The executive profile of children with Benign Epilepsy of Childhood with Centrotemporal Spikes and Temporal Lobe Epilepsy.

RATIONALE: Benign Epilepsy of Childhood with Centrotemporal Spikes (BECTS) and temporal lobe epilepsy (TLE) represent two distinct models of focal epilepsy of childhood. In both, there is evidence of executive dysfunction. The purpose of the present study was to identify particular deficits in the executive function that would distinguish children with BECTS from children with TLE. METHODS: We prospectively evaluated 19 consecutive children and adolescents with TLE with hippocampal sclerosis (HS) (57.9% male; mean 11.74years [SD 2.05]; mean IQ 95.21 [SD 15.09]), 19 with BECTS (36.8% male; mean 10.95years [SD 2.33]; mean IQ 107.40 [SD 16.01]), and 21 age and gender-matched controls (33.3% male; mean 11.86years [SD 2.25]; mean IQ 108.67 [15.05]). All participants underwent a neuropsychological assessment with a comprehensive battery for executive and attentional functions. We used ANOVA and chi-square to evaluate differences on demographic aspects among groups (BECTS, TLE-HS, and control groups). Group comparisons on continuous variables were complemented by MANOVA and Bonferroni posthoc comparisons. RESULTS: Patients with BECTS had worse performance than controls in: Matching Familiar Figures Test, time (p=0.001); Matching Familiar Figures Test, time×errors index (p<0.001); Verbal Fluency for foods (p=0.038); Trail Making Test, part B time (p=0.030); Trail Making Test, part B number of errors (p=0.030); and WCST, number of categories achieved (p=0.043). Patients with BECTS had worse performance than patients with TLE-HS on Matching Familiar Figures Test, time (p=0.004), and Matching Familiar Figures Test, time×errors index (p<0.001). Patients with TLE-HS had worse performance than controls on the following tests: Verbal Fluency for foods (p=0.004); Wisconsin Card Sorting Test, the number of categories achieved (p<0.001); and Wisconsin Card Sorting Test, the number of perseverative errors (p=0.028). Patients with TLE-HS had worse performance than patients with BECTS on Digit Backward (p=0.002); and the Wisconsin Card Sorting Test, the number of perseverative errors (p<0.001). CONCLUSIONS: Patients with TLE and BECTS present distinct cognitive profiles. Patients with TLE-HS had worse performance in mental flexibility, concept formation, and working memory compared to BECTS. Patients with BECTS had worse inhibitory control compared to children with TLE-HS. Both TLE-HS and BECTS had a higher number of errors on an inhibitory control test. However, patients with BECTS had a slower mental processing even when compared to patients with TLE-HS. Rehabilitation programs for children with epilepsy must include children with benign epilepsies and must take into account the epileptic syndrome and its particular neurocognitive phenotype.

Memory in children with temporal lobe epilepsy is at least partially explained by executive dysfunction.

An association between memory and executive dysfunction (ED) has been demonstrated in patients with mixed neurological disorders. We aimed to investigate the impact of ED in memory tasks of children with temporal lobe epilepsy (TLE). We evaluated 36 children with TLE and 28 controls with tests for memory, learning, attention, mental flexibility, and mental tracking. Data analysis was composed of comparison between patients and controls in memory and executive function; correlation between memory and executive function tests; and comparison between patients with mild and severe ED in memory tests. Children with TLE had worse performance in focused attention, immediate and delayed recall, phonological memory, mental tracking, planning, and abstraction. Planning, abstraction, and mental tracking were correlated with visual and verbal memory. Children with severe ED had worse performance in verbal and visual memory and learning tests. This study showed that ED was related to memory performance in children with TLE.

Neuropsychological assessment of children with rolandic epilepsy: executive functions.

