bluuebunny/crossref_metadata_2025_split huggingface dataset:
“Obtundation Status (Dravet)” Caused by Complex Partial Status Epilepticus in a Patient with Severe Myoclonic Epilepsy in Infancy
Authors: Wakai Shuji , Ikehata Masami , Nihira Hiroshi , Ito Nozomi , Sueoka Hirofumi , Kawamoto Yoshitaka , Hayasaka Hiroyuki , Chiba Shunzo 
Published: August 2005

Summary: Purpose: We report a 1‐year 7‐month‐old boy with severe myoclonic epilepsy in infancy (SME) who exhibited complex partial status epilepticus (CPSE), which was confirmed by ictal video‐EEG analysis. This boy first had a hemi‐convulsion in a hot bath at age 3 months. Thereafter, he exhibited both partial and generalized seizures that were extremely intractable. At age 9 months, he had a prolonged episode of impaired consciousness that fit the “obtundation status in SME” defined by Dravet et al. Methods and Results: Ictal EEG revealed persisting irregular spike‐and‐wave complexes over the left hemisphere, predominantly in the occipitotemporal area, and confirmed CPSE. The EEG abnormalities with decreased level of the consciousness continued ‐6 h after onset of the symptoms even with AED administration. Conclusions: Because SME features both generalized and focal seizures, both types of nonconvulsive status may be seen in SME. Although Dravet et al. already reported long‐lasting atypical absences in patients with SME as “obtundation status,” we demonstrated CPSE in an infant with SME who exhibited a prolonged stuporous state.
The α2‐Adrenoreceptor Agonist Clonidine Suppresses Seizures, Whereas the α2‐Adrenoreceptor Antagonist Idazoxan Promotes Seizures in Amygdala‐Kindled Kittens: A Comparison of Amygdala and Pontine Microinfusion Effects
Authors: Shouse Margaret N. , Langer James , Bier Michael , Farber Paul R. , Alcalde Oscar , Moghimi Robert , Richkind Melvyn , Szymusiak Ronald 
Published: August 2005

Summary: Purpose: We sought to determine whether local, in vivo microinfusion of an α2‐adrenoreceptor agonist and antagonist into either the amygdala or the pons (locus ceruleus, LC) would have contrasting effects on evoked amygdala‐kindled seizure susceptibility. Methods: The study population consisted of 6 amygdala‐kindled kittens, each undergoing the same protocol, in which the amygdala microinfusion paradigm preceded the pontine microinfusion series. Microinfusions (1 μl) of the α2‐agonist clonidine (CLON) and of the α2‐antagonist idazoxan (IDA) were made over 1 min through cannulas adjacent to stimulating electrodes in the kindled amygdala or through cannulas adjacent to recording electrodes in the ipsilateral LC. Order of administered drugs (CLON vs. IDA) and dosages (n = 3 each) was partly counterbalanced. Focal and convulsive seizure thresholds were evaluated 10–12 min postinfusion and compared to thresholds obtained during two interspersed control control conditions (vehicle control = 1 μl microinfusion of sterile saline; sham control = needle insertion only). Results: CLON significantly increased focal and generalized seizure thresholds, whereas IDA significantly reduced seizure thresholds when compared to controls. Magnitude of effects was dose dependent and more potent after pontine than amygdala microinfusion. Conclusions: Our results confirm and extent findings of previous researchers who used unlocalized in vivo manipulations to show that norepinephrine (NE) is a highly antiepileptic agent in the amygdala kindling preparation. With further investigation, the results may ultimately lead to development of microinfusion techniques as an alternative treatment option for limbic epilepsy.
Evaluation of Case Reports of Aplastic Anemia Among Patients Treated with Felbamate
Authors: Kaufman D. W. , Kelly J. P. , Anderson T. , Harmon D. C. , Shapiro S. 
Published: August 2005

