bluuebunny/crossref_metadata_2025_split huggingface dataset:
Epilepsy in Developing Countries
Authors: Jallon P. 
Published: August 2005

Summary: On June 6th and 7th, 1996, an international workshop on specific aspects of epilepsy in the developing world was organized in Geneva by the chairman of the ILAE Commission of Epilepsy in Developing Countries, P. Jallon, involving members of the ILAE, the World Health Organization (WHO), and a network of people who work with epilepsy patients in developing countries. Those taking part included all the members of the ILAE Commission on Epilepsy in Developing Countries, the chairmen of the ILAE Commissions on Tropical Diseases, Epidemiology, Education, Economics, and Drugs, as well as the president, treasurer, and past president of the ILAE; and the president of the IBE, Hanneke de Boer. There were representatives from Eastern European countries (Russia, Slovenia, Turkey), South America (Brazil, Colombia, Uruguay, Ecuador, Venezuela), Africa (Ethiopia, Senegal, South Africa, Togo, Tunisia), and Asia (India, Indonesia, Pakistan, Sri Lanka, The Philippines and China). Representatives from WHO joined the meeting on the last day (Drs. L. Prilipko, A. Janca, and C. L. Bolis).Three major topics were considered—epidemiology, medical assessment, and therapeutic aspects as well as some economic and social aspects of the disease. A second mission of this meeting was to work with WHO representatives to develop a program for action to care for people with epilepsy in these countries.
Relationship Between Seizure Frequency and Costs and Quality of Life of Outpatients with Partial Epilepsy in France, Germany, and the United Kingdom
Authors: van Hout Ben , Gagnon Dennis , Souetre Eric , Ried Sibylle , Remy Claude , Baker Gus , Genton Pierre , Vespignani HervéC , McNulty Pauline 
Published: August 2005

Summary: Purpose: The relationship between seizure frequency and both health care costs and quality of life (QOL) was investigated in a retrospective, cross‐sectional, multicenter study in France, Germany, and the United Kingdom. Methods: Three hundred outpatients with stable partial epilepsy were approximately evenly distributed among five seizure—frequency groups, ranging from seizure—free in the last 3 months (group 1) to daily seizures (group 5). Economic data, obtained through patient interviews and record abstraction, comprised direct medical costs, direct nonmedical costs, and indirect costs for the preceding 3 months. Total societal costs in the three countries were pooled and converted to United States dollar equivalents. QOL was assessed through a self—administered questionnaire, the Functional Status Questionnaire (FSQ). Results: Mean total costs increased from $780 in group 1 to $2,171 in group 5 (p = 0.0001), with significant increases in each cost category as seizure frequency increased. Greater seizure frequency also significantly (p = 0.0270) correlated with lower employment rates, which ranged from 57% in group 1 to 30% in group 5. QOL declined as seizure frequency increased. Particularly affected were basic and intermediate activities of daily living (ADL), mental health, social activity, and feeling about health. Conclusions: The study results show that higher seizure frequencies are associated with higher direct and indirect costs and with reduced QOL for patients with epilepsy.
Familial Unverricht‐Lundborg Disease: A Clinical, Neurophysiologic, and Genetic Study
Authors: Parmeggiani Antonia , Lehesjoki Anna‐Elina , Carelli Valerio , Posar Annio , Santi Andrea , Santucci Margherita , Gobbi Giuseppe , Pini Antonella , Rossi Paola Giovanardi 
Published: August 2005

Summary: Purpose: Progressive myoclonus epilepsies (PMEs) are a clinically and etiologically heterogeneous group of disorders. The authors report clinical, neurophysi‐ological, and genetic findings of a family from Southern Italy with three members affected with PME. Methods: All data about familial and personal antecedents, clinical history, neurologic examination, laboratory tests, neurophysiological findings, brain imaging studies, and DNA analysis were examined. Results: All results were compatible with the features of Unverricht‐Lundborg disease and patients were homozygous for the “Finnish” ancestral haplotype. Conclusions: Work is in progress to identify and characterize the common EPM1 mutation in the Finnish patients. Subsequently, it will be possible to verify the hypothesis on the existence of a common mutation in the Finnish patients and the Italian family under study, or even in other Mediterranean EPM1 families.
Multiple Subpial Transection for Control of Epileptic Seizures: Effectiveness and Safety
Authors: Hufnagel Andreas , Zentner Josef , Fernandez Guillen , Wolf Helmut K. , Schramm Johannes , Elger Christian E. 
Published: August 2005