Rolandic epilepsy (RE) is the most common type of childhood focal epilepsy. Although there is no intellectual deficit, children with RE may have specific cognitive impairments. The aims of this study were to identify changes in executive functions in children with RE and to verify the influence of epilepsy and seizure variables. We evaluated 25 children with RE and 28 healthy controls. A comprehensive neuropsychological battery was utilized. The results showed that the RE children had worse performance than the control group in some categories of the Wisconsin Card Sorting Test, the Trail Making Test part B, and the Verbal Fluency Test (FAS). Children with earlier onset of epilepsy had worse performance when compared with children with later onset of epilepsy. We conclude that children with RE may show a deficit in executive function despite their normal IQ. The set of tests was more extensive than what was previously used in other studies. Our study suggests that early seizures can interfere with brain development. Regarding cognition, the term benign should be used cautiously in RE.

Executive dysfunction in children and adolescents with temporal lobe epilepsy: is the Wisconsin Card Sorting Test enough?

The Wisconsin Card Sorting Test (WCST) is the gold standard in the evaluation of executive dysfunction (ED) in patients with temporal lobe epilepsy (TLE). We evaluated 35 children with TLE and 25 healthy controls with the WCST and with a more comprehensive battery. Among the children with TLE, 77.14% showed impairment on the WCST. On other tests (Wechsler Intelligence Scale for Children-Digit Forward, Matching Familiar Figures Test, Trail Making Test, Word Fluency, Finger Windows, and Number-Letter Memory), impairment was demonstrated in 94.29%. The authors concluded that the WCST is a good paradigm to measure executive impairment in children with TLE; however, it may be not enough. Evaluation performed only with the WCST not only underestimated the number of patients with ED, but also missed relevant information regarding the type of ED.

A new candidate locus for bilateral perisylvian polymicrogyria mapped on chromosome Xq27.

Polymicrogyria (PMG) is characterized by an excessive number of small and prominent brain gyri, separated by shallow sulci. Bilateral perisylvian polymicrogyria (BPP) is the most common form of PMG. Clinical signs include pseudobulbar paresis, mental retardation, and epilepsy. Familial forms of BPP have been described and a candidate locus was previously mapped to chromosome Xq28, distal do marker DXS8103. The objective of this study was to perform linkage analysis in one family segregating BPP. A total of 15 individuals, including 8 affected patients with BPP were evaluated. Family members were examined by a neurologist and subjected to magnetic resonance imaging scans. Individuals were genotyped for 18 microsatellite markers, flanking a 42.3 cM interval on ch Xq27-q28. Two-point and multipoint linkage analysis was performed using the LINKAGE package and haplotype reconstruction was performed by GENEHUNTER software. Our results showed a wide spectrum of clinical manifestations in affected individuals with BPP, ranging from normal to mild neurological abnormalities. Two-point linkage analysis yield a Zmax = 2.06 at theta = 0.00 for markers DXS1205 and DXS1227. Multipoint lod-scores indicate a candidate interval of 13 cM between markers DSXS1205 and DXS8043, on ch Xq27.2-Xq27.3. These results point to a new locus for BPP in a more centromeric location than previously reported.

Interictal EEG in temporal lobe epilepsy in childhood.

The authors clarified the value of interictal discharges and verified which extratemporal regions may also show epileptiform activity in temporal lobe epilepsy (TLE) in childhood. Thirty consecutive patients aged 3 to 18 years (mean age = 12.16 years; 16 male) with TLE associated with hippocampal atrophy were studied. Each patient had 1 to 15 interictal EEG recordings (mean: 5.6; total = 192 EEGs). Video-EEG monitoring was performed in 20 patients. All patients had MRI. The findings were compared with a control group of 53 consecutive TLE adult outpatients with hippocampal atrophy. Each adult patient underwent 3 to 21 routine EEGs (mean: 10.67; total = 566). Interictal EEGs of children with TLE showed extratemporal epileptiform discharges more frequently than EEGs of adults with TLE. Frontal, parietal, and occipital discharges were more frequently seen in children (P < 0.05). These results suggest a close interaction between temporal and other cerebral regions in children with epilepsy and provide further evidence of the existence of neural networks.

