bluuebunny/crossref_metadata_2025 huggingface dataset:
Association of furunculosis and familial deficiency of mannose‐binding lectin
Authors: Kars M. , Van Dijk H. , Salimans M. M. , Bartelink A. K. , Van De Wiel A.
Published: August 2005

Background  Polymorphisms in the mannose‐binding lectin gene reduce serum mannose‐binding lectin levels and are associated with enhanced risk of infection. In a family with recurrent staphylococcal disease presenting as furunculosis or carbuncles, an association with mannose‐binding lectin deficiency was investigated.

Materials and methods  Levels of functional mannose‐binding lectin were estimated and the genotypes of the mannose‐binding lectin gene were analysed on blood samples, collected from the members of one particular family with a high prevalence of furunculosis.

Results  Functional mannose‐binding lectin levels in sera of 13 of the 28 members of one family showed deficiency. Furunculosis or carbuncles appeared to be present in nine of the 28 family members, seven of which showing the pBly allele and mannose‐binding lectin deficiency. Four young family members of the second generation were pBly positive and mannose‐binding lectin deficient, but had not shown furunculosis yet.

Conclusion  Members of a particular family suffering from furunculosis differ from their ‘healthy’ relatives as to mannose‐binding lectin genotypes, indicating the relevance of normal mannose‐binding lectin levels in the defence against staphylococcal disease.
Is the intensity of the highest fat oxidation at the lactate concentration of 2 mmol L−1? A comparison of two different exercise protocols
Authors: Bircher S. , Knechtle B. , Knecht H.
Published: August 2005

Background  The exercise intensity eliciting highest fat oxidation is important for a variety of populations and its precise determination requires an adequate exercise protocol. The aim of this study was to compare fat oxidation, concentration of lactate and lactate threshold during an established exercise protocol using fixed workloads with a protocol based upon the subject's individual heart rate response to exercise.

Materials and methods  Highest fat oxidation, concentration of lactate and lactate threshold were compared between two different exercise protocols in moderately trained men (n = 48) and women (n = 30). In randomized order subjects completed a standardized (STAND) and an individual (IND) submaximal exercise test. The increments during IND were adapted by the subjects’ individual heart rate response to exercise compared to STAND with defined steps.

Results  In men, fat oxidation was significantly higher at the intensity eliciting highest fat oxidation in STAND than in IND (P = 0·019), but not in women. In both genders lactate concentration (P < 0·001) and heart rate (HR) (P < 0·001) were significantly higher in IND compared to STAND at this intensity. A significant correlation between O2 at lactate threshold and the intensity eliciting the highest fat oxidation was found in both genders in IND (women r = 0·73; men r = 0·43) and in STAND (women r = 0·57; men r = 0·56).

Conclusion  Different exercise increments and stage durations have an influence on lactate concentration and HR at the intensity eliciting the highest fat oxidation. The shorter test duration of STAND favours this protocol to determine maximal fat oxidation. For the untrained, start of exercise should be at very low intensity.
Statin‐induced blockade of prenylation alters nucleocytoplasmic shuttling of GTP‐binding proteins γ2 and β2 and enhances their suppressive effect on glucocorticoid receptor transcriptional activity
Authors: Kino T. , Kozasa T. , Chrousos G. P.
Published: August 2005

Background  We previously reported that the guanine tri‐phosphate‐binding proteins (G) β and γ are both localized in the nucleus, in addition to their expected cytoplasmic/plasma membrane localization. These proteins, as a heterodimeric complex, suppress glucocorticoid response element‐mediated transcriptional activity of the glucocorticoid receptor through direct physical interactions between Gβ and the glucocorticoid receptor.

Materials and methods  As Gγ is prenylated at a cysteine residue in its C‐terminal portion, and as this post‐translational modification is required for many of the known Gβ/Gγ activities, we examined the effect of its absence or diminution on Gβ/Gγ‐induced suppression of glucocorticoid receptor‐induced transcriptional activity.

Results  In a functional reporter assay, Gγ2C68S, which is defective at the prenylation site, was more potent than the wild‐type Gγ2 at increasing Gβ2‐induced suppression of glucocorticoid receptor transactivation. Interestingly, the enhanced green fluorescent protein fusion of this mutant Gγ2 was localized preferentially in the nucleus, while it was absent from the plasma membrane. Lovastatin, a 3‐hydroxy‐3‐methylglutaryl coenzyme A (HMG‐CoA) reductase inhibitor that abrogates the prenylation of Gγ, shifted the subcellular localization of enhanced green fluorescence protein‐fused Gγ2 and Gβ2 from the cytoplasm/plasma membrane to the nucleus and further suppressed glucocorticoid receptor‐induced transcriptional activity.

