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Background This article explores the role of initial treatment on control of spasms and other epilepsies at four years in children previously treated for West syndrome. Methods The Sri Lanka Infantile Spasm Study is a prospective clinical trial evaluating response to intra-muscular adrenocorticotropic hormone (ACTH) versus oral prednisolone. A previous report documented response through age 12 months. This article provides four-year follow-up data. Results At age four years, 65 of the original 97 were available for follow-up; another 13 had died, and 19 moved and could not be contacted. Of the 65 children, 37 (57%) continued to have seizures and 28 were seizure free. In the 37 children with ongoing epilepsy, 32.4% continued to have spasms, either alone or in combination with other seizure types. The epilepsy types seen in these children were focal epilepsy (59.4%), mixed focal and generalized epilepsy (24%), generalized epilepsy only (10.8%), and uncertain (5%). The majority of those still having epilepsy (66.7%) were controlled on medication. There was no significant difference in the rate of epilepsy or spasms or their control by medication between those treated with ACTH or oral prednisolone. Spasm control at day 14 did not influence the four-year spasm or epilepsy outcome. Conclusions A majority of children diagnosed with West syndrome continued to have seizures at age four years, although most were controlled on antiseizure medication. The long-term risk of developing epilepsy or its control was the same, regardless of whether ACTH or prednisolone was initially used as treatment.

Background Jeavons syndrome is an underreported epileptic syndrome characterized by eyelid myoclonia, eyelid closure-induced seizures or electroencephalography paroxysms, and photosensitivity. Drug-resistant epilepsy is common, but the prognostic factors and clinical course leading to drug resistance have not been well characterized. Methods We identified 30 patients who met the diagnostic criteria of Jeavons syndrome at a single institution between January 1, 2000 and December 15, 2016. Criteria for Jeavons syndrome included all of the following: (1) eyelid myoclonia with or without absences, (2) eye–closure-induced seizures or electroencephalography paroxysms, and (3) seizure onset after 12 months of age. We reviewed and described the epilepsy history, antiepileptic drug trials, and response to treatments. Results Mean age at seizure onset was 7.3 years, and 80% were female. Absence seizures (63%) and generalized tonic-clonic seizures (23%) were most common at onset. Diagnosis was delayed by an average of 9.6 years. After a median follow-up of two years, 80% of patients had drug resistant epilepsy and 70% experienced generalized tonic-clonic seizures. Generalized tonic-clonic seizures and seizure types other than absence seizures increased the risk of drug-resistant epilepsy (P values 0.049 and 0.03, respectively). Valproic acid, lamotrigine, ethosuximide, and levetiracetam were the most effective in reducing seizures by more than 50%. Conclusions The diagnosis of Jeavons syndrome is often delayed. Generalized tonic-clonic seizures and seizure types other than absence seizures may be predictors of drug-resistant epilepsy among patients with Jeavons syndrome.

Background The CACNA1H gene mutations encoding the α1H subunit of Cav3.2 T-type calcium channels have been associated with generalized epilepsy. Focal or multifocal epilepsy and systemic (immunologic and gastrointestinal) involvement associated with these mutations have not been described previously. We detail the clinical characteristics of five patients with CACNA1H mutations and expand its phenotypic spectrum. Methods A case series of five patients with pathogenic CACNA1H mutations was evaluated. The pathogenicity of the mutations was predicted by polymorphism phenotyping (Polyphen-2) and sorting-intolerant-from-tolerant analysis. Results Mean age of seizure onset was 8.2 ± 3.7 years. Three patients had de novo mutations in the CACNA1H gene, and two patients inherited the mutation from an asymptomatic parent. The patients experienced different types of seizures including absence, focal seizures without awareness, focal seizures with secondary generalization, and myoclonic, atonic, and generalized tonic-clonic seizures. Electroencephalography showed focal, multifocal, or generalized discharges. One patient had autism and global developmental delay. Two patients had failure to thrive and selective antibody deficiency. Conclusions CACNA1H mutations can be associated with susceptibility to develop generalized epilepsy and focal or multifocal epilepsy of varying severity. Phenotypic features involving other organ systems (immune, gastrointestinal) can occur in addition to epilepsy, developmental delay, and autism.

