Which children receive vigabatrin? Characteristics of pediatric patients enrolled in the mandatory FDA registry.

Vigabatrin (Sabril®) is an antiepileptic drug (AED) currently indicated in the US as a monotherapy for patients 1month to 2years of age with infantile spasms (IS) and as adjunctive therapy for patients ≥10years of age with refractory complex partial seizures (rCPS) whose seizures have inadequately responded to several alternative treatments and for whom the potential benefits outweigh the risk of vision loss. The approval required an FDA mandated registry. This article describes 5years of demographic and treatment exposure data from US pediatric patients (<17years). Participation is mandatory for all US Sabril® prescribers and patients. A benefit-risk assessment must be documented for patient progression to maintenance therapy. This includes demographic diagnosis and reports of ophthalmologic assessments (where available). Patient data were grouped by age as proxies for indication (IS: <3years, rCPS: ≥3 to <17years). As of August 26, 2014, 5546/6823 enrolled patients were pediatric/total; 4472 (81%) were vigabatrin-naïve. Seventy-one percent of patients were <3years of age; 29% were ≥3 to <17years of age. Etiologies of IS were identified as cryptogenic (21%), symptomatic tuberous sclerosis (17%), and symptomatic other (42%). The majority of patients with IS (56%) attempted no prior treatments; 16% received adrenocorticotropic hormone prior to vigabatrin. A third of patients with IS were receiving 1 concomitant treatment with vigabatrin. For patients with rCPS, 39% attempted 1-3 prior treatments; 27% were receiving 2 concomitant treatments at enrollment. A total of 1852 (41%) patients did not undergo baseline ophthalmological assessment; 25% of patients with IS and 42% of patients with rCPS were exempted for neurologic disabilities. Kaplan-Meier estimates predict that 71% and 65% of vigabatrin-naïve patients with IS and rCPS, respectively, would remain in the registry at 6months. Most pediatric vigabatrin patients have IS as an underlying diagnosis, especially those <3years of age. A proportion of those with rCPS remain on long-term vigabatrin despite the risk of adverse events.

Neurobehavioral comorbidities of pediatric epilepsies are linked to thalamic structural abnormalities.

PURPOSE: Neurobehavioral comorbidities are common in pediatric epilepsy with enduring adverse effects on functioning, but their neuroanatomic underpinning is unclear. Striatal and thalamic abnormalities have been associated with childhood-onset epilepsies, suggesting that epilepsy-related changes in the subcortical circuit might be associated with the comorbidities of children with epilepsy. We aimed to compare subcortical volumes and their relationship with age in children with complex partial seizures (CPS), childhood absence epilepsy (CAE), and healthy controls (HC). We examined the shared versus unique structural-functional relationships of these volumes with behavior problems, intelligence, language, peer interaction, and epilepsy variables in these two epilepsy syndromes. METHODS: We investigated volumetric differences of caudate, putamen, pallidum, and thalamus in children with CPS (N = 21), CAE (N = 20), and HC (N = 27). Study subjects underwent structural magnetic resonance imaging (MRI), intelligence, and language testing. Parent-completed Child Behavior Checklists provided behavior problem and peer interaction scores. We examined the association of age, intelligence quotient (IQ), language, behavioral problems, and epilepsy variables with subcortical volumes that were significantly different between the children with epilepsy and HC. KEY FINDINGS: Both children with CPS and CAE exhibited significantly smaller left thalamic volume compared to HC. In terms of developmental trajectory, greater thalamic volume was significantly correlated with increasing age in children with CPS and CAE but not in HC. With regard to the comorbidities, reduced left thalamic volumes were related to more social problems in children with CPS and CAE. Smaller left thalamic volumes in children with CPS were also associated with poor attention, lower IQ and language scores, and impaired peer interaction. SIGNIFICANCE: Our study is the first to directly compare and detect shared thalamic structural abnormalities in children with CPS and CAE. These findings highlight the vulnerability of the thalamus and provide important new insights on its possible role in the neurobehavioral comorbidities of childhood-onset epilepsy.

The adult seizure and social outcomes of children with partial complex seizures.

