bluuebunny/crossref_metadata_2025_split huggingface dataset:
Time Trends in Incidence, Mortality, and Case‐Fatality after First Episode of Status Epilepticus
Authors: Logroscino Giancarlo , Hesdorffer Dale C. , Cascino Gregory , Annegers John F. , Hauser W. Allen 
Published: March 2003

Summary:  Purpose: Status epilepticus (SE) is a medical emergency associated with a high mortality. Clinical series have suggested that mortality after SE has decreased. No studies have systematically examined trends in incidence, mortality, and case fatality after SE in a well‐defined population.Methods: All first episodes of SE receiving medical attention between January 1, 1935, and December 31, 1984, were ascertained through the Rochester Epidemiology Project Records‐Linkage System and followed up until death or study termination (February 1, 1996). We calculated incidence rates in the 50‐year period (1935–1984), while we considered mortality and case‐fatality in the last 30‐year period (1955–1984).Results: Incidence of SE increased over time to 18.1/100,000 (1975 through 1984). The increase was related to an increased incidence in the elderly and to the advent of myoclonic SE after cardiac arrest, a condition not seen in the early decades. In the last decade, ∼16% of the incidence was due to myoclonic SE. The mortality rates increased from 3.6 per year in the decade 1955–1965 to 4.0/100,000 per year between 1975 and 1984. The 30‐day case‐fatality (CF) was unchanged, although a trend toward improvement was shown after excluding myoclonic SE.Conclusions: Incidence and mortality rates of SE have increased in the last 30 years. Case fatality remained the same. The increased incidence and mortality are due to the occurrence in the last decade of myoclonic SE after cardiac arrest. The mortality in the elderly was twice that of the youngest age group, across all study periods. Changes in the age and cause distribution of SE over time are responsible for the stable survivorship. There is improvement in survivorship in the last decade when myoclonic SE is excluded.
Stupor from Lamotrigine Toxicity
Authors: Sbei Mouaz , Campellone Joseph V. 
Published: March 2003

Summary:  Purpose: To report that acute lamotrigine poisoning may result in severe encephalopathy.Methods: A 55‐year‐old woman became stuporous after overdose with lamotrigine (LTG) and valproic acid (VPA) tablets. Evaluation revealed elevated serum LTG levels and no other etiology for encephalopathy.Results: LTG was withheld, and the patient experienced a complete recovery in conjunction with reduced serum levels.Conclusions: LTG overdose may result in a severe but reversible encephalopathy, a previously undescribed phenomenon. LTG toxicity should be considered in patients taking this drug who have depressed mental status.
Photosensitivity in Relation to Epileptic Syndromes: A Survey from an Epilepsy Center in Japan
Authors: Shiraishi Hideaki , Fujiwara Tateki , Inoue Yushi , Yagi Kazuichi 
Published: March 2003

Summary:  Purpose: We examined the incidence and distribution of photosensitivity among the different age groups and different types of epilepsies and epileptic syndromes. Furthermore, we considered the influence of ethnic and geographic factors on the incidence of photoparoxysmal response (PPR) in epilepsy patients.Methods: We analyzed the responses to intermittent photic stimulation (IPS) by using a Grass PS22 or PS33 photic stimulator for in 2,187 unselected patients with epilepsy who were treated in our center.Results: The classic PPR was elicited in 37 (1.7%) patients. The mean age of these 37 patients was 17.0 years. The subpopulation of patients having PPR included 2.0% of all patients with symptomatic generalized epilepsy, 5.6% (p < 0.01) of those with idiopathic generalized epilepsy, 0.7% of those with symptomatic localization‐related epilepsy, and 2.9% of those with undetermined epilepsy. PPR accounted for 17.4 % (p < 0.01) of the patients with juvenile myoclonic epilepsy, 7.6% (p < 0.01) of those with grand mal on awakening, and 6.1% (p < 0.01) of those with symptomatic occipital lobe epilepsy. The incidence of PPR increased in patients up to age 15 years, and suddenly decreased after age 20 years.Conclusion: The present study presents the first report from eastern Asia, analyzing the incidence of PPR with a restricted definition comparable to the other studies, and the rate of PPR was relatively low compared with the studies performed in the European countries. We could confirm the clear relation between age and positive PPR.
Loss of the Potassium Channel β‐Subunit Gene, KCNAB2, Is Associated with Epilepsy in Patients with 1p36 Deletion Syndrome
Authors: Heilstedt Heidi A. , Burgess Daniel L. , Anderson Anne E. , Chedrawi Aziza , Tharp Barry , Lee Olivia , Kashork Catherine D. , Starkey David E. , Wu Yuan‐Qing , Noebels Jeffrey L. , Shaffer Lisa G. , Shapira Stuart K. 
Published: March 2003

