bluuebunny/crossref_metadata_2025_split huggingface dataset:
Temporal Lobe Epilepsy in Children: Etiology in a Cohort with New‐onset Seizures
Authors: Sztriha László , Gururaj Aithala K. , Bener Abdulbari , Nork Michael 
Published: March 2003

Summary:  Purpose: The aim of this study was to characterize the incidence and etiology of temporal lobe epilepsy (TLE) in a community‐based cohort of children with new‐onset disease.Methods: A community‐based cohort of 30 children with TLE was studied. The patients had new‐onset disease before age 14 years between 1995 and 1999. They underwent clinical, EEG, and magnetic resonance imaging investigations.Results: The patients could be divided in three main groups according to likely etiology, as suggested by Harvey et al. (Neurology 1997;49:960–8). Group 1 consisted of eight (26.7%) children with malformations or long‐standing, nonprogressive tumors (developmental TLE). Arachnoid cysts were found in three, dual pathology [cortical dysplasia and hippocampal sclerosis (HS)] in one, and focal cortical dysplasia with glioproliferative changes in one patient. Dysembryoplastic neuroepithelial tumor was responsible for the epilepsy in one, and ganglioglioma, in two children. Group 2 consisted of seven (23.3%) children with a significant antecedent and/or HS. Five children had a significant illness or event before the onset of TLE, including perinatal hypoxic–ischemic encephalopathy in one, encephalitis in one, traumatic brain injury in two, and complex febrile seizures in one. HS was found in the patients with traumatic brain injury and complex febrile seizures in the history in addition to two children without known antecedents. Group 3 comprised 15 (50%) children with no abnormality on neuroimaging and no significant antecedents (cryptogenic TLE).Conclusions: Etiologic differences between children with new‐onset TLE may have prognostic implications: children with TLE and significant antecedents/HS are expected to have the greatest risk of continued seizures and psychological problems.
Interictal Epileptiform Discharges Do Not Change before Seizures during Sleep
Authors: Natarajan Alamelu , Marzec Mary L. , Lin Xihong , Minecan Daniela , Malow Beth A. 
Published: March 2003

Summary:  Purpose: Whether interictal epileptiform discharges (IEDs) increase, decrease, or are unchanged before epileptic seizures has implications for the pathophysiology of epilepsy. Prior studies relating IEDs and seizures have not demonstrated a change in IEDs before seizures. However, they have not controlled for changes in the depth of sleep. Our objective was to test the hypothesis that IEDs are related to seizures during sleep while adjusting for log delta power (LDP), a continuous measure of sleep depth.Methods: Twenty‐two seizures during sleep were identified in 16 subjects with epilepsy admitted for presurgical monitoring. The IEDs that occurred in the hour of sleep before each seizure were used to test the relation between IEDs and seizure occurrence. Sleep depth was measured by LDP (quantity of 1‐ to 4‐Hz activity in 30‐s epochs), and records were scored visually for sleep staging and for IEDs. Multivariate logistic regression analyses were applied.Results: Adjusting for LDP, number of seizures before the current seizure, quartile of the night, and total number of IEDs that occurred during the night, IED did not increase or decrease before seizures (p > 0.1). The rate of IEDs increased directly with LDP (p = 0.0001), as shown in prior work.Conclusions: IEDs are not activated or suppressed before seizures during sleep, suggesting that different pathophysiologic processes underlie these two phenomena. These results corroborate prior studies, while providing a more advanced analysis by adjusting for sleep depth and applying multivariate logistic regression analyses.
Epidemiologic Features of Infantile Spasms in Slovenia
Authors: Primec Zvonka Rener , Kopač Štefan , Neubauer David 
Published: March 2003

