Subtle Neuropsychological Deficits Research Articles

Executive functioning of patients with congenital heart disease: 45 years after surgery

Abstract Background For children with congenital heart disease (CHD), subtle neuropsychological deficits have been reported. However, very little is known about executive functioning in their adulthood. Purpose To investigate the self- and informant-reported executive functioning in adults with CHD operated in childhood (<15 years old). Material and methods A cohort study of 194 patients (age 50 [46–54] years), operated in childhood between 1968 and 1980 for one of the following diagnosis: atrial septal defect (ASD), ventricular septal defect (VSD), pulmonary stenosis (PS), tetralogy of Fallot (ToF) or transposition of the great arteries (TGA), were evaluated 40–53 years after surgery. The “Behavior Rating Inventory of Executive Functions – Adult version” (BRIEF-A) questionnaire was used to assess self- and informant-reported executive functioning and compared to the general population. Results The CHD group did not show worse executive functioning compared to normative data. In addition, no significant difference was found between simple CHD (ASD, VSD and PS) and moderate/complex CHD (ToF and TGA). Higher education and NYHA class 1 were associated with better self-reported executive functioning, while male patients and patients taking psychiatric or cardiac medications reported worse executive functioning. Conclusions Overall, our findings suggest reassuring outcomes regarding executive functioning in adults with CHD. However, these findings should be confirmed with neuropsychological assessment studies. Funding Acknowledgement Type of funding sources: Foundation. Main funding source(s): Thorax Foundation

Treatment of Delayed Cerebral Ischemia in Good-Grade Subarachnoid Hemorrhage: Any Role for Invasive Neuromonitoring?

BackgroundGood-grade aneurysmal subarachnoid hemorrhage (Hunt and Hess 1–2) is generally associated with a favorable prognosis. Nonetheless, patients may still experience secondary deterioration due to delayed cerebral ischemia (DCI), contributing to poor outcome. In those patients, neurological assessment is challenging and invasive neuromonitoring (INM) may help guide DCI treatment.MethodsAn observational analysis of 135 good-grade SAH patients referred to a single tertiary care center between 2010 and 2018 was performed. In total, 54 good-grade SAH patients with secondary deterioration evading further neurological assessment, were prospectively enrolled for this analysis. The cohort was separated into two groups: before and after introduction of INM in 2014 (pre-INMSecD: n = 28; post-INMSecD: n = 26). INM included either parenchymal oxygen saturation measurement (ptiO2), cerebral microdialysis or both. Episodes of DCI (ptiO2 < 10 mmHg or lactate/pyruvate > 40) were treated via induced hypertension or in refractory cases by endovascular means. The primary outcome was defined as the extended Glasgow outcome scale after 12 months. In addition, we recorded the amount of imaging studies performed and the occurrence of silent and overall DCI-related infarction.ResultsSecondary deterioration, impeding neurological assessment, occurred in 54 (40.0%) of all good-grade SAH patients. In those patients, a comparable rate of favorable outcome at 12 months was observed before and after the introduction of INM (pre-INMSecD 14 (50.0%) vs. post-INMSecD 16, (61.6%); p = 0.253). A significant increase in good recovery (pre-INMSecD 6 (50.0%) vs. post-INMSecD 14, (61.6%); p = 0.014) was observed alongside a reduction in the incidence of silent infarctions (pre-INMSecD 8 (28.6%) vs. post-INMSecD 2 (7.7%); p = 0.048) and of overall DCI-related infarction (pre-INMSecD 12 (42.8%) vs. post-INMSecD 4 (23.1%); p = 0.027). The number of CT investigations performed during the DCI time frame decreased from 9.8 ± 5.2 scans in the pre-INMSecD group to 6.1 ± 4.0 (p = 0.003) in the post-INMSecD group.ConclusionsA considerable number of patients with good-grade SAH experiences secondary deterioration rendering them neurologically not assessable. In our cohort, the introduction of INM to guide DCI treatment in patients with secondary deterioration increased the rate of good recovery after 12 months. Additionally, a significant reduction of CT scans and infarction load was recorded, which may have an underestimated impact on quality of life and more subtle neuropsychological deficits common after SAH.