Summary:  Purpose: Clinical features associated with chromosome 1p36 deletion include characteristic craniofacial abnormalities, mental retardation, and epilepsy. The presence and severity of specific phenotypic features are likely to be correlated with loss of a distinct complement of genes in each patient. We hypothesize that hemizygous deletion of one, or a few, critical gene(s) controlling neuronal excitability is associated with the epilepsy phenotype. Because ion channels are important determinants of seizure susceptibility and the voltage‐gated K+ channel β‐subunit gene, KCNAB2, has been localized to 1p36, we propose that deletion of this gene may be associated with the epilepsy phenotype.Methods: Twenty‐four patients were evaluated by fluorescence in situ hybridization with a probe containing KCNAB2. Clinical details were obtained by neurologic examination and EEG.Results: Nine patients are deleted for the KCNAB2 locus, and eight (89%) of these have epilepsy or epileptiform activity on EEG. The majority of patients have a severe seizure phenotype, including infantile spasms. In contrast, of those not deleted for KCNAB2, only 27% have chronic seizures, and none had infantile spasms.Conclusions: Lack of the β subunit would be predicted to reduce K+ channel–mediated membrane repolarization and increase neuronal excitability, suggesting a possible relation between loss of this gene and the development of seizures. Because some patients with seizures were not deleted for KCNAB2, there may be additional genes within 1p36 that contribute to epilepsy in this syndrome. Hemizygosity of this gene in a majority of monosomy 1p36 syndrome patients with epilepsy suggests that haploinsufficiency for KCNAB2 is a significant risk factor for epilepsy.
The Effect of Vagus Nerve Stimulation on Epileptiform Activity Recorded from Hippocampal Depth Electrodes
Authors: Olejniczak Piotr W. , Fisch Bruce J. , Carey Michael , Butterbaugh Grant , Happel Lee , Tardo Carmela 
Published: March 2003

Summary:  Purpose: To assess the effect of vagus nerve stimulation (VNS) on interictal epileptiform activity in the human hippocampus. Clinical studies have established the efficacy of vagus nerve stimulation in patients with epilepsy (VNS Study Group, 1995), although the electrophysiologic effects of VNS on the human hippocampus and mesial temporal lobe structures remain unknown.Methods: We report a case study in which a patient with an implanted VNS underwent intracranial electrode recording before temporal lobectomy for intractable complex partial seizures. Epileptiform spikes and sharp waves were recorded from a depth electrode placed in the patient's left hippocampus. Spike frequencies and sharp‐wave frequencies before and during VNS were compared using both a 5‐ and a 30‐Hz stimulus. Different stimulation rates were tested on different days, and all analyses were performed using a Student's t test.Results: We found no significant differences in spike frequency between baseline periods and stimulation at 5 and 30 Hz. In contrast, stimulation at 30 Hz produced a significant decrease in the occurrence of epileptiform sharp waves compared with the baseline, whereas stimulation at 5 Hz was associated with a significant increase in the occurrence of epileptiform sharp waves.Conclusions: VNS produces a measurable electrophysiologic effect on epileptiform activity in the human hippocampus. Although a clinical response to VNS did not occur in our patient before surgery, 30‐Hz VNS suppressed interictal epileptiform sharp waves that were similar in appearance to those seen during the patient's actual seizures. In contrast, 5‐Hz stimulation appeared to increase the appearance of interictal sharp waves.
Infantile Spasms in Down Syndrome: Good Response to a Short Course of Vigabatrin
Authors: Nabbout R. , Melki I. , Gerbaka B. , Dulac O. , Akatcherian C. 
Published: March 2003

