bluuebunny/crossref_metadata_2025_split huggingface dataset:
Lamotrigine Therapy in Juvenile Neuronal Ceroid Lipofuscinosis
Authors: Åberg L. , Kirveskari E. , Santavuori P. 
Published: August 2005

Summary: Purpose: To evaluate the effects of lamotrigine (LTG) therapy on epileptic seizures and general well‐being in patients with juvenile neuronal ceroid lipofuscinosis (JNCL). Methods: LTG was initiated in 28 patients with JNCL. The mean age of the patients at the initiation of LTG was 13.7 years (range, 6.7–28.2 years). LTG was started at a dosage of 0.1–0.5 mg/kg/day and increased every 2 weeks until a maintenance dose of 1.25–15 mg/kg/day was reached. On the basis of the indication for LTG therapy, the patients could be divided into four groups. In the first group, LTG was initiated on an add‐on basis; in the second group, LTG was started as the first antiepileptic drug (AED) because of seizures, and in the third group, despite no preceding seizures, because of epileptiform activity in the whole‐night polysomnography; in the fourth group, LTG replaced valproate (VPA), which was discontinued because of adverse side effects. The efficacy was assessed after 1 year on LTG. The mean follow‐up time was 2.8 years (range, 1.3–5.8). Results: LTG had a favorable effect in 23 of 28 patients. A decrease in frequency of seizures of ≥50% was observed in 10 and a decrease in severity of seizures in nine of the 22 patients who had preceding seizures. Increases in well‐being were found in 18 of 28. During the follow‐up, LTG was continued as monotherapy in 13 of 19 patients. Conclusions: In light of our experiences, LTG seems to be a valuable drug in JNCL.
Differential Cognitive Effects of Carbamazepine and Gabapentin
Authors: Meador K. J. , Loring D. W. , Ray P. G. , Murro A. M. , King D. W. , Nichols M. E. , Deer E. M. , Goff W. T. 
Published: August 2005

Summary: Purpose: The cognitive effects of the newer antiepileptic drugs (AEDs) compared with the older standard AEDs are uncertain. Methods: We directly compared the cognitive effects of carbamazepine (CBZ) and gabapentin (GBP) in 35 healthy subjects by using a double‐blind, randomized crossover design with two 5‐week treatment periods. During each treatment condition, subjects received either GBP, 2,400 mg/day, or CBZ (mean, 731 mg/day) adjusted to a dose to achieve midrange standard therapeutic blood levels (mean, 8.3 μg/ml). Subjects were tested at the end of each AED treatment period and in four drug‐free conditions [two pretreatment baselines and two posttreatment washout periods (1 month after each AED)]. The neuropsychological test battery included 17 measures yielding 31 total variables. Results: Direct comparison of the two AEDs revealed significantly better performance on eight variables for GBP, but none for CBZ. Comparison of CBZ and GBP to the nondrug average revealed significant statistical differences for 15 (48%) of 31 the variables. Pairwise follow‐up analyses of the 15 variables revealed significantly better performance for nondrug average on 13 variables compared with CBZ, and on four compared with GBP. GBP was better than nondrug average on one variable. Conclusions: Although both CBZ and GBP produced some effects, GBP produced significantly fewer untoward cognitive effects compared with CBZ at the dosages used in this study.
Acute and Chronic Effects of Seizures in the Developing Brain: Lessons from Clinical Experience
Authors: Mizrahi Eli M. 
Published: August 2005

Summary:Seizures in the neonate are often considered a form of status epilepticus (SE) because they are relatively prolonged, difficult to control with antiepileptic drugs (AEDs), and may be associated with significant morbidity and mortality. Despite their clinical importance, there is still no clear understanding of how seizures may affect the developing brain. Although both basic neuroscience and clinical research have addressed these issues, there are difficulties in the design and analysis of each type of investigation. Animal studies should reflect the human condition, the most relevant studies being those that consider neocortical rather than hippocampal seizures. Clinical investigations should be based on precise, age‐specific definitions of seizures of epileptic origin and of SE. Treatment strategies should be standardized with defined rationale and end points. Outcome measures are best when defined and quantifiable. The relative effects of underlying CNS injuries that coexist with the onset of neonatal seizures may be difficult to differentiate from the effects of the seizures themselves or their treatment. Current clinical studies suggest that the overriding factors in determining the outcome of neonates with seizures are the cause, the degree, and the distribution of brain injury at the time of seizure occurrence. However, such studies have limitations and may not yet employ methodology sensitive enough to detect a full range of adverse effects of seizures themselves.
Acute and Chronic Effects of Seizures in the Developing Brain: Experimental Models
Authors: Jensen Frances E. 
Published: August 2005

