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Clonic Seizures (clonic + seizures)
Selected AbstractsInterobserver agreement in neonatal seizure identificationEPILEPSIA, Issue 9 2009Aileen Malone Summary Objectives:, Accurate diagnosis of neonatal seizures is critically important and is often made clinically, without EEG (electroencephalography) monitoring. This observational study aimed to determine the accuracy and interobserver reliability of healthcare professionals in distinguishing clinically manifested seizures from other neonatal movements, when presented with clinical histories and digital video recordings only. Methods:, Twenty digital video recordings of paroxysmal movements in term and preterm infants were selected from a video-EEG database. The movements were categorized as seizure and nonseizure using EEG. Health care professionals (n = 137) from eight neonatal intensive care units (NICUs) were shown the video recordings with additional relevant clinical data, excluding EEG findings. The observers were asked to indicate which movements they considered to be seizure or nonseizure. A multirater Kappa statistic was used to assess agreement between observers and with the true diagnosis. Results:, Twenty video clips (11 seizure, 9 nonseizure) were evaluated by 91 doctors and 46 other professionals. The average number of correctly identified events was 10/20. Clonic seizures were correctly identified most frequently (range 36.5,95.6% of observers). Subtle seizures were poorly identified (range 20.4,49.6% of observers). The interobserver agreement (Kappa) for doctors and other health care professionals was poor at 0.21 and 0.29, respectively. Agreement with the correct diagnosis was also poor at 0.09 for doctors and ,0.02 for other healthcare professionals. Discussion:, It is often impossible to accurately differentiate between seizure-related and nonseizure movements in infants using clinical evaluation alone. In addition, doctors do not have a higher capacity for discriminating between neonatal paroxysmal events than other health care professionals. Until reliable continuous neurologic monitoring of newborn babies is available, it is likely that some babies with seizures will remain undetected and others with nonseizure movements will continue to be treated with potentially harmful anticonvulsants. [source] Seizures and paroxysmal events: symptoms pointing to the diagnosis of pyridoxine-dependent epilepsy and pyridoxine phosphate oxidase deficiencyDEVELOPMENTAL MEDICINE & CHILD NEUROLOGY, Issue 7 2010BERNHARD SCHMITT Aim, We report on seizures, paroxysmal events, and electroencephalogram (EEG) findings in four female infants with pyridoxine-dependent epilepsy (PDE) and in one female with pyridoxine phosphate oxidase deficiency (PNPO). Method, Videos and EEGs were analysed and compared with videos of seizures and paroxysmal events archived from 140 neonates. PDE and PNPO were proven by complete control of seizures once pyridoxine or pyridoxal 5,-phosphate was administered and by recurrence when withdrawn. Mutations in the antiquitin gene were found in three patients and in the PNPO gene in one child. Results, Seizures began within 48 hours after birth in four newborns and at age 3 weeks in one. Frequent multifocal and generalized myoclonic jerks, often intermixed with tonic symptoms, abnormal eye movement, grimacing, or irritability, were observed in all infants with PDE and PNPO, but rarely in the other archived videos of neonates. EEGs were inconstant and frequently no discernable ictal changes were recorded during the seizures and the paroxysmal events. In addition, interictal EEGs were inconclusive, with normal and abnormal recordings. In older children tonic,clonic seizures, abnormal behaviour, inconsolable crying, frightened facial expression, sleep disturbance, loss of consciousness, paraesthesia, or intermittent visual symptoms were described during controlled and uncontrolled withdrawal or insufficient dosage. Interpretation, PDE or PNPO should be considered in infants with prolonged episodes of mixed multifocal myoclonic tonic symptoms, notably when associated with grimacing and abnormal eye movements. [source] Outcome of vagus nerve stimulation for epilepsy in BudapestEPILEPSIA, Issue 2010Katalin Müller Summary Vagus nerve stimulation (VNS) is a nonpharmacologic therapeutic option for patients with intractable epilepsy. Better clinical outcomes were recorded in nonfocal and Lennox-Gastaut syndrome (LGS). We conducted a 2-year, open label, prospective study to measure the seizure outcome of 26 VNS patients. The seizure numbers were assessed using clinician's global impression scale (CGI) and patient diaries. The average seizure reduction was 23% at the first year and 22% at the second year. Seizure reduction was more pronounced among patients with nonfocal than with focal epilepsy. The response rate was 50% at first year and 30% at the second year. The best CGI record for clinically significant improvement was 15% in the LGS group. The only statistically significant result was the reduction of the generalized tonic,clonic seizures (GTCS). The side-effect profile was good; however, the large number of mild and reversible effects influenced the stimulation parameters and thus probably the effectiveness of the therapy. We suggest that VNS is an optional treatment mostly in cases of therapy-resistant Lennox-Gastaut syndrome. Patients with GTCS may experience improvement such as reduction of seizure severity. We conclude that VNS is a safe neuromodulatory treatment, but future developments of neuromodulatory approaches are needed. [source] Hemiconvulsion,hemiplegia syndrome in a patient with severe myoclonic epilepsy in infancyEPILEPSIA, Issue 9 2009Takafumi Sakakibara Summary We report a 2-year-old girl who had repeated febrile or afebrile seizures since infancy. Prolonged left/right hemiconvulsions and myoclonus of the eyelids/extremities with generalization to tonic,clonic seizures, were refractory to antiepileptic agents. At age 1 year and 4 months, she contracted rotavirus infection, and developed status epilepticus with persistent right hemiclonic seizures. Left unilateral brain edema with subsequent emergence of cortical laminar necrosis and white matter lesions, and progressive atrophy of the left cerebral hemisphere were noted during this period. She showed residual right hemiparesis and mild intellectual disability, and