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ABSTRACT: This study aimed to identify the indications in which electroencephalography in the pediatric emergency department is most useful. We retrospectively reviewed the influence that the results of the emergent electroencephalogram had on the eventual disposition of patients at our pediatric emergency department. Sixty-eight children (mean age, 7.3 years; 32 males) underwent 70 emergent electroencephalograms. Fifty-seven emergent electroencephalograms were performed for the suspicion of ongoing seizures or status epilepticus. Thirteen of the 22 children (59.1%) discharged from the emergency department were sent home mainly based on the results of the emergent electroencephalogram, which prevented an admission. In particular, 11 of 38 children with frequent and recurrent paroxysmal events concerning for seizures and 2 of 19 children with suspected ongoing status epilepticus were discharged after excluding an epileptic disturbance. The emergent electroencephalogram provided meaningful clinical information that influenced disposition, especially in patients with ongoing events in which the clinical picture was clarified by a rapidly acquired electroencephalogram.
Journal of child neurology 04/2013; · 1.59 Impact Factor
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ABSTRACT: Guidance about how to practice child neurology has been around for decades. Recently, however, clinical practice guidelines, practice parameters, and standardized clinical assessment and management plans are gaining increasing attention. This overview, written for child neurologists, addresses such issues as the following: what are clinical practice guidelines, why are they needed, how are they created, how should they be created, how well are they accepted and adhered to, what influences acceptance and adherence, do guidelines improve care, do they reduce costs, will they be viewed by courts as the standard of care, how can they be updated and improved, and are there better alternatives?
Journal of child neurology 04/2013; · 1.59 Impact Factor
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ABSTRACT: Approximately one-third of patients with epilepsy continue to have seizures despite antiepileptic therapy. Many seizures occur in diurnal, sleep/wake, circadian, or even monthly patterns. The relationship between biomarkers and state changes is still being investigated, but early results suggest that some of these patterns may be related to endogenous circadian patterns whereas others may be related to wakefulness and sleep or both. Chronotherapy, the application of treatment at times of greatest seizure susceptibility, is a technique that may optimize seizure control in selected patients. It may be used in the form of differential dosing, as preparations designed to deliver sustained or pulsatile drug delivery or in the form of 'zeitgebers' that shift endogenous rhythms. Early trials in epilepsy suggest that chronopharmacology may provide improved seizure control compared with conventional treatment in some patients. The present article reviews chronopharmacology in the treatment of epilepsy as well as future treatment avenues.
Current Neurology and Neuroscience Reports 04/2013; 13(4):339. · 3.45 Impact Factor
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Sarah M Sanchez,
Jessica Carpenter,
Kevin E Chapman,
Dennis J Dlugos,
William B Gallentine,
Christopher C Giza,
Joshua L Goldstein,
Cecil D Hahn,
Sudha K Kessler, Tobias Loddenkemper,
James J Riviello,
Nicholas S Abend
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ABSTRACT: PURPOSE:: To describe current continuous EEG monitoring (cEEG) utilization in critically ill children. METHODS:: An online survey of pediatric neurologists from 50 US and 11 Canadian institutions was conducted in August 2011. RESULTS:: Responses were received from 58 of 61 (95%) surveyed institutions. Common cEEG indications are altered mental status after a seizure or status epilepticus (97%), altered mental status of unknown etiology (88%), or altered mental status with an acute primary neurologic condition (88%). The median number of patients undergoing cEEG per month per center increased from August 2010 to August 2011 (6 to 10 per month in the United States; 2 to 3 per month in Canada). Few institutions have clinical pathways addressing cEEG use (31%). Physicians most commonly review cEEG twice per day (37%). There is variability regarding which services can order cEEG, the degree of neurology involvement, technologist availability, and whether technologists perform cEEG screening. CONCLUSIONS:: Among the surveyed institutions, which included primarily large academic centers, cEEG use in pediatric intensive care units is increasing and is often considered indicated for children with altered mental status at risk for nonconvulsive seizures. However, there remains substantial variability in cEEG access and utilization among institutions.
