Attention and executive functions profile in childhood absence epilepsy

Childhood absence epilepsy (CAE) has been associated with executive functions and attention deficits. To clarify the issue of neurocognitive impairments in CAE, we investigated whether specific executive functions and attention deficit patterns were present in a well-defined group of children with CAE who were taking valproic acid. Participants included 15 children with CAE and 15 healthy controls aged 8–15 years and matched for sex, age and IQ. We compared the performances of the two groups in the following neuropsychological domains: planning and problem solving (TOL), verbal fluency (FAS and CAT), verbal short-term memory (DSF), verbal working memory (DSB), visuospatial memory (Corsi Block Tapping Test) and sustained and divided attention (TMT-A and TMT-B). No differences were found between the two groups on measures of intellectual functioning, verbal short-term memory and visuospatial memory. By contrast, significant differences were found in total time of planning task, phonological and category fluency and sustained and divided attention. Future studies that systematically examine different aspects of attention and executive functions are needed to outline a clear and specific neuropsychological profile in CAE.     

Executive functions in children with frontal and temporal lobe epilepsy.

Even though frontal lobe epilepsy (FLE) is a relatively common seizure type, no formal psychometric studies of children with FLE have been reported. We compared 12 children with FLE and 15 children with temporal lobe epilepsy (TLE) on neuropsychological tests of attention, memory, executive functioning, and adaptive functioning. The results of these tests indicated that the children with FLE had deficits in planning and executive functions, whereas their verbal and nonverbal memory was intact. The opposite pattern was observed in children with TLE. Measures of executive functioning and impulse control were the best predictors of adaptive functioning. The findings suggest that children with FLE have a pattern of cognitive deficits that differs markedly from the pattern seen in children with TLE. Children with FLE have prominent deficits in executive functioning that appear to be related to poor behavioral adaptation.     

Neuropsychological assessment in children with absence epilepsy

OBJECTIVE: To define cognitive deficits in children with absence epilepsy. BACKGROUND: Cognitive deficits have often been reported in children with epilepsy, but have rarely been characterized in patients with a specific epileptic syndrome. METHODS: Detailed neuropsychological testing was carried out on 16 right-handed children with absence epilepsy with similar clinical and EEG findings, and the findings were compared to 16 well-matched right-handed children without absence epilepsy. RESULTS: The authors found lower scores of measures of general cognitive functioning and visuospatial skills in patients with absence epilepsy, as compared to controls. Memory disturbances were also detected in absence epilepsy patients, with selective involvement of nonverbal memory and delayed recall. In contrast, verbal memory and language skills were relatively preserved. Patients whose seizures began at an earlier age seemed to have more severe cognitive deficits. CONCLUSION: Language skills tend to be relatively well preserved in children with generalized epilepsy, with more dysfunction seen in global terms rather than specific lateralizing deficits. Patients with absence epilepsy seem to show a similar neurocognitive profile that may be a reflection of the underlying epilepsy syndrome.     

Neurocognitive functions in euthymic bipolar patients

Objective: Meta‐analytic findings support the hypothesis of specific neurocognitive deficits for bipolar patients in the domains of attention, processing speed, memory and executive functions. This study aims to show neurocognitive impairment in euthymic patients with bipolar I disorder compared with healthy controls while detailing the impact of medication side‐effects or illness characteristics on neuropsychological test performance. Method: Forty euthymic patients with bipolar I disorder were compared with 40 healthy controls in a cross‐sectional design. Clinical features and neuropsychological measures of IQ, psychomotor speed, verbal fluency, learning and memory, executive functions and attention were assessed. Results: Patients without antipsychotic drug use did not differ significantly from healthy controls in any neuropsychological measure. Yet patients treated with antipsychotics showed significant underperformance in the domains of semantic fluency, verbal learning and recognition memory as well as executive functions related to planning abilities, even when clinical features were controlled for. Conclusion: The impact of antipsychotic medication needs to be further clarified for euthymic bipolar patients and should be considered when neuropsychological test performance is interpreted.     