Summary:  Purpose: To determine the epidemiologic features of infantile spasms (ISs) in Slovenia.Methods: Medical records of all children with ISs in Slovenia in the period from 1985 to 1995, based on community pediatrician referrals to four hospitals, including all pediatric EEG laboratories, were retrospectively studied. The outcome was assessed by a follow‐up study in 1998.Results: Forty‐seven children with ISs were identified over an 11‐year interval. The cumulative incidence was 2.06 per 10,000 live births. Among 29 (61.7%) children with symptomatic etiology, 14 cases had prenatal etiology [tuberous sclerosis (TS), seven; vascular insult, three; cerebral malformations, three; Down syndrome, one child], 14 perinatal and one postnatal cause: anoxic brain damage after cardiac surgery. Cryptogenic and idiopathic etiology were diagnosed in 13 (27.6%) and five (10.6%) of 47 cases, respectively. The age of onset of ISs ranged from 2 to 10 months. As initial treatment, steroids were used in 19 children (remission in 10); vigabatrin in seven (remission in four), and other antiepileptic drugs (AEDs) in 20 children (remission in six). According to the follow‐up study, 18 (38.3%) children were seizure free, and 14 of them had normal mental development. Among 29 mentally retarded children (14 severely), 26 belonged to the symptomatic group. Four children died.Conclusions: The incidence of ISs in Slovenia is similar to that in some parts of the United States, but lower than that in Finland or Sweden. The outcome depends mainly on etiology. Additional neuroimaging studies are needed for evaluation of cryptogenic cases.
Multiple Subpial Transection for Intractable Partial Epilepsy: An International Meta‐analysis
Authors: Spencer Susan S. , Schramm Johannes , Wyler Allen , O'Connor Michael , Orbach Darren , Krauss Gregory , Sperling Michael , Devinsky Orrin , Elger Christian , Lesser Ronald , Mulligan Lisa , Westerveld Michael 
Published: March 2003

Summary:  Purpose: Because the number and variety of patients at any single facility is not sufficient for clinical or statistical analysis, data from six major epilepsy centers that performed multiple subpial transections (MSTs) for medically intractable epilepsy were collected.Methods: A meta‐analysis was performed to elucidate the indications and outcome, and to assess the results of the procedure. Overall, 211 patients were represented with data regarding preoperative evaluation, procedures, seizure types and frequencies before and after surgery, postoperative deficits, and demographic information. Fifty‐three patients underwent MST without resection.Results: In patients with MST plus resection, excellent outcome (>95% reduction in seizure frequency) was obtained in 87% of patients for generalized seizures, 68% for complex partial seizures, and 68% for simple partial seizures. For the patients who underwent MST without resection, the rate of excellent outcome was only slightly lower, at 71% for generalized, 62% for complex partial, and 63% for simple partial seizures. EEG localization, age at epilepsy onset, duration of epilepsy, and location of MST were not significant predictors of outcome for any kinds of seizures after MST, with or without resection. New neurologic deficits were found in 47 patients overall, comparable in MST with resection (23%) or without (19%).Conclusions: These preliminary results suggest that MST has efficacy by itself, with minimal neurologic compromise, in cases in which resective surgery cannot be used to treat uncontrolled epilepsy. MST should be investigated as a stand‐alone procedure to allow further development of criteria and predictive factors for outcome.
A Rett Patient with a Typical Angelman EEG
Authors: Laan Laura A. E. M. , Vein A. A. 
Published: March 2003

Summary:  Purpose: To draw attention to the phenomenon that EEG characteristics of both Angelman syndrome (AS) and Rett syndrome (RS) can be found in the same patient, as evidenced by the description of one case. There are specific EEG patterns in AS patients, in which the most frequently occurring EEG characteristics are rhythmic triphasic 2‐ to 3‐Hz, high‐voltage (200–500 μV) activity, mixed with spikes or sharp waves, with a maximum over the frontal regions. EEG changes in RS patients are less specific and can show multifocal, mostly central or centrotemporal epileptiform discharges in combination with slow background activity.Methods: A 6‐year‐old girl with RS and a proven MECP2 mutation was described.Results: She had an EEG pattern at age 2 years comparable with the clinical diagnosis of RS, and an EEG at age 6 years comparable with an AS EEG.Conclusions: We wish to draw attention to this phenomenon, although there is as yet no evident explanation for it. We advise MECP2 examination in AS patients of unknown genetic etiology whose EEG examinations are/were pathognomonic for AS to exclude RS.
Focal Cortical Dysplasia of Taylor's Balloon Cell Type: A Clinicopathological Entity with Characteristic Neuroimaging and Histopathological Features, and Favorable Postsurgical Outcome
Authors: Urbach Horst , Scheffler Björn , Heinrichsmeier Thomas , Von Oertzen Joachim , Kral Thomas , Wellmer Joerg , Schramm Johannes , Wiestler Otmar D. , Blümcke Ingmar 
Published: March 2003