Clinical and electroencephalographic features of patients with polymicrogyria.

Polymicrogyria is a malformation of cortical organization. The aim of this historic cohort study was to describe clinical and EEG features of patients with polymicrogyria. Patients underwent clinical and neurologic examination and a prolonged routine EEG to allow recording during sleep. Neuroimaging data were classified as: perisylvian polymicrogyria (subdivided into holosylvian, posterior parietal, and generalized), hemispheric polymicrogyria, and frontal polymicrogyria. Forty patients were studied: 16 with holosylvian polymicrogyria, 14 with posterior parietal polymicrogyria, 4 with generalized polymicrogyria, 3 with hemispheric polymicrogyria, and 3 with frontal polymicrogyria. Patients with polymicrogyria usually did not have epilepsy and their EEGs were mostly normal (55%); the severity of the clinical and EEG features correlated with the extent of the cortical lesion. In perisylvian polymicrogyria, epileptiform abnormalities predominated in fronto-temporal regions. Dour patients had focal electrical status (FES) in awakeness and electrical status epilepticus of sleep (ESES); these four patients had right hemispheric polymicrogyria and asymmetric bilateral perisylvian polymicrogyria, mostly on the right hemisphere. The authors conclude that the EEG is usually normal in patients with polymicrogyria, despite it being associated with FES and ESES in certain patients.

Temporal lobe epilepsy in childhood: comprehensive neuropsychological assessment.

The neuropsychological features of children with temporal lobe epilepsy are not yet well defined. The aim of this study was to identify the neuropsychological deficits in children with temporal lobe epilepsy. We evaluated 25 patients and compared them with 25 normal children. All children underwent a comprehensive neuropsychological assessment. We found a significant difference in favor of the control group in the following measures: IQ; forward digit; Trail Making Test for Children B; Wisconsin Card Sorting Test; block design; Boston naming test, verbal fluency; and Wide Range Assessment of Memory and Learning verbal learning, visual learning, verbal memory, visual memory, delayed recall of verbal learning, delayed recall of stories, and recognition of stories. Our findings show that children with temporal lobe epilepsy present with several neuropsychological deficits, despite normal IQ. These findings point to a dysfunction of cerebral areas other than temporal lobe, particularly the frontal lobes.

Frontal lobe dysfunction in children with temporal lobe epilepsy.

There is evidence that adults with temporal lobe epilepsy present executive impairments. However, there is limited information in children, especially when using a comprehensive neuropsychologic battery. We aimed to: 1) investigate the presence and severity of executive dysfunctions in children with temporal lobe epilepsy, and 2) determine the implications of clinical variables (including etiology) in the occurrence and severity of executive dysfunction, using eight paradigms. Thirty-one children with temporal lobe epilepsy were evaluated and compared with 21 age-matched controls. Patients with temporal lobe epilepsy had significantly worse performance than controls. Intragroup analysis indicated that patients with symptomatic epilepsy were more impaired than those with cryptogenic epilepsy. In the former group, patients with mesial lesions performed worse than those with lateral lesions. Regarding the severity of executive dysfunction, 83.87% manifested severe to moderate executive impairment. Early age of onset, longer duration of epilepsy, and use of polytherapy were correlated with worse executive dysfunction. These findings indicated the presence of frontal lobe dysfunction in children with temporal lobe epilepsy, with worse performance in those with mesial temporal lobe epilepsy, early onset, longer duration of disease, and use of polytherapy. Our study corroborates the hypothesis that temporal lobe epileptogenic activity affects the extratemporal regions that mediate attentional and executive functions.

The impact of intelligence on memory and executive functions of children with temporal lobe epilepsy: Methodological concerns with clinical relevance.