Only subtle cognitive deficits in non–bulbar amyotrophic lateral sclerosis patients

Neuropsychological investigations of amyotrophic sclerosis (ALS) patients revealed considerable discrepancies regarding neurocognitive functions. Some, but not all studies have suggested executive dysfunctioning and memory impairment, and there is a wide range of applied neuropsychological tests and results. In this study, we investigated the neuropsychological performance of 15 non–bulbar ALS patients, 14 patients with neuromuscular symptoms, and 15 healthy controls. To avoid confounding effects of motor disability, performance was assessed using exclusively motor–free tests of frontal lobe functioning (specific memory functions, conditional–associative learning, attention, and executive functions). ALS patients exhibited poorer performance in two conditions (semantic and alternating condition, respectively) of the Verbal Fluency Test, suggesting a subtle executive deficit. No deficits were found in tests of memory, conditionalassociative learning, or attention. Assessed mood status was not related to neuropsychological performance.Verbal memory (CVLT) and verbal fluency (lexical condition) were positively associated with duration of disease. Our results support the view that there are only subtle cognitive deficits in ALS patients and we assume a possible effect of practice on cognitive tasks following reduced daily motor activity.     

Neuropsychological correlates of obstructive sleep apnea severity in patients with epilepsy

Aims. Obstructive sleep apnea affects up to 30% of patients with epilepsy. As obstructive sleep apnea represents a clinical risk factor for cognitive deficits, its occurrence in epilepsy patients may exacerbate cognitive deficits associated with this condition. However, the cognitive burden of obstructive sleep apnea in epilepsy remains poorly understood. We conducted a retrospective record review of adults with epilepsy who underwent a polysomnography and a neuropsychological assessment at Brigham and Women's Hospital. Methods. We examined the relationship between obstructive sleep apnea severity and cognitive functioning, particularly attention/executive functions, memory, and processing speed in untreated obstructive sleep apnea patients with epilepsy. Twenty patients with epilepsy and mild‐to‐severe obstructive sleep apnea were included in the analyses. Results. We found significant positive correlations between the oxygen saturation levels during rapid‐eye‐movement sleep and attention/executive tests (p<0.05), as well as time spent with saturation levels ≤90% and executive functioning (p=0.008). Similarly, worse verbal memory performances were associated with lower oxygen levels (p=0.003). In addition, more severe respiratory events during rapid‐eye‐movement sleep were associated with worse performances on attention tests (p=0.03). Conclusions. Our findings indicate that more severe obstructive sleep apnea‐related hypoxemia during sleep is associated with poorer cognitive performances on tests that assess attention/executive functions and verbal memory in patients with epilepsy. Overall, these results are consistent with the sleep apnea literature, and suggest that patients with epilepsy are also vulnerable to the effects of obstructive sleep apnea. Future prospective studies will help in determining whether treatment of obstructive sleep apnea may help improve cognitive functioning in patients with epilepsy.     

Neurocognitive function in unmedicated manic and medicated euthymic pediatric bipolar patients

OBJECTIVE: A systematic evaluation of neuropsychological functioning in individuals with pediatric bipolar disorder is necessary to clarify the types of cognitive deficits that are associated with acutely ill and euthymic phases of the disorder and the effects of medication on these deficits. METHOD: Unmedicated (N=28) and medicated (N=28) pediatric bipolar patients and healthy individuals (N=28) (mean age=11.74 years, SD=2.99) completed cognitive testing. Groups were matched on age, sex, race, parental socioeconomic status, general intelligence, and single-word reading ability. A computerized neurocognitive battery and standardized neuropsychological tests were administered to assess attention, executive function, working memory, verbal memory, visual memory, visuospatial perception, and motor skills. RESULTS: Subjects with pediatric bipolar disorder, regardless of medication and illness status, showed impairments in the domains of attention, executive functioning, working memory, and verbal learning compared to healthy individuals. Also, bipolar subjects with comorbid attention deficit hyperactivity disorder (ADHD) performed worse on tasks assessing attention and executive function than patients with bipolar disorder alone. CONCLUSIONS: The absence of differences in the deficits of neurocognitive profiles between acutely ill unmedicated patients and euthymic medicated patients suggests that these impairments are trait-like characteristics of pediatric bipolar disorder. The cognitive deficits found in individuals with pediatric bipolar disorder suggest significant involvement of frontal lobe systems supporting working memory and mesial temporal lobe systems supporting verbal memory, regardless of ADHD comorbidity.     

