Precipitating factors and therapeutic outcome in epilepsy with generalized tonic-clonic seizures

OBJECTIVES: The aim of the study was to evaluate the influence of precipitating factors and therapy on the outcome of epilepsy with generalized tonic-clonic seizures. PATIENTS AND METHODS: Retrospective analysis of data from 34 patients (mean age at seizure onset 19 years; mean duration of follow-up 9.2 years) suffering from epilepsy of either cryptogenic or remote symptomatic (n = 19), or idiopathic (n = 15) etiology. The total number of seizures in all patients was 146. RESULTS: Without treatment 97 seizures manifested during 90.5 years without treatment (1.07 seizures/year), during treatment with carbamazepine or valproate 49 seizures occurred within 224 years (0.2 seizures/year). The frequency of seizures was significantly lower during treatment. Precipitating factors were found in relation to 31% of seizures in patients with remote symptomatic or cryptogenic epilepsy, and for 51% of seizures in patients with idiopathic epilepsy. CONCLUSIONS: There was a low frequency of seizures in patients with generalized tonic-clonic seizures. Precipitating factors are common. Antiepileptic drug treatment is effective.     

Autism and epilepsy: a retrospective follow-up study

So-called "idiopathic" autism, which exhibited no major complications before diagnosis is well-known as one of the risk factors for epilepsy. This retrospective follow-up study aimed to clarify the characteristics of epilepsy in the autism; onset of seizure, seizure types, EEG findings and epilepsy outcome and the differences as a group between the autism with epilepsy and those without epilepsy. One hundred thirty individuals with autistic disorder or atypical autism diagnosed in childhood were followed up over 10 years and were evaluated almost every year up to 18-35 years of age. Their medical records related to perinatal conditions, IQ, social maturity scores and several factors of epilepsy were reviewed in October 2005. Thirty-three of the follow-up group (25%) exhibited epileptic seizures. The onset of epilepsy was distributed from 8 to 26 years of age. Two types of seizure were observed; partial seizure with secondarily generalized seizure and generalized seizure. Twenty of the epileptics (61%) showed the partial seizure. Although 18% of the non-epileptic group exhibited epileptic discharges on EEG, 68% of the epileptic group revealed epileptiform EEG findings before the onset of epilepsy. No differences were observed concerning the sex ratio, autistic disorder/atypical autism and past history of febrile seizures between the epileptic and non-epileptic groups. Lower IQ, lower social maturity score and higher frequency of prescribed psychotropics were observed in the epileptic group compared to the non-epileptics. Idiopathic autism was confirmed as the high risk factor for epilepsy. Epileptiform EEG findings predict subsequent onset of epileptic seizures in adolescence. Epilepsy is one of negative factors on cognitive, adaptive and behavioral/emotional outcomes for individuals with autism.     

Distribution of Epilepsy Syndromes in a Cohort of Children Prospectively Monitored from the Time of Their First Unprovoked Seizure

PURPOSE: To assess the distribution of epilepsy syndromes and their stability in children. METHODS: A cohort of 407 children with a first unprovoked seizure was prospectively recruited and followed up for a mean of 9.4 years. Etiology and epilepsy syndromes were classified by using the International League Against Epilepsy (ILAE) guidelines in the 182 children with two or more seizures. Classification was done both at time of second seizure and at last follow-up. Two-year terminal remission also was analyzed by etiology and epilepsy syndrome. RESULTS: Etiology of epilepsy syndromes was idiopathic in 45 (25%), cryptogenic in 89 (49%), and remote symptomatic in 48 (26%). In the initial classification, 114 (63%) children had a localization-based epilepsy syndrome including idiopathic in 26, cryptogenic in 34, and symptomatic based on localization or etiology in 54. Twenty-one (12%) children had a generalized epilepsy syndrome, including 19 with primary generalized epilepsy. Forty-seven (26%) cases were in the category of undetermined if focal or generalized. At last follow-up, there was a change in either etiology (n = 16) or the final epilepsy syndrome classification (n = 33) or both (n = 15) in 34 (19%) cases. At time of last follow-up, 144 (79%) of the children with epilepsy were in 2-year terminal remission, and 108 (59%) were in 2-year terminal remission without medications. Factors associated with a favorable prognosis included an idiopathic or cryptogenic etiology and having a localization-based idiopathic epilepsy syndrome. CONCLUSIONS: After two seizures, childhood-onset epilepsy can be classified by etiology and epilepsy syndrome. Prognosis is favorable in the majority of cases. However, the apparent syndrome may change with longer follow-up. The ability to classify these cases early in the clinical course is important if they are to be used for prognostic purposes.     

