bluuebunny/crossref_metadata_2025_split huggingface dataset:
Open‐field Behaviors and Water‐maze Learning in the F Substrain of Ihara Epileptic Rats
Authors: Okaichi Yoko , Amano Shigeru , Ihara Nobuo , Hayase Yoneko , Tazumi Tooru , Okaichi Hiroshige 
Published: January 2006

Summary: Purpose:Genetically epileptic model rats, Ihara epileptic rat (IER/F substrain), have neuropathologic abnormalities and develop generalized convulsive seizures when they reach the age of ∼5 months. Because the neuromorphologic abnormalities are centered in the hippocampus, we expected to observe spatial cognitive deficits. The present study aimed to evaluate emotionality and learning ability of the F substrain of IER.Methods:To determine whether deficits are caused by inborn neuropathologic abnormalities or by repeated generalized convulsions, we tested nine 6‐ to 12‐week‐old IER/F rats that had not yet experienced seizures (experiment 1) and nine 7‐ to 9‐month‐old IER/F rats that had repeatedly experienced seizures (experiment 2) with identical tasks: an open‐field test and the Morris water‐maze place and cue tasks.Results:Both groups of IER/Fs showed behaviors that were different from those of control rats in the open‐field test, and extensive learning impairments were seen in both the place task, which requires spatial cognition, and the cue task, which does not require spatial cognition but requires simple association learning. Their impaired performance of the cue task indicates that their deficiency was not limited to spatial cognition.Conclusions:Because young IER/F rats without seizure experiences also showed severe learning impairments, genetically programmed microdysgenesis in the hippocampus was suspected as a cause of the severe learning deficits of IER/Fs.
Reduction of AEDs in Postsurgical Patients Who Attain Remission
Authors: Berg Anne T. , Vickrey Barbara G. , Langfitt John T. , Sperling Michael R. , Shinnar Shlomo , Bazil Carl , Walczak Thaddeus , Spencer Susan S. ,   for the Multicenter Study of Epilepsy Surgery
Published: January 2006

Summary:  Purpose: Little evidence guides practice regarding the reduction of antiepileptic drugs (AEDs) in individuals who achieve significant periods of remission after resective surgery. Methods: In a prospective study of 396 individuals who underwent resective surgery for intractable seizures, the impact of reducing AEDs on relapse and predictors of relapse were examined. Results: Of 301 study subjects who attained a 1‐year seizure remission after surgery, 129 reduced from two to one or from one to no AEDs after achieving remission, and 162 did not reduce drugs. Ten patients who stopped all AEDs before attaining remission were excluded from analysis. In all, 114 (39%) patients relapsed: 73 (45%) of 162 in the nonreduction group and 41 (32%) of 129 in the reduction group (p = 0.02). Patients who reduced drugs were more likely to have remitted immediately after hospital discharge (p<0.0001). After adjustment for this factor, little or no apparent impact of drug reduction on the rate of relapse was noted [rate ratio (RR), 1.1; 95% CI, 0.72–1.71; p=0.64 for reducing from two to one AED, and RR, 0.85; 95% CI, 0.50–1.45; p=0.56 for reducing from one to no AEDs]. Within the 129 patients who reduced AEDs, only delayed remission after hospital discharge was significantly associated with an increased rate of relapse (RR, 2.26; 95% CI, 1.15– 4.48; p=0.02). Continued auras had a marginally significant association with relapse (RR, 2.06; p=0.07) Conclusions: Although many relapses in this cohort of postsurgical patients who achieved remission occurred in the context of reducing or completely eliminating AEDs, the risk was no higher than in those who continued AEDs. Randomized studies are needed to test the impact of drug reduction in seizure‐free postsurgical patients.
Efficacy and Safety of Levetiracetam (up to 2000 mg/day) in Taiwanese Patients with Refractory Partial Seizures: A Multicenter, Randomized, Double‐blind, Placebo‐controlled Study
Authors: Tsai Jing‐Jane , Yen Der‐Jen , Hsih Mo‐Song , Chen Shung‐Sheng , Hiersemenzel Reinhard , Edrich Pascal , Lai Chi‐Wan 
Published: January 2006