Summary: Purpose: To assess the efficacy and safety of multiple subpial transection (MST), a new technique in epilepsy surgery, alone and in combination with resection. Methods: MST was performed in 22 patients with intractable epilepsy, 10 of whom were treated with a combination of a resection and MST in functionally important cortex, 6 of whom were treated with a combination of a resection and MST performed outside functionally important cortex, and 6 of whom were treated with MST alone. Results: Of the 6 patients who received MST alone, none became seizure free and 4 showed > 50% reduction of all seizure types. In 2 patients, including 1 with Rasmussen's encephalitis, no change in seizure frequency or intensity occurred. Of the 16 patients in whom MST was combined with a resection, 9 (56%) became seizure free. Six of the remaining 7 patients showed > 95% reduction of all seizure types. Disappearance of epileptiform potentials in the postoperative EEG correlated significantly with complete relief from seizures. Subtle, permanent neurological deficits remained in 5 of 14 patients who received MST in functionally important brain areas. Conclusions: Reduction of the seizure frequency was substantial in 4 of 6 patients who received MST alone, but complete seizure control was not observed. MST surrounding a lesionectomy may be a new surgical approach which would minimize the excised volume and improve seizure control.
Anticonvulsant Action of Gabapentin During Postnatal Development in Rats
Authors: Mareš Pavel , Haugvicova Renata 
Published: August 2005

Summary: Purpose: To study the anticonvulsant effects of gabapentin (GBP) in immature animals. Methods: Motor seizures were induced by pentylenetetrazol in 7‐, 12‐, 18‐, 25‐day‐old, and adult rats. Animals pretreated with GBP (15, 30, or 60 mg/kg i.p.) were compared with controls. The incidence and latency of two types of seizures [minimal (i.e., clonic)] and generalized tonic‐clonic (GTCS) were recorded, and seizure severity was scored. Results: GBP suppressed or at least restricted the tonic phase of GTCS at all the developmental stages studied. In addition, minimal seizures were suppressed in 18‐day‐old rats. Both effects exhibited a tendency to a U‐shaped dose‐response curve. Conclusions: Anticonvulsant effects of GBP were demonstrated in immature and in adult rats.
Partial Cosegregation of Familial Hemiplegic Migraine and a Benign Familial Infantile Epileptic Syndrome
Authors: Terwindt G. M. , Ophoff R. A. , Lindhout D. , Haan J. , Halley D. J. J. , Sandkuijl L. A. , Brouwer O. F. , Frants R. R. , Ferrari M. D. 
Published: August 2005

Summary: Purpose: We studied a large Dutch‐Canadian family, in which two very rare hereditary paroxysmal neurologic disorders, familial hemiplegic migraine (FHM) and a “benign familial infantile epileptic syndrome” concur and partially cosegregate. FHM is a dominantly inherited subtype of migraine with attacks of hemiparesis, linked to chromosome 19p13 in 50% of the families tested. Recently mutations in a brain‐specific P/Q‐type Ca2+ channel α1 subunit gene (CACNLlA4) were identified in families with chromosome 19‐linked FHM. The infantile epileptic syndrome resembles to two other dominantly inherited benign epilepsies occurring in the first year of life, benign familial neonatal convulsions (BFNC), assigned to chromosomes 20q13.2 and 8q, and benign infantile familial convulsions (BIFC), as yet unlinked. Methods: Linkage analysis was performed for the known locations of FHM and BFNC. The question whether the two conditions in this family can be caused by a single gene defect was addressed by additional linkage analysis. Results: We excluded linkage of the infantile convulsions to markers on chromosome 20q13.2, Sq, or 19p13. This indicates the existence of a third locus for benign familial convulsions in the first year of life. Linkage of FHM to these markers was not formally excluded but seems very unlikely. Statistical analysis of whether, in this family, both conditions are caused by a single gene defect was inconclusive. Conclusions: We describe a “benign familial infantile epileptic syndrome“ with attacks of FHM at a later age. Further genetic studies in this family may help to unravel the genetic basis of epilepsy or migraine or both.
Dissociation in Epilepsy and Conversion Nonepileptic Seizures
Authors: Alper Kenneth , Devinsky Orrin , Perrine Kenneth , Luciano Daniel , Vazquez Blanca , Pacia Steven , Rhee Eun 
Published: August 2005