had generalized/eyelid myoclonia and hot water epilepsy after a 5-month seizure-free period. Analysis for SCN1A, the gene encoding the neuronal voltage-gated Na+ channel ,1 subunit revealed a nonsense mutation, R1892X. These indicate the potential risk in patients with severe myoclonic epilepsy in infancy (SMEI) to develop hemiconvulsion,hemiplegia (HH) syndrome. SCN1A mutations may need to be further explored in patients with HH syndrome without features of SMEI. [source] Seizures Lead to Elevation of Intracranial Pressure in Children Undergoing Invasive EEG MonitoringEPILEPSIA, Issue 6 2007Aash K Shah Summary:,Purpose: To study the effects of intracranial subdural grid electrode placement and seizures on intracranial pressure (ICP) in children undergoing invasive EEG monitoring. Methods: Sixteen children with pharmacoresistant epilepsy who underwent two-stage epilepsy surgery with subdural grid placement were included in the study. The ICP was recorded at baseline and with each seizure prospectively. A variety of seizure parameters including type of seizure, length of seizure, extent of seizure spread, and number of subdural grid electrodes inserted were analyzed retrospectively and correlated with the change in ICP. Results: A total of 48 seizures in 16 children were studied. The mean baseline ICP correlated positively with age of the child. Generalized tonic,clonic seizures were associated with the highest rise in ICP. Similarly, ICP rise was associated with seizures involving more electrodes indicating a larger area of brain participating in the seizure. Conclusion: Seizures in general and generalized tonic,clonic seizures, in particular, increase ICP temporarily in patients who are undergoing invasive EEG monitoring with subdural grids. [source] Intrinsic Ictal Dynamics at the Seizure Focus: Effects of Secondary Generalization Revealed by Complexity MeasuresEPILEPSIA, Issue 2 2007Christophe C. Jouny Summary:,Purpose: Partial seizures (PSs) may be self-limited regional events or propagate further and secondarily generalize. The mechanisms and dynamics of secondarily generalized tonic,clonic seizures (GTCSs) are not well understood. Methods with which to assess the dynamic of those events are also limited. Methods: Seizures were analyzed from patients with intractable partial seizures undergoing monitoring with intracranial electrodes. Inclusion in this study required patients to have at least one PS and one GTCS. From >120 patients, seven patients fulfilled these criteria, three with mesial temporal (MTLE) onset seizures and four with neocortical lesional (NCLE) onset seizures. In total, 50 seizures were analyzed by using the matching pursuit (MP) method and the Gabor atom density (GAD), a measure of signal complexity derived from the MP method. Results: The GAD complexity pattern at the seizure focus for the initial ictal period is remarkably consistent in a given patient, regardless of whether secondary generalization occurs. Secondary generalization produces greater modification of seizure activity at the focus in patients with NCLE than in patients with MTLE. In seizures from four patients with NCLE, secondary generalization resulted in an average increase of 115% in complexity at the focus compared to PSs. Conclusions: GAD shows that seizure dynamics of PSs are often very stereotyped from seizure to seizure in a given patient, particularly during early ictal evolution. Secondary generalization is more likely to produce changes in the duration and dynamics at the seizure focus in NCLE patients compared with MTLE patients. These observations suggest distinct mechanisms (e.g., feedback) that are operational during secondary generalization. [source] Altered Tryptophan Metabolism in the Brain of Cystatin B -Deficient Mice: A Model System for Progressive Myoclonus EpilepsyEPILEPSIA, Issue 10 2006Annika Vaarmann Summary:,Purpose: Progressive myoclonus epilepsy of the Unverricht,Lundborg type (EPM1) is a rare neurologic disorder, associated with mutations in the Cystatin B (Cstb) gene. Mice lacking Cstb, a cysteine protease inhibitor of the cathepsine family of proteases, provide a mammalian model for EPM1 by displaying similarly progressive ataxia, myoclonic seizures, and neurodegeneration. However, the linkage of Cstb deficit on the molecular level to pathologic features like myoclonic jerks or tonic,clonic seizures has remained unclear. We examined the tryptophan (TRP) metabolism, along the serotonin (5HT) and kynurenine (KYN) pathway in the brain of Cstb -deficient mice, in relation to their possible involvement in the seizure phenotype. Methods: TRP and its metabolites, along the 5HT and KYN pathways, were assayed in brain tissue by high-pressure liquid chromatography (HPLC) with electrochemical detection. The inverted wire grid and mild handling tests were used for evaluation of ataxia and myoclonic activity. Results: The Cstb -deficient mice had constitutively increased TRP, 5HT, and 5-hydroxyindole acetic acid (5HIAA) levels in the cerebral cortex and cerebellum and increased levels of KYN in the cerebellum. These neurochemical changes were accompanied with ataxia and an apparent myoclonic phenotype among the Cstb -deficient mice. Conclusions: Our findings suggest that secondary processes (i.e., overstimulation of serotoninergic transmission) on the cellular level, initiated by Cstb deficiency in specific brain regions, may be responsible for the myoclonic/seizure phenotype in EPM1. [source] Refractory Generalized Seizures: Response to Corpus Callosotomy and Vagal Nerve StimulationEPILEPSIA, Issue 1 2006Maromi Nei Summary:,Purpose: The vagal nerve stimulator (VNS) and corpus callosotomy can reduce seizure frequency when seizures are refractory to medications. However, the efficacy and safety of these two procedures have not been compared. This study evaluates the two procedures for generalized seizures. Methods: All patients with refractory generalized seizures (generalized tonic,clonic, tonic, or atonic) who underwent a corpus callosotomy (anterior or complete) (n = 53) without other forms of epilepsy surgery and those who underwent VNS placement (n = 25) were evaluated for this study. Seizure response and procedure complications were evaluated. Results: For those with a corpus callosotomy and generalized tonic,clonic seizures (n = 50), 79.5% had ,50% decrease in the frequency of generalized tonic,clonic seizures, and 60% had ,80% seizure reduction. For those with a VNS and generalized tonic,clonic seizures (n = 21), 50% had ,50% seizure reduction, and 33% had ,80% seizure reduction. Tonic and atonic seizures decreased after either VNS or a corpus callosotomy. The complication rate for corpus callosotomy was higher (21% all complications, 3.8% permanent) than that for VNS (8%; none permanent), but complications for both corpus callosotomy and VNS were rarely permanent. Conclusions: Both corpus callosotomy and VNS are effective in reducing generalized seizures. Corpus callosotomy is associated with greater efficacy but higher risk for complications, although these were generally transient. [source] Levetiracetam in the Treatment of Idiopathic Generalized EpilepsiesEPILEPSIA, Issue 2005Richard Grünewald Summary:, Since its introduction into clinical practice in 1999, levetiracetam, the S enantiomer of piracetam, has rapidly found a secure place, initially in the therapy of partial onset seizures and subsequently in the treatment of idiopathic generalized epilepsies (IGE). It has many of the properties of an "ideal" antiepileptic drug, including rapid absorption, linear pharmokinetics, and sparse drug interactions. Tolerabiliy is generally excellent in both adults and children, although tiredness is a common dose-limiting adverse effect. Occasionally the drug can precipitate behavioral abnormalities, especially in patients with learning disability. There is a wide safety margin in overdose. In common with most antiepileptic drugs its mode of action remains uncertain. Levetiracetam binds to a specific site in the brain, influences intracellular calcium currents and reverses negative allosteric modulators of GABA- and glycine-gated currents in vitro. Its effectiveness has been demonstrated in animal models of epilepsy and in clinical trials of partial onset and IGE. Treatment of IGEs may be straightforward, with many patients demonstrating an excellent and robust response to valproate monotherapy. However, there remains a significant minority of patients for whom valproate is unsuitable, including those who experience unacceptable adverse effects (e.g., weight gain or hair loss) and women of childbearing age in whom the teratogenic potential of valproate is unacceptable. Therapeutic response to lamotrigine in this group is often disappointing, and many clinicians now are turning to the choice of levetiracetam. Efficacy in generalized tonic,clonic seizures and myoclonus is usually apparent and some patients experience improvement in typical absences. Experience of combinations of levetiracetam with other antiepileptic drugs is limited in IGE and the responses are largely anecdotal. In our hands, patients with refractory IGEs may respond to combinations of levetiracetam with valproate, lamotrigine, and phenobarbital, and adverse effects when they occur are usually limited to tiredness. Levetiracetam does not interact with the oral contraceptive pill, simplifying treatment in women of childbearing age. Although animal data look encouraging, questions over levetiracetam's teratogenic potential and overall safety in pregnancy will remain for many years to come. [source] Focal Semiologic and Electroencephalographic Features in Patients with Juvenile Myoclonic EpilepsyEPILEPSIA, Issue 10 2005Naotaka Usui Summary:,Purpose: A few reports have described focal electroencephalographic or clinical features or both of juvenile myoclonic epilepsy (JME), but without video-EEG documentation. We examined focal clinical and EEG features in patients with JME who underwent video-EEG monitoring. Methods: Twenty-six patients (nine males and 17 females) who had seizures recorded during video-EEG monitoring were included. Age at seizure onset was 0 to 22 years (mean, 12.3 years), and age at monitoring was 12 to 44 years (mean, 26.5 years). In one patient with left parietooccipital epilepsy, primary generalized tonic,clonic seizures developed after resection of the parietal tumor. Two patients had both temporal lobe epilepsy and JME. Videotaped seizures in each patient were analyzed. Interictal and ictal EEG also were analyzed for any focal features. Results: Focal semiologic features were observed in 12 (46%) of 26 patients. Six patients had focal myoclonic seizures, and two had Figure 4 sign: one with version to the left, and another had left version followed by Figure 4 sign, and left arm clonic seizure. Their ictal EEGs were generalized at onset but with a lateralized evolution over the right hemisphere. The patient who had both JME and left parietooccipital epilepsy, right arm clonic seizure, and Figure 4 sign was seen during a generalized EEG seizure. Interictally, one patient had temporal sharp waves, and another had run of spikes in the right frontal region. Conclusions: Fourteen (54%) of 26 patients with JME exhibited focal semiologic or electroencephalographic features or both. Video-EEG was essential in reaching a correct diagnosis and choosing an appropriate antiepileptic drug regimen. [source] Genetic Architecture of Idiopathic Generalized Epilepsy: Clinical Genetic Analysis of 55 Multiplex FamiliesEPILEPSIA, Issue 5 2004Carla Marini Summary: Purpose: In families with idiopathic generalized epilepsy (IGE), multiple IGE subsyndromes may occur. We performed a genetic study of IGE families to clarify the genetic relation of the IGE subsyndromes and to improve understanding of the mode(s) of inheritance. Methods: Clinical and genealogic data were obtained on probands with IGE and family members with a history of seizures. Families were grouped according to the probands' IGE subsyndrome: childhood absence epilepsy (CAE), juvenile absence epilepsy (JAE), juvenile myoclonic epilepsy (JME), and IGE with tonic,clonic seizures only (IGE-TCS). The subsyndromes in the relatives were analyzed. Mutations in genes encoding ,1 and ,2 ,-aminobutyric acid (GABA)-receptor subunits, ,1 and ,1 sodium channel subunits, and the chloride channel CLC-2 were sought. Results: Fifty-five families were studied. 