Delay in diagnosing patients with right-sided glioblastoma induced by hemispheric-specific clinical presentation.

Cognitive functions are differentially represented in brain hemispheres. Aphasia is an "easy to recognize" symptom of diseases affecting the left side. In contrast, lesions in the right hemisphere cause subtle neuropsychological deficits such as neglect and anosognosia. We evaluated whether right-sided malignant brain tumors are on average larger at the time of first diagnosis as compared to left-sided tumors, and extrapolated the delay in diagnosing right-sided tumors compared to the left side. All first-ever diagnosed glioblastoma (GBM) patients between 2005 and 2012 were identified using our hospital-based prospective research registry. Baseline data, information on initial clinical presentation and imaging findings (including tumor volume) were collected. Extrapolation of time since tumor initiation was based on an established gompertzian growth model. We included 173 patients. Mean age of the study population was 58 ± 13years. Tumors located in the right hemisphere (n = 96) were larger as compared to tumors located in the left hemisphere (n = 77) (median 36.4mL [interquartile range 13.0-56.0; minimum 0.2, maximum 140.0] vs. 17.2mL [7.7-45.1mL; 0.4, 105.2]; p = 0.011). Right-sided tumors grew longer than left-sided tumors (378 ± 95days vs. 341 ± 74days; p = 0.006). Initial neuropsychological symptoms differed depending on the affected hemisphere. Right-hemispheric symptoms appear to be less clinically conspicuous resulting in a delayed diagnosis of GBM, which might be improved by raising awareness for the corresponding neuropsychological deficits. Whether our findings have prognostic implications needs to be evaluated in future studies.

SUBJECTIVE COGNITIVE DECLINE VERSUS SUBTLE DEFICITS AS INDICATORS OF PRECLINICAL AD: RESULTS FROM THE MULTICENTER OBSERVATIONAL STUDY ON PREDEMENTIA ALZHEIMER’S DISEASE (DELCODE) STUDY

In preclinical AD, subtle neuropsychological deficits occur in groups without mild cognitive impairment (MCI), although the detection of a subtle decline in individual cases may be difficult. Subjective cognitive decline (SCD) has been emphasized as being one of the first symptomatic manifestations of preclinical AD, possibly even preceding subtle deficits because compensation of slight impairments is still possible. Subjective and subtle cognitive decline are both mentioned in the NIA-AA-criteria-2018 as indicators for the Alzheimer's continuum in cognitively normals, but their overlap and their possibly complementary contribution in this regard is unclear. By applying an operational definition for subtle cognitive decline in SCD patients, we aim to clarify the degree of overlap of these definitions and their association with CSF-AD biomarkers. We analyzed 240 memory-clinic patients with a worrisome subjective cognitive decline (SCD) and 209 controls (CON) without SCD. Both groups performed within the normal range on a comprehensive test battery (>-1.5SD). Presence of subtle deficits was defined by mild neuropsychological or functional impairments (Table1). Prevalence of subtle impairment was calculated in SCD and CON to gauge the extent of overlap. With logistic regression, we analyzed whether subjective decline (SCD vs. CON) would predict CSF-AD markers, controlling for age. We repeated this analysis after excluding all subjects with subtle deficits. Most CON and SCD participants had no subtle cognitive impairments and groups didn't differ significantly in this regard (Table1). Subjective decline was associated with amyloid load (SCD>CON; p<.05) in cases with and without subtle impairment. Subtle impairment was associated with amyloid in CON and SCD (Figure1). SCD were 2.979 more likely Aβ42-positive compared to CON, even if excluding all subjects with subtle decline from analysis (Exp(B)=2.762).

A Retrospective Cross-Sectional and Longitudinal Study of the Effects of Age on CNS Vital Signs Scores in High-School Athletes.

