bluuebunny/crossref_metadata_2025_split huggingface dataset:
Direct Cost of Medical Management of Epilepsy among Adults in Italy: A Prospective Cost‐of‐Illness Study (EPICOS)
Authors: Beghi Ettore , Garattini Livio , Ricci Elena , Cornago Dante , Parazzini Fabio ,   EPICOS Group
Published: January 2004

Summary:  Purpose: To investigate the costs of epilepsy from a nationwide survey comparing adult patients included in different prognostic categories. Methods: A 12‐month prospective observational study was conducted in 15 epilepsy centers from Northern, Central, and Southern Italy. The study population included a random sample of individuals aged 18 years and older with newly diagnosed (ND) epilepsy, seizure remission (R), occasional seizures (OS), active non–drug‐resistant (NDR) seizures, drug‐resistant (DR) seizures, or surgical candidates (SC). Estimates of the direct costs of care of epilepsy were based on the use of diagnostic examinations, laboratory tests, specialist consultations, hospital admissions, day‐hospital days, and drugs, taking the Italian National Health Service perspective. Results: The sample included 631 patients (ND 62, R 158, OS 155, NDR 114, DR 128, and SC 14). The SC group had the highest total cost per patient (€3,619) followed by DR (€2,190), ND (€976), NDR (€894), OS (€830), and R (€561). For each epilepsy group, the main components of the total cost were drugs and hospital admissions. Drug costs increased from the R group to the DR group. The new antiepileptic drugs (AEDs) were the largest part of the cost of treatment. Conclusions: The costs of epilepsy in referral patients vary significantly according to the time course of the disease and the response to treatment. Hospital admissions and drugs are the major sources of expenditure.
Mutations of Neuronal Voltage‐gated Na+ Channel α1 Subunit Gene SCN1A in Core Severe Myoclonic Epilepsy in Infancy (SMEI) and in Borderline SMEI (SMEB)
Authors: Fukuma Goryu , Oguni Hirokazu , Shirasaka Yukiyoshi , Watanabe Kazuyoshi , Miyajima Tasuku , Yasumoto Sawa , Ohfu Masaharu , Inoue Takahito , Watanachai Aruchalean , Kira Ryutaro , Matsuo Muneaki , Muranaka Hideki , Sofue Fumiko , Zhang Bo , Kaneko Sunao , Mitsudome Akihisa , Hirose Shinichi 
Published: January 2004

Summary:  Purpose: Severe myoclonic epilepsy in infancy (SMEI) is a distinct epilepsy syndrome. Patients with borderline SMEI (SMEB) are a subgroup with clinical features similar to those of core SMEI but are not necessarily consistent with the accepted diagnostic criteria for core SMEI. The aim of this study was to delineate the genetic correlation between core SMEI and SMEB and to estimate the frequency of mutations in both phenotypes. Methods: We examined 96 healthy volunteers and 58 unrelated individuals whose clinical features were consistent with either core SMEI (n = 31) or SMEB (n = 27). We screened for genetic abnormalities within exons and their flanking introns of the genes encoding major subunits of the Na+ channels (SCN1A, SCN2A, SCN1B, and SCN2B) by using a direct sequencing method. Results: In both core SMEI and SMEB, various mutations of SCN1A including nonsense and missense mutations were identified, whereas no mutations of SCN2A, SCN1B, and SCN2B were found within the regions examined. All mutations were heterozygous and not found in 192 control chromosomes. Mutations were identified in 26 (44.8%) of the 58 individuals and were more frequent (p < 0.05) in core SMEI (19 of 31) than in SMEB (seven of 27), as assessed by the continuity‐adjusted χ2 test. Mutations resulting in a molecular truncation were found only in core SMEI. Among the mutations, two missense mutations were found in both core SMEI and SMEB. Conclusions: Our findings confirm that SMEB is part of the SMEI spectrum and may expand the recognition of SMEI and suggest other responsible or modifying genes.
Recurrence after a First Unprovoked Cryptogenic/Idiopathic Seizure in Children: A Prospective Study from São Paulo, Brazil
Authors: Scotoni Anna E. , Manreza Maria L. G. , Guerreiro Marilisa M. 
Published: January 2004