The Neuropsychological Profile of Infantile Duchenne Muscular Dystrophy

It has been shown that children with Duchenne muscular dystrophy (DMD) exhibit specific cognitive deficits. However, the neuropsychological profile has not yet been fully characterized. In order to control for the contribution of motor impairments as a confounding variable that is usually present when assessing children with muscular pathologies, we compared children with DMD to a group of children with an autoimmune pathology that does not entail either brain or cognitive dysfunction but does imply motor impairment: juvenile rheumatoid arthritis (JRA). An extensive neuropsychological evaluation was administered, including intelligence, perception, language, memory and learning, attention, executive function, praxis and reasoning. As the main result, in children with DMD we found significantly lower performances in verbal IQ, verbal short-term memory and phonological abilities, as well as in praxis and executive functioning domains. A wide range of cognitive domains, including verbal abilities, executive functioning, and praxis, must be taken into account when assessing neuropsychological functioning in children with DMD. In particular, subtle executive function impairment could be considered as a subclinical marker of cognitive impairment in developmental disorders.     

The neuropsychological profile of infantile Duchenne muscular dystrophy

It has been shown that children with Duchenne muscular dystrophy (DMD) exhibit specific cognitive deficits. However, the neuropsychological profile has not yet been fully characterized. In order to control for the contribution of motor impairments as a confounding variable that is usually present when assessing children with muscular pathologies, we compared children with DMD to a group of children with an autoimmune pathology that does not entail either brain or cognitive dysfunction but does imply motor impairment: juvenile rheumatoid arthritis (JRA). An extensive neuropsychological evaluation was administered, including intelligence, perception, language, memory and learning, attention, executive function, praxis and reasoning. As the main result, in children with DMD we found significantly lower performances in verbal IQ, verbal short-term memory and phonological abilities, as well as in praxis and executive functioning domains. A wide range of cognitive domains, including verbal abilities, executive functioning, and praxis, must be taken into account when assessing neuropsychological functioning in children with DMD. In particular, subtle executive function impairment could be considered as a subclinical marker of cognitive impairment in developmental disorders.     

Relationship of indicators of neuropathology, psychopathology, and effort to neuropsychological results in patients with epilepsy or psychogenic non-epileptic seizures

Previous research suggests epilepsy and psychogenic non-epileptic seizure (PNES) patients do not show consistent group differences on neuropsychological measures. However, both groups of patients show decreased neuropsychological performance when compared to a normal population (Cragar, Berry, Fakhoury, Cibula, & Schmitt, 2002). Swanson, Springer, Benbadis, and Morris (2000) have suggested epilepsy patients show decreased neurocognitive functioning due to neuropathology whereas PNES patients show decreased neurocognitive functioning due to psychopathology. Effort has also been implicated as an important factor in neuropsychological test results in general (Green, 2003). The purpose of this study was to investigate the relationships of medical history variables (e.g., duration of disorder), neuropathology, psychopathology, and effort to neuropsychological findings in epilepsy and PNES patients. Neuropsychological functioning was divided into six domains: intelligence, memory, language, executive functions, visual-spatial, and motor. Results indicated that medical history variables were related to intellectual, memory, language, and motor functioning. Psychopathology was associated with all cognitive domains except executive functioning. Effort was related to intellectual, memory, language, visual-spatial, and motor functioning. Neuropathology and diagnosis were related only to memory.     

