Epidemiological and clinical study of West syndrome in Nagasaki Prefecture, Japan

To determine the occurrence, outcome, and prognostic factors of West syndrome (WS), we performed a retrospective epidemiological study of WS occurred in 47 children (26 boys and 21 girls) in Nagasaki prefecture during a recent 10-year period from 1989 to 1998. The incidence of WS was 3.1/10,000 live births. The mean age at onset of spasm is 6.3 months (range: 2 to 12 months). Thirty-nine patients (83%) had symptomatic WS, in which the prenatal causes (patients) were most frequent, followed by low-birth weight infants (patients), perinatal (patients) and postnatal (patients). The brain CT was performed in 37 patients, and revealed congenital brain malformations (9 patients), destructive brain disorders (12 patients), and no structural abnormalities (16 patients). The seizure outcome was worse in the symptomatic WS than in the cryptogenic WS; seizure/disappeared in 39% of the former and in 75% of the latter/developmental delay before the onset of WS, relapse of WS and persistence of seizures were associated with poor seizure outcomes. Among the remaining seizures at the time of this surveillance, a tonic seizure was most frequently observed, followed by the partial seizures. Lennox-Gastaut syndrome was observed in 2 patients only. Epileptic discharge in the latest interictal EEG were diffuse in 4.3%, focal or multifocal in 60.7%, and absent in 35%, suggesting that many patients with WS had cortical epileptogenic foci. The developmental outcome was very poor in both the symptomatic and cryptogenic WS. The mean DQ in all the patients was 25, and only 4 patients (11%) had a normal DQ (> 75). DQ was lower in patients with congenital brain malformations than in those with destructive brain disorders.     

Epidemiological and clinical studies of West syndrome in Miyagi Prefecture, Japan

We performed a retrospective epidemiological study of West syndrome (WS) in Miyagi prefecture over a 3-year period (2000 -2003). Twenty-two children (6 boys, 16 girls) developed WS. The incidence of WS was 3.4/10,000 live births. It agreed with the incidence of previous report in Japan. The mean age at onset of spasms was 6.5 months (range 1 - 17 months). Thirteen patients (59%) had symptomatic WS; of these, eight patients had prenatal causes and five had perinatal causes. There were no familial cases, although four patients had family history that included neurological diseases. Periventricular leukomalacia was found in four of nine patients with abnormal magnetic resonance imagings (MRI) . Early seizure outcome was good in twenty-one patients (95%). Six patients (29%) had seizure recurrence. There were no significant differences between symptomatic WS and cryptogenic WS in terms of seizure recurrence and persistent EEG abnormalities. The Five patients (20%) with developmental quotients (DQ) more than 70 were all cryptogenic WS, while patients with DQ less than 70 were significantly frequent in symptomatic WS as compared with cryptogenic WS.     

Evolutional Changes and Outcome of West Syndrome: Correlation with Magnetic Resonance Imaging Findings

Summary: The prognosis and evolutional changes of 77 patients with West syndrome (WS) were studied after patients were classified into four groups on the basis of their magnetic resonance imaging (MRI) findings: anomaly, perinatal injury, normal, and the other groups. The average age at onset of spasms was earliest in the patients with anomalies and latest in patients with normal MRI findings. Patients with normal MRI findings had the shortest duration of spasms, and patients with anomalies had the longest duration of spasms. Antecedent seizures were observed in 6 patients (3 patients with anomalies, 1 patient with normal MRI findings, and 2 patients with other abnormalities). Thirty-five patients had subsequent seizures. Patients with anomalies often had partial seizures and patients with perinatal injuries often had generalized seizures. Seizures were infrequent in patients with normal MRI findings. Developmental outcome was best in the patients with normal MRI findings and worst in patients with perinatal injuries. Various types of epileptic syndromes occurred subsequent to WS in patients with anomalies, although nonspecific symptomatic generalized epilepsy was common in patients with perinatal injuries. These results suggest that seizure prognosis, evolutional changes in seizures, and developmental outcome are different among the types of brain lesions.     

