bluuebunny/crossref_metadata_2025_split huggingface dataset:
Lost Causes
Authors: Hoover Kevin D. 
Published: July 2004

Often papers begin with an idea. Once the paper is written, sometimes little sometimes much effort goes into finding a title. This lecture worked the other way round. It started with the title, which I passed on to Roy Weintraub, my successor as President of the Society, when I still had but the vaguest idea ol what I would write. When Roy heardmy title he pointed me to a passage from C. Vann Woodward, that he hadhimself quotedinStablizing Dynamics:Lost causes, especially those that foster loyalties and nostalgic memories are among the most prolific breeders of historiography. If survivors deem the cause not wholly lost andperhaps in some measure retrievable, the search of the past becomes more frantic and the books about it more numerous. Blame must be fixed, villains found, heroes celebrated, old quarrels settled, old dreams restored, and motives vindicated. Amid the ruins controversy thrives and books proliferate (quoted by Weintraub 1991, p. 125, from Vann Woodward 1986).
Oxygenation Prevents Sudden Death in Seizure‐prone Mice
Authors: Venit Elizabeth L. , Shepard Blythe D. , Seyfried Thomas N. 
Published: July 2004

Summary:  Purpose: Sudden unexpected death in epilepsy (SUDEP) is a significant health problem for some with epilepsy, but no preventive therapy has been developed for high‐risk patients. Sudden death in mice, after sound‐induced (audiogenic) seizures, can model the seizure‐associated respiratory arrest that contributes to SUDEP in some humans and may be useful for evaluating potential SUDEP therapies. This study evaluated the effects of oxygenation on sudden fatal audiogenic seizures (AGSs) in three mouse strains, DBA/2J (D2), B6SAS, and primed C57BL/6J (B6) that differ in genetic background and seizure etiology (idiopathic or acquired). Methods: The incidence of fatal AGSs was measured in (a) a normal air environment; (b), an oxygen‐rich environment; (c) a normal air environment 24 h after a nonfatal AGS; (d) an oxygen‐rich environment 24 h after a nonfatal AGS; and (e) a normal air environment 60 s after exposure to an oxygen‐rich environment. Results: Sudden, fatal AGS occurred in 100%, 100%, and 58% of the D2, B6SAS, and primed‐B6 mice, respectively, in the air environment. Oxygenation completely prevented fatal AGSs in each strain, but had no effect on seizure incidence or severity. Furthermore, the protective effect of oxygenation against fatal seizures occurred only when the mice were in an oxygen‐rich environment. Conclusions: These findings indicate that oxygenation prevents sudden death in seizure‐prone mice and suggest that oxygenation may protect some seizure‐prone humans at risk for SUDEP.
Preclinical Profile of Combinations of Some Second‐generation Antiepileptic Drugs: An Isobolographic Analysis
Authors: Luszczki Jarogniew J. , Czuczwar Stanislaw J. 
Published: July 2004

Summary:  Purpose: The need for an efficacious treatment of patients with intractable seizures is urgent and pressing, because ∼30% of epilepsy patients worldwide are still inadequately medicated with current frontline antiepileptic drugs (AEDs). This study sought to determine the interactions among some newer AEDs [topiramate (TPM), felbamate (FBM), oxcarbazepine (OXC), and lamotrigine (LTG)] in the maximal electroshock‐induced seizures (MES) and chimney test (motor performance) in mice, by using the isobolographic analysis. Methods: Evaluation of the anticonvulsant and acute adverse (neurotoxic) effects in mice produced by the AEDs in combinations at the fixed ratios of 1:3, 1:1, and 3:1 allowed the assessment of their preclinical profile and the determination of benefit indices (BIs) for all individual combinations. Results: Combinations of TPM+FBM at the fixed ratios of 1:3, 1:1, and 3:1 offered supraadditive (synergistic) interactions against electroconvulsions and subadditivity (antagonism) in terms of acute neurotoxic effects in the chimney test (BIs ranged between 1.90 and 2.59, the best combinations from a preclinical point of view). The examined combinations of TPM+OXC also were advantageous due to synergistic interactions in the MES, and additivity in terms of acute neurotoxic effects produced by the AEDs (BIs ranged between 1.35 and 1.71). In contrast, OXC+FBM exerted subadditive (antagonistic) interactions in the MES test and additive interactions in terms of acute motor impairment of animals (BIs ranged between 0.53 and 0.71). The worst combination was observed for OXC+LTG, at the fixed ratio of 1:1, displaying subadditivity (antagonism) against electroconvulsions and supraadditivity (synergy) with respect to neurotoxicity (BIs, 0.43). The remaining combinations of OXC+LTG tested (i.e., 1:3 and 3:1) exerted additivity in the MES test and supraadditivity in the chimney test (BIs 0.54 and 0.49, respectively). None of the studied AEDs affected the brain concentrations of other AEDs, so the existence of any pharmacokinetic interactions to be responsible for the observed effects is improbable. Conclusions: Based on the current preclinical data, the pharmacological profile of combinations of TPM+FBM and TPM+OXC evaluated with isobolography was beneficial and might be worth recommendation to further clinical practice. In contrast, utmost caution is required during the use of OXC+FBM or OXC+LTG in clinical practice, because of the high risk of neurotoxic adverse effect appearance.
Chromosomal Mapping of Genetic Loci Controlling Absence Epilepsy Phenotypes in the WAG/Rij Rat
Authors: Gauguier Dominique , Luijtelaar Gilles van , Bihoreau Marie Thérèse , Wilder Steven P. , Godfrey Richard F. , Vossen Jo , Coenen Anton , Cox Roger D. 
Published: July 2004

