The Absence Epilepsies

Four syndromes comprise the absence epilepsies. Each is classically associated with the absence seizure, although other syndromes also have absence attacks as part of their repertoire. The most common syndrome is childhood absence epilepsy; it usually occurs in the age range of 6–7 years. The absence seizures may occur many times daily, and the electroencephalographic (EEG) characteristics are the most typical of the absence epilepsies. The second form of absence epilepsies is juvenile absence epilepsy; it begins near puberty and may represent a continuum from the childhood form. Myoclonic seizures are more common than in the childhood form, and the spike-wave discharges in the EEG are often faster than that seen in childhood absence epilepsy. The third form of absence epilepsy is juvenile myoclonic epilepsy, characterized especially by myoclonic jerks in the morning; these attacks occasionally progress to generalized tonic-clonic seizures. The final form of absence epilepsy is epilepsy with myoclonic absences, a rare disorder with a specific form of absence seizures. The absence seizure itself is observed to a greater or lesser extent in all of these syndromes. This seizure is a curious event, and its causes are poorly explained by current knowledge of the fundamental mechanisms of the epilepsies. Although the etiology of the absence seizure at a biochemical level is unknown, some studies suggest that certain low-threshold calcium ion currents (T currents), which are partially controlled by GABA-B mechanisms, may activate burst firing of thalamic neurons, initiating an absence seizure. The evidence of a genetic predisposition for the absence epilepsies is overwhelming. Although the nature of the genetic abnormality remains unclear, promising investigations may soon reveal the location and the nature of the genetic defect.     

Update on the epidemiology and prognosis of pediatric epilepsy

Epilepsy is among the most common serious neurologic disorders in childhood. Epidemiologic studies over the past few decades have greatly increased current knowledge of the incidence and prognosis of seizures. Newer epidemiologic studies have used population- or community-based cohorts, and careful attention has been given to etiology and specific epilepsy syndromes, the two most important factors affecting prognosis. Risk of epilepsy is highest in patients with an associated serious neurologic abnormality, such as mental retardation or cerebral palsy. More than two thirds of patients with childhood-onset epilepsy ultimately achieve remission. Of those attaining remission on medications, approximately 70% remain seizure free when medications are discontinued. Mortality is increased in patients with epilepsy, but the increased mortality risk in childhood-onset epilepsy is primarily seen in patients with neurologic abnormalities or intractable epilepsy. Although long-term seizure outcomes are generally favorable, childhood-onset epilepsy is associated with adverse long-term psychosocial outcomes, even in patients attaining remission. This review summarizes recent data on the epidemiology and prognosis of pediatric epilepsy.     

Relation of photosensitivity to epileptic syndromes.

Photosensitivity is the most common mode of seizure precipitation. It is age-related, more frequent in females, and most often found in generalised epilepsies. Little is known about its relation to individual epileptic syndromes. This study on 1062 epileptic patients who had 4007 split screen video EEG investigations revealed that the relation to generalised epilepsy is even more close than generally believed. Versive seizures with visual hallucinations was the only focal seizure type related to photosensitivity. Of the syndromes of generalised epilepsy, only childhood absence epilepsy, juvenile myoclonic epilepsy, and epilepsy with grand mal on awakening were related to photosensitivity. The closest correlation was with juvenile myoclonic epilepsy. This is confirmed by a relation to the poly-spike wave pattern, and by an increase of myoclonic seizures by intermittent light stimuli. No relation was found with early childhood syndromes of generalised epilepsy, or generalised tonic-clonic seizures in the evening, or, most remarkably, with juvenile absence epilepsy. In generalised epilepsies with onset around puberty, photosensitivity could thus act as a pathoplastic factor. The female preponderance in both childhood absences and photosensitivity could be due to the same unknown factor.     

Predicting antiepileptic drug response in children with epilepsy

In clinical practice, after diagnosis and when treatment has begun, it is important to predict as soon as possible which children will become seizure-free and which are likely to develop medically intractable seizures. This article summarizes factors predicting seizure remission in childhood-onset epilepsy treated with antiepileptic drugs (AEDs). Sustained seizure remission can be expected in over 90% of idiopathic epilepsies of childhood and in neurologically normal children with epilepsy having infrequent seizures showing early remission after starting treatment with AEDs. Even in the presence of symptomatic etiology of epilepsy--focal seizures and syndromes; high seizure frequency prior to or during treatment; seizure clustering; and poor or delayed response to first adequate drug therapy--up to 60% of children with treated epilepsy are able to enter long-term remission. However, remission can be expected in only 30% or less of those with catastrophic epilepsies of childhood.     