PURPOSE: Patients with TLE are prone to have lower IQ scores than healthy controls. Nevertheless, the impact of IQ differences is not usually considered in studies that compared the cognitive functioning of children with and without epilepsy. This study aimed to determine the effect of using IQ as a covariate on memory and attentional/executive functions of children with TLE. METHODS: Thirty-eight children and adolescents with TLE and 28 healthy controls paired as to age, gender, and sociodemographic factors were evaluated with a comprehensive neuropsychological battery for memory and executive functions. The authors conducted three analyses to verify the impact of IQ scores on the other cognitive domains. First, we compared performance on cognitive tests without controlling for IQ differences between groups. Second, we performed the same analyses, but we included IQ as a confounding factor. Finally, we evaluated the predictive value of IQ on cognitive functioning. RESULTS: Although patients had IQ score in the normal range, they showed lower IQ scores than controls (p = 0.001). When we did not consider IQ in the analyses, patients had worse performance in verbal and visual memory (short and long-term), semantic memory, sustained, divided and selective attention, mental flexibility and mental tracking for semantic information. By using IQ as a covariate, patients showed worse performance only in verbal memory (long-term), semantic memory, sustained and divided attention and in mental flexibility. IQ was a predictor factor of verbal and visual memory (immediate and delayed), working memory, mental flexibility and mental tracking for semantic information. CONCLUSION: Intelligence level had a significant impact on memory and executive functioning of children and adolescents with TLE without intellectual disability. This finding opens the discussion of whether IQ scores should be considered when interpreting the results of differences in cognitive performance of patients with epilepsy compared to healthy volunteers.

Interictal electroencephalographic findings in children and adults with temporal lobe tumors.

OBJECTIVE: To characterize clinical and interictal electroencephalographic aspects of children and adults with temporal lobe epilepsy (TLE) due to tumoral lesions. METHOD: We performed a retrospective analysis of the clinical and interictal electroencephalographic aspects of 16 children (64 exams) and 12 adults (78 exams) with lesions in the temporal lobe. RESULTS: The most frequent etiologies were gangliogliomas, DNETs, followed by astrocytomas. Auras occurred in both groups, the most common being epigastric sensation. Other findings such as myoclonias, behavioral arrest and vomiting were more frequent in children. Temporal epileptiform and nonepileptiform activities, mostly unilateral, were found in both groups. Extratemporal epileptiform activities (frontal, parietal, central, occipital and generalized) were also found equally in both groups. CONCLUSION: Our data show that children and adults with TLE due to expansive lesions present with similar EEG findings.

Specific language impairment: linguistic and neurobiological aspects.

Specific language impairment (SLI) occurs when children present language maturation, at least 12 months behind their chronological age in the absence of sensory or intellectual deficits, pervasive developmental disorders, evident cerebral damage, and adequate social and emotional conditions. The aim of this study was to classify a group of children according to the subtypes of SLI and to correlate clinical manifestations with cortical abnormalities. Seventeen children with SLI were evaluated. Language assessment was based on standardized test (Peabody) and a non-standardized protocol, which included phonological, syntactical, semantical, pragmatical and lexical aspects of language. All children, except one, had abnormal MRI. Thirteen children presented perisylvian polymicrogyria. The MRI findings in the remaining three patients were: right frontal polymicrogyria, bilateral fronto-parietal atrophy, and hypogenesis of corpus callosum with Chiari I. The data show that patients with posterior cortical involvement tended to present milder form of SLI (no sign of articulatory or bucofacial praxis disturbance), while diffuse polymicrogyric perisylvian cortex usually was seen in patients who presented severe clinical manifestation, mainly phonological-syntactic deficit. In conclusion, SLI may be associated with perisylvian polymicrogyria and clinical manifestation may vary according to the extent of cortical anomaly.

[Perisylvian syndrome: report of one Brazilian family with focus on the genetic mode of inheritance and clinical spectrum]. / Síndrome peri-sylviana: estudo de uma família brasileira com ênfase na modalidade de transmissão genética e espectro clínico.