Summary: Purpose: We examined the dimensionality of the item content of the Dissociative Experiences Scale (DES) in relation to the clinical diagnosis of conversion nonepileptic seizures (C‐NES) versus complex partial epilepsy (CPE). Methods: The DES was administered to a sex‐ and agematched sample of 132 patients with C‐NES and 169 with CPE and was factor analyzed with principal components analysis (PCA) with varimax rotation. Results: The mean total DES score was 15.1 in the C‐NES group and 12.7 in the CPE group (p = 0.079). The factors obtained by PCA differentiated the CPE and C‐NES groups more strongly than did the total DES score. The factor accounting for the most variance, interpreted as “depersonalizationderealization,” was significantly greater in C‐NES than CPE (p = 0.005). An “absorption‐imaginative involvement” factor, which included some of the clinical features of posttraumatic stress disorder was elevated only in subjects reporting histories of childhood abuse (p = 0.001) regardless of the diagnosis of CPE or C‐NES. An “amnestic” factor appearing to represent memory problems related to neurologic impairment showed a trend toward elevation in CPE (p = 0.056) and may have confounded the CPE versus C‐NES distinction using total DES scores. Conclusions: The DES has separate underlying dimensions that appear to relate distinctively to depersonalization and derealization, childhood trauma, and neurologic impairment. The heterogeneous item content of the DES is a potential confound that should be appreciated when this instrument is used to study dissociation in neuropsychiatric populations.
The Relation Between Quantitative MRI Measures of Hippocampal Structure and the Intracarotid Amobarbital Test
Authors: Baxendale S. A. , Van Paesschen W. , Thompson P. J. , Duncan J. S. , Shorvon S. D. , Connelly A. 
Published: August 2005

Summary: Purpose: The increasing sophistication of quantitative magnetic resonance imaging (MRI) techniques has generated hopes that they may eventually supersede the intracarotid amobarbital procedure (IAP) in the presurgical screening for bilateral abnormalities in prospective candidates for temporal lobe epilepsy surgery. As the first step toward this aim, the purpose of this study was to examine the relationship between these measures of structural and functional integrity. Methods: We examined the relation between memory performance and pasdfail rates on the IAP and two MRI measures of hippocampal integrity: hippocampal volumes, adjusted for intracranial volume (HCvol) and hippocampal T2 relaxometry (HCT2), in 48 patients with medically intractable temporal lobe epilepsy, who underwent the IAP as part of their presurgical evaluation for temporal lobectomy. Results: The unilateral memory scores from the IAP were not significantly correlated with the corresponding HCvol or HCT2 measures in the right‐ and left‐temporal‐lobe groups. However, the MRI measures of hippocampal asymmetry (right minus left HCvol right minus left HCT2) were significantly correlated with our measure of functional asymmetry, the right minus left hemisphere memory score from the IAP, supporting the role of the IAP in lateralising temporal lobe dysfunction. Forty‐six patients with unilateral hippocampal sclerosis and concordant EEG studies passed the IAP. Two patients failed the memory component of the IAP. In both cases, other presurgical investigations suggested bilateral abnormalities. Conclusions: We conclude that patients with unilateral hippocampal sclerosis, established by a rigorous quantitative MRI protocol, and concordant ictal and interictal EEG findings may not be at risk for postoperative amnesia, despite baseline neuropsychological deficits suggestive of bilateral disturbance.
Widespread Cerebral Structural Changes in Two Patients with Gelastic Seizures and Hypothalamic Hamartomata
Authors: Sisodiya S. M. , Free S. L. , Stevens J. M. , Fish D. R. , Shorvon S. D. 
Published: August 2005

Summary: Purpose: We tested the hypothesis that widespread extralesional abnormalities of cerebral structure exist in association with apparently isolated hypothalamic hamartomata, providing a structural basis for the poor response of seizures to removal of the hamartoma or other apparently focal epilepto‐genic zones present. Methods: High‐resolution magnetic resonance imaging (MRI) brain scans of 2 patients with hypothalamic hamartomata were quantified by determination of regional distribution and symmetry of distribution of cortical gray matter and subcortical matter volumes. The results were compared with normal ranges for the distribution of such tissues in 33 controls. Results: Both patients had abnormalities of distribution of gray and subcortical matter, whereas control subjects did not. These abnormalities were beyond the hamartoma itself, in areas of cerebrum that on visual inspection alone appeared completely normal. Conclusions: Extralesional abnormalities of cerebral structure are present in the cerebrum of patients with hypothalamic hamartoma, as in most patients with other dysgeneses. These abnormalities may explain the poor outcome of epilepsy surgery in patients with this form of dysgenesis. These preliminary findings require further investigation.
Cyclic Alternating Pattern as a Provocative Factor in Nocturnal Paroxysmal Dystonia
Authors: Terzano Mario Giovanni , Monge‐Straws Marie‐France , Mikol Francois , Spaggiari Maria Cristina , Parrino Liborio 
Published: August 2005

