March 2006
Prevalence of Childhood Epilepsy and Distribution of Epileptic Syndromes: A Population-based Survey in Okayama, Japan
Eiji Oka, Yoko Ohtsuka, Harumi Yoshinaga, Nagako Murakami, Katsuhiro Kobayashi, and Tatsuya Ogino

Epilepsy consists of various types of clinical seizures and epileptic syndromes. Therefore, epidemiological findings are important not only for the public health but also clinical practice. However, the appearance rate or distribution of various types of epilepsies and epileptic syndromes has not been clarified. We carried out a population-based survey of childhood epilepsy within a large population in Okayama Prefecture, Japan. A total of 2,220 cases were identified from a background population of 250,997. The prevalence rate was 8.8 per 1,000. If we exclude patients who had experienced a single seizure or seizures occurring during febrile episodes, the prevalence rate was 5.3 per 1,000. Of the 2,220 cases, 2,030 (91.4%) were classified into three major categories by ILAE Classification. They consisted of 1,556 cases (76.7%) with localization-related epilepsy, 453 (22.3%) with generalized epilepsy, and 21 (1.0%) with undetermined epilepsy. A total of 309 of the 2,030 cases (15.2%) were classified into epileptic syndrome categories, and 84.8% of the total were nonspecific types of epilepsy. Epilepsia 2006;47(3).

Physical Activity In Children/Teens With Epilepsy Compared To Their Siblings Without Epilepsy

Judy Wong and Elaine Wirrell

e determined (a) if children and teens with epilepsy participate in less physical activity and have higher body mass index percentiles for age than their non-epileptic siblings and (b) what epilepsy-specific factors limit their participation. Cognitively normal children and teens 5-17 years, with a >3 month history of epilepsy, no major motor or sensory impairments, and at least one non-epileptic sibling in a similar age range who attended neurology clinic at the Alberta Children’s Hospital were identified. Parents completed a questionnaire regarding sedentary activities and group, individual and total sports activities. Children aged 11-15 years also completed the physical activity portion of the Health Behavior in School Aged Children questionnaire. Clinic charts were reviewed for seizure type, etiology, frequency, duration of epilepsy and number of anti-epileptic drugs ever taken. Teens with epilepsy participated in less group and total sports activities than their siblings without epilepsy and were more likely to be potentially overweight or overweight. Those who received three or more anti-epileptic medications in the past were less active. While there was a trend for those with higher seizure frequency to be less active, no other epilepsy specific factors nor prior seizures or seizure-related injury during a sports event correlated with participation in physical activity. Programs that promote exercise in adolescents with epilepsy should be encouraged to improve their physical, psychological, and social well-being. Epilepsia 2006;47(3).

Family Predictors of Psychopathology in Children with Epilepsy

Hesther Rozemarijn Rodenburg, Anne Marie Meijer, Maja Dekovic, and Albert P. Aldenkamp

Research on children with epilepsy indicates that family factors contribute to child psychopathology. Because child developmental research demonstrated that different types of family factors play different roles in relation to child outcome, the aim of this study was to examine the simultaneous contribution of distinct family factors to child psychopathology. These distinct family factors were differentiated according to their level of proximity to the child’s everyday life. As such, proximal family factors (parent-child relationship quality), distal family factors (parental characteristics), and contextual family factors (other family relationships) were discerned. Examining the relative contribution of each of these family factors and epilepsy-related factors (onset and severity) to the full range of psychopathology (measured with the Child Behavior Checklist) in 91 children with epilepsy and normal intelligence revealed that proximal family factors were most influential in explaining child psychopathology, whereas epilepsy-related factors were non-significant. Moreover, it was found that the effects of distal and contextual family factors influence child psychopathology via the proximal family factors. The article discusses the results regarding any possible causal relationships between family factors and child psychopathology and the implications for clinical practice. Epilepsia 2006;47(3).

Autonomic Symptoms during Childhood Partial Epileptic Seizures

András Fogarasi, József Janszky, and Ingrid Tuxhorn

Autonomic (vegetative) symptoms happen frequently during epileptic seizures. They can help us better understand the central representation of the autonomic nervous system and some of them can add further information to the assessment of the epileptogenic focus, which is especially useful in presurgical evaluation of intractable epilepsy. In this study, we systematically analyzed the occurrence and age-dependency as well as the localizing and lateralizing value of ictal autonomic symptoms during 514 video-recorded seizures of 100 children with partial epilepsy and compared our results with those of earlier adult studies. Sixty patients produced at least one vegetative sign; more frequently in patients with temporal (70%) than with extratemporal (44%) lobe epilepsy. Apnea/bradypnea happened rather in younger children, while the presence of other ASs was neither age- nor gender-related. Postictal coughing and epigastric aura localized to the temporal lobe, while no ASs lateralized the seizure onset zone. Summarized, our study showed that autonomic symptoms are common in childhood focal epilepsies appearing already in infants and young children. Similarly to adults, childhood central autonomic networks might have a close connection to temporal lobe structures but do not lateralize the seizure onset zone. Epilepsia 2006;47(3).

Early Pattern of Epilepsy in the Ring Chromosome 20 Syndrome

Dorothée Ville, Anna Kaminska, Nadia Bahi-Buisson, Arnaud Biraben, Perrine Plouin, Louise Telvi, Olivier Dulac, and Catherine Chiron

The characteristics of epilepsy in ring chromosome 20 have been reported in adolescents and adults. The mode of onset most often remains unprecise. In order to clarify this onset period, we studied the early onset features in our personal series and in the reported pediatric cases. Our series comprises one child with an onset of epilepsy in the neonatal period and five others with an onset before eight years of age. Case studies with an epilepsy onset before eight years from the literature were also reviewed. Seizures in the neonatal period were described as motor seizures. Our personal patient with a neonatal onset had severe psychomotor delay. In both infancy and early childhood, no long lasting seizure was recorded. Seizures with terror and hallucinations usually appeared from about four years of age. It is not before the age of eight years that the usual EEG pattern described in adolescence and adulthood appeared, but between four and six years of age, a particular EEG pattern with very slow waves and spike and waves predominating in frontal areas, has been described. In ring 20 chromosome, specific epilepsy features are lacking in the neonate, but the whole phenotype is particular, with a more severe expression in terms of mental delay. The typical EEG pattern does not appear before the age of eight years. Epilepsia 2006;47(3).

Analysing the Etiology of Benign Rolandic Epilepsy: A Multi-center Twin Collaboration

Lata Vadlamudi, Marianne J. Kjeldsen, Linda A. Corey, Marit H. Solaas, Mogen L. Friis, John M. Pellock, Karl O. Nakken, Roger L. Milne, Ingrid E. Scheffer, A. Simon Harvey, John L. Hopper, and Samuel F. Berkovic

Benign rolandic epilepsy (BRE) is a common childhood epilepsy and considered to be genetically determined. In BRE children or young adults around the age of puberty tend to have partial seizures affecting the face or hand, especially when falling asleep. The seizure focus is located around the motor (Rolandic) area of brain. BRE is called benign because of the propensity to outgrow the seizures. Here we analysed a large sample of twins from four international twin registers to understand the genetics of BRE. Large population-based twin registries of epilepsies from Odense (Denmark), Richmond (USA) and Oslo (Norway) were reviewed for BRE cases and added to our Australian twin data. Diagnosis of classic BRE was based on characteristic electroencephalogram and clinical criteria with normal neurological development. Cases with a compatible electroencephalogram and clinical criteria but abnormal neurological development were termed non-classic BRE. There were eighteen twin pairs identified (10 monozygous; 8 dizygous) where at least one twin was diagnosed with classic BRE amongst a total sample of 1952 twin pairs validated for seizures, and all were discordant (only one twin within the twin pair had BRE). There were four twin pairs (1 monozygous, 3 dizygous) with non-classic BRE and all co-twins had seizures.The twin data showing an absence of any concordant twin pairs with classic BRE suggest that non-inherited factors are of major importance in BRE. Genetic factors are probably more important in non-classic BRE. The etiology and mode(s) of inheritance of BRE are much more complicated than initially conceptualized. Epilepsia 2006;47(3).