Two epileptic syndromes, one brain: childhood absence epilepsy and benign childhood epilepsy with centrotemporal spikes

Childhood absence epilepsy (CAE) and benign childhood epilepsy with centrotemporal spikes (BCECTS), or benign rolandic epilepsy (BRE), are the most common forms of childhood epilepsy. CAE and BCECTS are well-known and clearly defined syndromes; although they are strongly dissimilar in terms of their pathophysiology, these functional epileptic disturbances share many features such as similar age at onset, overall good prognosis, and inheritance factors. Few reports are available on the concomitance of CAE and BCECTS in the same patients or the later occurrence of generalized epilepsy in patients with a history of partial epilepsy. In most cases described in the literature, absence seizures always started after the onset of benign focal epilepsy but the contrary has never occurred yet. We describe two patients affected by idiopathic generalized epileptic syndrome with typical absences, who experienced BCECTS after remission of seizures and normalization of EEG recordings. While the coexistence of different seizure types within an epileptic syndrome is not uncommon, the occurrence of childhood absence and BCECTS in the same child appears to be extremely rare, and this extraordinary event supports the hypothesis that CAE and BCECTS are two distinct epileptic conditions. However, recent interesting observations in animal models suggest that BCECTS and CAE could be pathophysiologically related and that genetic links could play a large role.     

Cognitive and behavioral outcomes of epileptic syndromes: implications for education and clinical practice

The educational and social progress of a child with epilepsy depends not only on seizure control but also on cognitive and behavioral factors. The various epilepsy syndromes of childhood and adolescence differ greatly in terms of cognitive and behavioral outcome. A high proportion of babies who have West syndrome and children who have Dravet syndrome (severe myoclonic epilepsy in infancy) will have long-term cognitive and behavioral problems. The Lennox-Gastaut syndrome also often has a poor prognosis in this regard. Children with the Landau-Kleffner syndrome have a variable prognosis, some regain speech and others have permanent speech impairment. Benign childhood epilepsy with centrotemporal spikes is now recognised as lying on a spectrum with the Landau-Kleffner syndrome: mild cases have few if any cognitive or behavioral problems but others may have quite severe difficulties. People with juvenile myoclonic epilepsy may have characteristics suggesting frontal lobe impairment. The educational and social impairments associated with the epilepsy syndromes of childhood and adolescence are of major importance but they have been the subject of remarkably few well-performed studies. The impairments are not always necessarily permanent and it seems highly likely that the cognitive and behavioural outcome of at least some of these syndromes can be influenced greatly by early effective treatment with either antiepileptic medication or surgery.     

Cognitive and Behavioral Outcomes of Epileptic Syndromes: Implications for Education and Clinical Practice

Summary:  The educational and social progress of a child with epilepsy depends not only on seizure control but also on cognitive and behavioral factors. The various epilepsy syndromes of childhood and adolescence differ greatly in terms of cognitive and behavioral outcome. A high proportion of babies who have West syndrome and children who have Dravet syndrome (severe myoclonic epilepsy in infancy) will have long-term cognitive and behavioral problems. The Lennox-Gastaut syndrome also often has a poor prognosis in this regard. Children with the Landau-Kleffner syndrome have a variable prognosis, some regain speech and others have permanent speech impairment. Benign childhood epilepsy with centrotemporal spikes is now recognised as lying on a spectrum with the Landau-Kleffner syndrome: mild cases have few if any cognitive or behavioral problems but others may have quite severe difficulties. People with juvenile myoclonic epilepsy may have characteristics suggesting frontal lobe impairment. The educational and social impairments associated with the epilepsy syndromes of childhood and adolescence are of major importance but they have been the subject of remarkably few well-performed studies. The impairments are not always necessarily permanent and it seems highly likely that the cognitive and behavioural outcome of at least some of these syndromes can be influenced greatly by early effective treatment with either antiepileptic medication or surgery.     

Childhood epilepsy: what is the evidence for what we think and what we do?