Most intellectually normal children with focal epilepsy have partial complex or focal with secondary generalization seizures without a precise epilepsy syndrome. Their long-term outcome is largely unknown. Cases were identified from the population-based Nova Scotia Childhood Epilepsy cohort. Those eligible had seizure onset at 1 month to 16 years between 1977 and 1985, normal intelligence, ≥10 years of follow-up, only focal seizures and no benign epilepsy syndromes. There were 108 patients with partial complex with or without secondary generalization as the only seizure type(s) throughout (partial complex group) and 80 with secondary generalization as the only seizure type (secondary generalization group). Average age ± standard deviation at onset was 7.3 ± 4.5 years and follow-up was 27.9 ± 5.4 years. At follow-up, 57% of the partial complex group were in remission versus 81% of the secondary generalization group (P = 0.001). The partial complex group was more likely to be intractable or have undergone epilepsy surgery (36% versus 5%, P = 0.000). In the partial complex group, 28% had <5 years seizure free versus 5% in the secondary generalized group (P = 0.000). More patients in the partial complex group had undergone mental health assessments (59% versus 32%, P = 0.000), and 33% had a psychiatric diagnosis versus 15% in the secondary generalized group (P = 0.004). More patients with partial complex seizures had specific learning disorders (63% versus 45%, P = 0.03). Seven markers of poor social outcome were more common in patients with partial complex seizures (>2 markers: 34% versus 10%, P = 0.000). During 25-30 years of follow-up, >50% of intellectually normal patients with childhood-onset partial complex seizures had difficult-to-control seizures and learning and psychiatric/social problems. Most with secondary generalized seizures only had remission and better academic and psychiatric/social outcomes.

[Oligoepilepsy: a real entity or the benign form of epileptic disorder?]. / Oligoepilepszia: onálló entitás vagy az epilepsziás készség benignus formája?

BACKGROUND AND PURPOSE: Although oligoepilepsy (OLE) is a used term in many protocols, guidelines and the everyday routine, it is found practically nowhere in the scientific literature. The aim of our study is to investigate and evaluate of the main characteristics of his subcategory of epilepsy. We try to find answer to the basic question of not only theoretical but also great practical importance whether the OLE does really exist, is it an independent entity of epilepsy or only its general benign clinical presentation. METHODS: We considered OLE if the patients had two seizures maximally in the last year of their course. We counted only the two most severe clinical types, the generalized tonic-clonic and the complex partial seizures. We divided the OLE into two subtypes: those patients who had OLE from the beginning of their epilepsy (OLE1) and those in whom the OLE was the result of the treatment (OLE2). We retrospectively analysed the data of 817 "OLE-suspicious" patients taken from our EPIMED database. RESULTS: We found 47 patients met the inclusion criteria (OLE1 = 34, OLE2 = 13). OLE patients did not differ from the general epileptic population according to the age and gender, the type of seizures, the electro-clinical diagnosis and the possible cause of their first seizure. But we found statistically significant differences in two measures. In OLE, far less seizure provoking factors were found in the sporadic seizures. Concerning the social conditions: while the range of employees was equal, the vast majority of OLE patients were able to work at their level of education. CONCLUSION: We found that more than 5% of people with epilepsy can belong to the OLE category. In the majority of OLE patients the seizure frequency is low from the beginning of the disease. The sporadic seizures in OLE are generally not provoked. The chances of OLE patients in the world of job are better for the OLE patients. In our opinion OLE rather seems to be an independent entity among epilepsy. Therefore larger prospective studies are needed to the exact description of OLE and to establish the special standards for the everyday medical practice.

Study of DSM-IV Axis I psychiatric disorders in patients with refractory complex partial seizures using a short structured clinical interview.

Psychiatric disorders are a source of significant comorbidity in patients with refractory epilepsy, yet are often underrecognized. We assessed the prevalence of DSM-IV Axis I psychiatric disorders using a short structured clinical interview (Mini-International Psychiatric Interview [MINI]) in patients with medically refractory complex partial seizures. Consecutive patients with refractory epilepsy being evaluated with video/EEG monitoring and imaging for seizure focus localization and lateralization underwent MINI evaluation to assess for the presence of psychiatric disorders. Among 117 patients (74 male, 43 female) studied, 57 (48.7%) had at least one psychiatric disorder; 19 (16.2%) had depression, 10 (8.5%) dysthymia, 27 (23.0%) anxiety disorder, and 11(9.4%) other disorders. Most clinical features and epilepsy-related variables had no significant association with psychiatric disorder on logistic regression analysis. Almost half of the patients with refractory focal seizures have a coexistent psychiatric disorder, and its presence or absence cannot be predicted by their clinical profiles. All patients should be assessed and treated for psychiatric comorbidity to improve overall quality of life.

