Systemic lupus erythematosus (SLE) and multiple sclerosis (MS) are chronic immunologic diseases that can cause cognitive dysfunction. MS is a central nervous system (CNS) disease characterized by demyelination and progressive brain atrophy. SLE is an autoimmune disease capable of damaging multiple organ systems, including the CNS. Cognitive disturbances are seen in both SLE and MS. The present study is concerned with understanding the similarities and differences between the cognitive profiles of SLE and MS as well as the relationship between cognitive impairment and vocational disability in these patients. We examined 47 SLE patients, 47 MS patients, and 44 healthy controls. The groups were well matched on demographics and the patient groups were also matched on disease duration and severity. Group comparisons revealed that generative verbal fluency and visual-spatial memory are more profoundly affected in MS than SLE; whereas depression, fatigue, and working memory deficits are similarly involved in both diseases. Logistic regression analysis revealed that executive function, in particular, was predictive of vocational outcomes in SLE and MS patients. (JINS, 2012, 18, 530–540)

Several authors have recently shown that anterograde amnesia is often associated with semantic memory impairment in amnesic MCI patients. Similarly, after the MCI condition, some patients who convert to Alzheimer's disease (AD) show the classic onset (cAD) characterized by the impairment of memory and executive functions, whereas other AD patients show isolated defects of episodic and semantic memory without deficits in other cognitive domains. The latter have been considered an AD variant characterized by ‘focal Temporal Lobe Dysfunction’ (TLD). The aim of the present study was to assess the differences in disease progression between cAD and TLD. For this purpose a continuous series of newly diagnosed probable AD patients presenting as cAD (n = 30) and TLD (n = 25), matched for severity, and 65 healthy controls underwent a comprehensive neuropsychological evaluation at baseline; TLD and cAD were re-evaluated at a 24-month follow-up. At follow-up, TLD patients showed no significant worsening of cognitive functions, whereas cAD subjects displayed a significant worsening in all explored cognitive domains. In conclusion, our results confirm that probable AD presenting as TLD represents a specific onset of AD characterized by a slower rate of progression. (JINS, 2012, 18, 144–150)

Literature suggests that individuals with mild traumatic brain injury (mTBI) show subtle abnormalities in the cognitive control process of performance monitoring. The neural bases of performance monitoring can be measured using the error-related negaitivity (ERN) and post-error positivity (Pe) components of the scalp-recorded event-related potential (ERP). Thirty-six individuals with mTBI and 46 demographically similar controls completed a modified color-naming Stroop task while ERPs were recorded. Separate repeated-measures analyses of variance were used to examine the behavioral (response times [RT] and error rates) and ERP (ERN and Pe amplitudes) indices of performance monitoring. Both groups showed slower RTs and increased error rates on incongruent trials relative to congruent trials. Likewise, both groups showed more negative ERN and more positive Pe amplitude to error trials relative to correct trials. Notably, there were no significant main effects or interactions of group for behavioral and ERP measures. Subgroup and correlational analyses with post-concussive symptoms and indices of injury severity were also not significant. Findings suggest comparable performance to non-injured individuals in some aspects of cognitive control in this sample. Neuropsychological implications and comparison with other cognitive control component processes in individuals with TBI are provided. (JINS, 2012, 18, 323–333)

Mutations in the progranulin (PGRN) gene have been identified as a cause of frontotemporal dementia (FTD). However, little is known about the neuropsychological abilities of asymptomatic carriers of these mutations. The aim of the study was to assess cognitive functioning in asymptomatic c.709-1G>A PGRN mutation carriers. We hypothesized that poorer neuropsychological performance could be present before the development of clinically significant FTD symptoms. Thirty-two asymptomatic first-degree relatives of FTD patients carrying the c.709-1G>A mutation served as study participants, including 13 PGRN mutation carriers (A-PGRN+) and 19 non-carriers (PGRN-). A neuropsychological battery was administered. We found that the A-PGRN+ participants obtained significantly poorer scores than PGRN- individuals on tests of attention (Trail-Making Test Part A), mental flexibility (Trail-Making Test Part B), and language (Boston Naming Test). Poorer performance on these tests in asymptomatic PGRN mutation carriers may reflect a prodromal phase preceding the onset of clinically significant symptoms of FTD. (JINS, 2012, 18, 1086–1090)