Journal of clinical neurophysiology: official publication of the American Electroencephalographic Society 04/2013; 30(2):156-160. · 1.47 Impact Factor
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ABSTRACT: The Affordable Care Act penalizes hospitals with high readmission rates. Children's hospitals are not yet among these hospitals, although that is likely to change. Because chronic neurologic conditions represent a sizable proportion of all children's hospitals costs, and because some/many of the readmissions might not be easily prevented, children's hospitals and neurologists who care for children might be inappropriately penalized for some readmissions. We encourage more study to identify the correlates of readmission of children who have a neurologic disorder.
Journal of child neurology 03/2013; · 1.59 Impact Factor
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ABSTRACT: Continuous electroencephalographic (CEEG) monitoring is used with increasing frequency in critically ill children to provide insight into brain function and to identify electrographic seizures. CEEG monitoring use often impacts clinical management, most often by identifying electrographic seizures and status epilepticus. Most electrographic seizures have no clinical correlate, and thus would not be identified without CEEG monitoring. There are increasing data showing that electrographic seizures and electrographic status epilepticus are associated with worse outcome. Seizure identification efficiency may be improved by further development of quantitative electroencephalography trends. This review describes the clinical impact of CEEG data, the epidemiology of electrographic seizures and status epilepticus, the impact of electrographic seizures on outcome, the utility of quantitative electroencephalographic trends for seizure identification, and practical considerations regarding CEEG monitoring.
Current Neurology and Neuroscience Reports 03/2013; 13(3):330. · 3.45 Impact Factor
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ABSTRACT: Methods for rapid and objective quantification of interictal spikes in raw, unprocessed electroencephalogram (EEG) samples are scarce. We evaluated the accuracy of a tailored automated spike quantification algorithm. The automated quantification was compared with the quantification by two board-certified clinical neurophysiologists (gold-standard) in five steps: 1) accuracy in a single EEG channel (5 EEG samples), 2) accuracy in multiple EEG channels and across different stages of the sleep-wake cycles (75 EEG samples), 3) capacity to detect lateralization of spikes (6 EEG samples), 4) accuracy after application of a machine-learning mechanism (11 EEG samples), and 5) accuracy during wakefulness only (8 EEG samples). Our method was accurate during all stages of the sleep-wake cycle and improved after the application of the machine-learning mechanism. Spikes were correctly lateralized in all cases. Our automated method was accurate in quantifying and detecting the lateralization of interictal spikes in raw unprocessed EEG samples.
Epilepsy & Behavior 01/2013; 26(2):143-152. · 2.34 Impact Factor
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ABSTRACT: Electrical status epilepticus in sleep involves an electroencephalographic pattern where interictal epileptiform activity is potentiated in the transition from wakefulness to sleep. Near-continuous spikes and waves that occupy a significant proportion of nonrapid eye movement sleep appear as a result of sleep-potentiated epileptiform activity. This electroencephalographic pattern appears in different electroclinical syndromes that present three common characteristics with different degrees of severity: seizures, sleep-potentiated epileptiform activity, and neuropsychologic regression. Continuous spikes and waves during sleep comprise the severest epileptic encephalopathy in the electroclinical spectrum. Landau-Kleffner syndrome presents with intermediate severity. Some "benign" pediatric focal epileptic syndromes represent the mildest end of this continuum. Based on published data, we provide a framework for clinical and electrical events. The underlying mechanisms leading to sleep potentiation of epileptiform activity in electrical status epilepticus in sleep are incompletely understood. A genetic basis or acquired early developmental insult may disrupt the normal maturation of neuronal networks. These factors may dynamically alter normal processes of brain development, leading to an age-related pattern of electroclinical expression of electrical status epilepticus in sleep.