Conclusions  These findings indicate that not only is the natural covalent addition of the prenyl residue to Gγ unnecessary for the transcriptional suppression induced by Gβ/Gγ on the glucocorticoid receptor, but rather helps retain the Gβ/Gγ complex away from the nucleus decreasing its antiglucocorticoid actions.
Differential expression of P2X‐purinoceptor subtypes in circular and longitudinal muscle of canine colon
Authors: Lee H. K . , Ro S . , Keef K. D . , Kim Y.‐h . , Kim H. W . , Horowitz B . , Sanders K. M .
Published: August 2005

Abstract  Adenosine triphosphate (ATP) mediates excitatory junction potentials through P2X receptors in many smooth muscles. However, relatively little is known about postjunctional intestinal P2X receptors. We examined the effect of exogenous ATP on circular and longitudinal myocytes of canine colon using the patch clamp technique at 32 °C. In both cell types, ATP induced inward currents (IATP) at −70 mV in a concentration‐dependent manner. The potency profile of ATP analogues in circular myocytes was: ATP ≈ 2‐methylthio‐ATP > α,β‐methylene ATP, and that in longitudinal myocytes was: α,β‐methylene ATP ≈ ATP > 2‐methylthio‐ATP. Pretreatment of circular myocytes with α,β‐methylene ATP inhibited the response to subsequent ATP, suggesting receptor desensitization. I–V relationships of IATP were linear with inward rectification and Erev of −13 mV. IATP at −70 mV was carried predominantly by Na+ as determined by shifts in Erev when extracellular Na+ was lowered. In RT‐PCR, circular myocytes expressed mRNAs encoding P2X2, 3 and 4, while longitudinal myocytes expressed mRNAs for P2X3 and 5. P2X7 was absent in both cells. Fragments of each subtype were cloned and sequenced. We failed to clone P2X1 and P2X6 genes. Overall, different P2X receptor subtypes are expressed in circular and longitudinal canine colonic myocytes. Their activation produces non‐selective cation currents that can depolarize and excite muscles of both layers.
Benign cutaneous Degos disease
Authors: Zamiri Mozheh , Jarrett Paul , Snow John
Published: August 2005

A 24‐year‐old woman presented with an 8‐year history of a recurrent asymptomatic rash characterized by small erythematous papules which evolved to form atrophic porcelain white scars with a telangectatic rim. She had never had gastrointestinal or neurological symptoms. A short trial of aspirin did not alter the behavior of the disease. Histology confirmed the clinical diagnosis of Degos disease. Degos disease is a rare disorder that has been classified into the benign or malignant variety. The malignant type has a poor prognosis. Gastrointestinal involvement is the most frequent cause of death. The existence of patients with a prolonged, purely cutaneous or benign form has been increasingly recognized. It may be impossible to classify a patient at the time of initial presentation. Her progress is consistent with the benign form.
Patient‐centred improvements in health‐care built environments: perspectives and design indicators
Authors: Douglas Calbert H. , Douglas Mary R.
Published: August 2005

Objective  To explore patients’ perceptions of health‐care built environments, to assess how they perceived health‐care built facilities and designs. To develop a set of patient‐centred indicators by which to appraise future health‐care designs.

Design  Qualitative and quantitative methodologies, including futures group conferencing, autophotographic study, novice‐expert exchanges and a questionnaire survey of a representative sample of past patients.

Setting and participants  The research was carried out at Salford Royal Hospitals NHS Trust (SRHT), Greater Manchester, UK, selected for the study because of planned comprehensive redevelopment based on the new NHS vision for hospital care and service delivery for the 21st century. Participants included 35 patients who took part in an autophotographic study, eight focus groups engaged in futures conferencing, a sample of past inpatients from the previous 12 months that returned 785 completed postal questionnaires.

Results  The futures group provided suggestions for radical improvements which were categorized into transport issues; accessibility and mobility; ground and landscape designs; social and public spaces; homeliness and assurance; cultural diversity; safety and security; personal space and access to outside. Patients’ autophotographic study centred on: the quality of the ward design, human interactions, the state and quality of personal space, and facilities for recreation and leisure. The novices’ suggestions were organized into categories of elemental factors representing patient‐friendly designs. Experts from the architectural and surveying professions and staff at SRHT in turn considered these categories and respective subsets of factors. They agreed with the novices in terms of the headings but differed in prioritizing the elemental factors. The questionnaire survey of past patients provided opinions about ward designs that varied according to where they stayed, single room, bay ward or long open ward. The main concerns were limitation of private space around the bed area, supportive of privacy and dignity, ward noise and other disturbances.

Conclusions  Patients perceived sustainable health‐care environments to be supportive of their health and recovery. The design indicators developed from their perspectives and from their considerations for improvements to the health‐care built environment were based on their visions of the role of the health‐care facilities. These were homely environments that supported normal lifestyle and family functioning and designs that were supportive of accessibility and travel movements through transitional spaces.
House‐dust mites and summer cottages
Authors: Korsgaard J. , Harving H.
Published: August 2005

Background:  Estimated indoor climate conditions in unheated summer cottages offers house‐dust mites unfavorable temperature and feeding conditions while giving them optimum humidity conditions. We aimed to estimate the exposure level of house‐dust mites in summer cottages.

Methods:  A total of 37 summer cottages were sampled for house dust and storage mites in three locations and the results compared with samples from 33 patients suspected of house‐dust mite allergy and living in ordinary houses. The processed dust samples were investigated by microscopy and exposure levels given as number of mites per 0.1 g dust.

Results:  The summer cottages were without exception all heavily infested with threefold or higher concentrations of both house dust and storage mites in comparison with ordinary houses. In unheated summer cottages were found tropical high concentrations of house dust and storage mites with a median concentration of house‐dust mites in mattress dust of 2000 house‐dust mites/g of dust which corresponds to an average of 40 μg allergen/g of mattress dust.

