The effect of duration of epilepsy on IQ in children with CLRE; a comparison to SLRE and IGE.

Up to one third of the children with epilepsy are classified as having cryptogenic localization epilepsy (CLRE). Unfortunately, not much is known on the prognosis of CLRE; it is assumed to be somewhere between the benign course of many idiopathic epilepsies and the more malignant course of most symptomatic epilepsies. The risk of cognitive decline over time seems to be increased for children with epilepsy, but no data are available specifically for CLRE. This study was a systematic cross-sectional open clinical and non-randomized investigation, which included 68 children with CLRE, 24 children with symptomatic localization related epilepsy (SLRE) and 21 children with idiopathic generalized epilepsy (IGE). One-way between-groups ANOVAs with Tukey post hoc testing were used to test differences in demographical variables and IQ between CLRE, SLRE and IGE and to test the influence of duration of epilepsy and other epilepsy factors on IQ. To test whether there were IQ differences between the three types of epilepsy regarding duration of epilepsy and other epilepsy factors, the Kruskal-Wallis test was used. In CLRE, intelligence is stable over time; IQ scores do neither improve nor deteriorate. Age at onset, seizure type, seizure frequency and the use of AED appeared to have no influence on intelligence in CLRE. Furthermore, over time, there appeared to be no differentiation regarding IQ between CLRE, SLRE and IGE.     

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.     

Cryptogenic localization related epilepsy in children from a tertiary outpatient clinic: Is neurological and neuropsychological outcome predictable?

OBJECTIVES: Up to one-third of the children with epilepsy are diagnosed with cryptogenic localization related epilepsy (CLRE). As yet, there is a lack of studies that specify the short- and long-term prognosis for this group. In this study, we systematically established neurological outcome (represented by seizure frequency) as well as neuropsychological outcome in a cohort of 68 children with CLRE who had been referred to our tertiary outpatient clinic. Also, we analysed correlations with risk and prognostic factors. PATIENTS AND METHODS: A systematic cross-sectional open clinical and non-randomized design was used including 68 children admitted to our epilepsy centre in a child neurological programme between January 1999 and December 2004. A model was defined, distinguishing risk factors with a potential effect on epileptogenesis (history of febrile seizures, family history of epilepsy, history of early mild development delay and serious diagnostic delay) and prognostic factors, with a potential effect on the course of the epilepsy (neurological symptoms or soft signs, age at onset, duration of epilepsy, seizure type, percentage of time with epileptiform activity, localization of epileptiform activity, treatment history and treatment duration). Seizure frequency was used as the primary outcome variable, whereas three neuropsychological outcomes (IQ, psychomotor delay and educational delay) were used as secondary outcome variables. RESULTS: The children experienced a broad range of seizure types with the 'absence-like' complex partial seizure as the most commonly occurring seizure type. Almost half of the children of the study sample had a high seizure frequency. They experienced several seizures per month, week or even daily seizures. Also a substantial impact on neuropsychological outcome was observed. Mean full scale IQ was 87.7, mean academic delay was almost 1 school year and 27 children showed psychomotor delay on the Movement ABC. Only 'having more than one seizure type' showed a prognostic value for seizure frequency, and no factors were found to be correlated with the secondary outcome measures. None of the risk factors show a differential impact on seizure outcome. CONCLUSION: CLRE has a non-predictable course; clinical variability is high and prognosis in many children with CLRE is obscure. Having more than one seizure type was the only factor correlated to seizure frequency. Further longitudinal studies are needed.     

Neuropsychological profile of children with cryptogenic localization-related epilepsy and its association with age at onset

The aim of this study was to investigate the neuropsychological profile of children with cryptogenic localization-related epilepsy (CLRE). Neuropsychological evaluations were performed in 16 CLRE children and 14 children with idiopathic localization-related epilepsy (ILRE) for control within 8 months (average 2.1 months) of initial seizure. The neuropsychological tests used in this study are as follows: the Wechsler Intelligence Scale for Children-Third Edition, Wechsler Intelligence Scale for Children-Revised, and Wechsler Preschool and Primary Scale of Intelligence. Age at onset and test differed significantly between CLRE and ILRE, while the duration between onset and test and the number of seizures before test did not. No marked difference was observed in the neuropsychological profile between 2 groups; however, the discrepancy between VIQ and PIQ was significantly larger in CLRE than in ILRE. This discrepancy was negatively correlated with age at the time of seizure onset (r = -0.615, and p = 0.011). The laterality in discrepancy between VIQ and PIQ was associated with the dominance of interictal discharge. In conclusion, children with lower age at the time of seizure onset were likely to have had a larger discrepancy between VIQ and PIQ.     

