Neurocognitive profile of a man with Dandy-Walker malformation: Evidence of subtle cerebellar cognitive affective syndrome.

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.

Psychometric properties of the Brief Fatigue Inventory in community-dwelling older adults.

OBJECTIVE: To investigate the psychometric properties of the Brief Fatigue Inventory (BFI) in community-dwelling older adults. DESIGN: Cross-sectional validation study. SETTING: Community based. PARTICIPANTS: Subjects (N=302) were nondemented older adults (mean age, 76.44 y; 54% women). INTERVENTIONS: Not applicable. MAIN OUTCOME MEASURES: BFI total, severity, and interference summation scores. RESULTS: A principal component analysis (PCA) yielded 2 factors, fatigue severity and interference, explaining 65.94% of the variance. Both factors had good reliability, with Cronbach alpha values of .867 for fatigue interference and .818 for fatigue severity. Higher fatigue scores were associated with older age and worse physical and cognitive functions. CONCLUSIONS: Fatigue is a common and debilitating symptom in the aging population. The current study provides novel findings in validating and establishing a bidimensional factor structure for the BFI in older adults. Severity and interference were differentially related to important health outcomes; therefore, using these subscales in addition to the total BFI score is recommended with older adults. Because of its relatively short administration time and established psychometric properties, the BFI can be successfully incorporated into longitudinal studies and clinical trials.

Acts of generating and their sources: predicting the effects of imagery encoding on false recognition errors.

In the two experiments reported here the basis of the beneficial effects of generating images on false recognition errors is investigated. Acts of generating (descriptions, images, or both) were manipulated while examining the effects of the source of descriptions guiding imagery generations (participant vs peer). False recognition errors were relatively high across encoding conditions except when imagery generations were based on participants' own descriptions (Experiments 1 and 2). These differences in the acts of generating were not attributable to differences in the cohesiveness of descriptions themselves. Acts of generating led to greater "remember" responses than "know" responses only when participants were not the source of the descriptions used to generate images (Experiment 2). Results highlight the importance of examining the effects of the source of descriptions for guiding imagery (participant or peer) when testing predictions about the effects of imagery encoding on false recognition errors.

Imagery encoding and false recognition errors: Examining the role of imagery process and imagery content on source misattributions.

Imagery encoding effects on source-monitoring errors were explored using the Deese-Roediger-McDermott paradigm in two experiments. While viewing thematically related lists embedded in mixed picture/word presentations, participants were asked to generate images of objects or words (Experiment 1) or to simply name the items (Experiment 2). An encoding task intended to induce spontaneous images served as a control for the explicit imagery instruction conditions (Experiment 1). On the picture/word source-monitoring tests, participants were much more likely to report "seeing" a picture of an item presented as a word than the converse particularly when images were induced spontaneously. However, this picture misattribution error was reversed after generating images of words (Experiment 1) and was eliminated after simply labelling the items (Experiment 2). Thus source misattributions were sensitive to the processes giving rise to imagery experiences (spontaneous vs deliberate), the kinds of images generated (object vs word images), and the ways in which materials were presented (as pictures vs words).