122 (13%) of 937 first- and second-degree relatives had seizures. Phenotypic concordance within families of CAE and JME probands was 28 and 27%, respectively. JAE and IGE-TCS families had a much lower concordance (10 and 13%), and in the JAE group, 31% of relatives had CAE. JME was rare among affected relatives of CAE and JAE probands and vice versa. Mothers were more frequently affected than fathers. No GABA-receptor or sodium or chloride channel gene mutations were identified. Conclusions: The clinical genetic analysis of this set of families suggests that CAE and JAE share a close genetic relation, whereas JME is a more distinct entity. Febrile seizures and epilepsy with unclassified tonic,clonic seizures were frequent in affected relatives of all IGE individuals, perhaps representing a nonspecific susceptibility to seizures. A maternal effect also was seen. Our findings are consistent with an oligogenic model of inheritance. [source] A Detailed Analysis of Symptomatic Posterior Cortex Seizure Semiology in Children Younger Than Seven YearsEPILEPSIA, Issue 1 2003András Fogarasi Summary: ,Purpose: To analyze the semiology of seizure onset and evolution in young children with posterior cortex epilepsy (PCE), compare this with adult reports, and assess age-related differences. Methods: We videotaped and analyzed 110 seizures from 18 patients with PCE, aged 3,81 months. All had a good prognosis after posterior epileptogenic zone removal. Ictal events were categorized by behavioral, consciousness, autonomic, and sensory features, as well as motor patterns, which included myoclonic, tonic, clonic, unclassified motor seizures, and epileptic spasm. A time-scaled data sheet was developed to record each epileptic event as onset, very early, early, or late manifestation. Results: Patients had a high seizure frequency with ,100 attacks/day; one third of them showed a cluster tendency. The mean duration of seizures was 67 s. The most common seizure components were motor manifestations (with myoclonic and tonic seizures), but psychomotor (automotor), hypomotor attacks, and isolated auras also were frequently observed. Clinical seizure spread was frequent; auras and visual sensory signs were difficult to record in this age. Typical phenomena during seizures included behavioral changes, ictal vocalization, smile, flush, head nod, oculomotor features, and late-appearing oral automatisms, whereas hypermotor and secondarily generalized tonic,clonic seizures were not seen. Conclusions: Our results suggest that PCE in infants and young children is very heterogeneous but shows important age-related features. Compared with adults, children with PCE have shorter but more frequent seizures; they rarely report aura or visual sensory signs, only sporadically develop hypermotor and secondarily generalized tonic,clonic seizures, whereas ictal smile, flush, head nod, and behavioral change are typical features at this age. Because of frequent subtle ictal phenomena, long-term video-EEG monitoring is a useful diagnostic tool with infants and young children with PCE. [source] Serum S-100 Protein Is Not a Suitable Seizure Marker in Temporal Lobe EpilepsyEPILEPSIA, Issue 10 2002Fritz Leutmezer Summary: ,Purpose: S-100 protein is a sensitive marker of various brain diseases; however, its role in epilepsy is controversially discussed in the literature. We therefore studied the temporal profile of serial concentrations of S-100 protein in serum after secondarily generalized tonic,clonic seizures during video-EEG monitoring. Methods: Ten patients with mesial temporal lobe epilepsy were prospectively studied. Serum S-100 protein was measured after a seizure-free period of ,24 h (baseline) and 30 min, 3, 6, 12, and 24 h after a secondarily generalized tonic,clonic seizure of temporal lobe origin in nine and a convulsive status epilepticus in one patient. Results: All S-100 levels were within the normal range, except for those of one patient at baseline. Mean values were 0.045 ,g/L (range, 0.003,0.13 ,g/L) at baseline, 0.038 ,g/L (range, 0.003,0.09 ,g/L) at 30 min, 0.036 ,g/L (range, 0.003,0.08 ,g/L) at 3 h, 0.034 ,g/L (range, 0.003,0.07 ,g/L) at 6 h, 0.034 ,g/L (range, 0.003,0.08 ,g/L) at 12 h, and 0.035 ,g/L (range, 0.003,0.09 ,g/L) at 24 h after seizure offset. There were no significant differences between mean concentrations at any interval postictally. Conclusions: We could not detect any significant alterations in serum S-100 protein concentration either after a single secondarily generalized tonic,clonic seizure or after convulsive status epilepticus in patients with temporal lobe epilepsy. Our data do not confirm previous work, which suggested serum S-100 protein to be a suitable marker for epileptic seizures. [source] Seizure Suppression by Adenosine-releasing Cells Is Independent of Seizure FrequencyEPILEPSIA, Issue 8 2002Detlev Boison Summary: ,Purpose: Intraventricular cellular delivery of adenosine was recently shown to be transiently efficient in the suppression of seizure activity in the rat kindling model of epilepsy. We tested whether the suppression of seizures by adenosine-releasing grafts was independent of seizure frequency. Methods: Adenosine-releasing cells were encapsulated and grafted into the lateral brain ventricle of rats kindled in the hippocampus. During 4 weeks after grafting, electric test stimulations were delivered at a frequency of either once a week or 3 times per week. Seizure activity was evaluated by visual scoring of seizure severity and by the recording of EEGs. Results: Adenosine released from encapsulated cells exerted potent antiepileptic activity for ,2 weeks. One week after grafting, treated rats displayed a complete protection from clonic seizures, and a protection from focal seizures was observed in the majority of animals. Seizure suppression was accompanied by a reduction of afterdischarges in EEG recordings. The protective efficacy of the grafted cells was the same irrespective of whether electrical test stimulations were delivered 1 or 3 times per week. Rats receiving control grafts continued to display full clonic convulsions. Conclusions: This study demonstrated that the frequency of test stimulations did not influence the seizure-suppressive potential of adenosine-releasing grafts. Thus the local delivery of adenosine is likely to be effective in seizure control over a threefold range of seizure-discharge frequency. [source] Nonsymptomatic Generalized Epilepsy in Children Younger than Six Years: Excellent Prognosis, but Classification Should Be Reconsidered after Follow-up: The Dutch Study of Epilepsy in ChildhoodEPILEPSIA, Issue 7 2002C. M. Middeldorp Summary: ,Purpose: To assess the prognosis and the accuracy of the epilepsy classification in young children with nonsymptomatic generalized epilepsy. Methods: Of the cohort of