February 2006
Seizure Recurrence and Risk Factors after Anti-epilepsy Drug Withdrawal in Children with Brain Tumors
Raja B. Khan and Arzu Onar

Brain tumors are the second most common tumor in children, after acute leukemia. Approximately 20% of children with brain tumors will develop seizures. The majority of these patients will achieve seizure control with appropriate medical therapy. Until now, risk for seizure recurrence was not known after discontinuation of seizure medications in brain tumor patients. We report rate and predictors of seizure recurrence in 62 children with brain tumors, in whom seizure medications were withdrawn. Based on our data, the estimated risk of seizure recurrence is 18% at two years and 27% at five years. We found that risk of seizure recurrence increased substantially if the child was treated with whole brain radiation therapy and required more than one brain surgery to treat the tumor. Younger age at tumor diagnosis and initial difficulty in controlling seizures may also increase the risk of seizure recurrence after medication withdrawal. Our data shows that the risk of seizure recurrence in childhood brain tumor patients is comparable to that in children with medically controlled seizures without brain tumors. We also show that seizure medications can be successfully withdrawn in carefully selected patients with childhood brain tumors and controlled seizures. Epilepsia 2006;47(2).

Epilepsy in Menkes Disease: Analysis of Clinical Stages

Nadia Bahi-Buisson, Anna Kaminska, Rima Nabbout, Christine Barnerias, Isabelle Desguerre, Pascale De Lonlay, Michele Mayer, Perrine Plouin, Olivier Dulac, and Catherine Chiron

Menkes disease (MD) is a rare X-linked recessive disorder with a primary generalized defect in copper transport. It results in a copper deficiency and subsequent impairment of activity of multiple copper dependent enzymes. Epilepsy is one of the main features of MD. In order to better determine the spectrum of epilepsy, we retrospectively analyzed the evolution of electroclinical features of 12 patients with confirmed MD based on clinical charts. Epilepsy could be divided into three successive periods. First, an early stage (median age 3 months) characterized by focal clonic status epilepticus, usually triggered by fever (10 patients). Ictal EEG showed runs of slow spike-waves and slow waves in the posterior regions, and interictal EEG multifocal and polymorphic slow waves (3 cases), or mixed slow spike-waves and slow waves (7 cases). Partial seizure control was obtained in 9 patients during 5.9 months. The second period or intermediate stage (median age 10 months) was characterized by intractable infantile spasms (11 patients) in which interictal EEG demonstrated modified hypsarrhythmia (7 cases), diffuse irregular slow waves and spike-waves (4 cases). Six patients died at the median age of 15 months. The third period or late stage in the 6 remaining patients (median age 25 months), was characterized by multifocal seizures, tonic spasms and myoclonus in 4 patients whereas 2 patients became seizure-free. Interictal EEG showed multifocal high amplitude activity, mixed with irregular slow waves in all 6 cases. These patients died at the median age of 3.6 years. Based on a relatively large series of MD patients with a quite prolonged survival, we individualized three successive periods in the course of epilepsy: early focal status, followed by infantile spasms, and then myoclonic and multifocal epilepsy after 2 years of age. Epilepsia 2006;47(2).

Benign Myoclonic Epilepsy in Infants: Electroclinical Features and Long-term Follow-up of 34 Patients.

Auvin Stéphane, Pandit Florence, De Bellecize Julitta, Badinand Nicole, Isnard Hervé, Motte Jacques, Villeneuve Nathalie, Lamblin Marie-Dominique, Vallée Louis, and the Epilepsy Study Group of the French Pediatric Neurology Society

Benign myoclonic epilepsy in infants (BMEI) is a rare epileptic syndrome characterized only by generalized myoclonic seizures (MS) in normal children during the first two years. Our aim was to assess the follow-up of this syndrome. Methods: BMEI was confirmed by electroencephalogram (EEG) in four neuropediatric units in France between 1981 and 2002. Clinical and electroencephalographic findings at diagnosis and during the follow-up were collected. The Vineland scale and/or Wechsler scale were used to perform neuropsychological evaluations. Results: We report 34 patients. BMEI is clinically characterized by myoclonic seizures involving the upper part of the body occurring many times a day. The ictal EEG showed a generalized discharge of polyspike, polyspike and wave or spike and wave. The interictal EEG was usually normal. Reflex MS were frequently observed and could suggest the diagnosis. A family history of febrile seizures (FS) or epilepsy was noted in six patients. A history of FS was noted in 11 patients. Monotherapy with valproate acid was effective in 23/30 treated patients. Four patients presented seizures after the initial symptoms. Juvenile myoclonic epilepsy developed in two patients and cryptogenic partial epilepsy in another. Neuropsychological outcome was evaluated in 20 patients (10 with Wechsler scales and 17 with Vineland scale). Cognitive functions were normal in 17 patients, while developmental delay was observed in three others.Despite a generally favorable neuropsychological outcome, cognitive impairment and developmental delay can be observed more frequently than in the general population. Further prospective studies are needed to better understand the existence of factors that contribute to these conditions. Epilepsia 2006;47(2).

Absence of Seizures Despite High Prevalence of Epileptiform EEG Abnormalities in Children with Autism Monitored in a Tertiary Care Center

Howard L. Kim, Joseph H. Donnelly, Anne E. Tournay, Teri M. Book, and Pauline Filipek

Seizures and EEG abnormalities are more common in children with autism than normal children, but the relationship is poorly understood. We studied 32 children with autism using prolonged video-EEG (VEEG) recording; twenty two of these children were suffering from recurrent seizures. The VEEG evaluations revealed that the majority of these children had epileptiform EEG abnormalities. These types of abnormalities can occur in children with epilepsy, or who have a higher risk for having epileptic seizures. However, in our study, these EEG abnormalities occurred in 60% of children who did not have seizures. Of the children who had their “seizures” recorded on VEEG, none of them were epileptic, even though 59% of these children had epileptiform EEG abnormalities. Most of the recorded “seizures” were staring spells, resembling absence seizures or bland complex partial seizures. The findings of our study demonstrate the peril of making a clinical diagnosis of epilepsy in children with autism. Our study also highlights the important role of video-EEG monitoring in establishing an accurate diagnosis of epilepsy in these children. Epilepsia 2006;47(2).

Thalamic Atrophy in Childhood Absence Epilepsy

Chow Huat (Patrick) Chan, Regula S. Briellmann, Gaby S. Pell, Ingrid E. Scheffer, David F. Abbott, and Graeme D. Jackson

Childhood absence epilepsy (CAE) is a common childhood epilepsy with frequent, but very short absence seizures. Patients with CAE have no abnormalities of their brain structure. Seizures may be caused by a functional disturbance of brain networks, prominently involving the cortex and the thalamus. We used automated voxel-by-voxel statistical assessment to assess for subtle volume differences of gray and white matter of cortex and thalamus. We recruited 13 patients with a clinical and EEG diagnosis of CAE (mean age at examination 17 ±8 years), and compared them to a consecutive series of 109 controls (mean age 29 ±9 years). Each subject performed a high-resolution magnetic resonance scan, which was analysed with an optimised protocol for voxel-based volume assessment. Compared to controls, CAE patients showed areas of grey matter decrease in the thalamus as well as less pronounced in other structures. White matter decrease was found around the thalamus, and in the white matter of the forebrain. Grey and white matter increase was restricted to small areas. Therefore, there is evidence of thalamic volume reduction in CAE patients. Bilateral thalamic atrophy may either be a result of damage from seizures or a reflection of a primary underlying pathology as the cause of absence seizures. Epilepsia 2006;47(2).