Summary:  Purpose: To evaluate the recurrence risk after a first unprovoked seizure in a large population of children and adolescents of a developing country. Methods: This prospective study was conducted at two tertiary hospitals, between September 1989 and August 1998. Children were enrolled if they had a first unprovoked cryptogenic/idiopathic seizure and maximal interval to the enrollment ≤90 days. EEG and computed tomography (CT) were performed in most patients. Potential predictors of recurrence were compared by using the Cox proportional hazards model in univariate and multivariate analyses. Survival analysis was performed by using the Kaplan–Meier curves. Results: Two hundred thirteen children were included. Recurrence occurred in 34% of the patients, and mean time for recurrence was 12 months. Statistical analysis showed significance for seizure recurrence only for patients with abnormal EEGs. CT was performed in 182 patients, and abnormalities were found in 9.5%. Small calcifications were the most frequent finding, and this was not a predictor for recurrence. Conclusions: The risk of recurrence after a first unprovoked seizure in children from a developing country is similar to that found in developed countries. An abnormal EEG is a risk factor for seizure recurrence in children with a cryptogenic/idiopathic seizure. Calcifications on CT do not increase the risk of recurrence.
Sulthiame in the Primary Therapy of West Syndrome: A Randomized Double‐blind Placebo‐controlled Add‐on Trial on Baseline Pyridoxine Medication
Authors: Debus Otfried Martin , Kurlemann Gerhard ,   Study Group
Published: January 2004

Summary:  Purpose: West syndrome (WS) is still one of the most difficult to treat epilepsies in infancy. Sulthiame (STM), which is commonly used in some countries in the treatment of benign focal epilepsies in childhood, has been suggested to be effective in WS too. This prospective, randomized placebo‐controlled study was designed to prove or refute this hypothesis. Methods: Thirty‐seven infants aged between 3.5 and 15 months with newly diagnosed WS received baseline therapy with pyridoxine (PDX). The children were randomized in a double‐blind fashion to STM (n = 20) or placebo (n = 17), starting at day 4 at a moderate dose of 5 mg/kg body weight. Without complete cessation of infantile spasms (ISs) and resolution of hypsarrhythmia as the definition criteria of a response, the dose was doubled at day 7. The final examination was undertaken at the end of day 9. Results: Based on the intention to treat, six (30%) of 20 patients responded to STM (p < 0.025), as did six (35%) of 17 infants fulfilling the study criteria (p < 0.01). Patients with tuberous sclerosis did not respond to STM (n = 3) No patient responded to placebo. One patient in the verum group was withdrawn because of reversible somnolence. Conclusions: As no child in the placebo group responded to the baseline PDX therapy, nor did any child in the STM group during the first 3 days of baseline therapy, PDX does not seem to be effective in WS. STM has a positive effect in the primary therapy of WS, comparable to that of vigabatrin.
Factor XIII Deficiency Associated with Valproate Treatment
Authors: Teich Martin , Longin Elke , Dempfle Carl‐Erik , König Stephan 
Published: January 2004

Summary:  Purpose: We present two children who developed a deficiency of factor XIII with valproate (VPA) treatment. This coagulation disorder has not been described in association with VPA treatment in children, and only very recently in one adult patient. Results: Both patients showed recurrent epistaxis as major clinical sign of a combination of decreased coagulation parameters (factor XIII deficiency with thrombocytopenia and decreased von Willebrand factor, respectively). A few days after reduction or withdrawal of VPA treatment, clinical symptoms disappeared, and laboratory findings were within normal range. Conclusions: VPA is known to influence the synthetic function of the liver and the number and function of megakaryocytes. Therefore an alteration of the factor XIII level by VPA is conceivable. Our case reports suggest that bleeding symptoms during VPA treatment may be caused or aggravated by a decreased factor XIII activity. A determination of factor XIII activity should be considered before surgical procedures during VPA treatment to minimize the risk of (severe) postsurgical bleeding complications.
Evaluation with α−[11C]Methyl‐l‐tryptophan Positron Emission Tomography for Reoperation after Failed Epilepsy Surgery
Authors: Juhász Csaba , Chugani Diane C. , Padhye Uma N. , Muzik Otto , Shah Aashit , Asano Eishi , Mangner Tom J. , Chakraborty Pulak K. , Sood Sandeep , Chugani Harry T. 
Published: January 2004

