Problems surrounding absence seizures]

The diagnosis, treatment, and prognosis of childhood absence epilepsy (CAE) and juvenile absence epilepsy (JAE) were reviewed with reference to 94 patients with typical absence seizures (82 with CAE, 12 with JAE) and the literature. The patients were separated into two groups based on clinical features, age at onset of seizures, and EEG findings. There has been much discussion on the age that represents the borderline between CAE and JAE. My view is that JAE begins with puberty, i.e. at around 10 years old. The treatment of choice for CAE is valproic acid (VPA). If the seizures are not controlled with VPA, add-on therapy with ethosuximide is recommended. For patients who respond poorly to these drugs, clonazepam in often effective. Lamotrigine, which is not yet commercially available in Japan, is effective when combined with VPA. As for school performance, some patients showed excellent results. However, about half of them performed weakly. Patients followed beyond 20 years were free of absence seizures in both groups, but suffered from GTCS with occurred sporadically in CAE as well as in JAE. The social prognosis in CAE and JAE may not be as good as we believed it to be.     

Long-term prognosis for childhood and juvenile absence epilepsy

Abstract. Purpose: To analyse prognostic factors for long term seizure remission in patients with childhood (CAE) and juvenile absence epilepsy (JAE). Study design: A retrospective analysis of a hospital based prevalence cohort. Methods: The cohort consisted of 163 patients (104 females, 59 males) treated at the Universitätsklinik für Neurologie, Innsbruck between 1970 and 1997. All had absences according to the ILAE classification. Follow up was in 1999 to 2000. We assessed multiple clinical and EEG factors as predictors of outcome and compared a classification according to the predominant pattern of seizure recurrence (pyknoleptic, PA or non pyknoleptic absence, NPA) with the ILAE classification with respect to prognosis. Results: The mean age at seizure onset was 10.9 years (range, 3 to 27); age at follow up was 36.7 years (range, 13 to 81); duration of follow up was 25.8 years (range, 3 to 69). Sixty four patients (39 %) had CAE and 64 (39 %) JAE, while 35 (22%) had typical absences but could not be clearly defined as either CAE or JAE, and were therefore called the overlap group. Patients with JAE or patients in the overlap group developed more often generalized tonic clonic seizures (GTCS) (p<0.001) and myoclonic attacks (p<0.05) during the course of the disease. At follow up 36 (56 %) of patients with CAE, 40 (62%) with JAE and 19 (54 %) of the overlap group were seizure free for at least two years (p=ns). When classified according to the predominant absence pattern at seizure onset 42 (51%) patients with PA and 53 (65%) with NPA were in remission (p=ns). In a stepwise binary logistic regression analysis the pattern of absence (PA or NPA) together with the later development of additional seizure types (myoclonias or GTCS), but not the CAE/JAE classification was predictive for long term lack of remission with a correct prediction of 66% of all patients. Conclusion: Only 58% of patients with absences were in remission after a long term follow up. CAE and JAE are closely related syndromes with large overlap of the age of onset. A classification according to the predominant seizure pattern at onset, together with later development of myoclonic attacks or GTCS is useful in predicting seizure remission in absence epilepsies.     

Absence Epilepsies

Summary: Individuals fulfilling diagnostic criteria for childhood absence epilepsy (CAE) and juvenile absence epilepsy (JAE) were selected from a large group of patients who were born between 1945 and 1973 and had presented with absence seizures (AS). Updated data allowed an analysis of 52 patients with CAE and of 62 patients with JAE aged ≥20 years. In CAE, complete control was achieved in 90% of patients (95%, AS only; 77%, AS + generalized tonic-clonic seizures, GTCS). Only 16% of patients with an onset <9 years had developed GTCS. In JAE, complete control was achieved in 37% of patients (47%, AS; 37%, GTCS). These figures support the validity of the International Classification of Epilepsy (ICE). Stricter diagnostic criteria are discussed.     