Pediatric Neurology 12/2012; 47(6):390-410. · 1.52 Impact Factor
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ABSTRACT: Purpose: The terms "electrical status epilepticus during sleep (ESES)" and "continuous spikes and waves during sleep (CSWS)" have been used interchangeably when referring to related but different concepts. In addition, the quantification of epileptiform activity has not been standardized, and different approaches to quantification have been used. The aim of this study was to evaluate the extent to which pediatric neurologists and epileptologists use a homogeneous terminology and conceptualization in CSWS and ESES and to characterize the current understanding of these conditions. Methods: A survey addressing the use of terminology in "ESES" and "CSWS" and the understanding of related concepts was distributed online to all members of the Child Neurology Society and the American Epilepsy Society mailing lists. Surveys were self-administered and collected using an online survey website (http://www.surveymonkey.com). Key Findings: Two hundred nineteen surveys were completed, 137 from the Child Neurology Society mailing list and 82 from the American Epilepsy Society mailing list. ESES and CSWS were considered synonymous by 117 respondents, not synonymous by 61, 21 respondents did not know, and 20 did not respond. Most respondents (63.1%) considered CSWS as a devastating epileptic encephalopathy with severe sequelae even if treated correctly, but 25.1% of respondents indicated that it does not leave sequelae if epilepsy was treated early and another 11.8% noted that cognitive difficulties resolved with age. Cognitive and/or language regression were considered mandatory for the diagnosis of CSWS by only 27% of the respondents. The diagnosis of CSWS was based on electroencephalography (EEG) assessment alone by 31% of respondents. Respondents used different methods for calculation of the epileptiform activity, different EEG samples for calculation, and considered differently the lateralized epileptiform activity. The cut-off values for percentage of the sleep record occupied by spike-waves were variable depending on the respondent. There was no agreement on whether these cutoff values were mandatory for the diagnosis of ESES and CSWS. Significance: Our data show that the professionals caring for children with ESES and CSWS in North America use the terms, concepts, and defining features heterogeneously. The lack of a common language may complicate communication among clinicians and jeopardize research in this field. We anticipate that our data will fuel the development of much needed common terminology and conceptualization of ESES and CSWS.
Epilepsia 11/2012; · 3.96 Impact Factor
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ABSTRACT: Seizures can evolve sequentially into different clinical phases. For example, a seizure may start as an aura (first phase), then evolve into a tonic seizure (second phase), and evolve further into a generalized tonic-clonic semiology (third phase). It is currently unknown whether specific seizure evolutions cluster at particular times of the day and/or during sleep/wakefulness. We aimed to describe the distribution of the clinical evolution of seizures across time of day and sleep/wake state. We included all patients with at least two seizure phases admitted for long-term electroencephalogram monitoring during a 5 year period. Two-hundred-and-fifteen patients (866 seizures) presented with two different phases and 87 patients (324 seizures) evolved into a third clinical phase. During phase two, evolution into clonic seizures differed across time (p = 0.047) with peaks at 0-3 h and 6-9 h and during sleep (p < 0.001), evolution into automotor seizures peaked during wakefulness (p = 0.015), evolution into tonic seizures differed across time (p = 0.005) with peaks at 21-12 h and during sleep (p = 0.0119), and generalized tonic-clonic seizures peaked during sleep (p = 0.0067). Findings remained statistically significant after multivariable analysis adjusting, separately, for potential confounders (semiology of the first phase, age, gender, days in the long-term electroencephalographic monitoring unit, abnormal neuroimaging, number of antiepileptic medications, and seizure localization). During phase three, seizure evolutions followed the same pattern of distribution as during phase two but differences did not reach statistical significance. Our data demonstrate that the evolution of seizures into different phases cluster at specific times of day and at specific phases of the sleep/wakefulness cycle.
Journal of Neurology 10/2012; · 3.47 Impact Factor
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ABSTRACT: Benign epilepsy with centrotemporal spikes, early-onset childhood occipital epilepsy (Panayiotopoulos syndrome [PS]) and late-onset childhood occipital epilepsy (Gastaut type [LOCE-G]) are the principal pediatric focal epilepsy syndromes. They share major common characteristics: the appearance and resolution of electroclinical features are age related, there is a strong genetic predisposition, the clinical course is often mild with infrequent and easy to control seizures, interictal epileptiform activity is disproportionately abundant when compared with the clinical correlate, and tends to potentiate and generalize during sleep. In this review, we outline the relevant pathophysiology underlying this electroclinical spectrum. Then, the initial description of individual syndromes is followed by a summary of overlapping features and intermediate presentations that question the boundaries between these entities and provide the basis for the concept of a childhood seizure susceptibility syndrome. Additionally, we outline the main features of the related epileptic encephalopathies. An outlook on potential future lines of research completes this review.