[Epilepsy in the elderly].

Elderly persons with epilepsy are expected to be an increasing larger group among patients with epilepsy in view of population aging and the higher onset of epilepsy in the elderly as compared to any other age group. Cerebrovascular disease is the most common cause of seizures in the elderly, and complex partial seizures (CPS) are the most common seizure type in this age group. CPS semiology is often different in the elderly compared to young adults, since strokes usually involve extratemporal regions. The absence of familiar clinical characteristics of seizures, such as aura and automatisms, can delay the correct diagnosis of epilepsy or Lead to misdiagnosis. As a result of age-related pharmacokinetic changes, the clearance of most old and new antiepileptic drugs (AEDs] is reduced by 20-40% in elderly persons compared to younger adults, and there may be a longer elimination half-life of certain AEDs. In addition to measurements of serum AED concentration, it is important to monitor clinical response, since age-related pharmacodynamic changes can alter the relationship between serum AED concentration and pharmacological effects. Newer AEDs have a lower potential for drug interactions and are better tolerated by the elderly compared to old generation AEDs. Monotherapy is the preferred therapeutic strategy for initial treatment of seizures due to fewer adverse events, decreased risk of drug interactions, improved adherence and lower treatment costs. Elderly patients with epilepsy are more likely to remain seizure-free on AED treatment than younger age groups. Elderly patients with refractory epilepsy and precise localization of the seizure focus can be appropriate surgical candidates, and a favorable prognosis is expected with curative surgical procedures.

Clinical spectrum and difficulties in management of hypothalamic hamartoma in a developing country.

AIM: We describe the clinical features, treatment and prognosis in a series of patients with epilepsy secondary to hypothalamic hamarthomas (HH) in a developing country. MATERIALS AND METHODS: Eight patients with epilepsy and HH were included between 1997 and 2006. We analyzed gender, age, age at seizure onset (ASO), seizure types (ST), mental retardation (MR), precocious puberty (PP), electroencephalogram (EEG)-magnetic resonance imaging (MRI) features and response to treatment. RESULTS: Mean age 25.1 years, 2/6 female/male, none had PP, ASO 4.5 years. Complex partial seizure were the most frequent (100%), mean similar to those seen in temporal (62.5%) or frontal lobe epilepsy (37.5%). Exactly 87.5% developed gelastic seizures (GS). Half of the patients showed MR. Mild-to-severe MR was associated with the presence of multiple ST including atonic and complex partial seizures with frontal semiology. Interictal EEG was abnormal in 87.5% patients. Video EEG was performed in three cases with unspecific findings. HH were small and sessile in seven patients whereas large and pedunculated in one. All patients were refractory to medical treatment. In five, an additional procedure was performed without any significant improvement. CONCLUSION: These series show the heterogeneous spectrum of this entity and the difficulties in its treatment in a developing country.

Clinical description of seizures in patients with systemic lupus erythematosus.

OBJECTIVE: To evaluate the frequency of and risk factors for epileptic seizures in patients with systemic lupus erythematosus (SLE) in a large cohort series. METHODS: One thousand two hundred patients with SLE were analyzed. The type and frequency of risk factors for seizures associated with SLE were studied and compared with two other series reported in the literature. RESULTS: One hundred and forty-two patients had seizures. Seventy-five patients were studied with a mean follow-up of 5 years from the first seizure episode. Fifty-eight (77%) patients had tonic-clonic seizures, 9 (12%) complex partial seizures (PS), 5 (7%) simple partial motor seizures and 3 (4%) secondary tonic-clonic seizures. In 41 (54%) patients, the seizures occurred within the first year of the SLE diagnosis. Recurrence occurred in 40 (53%) patients, and was associated with PS in 14 (35%; p = 0.006) and time of seizures with SLE onset in 5 (12.5%; p = 0.05). Less than one third of the patients had positive antiphospholipid antibodies. A concurrent infection was present in 16 (21%) patients. CONCLUSIONS: Epileptic seizures were more common during the first year after SLE diagnosis. Neither infection nor antiphospholipid syndrome was associated with the occurrence of seizures.