This article reviews the strength of the evidence that underlies the current approach to the management of childhood epilepsy. The authors reviewed published, peer-reviewed English literature accessed through PubMed and Cochrane reviews with evidence rated as Class 1 (strongest) to Class 4 (weakest). There is considerable inaccuracy in the diagnosis of seizures and epilepsy syndromes. Sound information supports the consensus that the diagnosis of epilepsy should await two unprovoked seizures. Population-based studies indicate that remission from childhood onset epilepsy occurs in at least 50% of children. It is easier to predict a good seizure outcome than a poor one. Absence of concomitant neurologic handicap and onset before about 12 years of age are the most consistent predictors of remission. Intractability is poorly defined and difficult to predict until several antiepilepsy drugs have been used and failed to control the seizures. Most epilepsy syndrome diagnoses do not yield an accurate prognosis. Social outcome appears unsatisfactory in about 50% of cases without intellectual handicap. Death is rare in childhood epilepsy. Those without severe neurologic handicaps have the same mortality as the general population. We identified only 27 published randomized trials of antiepilepsy drugs in children that compare the efficacy of antiepilepsy drugs, offer treatment of syndromes currently without successful treatment, or have negative effects. There is a pressing need for better definitions of seizures and epilepsy syndromes. The causes of poor social outcome are unclear. Intractability needs a clear definition and randomized trials comparing treatment regimes are sadly lacking.     

Clinical correlations of occipital epileptiform discharges in children.

OBJECTIVES: To determine the frequency of different causes of occipital epileptiform discharges (OEDs) in children and to analyze the EEG features that help predict epilepsy type and prognosis. METHODS: We identified children with OEDs in the absence of other focal discharges using an EEG database at our center; the presence of generalized spike-wave discharges (GSW) or slowing was not an exclusion criterion. Diagnosis, neurologic status, seizure semiology, and seizure remission status were recorded. RESULTS: Of 90 patients with OEDs, 50 (56%) had symptomatic seizures (18 with cerebral palsy, 11 with cerebral dysgenesis, 8 with genetic abnormalities); 31 (34%) had idiopathic seizures, including 6 with benign childhood epilepsy with occipital paroxysms (BCEOP), 8 (9%) had no seizures; and 1 (1%) had febrile seizures. Only two reported ictal visual symptoms. Eighty-seven percent with background slowing had symptomatic seizures, and 87% with normal backgrounds had idiopathic seizures (p < 0.001). Of 72 children with seizures and adequate follow-up, 28 of 45 (62%) with a normal background experienced seizure remission compared with 10 of 27 (37%) with background slowing (p = 0.04). Twenty of 81 patients with epilepsy had GSW. Twelve (60%) of the 20 GSW-positive patients had idiopathic epilepsy compared with 19 of 61 (31%) without GSW (p = 0.02). CONCLUSIONS: Most epilepsy in referred children with OEDs is symptomatic; syndromes such as BCEOP are rare. Visual ictal symptoms are rare. The presence of GSW or a normal background rhythm correlates with idiopathic seizures and a better prognosis.     

The Progression of Epilepsy

Summary:  Prognosis for seizure control and cognitive development varies considerably among syndromes. Several factors may interact to influence outcome of an epilepsy including a causative etiology, ictal and interictal discharges, seizure-related trauma or systemic perturbations, and antiepileptic drug (AED) effects. Clinical evidence convincingly supporting Gowers' hypothesis that seizures beget seizures is lacking. Short-term seizure suppression by early treatment does not appear to influence long-term prognosis.
Malignant epilepsy syndromes usually begin in infancy or childhood, have a high seizure frequency, resist the initial AED, and are often associated with progressive cognitive dysfunction. Prompt management of some severe epilepsy syndromes may lessen cognitive decline. However, aggressive AEDs therapy must be balanced against the potential for cognitive side effects, particularly if multiple AEDs are used.
Several experimental paradigms closely parallel human TLE as both have an initial precipitating injury (IPI), a latent period, then recurrent spontaneous seizures. In humans, an IPI is any medical event with neurological implications. Although transition from a latent period to a seizure disorder certainly constitutes "progression" of the disorder, convincing clinical evidence of subsequent worsening has not emerged. Substantial clinical and experimental evidence indicates some cognitive regression and focal atrophy with time for TLE and other intractable syndromes. However, seizure frequency and severity, established early in the disorder, appear stable in most patients, and even regress in benign syndromes. Factors mitigating or extinguishing epilepsies need to be further sought.     