Perisylvian syndrome (PS) refers to a variety of clinical manifestations associated with lesions in the perisylvian or opercular region. Acquired lesions such as cerebrovascular diseases or virus encephalitis and congenital lesions such as polymicrogyria (PMG) may be implied as etiological factors. The onset of the PS may occur in early childhood. The aim of this study was to report one family with PS in order to draw attention to this rarely diagnosed entity. Our family has five affected patients, three children and two male adults. All of them had developmental language disorder. Epilepsy, motor deficit and pseudobulbar signs (such as drooling) were detected in one child who had diffuse PMG along the Sylvian fissure. Subtle clinical manifestations correlated with either subtle MRI findings or normal MRI. Most reported families provide evidence suggestive of X-linked transmission. However, the most likely mode of inheritance in our family is autosomal dominant, since a male to male transmission was documented.

Clinical, electroencephalographic, and behavioral features of temporal lobe epilepsy in childhood.

This study describes the clinical, electroencephalographic, and behavioral features of 36 children with temporal lobe epilepsy. Patients were divided into two groups: group A, with 6 patients (< 6 years), and group B, with 30 patients (6-18 years). Statistical analysis was performed considering the significance level of .05. Regarding the clinical features of the focal seizures, motor components were more frequently seen in children younger than 6 years of age (P < .01), whereas automatisms were more frequently seen in patients older than 6 years of age (P < .05). Associated myoclonic seizures were more frequent in the younger age group (P < .01). Behavioral disorders such as hyperactivity and aggressiveness and speech delay were more common in the younger age group (P < .05). Temporal lobe epilepsy in children younger than 6 years of age is more frequently associated with motor components, myoclonic seizures, behavioral disorders, and speech delay. Conversely, temporal lobe epilepsy in older patients has frequent automatisms.

Rolandic epilepsy and dyslexia.

OBJECTIVE: Although benign epilepsy with centrotemporal spikes (BECTS) is an idiopathic, age-related epilepsy syndrome with favorable outcome, recent studies have shown impairment in specific neuropsychological tests. The objective of this study was to analyze the comorbidity between dyslexia and BECTS. METHOD: Thirty-one patients with clinical and electroencephalographic diagnosis of BECTS (group A) and 31 paired children (group B) underwent a language and neuropsychological assessment performed with several standardized protocols. Our findings were categorized as: a) dyslexia; b) other difficulties; c) without difficulties. Our results were compared and statistically analyzed. RESULTS: Our data showed that dyslexia occurred in 19.4% and other difficulties in 74.2% of our patients. This was highly significant when compared with the control group (p<0.001). Phonological awareness, writing, reading, arithmetic, and memory tests showed a statistically significant difference when comparing both groups. CONCLUSION: Our findings show significant evidence of the occurrence of dyslexia in patients with BECTS.

ADHD in idiopathic epilepsy.

Our aim was to clarify the correlation of attention deficit hyperactivity disorder (ADHD) with epilepsy and behavior problems. This was a cross-sectional study. Sixty children with idiopathic epilepsy were interviewed using the MTA-SNAP IV Teacher and Parent Rating Scale, Vineland Adaptive Behavior Scales and Conners' Rating Scales. We used the chi-square test to analyze the correlation of epilepsy variables in patients with and without ADHD with a significance level of 0.05. Eight patients had ADHD symptoms (13%), seven had the inattentive ADHD subtype and only three had behavioral problems. When epileptic patients with and without ADHD symptoms were compared we found no significant difference in regard to epilepsy variables. All patients were controlled and 43% were either without AED or undergoing withdrawal. Our study revealed a low comorbidity of ADHD symptoms and epilepsy due to low interference of seizures and drug treatment on the comorbid condition.

Memory in children with symptomatic temporal lobe epilepsy.