Long-term seizure outcome in patients with juvenile absence epilepsy; a retrospective study in a tertiary referral center

PurposeThe study aim was to evaluate pharmacotherapy effects and long-term seizure outcomes in patients with juvenile absence epilepsy (JAE) during a five-year follow-up period. The secondary aim was to identify factors from patient history and determine their influence on seizure control.MethodWe retrospectively studied 46 patients with JAE in the period between 2006 and 2011. The age at seizure onset, onset seizure type, family history of epilepsy, status epilepticus in history, medication history, and the rate of seizure control were studied.ResultsThere were 30 females (65.2%) and 16 males (34.8%) in the study. The mean age at seizure onset was 12.9 ± 5.6 years (ranged from 3 to 28 years). In 30 patients (65.2%), seizure onset was with absences, in 15 patients (32.6%) with generalized tonic-clonic seizure (GTCS), and in 1 patient (2.2%) with absence status. In 43 patients (93.5%), GTCS occurred in the course of the disease. Family history for epilepsy was positive in 10 patients (21.7%). In the five-year follow-up period, seizure freedom (Group 1) was achieved in 7 patients (15.2%). In total, 22 patients (47.8%) were classified into the groups involving very poor seizure control and antiepileptic drug resistance (Groups 5 and 6). The mean number of antiepileptic drugs (AEDs) used in the course of the disease in appropriate therapeutic doses was 3.8 ± 2.3 (1–10 AEDs).ConclusionThe study results show that almost half of JAE patients have poor seizure control with a high rate of pharmacoresistance. The outcome of JAE can be very uncertain.     

Factors influencing clinical features of absence seizures

Purpose: The clinical features of absence seizures in idiopathic generalized epilepsy have been held to be syndrome‐specific. This hypothesis is central to many aspects of epilepsy research yet has not been critically assessed. We examined whether specific factors such as epilepsy syndrome, age, and state determine the features of absence seizures. Methods: Children with newly presenting absence seizures were studied using video electroencephalography (EEG) recording. We analyzed whether a child's epilepsy syndrome, age, state of arousal, and provocation influenced specific clinical features of their absence seizures: duration, eyelid movements, eye opening, and level of awareness during the seizure. Results: Seizures (509) were evaluated in 70 children with the following syndromes: Childhood absence epilepsy (CAE), 37; CAE plus photoparoxysmal response (PPR), 10; juvenile absence epilepsy (JAE), 8; juvenile myoclonic epilepsy (JME), 6; unclassified, 9. Seizure duration was associated with epilepsy syndrome as children with JME had shorter seizures than in other syndromes, independent of age. Age independently influences level of awareness and eye opening. Arousal or provocation affected all features except level of awareness. Specific factors unique to the child independently influenced all features; the nature of these factors has not been identified. Discussion: The view that the clinical features of absence seizures have syndrome‐specific patterns is not supported by critical analysis. We show that confounding variables profoundly affect clinical features and that syndromes also show marked variation. Variation in clinical features of absence seizures results from a complex interaction of many factors that are likely to be genetically and environmentally determined.     

High-power,frontal-dominant ripples in absence status epilepticus during childhood

ObjectiveAbsence status epilepticus (ASE) is a form of non-convulsive status epilepticus characterized by ongoing or intermittent epileptic activity accompanied by behavioral and cognitive changes. Herein, we assessed high-frequency oscillations in the ripple band in patients with ASE and typical absence seizures.MethodsWe enrolled five patients with ASE, 26 patients with childhood absence epilepsy (CAE), and 15 patients with juvenile absence epilepsy (JAE). We performed time–frequency analysis of electroencephalogram data for ictal absence seizures at each electrode to assess the high frequency activity (HFA) rate, peak frequency, and peak power.ResultsThe average HFA rates were 60.7%, 20.8%, and 12.9% in ASE, CAE, and JAE patients, respectively. The average peak frequencies were 126.4 Hz, 120.9 Hz, and 126.1 Hz in ASE, CAE, and JAE patients, respectively. The average peak power values were 2,388.8 μV², 120.9 μV², and 126.1 μV² in ASE, CAE, and JAE patients, respectively, and all epilepsy groups exhibited frontal-dominant ripple distribution.ConclusionASE patients presented higher power and frontal dominant ripples of absence seizure, compared to CAE and JAE patients.SignificanceFuture studies should utilize scalp-recorded ripples as a biomarker of absence epilepsy. This may aid in the development of novel treatment strategies for ASE.     