The prognosis of idiopathic generalized epilepsy

Prognosis describes the trajectory and long‐term outcome of a condition. Most studies indicate a better prognosis in idiopathic generalized epilepsy (IGE) in comparison with other epilepsy syndromes. Studies looking at the long‐term outcome of different IGE syndromes are relatively scant. Childhood absence epilepsy appears to have a higher rate of remission compared to juvenile absence epilepsy. In absence epilepsies, development of myoclonus and generalized tonic–clonic seizures predicts lower likelihood of remission. Although most patients with juvenile myoclonic epilepsy (JME) achieve remission on antiepileptic drug therapy, <20% appear to remain in remission without treatment. Data on the prognosis of other IGE syndromes are scarce. There are contradictory findings reported on the value of electroencephalography as a predictor of prognosis. Comparisons are made difficult by study heterogeneity, particularly in methodology and diagnostic criteria.     

Benign Focal Epilepsies of Childhood

Until relatively recently, genetic influences in partial seizures were thought to be of minimal importance. However, with further identification of childhood benign partial seizures it is becoming clear that inheritance plays a major role in the pathogenesis of these seizures. Diagnostic criteria proposed for benign partial seizures include absence of neurologic or intellectual deficits, family history of epilepsy, onset of seizures after age 2 years, stereotyped brief seizures, frequent nocturnal occurrence, spontaneous remission in adolescence, and electroencephalograms (EEGs) demonstrating spikes with a distinctive morphology and localization superimposed on normal background activity. The two most commonly described benign partial epilepsies of childhood are benign Rolandic epilepsy (BRE) and benign occipital epilepsy (BOE). Both disorders begin in childhood, are associated with characteristic EEG patterns, have seizures that are easily controlled with medication, often are familial, and have an excellent prognosis. The other benign partial seizure disorders in children that have been described are not as well studied as BOE and BRE, and the role of inheritance pattern, if any, is less clear.     

Absence epilepsy with onset before age three years: a heterogeneous and often severe condition

PURPOSE: The classification of epilepsies and epileptic syndromes recognizes three syndromes with typical absences [TA, i.e., childhood and juvenile absence epilepsies (CAE and JAE), and epilepsy with myoclonic absences (EMA), none of which is characterized by onset in early childhood]. Although several other forms of absence epilepsies have been described recently, none concerns infants and very young children, and little is known about the nosology and prognosis of early-onset absences. METHODS: We retrospectively selected all cases with onset of absences as the only or major seizure type before age 3 years and >/=2 years of follow-up among cases newly referred between 1986 and 2002. Neuropsychological assessments (generally IQ measure), behavior patterns, and schooling situations were reviewed for each child. RESULTS: We found 10 patients (7 F, 3 M). No child had sensory or motor deficits: neuroimaging was performed in nine and was normal in eight, with aspecific findings in one. Only two could be characterized as CAE and EMA, respectively, both with seizure control and a good cognitive outcome. Among the remaining eight cases, four had a fairly homogeneous presentation with predominantly brief absences and clearly asymmetric interictal EEGs. All eight had neuropsychological and/or behavioral difficulties. Three had full seizure control, and five, persisting absences, with a follow-up ranging between 2 years 8 months to 9 years 4 months; only one child was older than 12 years. CONCLUSIONS: Great heterogeneity exists among absence epilepsies of early onset, which are rare conditions. Only a few patients can be categorized into well-known syndromes. The overall prognosis is poor. Early onset of absences is uncommon, and multicenter studies should help clarify the nosology and prognosis.     

Seizure-related factors and non-verbal intelligence in children with epilepsy. A population-based study from Western Norway.

