bluuebunny/crossref_metadata_2025_split huggingface dataset:
What’s shaking in the ICU? The differential diagnosis of seizures in the intensive care setting
Authors: Benbadis Selim R. , Chen Shirley , Melo Maria 
Published: August 2010

SummaryTo analyze what conditions mimic seizures in the intensive care unit (ICU) setting, we reviewed all bedside electroencephalography (EEG)–videos obtained in the adult ICU setting over an 18‐month period. Only those studies performed for “possible seizures” due to motor phenomena and whose clinical events were captured on video were analyzed. A total of 52 studies were performed. Fourteen patients (27%) had epileptic seizures. Thirty‐eight (73%) had nonepileptic events. These consisted of 12 (23%) with tremor‐like movements, 7 (13.5%) with multifocal myoclonic jerks without electrographic changes, 7 (13.5%) with slow semipurposeful movements, and 10 with other movements. Therefore, seizure mimics in the ICU setting were diverse and distinct from the usual differential diagnosis of seizures seen in ambulatory patients.
Comparative pharmacodynamic and pharmacokinetic analysis of two anticonvulsant halo derivatives of 2,2,3,3‐tetramethylcyclopropanecarboxamide, an amide of a cyclic analog of valproic acid
Authors: Pessah Neta , Kaufmann Dan , Yagen Boris , Hen Naama , Wlodarczyk Bogdan , Finnell Richard H. , Bialer Meir 
Published: August 2010

SummaryPurpose:  α‐Fluoro‐2,2,3,3‐tetramethylcyclopropanecarboxamide (α‐F‐TMCD) and α‐Cl‐TMCD, are α‐halo derivatives of TMCD, the corresponding amide of a cyclopropane analog of valproic acid (VPA). This study aimed to comparatively evaluate the pharmacodynamics and pharmacokinetics of α‐F‐TMCD and α‐Cl‐TMCD in rodent models of epilepsy and for antiepileptic drug (AED)–induced teratogenicity. The potential of α‐F‐TMCD as an antiallodynic and antinociceptive compound was also evaluated.Methods:  α‐F‐TMCD and α‐Cl‐TMCD were synthesized. α‐Cl‐TMCD anticonvulsant activity was evaluated in comparison to VPA in the mouse maximal‐electroshock‐seizure (MES), Metrazol (scMet), and 6‐Hz psychomotor‐seizure tests. Neurotoxicity was assessed by the Rotorod‐ataxia test. Induction of neural tube defects (NTDs) by α‐Cl‐TMCD and α‐F‐TMCD was evaluated after intraperitoneal administration to a mouse strain highly susceptible to VPA‐induced teratogenicity. The ability of α‐F‐TMCD to reduce pain was evaluated in the rat spinal nerve ligation (SNL) model for neuropathic pain and in the formalin test. α‐F‐TMCD and α‐Cl‐TMCD pharmacokinetics was evaluated following intraperitoneal (40 mg/kg) and oral (60 mg/kg) administration to rats.Results:  α‐F‐TMCD and α‐Cl‐TMCD had similar potencies in the 6‐Hz test and were more potent than VPA in this model and in the scMet test. Neither induced NTDs, and both exhibited wide safety margins. α‐F‐TMCD was active in the two pain models, and was found to be equipotent to gabapentin in the SNL model (ED50 = 37 and 32 mg/kg, respectively). Comparative pharmacokinetic analysis showed that α‐Cl‐TMCD is less susceptible to liver first‐pass effect than α‐F‐TMCD because of lower total (metabolic) clearance and liver extraction ratio.Conclusions:  Based on their potent anticonvulsant activity and lack of teratogenicity, α‐F‐TMCD and α‐Cl‐TMCD have the potential for development as new antiepileptics and central nervous system (CNS) drugs.
Semiologic aspects of epileptic seizures in 31 patients with hypothalamic hamartoma
Authors: Oehl Bernhard , Brandt Armin , Fauser Susanne , Bast Thomas , Trippel Michael , Schulze‐Bonhage Andreas 
Published: August 2010