In children with temporal lobe epilepsy (TLE), memory deficit is not so well understood as it is in adults. The aim of this study was to identify and describe memory deficits in children with symptomatic TLE, and to verify the influence of epilepsy variables on memory. We evaluated 25 children with TLE diagnosed on clinical, EEG and MRI findings. Twenty-five normal children were compared with the patients. All children underwent a neuropsychological assessment to estimate intellectual level, attention, visual perception, handedness, and memory processes (verbal and visual: short-term memory, learning, and delayed recall). The results allowed us to conclude: besides memory deficits, other neuropsychological disturbances may be found in children with TLE such as attention, even in the absence of overall cognitive deficit; the earlier onset of epilepsy, the worse verbal stimuli storage; mesial lesions correlate with impairment in memory storage stage while neocortical temporal lesions correlate with retrieval deficits.

Voxel-based morphometry and intellectual assessment in patients with congenital bilateral perisylvian syndrome.

Congenital bilateral perisylvian syndrome (CBPS) presents with heterogeneous clinical manifestations such as pseudobulbar palsy, language disorder, variable cognitive deficits, epilepsy, and perisylvian abnormalities (most frequently polymicrogyria) on imaging studies. We investigated the relationship between seizures and extent of gray matter (GM) and white matter (WM) abnormalities using voxel-based morphometry (VBM) of brain magnetic resonance imaging (MRI) as well the association between seizures, structural abnormalities and cognitive ability. In this cross-sectional study, we evaluated 51 healthy volunteers and 18 patients with CBPS with epilepsy (seizure group, n = 7) and without (non-seizure group, n = 11). We used VBM (SPM8/DARTEL) to investigate areas with excess and atrophy of both gray and white matter, comparing groups of patients with controls. Intellectual ability of patients was assessed by the WISC-III or WAIS-III. Both groups with CBPS and the control group were homogeneous with respect to gender (p = 0.07) and age (p = 0.065). Besides perisylvian polymicrogyria, the seizure group exhibited areas with GM and WM reduction including temporal, frontal, parietal and occipital lobes. In contrast, we identified fewer areas with GM and WM reduction in the non-seizure group. The seizure group presented worse intellectual performance (performance IQ and global IQ) than the non-seizure group. The seizure group presented with a more widespread pattern of cortical and sub-cortical abnormalities, as well as worse cognition. Our results suggest that patients with CBPS and epilepsy appear to have widespread neuronal damage that goes beyond the areas with MRI-visible perisylvian polymicrogyria.

Clinical and neuropsychological correlation in patients with rolandic epilepsy.

OBJECTIVES: To evaluate the presence of neurological soft signs (NSS) and to correlate them with the Wechsler Intelligence Scale for Children (WISC III) in patients with rolandic epilepsy (RE). METHODS: Forty children and adolescents aged between 9 and 15 years were studied. They were divided into two groups: G1 - patients with RE (n=20) - and G2 - healthy controls without epilepsy (n=20). They were assessed with the Quick Neurological Screening Test (QNST II) - clinical trial to search for NSS -, and the WISC III - neuropsychological test. RESULTS: No statistical difference between groups was found in WISC III and QNST II. However, children with poorer motor skills had worse performance in the QNST II and also in the execution intelligence quotient - IQ (p=0.001) and in total IQ (p=0.004), thus showing a positive correlation between them. CONCLUSIONS: The QNST II is a good screening tool for the neurologist to detect abnormalities in fine motor skills.

Quality of life and epilepsy surgery in childhood and adolescence.

UNLABELLED: Epilepsy can affect the quality of life (QOL) of patients. The temporal lobe epilepsy (TLE) is often refractory to medication, which has an adverse impact on QOL. The surgery can be a form to control the seizures and to improve the QOL of the patients. OBJECTIVE: The aim of this study was to verify the QOL of children and adolescents with TLE who underwent surgery for epilepsy, comparing QOL before and after surgery and investigating which parameters showed improvement. METHOD: We used semi-structured questionnaire in the pre-and post-surgery in 13 patients. The data were analyzed using the Wilcoxon test. RESULTS: The analysis showed that there was general improvement in the QOL postoperatively. There was improvement in general health issues, adverse effects of antiepileptic drugs and the relationship with parents. CONCLUSION: When properly indicated, epilepsy surgery improves quality of life of patients with TLE.