The phenotypic spectrum related to the human epilepsy susceptibility gene “EJM1”

Linkage studies of families ascertained through patients with juvenile myoclonic epilepsy (JME) suggest that an HLA-linked susceptibility gene on chromosome 6, designated “EJM1,” predisposes to a group of idiopathic generalized epilepsies (IGEs) comprising JME, juvenile absence epilepsy (JAE), childhood absence epilepsies (CAE), and epilepsies with generalized tonic–clonic seizures (GTCS). To explore the EJM1-related phenotypic spectrum, we conducted linkage studies with HLA-DQα restriction fragment length polymorphisms in 44 families ascertained through patients with CAE or JAE. Our results for the entire group of families provide evidence against a major susceptibility locus for idiopathic absence epilepsies and broader spectra of IGEs in the HLA region. Lod scores less than – 2 were obtained for a region from 10 cM up to 23 cM on either side of the HLA-DQα locus, depending on the assumed trait model. Suggestive evidence for linkage was found only for a subgroup of families with JME patients assuming an autosomal dominant mode of inheritance with 70% penetrance. A maximum lod score was obtained when family members with JME, JAE, CAE, and idiopathic GTCS were included into the affection status. Our results demonstrate that (1) the genetic susceptibility to idiopathic absence epilepsies and broader spectra of IGEs is heterogeneous, (2) the gene effect of EJM1 depends on the familial genetic background, and (3) EJM1 confers genetic susceptibility to idiopathic absence epilepsies and broader spectra of IGEs in the presence of family members with JME.     

Felbamate for refractory absence seizures

We treated 10 patients (aged 5–37 years) with medically refractory absence seizures with felbamate (FBM). Average duration of absence seizures was 16.2 years. All patients continued to have absence seizures at maximally tolerated doses of valproic acid and/or ethosuximide. At maximal FBM dosage (45 mg/kg/day or 3,600 mg/day), there was at least a 65% reduction in absence seizure frequency in eight patients; in four, there was a > 95% reduction. Mean duration of FBM therapy is 16.2 months (range, 5–31 months). Nine patients remain on FBM. Two patients are on FBM monotherapy; seven others are on FBM and one or two other antiepileptic drugs. FBM was discontinued in one patient who developed severe insomnia followed by a return to baseline absence seizure frequency after an initial good response, and an increase in generalized tonic-clonic seizures (GTCS). Three other patients also had a history of GTCS; in one there has been no significant change, whereas two have not had a GTCS during FBM therapy. Eight patients continue to have a clinically meaningful reduced absence seizure frequency, and eight patients reported fewer adverse effects with their current regimen including FBM. Tachyphylaxis to the antiabsence efficacy was observed in four cases. These preliminary findings suggest that FBM is useful in treating absence seizures.     

EEG features of absence seizures in idiopathic generalized epilepsy: Impact of syndrome, age, and state

Purpose:   Factors influencing the electroencephalography (EEG) features of absence seizures in newly presenting children with idiopathic generalized epilepsy (IGE) have not been rigorously studied. We examined how specific factors such as state, provocation, age, and epilepsy syndrome affect the EEG features of absence seizures.
Methods:   Children with untreated absence seizures were studied using video-EEG recording. The influence of state of arousal, provocation (hyperventilation, photic stimulation), age, and epilepsy syndrome on specific EEG features was analyzed.
Results:   Five hundred nine seizures were evaluated in 70 children with the following syndromes: childhood absence epilepsy (CAE) 37, CAE+ photoparoxysmal response (PPR) 10, juvenile absence epilepsy (JAE) 8, juvenile myoclonic epilepsy (JME) 6, and unclassified 9. Polyspikes occurred in all syndromes but were more common in JME. They were brought out by drowsiness and sleep in fragments of generalized spike and wave (GSW). Polyspikes were more likely to occur during photic stimulation, but were not influenced by age independently. GSW was more likely to be disorganized in JME than JAE, and in JAE than CAE. Increasing age and levels of arousal were more likely to result in organized GSW. Factors specific to each child independently influenced EEG features; the nature of these factors has not been identified.
Discussion:   The EEG features of absence seizures are influenced by a complex interaction of age, epilepsy syndrome, level of arousal, provoking factors, and other intrinsic factors. Epilepsy syndrome alone cannot predict specific features of GSW; however, JME is more frequently associated with polyspikes and disorganization of the paroxysm.     