Conclusions:  As a probable consequence of high indoor air humidity conditions in unheated summer cottages in winter, dust samples contained threefold or higher concentrations of house dust and storage mites. Indoor humidity conditions thus more than compensated for lack of continuous supply of skin scales from human beings and an unfavorable low indoor air temperature. The uniform high mite exposure in summer cottages is likely to imply disease deterioration in patients already allergic to mites.
Hypersensitivity reactions to paracetamol in children: a study of 25 cases
Authors: Boussetta K. , Ponvert C. , Karila C. , Bourgeois M. Le , de Blic J. , Scheinmann P.
Published: August 2005

Background:  Allergic‐like reactions to paracetamol (acetaminophen) are rare. Paracetamol allergic and nonallergic hypersensitivity (HS) has been diagnosed in a few patients with skin and/or respiratory symptoms, immediate and accelerated urticaria, and angioedema especially. Most patients with HS to paracetamol were also hypersensitive to anti‐inflammatory drugs (i.e. acetylsalicylic acid, ASA), suggesting that their reactions resulted from a nonallergic HS. However, anaphylactic reactions, and potentially harmful toxidermias, such as acute generalized exanthematic pustulosis and toxic epidermal necrolysis, have been related to specific paracetamol allergic HS, with tolerance to anti‐inflammatory drugs.

Patients and methods:  We report the results of a study performed in 25 children with suspected paracetamol HS. Diagnosis of paracetamol HS was based on a suggestive clinical history and a positive response in an oral challenge (OC) test.

Results:  Paracetamol HS was diagnosed in only one child (4%). In this child, a positive response to an OC with ASA diagnosed HS to anti‐inflammatory drugs.

Conclusions:  Our results in children agree with those of the literature, showing that paracetamol HS is rare, and is associated with HS to anti‐inflammatory drugs in most patients.
Influence of an asthma education program on parents with children suffering from asthma
Authors: Trollvik Anne , Severinsson Elisabeth
Published: August 2005

Abstract  Several studies have evaluated the effectiveness of various methods of asthma education for parents. This study adds a qualitative and inner perspective from the families of children suffering from asthma. The aim was to illuminate the influence of an asthma education program (AEP) for parents. The study sample consisted of nine parents of five children with moderate to severe asthma in the 2–6 years age group. Data were collected by interviews and qualitative analyses were used. The main findings were that the parents experienced an increased understanding of their child's experience of asthma after having participated in the program. Three themes were revealed: sharing experiences, accepting the illness, and partnership building. In conclusion, the parents in the study expressed that they promote increased self–understanding by meeting other parents in groups.
Adolescents’ perspectives on addiction: Qualitative study
Authors: Parvizy Soroor , Nikbahkt Alireza , Pournaghash Tehrani Said , Shahrokhi Sholeh
Published: August 2005

Abstract  Given that the adolescence period is one of the most crucial stages of development during one's lifetime and adolescents are prone to engage in behaviors which may negatively impact their health, it is very important to have a clear and transparent understanding of adolescents’ health needs and problems. A content analysis with a qualitative approach was conducted and 41 healthy adolescents were interviewed. The results of this study revealed the following themes: addiction causes and prevalence; unhealthy friendship and communication and increased rates of cigarette smoking; alcohol and drugs; barriers to and factors in addiction and health; family and addiction. The findings showed worrying attitudes of adolescents to addiction and its prevalence are alarming. It is concluded that exploring adolescents’ perspectives is not only important but is useful for maintaining a healthy society, and thus policy makers should develop effective prevention and intervention programs based on these themes.
Does off‐pump coronary artery bypass grafting beneficially affect renal function?
Authors: Ogawa Mitsugu , Yaku Hitoshi , Doi Kiyoshi , Okano Takahisa , Yamada Yoshiaki , Hayashida Kyoko , Fukumoto Atsushi
Published: August 2005

Background:  Off‐pump coronary artery bypass grafting (CABG) has been reported to beneficially affect renal function, but this remains to be confirmed. The purpose of the present paper was to study the effects of off‐pump CABG on renal function and analyse predictors of postoperative renal impairment in patients who received off‐pump CABG.

Methods:  A total of 451 patients who underwent isolated CABG between January 1999 and August 2003 were retrospectively studied. No patient was receiving dialysis. A total of 300 patients (228 men) underwent off‐pump CABG (off‐pump group) and 151 patients (104 men) underwent on‐pump CABG (on‐pump group). Perioperative serum creatinine levels and creatinine ratios (peak postoperative creatinine level/preoperative creatinine level) were compared between the groups.

Results:  Renal impairment (serum creatinine >1.5 mg/dL) developed postoperatively in 12.7% of the off‐pump group and 18.5% of the on‐pump group (P = 0.1). The creatinine ratio was significantly lower in the off‐pump group (1.2 ± 0.4) than in the on‐pump group (1.4 ± 0.7, P = 0.003). Logistic regression analysis demonstrated that the strongest predictors of postoperative renal impairment in off‐pump CABG were left ventricular dysfunction (odds ratio 10.8) and multivessel grafting (odds ratio 4.3).

Conclusions:  Off‐pump CABG provides better renal protection than on‐pump CABG. However, perioperative renal function should be closely monitored in patients who have left ventricular dysfunction or who undergo multivessel grafting, even when off‐pump CABG is performed.
Stimulation of the retina with a multielectrode extraocular visual prosthesis
Authors: Chowdhury Vivek , Morley John W. , Coroneo Minas T.
Published: August 2005

Background:  An extraocular approach to developing a retinal prosthesis for blind patients using electrodes placed on the outer surface of the eye is suggested. Experiments were carried out to determine the feasibility of this approach, and evaluate electrode configurations and parameters for stimulation.