Memory deficits for survivors of aneurysmal subarachnoid hemorrhage (SAH) are common, however, the nature of these deficits is not well understood. In this study, 24 patients with SAH and matched control participants were asked to study six lists containing words from four different categories. For half the lists, the categories were presented together (organized lists). For the remaining lists, the related words were presented randomly to maximize the use of executive processes such as strategy and organization (unorganized lists). Across adjoining lists, there was overlap in the types of categories given, done to promote intrusions. Compared to control participants, SAH patients recalled a similar number of words for the organized lists, but significantly fewer words for the unorganized lists. SAH patients also reported more intrusions than their matched counterparts. Separating patients into anterior communicating artery ruptures (ACoA) and ruptures in other regions, there was a recall deficit only for the unorganized list for those with ACoA ruptures and deficits across both list types for other rupture locations. These results suggest that memory impairment following SAH is likely driven by impairment in the executive components of memory, particularly for those with ACoA ruptures. Such findings may help direct future cognitive-therapeutic programs. (JINS, 2012, 18, 334–342)

Amnestic mild cognitive impairment (aMCI) represents a group of individuals who are highly likely to develop Alzheimer's disease (AD). Although aMCI is typically conceptualized as involving predominantly deficits in episodic memory, recent studies have demonstrated that deficits in executive functioning may also be present, and thorough categorization of cognitive functioning in MCI may improve early diagnosis and treatment of AD. We first provide an extensive review of neuropsychology studies that examined executive functioning in MCI. We then present data on executive functioning across multiple sub-domains (divided attention, working memory, inhibitory control, verbal fluency, and planning) in 40 aMCI patients (single or multiple domain) and 32 normal elderly controls (NECs). MCI patients performed significantly worse than NECs in all 5 sub-domains, and there was impairment (>1.0 SD below the mean of NECs) in all sub-domains. Impairment on each test was frequent, with 100% of MCI patients exhibiting a deficit in at least one sub-domain of executive functioning. Inhibitory control was the most frequently and severely impaired. These results indicate that executive dysfunction in multiple sub-domains is common in aMCI and highlights the importance of a comprehensive neuropsychological evaluation for fully characterizing the nature and extent of cognitive deficits in MCI. (JINS, 2012, 18, 541–555)

We examined two-wave longitudinal changes in two indicators of neurocognitive speed (i.e., mean rate, intraindividual variability) using one simple and three complex reaction time tasks. Participants included idiopathic Parkinson's disease (PD) patients, with and without incipient dementia, and normal controls. At baseline, there were 45 patients (26 men, 19 women) with idiopathic PD who ranged from 65 to 84 years (M = 71.3; SD = 4.5) and 47 matched controls (27 men, 20 women) who ranged from 65 to 84 years (M = 71.4; SD = 4.9). The 18-month longitudinal sample comprised of 74 returning participants (43 controls; 31 PD patients) who had no cognitive impairment or dementia at both waves. Ten of the 31 PD patients returning for Time 3 had dementia or cognitive impairment. These constituted the PD with incipient dementia (PDID) group. Repeated measures analyses of variance showed that the PD and PDID groups were slower over time on the reaction time tasks, whereas the controls improved their performance over time on all tasks. Inconsistency distinguished the two clinical groups (i.e., the PDID group but not the PD group became more inconsistent over time). Changes in neurocognitive speed and inconsistency may be valid clinical markers of PDID. (JINS, 2012, 18, 1–9)