Summary: Purpose:To assess the efficacy and safety of adjunctive levetiracetam (LEV) therapy in controlling partial‐onset seizures refractory to other antiepileptic drugs (AEDs) in a multicenter study in Taiwanese adults.Methods:Ninety‐four patients aged 16–60 years with refractory partial seizures were randomized to receive LEV (n = 47) or placebo (47) for 14 weeks and composed the intention‐to‐treat (ITT) population. After the first 2 weeks, LEV patients had their dosage increased from 500 mg twice daily to 1,000 mg twice daily. A 12‐week maintenance phase followed, after which patients switched to long‐term, open‐label LEV therapy or entered a 4‐week phase of medication discontinuation.Results:All patients from the ITT population, except one LEV‐treated patient with missing seizure‐count data, were included in the primary efficacy analysis. The least square mean of logarithmically transformed weekly partial‐seizure frequency was significantly lower in the LEV than in the placebo group (0.813 vs. 1.085; p = 0.001). LEV reduced log‐transformed weekly partial‐seizure frequency by 23.8% (95% confidence interval, 10.4–35.2%) relative to placebo. Significantly more LEV than placebo patients (43.5% vs. 10.6%) experienced a response of a ≥50% decrease from baseline in weekly frequency of partial seizures [odds ratio, 6.5 (95% CI, 2.2–19.3); p < 0.001]. Adverse events were reported in 34 (72.3%) of 47 LEV‐treated patients and 32 (68.1%) of 47 placebo patients. The three most common adverse events in the LEV and placebo groups were somnolence (40.4% and 14.9%), dizziness (14.9% and 8.5%), and headache (10.6% and 8.5%), respectively. Only four patients (three LEV‐treated patients and one placebo patient) were withdrawn from the study because of adverse events.Conclusions:Adjunctive LEV therapy, ≤1,000 mg twice daily, was significantly more effective than placebo and was generally well tolerated in Taiwanese adults with treatment‐resistant partial‐onset seizures.
Refractory Generalized Seizures: Response to Corpus Callosotomy and Vagal Nerve Stimulation
Authors: Nei Maromi , O'Connor Michael , Liporace Joyce , Sperling Michael R. 
Published: January 2006

Summary:  Purpose: The vagal nerve stimulator (VNS) and corpus callosotomy can reduce seizure frequency when seizures are refractory to medications. However, the efficacy and safety of these two procedures have not been compared. This study evaluates the two procedures for generalized seizures. Methods: All patients with refractory generalized seizures (generalized tonic–clonic, tonic, or atonic) who underwent a corpus callosotomy (anterior or complete) (n = 53) without other forms of epilepsy surgery and those who underwent VNS placement (n = 25) were evaluated for this study. Seizure response and procedure complications were evaluated. Results: For those with a corpus callosotomy and generalized tonic–clonic seizures (n = 50), 79.5% had ≥50% decrease in the frequency of generalized tonic–clonic seizures, and 60% had ≥80% seizure reduction. For those with a VNS and generalized tonic–clonic seizures (n = 21), 50% had ≥50% seizure reduction, and 33% had ≥80% seizure reduction. Tonic and atonic seizures decreased after either VNS or a corpus callosotomy. The complication rate for corpus callosotomy was higher (21% all complications, 3.8% permanent) than that for VNS (8%; none permanent), but complications for both corpus callosotomy and VNS were rarely permanent. Conclusions: Both corpus callosotomy and VNS are effective in reducing generalized seizures. Corpus callosotomy is associated with greater efficacy but higher risk for complications, although these were generally transient.
Ictal Brain Hyperperfusion Contralateral to Seizure Onset: The SPECT Mirror Image
Authors: Huberfeld Gilles , Habert Marie‐Odile , Clemenceau Stéphane , Maksud Philippe , Baulac Michel , Adam Claude 
Published: January 2006