Current recommendations for concussion management acknowledge the importance of objective assessments of neuropsychological (NP) ability, and computerized NP assessments have been widely integrated into the concussion management protocols of high schools. The optimal intervals for baseline test administration in high-school athletes are currently uncertain. The ability to accurately detect subtle NP deficits is particularly important for high-school athletes, in which concussions are increasingly recognized for adverse effects to the developing brain. The aim of this study was to assess the pattern of change in neurocognitive test performance, as well as changes in different domains of NP functioning over time. Baseline computerized NP assessments were conducted at six high schools over 4academic years using CNS Vital Signs, a battery consisting of seven well-established NP tests. Data were retrospectively examined for age differences in both cross-sectional (n=3015) and longitudinal (n=1221) analyses. Moderate changes were observed across several NP domains over time (Cohen's d=0.39-0.61), with the largest improvements observed in executive functioning (mean improvement 5.78, 95% confidence interval [CI] 5.41-6.14, p<0.001), psychomotor speed (mean improvement 4.59, 95% CI 3.97-5.22, p<0.001), cognitive flexibility (mean improvement 5.11, 95% CI 4.76-5.45, p<0.001), and reaction time (mean improvement -12.44ms, 95% CI -10.10 to -14.78, p<0.001). Improvements in NP performance were most pronounced between the freshman and senior years. There is an appreciable change that occurs each year of high school in one or more domains of an NP battery, with executive functioning indicating the greatest magnitude of change. Females performed better relative to males across all time pointsthough males exhibited more substantial improvement over time.

Chemotherapy and Cognitive Function in Breast Cancer Patients: The So-Called Chemo Brain.

Self-perceived problems of cognitive functioning after treatment for early-stage breast cancer have the potential to substantially affect the lives of patients. In the past two decades, neuropsychological studies have accumulated evidence of corresponding cognitive deficits that have mostly been attributed to neurotoxic effects of chemotherapy. Nevertheless, observations of impaired cognitive functioning already before the start of adjuvant or neoadjuvant chemotherapy question the singular role of chemotherapy for the causation of these deficits. The divergence between mostly subtle neuropsychological deficits and often dramatic subjective cognitive complaints as well as the lack of association between both in the majority of studies present an unsolved puzzle. Recent investigations that include brain imaging have begun to yield tentative answers in this regard. The present review aims at briefly summarizing and integrating the current evidence from clinical studies for purposes of patient counseling.

Elderly with autism: executive functions and memory.

Cognitive autism research is mainly focusing on children and young adults even though we know that autism is a life-long disorder and that healthy aging already has a strong impact on cognitive functioning. We compared the neuropsychological profile of 23 individuals with autism and 23 healthy controls (age range 51–83 years). Deficits were observed in attention, working memory, and fluency. Aging had a smaller impact on fluency in the high functioning autism (HFA) group than in the control group, while aging had a more profound effect on visual memory performance in the HFA group. Hence, we provide novel evidence that elderly with HFA have subtle neuropsychological deficits and that the developmental trajectories differ between elderly with and without HFA in particular cognitive domains.

Study design considerations: conducting global clinical trials in early Alzheimer's disease.

An increasing number of Alzheimer's disease (AD) clinical trials are being conducted in countries in which such trials have infrequently, if ever, been conducted. The infrastructure for conducting trials in many of these regions is not well developed, leading to particular challenges in collection of biomarkers, which are becoming increasingly important in trials in early AD. Linguistic and cultural differences make scale translation, adaptation, validation and implementation across countries and regions difficult. In addition, multiple translations and versions of scales and differences in their administration increase variability and thus decrease the chance of detecting a signal. These issues are magnified in trials in early AD, where detecting subtle neuropsychological deficits is even more challenging. Two additional significant factors for global AD research include: 1) Differing regulatory authority requirements resulting in the need for repeat studies to satisfy diverse regulatory requirements in different parts of the world; and 2) reimbursement and access may be limited due to different data requirements for country specific economic evaluations. While standardization of biochemical assays and neuroimaging protocols have recently been undertaken, there remains a pressing need for standardization of clinical measures (including translation, linguistic and cultural validation and administration). In addition, a global consensus on regulatory requirements for approval of drugs for the treatment of early AD and identification of universally accepted variables from a cost-effectiveness or value perspective would have significant impact on advancing drug development in early AD.