Journal of clinical neurophysiology: official publication of the American Electroencephalographic Society 10/2012; 29(5):425-40. · 1.47 Impact Factor
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Julie Blumberg,
Iván Sánchez Fernández,
Martina Vendrame,
Bernhard Oehl,
William O Tatum,
Stephan Schuele,
Andreas V Alexopoulos,
Annapurna Poduri,
Christoph Kellinghaus,
Andreas Schulze-Bonhage, Tobias Loddenkemper
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ABSTRACT: Purpose: To provide an estimate of the frequency of dacrystic seizures in video-electroencephalography (EEG) long-term monitoring units of tertiary referral epilepsy centers and to describe the clinical presentation of dacrystic seizures in relationship to the underlying etiology. Methods: We screened clinical records and video-EEG reports for the diagnosis of dacrystic seizures of all patients admitted for video-EEG long-term monitoring at five epilepsy referral centers in the United States and Germany. Patients with a potential diagnosis of dacrystic seizures were identified, and their clinical charts and video-EEG recordings were reviewed. We included only patients with: (1) stereotyped lacrimation, sobbing, grimacing, yelling, or sad facial expression; (2) long-term video-EEG recordings (at least 12 h); and (3) at least one brain magnetic resonance imaging (MRI) study. Key Findings: Nine patients (four female) with dacrystic seizures were identified. Dacrystic seizures were identified in 0.06-0.53% of the patients admitted for long-term video-EEG monitoring depending on the specific center. Considering our study population as a whole, the frequency was 0.13%. The presence of dacrystic seizures without other accompanying clinical features was found in only one patient. Gelastic seizures accompanied dacrystic seizures in five cases, and a hypothalamic hamartoma was found in all of these five patients. The underlying etiology in the four patients with dacrystic seizures without gelastic seizures was left mesial temporal sclerosis (three patients) and a frontal glioblastoma (one patient). All patients had a difficult-to-control epilepsy as demonstrated by the following: (1) at least three different antiepileptic drugs were tried in each patient, (2) epilepsy was well controlled with antiepileptic drugs in only two patients, (3) six patients were considered for epilepsy surgery and three of them underwent a surgical/radiosurgical or radioablative procedure. Regarding outcome, antiepileptic drugs alone achieved seizure freedom in two patients and did not change seizure frequency in another patient. Radiosurgery led to moderately good seizure control in one patient and did not improve seizure control in another patient. Three patients were or are being considered for epilepsy surgery on last follow-up. One patient remains seizure free 3 years after epilepsy surgery. Significance: Dacrystic seizures are a rare but clinically relevant finding during video-EEG monitoring. Our data show that when the patient has dacrystic and gelastic seizures, the cause is a hypothalamic hamartoma. In contrast, when dacrystic seizures are not accompanied by gelastic seizures the underlying lesion is most commonly located in the temporal cortex.
Epilepsia 07/2012; 53(10):1810-1819. · 3.96 Impact Factor
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ABSTRACT: To investigate the sleep/wake, day/night, and 24-h periodicity of pediatric evolution to generalized tonic-clonic seizures (GTC).
Charts of 407 consecutive patients aged 0-21 years undergoing continuous video-EEG monitoring for epilepsy were reviewed for the presence of GTC evolution. Seizures were characterized according to 2001 ILAE terminology. Charts were reviewed for EEG seizure localization, MRI lesion, and for seizure occurrence in 3-h time blocks, out of sleep or wakefulness, and during the day (6 AM-6 PM) or night. Analysis was done with binomial testing. Regression models were fitted using generalized estimating equations with patients as the cluster level variable.
71 patients (32 girls, mean age 12.63 ± 5.3 years) had 223 seizures with GTC evolution. Sleep/wake seizure distribution predicted tonic-clonic evolution better than time of day, with more occurring during sleep (p<0.001). Tonic-clonic evolution occurred most frequently between 12-3 AM and 6-9 AM (p<0.05). Patients with generalized EEG onset had more tonic-clonic evolution between 9 AM and 12 PM (p<0.05). Patients with extratemporal focal seizures were more likely to evolve during sleep (p<0.001); this pattern was not found in patients with temporal or generalized seizure onset on EEG. Patients without MRI lesions were more likely to evolve between 12 AM and 3 AM (p<0.05), in the sleeping state (p<0.001), and at night (p<0.05). Logistic regression revealed that sleep and older patient age were the most important predictors of GTC evolution.