Surgery for medically intractable temporal lobe epilepsy during early life.

PURPOSE: Temporal lobe epilepsy (TLE) in early life is often a catastrophic disorder with pharmacoresistant seizures and secondary neurological deterioration. there is little data available regarding epilepsy surgery performed in infants and young children and no prior study has focused on tle. METHODS: We analyzed the results of temporal resection for epilepsy as the primary indication in children less than age 5 years who had at least 2 years of follow-up. RESULTS: 20 children (14 males) were identified with a mean age at surgery of 26 months and a mean age at seizure onset of 12 months. Clinical presentation was diverse. Typical psychomotor seizures (n = 4; mean age at surgery 37 months) were followed by prominent motor changes (n = 7; 30 months) and were occasionally isolated (n = 3; 23 months). Epileptic spasms were noted in six patients and were frequently associated with lateralizing features. The interictal EEG was lateralizing in 15 patients and the ictal EEG was lateralizing in 18 patients. Brain MRI provided localizing value in 16 patients, ictal SPECT was concordant in 4/8 cases. Invasive EEG was employed in six cases. At mean follow-up of 5.5 years, 65% of the children were seizure-free and 15% had >90% seizure reduction. Morbidity included infection and hydrocephalus in one case and stroke-related hemiparesis in two cases. Cortical dysplasia was identified in eight children, tumors in eight including two DNET, two ganglioglioma, and four malignant tumors. Hippocampal sclerosis was present in four cases, always as dual pathology. CONCLUSION: TLE presents in early life with varied and severe manifestations. Excisional procedures in this age group are associated with favorable seizure reduction similar to older children and in adults.

Treatment of pediatric epilepsy: European expert opinion, 2007.

BACKGROUND: Childhood epilepsies are a heterogeneous group of conditions that differ in diagnostic criteria and management and have dramatically different outcomes. Despite increasing data on treatment of epilepsy, research findings on childhood epilepsy are more limited and many clinical questions remain unanswered, so that clinicians must often rely on clinical judgment. In such clinical situations, expert opinion can be especially helpful. METHODS: A survey on pediatric epilepsy and seizures (33 questions and approximately 650 treatment options) was sent to 57 European physicians specializing in pediatric epilepsy, 42 (74%) of whom completed it. In some questions, the experts were asked to recommend overall treatment approaches for specific syndromes (the order in which they would use certain strategies). Most of the questions asked the experts to rate options using a modified version of the RAND 9-point scale for medical appropriateness. Consensus was defined as a non-random distribution of scores by chi-square test, with ratings used to assign a categorical rank (first line/usually appropriate, second line/equivocal, and third line/usually not appropriate) to each option. RESULTS: Valproate was treatment of choice for symptomatic myoclonic and generalized tonic-clonic seizures. For initial monotherapy for complex partial seizures, carbamazepine and oxcarbazepine were treatments of choice, with valproate also first line. As initial therapy for infantile spasms caused by tuberous sclerosis, viagabatrin was treatment of choice. As initial therapy for infantile spasms that are symptomatic in etiology, vigabatrin was also treatment of choice, with adrenocorticotropic hormone (ACTH) and prednisone other first-line options. As initial therapy for Lennox-Gastaut syndrome, valproate was treatment of choice. For acute treatment of a prolonged febrile seizure or cluster of seizures, rectal diazepam was treatment of choice. Valproate was treatment of choice as preventive therapy for febrile seizures. For benign childhood epilepsy with centro-temporal spikes, valproate was treatment of choice. For childhood and juvenile absence epilepsy, valproate was treatment of choice, with lamotrigine another first-line option (ethosuximide was another first-line option for childhood absence epilepsy). For juvenile myoclonic epilepsy in adolescent males, valproate was treatment of choice, with lamotrigine another first-line option; for juvenile myoclonic epilepsy in adolescent females, lamotrigine was treatment of choice, with valproate another firstline option. As initial therapy for neonatal status epilepticus, intravenous (IV) phenobarbital was treatment of choice. As initial therapy for all types of pediatric status epilepticus, IV diazepam was treatment of choice. For generalized tonic-clonic status epilepticus, rectal diazepam and IV lorazepam were also treatments of choice; for complex partial status epilepticus, IV lorazepam was another first-line option. CONCLUSION: The expert panel reached consensus on many treatment options. Within the limits of expert opinion and with the understanding that new research data may take precedence, the experts' recommendations provide helpful guidance in situations where the medical literature is scant or lacking. The information in this report should be evaluated in conjunction with evidence-based findings.