Profile of West syndrome in North Indian children

To study the profile of West syndrome (WS) in North Indian Children, 165 cases of WS were analyzed. Details of seizure semiology, prenatal and perinatal events, developmental milestones, treatment received, physical and neurological examination and investigations were recorded. The response of seizures to various therapeutic modalities and the final developmental status were taken as primary outcome variables. Analysis was done to find the factors influencing these outcome variables. The age of onset of infantile spasms ranged from 1 to 19 (mean 6.1 +/- 3.4) months. Age at presentation ranged from 1.5 months to 4.5 years (mean 14.7 +/- 11.4 months); 74% had flexor spasms. Other types of seizures were associated in 31 children. Antenatal problems and adverse perinatal events were reported in 26.7 and 59.4%, respectively. Developmental delay was recognized in 69.7% prior to and in 27.9% after onset of spasms. Microcephaly was seen in 72.7%. Interictal EEG showed hypsarrhythmia in 44; generalized spike and slow waves in 31% and burst suppression in 7%. Computed tomography scan done in 94 cases showed cerebral atrophy in 15%, infarcts in 8%, tubers in 7%, developmental malformations in 5%. Magnetic resonance imaging done in 77 cases showed periventricular T2WI white matter hyper intensities in 33.8% and cerebral atrophy in 21%. Prednisolone and ACTH were used in 57 and 35 cases, respectively. Complete control of seizures was seen in 49 and 46% cases. No significant difference in seizure control or developmental outcome was found in the two groups. Overall, 42.4, 30.9 and 16% children showed complete, partial and no control of seizures. After therapy, developmental improvement was seen in 55.8% and no change in 23.6% cases. The type of spasms had no correlation with the other parameters including etiology, seizure or developmental outcome. An early age of onset correlated with presence of antenatal problems (P < 0.05). Seizure control and developmental improvement correlated significantly (P < 0.005). Developmental outcome was better in cryptogenic as compared to symptomatic cases (P < 0.05). No other significant correlations were found. In India WS is often diagnosed late because of lack of awareness. Adverse perinatal events are important etiological factors. Non-affordability of ACTH and Vigabatrin prompts the use of prednisolone in most cases.     

ACTH therapy for Taiwanese children with West syndrome -- efficacy and impact on long-term prognosis

To study the efficacy of adrenocorticotrophic hormone (ACTH) in treating Taiwanese children with West syndrome (WS) and the impact on long-term prognosis, 66 patients with WS (54 symptomatic and 12 cryptogenic) were collected from 1987 to 1998 in a medical center in Taiwan. A total of 53 patients were enrolled in this study and treated with ACTH at the dosage of 2.5IU/kg daily for 2 weeks with gradual tapering in subsequent 6 weeks. Immediate responses, side effects of ACTH and long-term outcomes of the patients including seizure and developmental status were evaluated during the average follow-up period of 35.6 months. The spasm-free percentage after one or two courses of ACTH treatment was 77.4%. Nine (17%) patients encountered severe side effects such as major infections, which prompted us to stop ACTH. At the end of follow-up, 22 (41.5%) patients had intractable seizures but 25 (47.2%) patients remained seizure free with or without anticonvulsants. The ACTH-responders had a better chance of remaining seizure free (P<0.05). Regarding the long-term developmental outcome, 12 (22.6%) patients had normal or borderline development; two thirds of them belonged to the crytpogenic group. Six (11.3%) patients expired and 24 (45.3%) were severely retarded; all but one of them belonged to the symptomatic group. The prognosis of WS heavily relies on whether a patient is cryptogenic or symptomatic (P<0.001). Good response to therapy or short treatment lag did not favorably affect the developmental outcomes of the symptomatic cases. We conclude that the long-term outcomes of WS in Taiwan were generally poor despite of treatment. Only cryptogenic patients had favorable prognosis. For symptomatic patients, ACTH therapy may be used to control the spasms and decrease the incidence of subsequent epilepsy, but it will not improve developmental outcome. Considering a high percentage of severe side effects in our study, a lower dosage of ACTH with adequate therapeutic efficacy but less side effects should be considered for treating Taiwanese children with WS.     

Long-Term Course of West Syndrome Associated with Tuberous Sclerosis

Summary: We retrospectively analyzed the long-term clinical profiles of 47 patients with West syndrome (WS) associated with tuberous sclerosis (TS) the follow-up study for >10 years showed that WS developed into epilepsies of various types in all patients. Their final diagnoses were symptomatic generalized epilepsy (SGE: 29 patients, 62%), symptomatic partial epilepsy (PE: 14 patients, 30%), and undetermined epilepsy (UE: 4 patients, 8%). Eighty-five percent of all patients continued to show the same types of epilepsy that they had at their first examination; but in 15% the diagnosis had to be revised at the end of the follow-up period. The outcome of seizures was unfavorable for the SGE group. In the PE group, however, seizures were controlled in 9 (64%) of the 14 patients, and disturbances of daily activity were mild. In patients with WS associated with TS, the seizure outcome and social activity varied depending on the type of epilepsy or epileptic syndrome that developed from WS. The complicated clinical developments from WS with TS multiple cortical tubers related to epileptogenesis in addition to impairment of brain development.     