Methods:  In anaesthetized cats, a 21‐electrode extraocular retinal prosthesis (ERP) array was sutured to the sclera over the lateral surface of the eye. Electrically evoked potentials (EEP) were recorded at the visual cortex bilaterally in response to retinal stimulation with the electrode array. Bipolar stimulation of the ERP array electrodes in horizontal and vertical configurations and at different interelectrode separations was investigated with biphasic constant‐current pulses.

Results:  Electrical stimulation of the lateral retina with an ERP elicited EEP that were higher in the ipsilateral visual cortex. The threshold for bipolar retinal stimulation was 500 µA. EEP amplitude increased with increases in stimulus pulse duration and current intensity. Retinal stimulation was slightly more effective with electrodes in a vertical as opposed to horizontal orientation. A larger interelectrode separation resulted in a higher EEP amplitude.

Conclusions:  Retinal stimulation with a prototype ERP array is demonstrated. The thresholds for retinal excitation are below safe charge‐density limits for chronic neural stimulation. Ipsilateral localization of the EEP suggests that localized retinal stimulation is occurring. An ERP is a new approach to retinal prosthesis research, and might lead to the development of a low‐resolution visual prosthesis for blind patients.
Surgical accountability in the 1880s: the death of Susan Nixon
Authors: Watters Greg R. , Walker David R.
Published: August 2005

Susan Nixon died in 1881 following a surgical error. Her surgeon, Dr W. E. Warren, excised a normal 7‐month gravid uterus under the misapprehension that he was removing a tubular pregnancy. It is believed that Mrs Nixon was the first woman in Australia to have an abdominal hysterectomy and the second to have a live Caesarean section. The surgical misadventure that resulted in Mrs Nixon's death became a public scandal, which gained currency through both parliamentary debate and the popular press.

The purpose of referring to this case is to explore the mechanisms of accountability that surgeons faced in the 1880s − a decade of rapid change in the practice of surgery. The response of late nineteenth century society to surgical error and the resultant reaction of the medical profession have resonances that are relevant to surgeons practising today.
Cooperation in collective action*
Authors: Bandiera Oriana , Barankay Iwan , Rasul Imran
Published: August 2005

The ability to cooperate in collective action problems – such as those relating to the use of common property resources or the provision of local public goods – is a key determinant of economic performance. In this paper we discuss two aspects of collective action problems in developing countries. First, which institutions discourage opportunistic behaviour and promote cooperation? Second, what are the characteristics of the individuals involved that determine the degree to which they cooperate? We first review the evidence from field studies, laboratory experiments, and cross community studies. We then present new results from an individual level panel dataset of rural workers.
A two‐center, double‐blinded, randomized trial testing the tolerability and efficacy of a novel therapeutic agent for cellulite reduction
Authors: Rao Jaggi , Gold Michael H , Goldman Mitchel P
Published: August 2005

Introduction  Cellulite is the unsightly dimpling and nodularity found on the thighs and buttocks of many postadolescent women. Unfortunately, poor understanding of its pathophysiology coupled with very few scientifically based studies have left us with limited treatment options that are tolerable and effective.

Purpose  To review current concepts of the etiology and nature of cellulite and summarize available treatment options. To evaluate a novel, pathophysiologically based, topical agent for treatment.

Materials and methods  A total of 40 women with a moderate degree of cellulite (20 from each of the two research centers) entered a double‐blinded, randomized trial where an anticellulite cream was applied on a nightly basis to the affected sites for four continuous weeks. Each subject was randomized to receive active cream on either the right or left leg, with the contralateral side serving as placebo control. Bioceramic‐coated neoprene shorts were worn overnight to enhance penetration of the topical agents by occlusion. High‐quality digital photography was taken before treatment and after 4 weeks, with tangential full‐spectrum lighting. Five blinded, independent physician reviewers assessed the photographs for improvement. Subject questionnaires were completed to assess tolerability and efficacy.

Results  Of the 34 subjects who completed the study, 62% (21/34) noticed an overall improvement in their cellulite, with 62% (13/21) reporting greater improvement in the thigh that received active product. All 34 subjects found the shorts and creams easy and pleasant to use. Overall, the average measured decrease in thigh circumference was 1.9 cm (range: 0.1–4.5) with active product, and 1.3 cm (range: 0.1–3.0) with placebo. Upon review of the pre‐ and poststudy photographs, dermatologist evaluators found thighs treated with active product showed greater improvement than thighs treated with placebo in 68% of subjects.

Conclusions  The active topical agent used in this study was found to be effective in reducing the appearance of cellulite. All subjects tolerated the formulation well with no adverse effects. The success of this research validates the pathophysiologic concepts used to formulate the topical compound. This study both increases our understanding of the nature of cellulite and establishes a tolerable, effective product to treat it.
Artificial nails and psoriasis
Authors: André Josette
Published: August 2005

When dealing with artificial nails, it is often mentioned that psoriasis is a contraindication because artificial nails may worsen nail psoriasis by Köbner phenomenon.1 However, although not thoroughly discussed, other interactions between psoriasis and artificial nails exist. On the basis of clinical cases, the following interactions are described: worsening of psoriasis, the fact that psoriatic patients wearing artificial nails are more exposed to infections, and allergic risks. The clinical similarities that may exist between nail psoriasis and nail alterations caused by allergic reactions to artificial nails are also emphasized.
Microdermabrasion followed by a 5% retinoid acid chemical peel vs. a 5% retinoid acid chemical peel for the treatment of photoaging – a pilot study
Authors: Hexsel Doris , Mazzuco Rosemari , Dal’Forno Taciana , Zechmeister Debora
Published: August 2005

Background  Both retinoid acid (RA) chemical peeling and microdermabrasion are minimally invasive procedures recently developed for the treatment of photoaging. The efficacy of each procedure has been proved, but the results of the combination of these two methods have not been studied until now.