SummaryPurpose: Characterization of seizure semiology in patients with hypothalamic hamartoma (HH) based on video–electroencephalography (EEG) monitoring (VEM).Methods: We retrospectively analyzed seizure semiology of 31 patients (20 male, mean age 23.5 years) who underwent VEM at the University Hospitals Freiburg or Heidelberg, Germany. Inclusion criteria were magnetic resonance evidence of an HH, no prior surgical or radiosurgical treatment, and at least two video‐documented seizures. A total of 263 seizures were included (mean number of seizures/patient 8.5, range 2–10). To analyze age‐dependent changes in seizure semiology, patients were grouped into “children” (3–11 years, n = 5), “adolescents” (12–17 years, n = 4), and “adults” (≥18 years, n = 22).Results: According to patient history, gelastic seizures had occurred in all patients, in 74% as the initial seizure type at epilepsy onset. In VEM, epileptic laughter varied from facial grinning to intense contractions of the diaphragm and body shaking. Unilateral motor signs were seen ipsi‐ and contralaterally to the HH. Tonic seizures were frequent and did not depend on the state of vigilance. Children, in contrast to adults and adolescents, did not show secondarily generalized tonic–clonic seizures, the gelastic component was the dominating and initial semiologic element, and seizures were significantly shorter.Conclusion: Seizure semiology is highly variable and age dependent. This may reflect network modulations with different propagation of ictal activity and/or secondary epileptogenesis. Detailed knowledge about such changes may contribute to both earlier recognition of seizures during childhood and better assignment of seizure types to a hypothalamic origin.
Generalized convulsive status epilepticus management in adults: A cohort study with evaluation of professional practice
Authors: Aranda Alexandre , Foucart Guillaume , Ducassé Jean Louis , Grolleau Sabrina , McGonigal Aileen , Valton Luc 
Published: August 2010

SummaryPurpose: Generalized convulsive status epilepticus (GCSE) needs immediate management. Despite guidelines, adherence to management protocols is often poor, this contributing to poor outcome. We aimed to evaluate the usefulness of a management protocol in GCSE.Methods: This is a prospective population‐based study of consecutive adults with GCSE in Haute‐Garonne district, France. Demographics and treatment procedures were documented. Factors associated with seizure termination and refractoriness were analyzed using logistic regression.Results: One hundred eighteen episodes in 111 adults (mean age 55 years) between October 2006 and February 2008 were included. SE was convulsive in 101 episodes. Incidence was 6.6 per 100,000 and case fatality was 9%. Adequate initial treatment according to protocol was observed in 38%; 64% were treated out‐of‐hospital, and SE was refractory in 27% [no response after two antiepileptic drugs (AED)]. Patients who received adequate first‐line treatment were 6.8 times [95% confidence interval (CI) 2.8–16.9] more likely to have seizure termination, with 74% versus 29% efficacy rate (p < 0.0001). Inadequate initial management was 4.7 times (1.9–11.1) more likely to need several benzodiazepine doses (p = 0.0004) and 9.1 times (3.7–20) more likely to require a long‐acting AED as next treatment (p < 0.0001). Seizure termination after initial treatment was associated with decreased intensive care unit stay [1 (1–2) versus 2 (1–5.5) days, p < 0.0001] and hospital stay [3 (2–11) versus 7 (3–18) days, p = 0.009].Discussion: GCSE termination and outcome seem clearly associated with adherence to treatment protocol. Results add to the debate on appropriate allocation of resources for out‐of‐hospital treatment, licensed drugs, and achievement of guideline implementation to improve SE outcome.
A case of SUDEP in a patient with Dravet syndrome with SCN1A mutation
Authors: Le Gal François , Korff Christian M. , Monso‐Hinard Christine , Mund Michael T. , Morris Michael , Malafosse Alain , Schmitt‐Mechelke Thomas 
Published: August 2010

SummaryA boy with a clinical history of pharmacologically resistant Dravet syndrome died suddenly after falling asleep. The autopsy concluded that the cause of death was sudden unexpected death in epilepsy (SUDEP). Postmortem molecular analysis of the SCN1A gene by multiplex ligation‐dependent probe amplification (MLPA), high‐resolution melting curve analysis (HRMCA), and sequencing revealed a frameshift duplication of adenosine at position 504. The incidence of this mutation is discussed as a potential cause of SUDEP.
Novel mutations in EPM2A and NHLRC1 widen the spectrum of Lafora disease
Authors: Lesca Gaetan , Boutry‐Kryza Nadia , De Toffol Bertrand , Milh Mathieu , Steschenko Dominique , Lemesle‐Martin Martine , Maillard Louis , Foletti Giovanni , Rudolf Gabrielle , Nielsen Jørgen Erik , á Rogvi‐Hansen Bjarke , Erdal Jesper , Mancini Josette , Thauvin‐Robinet Christel , M’Rrabet Amel , Ville Dorothée , Szepetowski Pierre , Raffo Emmanuel , Hirsch Edouard , Ryvlin Philippe , Calender Alain , Genton Pierre 
Published: August 2010