Metabolic and neuropsychological phenotype in women heterozygous for ornithine transcarbamylase deficiency

We compared neurocognitive indices with clinical status, mutation analysis, and urea synthetic capacity in 19 women heterozygous for ornithine transcarbamylase deficiency. Although as a group, these women had average IQ scores, they displayed a specific neuropsychological phenotype with significant strengths in verbal intelligence, verbal learning, verbal memory, and reading, and significant weaknesses in fine motor dexterity/speed and nonsignificant weaknesses in nonverbal intelligence, visual memory, attention/executive skills, and math. This suggests selective vulnerability of white matter and better preservation of gray matter. When the group was divided into symptomatic and asymptomatic subgroups, based on either clinical history or residual urea synthetic capacity, the asymptomatic subgroup outperformed the symptomatic subgroup on all tested domains of neuropsychological functioning. Furthermore, the amount of residual urea synthetic capacity was predictive of several end point cognitive measures. There was no correlation between neonatal versus late-onset mutation or between normal or abnormal allopurinol challenge and neuropsychological outcome. In sum, we identified a specific metabolic and neurocognitive phenotype in women heterozygous for ornithine transcarbamylase deficiency. The findings support the importance of maintaining meticulous metabolic control in children with urea cycle disorders, because even mildly symptomatic subjects demonstrate cognitive deficits.     

Neuropsychological profile of patients with juvenile myoclonic epilepsy: a controlled study of 50 patients

The purpose of this study was to verify possible cognitive dysfunction in patients with juvenile myoclonic epilepsy (JME) and its relationship to factors related to epilepsy and schooling. Fifty subjects diagnosed with JME and 50 controls underwent neuropsychological assessment evaluating intellectual functions, attention, memory, executive functions, and language. The patients were further divided into two subgroups on the basis of educational level: < or = 11 and >11 years of formal education. Participants diagnosed with JME scored significantly below age-, education-, and gender-matched controls on neuropsychological measures of attention, immediate verbal memory, mental flexibility, control of inhibition, working memory, processing speed, verbal delayed memory, visual delayed memory, naming, and verbal fluency. A positive correlation was observed between duration of epilepsy and cognitive decline. However, in the group of patients with >11 years of education, this correlation was not significant. In this series of patients with JME, neuropsychological evaluation suggests widespread cognitive dysfunction outside the limits of the frontal lobes. The duration of epilepsy correlated with cognitive decline, and patients with higher education manifested less progression of deficits.     

Working memory impairment and its associated sleep-related respiratory parameters in children with obstructive sleep apnea

Study ObjectiveWorking memory deficits in children with obstructive sleep apnea (OSA) have been reported in previous studies, but the results were inconclusive. This study tried to address this issue by delineating working memory functions into executive processes and storage/maintenance components based on Baddeley's working memory model.MethodsWorking memory and basic attention tasks were administered on 23 OSA children aged 8–12 years and 22 age-, education-, and general cognitive functioning-matched controls. Data on overnight polysomnographic sleep study and working memory functions were compared between the two groups. Associations between respiratory-related parameters and cognitive performance were explored in the OSA group.ResultsCompared with controls, children with OSA had poorer performance on both tasks of basic storage and central executive components in the verbal domain of working memory, above and beyond basic attention and processing speed impairments; such differences were not significant in the visuo-spatial domain. Moreover, correlational analyses and hierarchical regression analyses further suggested that obstructive apnea–hypopnea index (OAHI) and oxygen saturation (SpO₂) nadir were associated with verbal working memory performance, highlighting the potential pathophysiological mechanisms of OSA-induced cognitive deficits.ConclusionsVerbal working memory impairments associated with OSA may compromise children's learning potentials and neurocognitive development. Early identification of OSA and assessment of the associated neurocognitive deficits are of paramount importance. Reversibility of cognitive deficits after treatment would be a critical outcome indicator.     

Neurocognitive dysfunction in first-episode psychosis: correlates with symptoms, premorbid adjustment, and duration of untreated psychosis