the Dutch Study of Epilepsy in Childhood (n = 466), all children younger than 6 years with a diagnosis of idiopathic (IGE) or cryptogenic (CGE) generalized epilepsy either at intake (n = 108) and/or after 2 years of follow-up (n = 102) were included. The number of reclassifications after 2 years was determined, and the reasons for reclassification were analyzed. All children receiving a diagnosis of IGE or CGE at 2 years were followed up for 5 years to study their outcome in terms of terminal remission (TR). Data on their level of intellectual functioning were collected at the start of this analysis. Results: The epilepsy syndrome was reclassified in 17 children. In 14 of them, the seizure type also was reclassified, and in three, the course of the epilepsy determined the new epilepsy type. Two other children had a reclassification of their seizure types without a change of the epilepsy type. Many children were categorized as having IGE not otherwise specified. In all probability, this is a heterogeneous group, containing patients with various epilepsy syndromes, with generalized tonic,clonic seizures as a common hallmark. Of the 102 children with IGE or CGE at 2 years of follow-up, 75% had a TR of >6 months after 2 years, and 85% a TR of ,1 year after 5 years. Conclusions: In a fair proportion of children with nonsymptomatic generalized epilepsy in this age group, it is not possible to classify firmly the epilepsy and/or the seizures immediately after the intake. Instead, they are reclassified during the course of the disease. This and the apparent heterogeneity of the category IGE not otherwise specified point to inherent drawbacks of the current International League Against Epilepsy (ILAE) classification of epilepsy and epileptic syndromes. The prognosis of IGE at this young age is generally excellent. [source] Clinical and Electrographic Features of Epileptic Spasms Persisting Beyond the Second Year of LifeEPILEPSIA, Issue 6 2002Márcio A. Sotero De Menezes Summary: ,Purpose: Few reports detailing the electroclinical features of epileptic spasms persisting beyond infancy have been published. We sought to characterize this unique population further. Methods: We retrospectively reviewed the clinical and video-EEG data on 26 patients (4,17 years; mean, 93 months) with a confirmed diagnosis of epileptic spasms and who were evaluated at our tertiary referral center between 1993 and 2000. Results: In half of our cases, epileptic spasms were associated with disorders of neuronal migration, severe perinatal asphyxia, and genetic anomalies. Interictal EEGs showed generalized slowing in the majority of patients, and a slow-wave transient followed by an attenuation of the background amplitude was the most common ictal EEG pattern associated with an epileptic spasm (19 cases). Other seizure types (number of cases in parentheses) included tonic seizures with or without a preceding spasm (13), partial seizures (11), myoclonic seizures (11), generalized tonic,clonic seizures (six), atypical absence seizures (two), and atonic seizures (one). Cases with a more organized EEG background (especially with frequencies ,7 Hz) were more likely to have better cognition. Continued disorganization of the EEG background and persistence of hypsarrhythmia were associated with poor developmental outcome. Conclusions: Patients with epileptic spasms persisting beyond age 2 years constitute a truly refractory population, one that should be better recognized by clinicians. Interestingly, although many therapies resulted in a >50% reduction in seizures, neither neurocognitive function nor quality of life was substantially improved with intervention. The interictal EEG background is the most helpful in predicting neurologic outcome. [source] Cardiac Dysrhythmia Associated with the Immediate Postictal State after Maximal Electroshock in Freely Moving RatEPILEPSIA, Issue 4 2002Olivier Darbin Summary: ,Purpose: Cardiac autonomic changes accompany complex partial seizures and generalized tonic,clonic seizures, and participate, at least partially, in the sudden and unexpected death in epilepsy (SUDEP). The analysis of the heart rate variability (HRV) is one of the simplest ways of providing insight into autonomic functions. The entropy quantifies the repetition of complex patterns in a signal and refers to systems randomness, regularity, and predictability. Clinical investigations have reported that entropy decreases in patients with a high risk of sudden cardiac death. The goal of this study was to evaluate the effects of the maximal electroshock (MES) on the entropy of HRV, monitored in the immediate postictal stage in the model of the freely moving rat. Methods: Entropy changes were correlated with the high and low frequencies of spectral analysis, which reflect the participation of the sympathetic and parasympathetic activities. Results: MES-induced arrhythmia is characterized by an HRV increase, an imbalance in favor of the parasympathetic activity, and a decrease in the entropy. Entropy decrease was restricted to the duration of the arrhythmia, suggesting that the postictal arrhythmia may be associated with a higher risk of lethal cardiac complications. Nevertheless, entropy changes did not correlate with spectral changes. Conclusions: The results suggest that the imbalance demonstrated in the spectral domain explains only partially the contribution of each autonomic system in the complexity of the heart rate during the postictal state. [source] Epileptic Seizures after Treatment with ThiocolchicosideEPILEPSIA, Issue 8 2001Pier Luigi De Riu Summary: ,Purpose: To report the occurrence of epileptic seizures in humans, closely related to the use of the centrally acting muscle relaxant thiocolchicoside. Methods: Description of three case histories. Results: Two patients, affected with complex-partial seizures, sometimes secondarily generalized, receiving antiepileptic therapy, were seizure free for 7 and 9 years, respectively. They had the reappearance of tonic,clonic seizures few days after the continued use of thiocolchicoside, at a cumulative dose of the drug of 52 mg and 76 mg, respectively. The third patient was brain damaged and without a history of seizures. He had a sudden, convulsive seizure a few minutes after 4 mg intramuscular thiocolchicoside. Conclusions: Our case histories indicate that thiocolchicoside has a powerful epileptogenic activity. This drug should be