Corpus callosum agenesis and rehabilitative treatment

Corpus callosum agenesis is a relatively common brain malformation. It can be isolated or included in a complex alteration of brain (or sometimes even whole body) morphology. It has been associated with a number of neuropsychiatric disorders, from subtle neuropsychological deficits to Pervasive Developmental Disorders.Etiology and pathogenetic mechanisms have been better understood in recent years, due to the availability of more adequate animal models and the relevant progresses in developmental neurosciences. These recent findings are reviewed (through a MedLine search including papers published in the last 5 years and most relevant previously published papers) in view of the potential impact on children's global functioning and on the possible rehabilitative treatment, with an emphasis on the possibility to exploit brain plasticity and on the use of the ICF-CY framework.

Short term changes in plasma phenylalanine status and cognitive processing speed in adolescent and adult females with phenylketonuria (PKU)

Even with early and continuous diet therapy, subtle neuropsychological deficits exist in phenylketonuria (PKU). To clarify the relationship between compliance and processing speed, this study examines the relationship between short‐term changes in plasma phenylalanine (Phe) concentration and cognitive processing speed. Data were collected from twenty females with PKU (ages 12‐47 years) attending a summer camp intervention designed in part to reduce plasma Phe. Pre‐ and post‐intervention fasting plasma Phe was assessed using ion‐exchange chromatography. Pre‐ and post‐intervention processing speed was assessed in group format by Woodcock‐Johnson III timed tests of decision speed and pair cancellation. As expected, mean plasma Phe and processing speed scores significantly improved (p&lt;0.001) and pair cancellation negatively correlated with change in plasma Phe controlling for verbal ability (r=‐0.627, p=0.004). Changes in decision speed and plasma Phe, however, were unrelated. Pair cancellation may be more sensitive to improved Phe control due to the task's reliance on attention and concentration. Short‐term improved compliance may be associated with concurrent improved processing speed. Supported in part by PHS Grant M01 RR00039 from the General Clinical Research Center program, National Institutes of Health, National Center for Research Resources.

Executive and Attention Functioning Among Children in the PANDAS Subgroup

Evidence from past studies indicates that adults and children with Obsessive-Compulsive Disorder (OCD) and Tourette syndrome (TS) experience subtle neuropsychological deficits. Less is known about neuropsychological functioning of children and adolescents with a symptom course consistent with the PANDAS (Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal infection) subgroup of OCD and tics. To provide such information, we administered three tests of attention control and two of executive function to 67 children and adolescents (ages 5–16) diagnosed with OCD and/or tics and a symptom course consistent with the PANDAS subgroup and 98 healthy volunteers (HV) matched by age, sex, and IQ. In a paired comparison of the two groups, the PANDAS subjects were less accurate than HV in a test of response suppression. Further, in a two-step linear regression analysis of the PANDAS group in which clinical variables were added stepwise into the model and in the second step matching variables (age, sex, and IQ) were added, IQ emerged as a predictor of performance on this task. In the same analysis, ADHD diagnosis and age emerged as predictors of response time in a continuous performance task. Subdividing the PANDAS group by primary psychiatric diagnosis revealed that subjects with TS or OCD with tics exhibited a longer response time compared to controls than subjects with OCD only, replicating previous findings within TS and OCD. This study demonstrates that children with PANDAS exhibit neuropsychological profiles similar to those of their primary psychiatric diagnosis.