Background Genetic alterations are significant causes of epilepsy syndromes; especially early-onset epileptic encephalopathies and voltage-gated sodium channelopathies are among the best described. Mutations in the SCN2A subunit of voltage-gated sodium channels have been associated with benign familial neonatal-infantile seizures, generalized epilepsy febrile seizures plus, and an early-onset infantile epileptic encephalopathy. Method We describe two infants with medically refractory seizures due to a de novo SCN2A mutation. Results The first child responded to intravenous lidocaine with significant reduction in seizure frequency and was successfully transitioned to enteral mexiletine. Mexiletine was subsequently used in a second infant with reduction in seizure frequency. Conclusion Class 1b antiarrhythmic agents, lidocaine and mexiletine, may be useful in infants with medically refractory early infantile epileptic encephalopathy secondary to mutations in SCN2A .

Rufinamide is a new antiepileptic drug recently approved as adjunctive treatment for generalized seizures in Lennox-Gastaut syndrome. We undertook a retrospective analysis of 77 patients with refractory epilepsy and receiving rufinamide to evaluate the drug's efficacy, tolerability, safety, and dosing schedules. It appeared efficacious in diverse epilepsy syndromes, with the highest responder rate in focal cryptogenic epilepsies (81.1% of patients with >50% response rate), and in diverse seizure types, with the highest responder rate in tonic/atonic and partial seizures (48.6% and 46.7% of patients with >50% response rate, respectively). Rufinamide was well tolerated: only 13% of patients developed side effects necessitating drug withdrawal. These findings suggest that rufinamide may possess good efficacy and tolerability, and that its efficacy may extend to epilepsy syndromes beyond Lennox-Gastaut, including both partial and generalized epilepsy syndromes.

This study investigated whether the T-type calcium channel gene CACNA1I causes susceptibility in the Chinese Han population to childhood absence epilepsy, a form of idiopathic generalized seizure disorder. For this investigation, we searched for mutations in the 35 exons and exon-intron boundaries of the CACNA1I gene in 50 Han Chinese patients with childhood absence epilepsy. Seventeen single nucleotide polymorphisms were identified in the 35 exons. Using six single nucleotide polymorphisms as markers, the allele and genotype distributions of all of the identified single nucleotide polymorphisms were examined; there was no significant difference between the childhood absence epilepsy cases and the control groups. Thus, we do not consider the CACNA1I gene to be an important susceptibility gene for childhood absence epilepsy in the Chinese Han population.

In this study, oxcarbazepine was began as monotherapy to evaluate the efficacy and safety of the drug. Forty-two patients (19 females, 23 males) with partial or generalized epilepsy more than 4 years of age were included (mean age, 11.9 +/- 3.4 years). The mean age at epilepsy onset 8.9 4 years. Complete blood count, liver function tests, electrolytes, lipid levels, electrocardiography, electroencephalography, and magnetic resonance imaging were performed in all patients. Oxcarbazepine dose was begun at 10 mg/kg/day twice daily and increased to 30 mg/kg/day at the end of the second week. Patients with inadequate seizure control even with the dose of 45 mg/kg/day or intolerable side effects were excluded. Intolerable headache and leukopenia led to discontinuation of the drug in two patients. At the sixth month, 35 of the patients (87.5%) were seizure free (91.7% of the generalized epilepsy patients and 81.2% of the partial epilepsy patients). The most frequent tolerable side effect was drowsiness in 12 patients. As a result, we found oxcarbazepine safe and effective in children with either generalized or partial epilepsy. (C) 2003 by Elsevier Science Inc. All rights reserved.

Therapeutic options for intractable epilepsy include new and investigational antiepileptic drugs, ketogenic diet, epilepsy surgery, and, now, vagus nerve stimulation, which is approved by the U.S. Food and Drug Administration for the treatment of refractory partial seizures in adolescents and adults. The exact mechanisms of action are unknown. Although the use of vagus nerve stimulation in children has increased, including those younger than 12 years of age or those with generalized epilepsy, there has been no large controlled pediatric study to date. The identification of favorable prognostic indicators, especially in children, would be useful. Preliminary results suggest that children with Lennox-Gastaut syndrome may have a favorable response, with improvement in both seizure control and global evaluation scores. Improved global evaluation scores have occurred even without an associated improvement in seizure control.