Summary: Purpose: We made a polygraphic study of 6 patients with nocturnal paroxysmal dystonia (NPD) in which the cyclic alternating pattern (CAP) parameters were compared with those of a group of age‐ and sex‐matched controls. Methods: All patients met the requirements for NPD diagnosis, characterized by generalized stereotyped movements (dystonic‐dyskinetic), with a 1‐min centered duration but with no clear evidence of epileptic abnormalities in the waking EEG and during nocturnal recordings. Results: Besides the major events, the NPD polysomnograms also showed shorter, repeated episodes of shorter duration (generally <20 s) consisting of abrupt movements involving one or more body segments. Overall, the motor events in patients with NPD were closely related to periods of unstable non‐REM (NREM) sleep, as evidenced by the sequences of CAP, and began during an A phase. According to the conventional scoring parameters, NPD and controls differed only in sleep latency (+14 min in the NPD patients: p < 0.04). However, the architecture of sleep in the group with NPD was characterized by prolonged and irregular NREMREM cycles. In addition, the NPD recordings showed significantly higher values of CAP rate (p < 0.0001). When major motor attacks were suppressed by medication, sleep was characterized by a decrease in the excessive amounts of CAP rate and by a more regular architecture. Conclusions: The modulatory role of CAP on nocturnal motor events is reported.
Initial Human Experience with Ganaxolone, a Neuroactive Steroid with Antiepileptic Activity
Authors: Monaghan Edward P. , Navalta Laura A. , Shum Linyee , Ashbrook Donald W. , Lee David A. 
Published: August 2005

Summary: Purpose: Studies were conducted to establish the safety, tolerability, and pharmacokinetics of the antiepileptic drug (AED) ganaxolone. Ganaxolone belongs to a novel class of neuroactive steroids called epalons, which specifically modulate the γ‐aminobutyric acid type A (GABAA) receptor in the central nervous system (CNS). Chemically related to progesterone but devoid of any hormonal activity, the epalons have potent antiepileptic, anxiolytic, sedative, and hypnotic activities in animals. Methods: Ninety‐six healthy male and female volunteers received ganaxolone in a variety of formulations, doses, and dosing regimens. The pharmacokinetics of ganaxolone were systematically characterized, and adverse events associated with drug use were documented. Results: Ganaxolone was well tolerated after single doses (≥1,500 mg) and after multiple doses (≥300 mg b.i.d for 10 days). Steady‐state plasma levels (trough) occurred after ˜7 days of dosing, with mean steady‐state plasma concentrations (Cmax) in multiple dose studies of between 32 ng/ml (50‐mg doses) and 376 ng/ml (500‐mg doses). No serious or lifethreatening adverse events attributed to the drug were observed. The majority of adverse events reported were mild (82%) to moderate (14%) and were limited to headache, dizziness, somnolence, gastrointestinal disturbances, and malaise. Conclusions: Ganaxolone alone or formulated with pharmaceutical‐grade excipients is rapidly absorbed from the gastrointestinal tract after oral administration in doses ranging from 50 to 1,500 mg. Pharmacokinetic analysis revealed a linear and proportional increase in the area under the curve (AUC) and Cmax values with increasing dose within the expected therapeutic dose range. Safety and tolerability in the clinical program were unremarkable.
Landau‐Kleffner Syndrome: Consistent Response to Repeated Intravenous γ‐Globulin Doses: A Case Report
Authors: Fayad Michel N. , Choueiri Roula , Mikati Mohamad 
Published: August 2005

