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.     

Neuropsychological correlates of electroencephalograms in children with epilepsy

INTRODUCTION: This study examined the degree to which neurophysiological activity on routine clinical EEG is associated with neuropsychological deficiencies in children with epilepsy. METHODS: Ninety-five children with epilepsy (58 chronic, 37 recent-onset; mean age = 10.41 years, S.D. = 2.87 years; mean age at onset = 5.86 years, S.D. = 3.46 years) completed a neuropsychological battery. Neurophysiological data were collected from the most recent EEG. RESULTS: In the recent-onset sample, no neuropsychological scores were related to any EEG variable. In the chronic sample, however, presence of slow-wave activity was related to memory impairment (p < 0.01). Post-hoc analyses on other neuropsychological measures showed localization of epileptiform activity (EA) might be related to verbal learning. DISCUSSION: Children with slow-wave activity on EEG might be at increased risk for developing neuropsychological deficits. When these abnormalities are observed on a child's EEG, closer monitoring of cognitive and academic functioning seems warranted. Differences between these findings and past research suggest that conclusions drawn from adult surgical studies cannot be generalized to pediatric patients, especially recent-onset samples, without qualification. Differences between the recent-onset and chronic samples in this cross-sectional study raise the possibility that neurophysiological abnormalities have a cumulative effect on cognitive development.     

Neuropsychological outcomes in children of mothers with epilepsy.

The study investigated the nature of the effects of maternal epilepsy on cognitive performance of the offspring. One hundred fifty-four children of mothers with epilepsy aged 5 to 11 years (study group), along with 130 control children, comparable with respect to IQ, socio-economic status, age, and gender underwent a neuropsychological assessment using subtests from the NEPSY: A Developmental Neuropsychological Assessment, tapping attentional, auditory-verbal, visuomotor, fine motor, and memory abilities. The study group scored significantly lower than the controls on measures of attention, memory, and fine-motor function. Deficits were more marked in but not limited to the subset of the study group exposed to maternal medication in utero. Group differences on auditory attention were found only in younger children. Valproate-exposed children obtained lower scores on sentence repetition, as well as on the more demanding part of a test of auditory attention, than other children in the study group, suggesting weaknesses in working memory in the former subgroup. Confounding by maternal epilepsy type and polytherapy complicate interpretation of this finding. Differences between subsets of children not exposed to anti-epileptic drugs in utero and controls suggest that both drug exposure and genetic factors may contribute to cognitive deficits associated with maternal epilepsy.     

Neuropsychological findings in children with benign childhood epilepsy with centrotemporal spikes

Benign childhood epilepsy with centrotemporal spikes (BCECTS) is a well-known idiopathic age- and localization-related epileptic syndrome with characteristic clinical and EEG manifestations. Due to the reported benign evolution of this epilepsy syndrome, neuropsychological assessment has been considered unnecessary. However, the benign nature of BCECTS has recently been challenged: verbal dysfunction as well as impaired visuomotor coordination, specific learning disabilities, and attention deficit have been noticed. These findings prompted this research study in which all children with BCECTS attending our epilepsy clinic underwent neuropsychological assessment. Seventeen children (10 boys and seven girls) aged 7 to 14 years were investigated with a neuropsychological test battery focusing on immediate and delayed recall of auditory-verbal and visual material, verbal fluency, problem-solving ability, and visuospatial constructional ability. Raven's coloured matrices and questionnaires regarding school functioning and behaviour were also administered. The children were matched with control subjects for age, sex, and school. Children with BCECTS had significantly lower scores than their control subject partners on the neuropsychological items. Intellectual abilities did not differ and neither did school functioning or behaviour according to teachers. Parents, however, recognized greater difficulties with concentration, temperament, and impulsiveness in children with BCECTS.     

Outcome of children with juvenile absence epilepsy

The incidence and natural history of childhood absence epilepsy are well documented, but those of juvenile absence epilepsy are poorly delineated. We conducted a retrospective chart study to evaluate the incidence and outcome of patients with juvenile absence epilepsy by retrieving the medical records of consecutive patients with juvenile absence epilepsy who were evaluated in three pediatric neurology outpatient clinics in Israel. Inclusion criteria included the onset of epilepsy after the age of 10 years and follow-up until at least 15 years of age. The patients with an electroencephalogram (EEG) suggestive of myoclonic epilepsy (polyspike and wave) were excluded from the study. Seventeen patients (10 female and 7 male) fulfilled the inclusion criteria for juvenile absence epilepsy. They presented with epilepsy at a mean age of 11.94 years (range 10-16.5 years). The mean duration of follow-up was 6.05 years (range 2-12 years). Five patients (29.4%) had a family history of epilepsy. All 17 patients had a normal neurodevelopmental status. Eight patients (47%) experienced generalized tonic-clonic seizures. At follow-up, eight patients (43.7%) were seizure free. Only three (37.5%) of the patients who experienced generalized tonic-clonic seizures were seizure free during follow-up compared with five (55.5%) patients without generalized tonic-clonic seizures. Our results indicate that the outcome of patients with juvenile absence epilepsy is less favorable than children with childhood absence epilepsy and that the presence of generalized tonic-clonic seizures is a predictor for poorer outcome.     