Myoclonic jerks are commonly associated with absence seizures in early‐onset absence epilepsy

Aim. Typical absence seizures are observed in various epilepsy syndromes, however, few series have focused on early‐onset absence epilepsy (EOAE). We aimed to evaluate the occurrence of this seizure type in children under 4 years of age in order to evaluate their electroclinical characteristics and outcome. Methods. We retrospectively studied (2006–2014) the electroclinical features of children with normal development and typical absence seizures starting before the age of 4 (with available pre‐treatment video‐EEG). Results. Nine patients were included. Among them, eight patients had rhythmic myoclonic jerks involving the muscles of the upper face (eyebrows and eyelids) or neck, present from the onset to the end of the typical absence discharge. The myoclonia were synchronous with spike‐wave complexes. One patient with GLUT‐1 deficiency was refractory to antiepileptic polytherapy. The other eight became seizure‐free; five with one antiepileptic drug and three with a combination of two drugs. The treatment was successfully withdrawn in five of the six patients who achieved two years of seizure freedom. None of them exhibited any other seizure type. Four of the eight patients with normal schooling required some support. We observed a positive correlation between the duration of absence seizure and the age of the patient at examination. Conclusion. Most of the patients under four years with only typical absence seizures had EOAE, and the motor symptoms may represent a distinctive age‐related feature of EOAE. Further investigations are required to better correlate the role of brain maturation with the duration of the absence. [Published with video sequence on www.epilepticdisorders.com ]     

Cognitive phenotype of juvenile absence epilepsy: An investigation of patients and unaffected siblings

Objective

The cognitive profile of juvenile absence epilepsy (JAE) remains largely uncharacterized. This study aimed to: (1) elucidate the neuropsychological profile of JAE; (2) identify familial cognitive traits by investigating unaffected JAE siblings; (3) establish the clinical meaningfulness of JAE-associated cognitive traits; (4) determine whether cognitive traits across the idiopathic generalized epilepsy (IGE) spectrum are shared or syndrome-specific, by comparing JAE to juvenile myoclonic epilepsy (JME); and (5) identify relationships between cognitive abilities and clinical characteristics.

Methods

We investigated 123 participants—23 patients with JAE, 16 unaffected siblings of JAE patients, 45 healthy controls, and 39 patients with JME—who underwent a comprehensive neuropsychological test battery including measures within four cognitive domains: attention/psychomotor speed, language, memory, and executive function. We correlated clinical measures with cognitive performance data to decode effects of age at onset and duration of epilepsy.

Results

Cognitive performance in individuals with JAE was reduced compared to controls across attention/psychomotor speed, language, and executive function domains; those with ongoing seizures additionally showed lower memory scores. Patients with JAE and their unaffected siblings had similar language impairment compared to controls. Individuals with JME had worse response inhibition than those with JAE. Across all patients, those with older age at onset had better attention/psychomotor speed performance.

Significance

JAE is associated with wide-ranging cognitive difficulties that encompass domains reliant on frontal lobe processing, including language, attention, and executive function. JAE siblings share impairment with patients on linguistic measures, indicative of a familial trait. Executive function subdomains may be differentially affected across the IGE spectrum. Cognitive abilities are detrimentally modulated by an early age at seizure onset.     

Atypical childhood absence epilepsy with preceding or simultaneous generalized tonic clonic seizures

Objective

Although the current diagnostic criteria for childhood absence epilepsy (CAE) do not specifically exclude children with generalized tonic clonic seizures (GTCSs) occurring before or early in the course of the active absence seizures, some workers have suggested that they should be interpreted as doing so. The aim of this study was to compare the clinical features between children with typical CAE and those with atypical CAE with preceding or simultaneous episodes of GTCS (atypical CAE-GTCS).

Methods

A total of 11 patients with atypical CAE-GTCS and 30 with typical CAE were identified by using the current CAE criteria. Their clinical data, including age, sex, family history of epilepsy, personal history of febrile convulsions, onset ages of absences and GTCS, treatment, and outcome were statistically analyzed.

Results

The two groups had the same mean onset age of absences (6 years), and their seizure outcome was comparably favorable in terms of both absences and GTCS. There was no significant difference in other clinical data except for the onset age of GTCS between the groups.

Conclusion

These findings show the similarity in the main clinical features between the groups, suggesting that some patients with atypical CAE-GTCS may have a variant form of CAE with early onset of GTCS.     

Seizure outcome in patients with juvenile absence epilepsy

The purpose of this study was to identify seizure outcome and factors potentially predictive for seizure outcome in patients with juvenile absence epilepsy (JAE). In this case–control study all patients with JAE were recruited at the outpatient epilepsy clinic at Shiraz University of Medical Sciences from 2008 till 2012. All patients had to be under the care of the epileptologist for at least 18 months. We divided the patients into two groups: patients who were seizure free in the last 12 months of their follow-up period and those who had any seizures. During the study period, 2750 patients with epilepsy were registered at our epilepsy clinic; 641 patients (23.3 %) had idiopathic generalized epilepsy (IGE). Among patients with IGE, 81 patients (12.6 %) were diagnosed as having JAE and of these, 33 patients (20 women and 13 men) were eligible to enter into the study. Ten patients (30.3 %) were seizure free in the last 12 months of their follow-up and 23 (69.6 %) patients reported at least one seizure of any type. We could not identify any factor to be associated with seizure outcome in these patients. All studies in the literature suffer from small number of patients; so does our study. Besides, they used different methodologies. A large multicenter study is required to explore the variables that predict seizure outcome in patients with juvenile absence epilepsy. This is particularly needed to provide an appropriate counselling for patients and their families and also to formulate better individualized treatment plans for the patients.     