Objectives  To assess and compare the efficacy of microdermabrasion followed by a 5% RA chemical peel with that of a 5% RA chemical peel alone.

Methods  Six female patients with moderate degrees of photodamage were evaluated. Three submitted to microdermabrasion followed by the application of a 5% RA chemical peel and three submitted to a 5% RA chemical peel alone. Each patient underwent three treatment sessions, with intervals of 7–10 days. Photographs were taken, and both biopsies and histological assessments were carried out, before treatment and 7 days after the final treatment session.

Results  In the opinion of the patients, in both groups there was improvement in the texture, pigmentation, and appearance of the treated skin, and this was confirmed with the assessment of the pre‐ and post‐treatment photographs. The combination of microdermabrasion followed by a 5% RA peel showed slightly greater improvement in the histological alterations resulting from photoaging.

Conclusion  Both methods, microdermabrasion followed by a 5% RA chemical peel and a 5% RA chemical peel alone are effective in the treatment of photoaging.
Topical retinoids in the treatment of photoaging
Authors: Stefanaki Christina , Stratigos Alexander , Katsambas Andreas
Published: August 2005

A large number of different substances comprise the family of retinoids, which are traditionally described as vitamin A derivatives. By exerting their action through nuclear and cytoplasmic receptors they may improve photoaging. Tretinoin is the best studied retinoid in the treatment of photoaging. Others such as isotretinoin, retinaldehyde, and tazarotene, although less well studied, have given promising results.
Self‐Help Epilepsy Groups: An Evaluation of Effect on Depression and Schizophrenia
Authors: Becu Maximo , Becu Nora , Manzur Graciela , Kochen Silvia
Published: August 2005

The general prevalence of depression and schizophrenia was determined in a random outpatient population (n = 67) of refractory epileptic patients by Minnesota Multiphasic Personality Inventory (MMPI) and Beck Depression Inventory (BDI). Of those patients 25% showed scores of moderate or severe depression. Self‐help group intervention modifies significantly (p < 0.001) general mean depression rates, especially dystimia. Male and female differences emerged in our study for previous prevalence and pattern of recovery.
New Topographic Mapping of Temporal Lobe Seizures
Authors: Ives J. R. , Ichihashi K. , Gruber L. J. , Mainwaring N. R. , Blume H. W. , Schomer D. L.
Published: August 2005

A unique topographic map has been developed based on EEG data of ictal events originating from the basal/mesiotemporal lobe regions. This technique involves a new mapping method of temporal lobe seizures as opposed to the interictal activity maps of most commercially available software. The map integrates data from sphenoidal electrodes as well as the standard 10–20 surface electrodes recorded with bipolar montages. A basal view is ideal for visualization of onset of temporal lobe ictal discharges recorded with chronic sphenoidal electrodes. We used the last 150 ictal events from 40 patients with basal/mediotemporal lobe epilepsy to develop this technique. Results indicate that a topographic view incorporating sphenoidal and scalp electrodes may provide a useful adjunct for interpretation of EEG recordings and a basis for comparison between and among patient groups for both ictal and interictal epileptic discharges.
Familial Progressive Myoclonus Epilepsy: Clinical and Electrophysiologic Observations
Authors: Acharya J. N. , Satishchandra P. , Shankar S. K.
Published: August 2005

Summary: Progressive myoclonus epilepsy (PME) is a syndrome complex encompassing different diagnostic entities. Among the 30 cases of PME studied during 1982 and 1992 at the National Institute of Mental Health and Neurosciences, Bangalore, South India, the specific diagnoses included Lafora disease (LD), neuronal ceroid lipofuscinosis (NCL), Unverricht‐Lundborg disease (ULD), and myoclonus epilepsy and ragged‐red fibres (MERRF).We discuss the familial nature of PME and the clinical and electrophysiological abnormalities in asymptomatic siblings. Eight cases of LD were in three different families with 3 affected siblings in two families (L1, L2) and 2 siblings in the third family (L3). Occipital seizures and behavioral changes occurred in all 3 members of L1 but were absent in the other two families. Age of onset was similar in two families (L1, 11 years; L2, 14.5 years), but not in the third. Presymptomatic EEG abnormalities were observed as long as 6 years before onset in L2. ULD occurred in 2 sisters in one family. Both had identical clinical features and normal somatosensory evoked potentials (SSEPs). The asymptomatic sister of the patient MERRF had abnormal EEG and giant SSEPs for the past 2 years. Thus, although all variations are evident in the overall clinical pattern in each of the PME, affected member of individuals families tend to be similar. Once an index case is identified, electrophysiologic tests (EEG and SSEP) may be useful in identifying other affected siblings in the presymptomatic stage. Key Words: Progressive myoclonus epilepsy–Genetics–Electrophysiology–Presymptomatic.
Monotherapy or Polytherapy for Epilepsy Revisited: A Quantitative Assessment
Authors: Lammers M. W. , Hekster Y. A. , Keyser A. , Meinardi H. , Renier W. O. , van Lier H.
Published: August 2005