Summary:  Purpose: Reoperation after failed cortical resection can alleviate seizures in patients with intractable neocortical epilepsy, provided that previously nonresected epileptic regions are accurately defined and removed. Most imaging modalities have limited value in identifying such regions after a previous surgery. Positron emission tomography (PET) using α−[11C]methyl‐l‐tryptophan (AMT) can detect epileptogenic cortical areas as regions with increased tracer uptake. This study analyzed whether increased cortical AMT uptake can detect nonresected epileptic foci in patients with previously failed neocortical resection. Methods: Thirty‐three young patients (age 3–26 years; mean age, 10.8 years) with intractable epilepsy of neocortical origin, and a previously failed cortical resection performed at various epilepsy centers, underwent further presurgical evaluation for reoperation. AMT‐PET scans were performed 6 days to 7 years after the first surgery. Focal cortical areas with increased AMT uptake were objectively identified and correlated to ictal EEG data as well as clinical variables (age, postsurgical time, etiology). Results: Cortical increases of AMT uptake were detected on the side of the previous resections in 12 cases. In two patients scanned shortly (within a week) after surgery, diffuse hemispheric increases were observed, without any further localization value. In contrast, in 10 (43%) of 23 patients scanned >2 months but within 2.3 years after surgery, focal cortical increases occurred, concordant with seizure onset on ictal EEG. Age, etiology (lesional vs. cryptogenic), epileptiform EEG activity during PET, or time of the last seizure were not significantly related to the presence of increased AMT uptake. All patients with localizing AMT‐PET, who underwent reoperation, became seizure free (n = 5) or showed considerable improvement of seizure frequency (n = 2). Conclusions: AMT‐PET can identify nonresected epileptic cortex in patients with a previously failed neocortical epilepsy surgery and, with proper timing for the scan, can assist in planning reoperation.
Resective Surgery for Intractable Focal Epilepsy in Patients with Low IQ: Predictors for Seizure Control and Outcome with Respect to Seizures and Neuropsychological and Psychosocial Functioning
Authors: Bjørnæs Helge , Stabell Kirsten Engberg , Heminghyt Einar , Røste Geir Ketil , Bakke Søren Jacob 
Published: January 2004

Summary: Purpose:To investigate possible predictive factors for seizure control in a group of children and adults with low IQs (IQ, ≤70) who underwent resective surgery for intractable focal epilepsy and to study outcome with respect to seizures and neuropsychological functioning. We also studied psychosocial outcome in the adult patients.Methods:Thirty‐one patients (eight children younger than 18 years) with a Wechsler Full Scale IQ of 70 or less underwent comprehensive neuropsychological assessments before and 2 years after surgery. Adults also completed the Washington Psychosocial Seizure Inventory (WPSI). Univariate analyses were used to identify variables differentiating between patients who became seizure free and those who did not. Pre‐ and postoperative test results were compared byttest for dependent samples.Results:Forty‐eight percent of the patients became seizure free, 52% of those with temporal lobe resection and 38% of those with extratemporal resection. Only one variable was predictive for seizure outcome: duration of epilepsy. In one third of the patients, who had the shortest duration of epilepsy (<12 years), 80% became seizure free. Significant improvement was seen regarding vocational adjustment in adults (WPSI). Seizure‐free adults improved their Full Scale IQ scores. No cognitive changes were found in seizure‐free children or in patients who did not become seizure free.Conclusions:A good seizure outcome was obtained after resective surgery in patients with intractable focal epilepsy and low IQ, provided that treatment was done relatively shortly after onset of epilepsy. No adverse effects were seen on cognitive and psychosocial functioning.
Status Epilepticus in a Population‐based Virginia Twin Sample
Authors: Corey Linda A. , Pellock John M. , DeLorenzo Robert J. 
Published: January 2004

Summary:  Purpose: To characterize status epilepticus (SE) and estimate its frequency of first occurrence, as well as to assess the contribution of genetic factors to risk of SE occurrence in a sample of Virginia‐born twins ascertained from the population‐based Mid‐Atlantic Twin Registry. Methods: The occurrence of SE was determined in 13,506 unselected Virginia‐born twin pairs ascertained from birth records. Twins included in the study were between ages 2 and 75 years when surveyed. History of seizures and SE was validated through medical records and by detailed personal or parental interviews. Results: Among 381 twins included in 332 pairs with a verified history of seizures, 70 (18.4%) were validated to have had at least one episode of SE. The frequency of first SE in this sample was 309 per 100,000 twins. First SE occurred in conjunction with 21 of 158 febrile and 49 of 223 afebrile seizure cases, respectively. Mean length of SE episode was 76.2 ± 14.9 min. Age at first SE occurrence ranged from 2 months to 59 years. All concordant twin pairs in the sample were monozygotic (MZ), with a proband‐wise concordance rate estimated for SE in this population of 0.31 [95% confidence interval (CI), 0.14–0.52) overall, and 0.67 (95% CI, 0.35–0.90) in pairs concordant for epilepsy. Conclusions: These results provide a direct estimate of the frequency of SE in a defined population of twins and afford further evidence for a genetic contribution to risk for SE.
Seizure Recurrence after Planned Discontinuation of Antiepileptic Drugs in Seizure‐free Patients after Epilepsy Surgery: A Review of Current Clinical Experience
Authors: Schmidt Dieter , Baumgartner Christoph , Löscher Wolfgang 
Published: January 2004