Summary:Clinical experience suggests two major components to the relationship between brain development and epilepsy. First, the maturational state of the immature brain appears to generally decrease seizure threshold and contribute to a different seizure phenotype from the adult. Second, certain forms of seizures, when present during development, may modify brain maturation to result in chronic epilepsy and/or other neurocognitive deficits.Maturational studies in animals suggest there are numerous factors developmentally regulated in such a way as to increase excitability in immature neuronal networks in the forebrain. The developing brain appears to exhibit a transient overexpression of glutamate receptors, glutamate receptor subunit composition permissive of enhanced excitatory neurotransmission, a relative lack of GABAergic inhibitory transmission, and ion channel expression and homeostasis which enhance neuronal excitability. The increased excitatory “drive” that is likely to be critical for normal brain development may share common mechanisms with those responsible for rendering the immature brain more susceptible to seizures, seizure induced plasticity (epileptogenesis), and neuronal injury. Furthermore, the coincidence of seizures during early postnatal brain development may modify many of these parameters, which in turn may promote long term epilepsy.
Pharmacology of Antiepileptic Drug Polypharmacy
Authors: Ferrendelli James A. 
Published: August 2005

Summary:Therapeutic options for epilepsy are increasing, and all drugs can produce adverse side effects. Physicians should first use monotherapy when treating an epileptic patient. If one drug proves insufficient to control all seizures without intolerable side effects, then careful combination of the fewest drugs possible must be used. Combine drugs with different mechanisms of action rather than drugs with similar mechanisms of action. Closely monitor the patient for adverse side effects, which increase when multiple drugs are taken, and measure antiepileptic blood levels more frequently than with patients who are on monotherapy.
Gabapentin as Adjunctive Therapy for Partial Seizures
Authors: Bruni Joseph 
Published: August 2005

Summary:The safety, tolerability, and efficacy of gabapentin as adjunctive therapy were assessed in epileptic patients who had experienced up to four complex partial seizures per month while receiving phenytoin and/or carbamazepine. This was a multicenter, open‐label prospective study, with the treatment period lasting 20 weeks. The gabapentin dosage was titrated to effective tolerated dose up to 2400 mg/day. Quality of life was evaluated with the QOLIE‐10 questionnaire. A total of 141 patients were enrolled; 114 patients were evaluated for efficacy analysis. The mean maintenance dose of gabapentin was 1600 mg/day. Seventy‐one percent of patients (81 patients) experienced a 50% or greater reduction in seizure frequency and 46% (52 patients) became seizure free. The most frequent adverse effects included drowsiness (16%), dizziness (9%), and asthenia (6%). Sixteen patients (11%) discontinued the study prematurely because of adverse events. A significant improvement was observed in five of the 10 questions of the QOLIE‐10.
Hypothalamic Neuronal Loss and Altered Circadian Rhythm of Temperature in a Rat Model of Mesial Temporal Lobe Epilepsy
Authors: Quigg Mark , Clayburn Hope , Straume Martin , Menaker Michael , Bertram Edward H. 
Published: August 2005

Summary: Purpose: Numerous dysfunctions in endogenous hypothalamic function have been associated with mesial temporal lobe epilepsy (MTLE). One endogenous activity is the circadian rhythm of temperature (CRT). In this study we examined whether hypothalamically mediated function is altered in the electrically induced, self‐sustained, limbic status epilepticus model of MTLE. We then wished to determine whether there was a structural basis for regulatory alterations. Methods: We measured CRT with peritoneal temperature telemetry obtained in light‐entrained (LD) and in free‐running, constant‐dark (DD) conditions. CRT from epileptic and controls of normal animals and kindled animals were quantized by fast Fourier transform‐nonlinear least squares analysis to determine rhythmic complexity. Results: The circadian component of CRT was preserved in all animals. In DD, CRTs of epileptic animals were more complex than those of normal animals. CRT of kindled animals showed no increased complexity after electrically induced seizures. Neuronal density was decreased in regions of the anterior and posterior hypothalamus but not in the suprachiasmatic nuclei from the epileptic rats. Conclusions: Alterations in CRT due to the epileptic state were independent of isolated seizures. Altered circadian thermoregulation in epileptic rats corresponded to regional hypothalamic neuronal loss. Structural changes of the hypothalamus may explain alterations in endogenous rhythms in MTLE.
Nonepileptic Seizures After Resective Epilepsy Surgery
Authors: Glosser Guila , Roberts Delight , Glosser David S. 
Published: August 2005