The effectiveness of lamotrigine as a monotherapeutic agent for a variety of pediatric epilepsies was reviewed retrospectively. Children were categorized as having focal vs generalized epilepsy and according to whether they were antiepileptic drug naive or drug exposed. Data collected included dosages, side effects, length of follow-up, number of prior drugs, and treatment response. Treatment was considered successful if the patient was seizure free for 6 months or more. Eighty-three children were identified (average age = 8.7 years); 43 had focal epilepsy, 32 had generalized epilepsy, and eight were not classified. Twenty-nine patients were classified as having specific syndromes. Fourteen patients were drug naive. The median follow-up period was 8 months (mean = 8.5). Overall, 45% were seizure free, 44% with focal epilepsy and 36% with generalized epilepsy. All children with juvenile myoclonic epilepsy and benign rolandic epilepsy of childhood were seizure free, although not all had been treated for at least 6 months. One third of drug-naive patients were seizure free. Rash was the most common side effect and was reported in five patients (6%); two patients discontinued the drug. None had Stevens-Johnson syndrome. One quarter of children experienced nonquantifiable improvements, namely increased alertness and improved behavior regardless of seizure control. Lamotrigine is effective as a monotherapeutic agent in children for both focal and generalized epilepsies. Side effects are relatively uncommon. Lamotrigine may be an effective firstline agent.

Myoclonic epilepsy associated with ragged-red fibers is one of the mitochondrial encephalomyopathies. Pathogenic mitochondrial DNA mutations have been identified in the mitochondrial transfer RNA (tRNA)(Lys) at positions 8344 and 8356. Characteristics of myoclonic epilepsy associated with ragged-red fibers include myoclonic epilepsy, generalized epilepsy, hearing loss, exercise intolerance, lactic acidosis, and ragged-red fibers. The elevated lactate level is one of the most important symptoms needed to make a diagnosis of mitochondrial encephalomyopathy. In the present case, however, myoclonic epilepsy was associated with ragged-red fibers but without increased lactate levels. Therefore, myoclonic epilepsy associated with ragged-red fibers should be suspected in a patient who has myoclonic epilepsy that is difficult to control with antiepileptic medications and who has other symptoms of mitochondrial disease, such as mental retardation, even if the patient's lactate level is normal.

A 7-year-old female with benign childhood epilepsy with centrotemporal spikes developed epileptic negative myoclonus (ENM) seizures during carbamazepine (CBZ) treatment. She had experienced nocturnal partial seizures since 5 years of age. Interictal electroencephalography demonstrated typical rolandic discharges. Valproate was first initiated at 6 years of age, but the seizures were uncontrollable. Carbamazepine was added and valproate withdrawn. The frequency of partial seizures did not decrease. Moreover, she had brief episodes of tone loss in each or both arms and eye blinking several weeks after CBZ introduction. Unilateral loss of arm tone corresponded to spike-and-wave discharges in the contralateral centrotemporal region, and a loss of tone in arms was associated with bilateral synchronous discharges. Eye blinking was also related to bilateral synchronous discharges and classified as a myoclonic seizure. The ENM and myoclonic seizures disappeared soon after CBZ withdrawal. Therefore the authors concluded that CBZ induced the ENM and myoclonic seizures in this patient. CBZ sometimes induces generalized seizures in the treatment of partial epilepsy and generalized epilepsy. CBZ-induced ENM seizures should be considered when a brief lapse of tone appears during CBZ treatment.

Childhood-onset epilepsy is a common disorder. There is insufficient data on the distribution of epilepsy syndromes in the population and their effect on long-term prognosis. This report presents the data on epilepsy syndromes in a childhood-onset epilepsy cohort. A population-based active-prevalence cohort of all children under 16 years of age residing in the catchment area of Turku University Hospital with active epilepsy between 1961 and 1964 was monitored prospectively until 1992. Etiology, seizure type, and epilepsy syndromes were classified according to the recent guidelines of the International League Against Epilepsy. The etiology of seizures was idiopathic in 28%, cryptogenic in 22%, and remote symptomatic in 50%. Seizures were classifiable in 235 patients (96%) and included 157 (64%) with partial seizures and 88 (36%) with generalized seizures. Epilepsy syndromes could be classified in 223 patients (91%) and included 152 (62%) localization-related syndromes, 61 (25%) generalized epilepsy syndromes, and 10 (4%) with syndromes not determined as being focal or generalized. Prognosis for both survival and remission was dependent on etiology and on the specific epilepsy syndrome. The authors conclude that the International League Against Epilepsy guidelines can be successfully applied to a population-based cohort with childhood-onset epilepsy. Accurate classification of syndromes is important because in many cases long-term outcome may be largely determined by the underlying epilepsy syndrome.