Motor and cognitive functions in newly diagnosed adult seizure patients before antiepileptic medication

OBJECTIVE: Motor and cognitive functions in patients with partial or generalized onset of seizures were evaluated prior to the administration of antiepileptic medication. MATERIAL AND METHODS: Motor function, attention and memory of 52 consecutive newly diagnosed adult patients with partial or generalized seizures were assessed with neuropsychological tests. RESULTS: Patients with partial onset of seizures did not differ from patients with generalized seizures in tests of motor function or attention, nor in tests of learning and memory. Compared to controls patients with epilepsy performed significantly worse on visual motor tasks, mental flexibility and in delayed visual memory. Within the patient group as a whole lower education, higher age and symptomatic epilepsy with more abnormal CT scan findings tended to associate with worse performance in tests of concentration and mental flexibility and tests of memory. CONCLUSION: These findings indicate that newly diagnosed adult patients with partial or generalized onset of seizures prior to treatment with antiepileptic medication experience some problems in visual motor tasks, mental flexibility and memory even without the numerous risk factors for cognitive deficits in epilepsy. In newly diagnosed patients with epilepsy as a whole symptomatic etiology was associated with somewhat more pronounced cognitive problems.     

Seizure Risk in Offspring of Parents with Generalized Versus Partial Epilepsy

Genetic factors are commonly assumed to play a more important role in generalized than in partial epilepsy. This study tested this hypothesis by comparing risks of unprovoked seizures in offspring of individuals with generalized versus partial epilepsy. Overall, seizure incidence was no higher in offspring of persons with generalized epilepsy than in offspring of those with partial epilepsy. The number of affected offspring was about three times that expected from population incidence rates, regardless of whether the parent had partial or generalized epilepsy. For the subgroup of generalized cases with absence seizures, however, seizure incidence in offspring was about three times as high as for partial cases. The higher incidence in offspring of absence cases was only partly explained by a higher proportion of absence than partial cases with two factors associated with high risk in relatives, namely early age at onset and idiopathic epilepsy. Offspring of absence cases had higher risk than offspring of other cases not only for absence seizures, but for other seizure types as well, suggesting that absence epilepsy is not genetically distinct from other seizure types of epilepsy. These results suggest that the higher incidence sometimes observed in relatives of patients with generalized epilepsy is due to a small proportion of generalized cases with extremely high familial risks--most generalized epilepsies are no more likely than partial epilepsies to have a genetic basis.     

Patients with pseudoseizures: intellectual and cognitive performance

We compared cognitive and intellectual performance of patients with pseudoseizures (pseudoseizure-only group), pseudoseizures and epilepsy (mixed seizure group), and generalized epileptic seizures (generalized seizure group). The pseudoseizure-only group performed significantly better on all measures except those of simple motor function. There were no significant differences between those with mixed and generalized seizures. Therefore, cognitive and intellectual performances of patients with pseudoseizures are influenced by the presence or absence of concomitant epilepsy, and suggest that it is necessary to distinguish patients with and without epilepsy in studies of pseudoseizures.     