Summary:  Purpose: Ictal single‐photon emission computed tomography (SPECT) may help localize the seizure‐onset zone (SOZ) by detecting changes in regional cerebral blood flow induced by epileptic discharges. This imaging method also reveals hyperperfusions in areas of seizure propagation, including the hemisphere contralateral to the SOZ. We have studied the occurrence, the topography, and the clinical value of such contralateral ictal hyperperfusion areas (HPAs). Methods: We examined data from presurgical evaluations of 36 consecutive patients with pharmacoresistant partial epilepsy of various localizations. Ictal and interictal SPECT examinations were made with 99mTc‐ECD, and the scans were processed for coregistration, normalization, subtraction, and merging with MRI images. Results: Contralateral HPAs were observed in 72% of the patients: 50% of mesiotemporal epilepsy cases with hippocampal sclerosis, 85.7% of the other mesiotemporal epilepsies, 85.7% of neocortical lateral temporal epilepsies, and 87.5% of extratemporal epilepsies. Contralateral HPAs were usually symmetrical to the SOZ, forming a mirror image, observed in 57.1% of the patients. They could be slightly asymmetrical in mesiotemporal epilepsies, perhaps because of the particular anatomic pathways linking temporal lobes. In neocortical epilepsies, they were located in the cortex homotopic to the SOZ. Conclusions: We show that the symmetrical nature of the mirror image usually does not disturb SPECT interpretation. It can confirm the location of the SOZ (11 patients) and even occasionally improve the precision of its definition (nine patients) by restraining several potential SOZ‐related HPAs to a single one or by permitting a restricted localization of the SOZ in a large HPA.
Hippocampal Sclerosis in Children with Lesional Epilepsy Is Influenced by Age at Seizure Onset
Authors: Riney Catherine J. , Harding Brian , Harkness William J. F. , Scott Rod C. , Cross J. Helen 
Published: January 2006

Summary:  Purpose: Hippocampal sclerosis (HS) is the most common lesion underlying drug‐resistant temporal lobe epilepsy. Whether HS is a developmental or acquired pathology remains unclear. Whereas HS has been causally linked to prolonged febrile convulsions in childhood, evidence also exists that it may coexist with extrahippocampal abnormalities, the concept of “dual pathology.” The aims of this study were to address whether hippocampal abnormality consistent with HS (a) occurs in children with lesional extrahippocampal epilepsy, (b) is more commonly seen in association with developmental rather than acquired extrahippocampal pathologies, and (c) whether any effect of age at seizure onset is found on the occurrence of HS in lesional extrahippocampal epilepsy. Methods: Clinical and histopathologic data of patients having resective surgery for extrahippocampal epilepsy that included the hippocampus were investigated. Results: Twenty‐nine children were retrospectively included in this study, and 21 (72%) of 29 were found to have a hippocampal abnormality consistent with HS. No relation was noted between developmental or acquired extrahippocampal pathologies and the presence of hippocampal abnormality. Children with normal hippocampi on visual histologic assessment had a significantly younger age at seizure onset (p < 0.001). Duration of epilepsy was not correlated with the presence of hippocampal abnormality. Conclusions: Hippocampal abnormalities are seen in similar proportions with both acquired and developmental extra‐hippocampal pathologies, suggesting that these abnormalities are the result of seizures from the focus that is remote from the hippocampus. In addition, children who have their initial seizure at an early age are less likely to develop seizure‐induced hippocampal injury.
Hippocampal and Thalamic Diffusion Abnormalities in Children with Temporal Lobe Epilepsy
Authors: Kimiwada Tomomi , Juhász Csaba , Makki Malek , Muzik Otto , Chugani Diane C. , Asano Eishi , Chugani Harry T. 
Published: January 2006