Contemporary research indicates that brain development occurs during childhood and into early adulthood, particularly in certain regions. A critical question is whether premature or atypical hormone exposures impact brain development (e.g., structure) or function (e.g., neuropsychological functioning). The current study enrolled 40 girls (aged 6–8 years) diagnosed with premature adrenarche (PA) and a comparison group of 36 girls with on-time maturation. It was hypothesized that girls with PA would demonstrate lower IQ and performance on several neuropsychological tasks. The potential for a sexually dimorphic neuropsychological profile in PA was also explored. No significant univariate or multivariate group differences emerged for any neuropsychological instrument. However, effect size confidence intervals contained medium-sized group differences at the subscale level. On-time girls performed better on verbal, working memory, and visuospatial tasks. Girls with PA showed improved attention, but not a sexually dimorphic profile. These results, though preliminary, suggest that premature maturation may influence neuropsychological functioning. (JINS, 2012, 18, 151–156)

In addition to having difficulties with social communications, individuals with an autism spectrum disorder (ASD) often also experience impairment in higher-order, executive skills. The present study examined the effects of pharmacological modulation of the norepinephrine system on the severity of such impairments. A sample of 14 high-functioning adults with ASD and a demographically-matched comparison group of 13 typically developing individuals participated. An AX continuous performance test (AX-CPT) was used to evaluate working memory and inhibitory control. AX-CPT performance was assessed following administration of a single dose of propranolol (a beta adrenergic antagonist) and following placebo (sugar pill) administration. Individuals with ASD performed more poorly than non-ASD individuals in the working memory condition (BX trials). Importantly, administration of propranolol attenuated this impairment, with the ASD group performing significantly better in the propranolol condition than the placebo condition. Working memory performance of the non-ASD group was unaffected by propranolol/placebo administration. No group or medication effects were observed for the inhibition condition (AY trials). The present findings suggest that norepinephrine may play a role in some, but not necessarily all, cognitive impairments associated with ASD. Additional research is needed to fully understand whether this role is primarily causal or compensatory in nature. (JINS, 2012, 18, 556–564)

Individuals with anorexia nervosa (AN) are often characterized as possessing excessive self-control and are unusual in their ability to reduce or avoid the consumption of palatable foods. This behavior promotes potentially life-threatening weight loss and suggests disturbances in reward processing. We studied whether individuals with AN showed evidence of increased self-control by examining the tendency to delay receipt of a monetary, non-food related, reward. Underweight AN (n = 36) and healthy controls (HC, n = 28) completed a monetary intertemporal choice task measuring delay discounting factor. Individuals with AN reduced the value of a monetary reward over time significantly less than HC (F[1,61] = 5.03; p = 0.029). Secondary analyses indicated that the restricting subtype of AN, in particular, showed significantly less discounting than HC (F[1,46] = 8.3; p = 0.006). These findings indicate that some individuals with AN show less temporal discounting than HC, suggestive of enhanced self-control that is not limited to food consumption. This is in contrast to other psychiatric disorders, for example, substance abuse, which are characterized by greater discounting. Though preliminary, these findings suggest that excessive self-control may contribute to pathological processes and individuals with AN may have neuropsychological characteristics that enhance their ability to delay reward and thereby may help to maintain persistent food restriction. (JINS, 2012, 18, 1–8)

Septal nuclei, components of basal forebrain, are strongly and reciprocally connected with hippocampus, and have been shown in animals to play a critical role in memory. In humans, the septal forebrain has received little attention. To examine the role of human septal forebrain in memory, we acquired high-resolution magnetic resonance imaging scans from 25 healthy subjects and calculated septal forebrain volume using recently developed probabilistic cytoarchitectonic maps. We indexed memory with the California Verbal Learning Test-II. Linear regression showed that bilateral septal forebrain volume was a significant positive predictor of recognition memory accuracy. More specifically, larger septal forebrain volume was associated with the ability to recall item source/context accuracy. Results indicate specific involvement of septal forebrain in human source memory, and recall the need for additional research into the role of septal nuclei in memory and other impairments associated with human diseases. (JINS, 2012, 18, 157–161)