Integrity of cerebral white matter in type 1 diabetes

To the Editor: In a recent issue of Diabetologia, Weinger et al. [1] report evidence of only mild white matter hyperintensities (WMHs) in a sample of adults with type 1 diabetes. These WMHs were comparable to those seen in a demographically similar sample of non-diabetic adults, and no association between diabetes characteristics and WMHs was found. As stated by the authors and from what is known from the literature, there is no clear understanding of the clinical relevance of WMHs. However there is evidence to suggest that whole grey and white matter volume, as compared with lesion burden, is more closely related to neuropsychological performance and neuropsychiatric symptoms [2, 3]. Our investigations of total white and grey matter volume in patients with type 1 diabetes revealed a relationship between cerebral white matter volume and cognitive performance [4]. Patients with diabetes demonstrated several subtle neuropsychological deficits, and patients with a microvascular complication (defined as proliferative retinopathy) showed a decreased white matter volume that was associated with worse cognitive performance. This suggests that cognitive decline is at least partly mediated by microvascular disease. Despite the fact that these studies differ methodologically and focus on different aspects of the brain (i.e. WMHs vs white matter volume), both aimed to elucidate the underlying mechanism of cognitive decline in type 1 diabetes. It is therefore unfortunate that Weinger et al. did not include this specific consideration in their paper. Whether WMHs are associated with white matter volume is still under debate. The results of a study by Wen et al. [5] suggest that WMHs are associated with loss of brain volume, which could presumably be the result of white matter pathology [6]. In contrast, Smith at al. [7] reported that decreased white matter volume measured from magnetic resonance imaging was associated with dementia and with lower cognitive test scores, whereas no such association was found with periventricular WMHs identified on the same images. WMHs were not related to white matter volume, stroke or dementia. Nonetheless, low white matter volume was strongly related to dementia and poor cognitive performance in this sample. In the elderly, the periventricular WMHs have been shown to be unrelated to total white matter volume loss [8]. Additional data from diffusion-weighted and magnetisation transfer (MT) imaging studies [9, 10] reveal that fractional anisotropy and the magnetisation ratio (a measure of the integrity of the microstructure) in white matter significantly decrease with age. These decreases suggest that white matter volume loss may result from these microscopic structural changes, which include myelin and axonal loss, dilatation of perivascular spaces and gliosis. To understand the mechanisms by which diabetes might affect the brain, it is important to consider the apparent Diabetologia (2008) 51:1554–1555 DOI 10.1007/s00125-008-1011-2

Response Inhibition in Borderline Personality Disorder: Performance in a Go/Nogo Task

Background: Borderline personality disorder is associated with subtle neuropsychological deficits. A potential impairment of response inhibition is of major interest, since it could be related to impulsivity as a clinical feature of borderline personality disorder. Sampling and Methods: Response inhibition was studied in an auditory Go/Nogo paradigm in a sample of 20 female inpatients with borderline personality disorder and 18 healthy controls. The main measures of interest were general task performance, errors and reaction times. Results: Patients with borderline personality disorder performed worse in the Nogo task but not in the Go task. In the Nogo task, when response inhibition was essential, patients made more errors of commission, revealing problems to inhibit a prepotent response. Additionally, the borderline group was characterized by significantly shorter reaction times in both tasks compared to the nonclinical control group. The results for errors of commission in the Nogo task remained significant even after controlling for reaction time. Conclusions: The present results suggest a double impairment on this response inhibition task. First, borderline personality disorder patients have inadequately fast reaction times and a speed-accuracy tradeoff. Second, they show a genuine deficit of response inhibition. These results are discussed in the context of the conflicting literature on response inhibition and executive control in borderline personality disorder.

Neuropsychological functioning in adolescent marijuana users: Subtle deficits detectable after a month of abstinence

In adults, studies examining the long-lasting cognitive effects of marijuana use demonstrate subtle deficits in attention, executive function, and memory. Because neuromaturation continues through adolescence, these results cannot necessarily generalize to adolescent marijuana users. The goal of this study was to examine neuropsychological functioning in abstinent marijuana using and demographically similar control adolescents. Data were collected from 65 adolescent marijuana users (n=31, 26% females) and controls (n=34, 26% females) 16-18 years of age. Extensive exclusionary criteria included independent psychiatric, medical, and neurologic disorders. Neuropsychological assessments were conducted after>23 days of monitored abstinence. After controlling for lifetime alcohol use and depressive symptoms, adolescent marijuana users demonstrated slower psychomotor speed (p<.05), and poorer complex attention (p<.04), story memory (p<.04), and planning and sequencing ability (p<.001) compared with controls. Post hoc analysis revealed that the number of lifetime marijuana use episodes was associated with poorer cognitive function, even after controlling for lifetime alcohol use. The general pattern of results suggested that, even after a month of monitored abstinence, adolescent marijuana users demonstrate subtle neuropsychological deficits compared with nonusers. It is possible that frequent marijuana use during adolescence may negatively influence neuromaturation and cognitive development.