Cryptogenic localization related epilepsy in children from a tertiary outpatient clinic: is neurological and neuropsychological outcome predictable?

OBJECTIVES: Up to one-third of the children with epilepsy are diagnosed with cryptogenic localization related epilepsy (CLRE). As yet, there is a lack of studies that specify the short- and long-term prognosis for this group. In this study, we systematically established neurological outcome (represented by seizure frequency) as well as neuropsychological outcome in a cohort of 68 children with CLRE who had been referred to our tertiary outpatient clinic. Also, we analysed correlations with risk and prognostic factors. PATIENTS AND METHODS: A systematic cross-sectional open clinical and non-randomized design was used including 68 children admitted to our epilepsy centre in a child neurological programme between January 1999 and December 2004. A model was defined, distinguishing risk factors with a potential effect on epileptogenesis (history of febrile seizures, family history of epilepsy, history of early mild development delay and serious diagnostic delay) and prognostic factors, with a potential effect on the course of the epilepsy (neurological symptoms or soft signs, age at onset, duration of epilepsy, seizure type, percentage of time with epileptiform activity, localization of epileptiform activity, treatment history and treatment duration). Seizure frequency was used as the primary outcome variable, whereas three neuropsychological outcomes (IQ, psychomotor delay and educational delay) were used as secondary outcome variables. RESULTS: The children experienced a broad range of seizure types with the 'absence-like' complex partial seizure as the most commonly occurring seizure type. Almost half of the children of the study sample had a high seizure frequency. They experienced several seizures per month, week or even daily seizures. Also a substantial impact on neuropsychological outcome was observed. Mean full scale IQ was 87.7, mean academic delay was almost 1 school year and 27 children showed psychomotor delay on the Movement ABC. Only 'having more than one seizure type' showed a prognostic value for seizure frequency, and no factors were found to be correlated with the secondary outcome measures. None of the risk factors show a differential impact on seizure outcome. CONCLUSION: CLRE has a non-predictable course; clinical variability is high and prognosis in many children with CLRE is obscure. Having more than one seizure type was the only factor correlated to seizure frequency. Further longitudinal studies are needed.

Memory, emotional and vocational impairments before and after anterior temporal lobectomy for complex partial seizures.

PURPOSE: To assess the pre- and postsurgical frequency of memory, emotional, and vocational impairments in patients who underwent anterior temporal lobectomy (ATL), and to assess the relationship between emotional disturbance and memory abilities after ATL. METHODS: Retrospective analysis of data was performed on 90 patients with medically intractable complex partial seizures who underwent ATL between 1981 and 2003. Patients were evaluated an average of 5 months before surgery and 11.3 months after surgery. RESULTS: A moderate to high frequency of memory impairment (44.4%; verbal or nonverbal), emotional disturbance (38.9%) and unemployment (27.8%) existed in the same individuals both before and after surgery. There were small to moderate rates of new onset memory (18.9%), emotional (11.1%), and vocational (7.8%) difficulties after surgery often regardless of seizure control outcome. Patients who underwent left-ATL and had emotional disturbance after surgery had the lowest verbal memory test scores. CONCLUSIONS: Results highlight the importance of taking into account emotional status when assessing memory abilities after ATL. Results replicate the finding of moderate to high frequencies of memory impairment, emotional disturbance, and unemployment both before and after ATL. Results provide support for the rationale that cognitive, psychiatric and vocational interventions are indicated to mitigate the problems that exist before and persist after ATL.

Patient awareness of complex partial seizures.