抗癫痫药物应用专家共识

目的 编制我国抗癫痫药物应用专家共识.方法 采用无记名问卷调查形式,收集我国三级医院中成人神经科专科医生有关抗癫痫药物的应用经验与评价.调查内容为特发性全面性癫痫与症状性部分性癫痫的药物治疗,以及特殊人群与伴有共患病患者的抗癫痫药物应用.药物评价标准采用九级分级制.药物治疗策略评价根据专家评分所得均数、标准差与95%可信区间(95%CI),分为首选药物、一线、二线与三线药物.结果 发出问卷50份,回收49份(98%).49名专家中,男性38名(77.6%),女性11名(22.4%),年龄(53.9±10.8)岁(35～81岁),平均从业时间(17.9±10.2)年(3～45年),所有专家每月诊治癫痫患者20～800例(中位数100例).总体治疗策略中,有关特发性全面性癫痫与症状性部分性癫痫的首选治疗,100%的专家选择单药.丙戊酸是新诊断特发性全面性癫痫的一线药物且惟一的首选药物.症状性部分性癫痫的初始药物首选均为卡马西平和奥卡西平.在特发性全面性癫痫药物治疗中,丙戊酸是与其他药物联合治疗的首选药物.症状性部分性癫痫的药物治疗中,卡马西平(奥卡西平)+托吡酯、卡马西平(奥卡西平)+左乙拉西坦、卡马西平(奥卡西平)+丙戊酸、丙戊酸+拉莫三嗪等是常用配伍.拉莫三嗪为健康育龄期妇女特发性全面性癫痫与症状性部分性癫痫的首选用药;伴抑郁的癫痫患者,特发性全面性发作的首选用药为丙戊酸与拉莫三嗪,继发性部分性发作的首选用药为拉莫三嗪、奥卡西平与卡马西平;伴有乙肝的癫痫患者,无论肝功能是否正常,特发性全面性发作的首选用药为托吡酯与左乙拉西坦,肝功能正常的继发性部分性患者,首选用药为奥卡西平,肝功能指标异常时,首选用药为托吡酯与左乙拉西坦;急诊室中的癫痫患者(不确定类型)首选丙戊酸与左乙拉西坦.结论 本共识归纳专家的临床经验,将对癫痫的药物治疗有帮助.

Abstract：

Objective To prepare expert consensus opinion in treatment of epilepsy in China.Methods To sent an anonymous questionnaire on the treatment of adolescent and adult epilepsy syndromes to a group of hospital neurologists in the field of epilepsy. The questions were formatted to simulate real-world clinical situations in the treatment of symptomatic localization related epilepsy (SLRE), idiopathic generalized epilepsy (IGE), and treatment in special patient populations and patients with comorbidity. The experts were asked to rate treatment options based on a modified RAND 9-point scale (with "9" most appropriate and "1" least appropriate). Statistical analysis of data was performed as defined by the expert consensus method. The results were used to develop user-friendly recommendations concerning overall treatment strategies and choice of specific medications. Results Of the 50 experts to whom the survey was sent, 49 (98%) responded. Of the respondents, 11 (22.4%) were female and 38 (77.6%) male. Their mean age was 53.9 years, with a mean of 17.9 years in practice. The median number of patients seen per month was 100 ( range, 20 to 800 ). For initial monotherapy of IGE ( generalized tonic-clonic ( GTC ),absence, and myoclonic seizures), valproate was rated as treatment of choice. Treatment options were rated for 3 types of SLRE: simple partial seizures ( SPS), complex partial seizures ( CPS ) , and secondarily generalized tonic-clonic seizures (SGTC). In SLRE-SPS and SLRE-CPS, carbamazepine and oxcarbazepine were treatments of choice, with lamotrigine, topiramate and levetiracetam as second line agents. In SLRESGTC, carbamazepine, lamotrigine and oxcarbazepine were treatments of choice, while lamotrigine,topiramate, levetiracetam and valproate were also usually appropriate. Valproate was selected as treatment of choice when combined with other AEDs in IGE. For SLRE, combination/add-on therapy of carbamazepine ( oxcarbazcpine ) + topiramate, carbamazepine ( oxcarbazepine ) + levetiracetam, carbamazepine (oxcarbazepine) +valproate, valproate + lamotrigine were considered as treatment of choice. For women who are pregnant or trying to conceive, lamotrigine was treatment of choice for both idiopathic generalized epilepsy (IGE) and symptomatic localization related epilepsy (SLRE). For patients with school-age,lamotrigine was treatment of choice for IGE, with oxcarbazepine and lamotrigine for SLRE. In people with both epilepsy syndromes who have depression, valproate and lamotrigine were treatment of choice for IGE; in SIRE, lamotrigine, oxcarbazepine and carbamazepine were treatment of choice. In persons with epilepsy and hepatitis B, whether liver function was normal or not, topiramate and levetiracetam were treatment of choice for IGE; in SLRE with normal liver function, oxcarbazepine was treatment of choice, while topiramate and levetiracetam were selected for SLRE with liver function impairment. Valproate and levetiracetam were treatment of choice for seizures in the emergency department. Conclusions The expert consensus method concisely summarizes expert opinion, and this opinion may be helpful in situations in which the medical literature is scant or lacking.     