Summary:  Purpose: Previous studies using diffusion MRI in patients with temporal lobe epilepsy have shown abnormal water diffusion in the hippocampus. Because thalamus and lentiform nuclei are considered important for the regulation of cortical excitability and seizure propagation, we analyzed diffusion tensor imaging (DTI) abnormalities in these subcortical structures and in hippocampus of children with partial epilepsy with and without secondary generalization. Methods: Fourteen children with partial epilepsy involving the temporal lobe underwent MRI including a DTI sequence. Fractional anisotropy (FA) and apparent diffusion coefficient (ADC) values were obtained in the hippocampus, thalamus, and lentiform nucleus, and compared with DTI data of 14 control children with no epilepsy, as well as glucose positron emission tomography (PET) findings. Results: Decreased FA (p < 0.001) and increased ADC (p = 0.003) values were found in the hippocampi ipsilateral to the seizure focus. Significant FA decreases (p = 0.002) also were seen in the contralateral hippocampi, despite unilateral seizure onset and excellent surgical outcome in patients who underwent surgery. ADC values showed a trend for increase in the thalami ipsilateral to the epileptic focus in the seven children with secondarily generalized seizures (p = 0.09). No group differences of ADC or FA were found in the lentiform nuclei. The DTI variables did not correlate with regional glucose metabolism in any of the structures analyzed. Conclusions: Increased ADC values in hippocampus can assist in lateralizing the seizure focus, but decreased FA in the contralateral hippocampus suggests that it too may be dysfunctional despite unilateral seizure onset. Less‐robust thalamic abnormalities of water diffusion in patients with secondarily generalized seizures suggest secondary involvement of the thalamus, perhaps due to recruitment of this structure into the epileptic network; however, this must be confirmed in a larger population. DTI appears to be a sensitive method for detection abnormalities in children with partial epilepsy, even in structures without apparent changes on conventional MRI.
Community‐based, Prospective, Controlled Study of Obstetric and Neonatal Outcome of 179 Pregnancies in Women with Epilepsy
Authors: Viinikainen Katriina , Heinonen Seppo , Eriksson Kai , Kälviäinen Reetta 
Published: January 2006

Summary:  Purpose: This study evaluated obstetric and neonatal outcome in a community‐based cohort of women with active epilepsy (WWAE) compared with the general pregnant population receiving modern obstetric care. Methods: We reviewed the total population who gave birth between January 1989 and October 2000 at Kuopio University Hospital. Obstetric, demographic, and epilepsy data were collected prospectively from 179 singleton pregnancies of women with epilepsy and from 24,778 singleton pregnancies of unaffected controls. The obstetric data from the pregnancy register was supplemented with detailed neurologic data retrieved from the medical records. The data retrieved were comprehensive because of a follow‐up strategy according to a predecided protocol. Results: During pregnancy, the seizure frequency was unchanged, or the change was for the better in the majority (83%) of the patients. We found no significant differences between WWAE and controls in the incidence of preeclampsia, preterm labor, or in the rates of caesarean sections, perinatal mortality, or low birth weight. However, the rate of small‐for‐gestational‐age infants was significantly higher, and the head circumference was significantly smaller in WWAE. Apgar score at 1 min was lower in children of WWAE, and the need for care in the neonatal ward and neonatal intensive care were increased as compared with controls. The frequency of major malformations was 4.8% (−0.6–10.2%; 95% confidence interval) in the 127 children of WWAE. Conclusions: Pregnancy course is uncomplicated and neonatal outcome is good in the majority of cases when a predecided protocol is used for the follow‐up of WWAE in antenatal and neurologic care. Long‐term follow‐up of the neurologic and cognitive development of the children of WWAE is still needed.
Epilepsy in Colombia: Epidemiologic Profile and Classification of Epileptic Seizures and Syndromes
Authors: Velez Alberto , Eslava‐Cobos Jorge 
Published: January 2006

Summary: Purpose:A national study was performed in Colombia to determine the general and regional prevalence of epilepsy, clinical profiles, seizure types, and clinical syndromes.Methods:Based on the National Epidemiological Study of Neurological Diseases (EPINEURO), we evaluated and followed up for 1 year all the subjects with epilepsy from the National Sample. Clinical profiles were further assessed. Seizure types and epilepsy syndromes were established according to the international classifications.Results:General prevalence was found to be 11.3 per 1,000, with little variation among regions, except the eastern region, where prevalence was 23 per 1,000; prevalence for active epilepsy was 10.1 per 1,000. Women have a slightly greater (not statistically significant) risk. Most seizures are focal (partial), frequently with secondary generalization. The most frequent epilepsy syndrome encountered was partial symptomatic/cryptogenic (80%). Epilepsy onset in Colombia occurs most frequently in childhood.Conclusions:Prevalence rates of epilepsy in Colombia are similar to those reported in nations with comparable developmental status and have diminished over time. The study presents the distribution of seizures and syndromes. The most frequent types are focal syndromes.
Mutation in Filamin A Causes Periventricular Heterotopia, Developmental Regression, and West Syndrome in Males
Authors: Masruha Marcelo R. , Caboclo Luis O. S. F. , Carrete Henrique , Cendes Íscia L. , Rodrigues Murilo G. , Garzon Eliana , Yacubian Elza M. T. , Sakamoto Américo C. , Sheen Volney , Harney Megan , Neal Jason , Sean Hill R. , Bodell Adria , Walsh Christopher , Vilanova Luiz C. P. 
Published: January 2006