Postsurgical Outcome in Pediatric Patients with Epilepsy: A Comparison of Patients with Intellectual Disabilities, Sub-average Intelligence, and Average-range Intelligence

Ulrike Gleissner, Hans Clusmann, Robert Sassen, Christian E. Elger, and Christoph Helmstaedter

The chances of becoming seizure free after focal epilepsy surgery are often considered to be worse in developmentally delayed patients because intellectual disabilities frequently are associated with bilateral or diffuse morphologic brain damage thus increasing the risk of multiple epileptic foci. Moreover, the risk of postoperative cognitive deficits could increase since diffuse brain damage lowers the patient’s ability to compensate for surgically induced deficits. Recently, several studies in adult patients have indicated that the intelligence quotient (IQ) alone is not a good predictor of postoperative cognitive and seizure outcome but there is no study specifically evaluating this subject in children and adolescents. In our study, we compared pediatric patients with intellectual disabilities (IQ ? 70), sub-average intelligence ( IQ between 71 and 85), or average-range intelligence (IQ > 85) who were matched according to several clinical and etiological criteria to determine the isolated impact of IQ. The results indicated no dependency of seizure outcome, postoperative cognitive development, and behavioral outcome on the IQ level. Between 67 and 78% were seizure free one year after surgery. School placement remained unchanged for the majority of patients. We conclude, that IQ alone is not a good predictor of postoperative outcome in pediatric patients with epilepsy. As with patients of average-range intelligence, the decision to operate on intellectually disabled patients should depend on the results of the presurgical diagnostics. Diffuse functional cognitive impairment should not hinder further steps, if all other findings are consistent. Epilepsia 2006;47(2).

School Performance in Nigerian Adolescents with Epilepsy

Abiodun O. Adewuya, Abiodun O. Adewuya, and John A.O. Okeniyi

This study assesses the school performance, over the past one academic year, of 73 Nigerian adolescents with epilepsy, compared with their healthy classmates matched for age, gender, socio-economic status and presence of psychopathology. Results indicated that the mean school grades of adolescents with epilepsy are significantly lower than their healthy controls in all the subjects. The variables that significantly predict poor school performance of adolescents with epilepsy include psychopathology in the caretaker; adolescents’ perceived poor family functioning; adolescents’ attitude towards the illness; adolescents’ felt stigma; externalising symptoms in the adolescents and duration of illness. It is evident that the determinants of poor school performance in Nigerian adolescents with epilepsy are multivariate, with psychosocial factors most important. These should be noted for early identification and screening of those children at greatest risk for academic failure and the need for appropriate educational remediation services. Epilepsia 2006;47(2).

January 2006
Hippocampal Sclerosis in Children with Lesional Epilepsy is Influenced by Age of Seizure Onset
Catherine J. Riney, Brian Harding, William J.F. Harkness, Rod C. Scott, and J. Helen Cross

Hippocampal sclerosis (HS) is a condition where there is cell loss in a part of the temporal lobe of the brain called the hippocampus. HS leads to seizures that are difficult to control. The cause of HS is unclear. It may be due to the hippocampus not forming correctly during development. Or it may be due to damage to the hippocampus. For example, children who have a long seizure caused by high temperature may later develop HS. We used information from a group of 29 children with seizures from areas of abnormal brain outside the temporal lobe (extra-temporal lesions) to look further at the question of what causes HS. The extra-temporal lesions were removed surgically (to cure seizures) and the hippocampus was removed at the same time. Both areas were studied by a pathologist. In total, 21 of 29 (72%) children had HS. HS was seen both in those with abnormal brain development and in those with brain damage as the cause of their extra-temporal lesion. This suggests that HS is caused by the seizures spreading from the extra-temporal lesion, as this is the common phenomenon when the cause of the extra-temporal lesion is different (development or damage). The other interesting finding of this study is that children who were younger at the time that they had their first seizure were less likely to develop HS. Why they are protected in this manner is an area that needs further research. Epilepsia 2006;47(1).

Hippocampal and Thalamic Diffusion Abnormalities in Children with Temporal Lobe Epilepsy

Tomomi Kimiwada, Csaba Juhász, Malek Makki, Otto Muzik, Diane C. Chugani, Eishi Asano, and Harry T. Chugani

Magnetic resonance imaging (MRI) is a non-invasive diagnostic method to evaluate structural abnormalities underlying epileptic processes in the human brain. Diffusion tensor imaging (DTI) is a relatively novel MRI technology to measure changes in cerebral water diffusion. DTI can measure (1) the magnitude (quantified by the apparent diffusion coefficient, ADC) and the directional preference (characterized by the fractional anisotropy, FA) of the diffusional motion of water molecules. Previous studies in adults with temporal lobe epilepsy (TLE) showed DTI abnormalities in the epileptic hippocampi, even when other, conventional MRI techniques failed to provide accurate localizing information. In the present study, DTI was performed in 14 children with unilateral TLE. FA and ADC values were measured in the hippocampus, as well as in the thalamus and lentiform nucleus, two subcortical structures commonly involved in seizure propagation. Increased ADC values were found in the hippocampi on the same side as the seizure foci, while mean FA was decreased in the hippocampi on both sides, although lower on the side of the epileptic focus. In addition, a trend for increased ADC values was found in the thalami on the side of the epileptic foci in children with generalized seizures. The findings suggest that, in children with temporal lobe epilepsy, DTI can detect abnormalities in some brain structures that are commonly involved in generation and propagation of seizures originating from the temporal lobe, even if these structures appear normal on conventional MRI. Thus, DTI can assist localization of temporal lobe seizure foci in some children with TLE. Epilepsia 2006;47(1).

Efficacy of Dexamathasone on cerebral Swelling and Seizures during Subdural Grid EEG Recording in Children

Takashi Araki, Hiroshi Otsubo, Yuko Makino, Irene Elliott, Koji Iida, Ayako Ochi, Shelly K. Weiss, Sylvester H. Chuang, James T. Rutka, and O. Carter Snead III

Patients with epilepsy who have a presurgical-evaluation procedure called subdural grid electroencephalography (SGEEG) sometimes experience brain swelling. Dexamethasone is used to reduce this swelling; however, the steroid can also reduce the occurrence of seizures that SGEEG is trying to measure, so that the duration of the procedure and the risk of complications increase. We wanted to evaluate the impact of dexamethasone treatment on brain swelling and seizures during SGEEG monitoring. We reviewed data from 37 children with epilepsy who underwent SGEEG before surgery. Some patients had received dexamethasone before the procedure as a preventative to swelling and some had not. We observed patients’ CT scans for brain swelling after subdural grid placement and measured the frequency of seizures during SGEEG. We then correlated this information with whether or not patients received initial dexamethasone. Twenty-three patients received dexamethasone from the first day of SGEEG; only two had brain swelling. Fourteen patients received no initial dexamethasone; six had swelling. Of the 37 patients, seizures were recorded in 36; one of the two dexamethasone-treated patients with swelling experienced neurological deterioration, and we were unable to record this patient’s seizures. SGEEG seizure recording took longer in those who had dexamethasone than in those who did not. Our study showed that dexamethasone treatment in these patients reduced brain swelling during SGEEG. Although dexamethasone decreased seizure frequency and increased monitoring time, this steroid reduced the risk of complications from brain swelling during SGEEG. Epilepsia 2006;47(1).