Cryptogenic localization-related epilepsy with childhood onset: The problem of definition and prognosis

BACKGROUND: Up to one-third of children with epilepsy are diagnosed with cryptogenic localization-related epilepsy (CLRE). CLRE is a large nonspecific category within the ILAE classification. For this population no unequivocal prognosis exists. METHODS: Twenty-five articles describing aspects of CLRE were included in this review. RESULTS: As a result of the progress in epilepsy research, as well as more advanced investigation in individual cases, the population with CLRE constantly changes. Also, disagreement on interpretation of the classification has resulted in striking differences between the populations described. High remission rates are reported, but relapse occurs frequently, leaving the long-term prognosis unforeseeable. This is reflected in academic and psychosocial prognosis, which is described to be problematic in CLRE specifically. Possible prognostic factors of CLRE in children have been identified: age at onset, seizure semiology, seizure frequency, intractability, interictal epileptiform activity on EEG, and premorbid IQ. These factors are explored to define subgroups within the CLRE population. DISCUSSION: Prospective studies on well-defined CLRE cohorts are needed to identify factors that distinguish various prognostic subgroups. Specific attention should be focused on course of the epilepsy, scholastic achievement, and psychosocial outcome.     

Nonsymptomatic generalized epilepsy in children younger than six years: excellent prognosis,but classification should be reconsidered after follow-up: the Dutch Study of Epilepsy in Childhood

PURPOSE: To assess the prognosis and the accuracy of the epilepsy classification in young children with nonsymptomatic generalized epilepsy. METHODS: Of the cohort of the Dutch Study of Epilepsy in Childhood (n = 466), all children younger than 6 years with a diagnosis of idiopathic (IGE) or cryptogenic (CGE) generalized epilepsy either at intake (n = 108) and/or after 2 years of follow-up (n = 102) were included. The number of reclassifications after 2 years was determined, and the reasons for reclassification were analyzed. All children receiving a diagnosis of IGE or CGE at 2 years were followed up for 5 years to study their outcome in terms of terminal remission (TR). Data on their level of intellectual functioning were collected at the start of this analysis. RESULTS: The epilepsy syndrome was reclassified in 17 children. In 14 of them, the seizure type also was reclassified, and in three, the course of the epilepsy determined the new epilepsy type. Two other children had a reclassification of their seizure types without a change of the epilepsy type. Many children were categorized as having IGE not otherwise specified. In all probability, this is a heterogeneous group, containing patients with various epilepsy syndromes, with generalized tonic-clonic seizures as a common hallmark. Of the 102 children with IGE or CGE at 2 years of follow-up, 75% had a TR of >6 months after 2 years, and 85% a TR of >or=1 year after 5 years. CONCLUSIONS: In a fair proportion of children with nonsymptomatic generalized epilepsy in this age group, it is not possible to classify firmly the epilepsy and/or the seizures immediately after the intake. Instead, they are reclassified during the course of the disease. This and the apparent heterogeneity of the category IGE not otherwise specified point to inherent drawbacks of the current International League Against Epilepsy (ILAE) classification of epilepsy and epileptic syndromes. The prognosis of IGE at this young age is generally excellent.     