SummaryPurpose:  Lafora disease (LD) is an autosomal recessive form of progressive myoclonus epilepsy with onset in childhood or adolescence and with fatal outcome caused by mutations in two genes: EPM2A and NHLRC1. The aim of this study was to characterize the mutation spectrum in a cohort of unrelated patients with presumed LD.Methods:  Sequencing of the two genes and search for large rearrangements was performed in 46 unrelated patients with suspected LD, 33 originating from France and the others from different countries. Patients were classified into two groups according to the clinical presentation.Results:  Mutations of various types were found in EPM2A in 10 patients and in NHLRC1 in 4 patients. Mutations were found in 14 (93%) of 15 patients with classical clinical and electroencephalography (EEG) presentation of LD and in no patients with an atypical presentation. Ten mutations were novel, including the first substitution reported in a donor splice site of EPM2A, leading to the deletion of exon 2 at the RNA level. Four large deletions, including two deletions of exon 2 with different sizes and breakpoints, were found in EPM2A, corresponding to 20% of the alleles of this gene.Discussion:  We described several novel mutations of EPM2A and NHLRC1 and brought additional data to the genetic epidemiology of LD. This study emphasized the high mutation rate in patients with classical LD as well as the high negativity rate of skin biopsy.
Multicenter performance evaluation of a transcription‐mediated amplification assay for screening of human immunodeficiency virus‐1 RNA, hepatitis C virus RNA, and hepatitis B virus DNA in blood donations
Authors: Koppelman Marco H.G.M. , Assal Azzedine , Chudy Michael , Torres Pilar , De Villaescusa R. Garcia , Reesink Henk W. , Lelie P. Nico , Cuypers H. Theo M. 
Published: August 2010

BACKGROUND: The performance of the recently launched Procleix Ultrio (Chiron/Gen‐Probe) human immunodeficiency virus‐1 (HIV‐1), hepatitis C virus (HCV), and hepatitis B virus (HBV) blood screening assay was evaluated in a European multicenter study.STUDY DESIGN AND METHODS: Serial dilutions of reference materials were tested to determine the detection limits. Robustness and specificity were assessed by testing alternating high‐load HCV RNA–positive and –negative samples, and 2912 test pools of eight donations. The added value of minipool and single‐donation HBV nucleic acid testing protocols was compared to the currently used Prism (Abbott GmbH & Co. KG) hepatitis B surface antigen (HBsAg) and Auszyme (Abbott GmbH & Co. KG) dynamic HBsAg tests in 15 HBV seroconversion panels.RESULTS: The 95 percent detection limits (and 95% confidence interval [CI]) on the WHO International Standards was 26 (16‐58) IU per mL for HIV‐1 RNA, 4.6 (3.7‐6.5) IU per mL for HCV RNA, and 11 (7.3‐22) IU per mL for HBV DNA. No cross‐contamination was observed. Testing 2912 pools of eight donations revealed 16 initial reactive samples; 11 were confirmed. The specificity after initial testing and percentage of invalid results were 99.83 and 0.48 percent, respectively. The HBV window‐period (WP) reductions relative to HBsAg seroconversion in Prism and Auszyme dynamic HBsAg were, respectively, 6 days (95% CI, 3‐8) and 9 days (95% CI, 7‐12) in 1:8 minipool (MP) testing.CONCLUSION: The performance characteristics of Procleix Ultrio assay and the Procleix HIV‐1 and HCV assay are comparable. The sensitivity for HIV‐1 and HCV met the directives of the Paul‐Ehrlich Institute and the FDA. The assay can reduce the WP for HBV by 6 days to 2 weeks when used in small MP (<1:8) or single‐donation screening protocols.
Efficacy of apheresis platelets treated with riboflavin and ultraviolet light for pathogen reduction
Authors: AuBuchon James P. , Herschel Louise , Roger Jill , Taylor Harry , Whitley Pamela , Li Junzhi , Edrich Rick , Goodrich Raymond P. 
Published: August 2010