Background Childhood absence epilepsy is an age-dependent, idiopathic, generalized epilepsy with a characteristic seizure appearance. The disorder is likely to be multifactorial, resulting from interactions between genetic and acquired factors, but the debate is still open. We review recent studies on different aspects of childhood absence epilepsy and also to describe new concepts. Methods Data for this review were identified using Medline and PubMed survey to locate studies dealing with childhood absence epilepsy. Searches included articles published between 1924 and 2013. Results The diagnosis comprises predominant and associated seizure types associated with other clinical and electroencephalographic characteristics. Many studies have challenged the prevailing concepts, particularly with respect to the pathophysiological mechanisms underlying the electroencephalographic seizure discharges. Childhood absence epilepsy fits the definition of system epilepsy as a condition resulting from the persisting susceptibility of the thalamocortical system as a whole to generate seizures. This syndrome, if properly defined using strict diagnostic criteria, has a good prognosis. In some cases, it may affect multiple cognitive functions determining risk for academic and functional difficulties; the detection of children at risk allows tailored interventions. Childhood absence epilepsy is usually treated with ethosuximide, valproate, lamotrigine, or levetiracetam, but the most efficacious and tolerable initial empirical treatment has not been well defined. Conclusions We review recent studies and new concepts on the electroclinical features and pathophysiological findings of childhood absence epilepsy in order to highlight areas of consensus as well as areas of uncertainty that indicate directions for future research.

Background The relation between epileptic seizures and vaccinations is sometimes debated. In the present work, the impact of vaccination on seizure onset and clinical outcome of SCN1A mutation-positive patients is addressed. Methods Seventy-two patients diagnosed with Dravet syndrome or generalized epilepsy with febrile seizure plus, carrying SCN1A mutations or not, were included. Details on vaccination type, temporal relationship between vaccination and seizure occurrence, seizure type at onset and during development, cognitive functioning, and vaccination completion was obtained by reviewing clinical records. Patients were divided into two groups based on the temporal window between vaccination and seizure onset (proximate group: 48 hours). Results Vaccination-related seizures occurred in 25% of patients with SCN1A mutation and 18% of patients without the mutation (no significant difference). The proximate group showed an earlier age at seizure onset and a higher frequency of status epilepticus during development than did the distant group. No other significant differences were found. Subsequent vaccinations did not significantly alter the evolution of the disease. Conclusions Results from this relatively small series provide evidence that vaccinations do not significantly affect clinical and cognitive evolution of Dravet syndrome and generalized epilepsy with febrile seizure plus patients even if they carry SCN1A mutations.

The combination of lamotrigine and valproate/divalproex sodium has been shown to be effective in the treatment of refractory epilepsy. This study aims to evaluate the pharmacologic properties of using this combination in a pediatric population refractory to antiepileptic drugs, with an extended follow-up. We studied a group of 51 patients, ranging from 4 to 16 years of age. Sixteen patients (31.4%) had generalized epilepsy and 35 (69.6%) had focal epilepsy. The combination was effective in 39 patients (76.5%) in the first year of follow-up and in 36 patients (70.6%) in the second year, with a reduction in drop attacks observed in 22 (88.5%). Adverse effects included rash, leading to discontinuation in four patients (7.8%). Slower introduction of lamotrigine minimizes adverse effects, thereby improving quality of life and adherence to treatment. In addition, therapeutic efficacy is maintained with lower doses of lamotrigine, even after the first year of treatment.

Chromosomal abnormalities involving deletions and duplications are known to cause severe developmental disorders, including mental retardation, dysmorphism, and seizures, in children. As the technique of array-based comparative genomic hybridization is being applied more frequently in the diagnostic evaluation of children with developmental disorders, novel pathologic chromosomal abnormalities are being identified. We report the case of a 9-year-old girl with a history of pervasive developmental disorder, growth delay, mild dysmorphic features, and intractable primary generalized epilepsy with a de novo microdeletion of approximately 0.73-0.94 Mb within chromosome 15q26.1. A much larger (5 Mb) but overlapping microdeletion has been previously reported in a 30-month-old child with similar phenotype including intractable myoclonic epilepsy, growth delay, and dysmorphic features. This leads us to propose that a potential candidate gene or genes within the deleted region involved in the pathogenesis of some forms of generalized intractable epilepsy, previously considered to be idiopathic.