Which patients become seizure free with antiepileptic drugs? An observational study in 821 patients with epilepsy

Objectives –  Analysis of factors influencing seizure outcome in antiepileptic drug treatment of epilepsy.
Patients and methods –  Retrospective analysis of 500 patients with complete seizure control and 321 patients with refractory epilepsy (mean ages 33.3 and 32.1 years respectively).
Results –  The seizure-free group consisted of 377 patients with symptomatic/cryptogenic epilepsy (SCE; mean seizure control 45 months) and 123 patients with idiopathic generalized epilepsy (IGE; mean seizure control 61 months) ( P  = 0.02). Of the patients with SCE, 35.7% had achieved seizure control with monotherapy (MT), 29.6% with ≥2 AEDs. No single AED was superior in MT. Of the patients with IGE, 35.9% had become seizure free with MT, 15.6% on combination therapy (CT). Valproate MT was more commonly associated with seizure freedom than lamotrigine ( P  < 0.05).
Conclusions –  The results indicate that, in SCE, seizures can be controlled with carefully selected CT more commonly than suggested by previous studies. The seizure prognosis of patients with IGE presenting to a specialist in epilepsy may be worse than previously thought.     

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.     

Treatment of epilepsy in adults: expert opinion in China

ObjectiveThe goal of this study was to survey a group of epileptologists in China regarding the treatment of adult epilepsy.MethodsA questionnaire on treatment of adult epilepsy was sent to a group of opinion leaders in the field of epilepsy.ResultsFor initial monotherapy for idiopathic generalized epilepsy (IGE), valproate was rated as the treatment of choice. In symptomatic localization-related epilepsy (SLRE)/simple partial seizures and SLRE/complex partial seizures, carbamazepine and oxcarbazepine were the respective treatments of choice, whereas in SLRE/secondarily generalized tonic–clonic seizures, carbamazepine, lamotrigine, and oxcarbazepine were treatments of choice. For women who were pregnant or trying to conceive, lamotrigine was the treatment of choice for both IGE and SLRE. In people with epilepsy who were HBsAg positive, whether liver function was normal or not, topiramate and levetiracetam were treatments of choice for IGE. Valproate and levetiracetam were treatments of choice for seizures in the emergency department.ConclusionA high level of consensus was reached on most treatments of choice and first-line treatments for patients with epilepsy, which were in accordance with published US expert opinion.     

Classification of epilepsy syndromes and role of genetic factors

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

Photosensitivity in epileptic syndromes of childhood and adolescence.

PURPOSE: Photosensitivity, a reaction of the brain to external photic stimulation, can be graded from 1 to 4, and is most frequently seen in the first decades of life. This study investigated photosensitivity in children with epilepsy. METHODS: A retrospective study performed in the neuropaediatric department of the largest paediatric hospital in Kiel, treating patients at all medical care levels. The clinical data and EEG records of 566 patients with the most common epileptic syndromes were analyzed, in particular regarding photosensitivity. Their EEGs included application of intermittent light stimulation using standard techniques at twice the minimum. RESULTS: The proportion of photosensitive patients was significantly higher in the paediatric cohort than in adult patients, as published in the literature: 46% of patients with generalized epilepsies showed photosensitivity as compared to 20% with focal epilepsies. Photosensitivity was more common in idiopathic generalized epilepsy (IGE), (epilepsy with grand mal on awakening, 74%; juvenile absence epilepsy, 56%; juvenile myoclonic epilepsy, 50%; childhood absence epilepsy, 44%) than in focal types (idiopathic partial - Rolandic epilepsy, 23%; symptomatic/cryptogenic type of epilepsy, 16%), while in patients who experienced occasional seizures (neonatal/febrile seizures), this ranged between 40% and 23%, respectively. The generalized photoparoxysmal response, (PPR), grades 3 and 4 were found significantly more often in patients with IGE (92%) than in patients with focal epilepsies. Finally, the female preponderance was confirmed (37% to 27% of all epilepsies). CONCLUSIONS: Photosensitivity can be detected both in patients with IGE, with idiopathic and symptomatic/cryptogenic types of focal epilepsies, and with epileptic (occasional) seizures. PPR grades 3 and 4 are the most common in IGE.     