Summary:  Purpose: Familial periventricular heterotopia (PH) represents a disorder of neuronal migration resulting in multiple gray‐matter nodules along the lateral ventricular walls. Prior studies have shown that mutations in the filamin A (FLNA) gene can cause PH through an X‐linked dominant pattern. Heterozygotic female patients usually remain asymptomatic until the second or third decade of life, when they may have predominantly focal seizures, whereas hemizygotic male fetuses typically die in utero. Recent studies have also reported mutations in FLNA in male patients with PH who are cognitively normal. We describe PH in three male siblings with PH due to FLNA, severe developmental regression, and West syndrome. Methods: The study includes the three affected brothers and their parents. Video‐EEG recordings and magnetic resonance image (MRI) scanning were performed on all individuals. Mutations for FLNA were detected by using polymerase chain reaction (PCR) on genomic DNA followed by single‐stranded conformational polymorphism (SSCP) analysis or sequencing. Results: Two of the siblings are monozygotic twins, and all had West syndrome with hypsarrthymia on EEG. MRI of the brain revealed periventricular nodules of cerebral gray‐matter intensity, typical for PH. Mutational analyses demonstrated a cytosine‐to‐thymidine missense mutation (c. C1286T), resulting in a threonine‐to‐methionine amino acid substitution in exon 9 of the FLNA gene. Conclusions: The association between PH and West syndrome, to our knowledge, has not been previously reported. Males with PH have been known to harbor FLNA mutations, although uniformly, they either show early lethality or survive and have a normal intellect. The current studies show that FLNA mutations can cause periventricular heterotopia, developmental regression, and West syndrome in male patients, suggesting that this type of FLNA mutation may contribute to severe neurologic deficits.
A Novel SCN2A Mutation in Family with Benign Familial Infantile Seizures
Authors: Striano Pasquale , Bordo Laura , Lispi Maria Luisa , Specchio Nicola , Minetti Carlo , Vigevano Federico , Zara Federico 
Published: January 2006

Summary:  Benign familial infantile seizures (BFIS) is a clinical entity characterized by focal seizures with or without secondary generalization, occurring mostly in clusters, and usually first seen between 4 and 8 months of life. Psychomotor development is normal, and seizures usually resolve within the first year of life. BFIS is a genetically heterogenous condition with loci mapped to chromosomes 19 and 16. Mutations in the voltage‐gated sodium channel α2 subunit (SCN2A) gene on chromosome 2 were recently identified in families affected by neonatal and infantile seizures (benign familial neonatal–infantile seizures, BFNIS) with typical onset before 4 months of life. The identification of SCN2A mutations in families with only infantile seizures indicated that BFNIS and BFIS show overlapping clinical features.We report a pedigree showing three affected individuals over three generations. All subjects experienced clusters of focal seizures with or without secondary generalization and onset between 4 and 12 months of life. Response to antiepileptic drugs and the outcome were good. No subjects had other forms of epilepsy later in the life. Neonatal or febrile seizures did not occur in the family. Genetic study in this family revealed a novel heterozygous mutation c.3003 T>A in the SCN2A gene. Comparative analysis of different sodium channel α subunits indicates that the mutated residue is highly conserved throughout the evolution, suggesting an important functional role for this domain. Additional families with the infantile form of benign familial seizures should be investigated to corroborate that BFIS and BFNIS may share the same genetic abnormality.