Summary:Purpose:Felbamate (FBM) is a new antiepileptic drug (AED) that is often effective in seizure disorders refractory to other treatments; its use has been greatly restricted after cases of aplastic anemia were reported. To elucidate the putative association between FBM and aplastic anemia, we made a detailed evaluation of the first 31 reports.Methods:Hematologic review according to the criteria of the International Agranulocytosis and Aplastic Anemia Study (IAAAS) confirmed 23 cases (74%) as aplastic anemia; FBM was judged to be theonlyplausible cause for three; confounding (mostly by other drugs) was considered possible, but FBM remained the most likely cause for 11; and there was at least one other plausible cause for 9.Results:Using a denominator from sales data of 110,000 persons exposed and a numerator of the cases for which FBM was considered theonlyplausible cause, we established a lower limit of incidence of 27 cases of aplastic anemia per million users as compared with the general population rate of 2.0 per million per year. With all confirmed cases used as the numerator, the upper limit of incidence was 209 per million. The “most probable” incidence was estimated to be 127 per million.Conclusions:Intensive, systematic investigation can maximize the utility of case reports for assessing risks of newly released drugs. The present evaluation confirmed an association between FBM and aplastic anemia; however, confounding was significant for most cases and there was a tenfold range in the “best case” and “worst case” incidence estimates among users.
Early‐Childhood Progressive Myoclonus Epilepsy Presenting as Partial Seizures in Dentatorubral‐Pallidoluysian Atrophy
Authors: Hattori Haruo , Higuchi Yoshihisa , Okuno Takehiko , Asato Reinin , Fukumoto Manabu , Kondo Ikuko 
Published: August 2005

Summary: Purpose: We explored the characteristics of epileptic seizures of progressive myoclonus epilepsy (PME) in 2 brothers with dentatorubral‐pallidoluysian atrophy (DRPLA). Methods: We obtained the case history of the siblings and ictal and interictal EEGs. Postmortem examination or demonstration of elongated CAG repeat in the gene for DRPLA was used to confirm the diagnosis. Results: Two Japanese siblings developed PME characterized by versive or himiclonic seizures with or without secondarily generalized tonic‐clonic convulsions. The elder brother regressed mentally and exhibited increasing spasticity after age 1 year. Myoclonus and seizures developed at age 4 years. The younger brother had shown psychomotor retardation before age 4 years, when he began to deteriorate further neurologically as the elder brother had. He also developed myoclonus and seizures at that age. Seizures in both patients remained partial until their deaths at ages 19 and 15 years, respectively. Ictal EEG verified partial onset of seizure evolving to generalized tonic‐clonic seizure (GTCS). Interictal EEGs showed multifocal paroxysmal discharges with little or no diffuse paroxysms. Postmortem examination or genetic study confirmed the diagnosis of DRPLA. Conclusions: Seizures of patients with DRPLA may present as partial seizures in children with early‐onset PME.
Community‐Based Study of Lennox‐Gastaut Syndrome
Authors: Heiskala Hannu 
Published: August 2005

Summary: Purpose: Before 1986, the spectrum of childhood epilepsies, including Lennox‐Gastaut syndrome (LGS) and Doose syndrome (DS), known collectively as “epilepsia myoclonica astatica”, was believed to represent a single disease. More recently, some investigators have considered these syndromes to be parts of a continuum. To clarify these theories, neurobiologic factors of the syndromes were studied to determine which qualities were shared and which were unique. Methods: A retrospective (1975–985), community‐based (Helsinki metropolitan area and the province of Uusimaa) study was designed to seek children with features of LGS and DS. It was assumed that recall bias and the selection of documented history would be similar throughout the group. Ranks of increasing pathology were assigned to different seizure types, EEG results, and drug treatments. A similar procedure was applied to epidemiologic data. Spearman rank‐order correlations were calculated to determine which features correlated with LGS and which correlated with less severe epilepsy. Results: The survey comprised 75 patients with broadly defined LGS. The annual incidence was 2 in 100,000 children aged 0 to 14 years. Prenatal or perinatal abnormalities did not correlate with severity of epilepsy. As compared with the relatively favorable ranks, the severe epilepsy ranks were more often associated with an early onset of epilepsy, an infectious disease at the onset, delayed development before epilepsy, abnormalities in neurologic or neuroradiologic examinations, and a deteriorating course of the condition. Conclusions: Patients with LGS are more likely than patients with less severe epilepsy to have a younger age at onset of epilepsy, an infection or both, and a deteriorating course of the condition.
Ictal EEG and Single Photon Emission Computed Tomography in a Patient with Cortical Dysplasia Presenting with Atonic Seizures
Authors: Aihara Masao , Hatakeyama Kazuo , Koizumi Kiyoshi , Nakazawa Shinpei 
Published: August 2005