Summary: Some investigators argue that treating epilepsy with several antiepileptic drugs (AEDs) simultaneously (polytherapy) may give rise to more adverse effects than monotherapy, but this argument lacks supporting quantitative data. To reexamine this issue, we recruited a cohort of patients from the outpatients of the Special Centres for Epilepsy in The Netherlands and from the outpatients of the Department of Neurology, Nijmegen University, The Netherlands. Two tools were used for analysis. All daily doses of antiepileptic drugs (AEDs) were standardized by the ratio of prescribed daily dose to defined daily dose (PDD/DDD). The DDD is the assumed average effective daily dose for a drug used for its main indication in adults. The assignment of DDD Values is the task of the World Health Organization (WHO) Collaborating Centre for Drugs Statistics Methodology and Nordic Council on Medicines, which regularly publishes Guidelines for Defined Daily Doses. The severity of adverse effects (AE) was assessed by using the Neurotoxicity Index and the Systemic Toxicity Index as developed by the VA Cooperative Study Group for their recent studies comparing the efficacy and tolerability of AEDs. One hundred sixty‐one patients received monotherapy; all had a PDD/DDD ratio ≤2/day; 128 of 262 patients receiving polytherapy also had ≤2 PDD/DDD ratios/day. The mono‐ and polytherapy groups were stratified according to the PDD/DDD ratio. The prevalence of neurological AE for patients with similar PDD/DDD ratios was 50–80% for monotherapy patients and 50–80% for polytherapy patients. The difference between the mono‐ and polytherapy groups was not significant. The prevalence of neurological AE for patients receiving polytherapy with a PDD/DDD ratio >2.0 was 71–100%, whereas all patients with a PDD/DDD ratio >4.0 had neurological AE. This difference between patients with a PDD/DDD ratio ≤2.0 and those with >2.0 was statistically significant; p < 0.05. The severity of neurological AE also increased with dose, but appeared to peak at ∼3.5 PDD/DDD ratio. Our study underscores the usefulness of applying quantitative methods to the analysis of drug AE. Comparison of monotherapy and polytherapy showed no difference for equipotent doses. Because distribution of the AED doses was uneven between the groups receiving mono‐ and polytherapy, our study permits only a tentative statement that the frequency and severity of AE is independent of the use of either. In addition, frequency and intensity of AE apparently are not very sensitive to changes in dose. An experimental prospective study is planned to verify or refute the conclusions of this observational pilot study.
Action of GP 47779, the Active Metabolite of Oxcarbazepine, on the Corticostriatal System. I. Modulation of Corticostriatal Synaptic Transmission
Authors: Calabresi Paolo , Murtas Marco De , Stefani Alessandro , Pisani Antonio , Sancesario Giuseppe , Mercuri Nicola B. , Bernardi Giorgio
Published: August 2005

Summary: Oxcarbazepine (OCBZ) is the keto‐analogue of carbamazepine (CBZ). In humans, OCBZ is rapidly and almost completely metabolized to 10, 1 l‐dihydro‐10‐hydroxy‐CBZ (GP 47779), the main metabolite responsible for the drug's antiepileptic activity. The corticostriatal pathway is involved in the propagation of epileptic discharges. We characterized the electrophysiological effects of GP 47779 on striatal neurons by making intracellular recordings from corticostriatal slices. GP 47779 (3–100 pM) produced a dose‐dependent inhibition of glutamatergic excitatory postsynaptic potentials (EPSPs). This effect was not coupled either with changes of the membrane potential of these cells or with alterations of their postsynaptic sensitivity to excitatory amino acids (EAA) suggesting a presynaptic site of action. GP 47779 reduced the current‐evoked firing discharge only at concentrations >100 μM. GP 47779 did not affect the presynaptic inhibitory action of adenosine, showing that presynaptic adenosine receptors were not implicated in the GP 47779‐mediated reduction of corticostriatal EPSPs. Our data indicate that GP 47779 apparently acts directly on corticostriatal terminals to reduce the release of EAA, probably by inhibiting high‐voltage‐activated (HVA) calcium (Ca2+) currents (described in the accompanying article). The inhibitory action of GP 47779 on corticostriatal transmission may contribute to the antiepileptic effects of this drug.
Benzodiazepine‐GABAA Receptors in Idiopathic Generalized Epilepsy Measured with [11C]Flumazenil and Positron Emission Tomography
Authors: Prevett Martin C. , Lammertsma Adriaan A. , Brooks David J. , Bartenstein Peter A. , Patsalos Philip N. , Fish David R. , Duncan John S.
Published: August 2005

Summary The neurochemical basis of absence seizures and the mechanism of their suppression by valproate (VPA) are uncertain. We used positron emission tomography (PET) to determine whether an abnormality of [11C]flumazenil binding to benzodiazepine (BZD)‐GABAA receptors exists in patients with childhood and juvenile absence epilepsy and to examine the effects of VPA on [11C] flumazenil binding. The regional cerebral volume of distribution (Vd) of [11C]flumazenil in patients not treated with VPA was not different from that in normal controls; Vd was lower in patients treated with VPA, and the number of receptors available for binding was significantly reduced in such patients as compared with normal controls. There was no evidence of a primary abnormality of the BZD‐GABA, receptor in childhood and juvenile absence epilepsy (CAE/JAE), but the data suggest that treatment with VPA is associated with a reduction in [11C]flumazenil binding that may be relevant to its mode of action in CAE/JAE.
Improved Sensitivity of 18FDG‐Positron Emission Tomography Scans in Frontal and “Frontal Plus” Epilepsy
Authors: Swartz B. E. , Khonsari A. , Brown C. , Mandelkern M. , Simpkins F. , Krisdakumtorn T.
Published: August 2005