Summary: Purpose: Although several treatments have been tried for Landau‐Kleffner syndrome (LKS) too many patients are refractory to known therapies. We report an 8–year‐old girl who failed other therapies but who had a consistent response after treatment with intravenous (i.v.) γ‐globulin.Methods: We monitored the girl from the age of 6 years, when she presented with a 6–month history of loss of language with normal hearing, normal brain magnetic resonance imaging (MRI), increased cerebrospinal fluid (CSF) IgG index, and an EEG showing almost continuous, predominantly left‐sided spike‐ and slow‐wave complexes. She had no clinical seizures and did not respond to consecutive trials of valproate (VPA), clonazepam (CZP), prednisone, and carbamazepine (CBZ). She received three courses of intravenous (i.v.) γ‐globulin; after each course, clinical and electrographic improvement lasted a few months. After each of the initial two eourses, clinical improvement lasted 3–4 months but was followed by recurrence of the spikes on the EEG and by speech deterioration.Results: However, her last remission has been continuous for the past 16 months. Her CSF IgG index became normal after the first i.v. γ‐globulin infusion.Conclusions: Based on our experience with this patient and on other investigators' experience, we believe that further research into immunologic mechanisms and therapies of this syndrome are warranted.
Left Hemispheric Dominance of Epileptiform Discharges
Authors: Dean Andy C. , Solomon Gail , Harden Cynthia , Papakostas George , Labar Douglas R. 
Published: August 2005

Summary: Purpose: We reviewed 1,360 EEG reports for all patients studied in two different neurophysiology laboratories during 1 calendar year to determine whether epileptiform discharges have a hemispheric dominance.Methods: Both inpatients and outpatients, with or without epilepsy, were included.Results: Ninety‐four records (6.9%) demonstrated generalized epileptiform activity. Of 95 EEG reports indicating spikes solely from one hemisphere, spikes arose from the left in 61 and from the right in 34. Among 50 other records with bilateral independent spikes with lateralization, 40 were left hemisphere‘dominant and 10 were right hemisphere dominant.Conclusions: These findings raise the possibility that the left cerebral hemisphere may generate focal epilepsy more frequently than the right.
Cyclosporin A and Brain Excitability Studied In Vitro
Authors: Taubøll Erik , Gerdts Remo , Gjerstad Leif 
Published: August 2005

Summary: Purpose: Seizures are frequently observed after organ transplantations. This has been attributed to a direct effect of cyclosporin A (CsA) on the brain, although other mechanisms may also be of importance. The aim of this study was to investigate possible acute and direct effects of CsA on neuronal excitability. Methods: Female rat hippocampal slices were perfused with CsA solutions containing 400 (n = 4), 1,000 (n = 4), 2,000 (n = 6), 8,000 (n = 8) μg/L CsA or control (n = 8) for 30 min, or penicillin, 2,000 IE/ml (n = 7). Actual concentrations of CsA were measured in the perfusate drawn from the slice chamber. To study CsA accumulation in the slices, uptake of radioactive CsA was measured in 12 living and 11 dead slices. Results: Despite a significant accumulation of CsA in the living neuronal slices, no effects were observed on prevolley, field excitatory postsynaptic potential (EPSP), or population spike amplitude. Penicillin, however, led to epileptiform activity within 10 min in all cases. Concentrations of CsA in the perfusate from the slice chamber were about half the calculated levels, demonstrating that the slices had been exposed to actual CsA concentrations in the range of ε200–4,000 μg/L CsA. Conclusions: Our results demonstrate a lack of acute effects of CsA on neuronal excitability within clinically relevant concentrations despite an active accumulation of the drug in the slices. Long‐term effects on brain tissue, indirect metabolic effects, or synergistic effects may be responsible for the neurotoxicity of the drug.
Complications of the Ketogenic Diet
Authors: Ballaban‐Gil Karen , Callahan C. , O'Dell C. , Pappo M. , Moshé S. , Shinnar S. 
Published: August 2005

Summary: Purpose: The ketogenic diet has been successfully used in treatment of pediatric epilepsy for >70 years. Few serious complications caused by the diet have been reported. We report complications that have been experienced by children receiving the ketogenic diet. Methods: In a 22‐month period, we treated 52 children with the classic ketogenic diet and monitored them in a prospective manner. Results: Five children (10%) experienced serious adverse events (AE) after initiation of the diet. Four patients (80%) were treated with valproate (VPA) in addition to the diet, as compared with 25 (53%) of the other 47 children. Two patients developed severe hypoproteinemia within 4 weeks of initiation of the diet, and 1 of them also developed lipemia and hemolytic anemia. A third child developed Fanconi's renal tubular acidosis within 1 month of diet initiation. Two other children manifested marked increases in liver function tests, 1 during the initiation phase and the other 13 months later. Conclusions: Clinicians who wish to use the ketogenic diet must be aware of the potential of serious AE and possible interactions of the diet with VPA.