Cognitive performance in type 1 diabetes patients is associated with cerebral white matter volume

Cognitive performance in type 1 diabetes may be compromised as a result of chronic hyperglycaemia. The aim of this study was to investigate the cognitive functioning of patients with type 1 diabetes (including a subgroup with a microvascular complication) and nondiabetic controls, and to assess the relationship between cognition and cerebral grey and white matter volumes. Twenty-five patients with type 1 diabetes (of whom ten had proliferative retinopathy) and nine nondiabetic controls (matched in terms of sex, age and education) underwent a neuropsychological examination and magnetic resonance imaging of the brain. Fractional brain tissue volumes (tissue volume relative to total intracranial volume) were obtained from each participant. Compared with nondiabetic controls, patients with diabetes performed worse on tests measuring speed of information processing and visuoconstruction; patients with microvascular disease performed worse on the former cognitive domain (p = 0.03), whereas patients without complications performed worse on the latter domain (p = 0.01). Patients with a microvascular complication had a significantly smaller white matter volume than nondiabetic controls (p = 0.04), and smaller white matter volume was associated with worse performance on the domains of speed of information processing and attention and executive function. Patients with diabetes demonstrated several subtle neuropsychological deficits, which were found to be related to white matter volume. Since patients with diabetic retinopathy had a smaller white matter volume, this suggests that cognitive decline is at least partly mediated by microvascular disease. This needs to be addressed in future studies.

Neuropsychological deficits and functional impairment in bipolar depression, hypomania and euthymia

To examine whether patients with bipolar disorder (BD) have subtle neuropsychological deficits that manifest clinically as cognitive and functional compromise, and this study attempted to determine the pattern of such cognitive deficits and their functional impact across all three phases of BD. We hypothesised that euthymia does not equate with normal neuropsychological function and that each phase has a characteristic pattern of deficits, with disturbance in attention and memory being common across all phases of the illness: (i) bipolar depression - psychomotor slowing and impairment of memory; (ii) hypomania by frontal-executive deficits and (iii) euthymia - a mild disturbance of attention, memory and executive function. Twenty-five patients with a diagnosis of bipolar I disorder underwent neuropsychological testing over a period of 30 months in the natural course of their illness while hypomanic and/or depressed and/or euthymic. The results from these assessments were compared with findings from neuropsychological tests conducted on 25 healthy controls matched for age, sex, education and handedness. Initial analyses revealed modest impairment in executive functioning, memory and attention in both hypomanic and depressed bipolar patients, with additional fine motor skills impairment in the latter. Memory deficits, also noted in euthymic patients, were non-significant after controlling for confounding variables, although bipolar depressed patients remained significantly impaired on tests of verbal recall. Bipolar depressed and hypomanic patients differed with respect to the nature of their memory impairment. Depressed patients were more impaired as compared with euthymic patients on tests of verbal recall and fine motor skills. Psychosocial functioning was impaired across all three patient groups, but only in depressed and hypomanic patients did this correlate significantly with neuropsychological performance. The mood-state-related cognitive deficits in both bipolar depression and hypomania compromise psychosocial function when patients are unwell. In euthymic patients, subtle impairments in attention and memory suggest that an absence of symptoms does not necessarily equate to 'recovery'. The possibility of persistent cognitive deficits in BD is an issue of profound clinical and research interest that warrants further investigation; however, future research needs to adopt more sophisticated neuropsychological probes that are able to better define state and trait deficits and determine their functional impact.