PURPOSE: To assess self-awareness of complex partial seizures (CPSs) in unselected epilepsy patients through a thorough interview. METHODS: The study comprised 134 patients at our epilepsy clinic, whose CPSs had been documented by the patient's family members. We investigated the proportion and characteristics of patients unaware of their CPSs compared to those who were, and we monitored the evolution of unawareness of CPSs during the follow-up. RESULTS: Thirty-one (23%) patients were assigned to the unawareness group (complete, 23; incomplete, 8) and 103 (67%) patients to the awareness group. Patients in the unawareness group were older and had a later age of onset than patients in the awareness group. Interictal epileptiform discharges (IEDs) localized primarily to the temporal region and were more frequently detected in the unawareness group (94%) than the awareness group (55%). Bilateral independent IEDs were found more frequently in the unawareness group than in the awareness group (48% vs. 13%). The bilateral presence of lesions was also more frequent in the unawareness group than the awareness group (16.1% vs. 4.9%). Six (26%) of 23 patients with complete unawareness of their CPSs had experienced awareness of CPSs during the follow-up. Two of these patients even experienced the emergence of de novo aura. CONCLUSIONS: Our results indicate that a significant number of epilepsy patients are not aware of their CPSs. Unawareness of CPSs may be related to bitemporal dysfunction and a rapid and complete loss of consciousness caused by rapid spread of ictal discharges to the contralateral hemisphere in association with bilateral independent IEDs and bilateral presence of lesions.

Population-based survey on prevalence of adult patients with epilepsy in Taiwan (Keelung community-based integrated screening no. 12).

PURPOSE: To determine the prevalence rate and patterns of adult patients with epilepsy in Taiwan, we conducted a community-based neuroepidemiological survey. METHODS: Epilepsy was detected by neurologists using one-stage method. It was integrated into a community health screening service and performed from 1 January 2001 to 31 December 2001 in Keelung, a northern city in Taiwan. A total of 13,663 subjects aged 30 years or older participated in this survey. RESULTS: There were 52 patients with epilepsy in this study. Among them, 37 were patients with active epilepsy. The age-adjusted prevalence rate of active epilepsy above 30 years old was 2.77/1000 (to the 1980 US population) with the highest rate in subjects aged 40-49 years (4.0/1000). There was a trend of higher prevalence rate in male than in female. The most common seizure type was complex partial seizure (46.0%). Using one-stage detection method, we found nine (24.3%) patients with active epilepsy who had never been diagnosed before. Among the patients with active epilepsy, 35.1% were symptomatic cases. Head injury (13.5%) is the leading cause, followed by CNS infection (8.1%), stroke (5.4%) and perinatal insult (5.4%). The lifetime prevalence rate of epilepsy (including active epilepsy and epilepsy in remission) was 3.14/1000 for age above 30 years. CONCLUSIONS: Comparing to previous epilepsy survey in 1993, our results showed that the prevalence rate of epilepsy was rather stable over the past decade in northern Taiwan. Head injury is the leading cause responsible for active epilepsy. Improving public safety is an important public health issue which may help to reduce occurrence of epilepsy.

Complex partial seizure symptom endorsement in individuals with a history of head injury.

Research has shown that subjects at risk for cerebral dysfunction endorse more complex partial seizure symptoms than low risk controls. It has been suggested that seizure activity be regarded as occurring on a continuum of neurobehavioral dysfunction, rather than as a discrete syndrome. The present study assessed seizure symptom endorsement in individuals reporting a positive history of head injury. There were three groups of college student participants: head injury with loss of consciousness (LOC; n=31); head injury followed by a dazed period or alteration of consciousness (AOC; n=36); non-injured controls (NHI; n=60). In general, the LOC group reported greater frequency of symptomatology and a greater number of clinically significant symptoms (above the 90th percentile) than the NHI group, and a greater number of clinically significant symptoms than the AOC group. The AOC group did not differ from the NHI group on either variable. These results lend credence to the concept of a continuum representing an epilepsy spectrum disorder.

Long-term follow-up of patients with benign partial epilepsy in infancy.

PURPOSE: The aim of this study was to investigate the long-term outcome of children with benign partial epilepsy in infancy (BPEI). METHODS: A telephone-interview survey using a structured questionnaire was conducted with patients who were diagnosed as having possible BPEI at age 2 years and who were 8 years or older at the time of the survey. The data from 39 of 48 patients were available. The median age at the time of the survey was 11.3 years; 18 boys and 21 girls were included. RESULTS: Three patients had a recurrence of unprovoked seizure beyond age 2 years. Four patients had cognitive problems (mild mental retardation in three and Asperger syndrome in one). An association of paroxysmal kinesigenic choreoathetosis was observed in three patients, and another three had experienced seizures associated with mild gastroenteritis. Major behavioral problems were not recognized in any patients. Four patients were excluded from having definite BPEI at age 5 years, and another two were excluded for having definite BPEI at the last follow-up. Eventually, 33 of 39 patients were categorized as having definite BPEI beyond 8 years of age. CONCLUSIONS: A large majority of patients diagnosed as possibly having BPEI at age 2 years did not have a recurrence of unprovoked seizures and cognitive problems beyond 8 years of age.