Summary: Purpose: Ictal studies of atonic seizures in children with epilepsy are rare. To clarify the neurophysiologic mechanism of such seizures, we describe a cluster of atonic seizures in a 6‐year‐old girl with cortical dysplasia. Methods: Ictal activity was recorded as slow‐wave bursts beginning over the right frontocentral regions, where the cortical dysplasia was detected by magnetic resonance imaging (MRI), and propagating to the left hemisphere. Results: The polygraphic recording demonstrated that the atonia indicated by the sudden interruption of elecromyogram (EMG) discharges corresponded to the bilaterally synchronous spike‐and‐wave complexes. An ictal single photon emission computed tomography (SPECT) study with 99mTc hexamethyl propylene amine oxime ([99mTc]HMPAO) simultaneously performed during EEG recording showed marked bilateral mesial frontal and right frontoparietal hyperperfusion. Conclusions: Our findings suggest that the neurophysiological mechanism of the atonic seizures may be produced by a strong inhibition in the bilateral motor cortexes. Ictal SPECT is valuable in demonstrating the pathophysiology of both atonic seizures and secondary bilaterally synchronous spike‐and‐wave complexes in the EEG.
Carbamazepine Toxicity with Lamotrigine: Pharmacokinetic or Pharmacodynamic Interaction?
Authors: Besag F. M. C. , Berry D. J. , Pool F. , Newbery J‐J. E. , Subel B. 
Published: August 2005

Summary: Purpose: To determine whether the toxicity that occurs in some patients when lamotrigine (LTG) is added to carbamazepine (CBZ) is the result of either a pharmacokinetic or a pharmacokinetic or a pharmcodynamic interaction. Methods: Escalating LTG doses were added to ongoing CBZ treatmcnt in 47 patients. All patients had blood samples collected for drug concentration measurement, including the epoxide metabolite of CBZ, before starting LTG treatment and after stabilising at each dose escalation. Patients also were examined for signs of toxicity. Results: After LTG was introduced, nine patients demonstrated clinical signs of CNS toxicity, mainly diplopia and diziness. There was no significant (p = 0.05) change in the serum concentrations of either CBZ or its epoxide metabolite when LTG was added either to the group as a whole or to the nine patients who experienced adverse CNS effects. LTG serum concentrations also were below the level at which the common signs of LTG toxicity, such as nausea, vomiting, or unsteadiness, are more likely to occur. In seven of the nine patients who exhibited CNS toxicity. CBZ serum concentrations were >8 mg/L on LTG introduction. Conclusions: Toxicity is more likely to occur when LTG is added to CBZ if the initial CBZ level is high. typically >8 mg/L. This appears to be the result of a pharmacodynamic interaction. A reduction of CBZ dose usually resolves the toxicity, allowing the LTG dose to be escalated to maximal effect. It is not usually necessary to stop either drug.
Selective Amobarbital Test for the Determination of Language Function in Patients with Epilepsy with Frontal and Posterior Temporal Brain Lesions
Authors: Hajek Marketa , Valavanis Anton , Yonekawa TYasuhiro , Schiess Regula , Buck Alfred , Wieser Heinz Gregor 
Published: August 2005

Summary: Purpose: Selective amobarbital tests with selective temporary inactivation of the left frontal operculum and/or the left parietotemporal cortex were performed in 5 patients with left‐hemispheric epileptogenic lesions in or adjacent to classical Broca's and/or Wemicke's aréa. The aim was to assess language functions in these brain regions before surgery, to tailor the surgery according to the individual functional importance of these brain regions, and to predict postoperative outcome. Methods: Amobarbital was injected by transfemoral selective catheterization of the arteries supplying the target areas. Along with neuropsychological and neurological testing during the amobarbital procedure, EEG recordings were performed in all patients, and [199mTc]HMPAO‐single photon emission computed tomography (SPECT) in 2 patients. Results: After the amobarbital injection into the left frontal opercular region, there was no recognizable language dysfunction in 3 patients. In these 3 patients, the lesions in or adjacent to the frontal operculum were completely resected without postoperative language impairment. In the remaining 2 patients, temporary language impairment after the amobarbital injection into the left frontoopercular and Wemicke's region, respectively, suggested language functions in these areas. Surgery was restricted to the left mesiotemporal lobe in 1 patient. In the other patient, the tumor infiltrating the frontal operculum was restrictively resected. Postoperatively, the fist patient had no language impairment, but the latter had transient global aphasia, from which she recovered. Conclusions: Selective temporary amobarbital inactivation of brain regions that may be associated with language has clearly indicated the presence or absence of language functions in these regions. The test contributed substantially to planning of the surgical approach in each patient. The predictive alue of the amobarbital test was demonstrated by the postoperative outcome.
Predictive Value of Interictal Epileptiform Discharges During Non‐REM Sleep on Scalp EEG Recordings for the Lateralization of Epileptogenesis
Authors: Adachi Naoto , Alarcon Gonzalo , Binnie Colin D. , Elwes Robert D. C. , Polkey Charles E. , Reynolds Edward H. 
Published: August 2005