Side‐to‐side axial movements

Aim. To study new semiological signs which help distinguish between primary and secondarily generalised tonic‐clonic seizures (GTCS). Methods. We retrospectively studied 86 GTCS, 13 primary and 73 secondary, in 58 patients who underwent video‐EEG (vEEG) evaluation in our epilepsy monitoring unit. Eleven patients had generalised epilepsy and 47 focal epilepsy. Two expert epileptologists, blinded to diagnosis, examined the vEEGs independently for the presence of five semiological signs. Results. Asymmetry of limb movements in clonic phase, side‐to‐side axial movements, and asymmetric seizure termination occurred more frequently (p<0.05) in secondary GTCS compared to primary GTCS. Combining asymmetry of limb movements in clonic phase and side‐to‐side axial movements provided the greatest value in differentiating secondary GTCS from primary GTCS. Conclusion. Careful examination of GTCS seizure semiology can help differentiate primary from secondary GTCS. The semiological sign of side‐to‐side axial movements, which has not previously been studied in this context, may add to existing literature of semiological signs and be of value for the evaluation of surgical patients in the epilepsy monitoring unit. In the out‐patient setting, a clear history of these signs may help guide drug treatment choices. [Published with video sequences].     

A study of idiopathic generalised epilepsy in an Irish population.

Idiopathic generalised epilepsy (IGE) is subdivided into syndromes based on clinical and EEG features. PURPOSE: The aim of this study was to characterise all cases of IGE with supportive EEG abnormalities in terms of gender differences, seizure types reported, IGE syndromes, family history of epilepsy and EEG findings. We also calculated the limited duration prevalence of IGE in our cohort. METHODS: Data on abnormal EEGs were collected retrospectively from two EEG databases at two tertiary referral centres for neurology. Clinical information was obtained from EEG request forms, standardised EEG questionnaires and medical notes of patients. RESULTS: two hundred twenty-three patients met our inclusion criteria, 89 (39.9%) male and 134 (60.1%) females. Tonic clonic seizures were the most common seizure type reported, 162 (72.65%) having a generalised tonic clonic seizure (GTCS) at some time. IGE with GTCS only (EGTCSA) was the most common syndrome in our cohort being present in 94 patients (34 male, 60 female), with 42 (15 male, 27 female) patients diagnosed with Juvenile myoclonic epilepsy (JME), 23 (9 male, 14 female) with Juvenile absence epilepsy (JAE) and 20 (9 male, 11 female) with childhood absence epilepsy (CAE). EEG studies in all patients showed generalised epileptiform activity. CONCLUSIONS: More women than men were diagnosed with generalised epilepsy. Tonic clonic seizures were the most common seizure type reported. EGTCSA was the most frequent syndrome seen. Gender differences were evident for JAE and JME as previously reported and for EGTCSA, which was not reported to date, and reached statistical significance for EGTCA and JME.     

Focal clinical and electroencephalographic features in patients with juvenile myoclonic epilepsy

Jayalakshmi SS, Srinivasa Rao B, Sailaja S. Focal clinical and electroencephalographic features in patients with juvenile myoclonic epilepsy.
Acta Neurol Scand: 2010: 122: 115–123.
© 2009 The Authors Journal compilation © 2009 Blackwell Munksgaard. Objective – To identify prevalence and factors associated with occurrence of focal clinical and electroencephalogram (EEG) abnormalities in patients with juvenile myoclonic epilepsy (JME). Materials and methods – Clinical asymmetries in the seizures and focal EEG abnormalities were analyzed in 266 patients with JME. Results – All the patients had myoclonic jerks (MJ) and generalized tonic‐clonic seizures (GTCS); 56 (21%) had absence seizures. Asymmetry in clinical seizures was reported in 45 (16.9%) and focal EEG abnormalities were noted in 92 (45.5%) patients. Amplitude asymmetry or focal onset of generalized discharges was noted in 41 (44.6%) and independent focal EEG abnormalities in 30 (32.6%) patients. A statistically significant association was seen with the presence of GTCS and MJ (P = 0.007), a family history of epilepsy (P = 0.001) and drug resistance (P = 0.04) and the occurrence of focal EEG abnormalities. Conclusion – Patients with JME showed focal clinical and EEG features. These features should not be misinterpreted as indicative of partial epilepsy.     