BACKGROUND:Pathogen reduction technologies for platelet (PLT) components offer a means to address continued viral transmission risks and imperfect bacterial detection systems. The efficacy of apheresis PLTs treated with riboflavin (vitamin B2) plus ultraviolet (UV) light (Mirasol, Navigant Biotechnologies) was investigated in a single‐blind, crossover study in comparison to untreated PLTs.STUDY DESIGN AND METHODS:Normal subjects (n = 24) donated PLTs by apheresis on two occasions at least 2 weeks apart. Units were randomized to control or test arms, the latter receiving the addition of 28 mL of 500 µmol per L B2 and exposure to 6.2 J per mL UV light. PLTs were stored for 5 days with biochemical and hematologic analyses performed before and after illumination on Day 0 and at the end of storage. An aliquot of each unit was radiolabeled and returned to determine recovery and survival.RESULTS:The PLT content of treated units was maintained from Day 0 (4.1 × 1011 ± 0.4 × 1011) to Day 5 (4.0 × 1011 ± 0.4 × 1011). Treatment with B2 plus UV light was associated with an increase in lactate production with concomitant increases in glucose consumption. pH (control, 7.38 ± 0.07; test, 7.02 ± 0.10) was well maintained throughout storage. Recovery of treated PLTs (50.0 ± 18.9%) was reduced from that of control PLTs (66.5 ± 13.4%); survival was similarly shortened (104 ± 26 hr vs. 142 ± 26 h; p < 0.001).CONCLUSIONS:PLTs treated with B2 plus UV light demonstrate some alterations in in vitro measures but retain in vitro and in vivo capabilities similar to pathogen‐reduced and licensed PLT components that have been shown to have useful clinical applicability. The recovery, survival, and metabolic properties of Mirasol PLTs should provide sufficient hemostatic support in thrombocytopenia to justify patient clinical trials.
Tranexamic acid reduces allogeneic red cell transfusions in patients undergoing total knee arthroplasty: results of a meta‐analysis of randomized controlled trials
Authors: Cid Joan , Lozano Miguel 
Published: August 2010

BACKGROUND: The delayed bleeding associated with total knee arthroplasty (TKA) may be a result of a tourniquet‐induced imbalance of the procoagulant and fibrinolytic systems. There are conflicting results in the literature about tranexamic acid (TA) infusion in reducing postoperative blood loss and the number of transfused red cells (RBC) units. A meta‐analysis was performed to summarize the results of different research studies.STUDY DESIGN AND METHODS: Randomized controlled trials reported through October 2004 were retrieved, and nine studies met the criteria for meta‐analysis. Summary odds ratio (OR) of allogeneic transfusion and a weighted mean difference (WMD) of transfusions per patient in TA‐treated group versus placebo group were calculated across studies.RESULTS: The use of TA significantly reduced the proportion of patients requiring blood transfusion (summary OR, 0.10; 95% CI, 0.06‐0.18; p < 0.00001) and the number of RBC transfusions per patient (WMD, −1.72; 95% CI, −2.11 to −1.33; p < 0.00001) when compared with patients who received placebo.CONCLUSION: Our meta‐analysis shows that the use of TA for patients undergoing TKA is effective in reducing the requirements of allogeneic blood transfusion.
Sequestration of anti‐Cra in the placenta: serologic demonstration by placental elution
Authors: Weber Shanasey L. , Bryant Barbara J. , Indrikovs Alexander J. 
Published: August 2010

BACKGROUND: Few cases of antibodies to the Cromer (a) antigen have been described during pregnancy. Interestingly, the anti‐Cra titers decreased during pregnancy, and although the newborns were Cra+, the direct antiglobulin tests (DATs) were negative and hemolytic disease of the newborn (HDN) was not observed. Cromer antigens reside on the decay‐accelerating factor (DAF), which is expressed on the fetal derived syncytiotrophoblast layer of the placenta. It has been postulated that Cromer antibodies are not transported to the fetus, but are bound to placental DAF, thereby protecting the fetus from HDN and causing the disappearance of Cromer antibody in maternal plasma. This report is the first to demonstrate Cromer antibody sequestration by the placenta.CASE REPORT: A G4P1 woman with an anti‐Cra presented for prenatal care during her fourth pregnancy. The anti‐Cra titer decreased from 64 at 7 weeks gestation to undetectable after 25 weeks. At delivery, the infant had no evidence of HDN. The infant's DAT was negative, and the maternal plasma, cord plasma, and the cord blood eluate were negative with screening cells, the infant's cord cells, and the mother's cells. Placental eluates revealed anti‐Cra.CONCLUSIONS: This is the fourth case report of a Cromer (a–) woman producing anti‐Cra during pregnancy, and the first demonstrating anti‐Cra sequestration in the placenta. The presence of anti‐Cra in the placental eluate, but not in the cord plasma, maternal plasma, or cord blood eluate, strongly supports the hypothesis that DAF at the fetomaternal interface absorbs anti‐Cra from the maternal circulation blocking its passage to the fetus.
The influence of blood group differences in allogeneic hematopoietic peripheral blood progenitor cell transplantation
Authors: Erker Christian G. , Steins Martin B. , Fischer Rudolf‐Josef , Kienast Joachim , Berdel Wolfgang E. , Sibrowski Walter , Cassens Uwe 
Published: August 2010