Summary: Background and Purpose: Focal cortical dysplasia of Taylor's balloon‐cell type (FCD‐BC) are a frequent cause of pharmacoresistant epilepsy in young patients. In order to characterize FCD‐BC, we coupled MRI and histopathology, and analyzed the clinical outcome following epilepsy surgery.Methods: From an epilepsy data bank with 547 histological specimens, 17 FCD‐BC were re‐evaluated of which high resolution MRI was available. Five additional FCD‐BC were prospectively identified by MRI. Histopathological and immunohistochemical features were related to MRI. Outcome following lesionectomy was analyzed as determined on routine examinations 3, 6 and 12 months following surgery.Results: All but one lesion were located outside the temporal lobe. A markedly hyperintense funnel‐shaped subcortical zone tapering towards the lateral ventricle was the characteristic finding on FLAIR MRI. Histopathologically, the subcortical zone of the FCD‐BC displayed hypomyelinated white matter with radially oriented balloon cells and gliosis. Dysplastic neurons were found in the adjacent, disorganized cortex. All patients with complete lesionectomy were seizure free one year following surgery.Conclusion: Focal cortical dysplasias of Taylor's balloon‐cell type (FCD‐BC) have characteristic MRI and histopathological findings. MRI recognition is important, since outcome following resective surgery is favorable.
Standardization of Amygdalohippocampectomy with Intraoperative Magnetic Resonance Imaging: Preliminary Experience
Authors: Schwartz Theodore H. , Marks David , Pak Jayoung , Hill James , Mandelbaum David E. , Holodny Andrei I. , Schulder Michael 
Published: March 2003

Summary:  Purpose: Intraoperative magnetic resonance imaging (IMRI) is an extremely useful neurosurgical tool in surgeries in which the extent of resection is known to have a significant impact on outcome. Residual hippocampus is the most common cause of recurrent seizures after temporal lobectomy for medial temporal lobe epilepsy. Although the risk/benefit ratio of a policy of universal radical hippocampal resection is not known, we hypothesized that IMRI would aid in the intraoperative assessment of the extent of hippocampal resection and assist in accomplishing a complete hippocampectomy.Methods: Five consecutive patients with medically intractable medial temporal lobe epilepsy underwent a radical amygdalohippocampectomy as part of the their surgery for epilepsy. IMRI was used before surgery and after an initial resection. The quality of images was assessed. Postoperative MR images were evaluated by a radiologist to determine the extent of resection of the amygdala, hippocampus, and parahippocampal gyrus.Results: There were no perioperative infections. After a mean follow‐up of 10 months, all patients are seizure free. T1‐weighted coronal intraoperative images were judged adequate at visualizing the medial structures in all patients. T2 and fluid‐attenuated inversion recovery (FLAIR) images did not provide useful information. Postoperative MR images indicated that a complete hippocampectomy had been achieved in all patients.Conclusions: IMRI is a useful adjunct in the surgical treatment of medial temporal lobe epilepsy and perhaps the most reliable method of standardizing a complete hippocampectomy. T1‐weighted coronal images are the most helpful sequence.
Risk Factors for Suicide in Epilepsy: A Case Control Study
Authors: Nilsson Lena , Ahlbom Anders , Farahmand Bahman Y. , Åsberg Marie , Tomson Torbjörn 
Published: March 2003

Summary:  Purpose: Suicide is considered to be one of the most important causes of death contributing to the increased mortality of persons with epilepsy. We investigated the association between the risk of suicide in persons with epilepsy and clinical factors that might increase or have been suggested to increase the risk of suicide.Methods: A case–control study was nested within a cohort of 6,880 patients registered in the Stockholm County In‐Patient Register with a diagnosis of epilepsy. The study population was followed up through the National Cause of Death Register. Twenty‐six cases of suicide, 23 cases of suspected but not proven suicide, and 171 controls, living epilepsy patients, were selected from the cohort. Clinical data were collected through medical record review.Results: There was a ninefold increase in risk of suicide with mental illness and a 10‐fold increase in relative risk (RR) with the use of antipsychotic drugs. The estimated RR of suicide was 16.0 [95% confidence interval (CI), 4.4–58.3] for onset of epilepsy at younger than 18 years, compared with onset after 29 years. The risk of suicide seemed to increase with high seizure frequency and antiepileptic drug (AED) polytherapy, although the estimates were imprecise and the associations not statistically significant. Insufficient data on seizure frequency and changes in AED dosage due to incomplete case records were associated with high RRs. We found no association between risk of suicide and any particular AED, with type of epilepsy, or localization or lateralization of epileptogenic focus on EEG [RR = 0.3 (95% CI, 0.1–1.7)].Conclusions: The profile of the epilepsy patient who commits suicide that emerges from our study is a patient with early onset (particularly onset during adolescence) but not necessarily severe epilepsy, psychiatric illness, and perhaps inadequate neurologic follow‐up. Previous reports of an association with temporal lobe epilepsy could not be confirmed.
Serum S‐100 Protein Is Not a Suitable Seizure Marker in Temporal Lobe Epilepsy
Authors: Leutmezer Fritz , Wagner Oswald , Baumgartner Christoph 
Published: March 2003