avoided in patients with epilepsy or acute brain injury and possible disruption of the blood,brain barrier. [source] Enhanced Anticonvulsant Activity of Neuroactive Steroids in a Rat Model of Catamenial EpilepsyEPILEPSIA, Issue 3 2001Doodipala S. Reddy Summary: ,Purpose: Perimenstrual catamenial epilepsy may in part be due to withdrawal of the endogenous progesterone-derived neurosteroid allopregnanolone that potentiates ,-aminobutyric acidA (GABAA) receptor,mediated inhibition. Here we sought to determine whether the anticonvulsant potencies of neuroactive steroids, benzodiazepines, phenobarbital (PB), and valproate (VPA) are altered during the heightened seizure susceptibility accompanying neurosteroid withdrawal in a rat model of perimenstrual catamenial epilepsy. Methods: Test drugs were evaluated for their ability to alter the convulsant activity of pentylenetetrazol (PTZ) in young adult female rats, in pseudopregnant rats with prolonged exposure to high levels of progesterone (and its neurosteroid metabolites), and in pseudopregnant rats 24 h after acute withdrawal of neurosteroids by treatment with the 5,-reductase inhibitor finasteride. Test drugs were administered at doses equivalent to twice their ED50 values for protection against PTZ-induced clonic seizures in naive young adult female rats. Results: The anticonvulsant activity of allopregnanolone (5 mg/kg, s.c.), pregnanolone (5 mg/kg, s.c.), allotetrahydrodeoxycorticosterone (15 mg/kg, s.c.), and tetrahydrodeoxycorticosterone (10 mg/kg, s.c.) were enhanced by 34,127% after neurosteroid withdrawal. The anticonvulsant activity of PB (65 mg/kg, i.p.) was also enhanced by 24% in neurosteroid-withdrawn animals. In contrast, the anticonvulsant activity of diazepam (4 mg/kg, i.p.), bretazenil (0.106 mg/kg, i.p.), and VPA (560 mg/kg, i.p.) were reduced or unchanged in neurosteroid-withdrawn animals. Conclusions: The anticonvulsant activity of neuroactive steroids is potentiated after neurosteroid withdrawal, supporting the use of such agents in the treatment of perimenstrual catamenial epilepsy. [source] Anticonvulsant Action of Topiramate Against Motor Seizures in Developing RatsEPILEPSIA, Issue 10 2000Renata Haugvicová Summary Purpose: To study the anticonvulsant action of topiramate (TPM) in developing rats. Methods: Motor seizures were elicited by administering pentylenetetrazol (100 mg/kg subcutaneously) in five age groups of Wistar rats (7, 12, 18, 25, and 90 days old). TPM was administered intraperitoneally in doses from 10 to 640 mg/kg 2 hours before pentylenetetrazol. The time course of TPM action was studied in 12- and 25-day-oId rats up to 24 hours after the 160-mg/kg dose, and the incidence and pattern of seizures were evaluated. Results: TPM did not influence minimal seizures (clonus of forelimb and head muscles with preserved righting ability). Generalized tonic-clonic seizures, however, were reliably changed at all developmental stages studied. The tonic phase was suppressed so that the majority of animals exhibited generalized clonic seizures (with a loss of righting reflexes). In addition, the incidence of generalized seizures was decreased after the 20-, 40-, and 80-mg/kg doses in the 7-day-old rat pups. The specific suppression of the tonic phase of generalized seizures was observed up to 12 hours in the 12-day-old rat pups. The same result was obtained over 6 hours after TPM administration in the 25-day-old animals, and with longer intervals the incidence of generalized seizures decreased in this age group. Conclusions: TPM exhibits stable anticonvulsant action against the tonic phase of generalized tonic-clonic seizures throughout development. In addition, it suppresses all phases of generalized seizures in 7-day-old rats. The anticonvulsant action of TPM lasted longer in 25-day-old than in 12-day-old rats. The two actions of TPM might be ascribed to two different mechanisms of action. [source] Effect of Ganaxolone on Flurothyl Seizures in Developing RatsEPILEPSIA, Issue 7 2000a Liptáková Summary: Purpose: To determine the effects of a newly synthesized epalon, ganaxolone (GNX), on primarily generalized seizures in rats of various ages during development. Epalons are classified as neuroactive steroids that interact at unique site of the GABAA receptor-Cl, channel complex in the central nervous system. Methods: Sprague-Dawley male rats were used at 9, 15, 30, and 60 postnatal days (PN). GNX dissolved in 2-hydroxypropyl-,-cyclodextrine was administered intraperitoneally in different doses at various time points before flurothyl testing. The incidence and threshold of clonic and tonic-clonic flurothyl seizures were evaluated. Behavioral changes were also assessed. Results: In all age groups, the effects of GNX were dose dependent and more prominent 10 min after its administration. In PN 60 and PN 30 rats, GNX had dose-dependent anticon-vulsant effects; tonic-clonic seizures were more sensitive to GNX treatment than clonic seizures. In PN 15 and PN 9 rats, GNX demonstrated dose- and time-dependent anticonvulsant effects against both types of flurothyl-induced seizures. GNX was more effective in PN 15 rats than in other age groups, but at doses that altered motor behavior. Conclusions: GNX has anticonvulsant effects against flurothyl-induced seizures in all age groups tested. Its effects are more prominent in the two younger age groups, especially in PN 15 rats, but are associated with motor side effects. [source] Role of Nitric Oxide in Pentylenetetrazol-Induced Seizures: Age-Dependent Effects in the Immature RatEPILEPSIA, Issue 4 2000Anne Pereira de Vasconcelos Summary: Purpose: Seizure susceptibility and consequences are highly age dependent. To understand the pathophysiologic mechanisms involved in seizures and their consequences during development, we investigated the role of nitric oxide (NO) in severe pentylenetetrazol (PTZ)-induced seizures in immature rats. Methods: Four cortical electrodes were implanted in 10-day-old (P10) and 21-day-old (P21) rats, and seizures were induced on the following day by repetitive injections of subconvulsive doses of PTZ. The effects of NG -nitro- l -arginine methyl ester (l -NAME; 10 mg/kg) and 7-nitroindazole (7NI; 40 mg/kg), two NO synthase (NOS) inhibitors, and l -arginine (l -arg; 300 mg/kg), the NOS substrate, were evaluated regarding the mean PTZ dose, seizure type and duration, and mortality rate. Results: At P10, the postseizure