Serum serotonin levels in patients with epileptic seizures

Profound cardiovascular and/or respiratory dysfunction is part of the terminal cascade in sudden unexpected death in epilepsy (SUDEP). Central control of ventilation is mediated by brainstem rhythm generators, which are influenced by a variety of inputs, many of which use the modulatory neurotransmitter serotonin to mediate important inputs for breathing. The aim of this study was to investigate epileptic seizure–induced changes in serum serotonin levels and whether there are potential implications for SUDEP. Forty‐one epileptic patients were pooled into 2 groups based on seizure type as (1) generalized tonic–clonic seizures (GTCS) of genetic generalized epilepsy and focal to bilateral tonic–clonic seizures (FBTCS; n = 19) and (2) focal seizures (n = 26) based on clinical signs using surface video‐electroencephalography. Postictal serotonin levels were statistically significantly higher after GTCS and FBTCS compared to interictal levels (P = .002) but not focal seizures (P = .941). The change in serotonin (postictal‐interictal) was inversely associated with a shorter duration of tonic phase of generalized seizures. The interictal serotonin level was inversely associated with a shorter period of postictal generalized electroencephalographic suppression. These data suggest that peripheral serum serotonin levels may play a role in seizure features and earlier postseizure recovery; these findings merit further study.     

Lack of association between synapsin II (SYN2) gene polymorphism and susceptibility epilepsy: a case-control study and meta-analysis

Objective: The SYN2 rs3773364 A>G polymorphism has been proposed to be involved in susceptibility to epilepsy, but research results have been inconclusive. The aim of this study was to investigate the association between the SYN2 rs3773364 A>G polymorphism and susceptibility against epilepsy in a case–control study and a meta‐analysis. Methods: The SYN2 rs3773364 A>G polymorphism was successfully genotyped in 1182 samples (618 epilepsy patients) of Chinese, Indian, and Malay ethnicities. Meta‐analysis of the related studies, including this case–control study, was performed under alternative genetic models. Results: Data from the case–control study indicated no allelic and genotypic association of this locus with susceptibility to epilepsy in the tri‐ethnic Malaysian population. Similar finding was obtained by stratified analysis by epilepsy syndrome for idiopathic epilepsy. These results were verified by meta‐analysis of the related pooled data. Conclusions: Our study indicated that SYN2 rs3773364 A>G polymorphism is not a risk factor for susceptibility to epilepsy. Synapse 2011. © 2011 Wiley‐Liss, Inc.     

Prognostic Significance of Failure of the Initial Antiepileptic Drug in Children with Absence Epilepsy

PURPOSE: In children with childhood absence epilepsy (CAE) and juvenile absence epilepsy (JAE), to determine the impact of failure of initial antiepileptic drug (AED) for lack of efficacy in eventual seizure control and long-term remission of epilepsy. METHODS: Centralized EEG records for the province of Nova Scotia allowed identification of all children seen with CAE or JAE between 1977 and 1985. Information regarding success or failure of initial AED in fully controlling seizures and long-term seizure control and remission of epilepsy was collected by patient questionnaire and chart review. RESULTS: Eighty-six of 92 eligible patients were followed up (75 CAE, 11 JAE). Initial AED treatment was successful in 52 (60%) of 86. Success tended to be greater for valproate (VPA) than for other AEDs (p = 0.07), and lower if generalized tonic-clonic or myoclonic seizures coexisted (p < 0.004 and p < 0.03). Terminal remission was more likely if the initial AED was successful than if it had failed (69% vs. 41%; p < 0.02). Compared with those in whom the initial AED was successful, subjects whose initial AED had failed were more likely to progress to juvenile myoclonic epilepsy (JME) at last follow-up (32% vs. 10%; p < 0.02) and to develop intractable epilepsy (17% vs. 2%; p < 0.04). CONCLUSIONS: Initial AED was successful in 60% of children with AE. If the first AED failed, the outcome was less favorable, with a lower rate of terminal remission and a higher rate of progression to JME and intractable epilepsy.