Summary:  Purpose: S‐100 protein is a sensitive marker of various brain diseases; however, its role in epilepsy is controversially discussed in the literature. We therefore studied the temporal profile of serial concentrations of S‐100 protein in serum after secondarily generalized tonic–clonic seizures during video‐EEG monitoring.Methods: Ten patients with mesial temporal lobe epilepsy were prospectively studied. Serum S‐100 protein was measured after a seizure‐free period of ≥24 h (baseline) and 30 min, 3, 6, 12, and 24 h after a secondarily generalized tonic–clonic seizure of temporal lobe origin in nine and a convulsive status epilepticus in one patient.Results: All S‐100 levels were within the normal range, except for those of one patient at baseline. Mean values were 0.045 μg/L (range, 0.003–0.13 μg/L) at baseline, 0.038 μg/L (range, 0.003–0.09 μg/L) at 30 min, 0.036 μg/L (range, 0.003–0.08 μg/L) at 3 h, 0.034 μg/L (range, 0.003–0.07 μg/L) at 6 h, 0.034 μg/L (range, 0.003–0.08 μg/L) at 12 h, and 0.035 μg/L (range, 0.003–0.09 μg/L) at 24 h after seizure offset. There were no significant differences between mean concentrations at any interval postictally.Conclusions: We could not detect any significant alterations in serum S‐100 protein concentration either after a single secondarily generalized tonic–clonic seizure or after convulsive status epilepticus in patients with temporal lobe epilepsy. Our data do not confirm previous work, which suggested serum S‐100 protein to be a suitable marker for epileptic seizures.
Systematic Review and Meta‐analysis of Incidence Studies of Epilepsy and Unprovoked Seizures
Authors: Kotsopoulos Irene A. W. , Van Merode Tiny , Kessels Fons G. H. , De Krom Marc C. T. F. M. , Knottnerus J. André 
Published: March 2003

Summary:  Purpose: To evaluate the methodology of incidence studies of epilepsy and unprovoked seizures and to assess the value of their findings by summarizing their results.Methods: A Medline literature search from January 1966 to December 1999 was conducted. In each selected study, key methodologic items such as case definition and study design were evaluated. Furthermore, a quantitative meta‐analysis of the incidence data was performed.Results: Forty incidence studies met the inclusion criteria. There was considerable heterogeneity in study methodology, and the methodologic quality score was generally low. The median incidence rate of epilepsy and unprovoked seizures was 47.4 and 56 per 100,000, respectively. The age‐specific incidence of epilepsy was high in those aged 60 years or older, but was highest in childhood. Males had a slightly higher incidence of epilepsy (median, 50.7/100,000) than did females (median, 46.2/100,000), and partial seizures seemed to occur more often than generalized seizures. Developing countries had a higher incidence rate of epilepsy (median, 68.7/100,000) than did industrialized countries (median, 43.4/100,000). Similar results were found for unprovoked seizures. The incidence of epilepsy over time appears to decrease in children, whereas it increases in the elderly.Conclusions: The age‐specific incidence of epilepsy showed a bimodal distribution with the highest peak in childhood. No definitive conclusions could be reached for the incidence of unprovoked seizures and other specific incidence rates of epilepsy. More incidence studies with an adequate study methodology are needed to explore geographic variations and time trends of the incidence of epilepsy and unprovoked seizures.
A Detailed Analysis of Symptomatic Posterior Cortex Seizure Semiology in Children Younger Than Seven Years
Authors: Fogarasi András , Boesebeck Frank , Tuxhorn Ingrid 
Published: March 2003