Summary:  Purpose: The WAG/Rij rat is among the most appropriate models for the study of spontaneous childhood absence epilepsy, without complex neurologic disorders that are associated with some mouse models for absence epilepsy. Previous studies have allowed the identification of distinct types of spike–wave discharges (SWDs) characterizing seizures in this strain. The purpose of this study was to investigate the genetic basis of electroencephalographic (EEG) properties of SWDs. Methods: An intercross was derived from WAG/Rij and ACI inbred strains that are known to differ substantially in the number of SWDs. Phenotypic analyses based on 23‐h EEG recording in all progenies allowed the quantification of type I and type II SWD phenotypes. A genome‐wide scan was performed with 145 microsatellite markers, which were used to test for evidence of genetic linkage to SWD quantitative phenotypes. Results: We were able to map quantitative trait loci independently, controlling type I and type II SWD variables to rat chromosomes 5 and 9. Strongest linkages were obtained for D5Mgh15 and total duration of type II SWD (lod, 3.64) and for D9Rat103 and the average duration of type I SWD (lod, 3.91). These loci were denoted T2swd/wag and T1swd/wag, respectively. Conclusions: The independent genetic control of type I and type II SWDs underlines the complexity of the molecular mechanisms participating in SWDs. The identification of these genetic loci represents an important step in our fundamental knowledge of the architecture of SWDs and may provide new insights for resolving the genetic heterogeneity of absence epilepsy.
Lateralization of Temporal Lobe Epilepsy and Learning Disabilities, as Defined by Disability‐related Civil Rights Law
Authors: Butterbaugh Grant , Olejniczak Piotr , Roques Betsy , Costa Richard , Rose Marcy , Fisch Bruce , Carey Michael , Thomson Jessica , Skinner John 
Published: July 2004

Summary:  Purpose: Epilepsy research has identified higher rates of learning disorders in patients with temporal lobe epilepsy (TLE). However, most studies have not adequately assessed complex functional adult learning skills, such as reading comprehension and written language. We designed this study to evaluate our predictions that higher rates of reading comprehension, written language, and calculation disabilities would be associated with left TLE versus right TLE. Methods: Reading comprehension, written language, and calculation skills were assessed by using selected subtests from the Woodcock‐Johnson Psycho‐Educational Tests of Achievement–Revised in a consecutive series of 31 presurgical patients with TLE. Learning disabilities were defined by one essential criterion consistent with the Americans with Disabilities Act of 1990. Patients had left hemisphere language dominance based on Wada results, left or right TLE based on inpatient EEG monitoring, and negative magnetic resonance imaging (MRI), other than MRI correlates of mesial temporal sclerosis. Results: Higher rates of reading comprehension, written language, and calculation disabilities were associated with left TLE, as compared with right TLE. Nearly 75% of patients with left TLE, whereas fewer than 10% of those with right TLE, had at least one learning disability. Conclusions: Seizure onset in the language‐dominant hemisphere, as compared with the nondominant hemisphere, was associated with higher rates of specific learning disabilities and a history of poor literacy or career development or both. These results support the potential clinical benefits of using lateralization of seizure onset as a predictor of the risk of learning disabilities that, once evaluated, could be accommodated to increase the participation of patients with epilepsy in work and educational settings.
Perceived Impact of Childhood‐onset Epilepsy on Quality of Life as an Adult
Authors: Sillanpää Matti , Haataja Leena , Shinnar Shlomo 
Published: July 2004