Summary: Purpose:Although epilepsy surgery, especially temporal lobe epilepsy surgery, is well established to control seizures in patients remaining on antiepileptic drug (AED) treatment, less information is available about how many seizure‐free surgical patients will relapse after discontinuation of AEDs under medical supervision.Methods:A literature review yielded six retrospective clinical observations.Results:After planned discontinuation of AEDs in patients rendered seizure free after epilepsy surgery, most often various forms of temporal lobe surgery, the mean percentage recurrence rate in adults in four studies was 33.8%[95% confidence interval (CI), 32.4–35.2%], with maximum follow‐up ranging from 1 to 5 years. Seizure recurrence increased during the follow‐up of 1 to 3 years and occurred within 3 years of AED discontinuation. In one study of children with temporal lobe epilepsy, the recurrence rate was 20%. More than 90% of adult patients with seizure recurrence regained seizure control with reinstitution of previous AED therapy. Seizure recurrence was unaffected by the duration of postoperative AED treatment; as a consequence, delaying discontinuation beyond 1 to 2 years of complete postoperative seizure control seems to have no added benefit. The occurrence of rare seizures or auras after surgery did not eliminate the possibility of eventual successful AED discontinuation.Conclusions:AED discontinuation is associated with a seizure recurrence in one in three patients rendered seizure free by epilepsy surgery. These results will be useful in counseling patients about discontinuing AED treatment after successful epilepsy surgery.
Stimulus‐induced Rhythmic, Periodic, or Ictal Discharges (SIRPIDs): A Common EEG Phenomenon in the Critically Ill
Authors: Hirsch Lawrence J. , Claassen Jan , Mayer Stephan A. , Emerson Ronald G. 
Published: January 2004

Summary:  Purpose: To describe an underrecognized EEG phenomenon in critically ill patients undergoing continuous EEG monitoring (cEEG). We named these EEG patterns stimulus‐induced rhythmic, periodic, or ictal discharges (SIRPIDs). Methods: We reviewed 150 consecutive patients undergoing cEEG during a 9‐month period and compared those with and without SIRPIDs. SIRPIDs were defined as periodic, rhythmic, or ictal‐appearing discharges that were consistently induced by alerting stimuli. Results: We identified 33 patients with SIRPIDs (22%). SIRPID patterns included periodic epileptiform discharges in 21 patients (nine lateralized) and rhythmic patterns with evolution that fulfilled criteria for ictal discharges in 18 patients (12 unilateral). Eight patients had prior epilepsy; 24 had acute brain injury. Half the patients (17 of 33) had seizures, clinical or subclinical, during the acute illness in addition to SIRPIDs, and half (16 of 33) did not. No significant difference was found in the incidence of clinical seizures in patients with SIRPIDs (30%) compared with those without (45%). Clinical status epilepticus was more common in patients with focal (43%) or ictal‐appearing (33%) SIRPIDs than in those without SIRPIDs (17%). Conclusions: Rhythmic, periodic, or ictal‐appearing EEG patterns are commonly elicited by stimulation in critically ill, encephalopathic patients. Recording video, documenting patient stimulation, or repetitively examining patients during cEEG is necessary to recognize these patterns and to differentiate SIRPIDs from spontaneous seizures. Further research is necessary to determine the pathophysiologic, prognostic, and therapeutic significance of SIRPIDs.
Vanishing Points, Turning Points, and Points of No Return: Geographies of Somewhere, Elsewhere, and Nowhere
Authors: Houe Poul 
Published: January 2004