Neuropsychological assessment for temporal lobe epilepsy surgery

Neuropsychological assessment consists of a comprehensive evaluation of cognitive functioning and most often some evaluation of motor skills and sensory status also. Cognitive functions sampled typically include "intelligence" (IQ tests), attention, language skills, visuospatial abilities, "executive skills" and other abilities associated with frontal-lobe function, and learning and memory. Thus, the assessment samples vary widely among a variety of functions, providing a comprehensive picture of an individual's strengths and weaknesses. The resulting pattern points to the probable site of epileptic focus. Neuropsychological findings also serve to predict the risk for postsurgical cognitive decline and, when performance before and after operation is compared, they provide data on the impact of surgery upon cognitive functioning. Comprehensive evaluation of learning and memory is particularly important in this context, because of the frequency of temporal lobe epilepsy and the prominence of memory dysfunction associated with it. In addition, patients slated for elective surgery may also undergo an intracarotid amobarbital procedure (IAP), which is performed to determine the side of cerebral dominance for language and to test the memory capabilities of each hemisphere alone. All of these specialized neuropsychological tools are discussed in this paper.     

Neuropsychological features of benign partial epilepsy in children

Rolandic paroxysmal epilepsy (RPE) is a useful model for investigating the complex links between epilepsy and cognitive dysfunction. 44 children with RPE who met the following (among other) criteria: negative CT scan, freedom from drug treatment, and IQ≥80, were assigned to three subgroups by side of EEG focus: left, right and bilateral. A neuropsychological bactery elicited small differences in cognitive performance between the whole group and the controls and among the subgroups, only partially correlated with EEG side. A follow-up assessmt showed that the short falls has disappeared along with the seizures and EEG anomalies, thus confirming the benign nature of RPE. Our findings suggest too that the mere presence of paroxysmal cortical activity is enough to trigger cognitive dysfunction.

---

Sammario L'epilessia parziale benigna dell'infanzia a parossismi rolandici (EPR) può costituire un utile modello di indagine delle complesse relazioni esistenti fra epilessia e disfunzioni cognitive. Nell'ambito di una popolazione globale di 107 pazienti con EPR sono stati selezionati 44 bambini in base a numerosi parametri tra cui la negatività della TC, l'assenza di trattamento farmacologico, un Q.I.≧80; sono stati quindi distinti tre gruppi in rapporto alla sede emisferica destra, sinistra o bilaterale del focolaio EE-Grafico. La somministrazione di una estesa batteria neuropsicologica ha evidenziato che i bambini con EPR, rispetto ai controlli, presentano lievi anomalie della sfera cognitiva, solo parzialmente correlabili con il lato EEGrafico. Una successiva osservazione longitudinale ha permesso di individuare 11 casi in cui le alterazioni cliniche e strumentali tipiche della EPR erano assenti da almeno 4 anni rispetto al periodo della valutazione neuropsicologica. In questa seconda fase dell'indagine i pazienti hanno mostrato perstazioni equivalenti a quelle dei controlli; le anomalie cognitive sembrano quindi transitorie così come si verifica per gli episodi critici e le alterazioni EEGrafiche. I risultati da una parte sembrano confermare che la EPR è una sindrome benigna anche per quanto riguarda gli aspetti cognitivi e comportamentali, dall'altra suggeriscono che la pre quanto riguarda gli aspetti cognitivi e comportamentali, dall'altra suggeriscono che la presenza di una attività parossistica corticale può essere sufficiente da sola a determinare anomalie del funzionamento dei processi cognitivi.