BACKGROUND: Severe immunohematologic complications after ABO‐mismatched allogeneic blood peripheral blood progenitor cell (PBPC) transplantation (PBPCT), including pure red cell aplasia and immune hemolysis, have been described. Although several studies have addressed this issue, the clinical influence of blood group differences on transfusion requirements and survival is still discussed controversially, especially in the case of PBPCT.STUDY DESIGN AND METHODS: This single‐center study is based on 143 patients receiving PBPCT after standard or reduced‐intensity conditioning. The influence of blood group differences in the ABO, Rh, and Kell systems on red blood cell, platelet, and plasma transfusion requirements; length of hospitalization in transplantation unit; survival; and occurrence of graft‐versus‐host disease was investigated. Additionally, the influence of the conditioning regimen and irregular antibodies on the measures mentioned above was analyzed.RESULTS: Multivariate analysis demonstrated that minor and bidirectional ABO mismatch (p = 0.028) and Rh difference (p = 0.020) independently led to poorer survival. The Kell difference did not show significant influences on the measures mentioned above. A clinically relevant influence of blood group differences on transfusion requirements could not be demonstrated. Irregular antibodies also did not show significant influences.CONCLUSION: These findings indicate an influence of blood group differences in PBPCT on survival and must be studied in further detail.
Effect of cessation of tobacco use on the incidence of oral mucosal lesions in a 10–yr follow‐up study of 12 212 users
Authors: Gupta PC , Murti PR , Bhonsle RB , Mehta FS , Pindborg JJ 
Published: August 2010

OBJECTIVE: To study the effect of cessation of tobacco use on the incidence of lichen planus, leukoplakia and other oral mucosal lesions.DESIGN: A 10–yr cohort study in a rural population of Ernakulam district, Kerala, India.MATERIAL AND METHODS: Some 12 212 tobacco users were interviewed and examined in a basetine survey and re‐examined annually for 10 years. At each examination they were exposed to health educational programs to encourage them to quit their tobacco use. The incidence rates were calculated using person‐years method among those who stopped their tobacco use and all others.RESULTS: A total of 77 681 person‐years of observation accrued among men and 32 544 among women. Among men 6.5% of these and among women 14.4% were in the stopped category. The incidence of oral lichen planus did not show any consistent association with cessation of tobacco habits (incidence ratio I.35) but for leukoplakia there was a substantial drop in the incidence after cessation (incidence ratio 0.3I). Several other tobacco‐associated oral mucosal lesions such as oral lichen planus‐like lesion, smoker's palate, preteukoplakia, central papillary atrophy of the tongue and leukoedema showed either zero, or very small incidence, after cessation. CONCLUSION: The reported association between tobacco use and lichen planus appears to be indirect but for all other lesions it is direct. The cessation of tobacco use led to a substantial fall in the incidence of leukoplakia and other lesions implying a reduced risk for oral cancer after cessation of tobacco use.
Cytokine profiles of Porphyromonas gingivalis‐reactive T lymphocyte lines and clones derived from P. gingivalis‐infected subjects
Authors: Gemmell E , Kjeldsen M , Yamazaki K , Nakajima T , Aldred MJ , Seymour GJ 
Published: August 2010

Porphyromonos gingivolis is generally recognized as a major periodontopathogen such that a study of T cell responses to this organism may help to elucidate immune regulation in periodontal disease.OBJECTIVE The aim of this study was to examine interleukin (IL)‐4, interferon (IFN)‐gamma and IL‐10 production by p‐gingivolis‐responsive T cell lines and clones derived from the peripheral blood of two P. gingivolis‐infected subjects with different disease expression and from the gingival tissues of one of the P. gingivolis‐infected subjects.MATERIALS AND METHODS: FACS analysis was used to determine the percentage of T cells staining positive for cytoplasmic IL‐4, IFN‐gamma and IL‐10 and reverse transcriptase polymerase chain reaction (RT‐PCR) was performed to determine the presence of mRNA for IL‐4 and IFN‐gamma in the T cell lines and clones.RESULTS FACS analysis showed that virtually all the T cell lines and clones contained IL‐4– and IFN‐gamma‐pro‐ducing T cells. The RT‐PCR results generally supported this trend. However, a higher percentage of cells in the clones derived from one subject produced IL‐4 while a lower percentage produced IFN‐gamma compared with the clones derived from the other subject. FACS analysis also demonstrated that the lines and clones derived from the two subjects showed differences in IL‐10 production. CONCLUSION: This study has demonstrated that there may be differences in IL‐4 and 11–10 production by the P. gingivolis responsive lines and clones derived from P. gingivalis‐infected subjects with different disease expression. Any relationship to disease however, remains to be determined.