GTC evolution occurs most frequently out of sleep and in older patients. Our results may assist in seizure prediction, individualized treatment patterns, and potentially complication and SUDEP prevention.
Seizure 06/2012; 21(7):535-9. · 1.80 Impact Factor
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ABSTRACT: A retrospective review of children with epilepsy and obstructive sleep apnea, treated surgically for their obstructive sleep apnea from January 2008-October 2010, was performed for age, sex, type of epilepsy, antiseizure medications, sleep-study data, and changes in seizure frequency. Twenty-seven subjects (median age, 5 years) with no adjustment to their medications around their time of surgery were identified. Three months after surgery, 10 (37%) patients became seizure-free, three (11%) demonstrated >50% seizure-reduction, and six (22%) exhibited an amelioration of seizure frequency. Two (7%) demonstrated unchanged seizure-frequency, and six (22%) manifested a worsening of seizure frequency. Median seizure frequency before surgery was 8.5 (interquartile range, 2-90), and after surgery, three (interquartile range, 0-75), with a 53% median seizure reduction. Multivariate analysis demonstrated a trend toward seizure freedom with each percentile increase in body mass index and early age of surgery. We conclude that obstructive sleep apnea surgery may decrease seizure frequency, especially in children with elevated body mass index scores and younger age at time of surgery.
Pediatric Neurology 06/2012; 46(6):359-62. · 1.52 Impact Factor
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ABSTRACT: Epileptic spasms are seizures that occur predominantly in children and are characterized by clusters of brief axial movements. Epileptic spasms may occur in the context of a variety of syndromes. Previous research has found that epileptic spasms occur in a sleep/wake and diurnal rhythm. The purpose of this study was to identify these patterns in different age groups.
Charts of 2,021 patients with epilepsy undergoing video-electroencephalography (EEG) monitoring over a 10-year period were reviewed for presence of epileptic spasms and analyzed for their occurrence during the day (6 a.m. to 6 p.m.) or night, out of wake or sleep, and in 3-h time-blocks throughout the day. Exact epileptic spasm time, EEG localization, and the presence or absence of magnetic resonance imaging lesion were also recorded. Patients were separated into two age groups: A ages 3 and under, and over age 3. Statistical analysis of seizure occurrence in time bins was carried out using binomial calculations. p-Values <0.05 were taken as significant. Using exact seizure times, a generalized linear mixed model of the Poisson-family with a square root link function was used to calculate mean seizure times. Age, as a binary variable, and time, as a categorical variable, was treated as fixed effect predictors, and individual effects were modeled as random effects. For comparison between the two age groups, over age 3 and under age 3, seizure times were transformed into circular variables. A circular analysis of variance test was used to assess for the difference in mean seizure time, assuming a von Mises distribution of the circle.
We analyzed 219 clusters of epileptic spasms in 51 patients (15 girls; mean age 2.15 ± 2.22 years). Forty-two patients younger than 3 years of age had 163 seizures and nine patients older than 3 years had 56 seizures. Epileptic spasms occurred predominantly during wakefulness (p < 0.001) and during daytime (p < 0.001). Epileptic spasms occurred most frequently between 9 a.m. and noon (p < 0.05) and between 3 p.m. and 6 p.m. (p < 0.001). Patients without magnetic resonance imaging lesions had most seizures between 9 a.m. and noon (p < 0.01) and 3 p.m. and 6 p.m. (p < 0.001). Thirty-seven patients had 157 epileptic spasms (71.2%) with generalized EEG patterns and 14 patients had 62 epileptic spasms (28.8%) with focal EEG patterns. Generalized EEG seizures occurred more frequently than focal EEG seizures (p < 0.001). Following age stratification, patients younger than 3 years had most epileptic spasms between 9 a.m. and noon (p < 0.05) and 3 p.m. and -6 p.m. (p < 0.01) and patients older than 3 years had most epileptic spasms between 6 a.m. and -9 a.m. (p < 0.05) and a second peak between 3 p.m. and 6 p.m., although the difference was not statistically significant due to insufficient numbers. Using continuous time analysis, the mean seizure time in the under age 3 and the over age 3 groups was 2:24 p.m. and 11:40 a.m. Using a circular analysis of variance test, the difference between mean seizure times in these groups was found to be statistically significant (p = 0.038).