     

Neuropsychological status of children with newly diagnosed idiopathic childhood epilepsy

We characterized the neuropsychological status of children with newly diagnosed idiopathic childhood epilepsy and measured differences in IQ between children with different types of epilepsy. The Korean Education Development Institute-Wechsler Intelligence Scale for Children (KEDI-WISC) was administered to 72 patients (35 males and 37 females), of mean age 8.7 ± 2.6 years, with newly diagnosed idiopathic childhood epilepsy. Of these patients, 22 (30.6%) had generalized epilepsy, 48 (66.7%) localization-related epilepsy, and 2 (2.8%) mixed epilepsy. Children with generalized epilepsy and benign childhood epilepsy with centro-temporal spikes (BCECTS) were of similar verbal IQ and full-scale IQ, although performance IQ was significantly lower in patients with generalized epilepsy. Among children with BCECTS, those with unilateral spikes had higher full-scale and performance IQ scores than those with bilateral spikes. Follow-up studies on large numbers of patients are needed to determine the effects of epilepsy per se, and antiepileptic drugs, on intelligence.     

Natural history of absence epilepsy in children

Absence seizures may be seen in a variety of epileptic syndromes in childhood. Identification of the specific syndrome is important to determine medical prognosis. With childhood absence epilepsy, approximately two thirds of children can be expected to enter long-term remission, while in juvenile absence epilepsy, seizure control is often achieved, however, lifelong treatment is usually required. Other absence syndromes have a poorer prognosis, with lower rates of seizure control and remission. Psychosocial outcome is often poor, even in patients with more benign forms of absence epilepsy. Remission of epilepsy does not preclude psychosocial morbidity.     

Neuropsychological profiles and outcomes in children with new onset frontal lobe epilepsy

Frontal lobe epilepsy (FLE) is the second most frequent type of localization-related epilepsy, and it may impact neurocognitive functioning with high variability. The prevalence of neurocognitive impairment in affected children remains poorly defined.This report outlines the neuropsychological profiles and outcomes in children with new onset FLE, and the impact of epilepsy-related factors, such as seizure frequency and antiepileptic drug (AED) load, on the neurocognitive development.Twenty-three consecutive children (15 males and 8 females) with newly diagnosed cryptogenic FLE were enrolled; median age at epilepsy onset was 7 years (6–9.6 years). They underwent clinical and laboratory evaluation and neuropsychological assessment before starting AED treatment (time 0) and after one year of treatment (time 1).Twenty age-matched patients affected by idiopathic generalized epilepsy (10 male and 10 females) and eighteen age-matched healthy subjects (9 males and 9 females) were enrolled as controls and underwent the same assessment.All patients with FLE showed a significant difference in almost all assessed cognitive domains compared with controls, mainly in frontal functions and memory. At time 1, patients were divided into two groups according to epilepsy-related factors: group 1 (9 patients) with persisting seizures despite AED polytherapy, and group 2 (14 patients) with good seizure control in monotherapy. A significant difference was highlighted in almost all subtests in group 1 compared with group 2, both at time 0 and at time 1.In children with FLE showing a broad range of neurocognitive impairments, the epilepsy-related factors mostly related to a worse neurocognitive outcome are poor seizure control and the use of AED polytherapy, suggesting that epileptic discharges may have a negative impact on the functioning of the involved cerebral regions.     

Simultaneous EEG-fMRI in drug-naive children with newly diagnosed absence epilepsy

Purpose: In patients with idiopathic generalized epilepsy (IGE), blood oxygen level dependent (BOLD) EEG during functional MRI (EEG‐fMRI) has been successfully used to link changes in regional neuronal activity to the occurrence of generalized spike‐and‐wave (GSW) discharges. Most EEG‐fMRI studies have been performed on adult patients with long‐standing epilepsy who were on antiepileptic medication. Here, we applied EEG‐fMRI to investigate BOLD signal changes during absence seizures in children with newly diagnosed childhood absence epilepsy (CAE). Methods: Ten drug‐naive children with newly diagnosed CAE underwent simultaneous EEG‐fMRI. BOLD signal changes associated with ictal EEG activity (i.e., periods of three per second GSW) were analyzed in predefined regions‐of‐interests (ROIs), including the thalamus, the precuneus, and caudate nucleus. Results: In 6 out of 10 children, EEG recordings showed periods of three per second GSW during fMRI. Three per second GSW were associated with regional BOLD signal decreases in parietal areas, precuneus, and caudate nucleus along with a bilateral increase in the BOLD signal in the medial thalamus. Taking into account the normal delay in the hemodynamic response, temporal analysis showed that the onset of BOLD signal changes coincided with the onset of GSW. Discussion: In drug‐naive individuals with CAE, ictal three per second GSW are associated with BOLD signal changes in the same striato‐thalamo‐cortical network that changes its regional activity during primary and secondary generalized paroxysms in treated adults. No BOLD signal changes in the striato‐thalamo‐cortical network preceded the onset of three per second GSW in unmediated children with CAE.     