Valproate in children with newly diagnosed idiopathic generalized epilepsy

Holland KD, Monahan S, Morita D, Vartzelis G, Glauser TA. Valproate in children with newly diagnosed idiopathic generalized epilepsy.
Acta Neurol Scand: 2010: 121: 149–153.
© 2009 The Authors Journal compilation © 2009 Blackwell Munksgaard. Objectives – Sparse information on dose–response characteristics for initial antiepileptic drug monotherapy in children with idiopathic generalized epilepsy (IGE) is available. The aim of this study is to characterize the therapeutic dose of valproate in children with newly diagnosed IGE. Materials and methods – Effect of initial valproate monotherapy and doses associated with seizure freedom were examined in consecutive children with IGE identified from a New Onset Seizure Clinic. Results – Of 84 patients identified, 48 (57%) became seizure‐free on valproate monotherapy and another 10 patients became seizure‐free but discontinued VPA because of adverse effects. The mean dose in seizure‐free children was 15.7 mg/kg/day and over 95% of IGE patients will respond below 25 mg/kg/day. Conclusions – Half of children became seizure‐free on valproate monotherapy and did so at modest doses.     

Epilepsy in severe motor and intellectual disabilities syndrome (SMIDS)--a clinical and electroencephalographic study of epileptic syndromes

The subjects were 106 SMIDS with epilepsy. They were classified into four epileptic syndromes: (1) SE-MISF (34.0%), (2) SGE (25.5%), (3) SLRE (20.7%), and (4) epileptic discharge-free patients (EDFP) (19.8%). Clinical electroencephalographic studies elucidated the following result: (1) The seizure disappearance rate was the highest in SLRE (54.5%), and it decreased in the order of EDFP (47.6%), SE-MISF (36.1%), and SGE (11.1%). (2) Status epilepticus was most frequently seen in SGE (62.4%), but it was not so often seen in EDFP (14.3%) or SLRE (22.7%). (3) The age at seizures onset was the lowest in SE-MISF (0.84 years), and it increased in the order of SLRE (1.3), SGE (2.3), and EDFP (6.7). (4) The rate of Ohshima's classification 1 was highest in SE-MISF (61.1%) and lowest in SGE (40.7%). In conclusion, epileptic syndrome and EEG findings are good indicators for predicting the seizure prognosis and some of the clinical features, and the majority of epileptic syndromes could be classified by the very first EEG findings. Since epilepsy in SMIDS is so frequent (70.3%) and intractable (seizure disappearance rate more than 3 years, 36.2%), more attention should be paid to electroencephalography and epileptic seizures in SMIDS.     

Epidemiology of childhood epilepsy in a cohort of 309 Chinese children

The present study describes the characteristics of epilepsy in a cohort of Chinese epileptic children. All children with active epilepsy who were under 15 years of age and residing in the catchment area of Tuen Mun Hospital, Hong Kong were monitored. Etiology, seizure types, and epilepsy syndromes were classified according to the recent guidelines of the International League Against Epilepsy. A total of 309 children were recruited into the study. The etiology of epilepsy was idiopathic in 42% of the children, cryptogenic in 16.8%, and remote symptomatic in 40.8%. Perinatal factors were the most frequently found cause of epilepsy. Seizure types were partial in 48.5% of the children and generalized in 46.9%. Epilepsy syndromes could be classified in all but seven patients, with 48.2% localization related and 49.5% generalized. Generalized seizures were more prevalent in children less than 5 years of age. Additional neuroimpairments affected 36% of our epileptic children. Sixty-nine percent of patients were seizure free for more than 1 year. The authors conclude that the International League Against Epilepsy can be applied successfully to a population-based cohort of Chinese epileptic children. A larger, longitudinal epidemiologic study is needed to answer questions concerning the true prevalence, incidence, types, and etiologies in the Chinese population.     