Cloxazolam has been used mainly as an anxiolytic agent. The present study was designed to evaluate the effectiveness of cloxazolam as an add-on antiepileptic drug in patients with intractable epilepsy. A total of 32 patients with intractable epilepsy were treated with cloxazolam: 13 with generalized epilepsy, 15 with focal epilepsy, and 4 with undetermined type of epilepsy. The initial effects were evaluated at 1 month after reaching a maintenance dose (0.3-0.5 mg/kg). The long-term effects were investigated at 2 years after reaching a maintenance dose. With cloxazolam, seizure frequency was reduced by ≥ 50% in 19/32 patients (59%) during initial therapy and in 6/23 patients (26%) during long-term therapy. Two became seizure free throughout the cloxazolam therapy. During initial therapy, 8/32 patients (25%) developed 11 episodes of adverse events during the initial therapy, including 5 with drowsiness, 3 with hyperactivity, 2 with irritability, and 1 with loss of appetite. During long-term therapy, 2/23 (9%) developed drowsiness. The mean dose of cloxazolam in patients with an effective response was 0.30 ± 0.18 mg/kg for initial therapy and 0.26 ± 0.20 mg/kg for long-term therapy. Seven of the 19 effective responders developed tolerance (37%). Cloxazolam is an effective and safe antiepileptic drug for intractable epilepsy.

This study investigated sleep and sleep spindle parameters in children with primary generalized spike-and-wave discharges (untreated primary generalized group, nine patients; treated primary generalized group, six patients) and compared these with an age- and sex-matched nonepileptic control group (n = 47). In the untreated primary generalized group, stage 2 onset was significantly shorter, with less spindles in stage 2. In the last stage 2 period of the night, significantly less fast frequency spindles were observed, indicating abnormal dynamics of sleep architecture. In the treated group, sleep patterns were comparable to that of the control group. The data indicate sleep architecture dysfunctions in children with generalized spike-and-wave discharges. These dysfunctions could account for the frequently encountered sleep problems in children with primary generalized epilepsy.

The aim of this study was to evaluate the efficacy and safety of zonisamide monotherapy in a cohort of children and adolescents with various types of epilepsy. Retrospective review of charts of our institution from 2001 through 2004 identified 69 children (19 males and 50 females, mean age 13.2 years) with epilepsy on zonisamide monotherapy. Seizure count and side effect profile were maintained during therapy. Sixty-one percent had idiopathic generalized epilepsy, 4% symptomatic generalized epilepsy, and 35% partial-onset epilepsy. Zonisamide was the first-line and second-line monotherapy for 32% and 68% of patients, respectively. The mean duration of follow-up on treatment was 22 months (range 3-48 months). The overall efficacy of zonisamide was 75.4% (or = 50% seizure frequency reduction: good responders). Sixty-seven percent of good responders became seizure-free. Seventy-nine percent of patients with partial epilepsy and 71% with generalized epilepsy were good responders, of whom 79% and 63% were free of seizure, respectively. Eighteen (26%) patients developed side effects: weight loss (9), cognitive impairment (3), sleepiness (3), dizziness (2), and decreased appetite (1). In seven patients (10%), zonisamide had to be discontinued: four due to side effects and three because of poor seizure control. Zonisamide was demonstrated to be effective as monotherapy in children with epilepsy.

Steroids are often an effective treatment for the West's syndrome. There have been few reports of steroid use in children with epilepsy outside the first year of life. I report my experience with prednisone for the treatment of older children with intractable epilepsy. Twenty-eight children (17 boys, 11 girls) aged 18 months to 10 years with intractable epilepsy were studied. Prednisone 1 mg/kg/day for 12 weeks (6 weeks daily and 6 weeks alternate therapy) was prescribed in addition to their regular antiepileptic medications. The parents kept seizure diaries, and the patients were regularly assessed for seizure frequency and side effects. The follow-up period was for 1 to 5 years. Thirteen patients (46%) became seizure free on prednisone and another 18 (40%) had a significant decrease in seizure frequency. Five patients (19%) had no change in seizure frequency. The best outcomes were seen in the absence group in which six out of seven patients became seizure free and in the Lennox-Gastaut syndrome group in which seven out of 10 became seizure free. Side effects were uncommon and included weight gain in five patients and aggression in four patients. Prednisone therapy is a safe and effective adjunctive treatment for epilepsy. It should be considered as an alternative treatment for older children with intractable generalized epilepsy who have failed conventional antiepileptic therapy.