Surgery of children with frontal lobe lesional epilepsy: Neuropsychological study

Aim of the study was to provide new data about the evolution of neuropsychological findings in patients with lesional frontal lobe epilepsy (FLE) operated on with lesion excision. Patients and methods Twelve patients with lesional FLE underwent full clinical examination including neurological, neuropsychological and developmental assessments, high-resolution magnetic resonance imaging (MRI), ictal and interictal prolonged EEG monitoring and evaluation of seizure semeiology before and after surgery. The mean follow-up duration was 2 years and 10 months (range = 14 months-7 years). Another group of lesional temporal lobe epilepsy, matched for the age at surgery and side of surgery, was likewise studied in order to compare neuropsychological patterns and to try to find out specific features in frontal lobe epilepsy evolution. Results All patients resulted seizure free at outcome except one belonging to Engel’s class II. Before surgery general intelligence was similar in FLE as well as in TLE group. Executive functions and motor coordination were frequently affected in FLE whereas patients with TLE often presented with deficits in naming, visual memory and visuo-spatial attention. After surgery there was a frequent decline of IQ in FLE group together with a slight deterioration, especially of executive functions in some patients. An improvement of behaviour was often observed in both groups. Conclusions As already reported in literature, neuropsychological pre-surgical data confirms the involvement of attention and executive functions in lesional FLE. No significant neuropsychological improvement was produced by surgery that determined in some cases a slight decline of general intelligence and specific frontal abilities. Yet, generally behaviour improved and seizures were controlled.     

Neuropsychological assessment and clinical evaluation in temporal lobe epilepsy with associated cortical dysplasia

Temporal Lobe Epilepsy (TLE) is a chronic neurological disorder, often associated to cognitive deficits. Focal cortical dysplasia (FCD), frequently associated to high risk of epilepsy, can lead to abnormalities in cognition. The aim of this study was to explore neuropsychological performance and to identify potential risk factors for cognitive impairment in TLE subjects with associated FCD. Our sample was composed by 46 TLE patients with FCD (37.76 ± 12.60 years; 29 females and 16 males) and 44 healthy controls (41.05 ± 9.74 years; 25 females and 19 males). All subjects performed a neuropsychological battery associated to a measurement of depression and anxiety. Results showed a poor performance of all domains of cognitive functioning and identified age of epilepsy onset as potential risk factor of cognitive impairment. These findings support the importance to focus on cognitive impairment in TLE patients with FCD to better clarify the impact of epilepsy features and FCD in therapeutic and everyday management.     

Intellectual functioning in children with epilepsy: Frontal lobe epilepsy,childhood absence epilepsy and benign epilepsy with centro-temporal spikes

PurposeThe purpose of our study is to describe intellectual functioning in three common childhood epilepsy syndromes – frontal lobe epilepsy (FLE), childhood absence epilepsy (CAE) and benign epilepsy with centro-temporal spikes (BECTS). And also to determine the influence of epilepsy related variables, type of epilepsy, age at epilepsy onset, duration and frequency of epilepsy, and treatment on the scores.MethodsIntellectual functioning was examined in a group of 90 children with epilepsy (30 FLE, 30 CAE, 30 BECTS), aged 6–15 years, and compared with a control group (30). All subjects obtained a Full Scale IQ ≥ 70 and they were receiving no more than two antiepileptic medications. Participants completed the Wechsler Intelligence Scale for Children – Third Edition. The impact of epilepsy related variables (type of epilepsy, age at epilepsy onset, duration of epilepsy, seizure frequency and anti-epileptic drugs) on intellectual functioning was examined.ResultsChildren with FLE scored significantly worse than controls on WISC-III Verbal IQ, Full Scale IQ and Processing Speed Index. There was a trend for children with FLE to have lower intelligence scores than CAE and BECTS groups. Linear regression analysis showed no effect for age at onset, frequency of seizures and treatment. Type of epilepsy and duration of epilepsy were the best indicators of intellectual functioning.ConclusionIt is crucial that children with FLE and those with a longer active duration of epilepsy are closely monitored to allow the early identification and evaluation of cognitive problems, in order to establish adequate and timely school intervention plans.     

Neuropsychological outcome following focal cortical removal for intractable epilepsy in children

In children, surgery for epilepsy has been recognized as a viable treatment option since publication of S. Davidson and M.A. Falconer's outcome study in 1975 [Lancet North Am Ed 5:1260-3], which demonstrated that medical outcome of children who underwent anterior temporal lobectomy paralleled that of adults. Pediatric surgical programs and the literature on medical outcome have grown considerably since that time, with surgery being offered to children with temporal but also extratemporal epilepsy foci. Comparatively little work has been conducted in the area of neuropsychological outcome. This article outlines differences in adult and pediatric outcome studies, reviews the literature on the intellectual and memory outcome in children, and discusses shortcomings of the pediatric outcome research conducted to date.     