Resective surgery for intractable focal epilepsy in patients with low IQ: predictors for seizure control and outcome with respect to seizures and neuropsychological and psychosocial functioning

PURPOSE: To investigate possible predictive factors for seizure control in a group of children and adults with low IQs (IQ, < or =70) who underwent resective surgery for intractable focal epilepsy and to study outcome with respect to seizures and neuropsychological functioning. We also studied psychosocial outcome in the adult patients. METHODS: Thirty-one patients (eight children younger than 18 years) with a Wechsler Full Scale IQ of 70 or less underwent comprehensive neuropsychological assessments before and 2 years after surgery. Adults also completed the Washington Psychosocial Seizure Inventory (WPSI). Univariate analyses were used to identify variables differentiating between patients who became seizure free and those who did not. Pre- and postoperative test results were compared by t test for dependent samples. RESULTS: Forty-eight percent of the patients became seizure free, 52% of those with temporal lobe resection and 38% of those with extratemporal resection. Only one variable was predictive for seizure outcome: duration of epilepsy. In one third of the patients, who had the shortest duration of epilepsy (<12 years), 80% became seizure free. Significant improvement was seen regarding vocational adjustment in adults (WPSI). Seizure-free adults improved their Full Scale IQ scores. No cognitive changes were found in seizure-free children or in patients who did not become seizure free. CONCLUSIONS: A good seizure outcome was obtained after resective surgery in patients with intractable focal epilepsy and low IQ, provided that treatment was done relatively shortly after onset of epilepsy. No adverse effects were seen on cognitive and psychosocial functioning.     

Cognitive outcomes in children who present with a first unprovoked seizure

Purpose: To determine the long‐term cognitive and educational outcomes in children prospectively identified at the time of a first unprovoked seizure. Methods: A cohort of children with a first unprovoked seizure was enrolled and followed for a mean of 15 years. Cognitive function and educational outcomes were determined 10 or more years after the first seizure via standardized neuropsychological tests, school records, and structured interviews. Children with symptomatic etiology were excluded from the analysis. When available, siblings of study subjects were recruited as normal controls. Primary educational outcome was defined as enrollment into special education services or grade repetition. Results: Twenty‐eight percent of (43 of 153) of children with a single seizure and 40% (42 of 105) of children with epilepsy received special education service or repeated a grade (p = 0.05). There was a statistically significant trend in which the children with more seizures tended to require special education or repeat a grade more often (28% in single seizure group vs. 34% in 2–9 seizure group vs. 64% in ≥10 seizure group; p = 0.004). Of 163 subjects who completed neuropsychological testing, children with single seizures tended to score higher than children with epilepsy on Wide Range Achievement Test‐3 (WRAT) reading (p = 0.08), Test of Non‐Verbal Intelligence‐II (TONI‐II) (p = 0.02), and Wechsler Intelligence Scale for Children (WISC)/Wechsler Adult Intelligence Scale (WAIS) (p = 0.07). There was no statistically significant difference between children with a single seizure and sibling controls. Conclusion: The results suggest that children with a single seizure represent a group that is distinctly different from children with epilepsy and are more similar to sibling controls. In contrast, even children with very mild epilepsy have significantly worse educational outcomes.     

Cognitive decline in severe intractable epilepsy

PURPOSE: To explore the relation between seizure-related variables and cognitive change in patients with severe intractable epilepsy. METHODS: A retrospective analysis of data from 136 patients who had undergone a cognitive assessment on two occasions at an interval of > or =10 years. Cognitive measures included tests of memory and executive skills in addition to intelligence quotients (IQ). Details were available regarding seizure type and frequency in the intertest interval. RESULTS: Cognitive decline was severe and occurred across a wide range of cognitive functions. The frequency of generalised tonic-clonic seizures was the strongest predictor of decline. Complex partial seizure frequency was associated with a decline in memory and executive skills but not in IQ. Seizure-related head injuries and advancing age carried a poor cognitive prognosis, whereas periods of remission were associated with a better cognitive outcome. Early age at onset was not implicated, and duration of epilepsy was a much less potent predictor of cognitive decline than has been reported in cross-sectional studies. No evidence indicated that a higher level of cognitive function protected against cognitive decline. CONCLUSIONS: Our findings, together with those from animal studies and surgically treated patients, suggest that seizures can have a direct adverse effect on cognition and that good seizure control even after years of intractability can have a beneficial impact on cognitive prognosis. This study was based on individuals who merited two cognitive assessments > or =10 years apart and hence is biased in favor of those with the most severe forms of refractory epilepsy and those with decline.     