Three hundred eight patients with childhood and adolescent epilepsy were examined to clarify the incidence of epileptic discharges on initial and follow-up electroencephalograms. Epileptic discharges were found in 75.6% patients on the initial electroencephalogram, which is higher than figures previously reported for adults. The cumulative incidence of epileptic discharges was 92.3% by the third electroencephalogram recording. However, in 17.1% patients with nonspecific idiopathic generalized epilepsy, no epileptic discharges were found even after three electroencephalogram recordings. The incidence of epileptic discharges in patients with generalized epilepsy (84.3%) was significantly higher than in patients with localization-related epilepsy (71.6%). The incidence of epileptic discharges in patients with partial seizures was lower than those in patients with generalized seizures. The incidence of epileptic discharges was low in the 0- to 3-year-old and 15- to 20-year-old groups, and high in the 3- to 12-year-old groups. In the positive epileptic discharge patients, 38.8% of electroencephalograms were abnormal only during the waking or sleeping portion of the recordings. Knowing the incidence of epileptic discharges for each type of epilepsy will be useful in planning further electroencephalogram research and performing electroencephalograms in the clinical setting.

In this report the types of epilepsy syndromes seen in children in a tertiary referral center in Beirut, Lebanon were studied and the importance of consanguinity and family history in the occurrence of these syndromes was investigated. Records of 230 pediatric patients evaluated during a 1-year period with the diagnosis of single seizure, febrile seizure, or epilepsy were reviewed. Each patient was classified according to the International League Against Epilepsy classification. The occurrence of consanguinity, of family history of febrile seizures or epilepsy, and of other variables was noted. Thirty-six percent of patients were diagnosed with localization-related epilepsy, 21.7% with generalized epilepsy, 11.7% with undetermined generalized or focal, and 24.3% with special syndromes. Twelve percent of patients were diagnosed with idiopathic, 15.1% with symptomatic, and 30.3% with cryptogenic epilepsies. Consanguinity was more common in patients with symptomatic and cryptogenic epilepsies than in patients with idiopathic epilepsies or with incidental seizures (P < 0.05). Family history of epilepsy was more common in patients with symptomatic, cryptogenic, and idiopathic epilepsies than in patients with incidental seizures (P < 0.05). Family history of febrile seizures but not consanguinity was more common in patients with febrile seizures (P < 0.05). We conclude that genetic factors are important not only in idiopathic epilepsies and febrile seizures but also in cryptogenic and symptomatic epilepsies.

A 3-year-old girl is presented with benign familial neonatal convulsions. She had seizures during the neonatal period and at age 3 months. Seizure manifestations and ictal electroencephalography indicated that she had not experienced generalized seizures but partial seizures, although international classifications of epilepsy define benign familial neonatal convulsions as generalized epilepsy.

West syndrome, although classified as a generalized epilepsy, is associated with localized cerebral lesions in some cases. However, similar localized cortical abnormalities usually can result in partial epilepsy, instead of West syndrome. We performed this study to determine the additional factors that result in West syndrome instead of partial epilepsy in patients with localized cerebral lesions. We reviewed the pathologic features, topographic localization, and side of unilaterally defined cerebral lesions in relation to the seizure types in 39 epileptic patients, including five patients who presented with West syndrome. The lesions of all five patients with West syndrome involved the temporal or occipital lobes (or both). Among the nine with an occipital lesion, four had West syndrome. In the 19 with a temporal lesion, three had West syndrome. However, in the 16 patients with a frontal lesion, none exhibited epileptic spasms. In four of the five with West syndrome the lesions were on the right side; 23 had lesions on the right, 16 had them on the left. Temporo-occipital lesions and lesions on the right were related to the genesis of West syndrome, which would be in close correlation with normal brain maturation.

The role of lamotrigine (LTG) in childhood epilepsy is emerging. We evaluated the efficacy and adverse effects of LTG in an open, prospective study of 56 children with generalized epilepsies. Six (11%) children became seizure-free, and 24 (43%) had greater than 50% reduction in seizure frequency. LTG was effective against a broad range of generalized seizure types. Three of 15 patients with Lennox-Gastaut syndrome achieved complete seizure control and eight demonstrated 50 to 99% improvement in seizure control. Increase in seizures (7) and rash (5) were the most common side effects. After valproate was discontinued, LTG therapy was resumed, with no recurrence of rash in any patient. This study suggests that LTG may be a useful drug in the treatment of generalized epilepsies in children.