Summary: Purpose: To identify factors that are associated with the emergence of nonepileptic seizures (NES) after resective epilepsy surgery. Methods: Twenty‐two patients with medically refractory epilepsy in whom NESs were documented by EEG after resective surgery were compared with a larger series of epilepsy surgery patients on demographic, neurologic, and psychiatric variables. Results: NES tended to become apparent in the first few months after surgery. Patients who developed NESs did not differ from other epilepsy surgery patients in terms of age, IQ, or preoperative psychiatric diagnoses. However, surgical NES patients’ neurologic problems and seizures began later in life, the NES group included a larger proportion of female subjects and patients with right hemisphere surgery, and NES patients were more likely to develop non‐NES psychiatric problems after surgery. Conclusions: The heterogeneous collection of behaviors subsumed under the label NESs are determined by multiple factors. Several variables were found to be specifically associated with the development of NES after resective epilepsy surgery. A disproportionate number of postsurgical NES patients are female, they have primary neurologic dysfunction in the right hemisphere, and their epileptic seizures often began after adolescence. We propose that at least one group of patients with somatoform tendencies develop NESs as part of the psychiatric instability that occurs often in the few months after resective surgery.
Relation of Interictal Spike Frequency to 1H‐MRSI‐Measured NAA/Cr
Authors: Serles Wolfgang , Li Li Min , Caramanos Zografos , Arnold Douglas L. , Gotman Jean 
Published: August 2005

Summary: Purpose: Whereas EEG spiking and decreases of the neuronal marker N‐acetyl‐aspartate (NAA) both localize well the epileptic focus, the significance of the intensity of these variables is unclear. Therefore we investigated whether the frequency of interictal surface spikes is related to the degree of N‐acetyl‐aspartate/creatine (NAA/Cr) ratio decrease as measured by proton magnetic resonance (MR) spectroscopic imaging (1H‐MRSI) in patients with intractable partial epilepsy. Methods: We retrospectively studied 14 patients, nine with temporal lobe epilepsy and five with frontal lobe epilepsy. Spikes that occurred during prolonged video‐EEG monitoring from electrodes placed according to the International 10–20 system were counted blinded to the 1H‐MRSI results. Eight electrode positions (F3/4, C3/4, T3/4, T5/6) were assigned to underlying brain subregions in the 1H‐MRSI volume of interest. We converted NAA/Cr ratios into z‐scores (NAA/Crz) to compared NAA/Cr values directly across subregions. We calculated Spearman rank‐order (ρ) and Pearson product‐moment (r) correlations between spike frequency and NAA/Crz values overall, as well as within each brain subregion. Results: We found an overall negative relation between spike‐frequency data and NAA/Crz data (ρ=−0.341). When analyzing only spiking subregions, this negative relation became slightly stronger (ρ=−0.442; r=−0.338). When data from the eight sites were considered separately, this negative relation remained in most instances. Conclusions: Our results reveal a trend toward higher interictal spike frequencies on surface EEG in regions of pronounced neuronal metabolic damage or dysfunction. This suggests that both variables parallel an underlying pathologic substrate, although the pathophysiologic processes may be distinct.
Epidemiologic Study of Epilepsy in Young Singaporean Men
Authors: Kun Loh Ngai , Ling Lee Wei , Wah Yew Woon , Lian Tjia Tjoei 
Published: August 2005