Clinical and pedigree study on familial cases of West syndrome in Japan.

Nationwide survey on familial cases of West syndrome (WS) in first- and second-degree relatives was conducted by mailing a questionnaire to 64 major university hospitals, children's hospitals, and epilepsy centers in Japan, and by review of the Japanese cases in the literatures. Thirty-four familial cases, 20 males and 14 females, were obtained in 15 families including one with five affected members in two generations and another with three affected male siblings including a half brother by a different father (X-linked WS). A mother and the child or children were involved in three families. Nine families had 21 cryptogenic cases and six families had 13 symptomatic cases, and the etiologies were same among the affected members in each family. Familial cases of WS have characteristic clinical features and genetic mechanisms. Age of onset, seizure types, electroencephalographic abnormalities, early seizure outcome, effective treatment, long-term seizure prognosis, and long-term developmental prognosis were concordant among the affected members in each family. Long-term seizure and developmental prognoses were far better than those in WS in general, with seizure-free rate of 82% and normal mental development rate of 44%. Poor prognosis was limited to specific symptomatic cases. Adrenocorticotropic hormone (ACTH) was a treatment of choice, and even in relapse of WS after ACTH therapy, the patients well responded to antiepileptic drugs. Specific inheritance pattern was difficult to imagine in the majority of the present cases, except for one family with X-linked WS and another family with five patients of maternal inheritance. These results are helpful for the treatment choice and prognostication of clinical course for familial cases of WS.     

Long-term follow-up study of West syndrome associated with tuberous sclerosis.

AIM: To analyze the evolution of seizures, electroencephalographic (EEG) features, seizure outcomes, and social outcomes, in order to formulate a basis for the long-term prognosis of West syndrome (WS) associated with tuberous sclerosis (TS). METHOD: The clinical profiles of 50 patients with TS, who presented with WS in infancy and had been monitored for more than 10 years after the initial examination, were analyzed. RESULTS: In 86% of the patients the seizure type did not change throughout the course of this study. However, 14% of patients had developed other types of seizures by the end of the follow-up period.The seizure outcome was unfavorable for patients with generalized seizures. Partial seizures were controlled in nine (64%) of the 14 patients, and normal or slightly subnormal intelligence accounted for seven of the 14 patients. CONCLUSIONS: Therefore, in patients with WS associated with TS, the seizure outcome and social activity depended on the type of seizure that developed during the course of this disorder. The outcome of patients with WS associated with TS seems to be better than that of WS in general.     

Prognosis of patients with seizures occurring in the first 2 years

The aim of this study is to determine the prognosis of patients with seizure onset from 1 to 24 months of age in respect to epilepsy, developmental outcome, and neurological status. It also aims to determine predictive factors regarding an unfavorable prognosis. Seventy-five patients were retrospectively analyzed. Univariate analysis revealed the following findings: (1) mental retardation at initial admission, abnormal neurological finding, infantile spasm, use of more than 1 antiepileptic drug, epileptic activity on electroencephalography (EEG) of neonatal seizure, and perinatal anoxia were significant risk factors with regard to developmental outcome; (2) mental retardation at initial admission, abnormal neurological finding, infantile spasm, use of more than 1 antiepileptic drug, epileptic activity on EEG, symptomatic etiology, history of neonatal seizure, and perinatal anoxia were significant risk factors regarding neurological status; and (3) mental retardation at initial admission, neurological abnormality, infantile spasm, use of more than 1 antiepileptic drug, epileptic activity on EEG, status epilepticus, symptomatic etiology, seizure frequency of more than once per week, history of perinatal anoxia, and neonatal seizure were significant risk factors regarding epilepsy prognosis. In addition, multivariate analysis revealed that neurological abnormality and use of more than 1 antiepileptic drug were significant for developmental outcome, that epileptic activity on EEG and use of more than 1 antiepileptic drug were significant for neurological status, and that perinatal anoxia, infantile spasm, and status epilepticus were significant for epilepsy prognosis. These findings suggest that neurological abnormality, use of more than 1 antiepileptic drug, infantile spasm, status epilepticus, and perinatal anoxia are unfavorable predictive risk factors regarding the prognosis of patients with seizures that have an onset from 1 to 24 months of age.     