Long-term Follow-up of Patients with Benign Partial Epilepsy in Infancy

Akihisa Okumura, Kazuyoshi Watanabe, Tamiko Negoro, Fumio Hayakawa, Toru Kato, Koichi Maruyama, Tetsuo Kubota, Motomasa Suzuki, Hirokazu Kurahashi, and Yoshiteru Azuma

Benign partial epilepsy in infancy (BPEI) is infantile focal epilepsy with very favorable outcome. We studied the long-term outcome of children with BPEI. A telephone interview survey was conducted with patients who were diagnosed as having possible BPEI at 2 years of age. The data from 39 of 48 patients were available. The median age at the time of the survey was 11.3 years. There were 18 boys and 21 girls.

Three patients had a recurrence of unprovoked seizures beyond two years of age. Four patients had cognitive problems (mild mental retardation in three and Asperger syndrome in one). An association of paroxysmal kinesigenic choreoathetosis, which is a disorder involving sudden involuntary twisting or stiffening movements) was observed in three patients, and another three experienced seizures associated with mild gastroenteritis. Major behavioral problems were not recognized in any patient. Eventually, 33 of 39 patients were categorized as having definite BPEI beyond 8 years of age. A large majority of patients diagnosed as possibly having BPEI at 2 years of age did not have a recurrence of unprovoked seizures and they also showed no more risk than normal for cognitive problems during school age through adolescence. These results confirm that BPEI truly is a benign epilepsy. Epilepsia 2006;47(1).

December 2005
The Prevalence of Autistic Spectrum Disorder in Children Surveyed in a Tertiary Care Epilepsy Clinic
Dave F. Clarke, Wendy Roberts, Mina Daraksan, Annie Dupuis, Jane McCabe, Halyey Wood, O. Carter Snead III, and Shelly K. Weiss

Autistic Spectrum Disorders (autism, and other pervasive developmental disorders) are a spectrum of disorders, characterized by impaired socialization, impaired communication, and repetitive, restricted patterns of behavior. Children with autistic spectrum disorder (ASD) have an increased prevalence of seizures; however, studies have not been done to evaluate the prevalence of ASD in children with epilepsy. This co-morbidity is important to define as early diagnosis and intervention in some children with ASD has been shown to improve outcome. Children with epilepsy seen in the epilepsy clinic at the Hospital for Sick Children in Toronto were evaluated using an autism screening questionnaire (ASQ). In addition, questions about sleep related problems, behavior, seizure characteristics, anti-epileptic medications, and weight were requested. An attempt was then made to determine if there was a relationship between the factors identified and a higher risk of autism found in some of these children. 97 questionnaires were properly completed and used in this study. Approximately 32% of children were found to be at risk of having autistic spectrum disorder. Most children had not previously been diagnosed. Worst behavior and increased daytime sleepiness was seen in those at greater risk, and seizures occurred at an earlier age (approximately two years in these children). Behavioral difficulties and daytime sleepiness identified in these children could potentially affect their ability to learn. Though confirmatory diagnostic evaluations are needed, this questionnaire-based study suggests that children with epilepsy are at greater risk of having ASD, and illustrates the need for more clinical vigilance. Epilepsia 2005;46(12).

Status Epilepticus and Frequent Seizures: Incidence and Clinical Characteristics in Pediatric Epilepsy Surgery Patients

Susan Koh, Gary W. Mathern, Gabrielle Glasser, Joyce Y. Wu, W. Donald Shields, Rinat Jonas, Sue Yudovin, Carlos Cepeda, Noriko Salamon, Harry V. Vinters, and Raman Sankar

This is a study based on a review of children who underwent surgery for status epilepticus (SE), which is defined as continuous seizures lasting more than 20 minutes in length or three seizures of any length within an hour. We looked at our database for all epilepsy surgeries performed from 2000-2005, and then divided these patients into three groups: a continuous SE group (where seizures lasted for more than 20 minutes in length), intermittent SE group (where there were three seizures of any length within an hour) and non-SE group. Then, we compared these patients based on several factors, including the disease that caused the seizures to occur, the type of surgery that they had, any complications that these patients had, and if the patients became seizure free. We found that continuous SE occurred in 5% of all our patients who underwent epilepsy surgery and intermittent SE in 15%. All of the SE patients had seizures originating from outside the temporal lobe. Compared with non-SE cases, the continuous and intermittent SE groups required a larger number of anti-seizure medications before surgery and six-months after surgery, underwent hemispherectomy (a type of epilepsy surgery where we remove approximately half of the brain) more frequently, and had an increased incidence of hemimegalencephaly (a type of malformation of the brain) and Rasmussen syndrome (a presumed auto-immune illness) and a lower occurrence of stroke and infections. Seizure control was over 71% up to two years after surgery, and there were no differences between patient groups. We concluded that children presenting with continuous or intermittent SE are not rare in pediatric epilepsy surgery centers, and such cases are more commonly associated with infantile spasms, Rasmussen’s syndrome, and hemimegalencephaly. Seizure outcome after surgery was not altered in children because they had presented with continuous or intermittent SE. Epilepsia 2005;46(12).

Taylor-type Focal Cortical Dysplasia in Infants: Some MRI Lesions Almost Disappear with Maturation of Myelination

Christin M. Eltze, Wui K. Chong, Sanjay Bhate, Brian Harding, Brian G. R. Neville, and J. Helen Cross

In young children with epilepsy that is not improving with medication, identification of localized areas of abnormally developed cortex (focal cortical dysplasia, FCD) on magnetic resonance (MR) brain scans is important. Surgical removal of abnormal cortex may offer improvement of seizure control and subsequent developmental progress. However the appearances of the abnormal cortex on MR images may change when the brain matures as children become older. We report four children with focal epilepsy resistant to medical treatment, whose MR brain scans in early childhood showed signal abnormalities (low intensity on T2 and high intensity on T1-weighted images) in localized areas of cortex and underlying white matter, that suggested abnormally developed cortex. The visibility of these lesions was more difficult on MR brain scans performed at a later age. The indicator of abnormal cortex in three patients was a subtle signal abnormality: blurring of the border between cortex and underlying white matter, which was only recognized after comparison with the earlier images. Taylor-type FCD (a type of abnormally developed cortex. defined by the appearance of the abnormal brain tissue under the microscope) was subsequently confirmed in all patients following surgical removal of the lesions. MR brain scans performed early within the first year of life in children with epilepsy are important to identify areas of abnormally developed cortex. The appearances on later scans can be very subtle, escaping recognition such that conclusions may be misleading with respect to diagnosis and appropriateness of surgical treatment. Epilepsia 2005;46(12).

Levetiracetam Efficacy in Epileptic Syndromes with Continuous Spikes and Waves during Slow Sleep: Experience in 12 Cases

Alec Aeby, Nathalie Poznanski Denis Verheulpen, Catherine Wetzburger, and Patrick Van Bogaert

Epileptic syndromes with continuous spikes and waves during slow sleep (CSWS) refer to childhood epilepsies characterized by strong activation of seizure-like activity during sleep, occupying at least 85% of non-dreaming sleep time, coupled with cognitive dysfunction. There is strong evidence that cognitive functioning may improve if epileptic activity is reduced using anti-epileptic drugs (AED). The goal of this study was to assess the add-on efficacy of levetiracetam on the EEG, behavior and cognition of children with CSWS. Twelve patients with behavioral or cognitive deterioration associated with the CSWS pattern received levetiracetam at 50 mg/kg/d as add-on treatment. After two months, seven patients (58.3 %) showed improvement of the EEG. together with improvement in behavioral or cognitive function. Two patients improved in terms of neuropsychological evaluation, despite the lack of EEG improvement. Eight patients (66.6 %) continued levetiracetam treatment after two months. After one year, four patients were still on levetiracetam, two because sustained effect on the EEG and behavior and the two others because of improvement in neuropsychological testing, despite an unchanged EEG. Levetiracetam was discontinued in the other four patients because of neuropsychological or behavioral deterioration associated with reappearance of the CSWS pattern, 9-11 months after treatment initiation. This retrospective study suggests that levetiracetam has a positive effect on the EEG, behavior and cognition of children with CSWS. Additional studies are warranted in order to assess the place of this drug in this condition. Epilepsia 2005;46(12).