Summary:  Purpose: To evaluate the efficacy of vigabatrin (VGB) in the treatment of infantile spasms (ISs) associated with Down syndrome (DS) and to assess the feasibility of early discontinuation to reduce the possible retinal toxicity.Methods: Five children with ISs with DS were treated with vigabatrin as first‐line monotherapy in an open prospective study. The short‐term response was evaluated, and VGB was continued in responders. The treatment was stopped after 6 months in children who were still spasm free.Results: Four children of five became spasm free with VGB, three of them responding within 1 week. This response was maintained during the 6 months of VGB treatment. After VGB discontinuation, and with a follow‐up ranging from 2 to 4 years, none of the responders experienced spasm recurrence or other types of seizures.Conclusions: This study confirms the efficacy of VGB in ISs associated with DS. Moreover, it shows that the duration of VGB treatment can be reduced to 6 months without relapse of ISs. This short treatment might reduce the risk of developing visual field constriction.
MEG Predicts Epileptic Zone in Lesional Extrahippocampal Epilepsy: 12 Pediatric Surgery Cases
Authors: Otsubo Hiroshi , Ochi Ayako , Elliott Irene , Chuang Sylvester H. , Rutka James T. , Jay Venita , Aung Maung , Sobel David F. , Snead O. Carter 
Published: March 2003

Summary:  Purpose: To discover whether the spatial distribution of spike sources determined by magnetoencephalography (MEG) provides reliable information for planning surgery and predicting outcomes in pediatric patients with lesional extrahippocampal epilepsy.Methods: We retrospectively studied 12 children with extrahippocampal epilepsy secondary to cortical dysplasia (CD), tumor, or porencephalic cyst. We compared interictal MEG spike source locations and somatosensory evoked fields derived from equivalent‐current dipole modeling with intraoperative or extraoperative electrocorticography (ECoG).Results: MEG spike sources were found in proximity to the lesion in all patients and extended from lesions in five patients with CD. Marginal spike sources were noted in three patients with tumors, one patient with a cyst, and one with CD, and extramarginal sources in three patients with tumors. Three patients with tumors underwent lesionectomy only; two had further cortical excisions. One patient with CD underwent lesionectomy only, three had lesionectomy and cortical excisions, and two had lesionectomy and multiple subpial transection. Asymmetric MEG spike sources correlated with ECoG findings in all patients. Residual epileptiform discharges on postexcisional ECoG corresponded to spike sources in three patients with tumors and one patient with a cyst. Eleven patients have been seizure free for 1–6 years (mean, 4 years). One patient had residual seizures after incomplete excision of right temporal CD.Conclusions: MEG delineated asymmetric epileptogenicity surrounding lesions and the eloquent cortex. Complete tumor resection produced favorable outcomes despite residual postexcisional ECoG spikes and extramarginal MEG spike sources. CD characterized by clusters of MEG spike sources within and extending from lesions seen on magnetic resonance imaging (MRI) should be removed to prevent seizures.
The Health‐Related Quality of Life of Children with Refractory Epilepsy: A Comparison of Those With and Without Intellectual Disability
Authors: Sabaz Mark , Cairns David R. , Lawson John A. , Bleasel Andrew F. , Bye Ann M. E. 
Published: March 2003

Summary:  Purpose: To determine whether refractory epilepsy affects the health‐related quality of life (HRQOL) of children with or without intellectual disability (ID), and if the presence of ID independently compromises HRQOL in children with refractory epilepsy.Methods: Subjects were parents of children with refractory epilepsy, whose syndrome had been defined using ILAE (International League Against Epilepsy) criteria and video‐EEG monitoring. Children had the presence or absence of ID determined by formal neuropsychological or educational assessment. The relative effect of epilepsy on the two intellectual ability groups was determined using relevant clinical variables. Parents completed a valid epilepsy‐specific HRQOL questionnaire for children, the Quality of Life in Childhood Epilepsy Questionnaire (QOLCE), and, depending on intellectual ability level, the Child Behaviour Checklist or Developmental Behaviour Checklist.Results: Both intellectually normal children with epilepsy and children with epilepsy and ID were more likely to have psychosocial problems compared with their respective intellectual ability reference populations. The results also revealed that children with ID had reduced HRQOL compared with intellectually normal children; a result independent of epilepsy. Analysis of the relationship between epilepsy variables and HRQOL revealed that the QOLCE was the most sensitive in detecting variation in age at onset, seizure frequency, and medications taken.Conclusions: The HRQOL of children with refractory epilepsy is greatly affected, regardless of intellectual ability level. The presence of ID in children with epilepsy independently depresses HRQOL outcomes. Compared with two generic HRQOL measures, the QOLCE was the most sensitive measure to variation in epilepsy variables.
Cryptogenic Epilepsy: An Infectious Etiology?
Authors: Stommel Elijah W. , Seguin Rosanne , Thadani Vijay M. , Schwartzman Joseph D. , Gilbert Karen , Ryan Kathleen A. , Tosteson Tor D. , Kasper Lloyd H. 
Published: March 2003