PURPOSE: To study the relationship between seizure-related factors, non-verbal intelligence, and socio-economic status (SES) in a population-based sample of children with epilepsy. METHODS: The latest ILAE International classifications of epileptic seizures and syndromes were used to classify seizure types and epileptic syndromes in all 6-12 year old children (N=198) with epilepsy in Hordaland County, Norway. The children had neuropediatric and EEG examinations. Of the 198 patients, demographic characteristics were collected on 183 who participated in psychological studies including Raven matrices. 126 healthy controls underwent the same testing. Severe non-verbal problems (SNVP) were defined as a Raven score at or <10th percentile. RESULTS: Children with epilepsy were highly over-represented in the lowest Raven percentile group, whereas controls were highly over-represented in the higher percentile groups. SNVP were present in 43% of children with epilepsy and 3% of controls. These problems were especially common in children with remote symptomatic epilepsy aetiology, undetermined epilepsy syndromes, myoclonic seizures, early seizure debut, high seizure frequency and in children with polytherapy. Seizure-related characteristics that were not usually associated with SNVP were idiopathic epilepsies, localization related (LR) cryptogenic epilepsies, absence and simple partial seizures, and a late debut of epilepsy. Adjusting for socio-economic status factors did not significantly change results. CONCLUSIONS: In childhood epilepsy various seizure-related factors, but not SES factors, were associated with the presence or absence of SNVP. Such deficits may be especially common in children with remote symptomatic epilepsy aetiology and in complex and therapy resistant epilepsies. Low frequencies of SNVP may be found in children with idiopathic and LR cryptogenic epilepsy syndromes, simple partial or absence seizures and a late epilepsy debut. Our study contributes to an overall picture of cognitive function and its relation to central seizure characteristics in a childhood epilepsy population and can be useful for the follow-up team in developing therapy strategies that meet the individual needs of the child with epilepsy.     

Seizure and Psychosocial Outcomes of Childhood and Juvenile Onset Generalized Epilepsies: Wolf in Sheep's Clothing,or Well-Dressed Wolf?

Studies of generalized electroclinical syndromes can provide guidance regarding long-term seizure, cognitive, and psychosocial outcomes. Childhood absence epilepsy, juvenile absence epilepsy, juvenile myoclonic epilepsy, and idiopathic generalized epilepsy with generalized tonic–clonic seizures alone are electroclinical syndromes typically associated with normal intellect and good response to antiseizure medications. However, studies have demonstrated significantly poorer psychosocial outcomes than expected for these syndromes, regardless of seizure control. Potential causes for this include underlying abnormalities in social skills, social stigma, and underlying abnormalities in brain development and maturation.When children are diagnosed with epilepsy, parents want to know the long-term outcome, such as whether the seizures will be pharmacoresponsive, will the epilepsy be outgrown, and how it will affect learning and development. These are difficult questions for the physician to answer. However, studies of long-term outcomes of specific electroclinical syndromes can help provide guidance.The previously labeled “idiopathic generalized epilepsy” syndromes—including childhood absence epilepsy, juvenile absence epilepsy, juvenile myoclonic epilepsy, and generalized epilepsy with generalized tonic–clonic seizures alone—are typically associated with normal intellect and good response to antiseizure medications. Therefore, it was previously felt these syndromes had a relatively benign course. However, further long-term studies have demonstrated this is not necessarily the case, suggesting they are “wolves in sheep''s clothing” (1). This article will review the long-term seizure outcome, as well as the cognitive and psychosocial outcomes of these syndromes.     

Epileptic Syndromes in Childhood

Summary: Epileptic syndromes are clusters of signs and symptoms regularly occurring together. These may include type of seizure(s), time and circumstances of occurrence, and neurological and EEG findings. Some syndromes have common signs and a predictable course (e.g., benign rolandic epilepsy). Others, such as absence seizures, are less specific and may include several subgroups with different outcomes and different associated features. Still others are rather loose collections of a few common characteristics irregularly linked together. Widely accepted syndromes in childhood include the West syndrome and Lennox-Gastaut syndromes, several myoclonic syndromes, febrile seizures of infancy, absence epilepsy, benign partial epilepsy, and juvenile myoclonic epilepsy. If the concept of epileptic syndromes is to be practically useful, it should be limited to clusters that are unequivocally identifiable. Heterogeneous epileptic syndromes such as West syndrome or absence epilepsies give only a limited guide to prognosis but may be important in determining investigations and treatment. A classification based on syndromes has the advantage of dispensing with most assumptions required by other systems. However, it cannot cover all the clinical aspects of epilepsy and gives no guide to the pathophysiology, genetics, and etiology of a seizure disorder. The ultimate goal remains the delineation of disease entities.     