mortality rate increased from 18,29% for the rats receiving PTZ only to 100% and 89% for the rats receiving l -NAME and 7NI, respectively; whereas l -arg had no effect. Conversely, at P21, NOS inhibitors did not affect the 82,89% mortality rate induced by PTZ alone, whereas l -arg decreased the mortality rate to 29%. In addition, all NO-related drugs increased the duration of ictal activity at P10, whereas at P21, L -arg and L -NAME affected the first seizure type, producing clonic seizures with L -arg and tonic seizures with L -NAME. Conclusions: The relative natural protection of very immature rats (P10) against PTZ-induced deaths could be linked to a high availability of L -arg and, hence, endogenous NO. At P21, the modulation of seizure type by NO-related compounds may be related to the maturation of the brain circuitry, in particular the forebrain, which is involved in the expression of clonic seizures. [source] Monitoring of CBFV and time characteristics of oxygen-induced acute CNS toxicity in humansEUROPEAN JOURNAL OF NEUROLOGY, Issue 7 2008A. E. Koch Background:, Hyperbaric oxygen can cause central nervous system (CNS) toxicity with seizures. We tested the hypothesis that CNS toxicity could be predictable by cerebral blood flow velocity (CBFV) monitoring. Method:, We monitored 369 mandatory oxygen tolerance tests (30 min, 280 kPa O2) by video-documentation and since May 2005 by additional CBFV registration (n = 61). Results:, The onset of early manifestations of CNS toxicity was documented in 11 of 369 tests within 22 ± 3 min. These included twitches and/or agitation, 6 of 11 and tonic,clonic seizures in 5 of 11 cases. In both cases with CBFV monitoring, an increase in CBFV preceded symptom onset, once followed by seizure, once without seizure after timely oxygen reduction. Conclusions:, During exposure to 280 kPa oxygen at rest a constant delay of approximately 20 min precedes the onset of central nervous oxygen toxicity. An increase in CBFV may indicate the impending seizure. [source] Naturally occurring fatal herpes simplex virus 1 infection in a family of white-faced saki monkeys (Pithecia pithecia pithecia)JOURNAL OF MEDICAL PRIMATOLOGY, Issue 1 2003M.D. Schrenzel Abstract: A family of three white-faced saki monkeys (Pithecia pithecia pithecia) died 48,96 hours after the onset of anorexia, nasal discharge, pyrexia and oral ulceration. One animal also had clonic seizures. Lesions found post-mortem consisted of oral and esophageal ulcers, hepatic and intestinal necrosis, meningoencephalitis and sporadic neuronal necrosis. Intranuclear inclusion bodies and syncytial cells were present in oral lesions and affected areas of liver. Herpes simplex virus 1 (HSV-1) was identified as the etiology of disease by virus isolation, polymerase chain reaction, or in situ hybridization in all three animals. Immunohistochemistry for detection of apoptotic DNA and activated caspase-3 showed significant levels of apoptosis in oral and liver lesions and occasional apoptotic neurons in the brain. These findings demonstrate the vulnerability of white-faced saki monkeys to HSV-1 and provide initial insight into the pathogenesis of fatal HSV-1-induced disease, indicating that apoptosis plays a significant role in cell death. [source] Oral health status in epileptic childrenPEDIATRICS INTERNATIONAL, Issue 2 2010Taskin Gurbuz Abstract Background:, The aim of the present study was to evaluate the oral hygiene status and dental treatment requirements in children with epilepsy. Methods:, The treatment group consisted of 211 children with epilepsy (120 boys and 91 girls, 4,15 years old, mean age 7.85 ± 2.98 years). The control group consisted of healthy children, matched by age and gender. Clinical features of the patients were obtained from hospital records. Clinical examinations were conducted, under standard light, using a plane buccal mirror, a dental probe and air drying to evaluate caries experience and to record the periodontal health of each child. Statistical analysis was performed using ,2 test, Fisher exact test and anova. Results:, The number of decayed and missing teeth, the degree of abrasion and periodontal indexes were significantly worse in patients with epilepsy, compared to the control group (P < 0.001). Gingival enlargement was documented in 42% of patients on valproate monotherapy compared to only in 16% of patients on phenobarbital. Dental caries and halitosis were the most common oral disorders. Generalized tonic,clonic seizures often cause minor oral injuries and traumatized anterior teeth. Conclusions:, Epileptic children are at an increased risk of developing caries and gingivitis compared with healthy subjects. [source] Anticonvulsant activities of nutmeg oil of Myristica fragransPHYTOTHERAPY RESEARCH, Issue 2 2009Abdul Wahab Abstract The purpose of this study was to investigate the anticonvulsant activity of the volatile oil of nutmeg, the dried seed kernel of Myristica fragrans Houtt, using well-established animal seizure models and to evaluate its potential for acute toxicity and acute neurotoxicity. The volatile oil of nutmeg (nutmeg oil) was tested for its effects in maximal electroshock, subcutaneous pentylenetetrazole, strychnine and bicuculline seizure tests. All the experiments were performed at the time of peak effect of nutmeg oil. Nutmeg oil showed a rapid onset of action and short duration of anticonvulsant effect. It was found to possess significant anticonvulsant activity against electroshock-induced hind limb tonic extension. It exhibited dose dependent anticonvulsant activity against pentylenetetrazole-induced tonic seizures. It delayed the onset of hind limb tonic extensor jerks induced by strychnine. It was anticonvulsant at lower doses, whereas weak proconvulsant at a higher dose against pentylenetetrazole and bicuculline induced clonic seizures. Nutmeg oil was found to possess wide therapeutic margin, as it did not induce motor impairment when tested up to 600 µL/kg in the inverted screen acute neurotoxicity test. Furthermore, the LD50 (2150 µL/kg) value was much higher than its anticonvulsant doses (50,300 µL/kg). The results indicate that nutmeg oil may be effective against grand mal and partial seizures, as it prevents seizure spread in a set of established animal models. Slight potentiation of clonic seizure activity limits its use for the treatment of myoclonic and absence seizures. Copyright © 2008 John