Predictors of Intractable Childhood Epilepsy

Our study sought to identify early predictive factors of medically intractable childhood epilepsy. A cohort of epileptic children from the city of Mersin was retrospectively investigated. All patients received care from the same Department of Pediatric Neurology. The epileptic cohort was divided into a drug-responsive epilepsy group and an intractable epilepsy group. Intractable epilepsy is defined as continued seizures in children despite adequate therapy with two or more antiepileptic drugs for more than 18 months. Strong univariate association was observed between intractability and several factors: age of onset, high initial seizure frequency, symptomatic etiology, mixed seizure types, previous history of status epilepticus, febrile and neonatal seizures, mental and motor developmental delay, multiple seizures in 1 day, electroencephalogram abnormalities, magnetic resonance imaging findings, and specific epileptic syndromes. Logistic regression analysis revealed that a previous history of epilepticus status, abnormal electroencephalogram results, and multiple seizures in 1 day comprise independent predictors of medically intractable childhood epilepsy. We suggest that medical intractability in childhood epilepsy can be predicted by monitoring these factors. Along with early prediction, alternative therapies may be designed to provide patients better seizure control and quality of life.     

Cognitive function in preschool children after epilepsy surgery: rationale for early intervention

PURPOSE: The detrimental effect of frequent early seizures on the cognitive potential of children is a significant clinical issue. Epilepsy surgery in childhood offers a good prognosis for seizure control and improved developmental outcome. We studied the postoperative outcome and the developmental velocity after surgery and analyzed risk factors for developmental delay in 50 consecutive preschool children treated surgically for severe epilepsy at ages 3 to 7 years. METHODS: Pre- and postoperative developmental quotients (DQs) were analyzed with analysis of variance; stepwise linear regressions were performed on preoperative DQs and on a difference score between post- and preoperative DQs to determine risk factors for preoperative development and factors influencing postoperative development. RESULTS: Of the 50 patients, 70% were retarded, with IQ < 70; 16% were of average intelligence, with IQ ranging from 85 to 115. Age at seizure onset and extent of lesion were predictive variables for preoperative cognitive development. Six to 12 months after surgery (early postoperative phase), 66% were seizure free (Engel outcome class I), 26% had substantial to worthwhile seizure reduction (classes II and III), and 8% were unchanged (class IV). Forty-one (82%) children showed stable velocity of development; three children showed gains of >/=15 IQ points; three had developmental decline (loss of >/=10 IQ points), which was transient in two children; and three children moved from not assessable to assessable. At last follow-up (6 months to 10 years after surgery), 11 children showed IQ/DQ gains of >/=15 IQ points. Gains in IQ were observed only in seizure-free children and were stable over time. Shorter duration of epilepsy was significantly associated with a postoperative increase in DQ. CONCLUSIONS: (a) Substantial global mental delay is common in young children treated for epilepsy with surgery; (b) In most patients, postoperative development proceeded at a stable velocity; (c) Catch-up development may occur but only in seizure-free patients; (d) Substantial cognitive losses were noted in only one child. and (e) Early seizure control stabilized developmental velocity in this patient cohort.     

Convulsive Status Epilepticus in Children

Summary: Status epilepticus (SE) occurs most commonly in infancy and childhood. Children with prior neurological abnormalities are most susceptible. More than 90% of cases are convulsive and the majority are generalized. SE may occur in the setting of an acute illness, in patients with established epilepsy or as a first unprovoked seizure. The etiology can be classified as idiopathic, remote symptomatic, febrile, acute symptomatic, or associated with a progressive encephalopathy. The morbidity and mortality of status have dramatically declined in recent years. Overall mortality in recent pediatric series was 3–10%, with almost all fatalities associated with acute central nervous system insults or progressive neurologic disorders. Neurological sequelae in children with idiopathic or febrile status are rare. Neurologically normal children with SE as their first unprovoked seizure have the same risk of experiencing subsequent seizures of any type as children who present with a brief first seizure. The risk of recurrent episodes of convulsive SE approaches 50% in neurologically abnormal children but is very low in neurologically normal children. The favorable outcome of SE in children may be related to advances in therapy and to the resistance of the immature brain to damage from seizures.     