Possibilities of non-pharmacological conservative treatment of epilepsy.

This study set out to assess the effect of non-pharmacological conservative (NPC) interventions as alternatives to antiepileptic pharmacotherapy. A prospective follow-up cohort study was conducted in an outpatient seizure clinic of a referral center for epilepsy. Twenty-five patients (nine males, 16 females) aged 16-45, with at least two well-described epileptic seizures, were included who had rejected antiepileptic pharmacotherapy. Twelve had idiopathic generalized epilepsy, 11 had symptomatic or cryptogenic localization-related epilepsy, and two had epilepsy with generalized and focal signs. Twenty-three of the patients were followed for more than 2 years. The patients were treated with arrest after focal seizure onset (2 cases), sensory protection against reflex seizures (3 cases), avoidance of non-specific seizure-precipitating factors ('life hygiene', 16 cases), and/or miscellaneous interventions (8 cases). The main outcome measures were complete seizure control (more than 2 years) or sufficient improvement to continue with NPC treatment alone. Eight of the 23 patients were completely seizure free for more than 4 years, and three were sufficiently improved to continue NPC treatment without drugs. Trends were observed for patients with idiopathic generalized epilepsies with less than seven convulsive seizures, and with only one seizure type to respond better to NPC treatment. The duration of epilepsy, and the finding of generalized epileptiform discharge in the EEG had no influence on the outcome. Rational NPC treatments which are aimed at specific factors in the precipitation and development of epileptic seizures can be useful therapeutic alternatives for patients with milder forms of epilepsy. Apart from photosensitive patients, those most likely to profit are patients with idiopathic generalized epilepsy, a maximum of six generalized tonic-clonic seizures which were precipitated by lack of sleep or excessive alcohol intake, and with no or rare concomitant absences. In such cases, NPC treatment may be as effective as pharmacotherapy and gives the patient a positive experience of regained self-control.     

Seizure recurrence after a first unprovoked seizure in childhood: a prospective study

PURPOSE: To study the risk of recurrence after a first unprovoked seizure in childhood. METHODS: All consecutive patients aged less than 14 years with one or more unprovoked seizures who were attended between January 1, 1987, and June 1, 1996, were included in a prospective study. Clinical features of patients attended after a first seizure and those attended after two or more seizures were compared. Recurrence risk in both groups was estimated by Kaplan-Meier curves. Univariate and multivariate analyses of the potential predictors of recurrence risk were performed for the group of patients attended after a first seizure using the Cox proportional hazards model. RESULTS: Included in the study were 217 children. Kaplan-Meier estimate of recurrence risk was 64% at 5 years, when only patients being attended after a first epileptic seizure were included, compared with 74% when all patients were included. Significant differences in several clinical features were found between patients attended after a first seizure and those attended after two or more seizures. Univariate and multivariate analyses showed that in the overall cohort of patients attended after a first seizure, a symptomatic etiology increased the risk of recurrence, whereas a patient age of 3 to 10 years decreased this risk. In particular, the recurrence risk was 96% at 2 years for symptomatic seizures, compared with 46% for idiopathic/cryptogenic seizures. In the group of patients with idiopathic/cryptogenic seizures, an abnormal electroencephalogram and the occurrence of seizures during sleep increased the recurrence risk, whereas a patient aged 3 to 10 years reduced it. In the group of patients with symptomatic etiology, univariate analysis revealed that there was a lower recurrence risk for patients aged 3 to 10 years. This last finding was not maintained, however, in multivariate analysis. CONCLUSIONS: The recurrence risk depends on the inclusion criteria for enrolling patients. Several factors enable us to predict the recurrence risk after a first unprovoked seizure; the most important of these factors is the etiology of the seizures.     