Summary:  Purpose: Cryptogenic epilepsy, the group of epilepsy syndromes for which an etiology is unknown, comprises ∼20% of all epilepsy syndromes. We selected patients in this subgroup of epilepsy and tested them for evidence of Toxoplasma gondii IgG antibodies by the enzyme‐linked immunosorbent assay. T. gondii is found in up to 20% of the U.S. population forming dormant brain cysts in the latent bradyzoite form. We investigated the hypothesis that dormant T. gondii infection might be associated with cryptogenic epilepsy.Methods: We selected patients with cryptogenic epilepsies and tested them for evidence of T. gondii IgG antibodies by the enzyme‐linked immunosorbent assay. A control group was also tested for comparison.Results: We have found a statistically‐significant elevation of T. gondii antibodies among cryptogenic epilepsy patients as compared to controls [59% increase in optical density (OD), p = 0.013]. This association persisted after adjustment for subjects' gender and age in a multiple logistic regression model; however, it was no longer as statistically significant.Conclusions: Our results suggest that chronic T. gondii infection with brain cysts may be a cause of cryptogenic epilepsy.
Study on Photo‐Pattern Sensitivity in Patients with Electronic Screen Game–Induced Seizures (ESGS): Effects of Spatial Resolution, Brightness, and Pattern Movement
Authors: Funatsuka Makoto , Fujita Michinari , Shirakawa Seigo , Oguni Hirokazu , Osawa Makiko 
Published: March 2003

Summary:  Purpose: With the ever‐increasing popularity of computers, electronic screen game–induced seizure (ESGS) is beginning to pose a serious social problem. To elucidate the pathophysiology of ESGS, with the ultimate goal of prevention, we have been studying photo‐pattern sensitivity in detail with a pattern‐stimulation test using a CRT (cathode ray tube) display. This method is referred to as the “CRT‐pattern test.”Methods: We studied 17 patients brought to our department for evaluation of ESGS. EEG responses were recorded during exposure to various patterns consisting of three elements: spatial resolution, brightness perception, and pattern‐movement recognition displayed on a CRT monitor. Photo‐paroxysmal response (PPR) frequencies were compiled for each stimulation.Results: PPR was induced by the CRT‐pattern test in nine of the 17 cases. In four cases, PPR induction was obtained only after introducing CRT‐pattern tests in addition to standard intermittent photic stimulation (IPS). The rate of PPR induction differed according to the type of pattern, spatial frequency, and pattern‐reversal frequency. However, neither the clarity of the edges of a pattern nor changes in the brightness of a pattern element had any effect on the rate of PPR induction. With the exception of a few subjects, the stimulation caused by pattern movement was not effective in eliciting PPR. Six cases in whom spatial resolution was involved showed occipital dominance in PPR provocation, and three in whom brightness perception and pattern movement recognition was involved showed frontal dominance.Conclusions: The CRT‐pattern test is useful for identifying patients with photosensitivity among patients considered to have incidental or nonphotosensitive seizures unresponsive to standard IPS. Patients with ESGS caused by photosensitivity can be divided into two groups: those with occipital dominance for PPR provocation, in whom spatial resolution is involved; and another group with frontal dominance, in whom brightness perception and pattern‐movement recognition (or possibly perception of colors) are involved.
A Possible Role for Gap Junctions in Generation of Very Fast EEG Oscillations Preceding the Onset of, and Perhaps Initiating, Seizures
Authors: Traub Roger D. , Whittington Miles A. , Buhl Eberhard H. , LeBeau Fiona E. N. , Bibbig Andrea , Boyd Stewart , Cross Helen , Baldeweg Torsten 
Published: March 2003