Summary We evaluated three techniques of analyzing 18FDG‐positron emission tomography‐(PET) scans in 23 cases of presumed frontal lobe epilepsy (FLE): routine visual or “qualitative linear,”“qualitative normalized,” and quantitative normalized approaches. Patients were then classified as having pure frontal, probable frontal, frontoparietal and frontotemporal epilepsy based on prolonged surface EEG monitoring with video, magnetic resonance imaging (MRI), chronic intracranial recording (CIR), and results of surgical excision. Overall sensitivity and accuracy of the scans was 52 and 48% for qualitative linear analysis, which was equivalent to that of MRI, and 69 and 43% for qualitative normalized analysis. Quantitative normalized analysis had 96% sensitivity and 74–78% accuracy and also detected 9 of 11 (81%) abnormalities in nonlesional cases, improving routine sensitivity from 1 of 11 (9%). We conclude that qualitative linear (routine) analysis is inadequate for diagnosis of FLE lobe or “frontal‐plus” epilepsies and does not add to the MRI scan. Because qualitative normalized images improve on routine analysis only slightly, quantitative techniques should be applied for preoperative evaluations.
Recurrent Seizures Alter Renal Function and Plasma Atrial Natriuretic Peptide Levels in Rats
Authors: Perrone Barbara L. , Vari Richard C. , Kreisman Norman R.
Published: August 2005

Summary: Status epilepticus can lead to impaired renal function, which has been attributed to complications of myoglobinuria. We confirmed changes in renal function in the absence of myoglobinuria by measuring renal hemodynamics, fluid and electrolyte excretions, and plasma levels of renin and atrial natriuretic peptide (ANP) before and after a 30‐min period of recurrent generalized seizures in anesthetized, paralyzed rats. Renal plasma flow (RPF), renal blood flow (RBF) and glomerular filtration rate (GFR) decreased by ∼60% after seizures. In contrast, urinary sodium excretion, urine flow, and plasma ANP levels increased approximately threefold. Urinary potassium excretion and plasma renin levels were unchanged. Renal function is profoundly altered after 30 min of seizures, primarily due to intense renal vasoconstriction precipitating a dramatic reduction in GFR. The concomitant increases in sodium and urine excretion may be mediated by the marked increase in plasma ANP levels. The decreases in GFR and RBF might contribute to the renal failure observed in some patients after status epilepticus.
Neuroepidemiological Study of Childhood Epilepsy by Application of International Classification of Epilepsies and Epileptic Syndromes (ILAE, 1989)
Authors: Oka Eiji , Ishida Sumio , Ohtsuka Yoko , Ohtahara Shunsuke
Published: August 2005

Summary: A population‐based survey of childhood epilepsy was made in 1975 on the total population of children aged >10 years living in Okayama Prefecture (n = 2,378 patients). Using the data obtained, we attempted to re‐classify the various types of epilepsy according to the international classification (ILAE, 1989). Reclassification was possible in 1,872 (78.7%) of the 2,378 cases. The 1,872 cases consisted of 1,045 (55.8%) with localization‐related epilepsies, 824 (44.0%) with generalized epilepsies, and 3 (0.2%) with epilepsies undetermined whether focal or generalized. Classification of the epilepsies in a population‐based survey using the international classification involves difficulties, because both clinical and EEG findings are essential. However, if an appropriate area is selected, classification of epilepsies and epileptic syndromes in a population‐based survey is possible by referring to all medical records stored at every hospital and practitioner's clinic that administers treatment to patients with epilepsy in the area.
Electrocorticography and Temporal Lobe Epilepsy: Relationship to Quantitative MRI and Operative Outcome
Authors: Cascino Gregory D. , Trenerry Max R. , Jack Clifford R. , Dodick David , Sharbrough Frank W. , So Elson L. , Lagerlund Terrence D. , Shin Cheolsu , Marsh W. Richard
Published: August 2005

Summary: We investigated the relationship between electrocorticography (ECoG), quantitative magnetic resonance imaging (MRI), and surgical outcome in 165 patients with intractable nonlesional temporal lobe epilepsy (NLTLE). A standard mesial temporal resection was performed in all patients. Patients with an operative follow‐up <1 year were excluded from the study. The extent of the lateral temporal neocortex resection (LCR) was guided by ECoG and the side of surgery. The extent of the LCR was not predictive of seizure outcome in patients with or without hippocampal formation atrophy (p > 0.5). Patients undergoing a right anterior temporal lobectomy had a larger LCR (p < 0.000l), but the side of surgery was not of predictive value in determining seizure outcome (p > 0.1). The topography of the acute intracranial spikes did not correlate with operative outcome (p > 0.5) and was independent of hippocampal volumetric studies (p > 0.5). The postexcision ECoG was also shown not to be of prognostic importance (p > 0.5). Our results indicates that the extent of the lateral temporal cortical resection and the ECoG findings are not important determinants of surgical outcome in patients with NLTLE.
Intracarotid Amobarbital Procedure as a Predictor of Material‐Specific Memory Change After Anterior Temporal Lobectomy
Authors: Kneebone Anthony C. , Chelune Gordon J. , Naugle Richard I. , Dinner Dudley S. , Awad Issam A.
Published: August 2005