Factors Affecting the Outcome of MMPI and Neuropsychological Assessments of Psychogenic and Epileptic Seizure Patients

The literature reveals conflicting information regarding the extent to which psychogenic seizure patients have emotional disorders and neuropsychological deficits as compared with patients with epileptic seizures. Among groups matched for numbers of subjects, age, sex, and years of education, we observed that patients who had solely psychogenic seizures with either a major component of affectual expression or relatively minor motor activity were more disturbed emotionally than epileptic patients with partial seizures. In contrast, the personality characteristics of psychogenic seizure patients whose attacks had either little affectual display or prominent motor expression could not be distinguished from those of epileptic patients with convulsive generalized seizures. Closely matched groups of patients with solely psychogenic seizures, partial seizures, or generalized seizures did not differ in neuropsychological performance. However, psychogenic seizure patients performed much better on neuropsychological measures than a less-well-educated, but otherwise matched group of patients with generalized epileptic seizures.     

Direct and indirect costs of refractory epilepsy in a tertiary epilepsy center in Germany

PURPOSE: There are only few studies on the costs of epilepsy in Germany. Therefore, we performed a pilot study to estimate the direct and indirect costs of refractory epilepsy in a German epilepsy center. METHODS: A "prevalence-based," cross-sectional convenience sample of adults with active epilepsy attending the outpatient clinic of our tertiary epilepsy center was evaluated. Seizure-free patients and patients presenting with their first seizure were excluded. Direct and indirect costs were prospectively recorded over a three-month period using questionnaires and a patient diary. Cost driving factors were identified. RESULTS: One hundred one patients were included (40.7+/-15.2 years; disease duration: 18.1+/-15.3 years; 6 patients had focal epilepsy with simple partial seizures only, 28 with complex partial seizures, 43 with secondarily generalized tonic-clonic seizures; 20 had idiopathic generalized epilepsy with generalized tonic-clonic seizures). The total costs of epilepsy per patient were in average euro 2610+/-4200 over the three-month period. Direct cost contributed 39% to the total costs. Costs of anticonvulsant medication were the main contributor to the direct costs while indirect costs were caused mainly by losses due to early retirement. Cost driving factors included higher seizure frequency, longer disease duration, ictal falls, and situationally inappropriate complex behavior during or after the seizure. CONCLUSIONS: Indirect costs were higher than direct costs in adult patients with active epilepsy attending a German epilepsy center. Medication contributed the most to the direct costs and early retirement was the main factor for the indirect costs. The costs of poorly controlled epilepsy in this German study were above average of the European costs of epilepsy.     

Epileptic Syndromes in Infancy and Childhood: Recent Advances

Summary: Epileptic syndromes are more reliable than etiology in determining prognosis and optimal treatment in infants and children. Benign neonatal and infantile convulsions are either idiopathic or dominantly inherited, the latter being genetically nonallelic. Forms of idiopathic generalized epilepsy (IGE) include absence epilepsy, benign myoclonic epilepsy of infancy, and epilepsy with generalized myoclonic atonic seizures. Forms of idiopathic partial epilepsy include benign partial epilepsy with centrotemporal spikes (BECT), benign partial epilepsy with occipital paroxysms, idiopathic partial epilepsy with affective seizures, and benign partial epilepsies with extreme somatosensory evoked potentials (SEPS). These idiopathic epilepsies appear to result from a multifactorial genetic predisposition. The diagnosis is based on strict clinical and EEG criteria. In epileptogenic encephalopathies, cognitive disorders are the main feature and are linked to so–called interictal abnormalities. Some epileptic syndromes, particularly myoclonic epilepsies, remain unclassifiable because of poor understanding of nosology or heterogeneous outcome.     