November 2005
Childhood Absence Epilepsy: Evolution and Prognostic Factors
Salvatore Grosso, Daniela Galimberti, Piero Vezzosi, Mariangela Farnetani, Rosanna Maria Di Bartolo, Simone Bazzotti, Guido Morgese, and Paolo Balestri

Evolution and prognosis of childhood absence epilepsy (CAE) remain to be defined. This is because of the wide range of diagnostic criteria used throughout the studies. Unfavorable prognostic factors have been considered to be age at onset, appearance of generalized tonic–clonic seizures (GTCS), type of absences, response to treatment, and type of abnormalities in the EEG background. We evaluated remission rates in patients with CAE in relation to the International League Against Epilepsy (ILAE) criteria and the stricter diagnostic criteria of Panayiotopoulos, respectively. Two main groups of patients were therefore identified: Group 1 fulfilling ILAE criteria and Group2 fulfilling stricter diagnostic criteria. Patients identified by using stricter diagnostic criteria had significantly higher rates of seizure control, remission, and patients under monotherapy. Fewer GTCS and shorter mean periods of treatment were also observed in patients from group 2. Therefore, remission rates of patients with CAE are greatly influenced by the classification criteria used for selection. When stricter diagnostic criteria are applied we may identify a homogeneous group of patients with excellent prognosis. Factors predicting unfavorable prognosis resulted GTCS concomitant with absences, myoclonic jerks, eyelid myoclonia or perioral myoclonia, and EEG features atypical for CAE. Epilepsia 2005;46(11).

October 2005
Hyperhomocysteinemia in Children Treated with Antiepileptic Drugs is Normalized by Folic Acid Supplementation
Martina Huemer, Bernd Ausserer, Günther Graninger, Michael Hubmann, Christian Huemer, Kurt Schlachter, Arthur Tscharre, Hanno Ulmer, and Burkhard Simma

Homocysteine (Hcy) is a metabolite involved in the metabolism of the amino acid methionine. Hcy is tightly connected to vitamin B12, B6, B2 and folate pathways. Mild to moderate elevations of Hcy may be caused by vitamin deficiency or by variations in genes coding for enzymes involved in the Hcy pathway. Hyperhomocysteinemia is an independent risk factor for thrombosis, atherosclerosis and neural tube defects in the offspring of affected females. Antiepileptic drugs (AEDs) are known to interfere with Hcy metabolism and long-term AED treatment may be associated with hyperhomocysteinemia. The prevalence of hyperhomocysteinemia was assessed in 123 pediatric patients treated with AEDs and the effect of oral folic acid (1 mg/d) supplementation for 3 months versus placebo on total Hcy (tHcy) concentrations was evaluated in patients with hyperhomocysteinemia. Hyperhomocysteinemia (tHcy > 10.4 _mol/L) was present in 19/123 patients (15.5 %). Patients with hyperhomocysteinemia were older (13.7 + 4 versus 11.0 + 3.9 yrs) and had significantly lower folate and vitamin B12 concentrations. Multidrug (>2) AED treatment and duration of therapy correlated significantly with elevated tHcy and low folate. In contrast, genetic polymorphisms (MTHFR 677 CèT, 1298 AèC, 1793 GèA) had no significant impact on tHcy. Nine out of 19 patients with hyperhomocysteinemia were randomized to placebo while the remaining 10 patients received folic acid supplementation. Folic acid supplementation resulted in a significant increase of folate and decrease of tHcy while both parameters remained unchanged in the placebo group. We recommend measurement of serum folate and plasma tHcy in children receiving AEDs. Epilepsia 2005;46(10).

Benign Partial Epilepsy in Childhood: Selective Cognitive Deficits are Related to the Location of Focal Spikes Determined by Combined EEG / MEG

Markus Wolff, Nikolaus Weiskopf, Emilio Serra, Hubert Preissl, Niels Birbaumer, and Ingeborg Krägeloh-Mann

Benign Partial Epilepsy (BPE) is a childhood epilepsy with genetic background and a favorable outcome. Children typically exhibit rare epileptic seizures which disappear around puberty. In contrast to the rare seizures the electroencephalogram (EEG) shows usually frequent high voltage spikes and waves with variable location (“focal spikes”). It is known that children with BPE suffer more often than healthy children from selective cognitive deficits, i.e. dyslexia, dyscalculia or others. Up to now it is unclear whether these deficits are due to the focal spike activity in the EEG or not. Therefore, we performed a study to investigate the relationship between the sites of the focal spikes in the brain on the one hand and the type of cognitive deficit on the other hand in twenty children with BPE. We used magneto-encephalography (MEG), a new technique that improves the accuracy of spike localization in the brain, and specific neuropsychological tests. We found that children with focal spikes in the language area (left temporal hemisphere) had significantly more language problems compared to the others; and children with focal spikes in the visual cortex (occipital region) had difficulties in complex visual transformation tasks, i.e. construction of triangles or arrangement of photo series. Hence, these findings indicate that focal spikes in the EEG may interfere with complex cognitive functions. Treatment strategy in these children should not only concentrate on the overt seizures, but also on the spike activity in the EEG in order to prevent selective cognitive deficits. Epilepsia 2005;46(10).

September 2005
The Accuracy of Outcome Prediction Models for Childhood-onset Epilepsy
Anne Olde Boerrigter and Miranda Geelhoed (co-first authors), Peter Camfield, Ada T. Geerts, Willem Arts, Bruce Smith, and Carol Camfield

On the day of diagnosis it would be highly desirable to know which children with epilepsy will eventually become seizure free, be able to stop daily medication treatment and have remission from (outgrow) their epilepsy. Two large studies (Nova Scotia and the Netherlands) identified children when their epilepsy started and then followed them for at least 5 years to see which children had remission. We combined these two studies to allow consideration of 1055 children with epilepsy and used powerful statistical methods (classification tree models and stepwise logistic regression) to see if clinical aspects of the child’s epilepsy at diagnosis and results of electroencephalographic (EEG) testing could be used to predict which children would have remission from their epilepsy and which would not.

Overall 59% of the children were in remission at the end of follow up (seizure-free and no longer receiving daily medication). The statistical models allowed us to predict the outcome correctly for 70%. Factors that helped to predict the outcome were the type of epilepsy (syndrome), age of first seizure <12 years of age, number of seizures before treatment, abnormalities in neurological function and intelligence. However even in this large group of children our prediction schemes were incorrect in 30% - some were expected to remit and did not while others had a surprise remission when it was not anticipated. Based on currently available clinical and EEG variables, predicting the outcome of childhood epilepsy is difficult and appears to be incorrect in about one of every three patients. Epilepsia 2005;46(9).

Characterizing Magnetoencephalographic Spike Sources in Children with Tuberous Sclerosis Complex

Koji Iida, Hiroshi Otsubo, Ismail S. Mohamed, Chiyuki Okuda, Ayako Ochi, Shelly K. Weiss, Sylvester H. Chuang, and O. Carter Snead III

In tuberous sclerosis complex (TSC), tubers and sclerotic patches on the brain cause seizures. Magnetoencephalography (MEG) measures magnetic fields caused by electrical activity in cells and locates centers of seizure activity as spike sources (SSs) for surgical planning. We evaluated data from seven children with TSC, characterized the MEG SS results, and correlated them with prolonged video-electroencephalography (VEEG) and MRI.