Cerebral embolism and epileptic seizures — the role of the embolic source

Objectives – To study the clinical outcome of patients with epileptic seizures due to ischemic stroke (IS) of cardiac or artery-to-artery embolism. Methods – Seizures due to IS of cardiac or artery-to-artery embolism are differentiated by clinical, neuroimaging and cardiovascular test data. Results – From 174 cases with supratentorial IS, 13 patients suffered from epileptic seizures due to cardiac embolism, 11 patients due to artery-to-artery embolism. The patients with cardiac IS showed an equal sex distribution and EEG abnormalities in 6 patients, the initial seizure occurred on average after 222 days (SD, ±69 days). Among the 11 patients with artery-to-artery embolic IS, there were 9 males and 2 females and EEG abnormalities in 10 patients. The initial seizure occurred on average after 447 days (SD, ±177 days). Conclusion – In seizures due to artery-to-artery embolism, there is a male preponderance and a higher incidence of EEG abnormalities, symptomatic seizures appear later compared to IS due to cardiac embolism.     

Seizure control and educational outcome in childhood-onset epilepsy.

Patients with epilepsy are more prone to have learning disabilities. This study investigated the therapeutic and educational outcome of 102 epileptic children. Analyzed data included age at onset, etiology, presence of underlying brain lesions, seizure type, and electroencephalographic (EEG) patterns. Cryptogenic seizures, remote symptomatic seizures, and underlying brain lesions were found in 29, 26, and 14 patients, respectively, whereas 47 patients had idiopathic seizures. Eighty-three patients achieved seizure control (46 remained seizure free), and 19 patients remained poorly controlled. Sixty-five patients were in regular schools, and 37 required special education (17 with mental retardation). Predictors for poor seizure control were remote symptomatic seizures, underlying brain lesions, and (when grouped together) hypsarrhythmia and mixed EEG patterns (P < .001). Predictors for special education needs were young age at onset, remote symptomatic seizures, underlying brain lesions, hypsarrhythmia and mixed EEG patterns, and poor seizure control (P < .001). We conclude that in childhood epilepsy, the need for special education is substantial and more common than treatment failure.     

Phenotypic and imaging features of FLNA-negative patients with bilateral periventricular nodular heterotopia and epilepsy

PurposePeriventricular nodular heterotopia (PVNH) is a malformation of cortical development due to impaired neuronal migration resulting in the formation of nodular masses of neurons and glial cells in close proximity to the ventricular walls. We report the clinical characteristics of the largest case series of FLNA-negative patients with seizures and bilateral periventricular heterotopia.MethodsParticipants were recruited through the Epilepsy Phenome/Genome Project (EPGP), a multicenter collaborative effort to collect detailed phenotypic data and DNA on a large number of individuals with epilepsy, including a cohort with symptomatic epilepsy related to PVNH. Included subjects had epilepsy, and MRI confirmed bilateral PVNH. Magnetic resonance imaging studies were visually and quantitatively reviewed to investigate the topographic extent of PVNH, symmetry, and laterality.Key findingsWe analyzed data on 71 patients with bilateral PVNH. The incidence of febrile seizures was 16.6%. There was at least one other family member with epilepsy in 36.9% of this population. Developmental delay was present in 21.8%. Focal onset seizures were the most common type of seizure presentation (79.3%). High heterotopia burden was strongly associated with female gender and trigonal nodular localization. There was no evidence for differences in brain volume between PVNH subjects and controls. No relationship was observed between heterotopic volume and gender, developmental delay, location of PVNH, ventricular or cerebellar abnormalities, laterality of seizure onset, age at seizure onset, and duration of epilepsy.SignificanceA direct correlation was observed between high heterotopia burden, female gender, and trigonal location in this large cohort of FLNA-negative bilateral PVNH patients with epilepsy. Quantitative MRI measurements indicated that this correlation is based on the diffuse nature of the heterotopic nodules rather than on the total volume of abnormal heterotopic tissue.     

Neonates with seizures: what predicts development?