Summary: Purpose: This survey of 20,542 Singaporean men born in 1974 studied the clinical features of young men diagnosed with epilepsy on preenlistment screening. Methods: All male citizens in Singapore are medically screened at age 18 years before enlistment for compulsory military service. Patients suspected to have epilepsy are then referred to government hospitals for further management. We interviewed the patients and their parents and reviewed their hospital records. Results: Eighty‐nine patients with epilepsy were identified, indicating a lifetime prevalence of 4.9/1,000 males by age 18 years. The lifetime prevalence of epilepsy among Chinese, Malays, and Indians were 5.2, 2.8, and 6.4/1,000, respectively; these differences were not statistically significant. The mean age of seizure onset was 11.1 years. Generalized seizures (65.2%) were commoner than partial seizures (34.8%); common seizure types included generalized tonic‐clonic seizures (52.8%), complex partial seizures with secondary generalization (24.7%), and myoclonic seizures (5.6%). Common epileptic syndromes included temporal lobe epilepsy (16.9%), juvenile myoclonic epilepsy (5.6%), and frontal lobe epilepsy (2.2%). Eighty‐four (94.4%) patients sought medical treatment, and seven (7.9%) patients sought additional traditional treatment. Although 70 (78.7%) patients responded to medication, 14 (15.7%) patients remained refractory to treatment. Conclusions: The lifetime prevalence of epilepsy in young Singaporean men was 4.9/1,000. The majority (65.2%) had generalized seizures. Temporal lobe epilepsy was the commonest (16.9%) defined epilepsy syndrome. More patients with epilepsy (94.4%) sought medical treatment, although 15.7% remained refractory to medication.
Longitudinal Follow‐Up in 145 Patients with Medically Refractory Temporal Lobe Epilepsy Treated Surgically Between 1984 and 1995
Authors: Salanova Vicenta , Markand Omkar , Worth Robert 
Published: August 2005

Summary: Purpose: There are few studies of prolonged longitudinal follow‐up after temporal resections. Methods: We analyzed 145 consecutive patients with temporal lobe epilepsy treated surgically. Patients had a comprehensive presurgical evaluation, including video‐EEG, psychometric testing, magnetic resonance imaging (MRI), single‐photon emission computed tomography (SPECT), intracarotid amobarbital procedure (IAP), and recently, volumetric head MRIs and F‐fluorodeoxyglucose‐positron emission tomography (FDG‐PET) scans. Most had en bloc temporal resections, and a few had lesionectomies and resection of the epileptogenic zone. There was no surgical mortality. Longitudinal follow‐up data of the seizure outcome were analyzed by actuarial analysis. Patients were followed up at 6 weeks, 3 months, 6 months, and then on a yearly basis. The mean follow‐up was 5.6 years. Results: Sixty‐six percent were seizure free at 1 year, 63% at 2 years, 60% at 5 years, and 55% at 10 years follow‐up. Moreover, 85%, became seizure free for ≤2 at the time of last follow‐up or had rare seizures. Patients who were seizure free for 1 and 2 years after surgery, had an 83% and 92% probability, respectively, of remaining seizure free at the time of last follow‐up. Ninety‐one percent of patients with small tumors and cavernous angiomas became seizure free compared with 69% of patients with hippocampal sclerosis. Conclusions: Actuarial analysis showed that the long‐term surgical outcome of temporal lobe epilepsy remains favorable. Follow‐up at 1 and 2 years is highly predictive of the long‐term outcome. Patients with discrete lesions had the best outcome. Most of the patients with late recurrences had hippocampal sclerosis or temporal lobe gliosis. Some patients with postoperative seizures eventually became seizure free, reflecting the running‐down phenomenon.
Epileptic Spasms Preceded by Partial Seizures with a Close Temporal Association
Authors: Kubota Toshiko , Aso Kosaburo , Negoro Tamiko , Okumura Akihisa , Natsume Jun , Takada Hiroyuki , Itomi Kazuya , Watanabe Kazuyoshi , Yamamoto Naoki 
Published: August 2005