Familial Occurrence of Epilepsy in Children with Newly Diagnosed Multiple Seizures: Dutch Study of Epilepsy in Childhood

Summary: Purpose: To study the familial occurrence of epilepsy in children with newly diagnosed multiple unprovoked seizures. Methods: Between August 1988 and September 1992, 462 children with two or more unprovoked seizures were included in the prospective Dutch Study of Epilepsy in Childhood. Seizures and epilepsy syndromes of probands were classified according to the International Classifications. Probands with at least 1 first-degree relative with epilepsy were selected. Seizures and syndromes of their relatives were classified using medical files and telephone interviews. Results: In 42% of the probands, the epilepsy was classified as localization-related, in 57% as generalized, and in 1% as undetermined whether focal or generalized. The 47 (10.2%) children with at least 1 first-degree relative with epilepsy less frequently had localization-related epilepsy (23%) and more often had generalized epilepsy (77%) as compared with the total group of probands. Fifty-eight first-degree and 21 other relatives had epilepsy. Thirty-three of the 40 (83%) first-degree relatives with idiopathic or cryptogenic epilepsy had the same seizure type as the proband, but detailed information about their seizures was sometimes difficult to obtain. Of the 12 first-degree relatives with epilepsy syndromes classifiable according to the International League Against Epilepsy (ILAE) 7 (58%) had the same syndrome as the proband. Conclusions: In 10% of children with newly diagnosed epilepsy, the condition is familial. Relatively more often, these children have generalized epilepsy syndromes as compared with children with a negative family history. Most of the relatives with idiopathic or cryptogenic epilepsy had the same sei- zure type as the proband. These findings confirm the role of genetic factors in the pathogenesis of epilepsy.     

The Syndromic Classification of the International League Against Epilepsy: A Hospital-Based Study from South India

Summary: Purpose: To determine the distribution of various epilepsies and epileptic syndromes in the epileptic population treated in a university hospital in a developing country. Methods: Data concerning 2,531 patients with epilepsy seen between January 1989 and June 1994 were analyzed using the international League Against Epilepsy (ILAE) classification. Results: Of 2,531 cases, 48% fell into ILAE categories 1.3, 3.2, or 4.1 (cryptogenic, without unequivocal generalized or focal seizures; or situation-related seizures, respectively). Localization-related epilepsies (LREs) and epileptic syndromes (1.1, 1.2, 1.3) were found in 1,591 (62.9%) patients; of these patients, symptomatic localization-related epilepsies totaled 62.7%, and idiopathic localization-related epilepsies accounted for only 0.7%. Juvenile myoclonic epilepsy was the most common type of idiopathic generalized epilepsy (IGE), comprising 4.9% of the total study population and 7.7% of patients registered in the epilepsy clinic. A combination of childhood and juvenile absence epilepsies were found in only 0.4% of the total study population. Single computed tomography (CT) enhancing lesion (SCTEL) and focal cerebral calcification (FCC) accounted for 22% of the etiologic factors for localization-related epilepsies. Neurologic deficits were found in 9.5% of patients with SCTEL; none were found with FCC. None of the patients with these lesions had any history of antecedent events that suggested CNS involvement. In patients with localization-related epilepsies with unremarkable clinical data, the proportion of CT scans showing SCTELs was 39 (95% confidence interval [CI], 0.35–0.43) and 0.18 (95% CI, 15–0.21) for FCCs. The proportion for both lesions together was 0.57 (95% CI, 0.53–0.61). Seizures did not recur once the lesion resolved in patients with SCTELs. In patients with FCCs, seizure remission was 71.5% (95% CI, 53.7–85.4) at 3 years. Conclusions: This study illustrates the rarity in one patient population of some of the syndromes and categories described in the ILAE classification. Childhood and juvenile absence epilepsies together formed a small proportion. SCTEL and FCC were important etiologic factors for localization related epilepsies. The epilepsy associated with SCTEL was a form of benign epilepsy; epilepsy associated with FCC had remission rates similar to other remote symptomatic epilepsies. Without neuroimaging evidence, these 2 lesions would have been missed and the patients might have been grouped under cryptogenic localization related epilepsy. For this reason, we emphasize the need for neuroimaging in patients with localization related epilepsies with unremarkable clinical findings, before classification into the cryptogenic category. In the absence of neuroimaging, such patients should be classified as “probably cryptogenic.”     