Summary:  Purpose: Childhood‐onset epilepsy is a common disorder. The long‐term impact of having childhood epilepsy on quality of life (QOL) as an adult, whether or not seizures are in remission, has not been systematically studied. Methods: A population‐based cohort of 245 children younger than 16 years with active epilepsy between 1961 and 1964 residing in the catchment area of Turku University Hospital was followed up prospectively until 1997. Of the 99 surviving cases with uncomplicated epilepsy and 99 matched population controls, 91 subjects and controls completed questionnaires on QOL and psychosocial outcomes. Results: Of the 91 subjects, 61 (67%) were in remission off medication, 13 (14%) in remission on medications, and 17 (19%) were not in remission. Subjects on medication, whether in remission or not, had worse scores on both general measures of QOL and epilepsy‐specific measures than did either controls or subjects in remission off medications. They also had significantly higher rates of unemployment (p < 0.001) and lower socioeconomic status. These differences could not be accounted for by differences in education or seizure frequency. Subjects in remission off medication had rates of employment and socioeconomic status similar to those of controls. All subjects, regardless of remission status, had lower rates of marriage and of having children than did controls (p < 0.001). Conclusions: Childhood‐onset epilepsy has a persistent long‐term adverse impact on health‐related quality of life. The major impact is on those still on medications as adults, whether or not they are in remission. The impact on those in remission off medications is relatively modest.
Dystonic Posturing Associated with Putaminal Hyperperfusion Depicted on Subtraction SPECT
Authors: Mizobuchi Masahiro , Matsuda Kazumi , Inoue Yushi , Sako Kazuya , Sumi Yoshihiro , Chitoku Shiro , Tsumaki Katsumi , Takahashi Masaaki 
Published: July 2004

Summary:  Purpose: Dystonic posturing (DP) is one of the most reliable lateralizing indicators for temporal lobe epilepsy (TLE). We evaluated the ictal hyperperfusional areas in patients with DP by using ictal–interictal subtraction single‐photon emission computed tomography (SPECT). Methods: Ninety‐seven patients were treated surgically for intractable TLE, and 39 patients underwent ictal and interictal SPECT studies with the same isotope. These patients were divided into three groups: group I with DP of the contralateral side extended to the epileptogenic focus, group II with elevated muscle tonus but without DP, and group III without DP or alteration of muscle tonus. Ictal, interictal SPECT and thin‐slice magnetic resonance imaging (MRI) were overlaid by using the automatic multimodality registration program to construct ictal–interictal subtracted images of SPECT on MRI. Results: Thirteen patients belonged to group I; 14, to group II; and 12, to group III. A statistically significant difference in hyperperfusion rate was observed in the putamen (10 patients in group I, three in group II and two in group III; p < 0.01) and mesial temporal lobe (10 patients in group I, seven in group II, and two in group III; p < 0.05) on the ipsilateral side of the epileptogenic focus. No statistically significant difference was observed for other ictal symptoms except ipsilateral upper‐limb automatism (eight patients in group I, three in group II, and none in group III; p < 0.01). Conclusions: A strong correlation between DP and hyperperfusion in the putamen and mesial temporal lobe was demonstrated. Some patients showed a wide hyperperfusion area extending from the mesial temporal lobe to putamen, which may correspond to the propagation of epileptic discharges. Our results suggest a correlation between hyperperfusion of putamen and contralateral dystonic posturing.
Evaluating the Utility of Inpatient Video‐EEG Monitoring
Authors: Ghougassian Daniel F. , D'Souza Wendyl , Cook Mark J. , O'Brien Terence J. 
Published: July 2004

Summary:  Purpose: Inpatient video‐EEG monitoring (VEM) is widely used for the diagnosis, seizure classification, and presurgical evaluation of patients with seizure disorders. It is resource intensive and relatively expensive, so its utility continues to be debated. Few studies have specifically evaluated the utility of inpatient VEM in altering diagnosis or management of patients with seizure disorders. We sought to assess the proportion of patients for whom the preadmission diagnosis and management were altered after inpatient VEM of patients admitted for diagnostic and presurgical evaluation of seizure disorders. Methods: Data from a consecutive cohort of patients admitted over a 3‐year period to an inpatient VEM unit in a tertiary referral hospital were retrospectively analyzed. The preadmission diagnosis and management by the referring neurologist was compared with the diagnosis and management after the VEM. Results: Of 131 patients, 91 (70%) were admitted for diagnostic evaluation and 39 (30%) for a presurgical workup. Mean evaluative period was 5.6 days. Mean number of seizures recorded was 2.9. No seizures were recorded in 31% of patients. Interictal EEG showed epileptiform changes in 56 (43%). In 76 (58%), the diagnosis was altered as a result of the VEM, with the greatest change being an increase in the nonepileptic diagnosis group (7% to 31%) and the generalized diagnosis group (5% to 11%). Management was changed after the VEM in 95 (73%). Conclusions: The results of this study demonstrate that inpatient VEM has a high yield in changing diagnosis and management. Future long‐term cost–benefit studies of the management changes resulting from VEM evaluation will aid in further reinforcing its role.
Prescience as an Aura of Temporal Lobe Epilepsy
Authors: Sadler and R. Mark , Rahey Susan 
Published: July 2004