Wiley & Sons, Ltd. [source] Afebrile benign convulsions with mild gastroenteritis: a new entity?ACTA NEUROLOGICA SCANDINAVICA, Issue 2 2009A. Verrotti Afebrile seizures in children usually necessitate investigations in order to determine the etiology and estimate the prognosis. Recently, convulsions that are described as benign but afebrile have been documented in children, in association with diarrhea, and are now recognized as a distinct entity. Benign afebrile seizures with mild gastroenteritis are defined as convulsions accompanying symptoms of mild diarrhea without dehydration or electrolyte derangement and without fever before and after the seizures in healthy children without meningitis, encephalitis or encephalopathy. The convulsions are short, symmetrical, generalized tonic,clonic seizures, occurring in clusters. Laboratory studies (full blood count, blood glucose, creatinine, serum electrolytes, cerebrospinal fluid, bacterial and viral cultures) are usually normal, and other investigations (neuroimaging and electroencephalogram) are not necessary. Prognosis is always favorable (normal psychomotor development, no recurrences of seizures), and anticonvulsant therapy is not warranted. Recognition of this benign infantile convulsion avoids extensive evaluation and long-term anticonvulsant therapy; physicians may reassure the parents regarding the lack of long-term sequelae. In conclusion, this type of seizure seems to be a new entity, but it awaits a correct place in the large group of infantile convulsion disorders. [source] Role of valproate across the ages.ACTA NEUROLOGICA SCANDINAVICA, Issue 2006Treatment of epilepsy in children In June 2005 a team of experts participated in a workshop with the objective of reaching agreement on the place of valproate use in the treatment of paediatric epilepsy patients. A general ,consensus of the meeting' was that the initiation of antiepileptic drug (AED) treatment should be based on a seizure-syndromic approach in children. Participants of the meeting also agreed that valproate is currently the AED with the broadest spectrum across all types of seizures and syndromes. Its superiority has been shown over almost 40 years of clinical experience. The best results are seen in idiopathic generalized epilepsy with or without photosensitivity, idiopathic focal and symptomatic generalized tonic,clonic seizures (GTCS). Evidence supports the use of valproate, ethosuximide and lamotrigine in absence epilepsies and the use of carbamazepine, lamotrigine, oxcarbazepine, phenytoin, topiramate, valproate and phenobarbital for primary GTCS. For new AEDs trials have been undertaken to define their therapeutic role but studies comparing their role to ,old' broad-spectrum drugs in specific syndromes are missing. Experts concluded that intravenous (i.v.) valproate is a useful agent in the treatment of non-convulsive status epilepticus (SE). There is an easy transition to oral treatment following i.v. valproate use. The discussion also concluded that, despite the lack of studies, valproate is an interesting, underutilized alternative in convulsive SE but more controlled studies are needed. The side effects of valproate use are well documented. Its effect on cognition and behaviour is more favourable than many of the other AEDs which is an important consideration in children. Overall, the clinical consensus of the meeting was that valproate's well established therapeutic properties far outweigh the negative side effects. Contraindication or withdrawal should be assessed individually. [source] Complex epileptic (Foix,Chavany,Marie like) syndrome in a child with neurofibromatosis type 1 (NF1) and bilateral (opercular and paracentral) polymicrogyriaACTA PAEDIATRICA, Issue 4 2009Mario Mastrangelo Abstract The association of brain malformations and symptomatic epilepsy in the setting of neurofibromatosis type 1 (NF1) is rarely reported. When it occurs, patients can present clinically with infantile spasms, focal seizures, generalized tonic clonic seizures or atypical absences. We report on a 10-year-old (molecularly proven) NF1 girl manifesting a complex epileptic syndrome resembling the Foix,Chavany,Marie spectrum (also known as opercular syndrome) associated with bilateral (opercular and paracentral lobular) polymicrogyria (PMG). Anecdotal cases of unilateral PMG in the setting of NF1 have been described in association with other-than-opercular epileptic syndromes. The typical clinical opercular syndrome consisting in mild mental retardation, epilepsy and pseudobulbar palsy is usually associated to bilateral perisylvian PMG (BPP) Conclusion: To the best of our knowledge, the complex epileptic syndrome hereby reported has not been previously recorded in the setting of NF1. In addition, the present girl manifested all the clinical features of an opercular syndrome but had an asymmetrical PMG (not a BPP). [source] Molecular and clinical consequences of novel mutations in the arylsulfatase A geneCLINICAL GENETICS, Issue 1 2009ugowska Metachromatic leukodystrophy (MLD), a severe neurodegenerative metabolic disorder, is caused by deficient activity of arylsulfatase A (ARSA; EC 3.1.6.8), which leads to a progressive demyelinating process in central and peripheral nervous systems. In this study, a DNA sequence analysis was performed on six Polish patients with different types of MLD. Six novel mutations were identified: one nonsense (p.R114X), three missense (p.G122C, p.G293C, p.C493F) and two frameshift mutations (g.445_446dupG and g.2590_2591dupC). Substitutions p.G293C and p.C493F and duplication g.445_446dupG caused a severe reduction of enzyme activity in transient transfection experiments on mammalian cells (less than 1% of wild-type (WT) ARSA activity). Duplication 2590_2591dupC preserved low-residual ARSA activity (10% of WT ARSA). In summary, the novel MLD-causing mutations in the exons 2, 5 and even in 8 of the ARSA gene described here can be classified as severe type 0, leading in homozygosity to the late infantile form MLD. Growth retardation, delayed motor development, gait disturbances, tonic,clonic seizures and non-epileptic muscle spasms were the first onset symptoms in patients with late infantile form of MLD. In individual with juvenile type MLD gait disturbances evidenced the onset of the disease, while in a patient with late juvenile MLD, difficulties at school were displayed. [source] | |||||||||||||||||||||||||