Atypical language representation in patients with chronic seizure disorder and achievement deficits with magnetoencephalography.

PURPOSE: To characterize the relation between hemispheric asymmetries in language-specific brain activity and reading/spelling achievement by using magnetoencephalography (MEG). METHODS: Patients (n = 83) with medically intractable complex partial seizures of either left- or right-hemisphere origin were classified as having reading and/or spelling deficits (RS) or as not impaired (NI) by using standard achievement tests. All patients had undergone noninvasive functional mapping of receptive language cortex by using MEG as part of a preoperative seizure surgery evaluation. RESULTS: RS patients with left-hemisphere seizure onset exhibited relatively greater activation and earlier onset of late, language-specific MEG activity in posterior temporal and inferior parietal areas of the right as compared with the left hemisphere than did NI patients. These findings also were evident on an individual basis and were independent of global intellectual abilities. CONCLUSIONS: Reading and spelling achievement deficits in patients with complex partial seizures of left-hemisphere origin are associated with atypical language organization, possibly secondary to reorganization of language function to right-hemisphere areas that are not as efficient as homotopic areas in the left hemisphere in supporting reading and spelling functions.

Depression and anxiety disorders in pediatric epilepsy.

PURPOSE: This study examined affective disorders, anxiety disorders, and suicidality in children with epilepsy and their association with seizure-related, cognitive, linguistic, family history, social competence, and demographic variables. METHODS: A structured psychiatric interview, mood self-report scales, as well as cognitive and language testing were administered to 100 children with complex partial seizures (CPSs), 71 children with childhood absence epilepsy (CAE), and 93 normal children, aged 5 to 16 years. Parents provided behavioral information on each child through a structured psychiatric interview and behavior checklist. RESULTS: Significantly more patients had affective and anxiety disorder diagnoses (33%) as well as suicidal ideation (20%) than did the normal group, but none had made a suicide attempt. Anxiety disorder was the most frequent diagnosis among the patients with a diagnosis of affective or anxiety disorders, and combined affective/anxiety and disruptive disorder diagnoses, in those with suicidal ideation. Only 33% received some form of mental health service. Age, verbal IQ, school problems, and seizure type were related to the presence of a diagnosis of affective or anxiety disorder, and duration of illness, to suicidal ideation. CONCLUSIONS: These findings together with the high rate of unmet mental health underscore the importance of early detection and treatment of anxiety disorders and suicidal ideation children with CPSs and CAE.

Psychopathology and pediatric complex partial seizures: seizure-related, cognitive, and linguistic variables.

PURPOSE: This study examined the role of cognition, language, seizure-related, and demographic variables in the psychopathology of children with complex partial seizure disorder (CPS) of average intelligence. METHODS: One-hundred one CPS and 102 normal children, aged 5.1 to 16.9 years, had a structured psychiatric interview and cognitive and language testing. Parents provided demographic, perinatal, and seizure-related information, as well as behavioral information through the Child Behavior Checklist (CBCL) and a structured psychiatric interview about the child. RESULTS: Significantly more CPS patients had psychopathology, cognitive deficits, and linguistic deficits than did those in the normal group. Among the patients, Verbal IQ predicted the presence of a psychiatric diagnosis, as well as CBCL scores in the borderline/clinical range. Seizure, linguistic, and demographic variables were unrelated to psychopathology. The cognitive and linguistic deficits of the CPS group, however, were predicted by seizure factors (e.g., prolonged seizures/febrile convulsions; seizure frequency/number of antiepileptic drugs) and demographic factors (e.g., minority status). CONCLUSIONS: Because subtle verbal cognitive deficits predict behavioral disturbances in pediatric CPSs, the study's findings highlight the importance of assessing behavior, cognition, and language in these children. They also underscore the negative impact of prolonged seizures, febrile convulsions, seizure frequency, and antiepileptic drug polytherapy on cognition and language in pediatric CPSs.