Summary:  Purpose: A patient with a distinct aura of prescience as a manifestation of temporal lobe epilepsy was encountered. The experience prompted a review of this ictal phenomenon among patients attending a tertiary care epilepsy outpatient clinic. Methods: A computer epilepsy database was searched for patients with simple partial sensory seizures and complex partial seizures with auras. Identified patients had charts reviewed for details of the auras; patients were contacted and asked to provide written descriptions of their experiences. Literature searches (PubMed) were done by using the terms “precognition” or “prescience” and “seizures” or “epilepsy.” Standard comprehensive epilepsy textbooks were reviewed. Results: The charts of 218 patients were reviewed from 927 in the database; three had prescience as an ictal feature. The patients' descriptions were very similar in all cases (a profound sense of “knowing” what was going to happen in their environment in the immediate future). The experience was distinct from déjà vu and other psychic experiences. All patients probably have temporal lobe epilepsy. Only one other description of prescience as an ictal feature was found in the literature. Conclusions: Prescience can occur as an ictal feature of temporal lobe epilepsy and represents a previously underreported psychic phenomenon. The potential lateralizing value of this symptom is yet to be determined.
Risk factors for hypospadias in Norwegian boys – association with testicular dysgenesis syndrome?
Authors: Aschim Elin Leirvoll , Haugen Trine B. , Tretli Steinar , Daltveit Anne Kjersti , Grotmol Tom 
Published: July 2004

SummaryIt has been proposed that hypospadias, cryptorchidism and testicular cancer, as well as decreasing sperm quality are symptoms of an underlying entity called testicular dysgenesis syndrome (TDS). We wanted to study the risk factors for hypospadias and compare them with those of the other conditions belonging to TDS. A large case–control study was undertaken on data on all live‐born boys registered in the Medical Birth Registry of Norway during the period 1967–1998 (n = 961 396; hypospadias cases = 2382). Logistic regression analysis was used to study the association between potential risk factors and hypospadias, estimated by odds ratio (OR). The risk factors for hypospadias were divided into four categories: (i) maternal characteristics, e.g. low parity [p(trend) < 0.001], hypertension (OR = 1.49) and bleeding (OR = 1.39) during index pregnancy, and (pre)eclampsia (OR = 1.84); (ii) complications during delivery, e.g. retained placenta (OR = 1.67) or Caesarean section (OR = 1.36); (iii) characteristics of the newborn, e.g. low birth weight [p(trend) < 0.001], small for gestational age (OR = 2.16), and presence of congenital malformations other than hypospadias (OR = 2.72), e.g. inguinal hernia (OR = 5.65); (iv) prevalence among relatives of hypospadias cases, e.g. brother with hypospadias (OR = 20.81). The novel finding of retained placenta as a risk factor indicates that early malfunction of placenta could be a causative factor for hypospadias. When comparing with previously published risk factors for hypospadias, cryptorchidism and testicular cancer, we found that the following risk factors were common to all three conditions: low parity, low birth weight, low gestational age, inguinal hernia, bleeding during pregnancy and Caesarean section. In conclusion, our results support the notion that the conditions of TDS share risk factors.
EAA/EMQN best practice guidelines for molecular diagnosis of y‐chromosomal microdeletions. State of the art 2004
Authors: Simoni M. , Bakker E. , Krausz C. 
Published: July 2004