Neuropsychological profiles and outcomes in children with new onset frontal lobe epilepsy

Frontal lobe epilepsy (FLE) is the second most frequent type of localization-related epilepsy, and it may impact neurocognitive functioning with high variability. The prevalence of neurocognitive impairment in affected children remains poorly defined.This report outlines the neuropsychological profiles and outcomes in children with new onset FLE, and the impact of epilepsy-related factors, such as seizure frequency and antiepileptic drug (AED) load, on the neurocognitive development.Twenty-three consecutive children (15 males and 8 females) with newly diagnosed cryptogenic FLE were enrolled; median age at epilepsy onset was 7 years (6–9.6 years). They underwent clinical and laboratory evaluation and neuropsychological assessment before starting AED treatment (time 0) and after one year of treatment (time 1).Twenty age-matched patients affected by idiopathic generalized epilepsy (10 male and 10 females) and eighteen age-matched healthy subjects (9 males and 9 females) were enrolled as controls and underwent the same assessment.All patients with FLE showed a significant difference in almost all assessed cognitive domains compared with controls, mainly in frontal functions and memory. At time 1, patients were divided into two groups according to epilepsy-related factors: group 1 (9 patients) with persisting seizures despite AED polytherapy, and group 2 (14 patients) with good seizure control in monotherapy. A significant difference was highlighted in almost all subtests in group 1 compared with group 2, both at time 0 and at time 1.In children with FLE showing a broad range of neurocognitive impairments, the epilepsy-related factors mostly related to a worse neurocognitive outcome are poor seizure control and the use of AED polytherapy, suggesting that epileptic discharges may have a negative impact on the functioning of the involved cerebral regions.     

Seizures in childhood ischemic stroke in Taiwan

In this retrospective study, we collected clinical and radiographic data on children (age range, 1 month to 18 years) with symptoms and radiographic confirmation of seizure after ischemic stroke for the period of January 1996 to July 2006. Thirty-nine out of 94 children with ischemic stroke had poststroke seizures. Thirty-three out of 39 children with poststroke seizures had new onset seizures but only data of 28 were available. Infection was the most common etiology in the early poststroke seizure group (52.4%) but not in the late poststroke seizure group (0%). Infarction involving arterial ischemic stroke of anterior circulation were the most common in both the early poststroke seizure (61.9%) and the late poststroke seizure group (57.1%). Epilepsy was the most common sequelae in both the early poststroke seizure (38.1%) and late poststroke seizure group (100%). Children who had initial focal neurological sign (100% vs. 38.1%; P=0.007) or the focal cortical dysfunction on EEG (85.7% vs. 33.3%; P=0.029) were prone to develop late poststroke seizures. Late poststroke seizures had a high risk of developing poststroke epilepsy (100% vs. 38.1%; P=0.007). We conclude that seizures commonly occur in childhood ischemic stroke. Most poststroke seizures developed at an early stage. Infection was the most common etiology that caused early poststroke seizures in childhood ischemic stroke. Initial focal neurological signs and focal cortical dysfunction on EEG are risk factors for developing epilepsy. Poststroke seizures did not affect mortality, but there was a significant difference in normal outcome and epilepsy between those with or without poststroke seizures.     

Remission and relapse in a drug-resistant epilepsy population followed prospectively

Purpose: We investigated the cumulative probability of seizure remission and relapse in an adult population with drug‐resistant epilepsy and frequent seizures. In addition, we determined clinical predictors of remission and relapse in this population. Methods: In 2003, we identified 246 patients at a single center with drug‐resistant epilepsy defined as at least one seizure per month and failure of at least two antiepileptic drugs. These patients were followed prospectively (cohort design). We examined the cumulative probability of seizure remission and relapse in this population using Kaplan‐Meier methodology. Clinical predictors of remission and relapse were also evaluated using Cox regression analysis. Key Findings: The estimated cumulative probability of 12‐month seizure remission was 34.6% at 7 years in the entire population and 33.4% when limited to those without surgery. The risk for relapse after a 12‐month period of seizure remission was 71.2% at 5 years. Negative predictors of seizure remission included developmental delay, symptomatic generalized epilepsy syndrome, duration of intractability, and number of antiepileptic drugs failed. Localization‐related epilepsy was the only negative predictor of relapse. Significance: Among patients with drug‐resistant epilepsy, 5% per year enter seizure remission even with a follow‐up of 6 years. However, a substantial proportion of these patients relapse after the first year following a remission. The large proportion of patients entering a significant remission gives these patients hope; however, caution should be advised when discussing the likelihood of future seizures.     