We classified MEG results according to whether the SS were in clusters or were scattered, and whether the SSs were anatomically related to tubers.

Two patients had a single, unilateral SS cluster with other scattered SSs.. The clustered SSs corresponded to prominent tubers on MRI and active seizure areas on VEEG. Two patients had bilateral clustered SSs, with other scattered SSs. The cluster locations partly overlapped multiple prominent tubers. VEEG discharges recorded between seizures were bilateral or diffuse. Three patients had only bilateral scattered SSs. These scattered SSs partly overlapped seizure-onset regions and between-seizure-onset regions on VEEG. In one patient with equally bilateral scattered SSs, the SSs overlapped a prominent tuber and seizure-/between-seizure-onset zones in the right frontal brain.

The significance of the study is that the characteristics of noninvasively determined MEG SSs can help define the primary epileptogenic zone (single cluster, scattered SS, in unilateral hemisphere) and reduce the extent of invasive VEEG needed for surgical planning. When MEG SSs are focal, intracranial VEEG grids need to cover only the focal area; when MEGSSs are scattered, however, VEEG must cover at least the entire area of MEG SSs. Epilepsia 2005;46(9).

August 2005
A Distinct Asymmetric Pattern of Cortical Malformation: Large Unilateral Malformation of Cortical Development with Contralateral Periventricular Nodular Heterotopia in Three Pediatric Cases
Annapurna Poduri, Anna Golja, James J. Riviello, Jr., Blaise F.D. Bourgeois, Frank H. Duffy, and Masanori Takeoka

Epilepsy affects about one percent of the world’s population. Magnetic resonance imaging (MRI) performed in patients with difficult to control epilepsy often reveals malformations of cortical development (MCD), or abnormalities in the formation of the brain. In some MCD, a small area of brain is affected, while in others the entire brain may be affected. If only part of the brain is affected, the abnormal region may represent a group of neurons that did not travel to the proper place in development; this occurs in lesions called periventricular nodular heterotopia (PNH). In other cases, a large region of the brain may be abnormally formed and represent a region in which neurons traveled to some extent but failed to result in a normally organized cortex. Examples include subcortical and transmantle heterotopia. PNH can occur on both sides of the brain as a result of genetic abnormalities but also in isolation on one side of the brain. Subcortical and transmantle heterotopia usually occur in isolation in an individual patient. We observed three patients in whom PNH occurred on one side and the larger heterotopia occurred on the opposite side of the brain. This unusual pattern suggests that in the same person, perhaps the same underlying problem (for example, a genetic abnormality) can affect brain development on the right and left sides of the brain at different stages of brain development. Future study of cases like these should contribute to a deeper understanding of brain development and of epilepsy that results from MCD. Epilepsia 2005;46(8).

Anti-glial Cell Autoantibodies and Childhood Epilepsy

Agathe Roubertie, Hassan Boukhaddaoui, Victor Sieso, Anne de Saint-Martin, Arielle Lellouch-Tubiana, Edouard Hirsch, Bernard Echenne, and Jean Valmier

We report the case of a patient with severe partial epilepsy associated with a focal motor cortex, pathologically proven, cortical dysplasia, a congenital defect of brain cells in an abnormal location. By measuring intracellular calcium concentrations, functional antibodies produced against glia (the supporting cells of the brain) were identified in the serum of this patient. Antibodies that interfere with function of a portion of the glutamate receptor on both glia and brain cells were detected. This observation provides new clues about the involvement of immunological (allergic) mechanisms in epileptic disorders. Epilepsia 2005;46(8).

Predictive Clinical Factors for the Differential Diagnosis of Childhood Extratemporal Seizures

András Fogarasi, Ingrid Tuxhorn, Márta Hegyi, and József Janszky

While temporal lobe epilepsy is the most common form of adults’ partial epilepsies, children frequently have extra-temporal epilepsies. They can originate from the frontal lobe or the posterior cortex (parietal and occipital lobes) of the brain. In our study we looked for clinical factors helping to differentiate between childhood frontal lobe epilepsy (FLE) and posterior cortex epilepsy (PCE).

Analyzing 177 seizures from 35 (20 FLE and 15 PCE) children <12 years we found some similarities and differences. Patients from both groups had daily seizures without significant difference in frequency, but with higher nocturnal dominance in children with FLE. Visual aura, nystagmus and versive (turning/twisting) seizures were observed exclusively in the PCE group, while somatosensory aura and hypermotor seizures appeared only in FLE. Tonic seizures were significantly more frequent in FLE, while the presence of clonic seizure (FLE) and postictal nose-wiping (PCE) showed only a trend to localize the seizure onset zone. Myoclonic seizures, epileptic spasms, psychomotor seizures, atonic seizures, oral and manual automatisms, as well as vocalization and eye deviation appeared in both groups without significant difference in their frequency. We can conclude that characteristic features described in adults’ FLE and PCE were frequently missing during childhood extra-temporal seizures. Ictal features help only a little in differentiating childhood FLE from PCE. Nocturnal appearance and the type of aura have high localizing value therefore an accurate history taking is still an essential element of pediatric presurgical evaluation. Epilepsia 2005;46(8).

July 2005
Origin and Propagation of Epileptic Spasms Delineated on Electrocorticography
Eishi Asano, Csaba Juhász, Aashit Shah, Otto Muzik, Diane C. Chugani, Jagdish Shah, Sandeep Sood, and Harry T. Chugani

Epileptic spasms (infantile spasms) are one of the catastrophic pediatric epilepsies characterized by clusters of short body contractions. Spasms often are associated with arrest in psychomotor development. Surgical removal of epileptic cortex is effective to eliminate medically-uncontrolled spasms in a subset of patients. In general, intracranial EEG and neuroimaging are utilized to localize the epileptic focus in children with uncontrolled seizures. Yet, it has been unclear how focal features on intracranial EEG recordings before or during spasms can be used to tailor cortical removal for alleviating spasms. In the present study of 15 children with medically-uncontrolled spasms, we analyzed intracranial EEG data using a novel analytic method called as ‘subtraction ictal electrocorticography co-registered to MRI (SIECOM)’. Sequential intracranial EEG changes associated with spasms were animated on a 3-dimensional MRI. We found that spasms were associated with either a ‘leading’ spike followed by fast wave bursts or fast wave bursts without a ‘leading’ spike on intracranial EEG recording. While ‘leading’ spikes appeared focal, fast wave bursts associated with spasms involved extensive neocortical regions within a second. Following epilepsy surgery, 10 children became seizure-free. Failure to remove the cortex showing a ‘leading’ spike was associated with poor surgical outcome. In conclusion, we speculate that spasms may be triggered by a focal neocortical impulse in a subset of patients, and that a ‘leading’ spike, if present, may be utilized as a marker of the trigger zone for epileptic spasms. Epilepsia 2005;46(7).

Characteristics of Slow Waves on EEG Associated with Epileptic Spasms

Katsuhiro Kobayashi, Makio Oka, Takushi Inoue, Tatsuya Ogino, Harumi Yoshinaga, and Yoko Ohtsuka

West syndrome is a grave epileptic disorder showing spasms in series in infancy and early childhood. The high-voltage slow waves (HVS) on EEG associated with epileptic spasms were investigated to clarify their characteristics and their relation to the pathophysiology of spasms in West syndrome and related disorders.