Epileptic spasms occur more frequently in the waking state and daytime. Younger patients have epileptic spasms mostly between 9 a.m. and noon and 3 p.m. and -6 p.m., and older patients have epileptic spasms mostly between 6 a.m. and 9 a.m. These findings emphasize age-related changes in epileptic spasm pathophysiology or potentially evolution of disease with age.
Epilepsia 05/2012; 53(7):1170-7. · 3.96 Impact Factor
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ABSTRACT: Currently, in continuous spikes and waves during sleep (CSWS) there is a lack of systematic assessments of the clinically relevant stages and the evolution of the electroencephalographic features. The aim of this study is to describe the evolution over time of clinical and electroencephalographic features in CSWS.
We enrolled patients from our video-electroencephalography (EEG) monitoring unit with CSWS and with overnight EEG studies with at least one overnight assessment per year over a minimum period of 3 years. We studied clinical presentation and electroencephalographic features. We calculated the (1) spike-wave percentage (SWP) as the percentage of 1-s bins containing at least one spike-wave complex and (2) spike frequency (SF) as the number of spikes per 100 s.
Nine children (six boys) met the inclusion criteria during a 15-year period. Seven (78%) had an abnormal development prior to the epilepsy onset, and in two (22%) seizures were the only presenting symptom. Median age at epilepsy onset was 2 years (range 2 days to 4 years), at neuropsychological regression 5.1 years (4-7.7 years), and at seizure freedom 8.6 years (6.5-11.4 years). Median duration and range of clinically relevant stages were as follows: dormant stage (birth-epilepsy onset median 2 years, range 2 days-4 years), prodromal stage (epilepsy onset-neuropsychological regression 3.9 years, range 0.9-7.7 years), acute stage (neuropsychological regression-seizure freedom 2.9 years, range 2.1-6.6 years), and residual stage (after seizure freedom). Seven patients (78%) had a structural lesion on neuroimaging. At last follow-up (median 11.4 years, range 7.2-20.3 years), eight patients (89%) were receiving antiepileptic treatment, and all patients had residual neurocognitive deficits. During the acute stage, SWP was <85% in 13 (42%) of 31 assessments, and after seizure freedom, 3 of 5 patients (60%) had SWP >85%. Evolution of electroencephalographic patterns included increasing-decreasing, continuously elevated, and fluctuating patterns (33.3% each). There was good correlation between SWP and SF (Spearman correlation-coefficient = 0.942; p < 0.0001). SF, which can exceed 100%, reflected changes in electroencephalography pattern in more detail than SWP, which cannot exceed 100% and therefore has a ceiling effect.
Our series systematically studied the age of occurrence of the significant clinical events. These may assist in defining clinical stages, which can provide a useful framework for future clinical trials in patients with CSWS. The severity of the epileptiform discharges on EEG did not always correlate with seizure frequency and severity; epileptiform discharges could be prominent after seizure freedom and fluctuated along the course of the disease. The values of SWP and SF correlated well, but SWP based on 1-s bins has the potential disadvantage of a ceiling effect.