Summary: Purpose: EEG recording during sleep is widely used in the assessment of epilepsy, particularly in candidates for surgery, yet the diagnostic value of this procedure is not well established. We evaluated the predictive reliability of interictal epileptiform discharges (IEDs) for localization in pre‐surgical patients with temporal lobe epilepsy (TLE) during non‐REM sleep. Methods: Preoperative scalp EEG recordings with waking and sleep states were assessed in 83 patients with TLE in whom localization of the epileptogenic zone was subsequently confirmed by successful surgical treatment (patient seizure‐free >1 year). Results: The accuracy of EEG recordings for prediction of lateralization significantly changed from 51.8% during waking to 78.3% during sleep. After exclusion of patients who showed no discharges, the predictive value changed from 74.1 to 86.7%. However, in patients in whom the waking scalp EEG lateralized incorrectly, no improvement in reliability was achieved by sleep recording. Conclusions: Our results suggest that IEDs occurring in non‐REM sleep provide more accurate information for lateralization of epileptogenesis than do those occurring during waking. This gain of diagnostic information was obtained in patients who showed either bilateral or no discharges in waking records, because unilateral discharges arising de novo in sleep were always correctly lateralizing. On the other hand, in patients who showed unilateral discharges in the awake state, whether ipsilateral or contralateral to the epileptogenic zone, the findings were generally unchanged during sleep.
Halothane as a Neuroprotectant During Constant Stimulation of the Perforant Path
Authors: Walker M. C. , Perry H. , Scaravilli F. , Patsalos P. N. , Shorvon S. D. , Jefferys J. G. R. 
Published: August 2005

Summary: Purpose: To determine the neuroprotective effects of halothane during constant stimulation of the perforant path. Methods: Male Sprague‐Dawley rats had electrodes implanted into the perforant path and dentate granule cell layer under halothane anaesthesia (1‐2% in oxygen). They were then divided into four groups. In group 1 (n = 9), the perforant path was stimulated at 20 Hz for 2 h under halothane anaesthesia (1‐2%). In group 2 (n = 3), the animals were unstimulated but maintained under halothane anaesthesia (1‐2%) for 2 h with the electrodes in place. Both groups 1 and 2 had the electrodes removed and were then allowed to recover fully from the anaesthetic. In groups 3 and 4, the electrodes were held in place with dental acrylic. Both of these groups were allowed to recover fully from anaesthesia. In group 3 (n = 3), 24‐48 h after recovery from anaesthesia, the perforant path was stimulated at 20 Hz for 2 h. Group 4 (n = 3) received no stimulation. After 14‐17 days, the rats were killed, and morphometry and cell counts were performed on the hippocampi from rats in groups 1 and 2. Results: Cell densities were not significantly different between control (group 2), unstimulated rats, and animals stimulated under halothane anaesthesia (group 1). Stimulation in the unanaesthetised rats resulted in severe neuronal loss in hilus, CA1, and CA3. Conclusions: Halothane protects hippocampal neurons against damage induced by constant stimulation of the perforant path.
Clinical Patterns of Patients with Temporal Lobe Epilepsy and Pure Amygdalar Atrophy
Authors: Guerreiro C. , Cendes F. , Li L. M. , Jones‐Gotman M. , Andermann F. , Dubeau F. , Piazzini A. , Feindel W. 
Published: August 2005