OBJECTIVE: The authors examined the relationship of neurocognitive function with duration of untreated psychosis, premorbid illness factors, and clinical symptoms to determine whether long duration of untreated psychosis independently compromises cognitive function. METHOD: Patients recruited to a study of the effect of an early detection program on the duration of untreated first-episode psychosis in two catchment areas were compared to patients in a similar treatment program in two other catchment areas without an early detection program. The median duration of untreated psychosis was 10.5 weeks for all patients. A total of 301 patients entered the study, and 207 completed a comprehensive neuropsychological test battery that assessed working memory/fluency, executive function, verbal learning, impulsivity, and motor speed. The median time from start of treatment to neuropsychological testing was 108 days; all patients were tested within 9 months. RESULTS: No significant association was found between duration of untreated psychosis and any of the cognitive measures. Strong associations were demonstrated between poorer premorbid school functioning and neurocognitive deficits, especially in verbal learning and working memory. No relationship was found between neurocognitive functions and clinical measures, except for an inverse correlation of Positive and Negative Syndrome Scale negative symptoms and working memory and a positive correlation between positive symptoms and motor speed. CONCLUSIONS: The data contribute to a disconfirmation of the hypothesis of an association between duration of untreated psychosis and neurocognitive performance at baseline.     

Neuropsychological performance in childhood OCD: A preliminary study

Background: Neuropsychological deficits have often been found in studies of adults with obsessive compulsive disorder (OCD). However, few studies have examined such impairment in children with OCD and of those studies published, the results are mixed. Methods: In the present study, 14 OCD children were compared to 24 healthy developing children of similar age and intellectual ability on a series of neuropsychological tests that assess response inhibition, abstract reasoning and problem solving, planning ability, verbal and nonverbal fluency, working memory, attention and information processing speed, and visual and verbal memory and learning. Results: No significant differences emerged between the children with OCD and healthy controls for working memory, verbal fluency, attention, information processing speed, concept formation/abstraction, and response inhibition. We observed some deficits and a trend toward performance differences between the groups for psychomotor speed and attention, cognitive flexibility, nonverbal fluency, planning ability, and verbal memory and learning. Results are partially consistent with those found in adults with OCD. Findings were not related to depressive symptoms or self‐report feeling of anxiety. Conclusions: This preliminary survey indicates that OCD children may have deficits for cognitive flexibility and planning ability and differ from adults with OCD in not presenting with poor response inhibition or memory deficits. Larger, multi‐site studies are warranted to help delineate the neurocognitive deficits associated with childhood OCD. Depression and Anxiety, 2010. © 2009 Wiley‐Liss, Inc.     

Broad Cognitive Profile in Children and Adolescents with HF-ASD and in Their Siblings: Widespread Underperformance and its Clinical and Adaptive Correlates

Despite evidence supporting the presence of cognitive deficits in children and adolescents with high-functioning autism spectrum disorder (HF-ASD), the nature of these deficits and their clinical and adaptive correlates remain unclear. Moreover, there are few cognitive studies of ASD siblings as a high risk population. We compared 50 children and adolescents with HF-ASD, 22 unaffected siblings of the HF-ASD sample and 34 community controls using an extensive neuropsychological battery. Planning, cognitive flexibility, verbal and working memory, visual local–global processing and emotion recognition are impaired in HF-ASD. Worse cognitive performance, especially in verbal and working memory, was significantly correlated with more severe symptoms and poorer adaptive functioning, also when controlling for intelligence quotient. Results in siblings may suggest an intermediate profile.     

Cognitive functioning over 3 years in community dwelling older adults with chronic partial epilepsy

Little is known about cognitive functioning of older adults with chronic partial epilepsy. We examined cognitive performance of this epilepsy patient group over 2-3 years. Seventeen older adults with epilepsy and 17 healthy older adults were administered measures of overall cognition and verbal memory at baseline and 2-3 years later. At baseline, older adults with epilepsy performed below controls on overall cognition and verbal memory (p's<0.001). These deficits generally remained stable at follow-up, although executive control appeared to decline (p<0.05). Older adults with epilepsy showed a failure to benefit from practice on a verbal memory measure (p=0.017). Older adults with epilepsy demonstrated cognitive deficits that generally are not progressive. A failure to benefit from repeat exposure to a Delayed Recall task could indicate learning deficits. These patients may also progressively lose executive control, possibly as a result of accelerated aging.     