Generalized spike-and-wave patterns in children: clinical correlates

All electroencephalograms performed in our institution between 1980 and 1990 were reviewed. The clinical characteristics of children with epilepsy and generalized spike-and-wave (SW) patterns were analyzed. The SW patterns were classified according to their frequency. Electroencephalograms of 154 children with epilepsy revealed SW patterns. Absence seizures were the most common first seizure, but partial seizures were frequent. More than 40% had several types of seizures. Sixty percent of the epileptic syndromes were generalized, but almost 25% were partial. The typical SW pattern was associated with absence seizures, a normal examination and computed tomographic scan, idiopathic generalized epilepsies, monotherapy, freedom from seizures, and lack of recurrence. The slow SW pattern was associated with West syndrome; a younger age at seizure onset; atonic, myoclonic, tonic, and partial simple seizures; an abnormal examination and computed tomographic scan; cryptogenic or symptomatic generalized epilepsy or symptomatic partial epilepsy; polytherapy; and poor seizure control. The fast SW pattern was associated with secondary generalized, partial, tonic-clonic, and complex partial seizures; a normal computed tomographic scan; cryptogenic partial epilepsy; isolated seizures; and seizure recurrence. Epilepsy with a typical SW pattern should be considered benign, epilepsy with a slow SW pattern malignant, and epilepsy with a fast SW pattern treacherous.     

Entorhinal cortex MRI assessment in temporal,extratemporal, and idiopathic generalized epilepsy

PURPOSE: We previously showed a reduction in the volume of the entorhinal cortex (EC) ipsilateral to the seizure focus in patients with intractable temporal lobe epilepsy (TLE). The purpose of this study was to examine the specificity of EC atrophy in epilepsy. METHODS: We performed volumetric measurement of the EC on high-resolution magnetic resonance imaging (MRI) in patients with TLE (n = 70), extratemporal lobe epilepsy (ETE; n = 18), and idiopathic generalized epilepsy (IGE; n = 20). EC volumes of epilepsy patients were compared with those of 48 age- and sex-matched normal controls. Within the TLE group, 63 patients were selected prospectively with hippocampal atrophy ipsilateral to the seizure focus. The remaining seven patients were chosen retrospectively based on normal volumetric MRI of the hippocampus and amygdale, as well as normal histopathologic examination of the resected tissue. RESULTS: Compared with normal controls, EC volume was smaller ipsilateral but not contralateral to the seizure focus in patients with TLE (p < 0.001). No difference in the EC volumes ipsilateral and contralateral to the seizure focus was seen in patients with ETE and IGE compared with normal controls. The individual analysis showed that the EC was atrophic in 73% of TLE patients with hippocampal atrophy. Three of the seven TLE patients with normal volumetric MRI of the hippocampus and amygdala and normal histopathologic examination had EC atrophy ipsilateral to the seizure focus. In no patient with ETE or IGE was the EC found to be atrophic. CONCLUSIONS: EC atrophy ipsilateral to the seizure focus appears to be specific to mesial temporal lobe structural damage associated with TLE.     

Prevalence, classification, and severity of epilepsy in children in western Norway

PURPOSE: To determine prevalence of active epilepsy in school children in a defined area and assess the usefulness of International League Against Epilepsy classification of seizures and epileptic syndromes, with special emphasis on frequency, additional handicaps, and therapeutic problems of severe cases. METHODS: The latest International League Against Epilepsy International Classification of Epileptic Seizures (ICES, 1981) and Epilepsies and Epileptic Syndromes (ICE, 1989) were used for determination of prevalence rates, seizure types, epilepsies and epileptic syndromes, and additional neurological deficits in all 6-to 12-year-old children with epilepsy in a Norwegian county. Children had neuropediatric and EEG examination, intelligence evaluation, and, when necessary, additional investigations. RESULTS: Prevalence of active epilepsy on January 1, 1995, was 5.1 per 1,000. Main seizure type and epilepsy syndrome could be classified in 98% and 90% of patients, respectively. Seizure types/epileptic syndromes were more often partial/localization related than generalized. Among generalized epilepsies, idiopathic forms were more frequent in girls, and cryptogenic and symptomatic forms more frequent in boys. Epileptogenic EEG activity was most often generalized or localized to one or two areas of the brain and was never found in 14% of patients. Symptomatic etiology was found in 46% of all children and in 81% of therapy-resistant cases, respectively. Over the years, 11% of children had never used antiepileptic drugs (AED), 62% had tried one or two AEDs, and 26% had tried from three to 15 AEDs. Twenty-five percent of children were without present AED treatment. Complementary/alternative medicine had been tried by 12% of children. CONCLUSIONS: Although most epilepsies could be classified, the number of cases in non-specific categories was relatively high. Symptomatic etiology was frequent, especially in therapy-resistant cases. Multidisciplinary therapeutic and habilitation approaches are often needed in childhood epilepsy.