Summary: Purpose: MRI volumetric measurements (MRIvol) have been proven reliable in determining mesial temporal atrophy in patients with TLE. We attempted to correlate the clinical features with different patterns of hippocampal formation (HF) and amygdala (AM) atrophy in patients with TLE without foreign tissue lesion. Methods: We studied 65 patients with refractory TLE. They were divided into five groups according to MRIvol results: pure AM atrophy (n = 1 1, 10 unilateral and one bilateral), unilateral HF atrophy (n = 16), bilateral HF atrophy (n = 12), unilateral AM+HF atrophy (n = 13), and patients with normal volumes of AM and HF (n = 13). MRIvol of AM and HF were performed by using a protocol previously described by Watson et al. (Neurology 1992;42: 1743–50). Results: Patients with AM atrophy had later onset of seizures compared with those with unilateral HF atrophy (p < 0.01). History of febrile convulsions (p < 0.0001) and frequent secondarily generalized tonic‐clonic seizures (GTCSs) were more often found in patients with HF atrophy compared with those with pure AM atrophy and those with normal volumes (p = 0.04). Prolonged postictal confusion was more often found with AM atrophy (p = 0.05). Memory impairment was more severe in patients with HF atrophy than in those with AM atrophy only or in those with normal volumes (p = 0.03). There were no significant differences among the five groups in the following parameters: age, duration of epilepsy, seizure frequency, and presence and type of aura. Conclusions: Prolonged postictal confusion appeared to be related to AM atrophy, in keeping with previous clinical observations. These patients also had a lower incidence of early febrile convulsions, older age at epilepsy onset, lower frequency of secondary GTCS, and lesser memory dysfunction compared with patients with hippocampal atrophy.
Family Interactions as Targets for Intervention to Improve Social Adjustment After Epilepsy Surgery
Authors: Langfitt John T. , Wood Beatrice L. , Brand Kerry L. , Brand Jennifer , Erba Giuseppe 
Published: August 2005

Summary: Purpose: To identify family interactions associated with psychosocial outcome of epilepsy surgery, to design interventions to improve patient outcome. Methods: A cross‐sectional, case series study of relations among observed family behavior and psychosocial outcome of 43 patients after temporal lobectomy. Videotaped family behavior during family discussion tasks was rated for predominant family affect, affective range, and support of patient autonomy. Multiple regression analyses tested the relation of observed family characteristics to outcomes, controlling for seizure control and other psychological and disease characteristics. Results: Predominant family affect predicted patients' social adjustment independent of postoperative seizure status and other disease characteristics. The relation between predominant affect and social adjustment was stronger among patients with persisting complex partial seizures (CPSs; r =−0.91), versus patients with auras (r =−0.38) and seizure‐free patients (r =−0.28; multiple R = 0.71; p < 0.05). Families with a positive affective climate supported patients' autonomy. Conclusions: Two potential targets were identified for family intervention to improve postsurgical social adjustment: (a) family interactions that support a predominantly positive affective climate, and (b) family interactions that support patient autonomy. These findings are consistent with findings in normal and other clinical populations. They identify specific interactions that give rise to positive versus negative affective climate and support versus undermining of autonomy. These results lay the groundwork for intervention studies targeting these specific family interactions. Such intervention studies would clarify the direction of effect of the observed relationships and would test the efficacy of family intervention for improving psychosocial outcomes for patients with epilepsy.
Quantitative EEG Analyses and Surgical Outcome After Corpus Callosotomy
Authors: Matsuzaka Tetsuo , Ono Kenji , Baba Hiroshi , Matsuo Mitsuhiro , Tanaka Shigeki , Kamimura Naohisa , Tsuji Yoshiro 
Published: August 2005

Summary: Purpose: To clarify the relation between quantitative electroencephalogram (EEG) findings and outcome following corpus callosotomy (CC). Methods: The degree of bilateral synchrony and morphologic similarity of spike‐wave discharges was analyzed by using a cross‐correlation analysis and the measurements of amplitude differences between bilateral homologous regions in 22 patients who underwent anterior CCs for intractable symptomatic generalized epilepsies (SGE; 17 patients) and frontal lobe epilepsy (five patients). Results: Interictal generalized synchronous spike‐wave (GSSW) bursts in the SGE patients were disrupted and changed to unilateral spike‐waves (USWs) in 11 patients and to bilaterally independent spike‐waves (BISWs) in six. The USW group had better surgical outcome than the BISW group. Preoperatively, the USW group had significantly lower interhemispheric synchrony (IS) and fewer regional changes in the side leading in time and the side dominant for amplitude, suggesting unilaterally predominant epileptogenesis that triggered the secondary bilateral synchrony. Postoperatively, the BISW group had a more marked reduction in IS because of independent discharges from bilateral epileptogenic areas, and the USW group had a greater amplitude difference because of unilateralized spike‐waves. In addition, an excellent surgical outcome was related to (a) the preoperative degree of the morphologic similarity of the bilateral spike‐waves (only a small variation during a burst of spike‐waves) and the few instances of regional changes in the side leading in time and in the side dominant for amplitude; and (b) to large postoperative amplitude differences. Conclusion: Preoperative quantitative EEG analyses enabled us to predict the underlying conditions of epileptogenesis and the surgical outcomes in patients undergoing CC.