Summary: Memory testing during the intracarotid amobarbital procedure (IAP) is used extensively to identify temporal lobe surgery candidates “at risk” for developing severe postoperative anterograde amnesia. However, the utility of the IAP in predicting commonly observed material‐specific memory deficits has not been thoroughly investigated. We examined the utility of contralateral IAP memory testing, as an index of the functional capacity of the surgical temporal lobe, to predict postoperative material‐specific memory changes on the Wechsler Memory Scale‐Revised (WMS‐R) in patients with left hemisphere speech dominance undergoing left (n = 32) and right (n = 31) temporal lobectomy (TL). Left TL patients who “passed” contralateral IAP memory testing (368% recognition of memory items) had significantly greater verbal memory decrements than those who “failed” the IAP, presumably as a result of removal of functional tissue. A similar relationship between contralateral IAP performance and visual memory performance was not observed among right TL patients. Thus, the functional adequacy of the tissue to be resected appears to be inversely related to postoperative verbal memory decrement, at least among left TL patients. This relationship is consistent with results of recent studies demonstrating an inverse relationship between verbal memory decrements after left TL and preoperative neuropsychological verbal memory performance, magnetic resonance imaging (MRI) hippocampal volumes, and degree of mesiotemporal sclerosis (MTS).
Asymmetric and Asynchronous Infantile Spasms
Authors: Gaily Eija K. , Shewmon D. Alan , Chugani Harry T. , Curran John G.
Published: August 2005

Summary: Infantile spasms most commonly show symmetric behavioral and electroencephalogram (EEG) manifestations. Asymmetric and asynchronous behavioral spasms occur occasionally, but their relationship to ictal EEG and to other localizing studies has not received much attention. We reviewed 75 consecutive video‐EEG recordings, done at UCLA from 1982 to 1992, that contained infantile spasms; 8,680 spasms were scored for behavioral and EEG asymmetry and asynchrony. Of the recorded spasms, 25% were asymmetric and 7% were asynchronous. Most asymmetric or asynchronous spasms were associated with an ictal EEG discharge that was contralateral to the behaviorally more involved side. In 12 of the 60 patients (20%), more than half of the recorded spasms were asymmetric or asynchronous,. Baseline EEG, magnetic resonance imaging, positron emission tomography, and neurological examination revealed structural and functional brain abnormalities that involved the contralateral central region significantly more often in the children with >50% spasm asymmetry or asynchrony than in the other children. Partial seizures with lateralized motor behavior also occurred frequently in these children. The findings suggest that asymmetric and asynchronous spasms are generated by a cortical epileptogenic region that involves the primary sensorimotor area. The combination of asymmetric and asynchronous infantile spasms, partial motor seizures involving the same side of the body, and pathology in the contralateral central region may represent a unique subset of symptomatic localization‐related infantile epilepsy.
Intraoperative Thermal Inactivation of the Hippocampus in an Effort to Prevent Global Amnesia After Temporal Lobectomy
Authors: Lee Gregory P. , Smith Joseph R. , Loring David W. , Flanigin Herman F.
Published: August 2005

Summary: In an effort to assess risk of amnesia after antenor temporal lobectomy (ATL), we conducted localized thermal inactivation (cooling) of the hippocampus with memory testing. Thirty‐three ATL patients whose preoperative evaluation suggested risk for postoperative amnesia underwent hippocampal cooling. Cooling consisted of inserting a catheter in the temporal horn and irrigating it with an iced solution until a stable hippocampal temperature of ‐20°C was reached. Memory was assessed before and after cooling. In 12 of the 33 patients, memory testing was either aborted or suggested poor contralateral support, and the hippocampus was resected in 2 of these patients. The remaining 21 patients showed evidence of contralateral memory support, and the hippocampus was resected in 18. No patient became amnestic. These results suggest that intraoperative hippocampal cooling may be useful in selected cases. However, even among many patients who could cooperate with testing, discomfort, sedation, attentional deficits, confusion, and anxiety made test interpretation difficult.
Epilepsy in Literature*
Authors: Wolf Peter
Published: August 2005

Summary: Literary accounts of epilepsy deserve our attention because they form part of the cultural history of the disease and reflect responses to it by society. Epilepsy has been reviewed, mainly in the British literature, from Shakespeare to contemporary fiction, and reveals a wide range of images from the objective to the bizarre, based on observation, empathy, or manifold prejudices about the disorder. Epilepsy has been used in the plot of detective stories and as a challenge to movie actors. Some authors are factual and are well‐informed, whereas others emphasize a fascination with ancient beliefs relating seizures to prophecy and divinity. In contemporary literature physicians are often represented as only marginally involved, distant, and helpless. Epilepsy is rarely viewed as a treatable disorder.
The Outcome of Epilepsy and Its Measurement
Authors: Meinardi Harry
Published: August 2005

Summary: Epilepsy outcome data should be concerned not only with seizure frequency, but should also assess present potential as a function of preexisting capabilities. Reported findings on outcome are not consistent, although view of epilepsy outcome since the 1970'sis much more optimistic. The founding of an Institute of Epileptology can stimulate concerted action to provide the scientific community with improved information on measurement and analysis of the ways in which epilepsy changes the lives of the patient and those immediately involved.