measures obtained immediately before and after the walk. Pulse pressure (PP) was derived from BP as an indicator of vascular auto-regulation and composite scores were computed for each cognitive domain assessed. As predicted, worse executive function scores exhibited a stronger relationship with post-exercise PP than pre-exercise PP. Results suggest that PP following system stress in the form of walking may be more reflective of the state of vascular integrity and associated executive dysfunction in older adults than baseline physiologic measures. [ABSTRACT FROM PUBLISHER]

Exercise "stress tests" are widely used to assess cardiovascular function and to detect abnormalities. In line with the view of exercise as a stressor, the present study examined the relationship between cognitive function and cardiovascular activity before and after light physical exercise in a sample of 84 non-demented community-dwelling older adults. Based on known relationships between hypertension, executive function and cerebral white matter changes, we hypothesized that greater post-exercise reactivity, as indexed by higher pulse pressure, would be more related to worse performance on frontal-executive tasks than pre-exercise physiologic measures. All participants were administered a comprehensive neuropsychological battery and underwent a Six Minute Walk Test (6MWT), with blood pressure (BP) measures obtained immediately before and after the walk. Pulse pressure (PP) was derived from BP as an indicator of vascular auto-regulation and composite scores were computed for each cognitive domain assessed. As predicted, worse executive function scores exhibited a stronger relationship with post-exercise PP than pre-exercise PP. Results suggest that PP following system stress in the form of walking may be more reflective of the state of vascular integrity and associated executive dysfunction in older adults than baseline physiologic measures.