Summary:  Purpose: To analyze the semiology of seizure onset and evolution in young children with posterior cortex epilepsy (PCE), compare this with adult reports, and assess age‐related differences.Methods: We videotaped and analyzed 110 seizures from 18 patients with PCE, aged 3–81 months. All had a good prognosis after posterior epileptogenic zone removal. Ictal events were categorized by behavioral, consciousness, autonomic, and sensory features, as well as motor patterns, which included myoclonic, tonic, clonic, unclassified motor seizures, and epileptic spasm. A time‐scaled data sheet was developed to record each epileptic event as onset, very early, early, or late manifestation.Results: Patients had a high seizure frequency with ≤100 attacks/day; one third of them showed a cluster tendency. The mean duration of seizures was 67 s. The most common seizure components were motor manifestations (with myoclonic and tonic seizures), but psychomotor (automotor), hypomotor attacks, and isolated auras also were frequently observed. Clinical seizure spread was frequent; auras and visual sensory signs were difficult to record in this age. Typical phenomena during seizures included behavioral changes, ictal vocalization, smile, flush, head nod, oculomotor features, and late‐appearing oral automatisms, whereas hypermotor and secondarily generalized tonic–clonic seizures were not seen.Conclusions: Our results suggest that PCE in infants and young children is very heterogeneous but shows important age‐related features. Compared with adults, children with PCE have shorter but more frequent seizures; they rarely report aura or visual sensory signs, only sporadically develop hypermotor and secondarily generalized tonic–clonic seizures, whereas ictal smile, flush, head nod, and behavioral change are typical features at this age. Because of frequent subtle ictal phenomena, long‐term video‐EEG monitoring is a useful diagnostic tool with infants and young children with PCE.
Widespread Upregulation of Drug‐resistance Proteins in Fatal Human Status Epilepticus
Authors: Sisodiya Sanjay M. , Thom Maria 
Published: March 2003

Summary:  Purpose: Accumulating evidence implicates drug‐transporter proteins ABCB1 and ABCC1 in resistance to multiple antiepileptic drugs (AEDs) in refractory epilepsy. These proteins are upregulated in surgically resected human brain tissue containing epileptogenic pathologies, including cortical dysplasia. In surgically resected cases, no upregulation is seen in normal adjacent tissue, suggesting that neither seizures nor prolonged exposure to AEDs need induce ABCB1 or ABCC1 expression. We wished to determine if status epilepticus might cause upregulation of these proteins.Methods: Immunohistochemistry was performed for ABCB1 and ABCC1 in postmortem human brain tissue from a patient who died in status epilepticus and was found to have unihemispheric cortical dysplasia.Results: There was upregulation of both proteins, as expected, in the hemisphere containing dysplasia. There also was widespread upregulation of both proteins in glia from the normal hemisphere. Previous work shows that drug treatment does not cause such upregulation.Conclusions: Upregulation of these proteins in histologically normal brain tissue is most likely the result of seizures in status, as seen in animal models. The findings provide a possible explanation for the appearance of AED resistance in prolonged status and emphasise the importance of prompt treatment of status epilepticus.
The Genetic Absence Epilepsy Rat from Strasbourg (GAERS), a Rat Model of Absence Epilepsy: Computer Modeling and Differential Gene Expression
Authors: Lakaye Bernard , Thomas Elisabeth , Minet Arlette , Grisar Thierry 
Published: March 2003

Summary:  Purpose: We present results obtained by computer modeling of the thalamic network and differential gene expression analysis in a rat strain with absence epilepsy, the genetic absence epilepsy rat from Strasbourg (GAERS).Methods: (a) Computer modeling used equations from the Hodgking–Huxley model with a circuit of 13 reticular thalamic (nRt) and 39 thalamocortical (TC) neurons; (b) gene‐expression analysis using differential mRNA display (DD), in situ hybridization, Northern blotting, and competitive reverse transcriptase–polymerase chain reaction (RT‐PCR).Results: (a) Computer modeling showed an increased network synchrony in the thalamic circuit as the value of conductance of low‐voltage activated calcium channel (LVACC) is increased. (b) Using differential mRNA display, a 40% upregulation of the H‐ferritin mRNA in the hippocampus was demonstrated. Looking for some candidate genes of the VACC family, no difference was found in the α1G mRNA expression between GAERS and control animals, whereas a decreased expression of the α1E subunit was observed in the cerebellum and the brainstem of the GAERS. This phenomenon was not observed in young animals when the epileptic phenotype is not expressed.Conclusions: The use of computer modeling appeared to be an efficient way to evaluate the impacts of electrophysiologic findings in vivo from single cells on an entire circuit. No clear single gene defect was revealed so far in GAERS. More information could arise from linkage analysis. However, some brain structures like hippocampus or cerebellum classically not known to be involved in the production of absence spike‐and‐wave discharges could in fact participate in the development of this phenotype.