Summary: Purpose: To investigate the distinctive features of patients with West syndrome who had partial seizures followed by epileptic spasms (PS‐ES). Methods: We examined 45 patients with West syndrome whose epileptic spasms were recorded with simultaneous video‐electroencephalography (EEG) monitoring between 1982 and 1996. We investigated the patients who had PS‐ES and compared the PS‐ES patients with the 37 patients without PS‐ES. Results: Of the 45 patients who had epileptic spasms in clusters (ES) and hypsarrhythmia on the interictal EEG, eight (17%) had ES preceded by partial seizures (PS) with a close temporal association. Seven of these eight were female patients. The underlying disorders were tuberous sclerosis (one patient), Aicardi syndrome (one), nonketotic hyperglycinemia (one), and focal cortical dysplasia (one). The etiology was unknown in the remaining four patients, but was suspected to be of prenatal origin in three. Three types of seizure sequence were identified: PS followed several seconds later by ES (two patients), alternating PS and ES starting with PS (three), and PS gradually replaced by ES with overlapping of the two (three). PS‐ES disappeared or was replaced by other types of seizures in 1–34 months. Six patients could not walk, and all patients could not speak any sentences at age 3 years. Conclusions: Compared with patients without PS‐ES, those with PS‐ES more often had organic brain lesions of prenatal origin, other types of seizures before the onset of ES, asymmetric hypsarrhythmia on the EEG, and poor psychomotor outcome.
Slow Hyperpolarization in Cortical Neurons: A Possible Mechanism Behind Vagus Nerve Simulation Therapy for Refractory Epilepsy?
Authors: Zagon Aniko , Kemeny Andras A. 
Published: August 2005

Summary: Purpose: Recent studies have shown that chronic, intermittent stimulation of the left vagus nerve (VNS) decreases the frequency, duration, and/or intensity of seizures in some patients with medically refractory focal seizures. Although VNS is being used in an increasing number of patients, the neuronal mechanism behind VNS therapy of refractory epileptic seizures is yet unclear. Methods: In vivo intracellular recordings were used to study responses elicited by the VNS in pyramidal neurons of the parietal association cortex in anesthetized rats. Results: Low‐intensity trains of VNS, which activated predominantly myelinated fibers (100 μA, 30 Hz, 0.5 millisecond, 20 seconds), elicited a slow hyperpolarization (onset latency 17.4 ± 2.0 seconds, amplitude –4.7 ± 0.6 mV, duration 35 ± 3.2 seconds; n = 19). Increasing the intensity of VNS to recruit nonmyelinated vagal fibers (200 μA) led to an increase in the magnitude of the response in some neurons while failed to evoke a response in others. On increasing the stimulus intensity to 500 μA, only one in nine neurons exhibited a visible response. All recorded and visualised neurons were pyramidal cells in cortical layer V. Conclusions: Stimulus intensities that activate predominantly myelinated fibers (less than 200 μA) were most effective to induce slow vagal hyperpolarization. It is suggested that slow hyperpolarization may be one of the mechanisms that underlie the seizure‐reducing effect of VNS, by means of reducing the excitability in neurons that would be involved in propagation of seizure activity. As the balance of activity in myelinated and nonmyelinated primary vagal afferents influenced the effect of VNS stimulation, it is likely that the effect of VNS is modulated as changes occur in the underlying vagal tone.
Electro‐Oculography, Electroretinography, Visual Evoked Potentials, and Multifocal Electroretinography in Patients with Vigabatrin‐Attributed Visual Field Constriction
Authors: Harding G. F. A. , Wild J. M. , Robertson K. A. , Lawden M. C. , Betts T. A. , Barber C. , Barnes P. M. F. 
Published: August 2005

Summary: Purpose: Symptomatic visual field constriction thought to be associated with vigabatrin has been reported. The current study investigated the visual fields and visual electrophysiology of eight patients with known vigabatrin‐attributed visual field loss, three of whom were reported previously. Six of the patients were no longer receiving vigabatrin. Methods: The central and peripheral fields were examined with the Humphrey Visual Field Analyzer. Full visual electrophysiology, including flash electroretinography (ERG), pattern electroretinography, multifocal ERG using the VERIS system, electro‐oculography, and flash and pattern visual evoked potentials, was undertaken. Results: Seven patients showed marked visual field constriction with some sparing of the temporal visual field. The eighth exhibited concentric constriction. Most electrophysiological responses were usually just within normal limits; two patients had subnormal Arden electro‐oculography indices; and one patient showed an abnormally delayed photopic b wave. However, five patients showed delayed 30‐Hz flicker b waves, and seven patients showed delayed oscillatory potentials. Multifocal ERG showed abnormalities that sometimes correlated with the visual field appearance and confirmed that the deficit occurs at the retinal level. Conclusion: Marked visual field constriction appears to be associated with vigabatrin therapy. The field defects and some electrophysiological abnormalities persist when vigabatrin therapy is withdrawn.