Neuropsychological Profiles in Different At-Risk States of Psychosis: Executive Control Impairment in the Early—and Additional Memory Dysfunction in the Late—Prodromal State

Impairments in neuropsychological functioning have been described in subjects clinically at high risk for psychosis, but the specific cognitive deficits in different clinical high-risk groups remain to be elucidated. The German Research Network on Schizophrenia employs a heuristic 2-stage model: a putatively late prodromal state (LPS), characterized by the onset of attenuated positive or brief psychotic symptoms, and an early prodromal state (EPS), mainly characterized by the presence of basic symptoms, which are predictive for psychosis within the next 10 years.A total of 205 subjects met the criteria for either an EPS or an LPS of psychosis and were assessed with a comprehensive neuropsychological test battery. Neurocognitive profiles of high-risk groups were compared with data of 87 healthy controls comparable with regard to gender, age, and premorbid verbal IQ.Patients in the LPS were impaired in all neurocognitive domains (memory/learning, executive control/processing speed, and working memory) examined, with memory being the worst. Deficits were less pronounced in patients in the EPS, with a specific deficit in the executive control/processing speed domain. Consistent with a progressive neurodevelopmental disorder, some cognitive abilities were already impaired in patients in the EPS, followed by further deterioration in the LPS. Specifically, deficits in executive control functioning were related to the presence of basic symptoms, indicating a vulnerability for psychosis. Memory deficits were associated with the onset of psychotic symptoms indicating further disease progression in the trajectory to psychosis and, thus, may be useful in predicting psychosis and targeting early intervention.     

Cognitive function across manic or hypomanic, depressed, and euthymic states in bipolar disorder

OBJECTIVE: The study aims were to address neuropsychological functioning across different states of bipolar illness and to determine relationships among clinical features, neuropsychological performance, and psychosocial functioning. METHOD: Several domains of cognitive function were examined in 30 depressed bipolar patients (DSM-IV criteria for major depression, Hamilton Depression Rating Scale score > or = 17), 34 manic or hypomanic bipolar patients (DSM-IV criteria for manic or hypomanic episode, Young Mania Rating Scale score > or = 12), and 44 euthymic bipolar patients (6 months of remission, Hamilton depression scale score < or = 8, and Young Mania Rating Scale score < or = 6). The comparison group consisted of 30 healthy subjects without history of neurological or psychiatric disorders. A neuropsychological battery assessed executive function, attention, and verbal and visual memory. RESULTS: The three groups showed cognitive dysfunction in verbal memory and frontal executive tasks in relation to the comparison group. Low neuropsychological performance was associated with poor functional outcome. Impairment of verbal memory was related to the duration of illness and the numbers of previous manic episodes, hospitalizations, and suicide attempts. CONCLUSIONS: A poorer performance was observed in all bipolar groups regarding executive function and verbal memory in relation to the healthy comparison subjects. These cognitive difficulties, especially related to verbal memory, may help explain the impairment regarding daily functioning, even during remission. Further studies should focus on testing, whether optimizing prophylactic pharmacological treatment and psychoeducation might reduce cognitive impairment, and whether bipolar patients would benefit from neuropsychological rehabilitation in order to reduce the impact of cognitive impairment in their overall functioning.     

Neurocognition and suicidal behaviour in an Irish population with major psychotic disorders

OBJECTIVES: Although neurocognitive deficits are seen as core to schizophrenia the association between suicidality and neurocognition has received little attention. Our aim was to examine the relationship between neurocognitive variables and suicidal behaviour in patients with schizophrenia and schizoaffective disorder. METHODS: Seventy-eight patients with DSM-IV diagnoses of schizophrenia or schizoaffective disorder were categorised as either having attempted suicide or not having attempted suicide based on clinical interview and chart review. Attempters and non-attempters were compared on an extensive neuropsychological battery examining pre-morbid and current general cognitive functioning, episodic memory, and executive functioning. RESULTS: Suicide attempters tended to out perform non-attempters across all areas of executive functioning, and showed significantly better performances on measures of attention and verbal fluency. After controlling for relevant clinical and demographic variables, the differences between attempters and non-attempters remained significant for measures of attention (F = 4.97, p = 0.03) and verbal fluency (F = 4.28, p = 0.04). CONCLUSION: This study adds to existing data that suicide attempters with schizophrenia or schizoaffective disorder may have higher cognitive functioning than non-attempters. In particular, the preservation of higher executive function may influence the ability to initiate and plan suicidal behaviour.