In 14 patients, digitally recorded EEG segments showing the ictal HVS were extracted and their traces were overlaid using an average reference. The ictal HVS were also averaged to build maps for investigation of the pattern of potential distribution over the scalp. In a total of 685 recorded spasms, 346 (50.5%) with minimal artifacts were selected to demonstrate that the ictal HVS had a largely consistent waveform and distribution in each patient. The ictal HVS were symmetric in ten patients and asymmetric in the other four, and were relatively negative over the posterior region and positive over the frontal or temporal regions in 11 patients. Two symptomatic patients showed a marked deviation of the distribution of HVS to the pathologically more involved hemisphere. An infant with Aicardi syndrome had two different types of spasms, each type showing a consistent pattern of HVS with a lateralized distribution. Therefore, the patterns of distribution of the ictal HVS may be related to the abnormal activation of the brain in the generation of spasms. Epilepsia 2005;46(7).

A Magnetoencephalographic Study of Patients with Panayiotopoulos Syndrome

Osamu Kanazawa, Jun Tohyama, Noriyuki Akasaka, and Takanori Kamimura

Panayiotopoulos syndrome (PS) is a newly identified type of benign childhood epilepsy characterized by ictal vomiting and eye deviation. It is usually accompanied by occipital spike discharges; however, its classification as an early-onset benign childhood occipital epilepsy is controversial. To further characterize this condition, we examined the localization of equivalent current dipoles (ECDs) of spike discharges by magnetoencephalography (MEG) in patients with PS. We studied thirteen patients with a mean age at time of examination of 5 years (range 3-14 years). MEG was measured by using a whole-head 204-channel neuromagnetometer with simultaneous EEG recordings. The estimated locations of ECDs of each peak of the spike discharges were overlaid on magnetic resonance images of the brain. Eleven patients (84.6%) showed clustered ECDs in the areas alongside the parieto-occipital sulcus (8/13: 61.5%) or the calcarine sulcus (4/13: 30.8%). Seven patients showed ECDs in the parietal lobe, while occipital spikes in the scalp EEG. Despite Fp-O synchronization of the spike discharges in the scalp EEG of five patients, no frontal locations of ECD were found. All five (38.5%) male patients with sylvian seizures, who also showed clustered ECDs in rolandic areas, had an earlier age of onset and higher seizure frequency than other patients. ECD orientations were regular in all but one patient who showed irregular and dispersed ECDs alongside bilateral calcarine sulci. Our results demonstrate localized cortical hyper-excitability in the areas alongside major cortical sulci in PS, and indicate that PS is closely related to benign childhood epilepsy with centro-temporal spikes. Epilepsia 2005;46(7).

June 2005
Nonfebrile Illness Seizures: A Unique Seizure Category?
Danielle M. Zerr, Heidi K. Blume, Anne T. Berg, Mark A. Del Beccaro, Sidney M. Gospe Jr., Amanda L. Allpress, and Dimitri A. Christakis

In contrast to febrile seizures and unprovoked seizures, little is known about nonfebrile seizures occurring in the setting of mild illness. The aims of this study were to describe the clinical characteristics of children presenting with a first-time nonfebrile seizure in the setting of mild illness and to test the hypothesis that these seizures are associated with illness characterized by diarrhea. This retrospective cohort study was performed in a pediatric emergency department. Patients 6 months to 6 years of age who were evaluated with first-time seizures were eligible for inclusion. Subjects were divided into three groups on the basis of symptoms accompanying their seizure: febrile (temperature >38.0º C with the seizure), unprovoked (no symptoms of illness), and nonfebrile illness (no fever at the time of seizure, but other symptoms of illness present). Of the 323 children with first-time seizures, 247 (76%) had febrile seizure, 37 (12%) had unprovoked seizure and 39 (12%) had nonfebrile illness seizures. Children with nonfebrile illness seizures were more likely than children with febrile seizures to have diarrheal illnesses accompanying their seizure (44 versus 16%, p = 0.001). Frequency of cough, rhinorrhea, and rash did not differ significantly between children with febrile and nonfebrile illness seizures. Diagnostic testing for infectious etiologies was not performed frequently in either group. We conclude that nonfebrile illness seizures may represent a distinct group of seizures with unique epidemiology. Further study to better define these seizures is warranted. Epilepsia 2005;46(6).

Early Thalamic Injury associated with Epilepsy and Continuous Spike-wave during Slow Sleep

Francesco Guzzetta, Domenica Battaglia, Chiara Veredice, Valeria Donvito, Marika Pane, Donatella Lettori, Francesca Chiricozzi, Daniela Chieffo, Tommaso Tartaglione, and Charlotte Dravet

The condition of continuous spike-waves, seen with an EEG recording taken during the slow-wave stage of sleep (CSWS) is in an infrequent, but potentially very serious, epileptic disorder. The causes of this syndrome are multiple, and often not known. However, a few cases have been associated with early injury to the thalamus in the central region of the brain. This study investigated epileptic-like activation of the EEG during sleep, its characteristics, and possible relationships with neuroimaging and neuropsychological features in a series of children with early injury of thalamus. Thirty-two patients with a brain injury involving thalamus, aged between 4 and 12 years (when CSWS usually develops), and consequently admitted to a specialty Hospital between 1999 and 2003, were enrolled in the study. In 32 studied patients, 29 showed major EEG activation during sleep. Among these 29 patients, two different groups were distinguished. The first included those with typical CSWS (12 cases), generally with symmetry of spikes and waves (SWs), and often with no normal EEG sleep spindles. Those in the other group presented with an usual asymmetry of SWs and presence or reduction of EEG sleep spindles, plus other atypical features concerning synchronism and morphology of SWs. Behavioral disorders were more prevalent in patients with true CSWS; improvement of their cognitive functions paralleled the disappearance of CSWS. Early thalamic injury thus could be considered to be a co-factor predisposing to CSWS. Detection of CSWS in infants or early childhood with EEG-sleep monitoring could lead to early treatment, which might prevent or ameliorate the cognitive deterioration and the behavioral disorders that are usually associated with CSWS. Epilepsia 2005;46(6).

Computerized Motion Analysis of Videotaped Seizures in Neonates with Epilepsy

Nicolaos B. Karayiannis, Guozhi Tao, Yaohua Xiong, Abdul Sami, Bindu Varughese, James D. Frost, Jr., Merrill S. Wise, and Eli M. Mizrahi

The main objective of the research outlined in this paper is the automated recognition and characterization of the type of neonatal seizures by processing and analyzing video recordings of infants monitored for seizures. This paper describes the results of our ongoing project, which aims at the extraction, processing, and analysis of novel quantitative information regarding the behavioral characteristics of videotaped neonatal seizures. The long-term goal of this research is the integration of the computational procedures and tools outlined in this paper into the development of a stand-alone automated system that could be used as a supplement in the neonatal intensive care unit (NICU) to provide 24-hour a day noninvasive monitoring of infants at risk for seizures without the distractions that currently limit the effectiveness of monitoring of neonatal seizures by NICU nurses. Such a system would be capable of detecting neonatal seizures, processing video recordings of neonatal seizures, and facilitating the analysis of videotaped neonatal seizures by physicians during retrospective review. The development of such a system involves the following three tasks: (1) the extraction of quantitative motion information from video recordings of neonatal seizures, (2) the selection of quantitative features that convey some unique behavioral characteristics of neonatal seizures, and (3) the training of artificial neural networks to distinguish neonatal seizures from infant behaviors not associated with seizures and to differentiate between different types of neonatal seizures. Epilepsia 2005;46(6).