Epilepsia 05/2012; 53(7):1185-95. · 3.96 Impact Factor
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Iván Sánchez Fernández,
Stavros Hadjiloizou,
Yaman Eksioglu,
Jurriaan M Peters,
Masanori Takeoka,
Emir Tas,
Imane Abdelmoumen,
Alexander Rotenberg,
Sanjeev V Kothare,
James J Riviello, Tobias Loddenkemper
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ABSTRACT: We describe the short-term effects of high-dose oral diazepam on sleep-potentiated epileptiform activity in patients with electric status epilepticus during sleep. We enrolled patients treated with high-dose oral bedtime diazepam from 2001-2009. We defined spike percentage as the percentage of 1-second bins containing at least one spike, and calculated it during three randomly selected 5-minute samples of wakefulness throughout the day and during the first 5 minutes of every hour of non-rapid eye movement sleep at night. In this study, patients were considered to demonstrate sleep-potentiated epileptiform activity when their spike percentage during sleep was increased by ≥50% compared with wakefulness. Twenty-nine children (18 boys) were included (median age, 7.4 years). Twenty-four hours after receiving high-dose diazepam, epileptiform activity was significantly reduced (76.7% at baseline vs 40.8% 24 hours after high-dose diazepam; Wilcoxon signed ranks test, Z = -4.287, P < 0.0001). Seven patients (24.1%) manifested mild, reversible side effects during the first 48 hours after diazepam administration. High-dose oral diazepam effectively and safely reduced epileptiform activity in patients with electric status epilepticus during sleep.
Pediatric Neurology 05/2012; 46(5):312-8. · 1.52 Impact Factor
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ABSTRACT: Retrospective review was performed of children aged <3 years with epileptic spasms at our center from 2004-2010. Short-term (<6 months) and long-term (≥6 months) outcomes were assessed. We included 173 children (104 boys; median age of onset, 6.8 months) with epileptic spasms of known (62%) and unknown (38%) etiology. Treatments included adrenocorticotropic hormone (n = 103), vigabatrin (n = 82), phenobarbital (n = 34), and other agents (n = 121). Short-term treatment with adrenocorticotropic hormone and vigabatrin provided better epileptic spasm control in groups with known and unknown etiology than other agents. At follow-up (6-27 months), 54% of children manifested seizures, and 83% manifested developmental delay. Known etiology was a predictor of poor developmental outcome (P = 0.006), whereas bilateral/diffuse brain lesions predicted both poor development and seizures (P = 0.001 and 0.005, respectively). Initial presentations of epileptic spasms with hypotonia or developmental delay most strongly predicted both seizures and neurodevelopmental outcomes (P < 0.001). In a child presenting with epileptic spasms with developmental delay or hypotonia, no specific treatment may offer superior benefit.
Pediatric Neurology 05/2012; 46(5):276-80. · 1.52 Impact Factor
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ABSTRACT: The study objective was to compare qualitatively the clinical features of patients with electrical status epilepticus in sleep with focal versus generalized sleep potentiated epileptiform activity. We enrolled patients 2 to 20 years of age, studied between 2001 and 2009, and with sleep potentiated epileptiform activity defined as an increase of epileptiform activity of 50% or more during non-rapid eye movement sleep compared with wakefulness. Eighty-five patients met the inclusion criteria, median age was 7.3 years, and 54 (63.5%) were boys. Sixty-seven (78.8%) patients had focal sleep potentiated epileptiform activity, whereas 18 (21.2%) had generalized sleep potentiated epileptiform activity. The 2 groups did not differ with respect to sex, age, presence of a structural brain abnormality, epilepsy, or other qualitative cognitive, motor, or behavioral problems. Our data suggest that there are no qualitative differences in the clinical features of patients with focal versus generalized sleep potentiated epileptiform activity.
Journal of child neurology 04/2012; · 1.59 Impact Factor
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ABSTRACT: EEG monitoring is used routinely during the Wada test in children. We quantified EEG asymmetry using the Brain Symmetry Index (BSI) to reduce subjectivity of EEG interpretation. Clinical and procedural variables were obtained and EEG data were retrieved from 46 patients with a total of 89 injections. The BSI, the absolute value of the relative difference of the average spectral density of the right and left hemisphere, was calculated over time for all EEGs. Lateralized slowing was correctly identified in all procedures. Asymmetry was minimal at baseline (BSI 0.16) and increased with injection of amobarbital (BSI 0.49). Various patterns of the BSI were seen in distinct clinical and procedural scenarios. In this retrospective analysis, the BSI could not predict an unsuccessful Wada procedure. Our results suggest application of the BSI during the Wada test in children is feasible. Real-time calculation of the BSI during EEG monitoring in the angiography suite is warranted for further validation.
Epilepsy & Behavior 02/2012; 23(3):247-53. · 2.34 Impact Factor