This article mines textual representations – by Max Frisch, Klaus Mann, Rainer Maria Rilke, and Peter Weiss – of typically discounted twentieth century Western positions and traditions for their possible clues as to the kind of cultural displacements of which Western civilization is said to be uniquely unaware, disinterestedly aware or disinterested in. If current modulations of cultural strangeness and estrangement have indeed aggravated the erosion of representational prospects, it lies near at hand to visit this recent decline as an instance of Eurocentric ways as still encoded, perhaps encrypted, in their subaltern Other(s).Univocal subject positionality‐or identity‐is the foremost casualty in all the texts under scrutiny, and painful after‐effects ensue from the implicit moments of textual crisis. Yet subsequent exilic mindsets and mental geographies of repositioning show little evidence of radical reconfiguration, let alone multiplication, of foundational priorities or sensibilities. In most instances, the sense of cultural landscape and cultural mobility merely contracts to inescapable points of historical gravity, from which the interpretational trajectory rarely bespeaks a turning point, but all the more so a point of no return or a virtual vanishing point.All the same, disfiguration of positionality and identity remains potentially translatable into transfiguration. The original and traditional‐the home‐enables a process of travel, hybridization, and othering, provided the cultural privilege of seeing is unlearned along the way. For that to happen, the process of unlearning must incorporate the process of learning as its sine qua non.
Five‐Finger Exercises: Mika Waltari's Detective Stories
Authors: Pyrhönen Heta 
Published: January 2004

This essay addresses the question of what happens when authors import into their own culture a genre whose structures and conventions have been moulded in another culture. If the imported structures and conventions include a certain value system, does an author's adaptation cause them to express markedly different values than they do in their original context? I explore this question by analysing the detective stories by Mika Waltari, a reowned Finnish author, who used both the British whodunit and Edgar Allan Poe's Dupin stories as models.I first consider Waltari's use of specific generic conventions and consider the national values he makes them express. I then analyse Waltari's insertion of himself into the textual roles of detective and culprit in order to examine the link between writing detective stories and ideology. I show how Waltari creates a fundamental discrepancy between the whodunit world and the Finnish context in which he sets this world in order to emphasize the literariness of the imitated model. In his hands, writing detective stories becomes first and foremost a literary exercise that enables him to show his skilful, self‐reflexive, and ironic play with literary forms and conventions.
Helena Fourment's Het Pelsken
Authors: Thøfner Margit 
Published: January 2004

This article focuses on Het Pelsken, a portrait by Peter Paul Rubens depicting his second wife Helena Fourment in a state of undress. It takes its cue from the fact that, for eighteen years of her life, Helena was the undisputed owner of Het Pelsken. The central aim is to give an historically grounded yet nuanced account of Helena's spectatorship in relation to an erotically charged and thus, in the early modern period, potentially troubling image. This, in turn, may function as a starting point for more profound inquiries into how women use their eyes when looking at erotically charged imagery. Margit Thøfner is a lecturer in art history in the School of World Art Studies and Museology at the University of East Anglia. She has published articles in the Oxford Art Journal, in the Nederlands Kunsthistorisch Jaarboek and in Emblematica. She has received an AHRB grant to investigate courtly and civic festivals in the Habsburg Netherlands in the early modern period.
Icon and narration in eighteenth‐century Christian Egypt: the works of Yuhanna al‐Armani al‐Qudsi and Ibrahim al‐Nasikh
Authors: Tribe Tania C. 
Published: January 2004

The many icons painted by Yuhanna, an Armenian from Jerusalem, and/or Ibrahim, a Coptic scribe, suggest that they headed an important workshop in eighteenth‐century Cairo, with links to the post‐Byzantine schools of Greater Syria, particularly Aleppo and Jerusalem. Their depictions of biblical episodes and hagiographical narratives often articulate words and images to clarify the story being told and the identity of the holy person represented. The gradual breakdown of Ottoman organization in the eighteenth century, partly due to Western influence, led to an influx of Christians from Greater Syria and a renewal of confidence in the local Coptic community. Coptic patrons shared in the wider revival of Egyptian Muslim scholarship, applying the common emphasis on grammar and rhetoric to the recovery of their own cultural memory. This was reflected in the taste for complex verbal‐visual devices in icons, the iconography of which included many local martyr saints, reinforcing Coptic identity. Tania C. Tribe lectures in art history and theory in the School of Oriental and African Studies, University of London. She has a special interest in the arts of Christian north‐eastern Africa, and has published several articles in this field. She is currently preparing a book on the visual culture of Christian Egypt.