Hypnic Tonic Postural Seizures in Healthy Children Provide Evidence for a Partial Epileptic Syndrome of Frontal Lobe Origin

Summary: In the pediatric age period, three idiopathic partial epileptic syndromes are recognized: benign childhood epilepsy with centrotemporal spikes, childhood epilepsy with occipital paroxysms, and primary reading epilepsy. All other partial epilepsies are considered cryptogenic, and no other idiopathic partial epilepsies have been recognized. We observed 10 children with tonic partial postural seizures, mainly hypnic, coinciding with a recognizable ictal epileptiform EEG pattern. The children all had normal neuropsychological development before and after seizure onset. The seizures were tractable in all. Onset was in the early pediatric age period; no other type of seizure was recognized. The occurrence of a family history of epilepsy was high. The seizure pattern was typical of supplementary motor area origin. Because of the normal neuropsychological status, high familial incidence of epilepsy, and benign course of this partial epilepsy, we believe it should be considered idiopathic, using the criteria of the International Classification of Epilepsies and Epileptic Syndromes. We therefore suggest the existence of a partial idiopathic epileptic syndrome having onset with seizures of frontal lobe origin.     

Medically refractory epilepsy in autism

Purpose: Epilepsy and electroencephalographic abnormalities are frequent in idiopathic autism, but there is little information regarding treatment‐resistant epilepsy (TRE) in this group. We sought to define the clinical and electrophysiologic characteristics and treatment outcomes in these patients. Methods: We retrospectively reviewed clinical and laboratory data of patients with idiopathic autism evaluated at NYU Epilepsy Center during a 20‐year period. Key Findings: One hundred twenty‐seven patients had idiopathic autism and at least one epileptic seizure; 33.9% had TRE and 27.5% were seizure free. The remaining 38.6% of patients had infrequent seizures or insufficient data to categorize. Patients with TRE had a significantly earlier onset of seizures than seizure‐free patients, and a trend for more developmental regression and motor and language delays. Three patients had surgical resection (two had limited improvement and one had no improvement) and one had an anterior callosotomy (no improvement). Vagus nerve stimulator (VNS) implantation provided limited improvement (2 patients) and no improvement (7). Significance: This study found that TRE is common in idiopathic autism and more common with early age of seizure onset. Relatively few patients underwent surgical resection due to multifocal partial epilepsy, comorbid generalized epilepsy, or limited impact of ongoing partial seizures given other problems related to autism. Our small sample suggests that surgical and VNS outcomes in this group are less favorable than in other TRE populations.     

Epilepsy With Myoclonic Atonic Seizures: An Electroclinical Study of 69 Patients

Epilepsy with myoclonic-atonic seizures is characterized by myoclonic-atonic, absence, tonic-clonic, and eventually tonic seizures, appearing in previously normal children at ages 18-60 months. We analyzed the electroclinical features, treatment, and outcome of 69 patients with myoclonic-atonic seizures; these patients were followed between 1990 and 2012 at the Juan P. Garrahan Pediatric Hospital, Buenos Aires, Argentina. No structural or metabolic etiology was identified. Based on the electroclinical features and evolution, two groups could be distinguished. The first group of 39 patients with myoclonic and myoclonic-atonic seizures with or without generalized tonic-clonic seizures and absences associated with generalized spike- and polyspike-and-wave paroxysms had excellent prognoses. The second group of 30 patients had myoclonic jerks and myoclonic-atonic seizures associated with other seizure types including tonic seizures; some had myoclonic status epilepticus and cognitive deterioration. The interictal EEG showed frequent generalized spike- and polyspike-and-wave paroxysms. In 16 patients, the seizures remitted within 3.6 years. The two groups were distinguished in retrospect, when enough time had elapsed to evaluate cognitive deterioration and different seizure types. In conclusion, epilepsy with myoclonic atonic seizures is an epileptic syndrome with a broad clinical spectrum and variable prognosis.