SummaryMicrodeletions of the Y chromosome are the second most frequent genetic cause of spermatogenetic failure in infertile men after the Klinefelter syndrome. The molecular diagnosis of Y‐chromosomal microdeletions is routinely performed in the workup of male infertility in men with azoospermia or severe oligozoospermia. Since 1999, the European Academy of Andrology (EAA) and the European Molecular Genetics Quality Network (EMQN) support the improvement of the quality of the diagnostic assays by publication of the laboratory guidelines for molecular diagnosis of Y‐chromosomal microdeletions and by offering external quality assessment trials. The present revision of the 1999 laboratory guidelines summarizes the results of a ‘Best Practice Meeting’ held in Florence (Italy) in October 2003. The basic protocol for microdeletion screening suggested in the 1999 guidelines proved to be very accurate, sensitive and robust. In the light of the recent advance in the knowledge of the Y chromosome sequence and of the mechanism of microdeletion it was agreed that the basic 1999 protocol, based on two multiplex polymerase chain reactions each covering the three AZF regions, is still fully valid and appropriate for accurate diagnosis.
Somatostatin‐14 Actions on Dopamine‐ and Pituitary Adenylate Cyclase‐Activating Polypeptide‐Evoked Ca2+ Signals and Growth Hormone Secretion
Authors: Yunker W. K. , Chang J. P. 
Published: July 2004

AbstractUsing single‐cell Ca2+ imaging and a growth hormone (GH) radioimmunassay, we investigated somatostatin‐14 (SS14) inhibition of cAMP‐dependent, stimulated GH secretion from primary cultures of dispersed goldfish pituitary cells. The dopamine‐D1 receptor agonist SKF‐38393, and the hypothalamic neuropeptide pituitary adenylate cyclase‐activating polypeptide (PACAP) both elevated intracellular Ca2+ concentration ([Ca2+]i) and stimulated GH release. When increases in [Ca2+]i were prevented by intracellular loading of BAPTA, a Ca2+ chelator, SKF‐38393‐ and PACAP‐stimulated GH release were inhibited, suggesting that these Ca2+ signals are required for stimulated GH release. SS14 inhibited SKF‐38393‐ and PACAP‐stimulated GH release, but did not prevent these Ca2+ signals. Kinetic analysis revealed that SS14 lowered the maximum amplitude of the SKF‐38393‐ and PACAP‐evoked Ca2+ responses, but had no effect on other aspects of the Ca2+ signal. We then examined the ability of SS14 to act subsequent to dopamine‐D1 or PACAP receptor activation using the adenylate cyclase activator forskolin, or the membrane permeant cAMP analogue 8Br‐cAMP. Forskolin and 8Br‐cAMP both increased [Ca2+]i and GH secretion and, as expected, SS14 inhibited the resultant GH release. Although SS14 significantly increased the time to maximum amplitude of the forskolin‐evoked Ca2+ signals, it had no detectable effect on any of the kinetic parameters used to describe the Ca2+ signals evoked by 8Br‐cAMP. Taken together, these results establish that SS14 has the ability to suppress Ca2+‐dependent exocytosis by acting distal to elevations in [Ca2+]i. Furthermore, it appears likely that the cellular mechanisms underlying the observed effects of SS14 on Ca2+ signalling are upstream of cAMP and may be unrelated to those responsible for inhibiting GH release.
Glutamic Acid Decarboxylase 67 (GAD67) Gene Expression in Discrete Regions of the Rostral Preoptic Area Change During the Oestrous Cycle and with Age
Authors: Cashion A. B. , Smith M. J. , Wise P. M. 
Published: July 2004

AbstractGamma‐aminobutyric acid (GABA), an inhibitory neurotransmitter, is important to the timing and amplitude of the gonadotrophin‐releasing hormone (GnRH)‐induced luteinizing hormone (LH) surge on pro‐oestrus. Data suggest that GABA input in the preoptic area must decrease for a normal LH surge to occur in young rats. We have previously found that ageing alters the timing and amplitude of the LH surge. Therefore, this study focused on changes in GAD67 gene expression, a reflection of GABA synthesis, in two regions of the rostral preoptic area, the organum vasculosum of the lamina terminalis (OVLT) and the anteroventral periventricular nucleus (AVPV) during the oestrous cycle and with age. We assessed the possibility that the expression of GAD67 mRNA in these regions displays time‐related and age‐dependent changes on pro‐oestrus. Our results demonstrate that, with age, overall expression of GAD67 mRNA decreases in the area surrounding the OVLT and in the AVPV. Young rats display a diurnal rhythm in GAD67 mRNA in both regions. GAD67 mRNA expression is high during the early morning hours of pro‐oestrus and then declines around the time of the GnRH‐induced LH surge. In addition, the diurnal rhythm disappears in the AVPV and is attenuated in the area surrounding the OVLT of middle‐aged proestrous rats. These findings suggest that a loss of rhythmicity in GAD67 gene expression and maintenance of inhibitory tone on proestrous afternoon may alter the timing and amplitude of the LH surge, as previously observed in middle‐aged rats.