Clinical and neurophysiologic spectrum associated with atypical absence seizures in children with intractable epilepsy

The aim of this study was to describe the clinical and neurophysiologic correlates of atypical absence seizures in children with intractable epilepsy. In a retrospective review, 19 children with videoelectroencephalographic monitoring (female n=14; male n=5) fulfilled the electroclinical criteria for this seizure type. Atypical absence seizures occurred in a spectrum of clinical conditions associated with educational disability and intractable seizures. In comparison with children with only atypical absence seizures, children with atypical absence in association with multiple seizure types were more likely to have severe educational disability (n=11 of 13; P = .01), a slower ictal frequency (n=10 of 13; P = .01), and slow background rhythms for age (n = 13 of 13; P = .03). This study illustrates the broad clinical spectrum in which atypical absence seizures are encountered. Differentiation between children with only atypical absence seizures and children with multiple seizure types can be useful with respect to potential academic ability.     

Neuropsychological profile of children with cryptogenic localization-related epilepsy and its association with age at onset

The aim of this study was to investigate the neuropsychological profile of children with cryptogenic localization-related epilepsy (CLRE). Neuropsychological evaluations were performed in 16 CLRE children and 14 children with idiopathic localization-related epilepsy (ILRE) for control within 8 months (average 2.1 months) of initial seizure. The neuropsychological tests used in this study are as follows: the Wechsler Intelligence Scale for Children-Third Edition, Wechsler Intelligence Scale for Children-Revised, and Wechsler Preschool and Primary Scale of Intelligence. Age at onset and test differed significantly between CLRE and ILRE, while the duration between onset and test and the number of seizures before test did not. No marked difference was observed in the neuropsychological profile between 2 groups; however, the discrepancy between VIQ and PIQ was significantly larger in CLRE than in ILRE. This discrepancy was negatively correlated with age at the time of seizure onset (r = -0.615, and p = 0.011). The laterality in discrepancy between VIQ and PIQ was associated with the dominance of interictal discharge. In conclusion, children with lower age at the time of seizure onset were likely to have had a larger discrepancy between VIQ and PIQ.     

Non-invasive assessment of cardioregulatory autonomic functions in children with epilepsy

OBJECTIVES: We aimed to evaluate the interictal cardiovascular autonomic functions in pediatric patients with idiopathic epilepsy, both partial and generalized. MATERIALS AND METHODS: The study included 25 patients with idiopathic epilepsy and 50 control subjects. Patients underwent five standardized clinical cardiovascular reflex autonomic tests [resting heart rate (HR), HR response to deep breathing and to Valsalva maneuver, the 30:15 ratio of HR response to standing, and blood pressure response to standing], as well as a 12 lead surface electrocardiogram. Heart rate variability (HRV) was tested via 24-h Holter monitoring and the time domain parameters (SDNN, PNN50, rMSDD) were assessed. Excretion of vanillyl mandelic acid and metanephrine was measured in 24-h urine collection. RESULTS: Clinical reflex autonomic tests showed mild dysfunction in 8%, moderate dysfunction in 44% and severe dysfunction in 4% of patients. The HRV parameter, SDNN, was reduced in all age groups, while rMSDD and PNN50 were reduced only in the older age group. Metanephrine levels were significantly reduced in the patients group. Patients with uncontrolled epilepsy had a significantly higher frequency of autonomic dysfunction as assessed by clinical scoring. CONCLUSION: Cardiac autonomic dysfunction is not uncommon in pediatric patients with epilepsy. Altered cardiovascular regulation seems to be related to the epilepsy itself rather than to the characteristics of the disorder.     

Cognitive assessment in epilepsy surgery of children

Introduction Although the neurocognitive assessment in children as in the adults is an important step before and after surgery, in the literature, the data about pre- and postoperative neurocognitive evaluations in children are very few.Objective The purpose of this paper is to consider some peculiar aspects of the neurocognitive assessment during development, and report literature data about neuropsychological outcome of epileptic children treated with focal resection and hemispherectomy.Results and discussion The second section concerns our personal experience about a cohort of 45 children with refractory epilepsy operated on before 7 years. The results suggest that early surgical treatment is generally effective for seizure control and behavior improvement in children with refractory epilepsy. Concerning cognitive outcome, we found that the neurocognitive level was unchanged in the majority of the patients.Conclusion We underline the importance of multicentric studies with standardized neuropsychological assessments in large series of young children.