Moderate and severe pediatric traumatic brain injuries (TBI) are associated with significant familial distress and child adaptive sequelae. Our aim was to examine the relationship between parental psychological distress, parenting practices (authoritarian, permissive, authoritative), and child adaptive functioning 12–36 months following TBI or orthopedic injury (OI). Injury type was hypothesized to moderate the relationship between parental distress and child adaptive functioning, demonstrating a significantly stronger relationship in the TBI relative to OI group. Authoritarian parenting practices were hypothesized to mediate relationship between parental distress and child adaptive functioning across groups. Groups (TBI n = 21, OI n = 23) did not differ significantly on age at injury, time since injury, sex, race, or SES. Parents completed the Brief Symptom Inventory, Parenting Practices Questionnaire, and Vineland-II. Moderation and mediation hypotheses were tested using hierarchical multiple regression and a bootstrapping approach, respectively. Results supported moderation and revealed that higher parental psychological distress was associated with lower child adaptive functioning in the TBI group only. Mediation results indicated that higher parental distress was associated with authoritarian parenting practices and lower adaptive functioning across groups. Results suggest that parenting practices are an important area of focus for studies attempting to elucidate the relationship between parent and child functioning following TBI. (JINS, 2012, 18, 343–350)

Decreased productivity on verbal fluency tasks by persons with schizophrenia has been attributed to semantic system abnormalities. Semantic structure is often assessed using multidimensional scaling (MDS) to detect normal and aberrant semantic clustering. However, MDS has limitations that may be particularly problematic for such assessments. Here, we introduce a different clustering technique, singular value decomposition (SVD), to elucidate abnormalities of the semantic system in schizophrenia. We compared 102 treated outpatients with schizophrenia to 109 healthy adults on two category-cued word fluency tasks. Patients with schizophrenia showed semantic clustering patterns that differ markedly from those of healthy adults. However, SVD revealed more detailed and critical semantic system abnormalities than previously appreciated using MDS. Patients with schizophrenia showed less coherent semantic clustering of both low- and high-frequency category exemplars than healthy adults. These results suggest the intriguing possibility that impaired automatic activation of semantic information is a key deficit in schizophrenia. (JINS, 2012, 18, 565–575)

Accessing the temporal position of events (early or late in the event sequence) can influence the generation of predictions about upcoming events. However, it is unclear how the temporal position is processed strategically. To investigate this, we presented event pairs to 23 healthy volunteers manipulating temporal order (chronological, inverse) and temporal position (early, late). Pupil dilation, eye movements, and behavioral data, showed that chronological and early event pairs are processed with more ease than inverse and late event pairs. Indexed by the pupillary response late events and inversely presented event pairs elicited greater cognitive processing demands than early events and chronologically presented event pairs. Regarding eye movements, fixation duration was less sensitive to temporal position than to temporal order. Looking at each item of the event sequence only once was behaviorally more effective than looking multiple times at each event regardless of whether temporal position or temporal order was processed. These results emphasize that accessing temporal position and temporal order information results in dissociable behavioral patterns. While more cognitive resources are necessary for processing late and inverse items, change of information acquisition strategies turns out to be most effective when temporal order processing is required. (JINS, 2012, 18, 351–360)

Although dialyzed patients often have cognitive problems, little is known about the nature of these deficits. We hypothesized that, in contrast to semantic fluency relying mainly on temporal lobes, phonemic fluency, preferentially depending on functions of frontal-subcortical systems, would be particularly sensitive to the constellation of physiological pathological processes associated with end-stage renal disease and dialysis. Therefore, we longitudinally compared phonemic and semantic fluency performance between 49 dialyzed patients and 30 controls. Overall, patients performed below controls only on the phonemic fluency task. Furthermore, their performance on this task declined over time, whereas there was no change in semantic fluency. Moreover, this decline was related to the presence of hypertension and higher blood urea nitrogen. We suggest that these findings may be due to a combination of vascular and topic effects that impact more on fronto-subcortical than temporal lobe networks, but this speculation requires direct confirmation. (JINS, 2012, 18, 162–167)