Epileptic Syndromes in Childhood

Summary: Epileptic syndromes are clusters of signs and symptoms regularly occurring together. These may include type of seizure(s), time and circumstances of occurrence, and neurological and EEG findings. Some syndromes have common signs and a predictable course (e.g., benign rolandic epilepsy). Others, such as absence seizures, are less specific and may include several subgroups with different outcomes and different associated features. Still others are rather loose collections of a few common characteristics irregularly linked together. Widely accepted syndromes in childhood include the West syndrome and Lennox-Gastaut syndromes, several myoclonic syndromes, febrile seizures of infancy, absence epilepsy, benign partial epilepsy, and juvenile myoclonic epilepsy. If the concept of epileptic syndromes is to be practically useful, it should be limited to clusters that are unequivocally identifiable. Heterogeneous epileptic syndromes such as West syndrome or absence epilepsies give only a limited guide to prognosis but may be important in determining investigations and treatment.
A classification based on syndromes has the advantage of dispensing with most assumptions required by other systems. However, it cannot cover all the clinical aspects of epilepsy and gives no guide to the pathophysiology, genetics, and etiology of a seizure disorder. The ultimate goal remains the delineation of disease entities.     

How to diagnose and classify idiopathic (genetic) generalized epilepsies

Idiopathic or genetic generalized epilepsies (IGE) constitute an electroclinically well‐defined group that accounts for almost one third of all people with epilepsy. They consist of four well‐established syndromes and some other rarer phenotypes. The main four IGEs are juvenile myoclonic epilepsy, childhood absence epilepsy, juvenile absence epilepsy and IGE with generalized tonic‐clonic seizures alone. There are three main seizure types in IGE, namely generalized tonic‐clonic seizures, typical absences and myoclonic seizures, occurring either alone or in any combination. Diagnosing IGEs requires a multidimensional approach. The diagnostic process begins with a thorough medical history with a specific focus on seizure types, age at onset, timing and triggers. Comorbidities and family history should be questioned comprehensively. The EEG can provide valuable information for the diagnosis, including specific IGE syndromes, and therefore contribute to their optimal pharmacological treatment and management.     

Two-year remission and subsequent relapse in children with newly diagnosed epilepsy

PURPOSE: Although remission is the ultimate measure of seizure control in epilepsy, and epilepsy syndrome should largely determine this outcome, little is known about the relative importance of syndrome versus other factors traditionally examined as predictors of remission or of relapse after remission. The purpose of this study was to examine remission and relapse with respect to the epilepsy syndrome and other factors traditionally considered with respect to seizure outcome. METHODS: A prospectively identified cohort of 613 children with newly diagnosed epilepsy was assembled and is actively being followed to determine seizure outcomes. Epilepsy syndrome and etiology were classified at diagnosis and again 2 years later. Remission was defined as 2 years completely seizure-free, and relapse as the recurrence of seizures after remission. Multivariable analysis was performed with the Cox proportional hazards model. RESULTS: Five hundred ninety-four of the original 613 children were followed > or = 2 years (median follow-up, 5 years). Remission occurred in 442 (74%), of whom 107 (24%) relapsed. On multivariable analysis, idiopathic generalized syndromes and age at onset between 5 and 9 years were associated with a substantially increased remission rate, whereas remote symptomatic etiology, family history of epilepsy, seizure frequency, and slowing on the initial EEG were associated with a decreased likelihood of attaining remission. Young onset age (<1 year) and seizure type were not important after adjustment for these predictors. Relapses occurred more often in association with focal slowing on the initial EEG and with juvenile myoclonic epilepsy. Benign rolandic epilepsy and age at onset <1 year were associated with markedly lower risks of relapse. About one fourth of relapses were apparently spontaneous while the child was taking medication with good compliance, and more than half occurred in children who were tapering or had fully stopped medication. CONCLUSIONS: A large proportion of children with epilepsy remit. Symptomatic etiology, family history, EEG slowing, and initial seizure frequency negatively influence, and age 5-9 years and idiopathic generalized epilepsy positively influence the probability of entering remission. Factors that most influence relapse tend to be different from those that influence remission.     

Abnormal behavior in children with temporal lobe epilepsy and ganglioglioma

Temporal lobe epilepsy in childhood is characterized by great clinical, electroencephalographic, and etiological diversity. The prognosis after temporal lobe epilepsy surgery in childhood is usually good, with most patients achieving complete seizure control. However, in some children behavior deteriorates postoperatively. We report two girls (2 and 6 years of age) with refractory seizures due to temporal lobe ganglioglioma. They exhibited aggression and hyperactivity since the beginning of their epilepsy. In both patients, behavioral disturbances worsened postoperatively, despite complete seizure control. Patients and parents should be advised about possible behavioral disturbances after epilepsy surgery, especially in the presence of a temporal lobe developmental tumor, even when seizure control is achieved postoperatively.