Summary:  Purpose: We propose an experimentally and clinically testable hypothesis, concerning the origin of very fast (>∼70 Hz) EEG oscillations that sometimes precede the onset of focal seizures. These oscillations are important, as they may play a causal role in the initiation of seizures.Methods: Subdural EEG recordings were obtained from children with focal cortical dysplasias and intractable seizures. Intra‐ and extracellular recordings were performed in rat hippocampal slices, with induction of population activity, as follows: (a) bath‐applied tetramethylamine (an intracellular alkalinizing agent, that opens gap junctions); (b) bath‐applied carbachol, a cholinergic agonist; and (c) focal pressure ejection of hypertonic K+ solution. Detailed network simulations were performed, the better to understand the cellular mechanisms underlying oscillations. A major feature of the simulations was inclusion of axon–axon gap junctions between principal neurons, as supported by recent experimental data.Results: Very fast oscillations were found in children before seizure onset, but also superimposed on bursts during the seizure, and on interictal bursts. In slice experiments, very fast oscillations had previously been seen on interictal‐like bursts; we now show such oscillations before, between, and after epileptiform bursts. Very fast oscillations were also seen superimposed on gamma (30–70 Hz) oscillations induced by carbachol or hypertonic K+, and in the latter case, very fast oscillations became continuous when chemical synapses were blocked. Simulations replicate these data, when axonal gap junctions are included.Conclusions: Electrical coupling between principal neurons, perhaps via axonal gap junctions, could underlie very fast population oscillations, in seizure‐prone brain, but possibly also in normal brain. The anticonvulsant potential of gap‐junction blockers such as carbenoxolone, now in clinical use for treatment of ulcer disease, should be considered.
Cerebrospinal Fluid and Serum Neuron‐Specific Enolase Levels After Febrile Seizures
Authors: Tanabe Takuya , Suzuki Shuhei , Hara Keita , Shimakawa Shuiti , Wakamiya Eiji , Tamai Hiroshi 
Published: March 2003

Summary:  Purpose: Neuron‐specific enolase (NSE) has been established as a reliable marker of neuronal damage in various neurologic disorders. The aim of this study was to evaluate whether febrile seizures (FS) cause brain damage, based on the serum and cerebrospinal fluid (CSF) levels of NSE.Methods: Fifty‐three patients aged from 6 months to 7 years were enrolled. Among them, 36 patients had generalized seizures, and 17 had partial seizures. The maximal seizure duration was 90 min. Blood and CSF samples for measurement of NSE were obtained immediately after the seizure. NSE was measured using an enzyme immunoassay (EIA).Results: Serum and CSF levels of NSE ranged up to 10 ng/mL, but very high levels were not observed. In patients with partial seizures, the NSE level in the CSF and the ratio of the CSF to serum NSE levels showed a strong correlation with seizure duration. Conversely, there were no correlations between NSE levels and seizure duration in the patients with generalized seizures.Conclusions: These results indicate that FS seldom cause severe neurologic damage, but prolonged partial seizures may cause slight neuronal injury.
Patients' Aims for Epilepsy Surgery: Desires Beyond Seizure Freedom
Authors: Taylor David C. , McMackin Deirdre , Staunton Hugh , Delanty Norman , Phillips Jack 
Published: March 2003

Summary:  Purpose: To evaluate prospectively patient's aims for epilepsy surgery as previously outlined theoretically by Taylor et al. (Epilepsia 1997;3:625–30).Methods: Ninety‐three consecutive patients were interviewed by a psychiatrist as part of their evaluation for epilepsy surgery. Open‐ended questions about the patient were asked, and carers' aims or ambitions for change as a result of putative relief of seizures were elicited. The interviewer aimed to obtain a maximum of five aims for later follow‐up purposes. These questions were part of an extensive psychiatric interview that is described.Results: The aims of 69 patients or carers were analyzed. The 204 statements of aims were grouped into 59 categories initially. The five most frequently cited constituted 50% of all the aims listed. These aims were desire for work, driving of motor vehicles, independence, socializing, and freedom from drugs. The patients rarely identified a desire for improvement in cognitive functioning as an aim for epilepsy surgery. A final analysis into six categories showed that changes in social process predominated, even over changes in personal behavior.Conclusions: The social and personal aims to accompany relief of epilepsy identified by patients are consistent with the literature on psychosocial adjustment to epilepsy.