Animal and human studies indicate that neonatal seizures are detrimental to the developing nervous system. This study addressed whether parameters of seizure severity and treatment were predictive of outcome and influenced the incidence of epilepsy. The outcome of babies with neonatal seizures was assessed based on follow-up examination, record review, and school performance. Epilepsy was assessed relative to developmental outcome and imaging abnormalities. There was no association between response to therapy and outcome. Neonates with mild or moderate seizure severity and decreasing severity over time, prior to anticonvulsant treatment, were more likely to have normal or moderately abnormal development than a severe outcome or death. Babies who had the highest seizure severity following treatment were more likely to have adverse outcomes. Those with normal imaging studies were more likely to have better outcome than those with diffuse severe abnormalities. Children with epilepsy were more likely to have abnormal development and imaging.     

Epilepsy in patients with severe motor and intellectual disabilities: a long-term follow-up

Long-term prognosis of epilepsy was investigated on 117 institutionalized patients with severe motor and intellectual disabilities, who were above 15 years of age in 1977, for a 20-year-period from 1977 to 1997. The incidence of epilepsy was 64.1% (75 patients), which was active in 28 patients (37.3%). The patients with the most severe psychomotor disabilities (bedridden and DQ < 20) showed the highest incidence of epilepsy (85.0%). Patients who died during the follow-up period showed higher incidence of active epilepsy (p < 0.01). During the follow-up of 94 surviving patients, persistence, relapse, and onset of seizures were frequent in patients with most severe intellectual disability, whereas those with less severe intellectual disability (20 < DQ < 35) were all seizure-free. Twenty-one patients had active epilepsy; symptomatic partial epilepsy in 17 (81.0%) and generalized epilepsy in 4 (19.0%). Notably, 5 of the 6 patients with persistent frequent seizures had age-dependent epileptic encephalopathy; persistent Lennox-Gastaut syndrome (LGS) (2 patients), severe epilepsy with multiple independent spike foci evolved from West syndrome (WS) and LGS (2 patients), and partial epilepsy with the history of LGS (1 patient).     

Febrile seizures in patients with complex partial seizures

Febrile seizures occurred in 14 of 155 (9%) out-patients with complex partial seizures. Twelve patients had prolonged or recurrent febrile seizures, convulsive status epilepticus or a transient postictal neurological deficit. Febrile seizures were associated with perinatal abnormalities, an earlier onset of epilepsy and with a poor seizure control. Recurrent febrile seizures or those with complicating features are associated with an unfavourable therapeutic outcome in adult patients with complex partial seizures.     

The perfusion defect seen with SPECT in West syndrome is not correlated with seizure prognosis or developmental outcome

We used interictal single photon emission computed tomography (SPECT) on 40 patients with West syndrome to determine whether cortical perfusion abnormalities are closely related to the development of West syndrome and whether they are correlated with the long-term seizure prognosis or the developmental outcome. Localized cortical perfusion abnormalities were seen in 24 patients (60%), while 15 patients (38%) were classified as normal. The remaining patient showed hyperperfusion of the basal ganglia bilaterally. Of 24 patients with localized perfusion abnormalities, unifocal cortical hypoperfusion was present in 11, multifocal hypoperfusion in 10, multiple cortical hypo- and hyperperfusion in one, hyperperfusion of the bilateral frontal cortices and brain stem in one, and focal hyperperfusion in the residual frontal cortex in one. For statistical analysis, we focused on 26 patients (cryptogenic; 10, symptomatic; 16), who were followed for more than 2 years after the onset of tonic spasms (mean 5.0 years). The results showed that focal cortical perfusion abnormalities were not correlated with the long-term seizure prognosis, the developmental outcome, or the response to ACTH therapy. In agreement with previous reports, the results of interictal SPECT suggested that focal cortical lesions play an important role in the development of West syndrome. However, statistical analysis showed that the existence of cortical dysfunction as defined by SPECT did not predict the seizure prognosis or the developmental outcome.     

Clinical predictors for outcome in infants with epilepsy

In this prospective study, the clinical parameters predictive for the outcome in infants with onset of epilepsy below 12 months of age (n = 60) were determined. At the end of the follow-up period, patients were included in a symptomatic or idiopathic group. In approximately 60% of all children, epilepsy could be controlled with standard antiepileptic monotherapy. The epilepsy was more difficult to control in the symptomatic group, reflected by the larger number of drug switches during follow-up. Seizure outcome was not influenced by age of onset, type of seizures, or electroencephalographic abnormalities at the epilepsy onset. Developmental outcome was significantly worse in the symptomatic group. In the idiopathic group, the control of epilepsy was the major developmental outcome factor, indicating a possible deleterious effect of seizures on brain development in that group.