Benign childhood epilepsy with occipital paroxysms: Neuropsychological findings

Benign childhood epilepsy with occipital paroxysms is classified among childhood benign partial epilepsies. The absence of neurological and neuropsychological deficits has long been considered as a prerequisite for a diagnosis of benign childhood partial epilepsy. Much evidence has been reported in literature in the latest years suggesting a neuropsychological impairment in this type of epilepsy, particularly in the type with Rolandic paroxysms. The present work examines the neuropsychological profiles of a sample of subjects affected by the early-onset benign childhood occipital seizures (EBOS) described by Panayotopulos. The patient group included 22 children (14 males and 8 females; mean age 10.1 ± 3.3 years) diagnosed as having EBOS. The patients were examined with a set of tests investigating neuropsychological functions: memory, attention, perceptive, motor, linguistic and academic (reading, writing, arithmetic) abilities. The same instruments have been given to a homogeneous control group as regards sex, age, level of education and socio-economic background. None of the subjects affected by EBOS showed intellectual deficit (mean IQ in Wechsler Full Scale 91.7; S.D. 8.9). Results show a widespread cognitive dysfunction in the context of a focal epileptogenic process in EBOS. In particular, children with EBOS show a significant occurrence of specific learning disabilities (SLD) and other subtle neuropsychological deficits. We found selective dysfunctions relating to perceptive-visual attentional ability ( p < 0.05), verbal and visual-spatial memory abilities ( p < 0.01), visual perception and visual-motor integration global abilities ( p < 0.01), manual dexterity tasks ( p < 0.05), some language tasks ( p < 0.05), reading and writing abilities ( p < 0.01) and arithmetic ability ( p < 0.01). The presence of cognitive dysfunctions in subjects with EBOS supports the hypothesis that epilepsy itself plays a role in the development of neuropsychological impairment. Supported by other studies that have documented subtle neuropsychological deficits in benign partial epilepsy, we stress the importance of reconsidering its supposed “cognitive benignity”, particularly in occipital types.

Speed of decision-making and set-switching: Subtle executive deficits in children with treated phenylketonuria

Phenylketonuria (PKU) is an inborn error of protein metabolism affecting about 1 in 10,000 children in which conversion of dietary phenylalanine into tyrosine is impaired. Unmetabolised phenylalanine accumulates in body tissue and is neurotoxic to the developing organism. The untreated child is almost invariably brain damaged and severely learning disabled. These gross neuropathic effects can be prevented by early implementation of a phenylalanine-restricted diet. Nonetheless, treated children may suffer subtle neuropsychological deficits in the form of mild executive dysfunction, possibly because of depletion of the neurotransmitter dopamine in prefrontal cortex. Dopamine is a downstream metabolite in the phenylalanine pathway but, because of the phenylalanine-tyrosine block, may be in short supply in treated PKU. We investigated the dopamine/executive hypothesis by comparing the performance of 22 early and continuously treated PKU children with controls, matched for non-executive ability, on the test of everyday attention for children. We found the PKU children did not show deranged selective or sustained attention, but were significantly slow on set-switching (creature counting), stop-go (walk/don’t walk), stroop-like (opposite worlds) and target-detection (sky search) tasks. These findings add weight to a growing body of evidence suggesting that rapid decision-making is subtly impaired in PKU, possibly due to prefrontal neurotransmitter insufficiency, and that speeded strategic and choice-reaction tests are particularly sensitive tools for investigating executive dysfunction. How these findings translate into daily domestic and scholastic functioning has yet to be established.

Personality dimensions and neuropsychological performance in first-degree relatives of patients with schizophrenia and affective psychosis

Several studies have found a significant increase in the prevalence of some personality disorders in the first-degree relatives of patients with schizophrenia; other studies have found subtle neuropsychological deficits in these relatives. However, little is known about the specificity of the personality traits reported or about the relationship between these traits and the neuropsychological deficits. One-hundred first-degree relatives of patients with schizophrenia (SR) and 88 first-degree relatives of affective psychotic patients (APR) completed the Eysenck Personality Questionnaire which measures extraversion, neuroticism, and psychoticism; they were also administered the National Adult Reading Test (NART), the Trail Making Test (TMT) and a Verbal Fluency Test (VFT). The male relatives of patients with schizophrenia scored significantly higher on the psychoticism scale than the male relatives of affective psychotic patients. In the SR group, there were significant correlations between the TMT performance and the extraversion scores and, between the IQ scores and the psychoticism scores. However, when logistical regression analyses were performed, none of the three personality scores predicted any of the neuropsychological performance in either the SR or the APR group. These results indicate some specificity as well as sex differences in the psychoticism dimension. Moreover, the relationship between the personality dimensions and the neuropsychological performance could indicate that psychoticism increases vulnerability to psychosis whereas extraversion decreases it.

Effects of diabetes on learning in children.

Effects of diabetes on learning in children.