Epilepsy in Young Adults with Autism: A Prospective Population-based Follow-up Study of 120 Individuals Diagnosed in Childhood

Susanna Danielsson, I. Carina Gillberg, Eva Billstedt, Christopher Gillberg, and Ingrid Olsson

Individuals with autism have qualitative impairment in reciprocal social interaction and communication abilities and a restricted pattern of behavior with onset early in childhood. The present study relates to individuals who met the typical definitions for autism fifteen to thirty years ago, the majority having developmental delay (mental retardation). We followed 120 individuals with autism from childhood into adulthood, and found that the majority still had autism and developmental delay as adults. The psychosocial outcome was poor and they constituted a severely disabled group. One of three had epilepsy. Epilepsy often started early in life, and persisted. Severe developmental delay and autism were associated with epilepsy, especially in females. On a group basis, both the cognitive level and the adaptive behavior level were lower in the epilepsy group than in the non-epilepsy group. Partial seizures with or without secondarily generalized seizures were the dominating seizure type. Seizure frequency had a great impact on the individuals’ lives. Relatives often expressed frustration about the difficulty for these individuals to “fit” into the system of medical services for adults. Collaboration across different disciplines and specialist medical care are needed for these severely disabled individuals, not only in childhood, but also in adulthood. Epilepsia 2005;46(6).

The Neuropsychological and Language Profile of Children with Benign Rolandic Epilepsy

Ellen Northcott, Anne M. Connolly, Anna Berroya, Mark Sabaz, Jenny McIntyre, Jane Christie, Alan Taylor, Jennifer Batchelor, Andrew F. Bleasel, John A. Lawson, and Ann M.E. Bye

Benign Rolandic Epilepsy (BRE) is the most common idiopathic (of unknown cause) partial epilepsy in childhood. BRE has an excellent prognosis for seizures, with remission typically seen during puberty. Recent research has raised concerns that some children may have cognitive difficulties, but reports about the nature of these difficulties have been inconsistent. Methodological problems related to patient selection and cognitive assessment processes are evident in previous studies. In the current study, patients were selected according to defined criteria and cognitive assessments were comprehensive. The aim of this study was to define the cognitive profile of children with BRE and to assess the effect of brainwave activity using EEG. Patients (n=42) were recruited from six EEG laboratories. Comprehensive neuropsychological and language assessments were conducted. The study demonstrated that children with BRE have normal intelligence and language ability. However, a specific pattern of difficulties in memory and phonological awareness was found. Phonological awareness is the awareness that spoken language is made up of discrete sounds. Difficulties in phonological awareness impact on reading, writing and spelling. Memory problems affect academic performance. Brainwave activity (numbers of spikes and location of spikes) was minimally associated with cognitive difficulties, and there was no correlation with memory indices and tests of phonological awareness. Some children with BRE have specific difficulties in memory and phonological processing skills, not explained by brainwave activity. If difficulties are suspected, neuropsychological assessment targeting phonological awareness and memory is recommended, as they may not be reflected in overall intellectual and language ability. Epilepsia 2005;46(6).

May 2005
Depression and Anxiety Disorders in Pediatric Epilepsy
Rochelle Caplan, Prabha Siddarth, Suresh Gurbani, Rebecca Hanson, Ramen Sankar, and W. Donald Shields

This study examined 100 children with complex partial seizures (CPS) and 71 children with childhood absence epilepsy (CAE). The children with epilepsy were more depressed and anxious, and had more suicidal behavior than 93 normal children, aged 5-16 years. The children and their parents were interviewed and filled out questionnaires about the child’s feelings, emotions, and behavior. The children also had testing of their intelligence and language. The parents provided information on the children’s seizure disorder. We found a much higher rate in the children with epilepsy compared to the normal children for depression and anxiety disorder (30%), as well as for suicidal thoughts (20%). None of the children had made a suicide attempt. Anxiety was more frequent than depression in the children with epilepsy. Only 33% of the children received some form of mental health service. Younger age, lower verbal skills, and school problems were related to the presence of a diagnosis of depression or anxiety disorder. Suicidal thoughts were found more in children who had epilepsy for longer periods of time. The children with CPS had more depression than anxiety; whereas, those with CAE had more anxiety. These findings, together with the high rate of unmet mental health needs for children with epilepsy, emphasize the importance of early identification and treatment of anxiety disorders and suicidal ideation in children with CPS and CAE. Epilepsia 2005;46(5).

Adding Video Recording Increases the Diagnostic Yield of Routine Electroencephalograms in Children with Frequent Paroxysmal Events

Nathan Watemberg, Barak Tziperman, Ron Dabby, Mariana Hasan, Liora Zehavi, and Tally Lerman-Sagie

Prolonged video-EEG monitoring has long been utilized in the diagnosis evaluation of epileptic patients. More recently, outpatient short-term video-EEG of 2 – 8 hours has been implemented for patients with frequent attacks. We have utilized a much shorter approach, involving the addition of video recording to routine EEG studies in children. An analysis of the efficacy of this diagnostic approach between 2000 and 2004 was undertaken. Video recording was added to routine studies of children referred with very frequent clinical episodes. Indications for EEG included: paroxysmal (sudden) eye movements, tremor, suspected seizures, myoclonic jerks, staring episodes, tics, absence epilepsy follow-up, cyanotic events, and suspected nonepileptic seizures. Video recording was added to 137 of 666 EEG studies. Mean patient age was 4.8 years. Twenty five percent were inpatients. The nature of the event could be determined in 61 (45%) studies, compared with 68% to 95% success rates of published studies using the 2 – 8 hour outpatient video-EEG recording. Average EEG duration was 26 minutes. Paroxysmal eye movements, staring episodes, tics, and myoclonus showed the highest diagnostic yield. The rate of success was higher (55%) among children with mental retardation. Conclusions: Adding video recording to routine EEG helped solve the clinical question in 45% of cases referred for frequent paroxysmal events. This technique was useful for hospitalized and ambulatory children. Infants and children with paroxysmal eye movements, staring episodes, staring episodes, tics, and myoclonus especially benefited from this diagnostic means. Due to its low cost and the little discomfort imposed on the patient and his or her family, this technique should be considered as a first diagnostic step in children with frequent paroxysmal events. Epilepsia 2005;46(5).

Hippocampal Sclerosis in Severe Myoclonic Epilepsy in Infancy: A Retrospective MRI Study

Zsuzsanna Siegler, Peter Barsi, Magdolna Neuwirth, Judit Jerney, Maria Kassay, Jozsef Janszky, Eva Paraicz, Marta Hegyi, and Andras Fogarasi

The hippocampus is a structure in the brain’s temporal lobe, known to be very involved with generation of seizures in some patients. Many patients with temporal lobe epilepsy show a type of scarring or “hardening” of the hippocampus, called hippocampal sclerosis (HS). Whether the HS results from seizures, causes seizures, or both, remains controversial. During the last ten years, the causal relationship of prolonged febrile convulsions and hippocampal sclerosis (HS) has been of great interest. Febrile convulsions, or seizures with fever, are common in children age 6 months to 6 years. Usually they are benign and do not lead to epilepsy. However, prolonged febrile convulsions are typical of the condition called severe myoclonic epilepsy in infancy (SMEI). We reviewed the magnetic resonance images (MRIs) of patients with SMEI to assess if they had developed HS during the course of disease. SMEI is a rare, difficult-to-treat epilepsy syndrome beginning in a healthy infant with prolonged one-sided or generalized tonic-clonic febrile seizures. In the second year of life, or near this time, new types of seizures appear, including atypical absences, myoclonic jerks and complex partial seizures. Developmental delay becomes evident in the second year. In this study, 28 MRIs of 14 patients treated in our hospitals between 1996 and 2004 were reviewed. Ten of 14 showed HS. Four children’s initial MRI, made only years after the beginning of the disease, showed HS, but six patient’s initial MRI didn’t show any abnormality and they developed HS during the course of the disease. Our data support the hypothesis that prolonged febrile convulsions might be responsible for HS, but other factors (individual sensitivity, genetic factors) could play a role, too. In addition, the lessons from this type of myoclonic epilepsy might not apply to other types of epilepsy. Epilepsia 2005;46(5).

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