Ring chromosome 20 with nonconvulsive status epilepticus: electroclinical correlation of a rare epileptic syndrome.

The electroclinical features of two Thai women with ring chromosome 20 and nonconvulsive status epilepticus (NCSE) were studied. Both have also had generalized tonic-clonic seizures and complex partial seizures of varying frequencies since adolescence. Their intellectual functions were normal. Twenty-four-hour video/EEG telemetry recorded during the NCSE showed fluctuating consciousness between overt unresponsiveness and normal awareness. The EEG consisted of long-lasting generalized rhythmic 3-5 Hz sharp or slow waves with a few spikes, lasting several days. Despite the continuous discharges, the patients had relatively subtle clinical episodes of seizures, during which they were sometimes responsive to verbal stimuli. Intravenous antiepileptic drugs (AED) had little effect on the rhythmic EEG. No lesion in their MRIs contributed to NCSE. Ring chromosome 20 was found in 20% of female karyotype in both patients [46,XX,r(20) (p13 q13)/46,XX] but were negative in four healthy siblings. Oral AEDs decreased more than 75% of the overt CPS episodes in both patients at 22 and 26 months of follow-up but had no effect on the natural history of electrical NCSE. The patients' daily activities were minimally affected by the ongoing electrical discharges. These are the first two cases reported of ring chromosome 20 with NCSE in Thailand. Our patients present a rather benign and pharmacologically responsive course probably because of the low percentage of r(20) mosaicism. The electroclinical correlations in our cases raise the possibility that the mechanism of continuous rhythmic waves in this syndrome may be unrelated to epilepsy. Assessing the severity of this syndrome using both clinical seizures and EEG is crucial.     

Nonconvulsive status epilepticus in patients with brain tumors

PurposeThe prevalence of nonconvulsive status epilepticus (NCSE) in brain tumor patients is unknown. Since NCSE has been associated with significant mortality and morbidity, early identification is essential. This study describes the clinical and EEG characteristics, treatment, and outcome in brain tumor patients with NCSE.MethodAll patients admitted to Mount Sinai Hospital from 2009 to 2012 with an ICD-9 brain tumor code were cross-referenced with the epilepsy department's database. EEGs from matching patients were reviewed for NCSE. Relevant information from the medical records of the patients with NCSE was extracted.Results1101 brain tumor patients were identified, of which 259 (24%) had an EEG and 24 (2%) had NCSE. The vast majority of seizures captured were subclinical with 13 patients (54%) having only subclinical seizures. Treatment resolved the NCSE in 22 patients (92%) with accompanying clinical improvement in 18 (75%) of those patients. Tumor recurrence or progression on MRI was associated with decreased 2-month survival (75% mortality, p = 0.035) compared to stable tumors (20% mortality). Patients with metastatic disease had median survival from tumor diagnosis of 1.2 months.ConclusionNCSE in brain tumor patients may be under diagnosed due to the frequent lack of outward manifestations and highly treatable with improvement in the majority of patients. NCSE patients with progressing brain lesions, tumor recurrence, or metastatic disease are at serious risk of mortality within 2 months. Continuous EEG monitoring in brain tumor patients with recent clinical seizures and/or a depressed level of consciousness may be critical in providing appropriate care.     

Utilidad del electroencefalograma en el manejo de la epilepsia en el Servicio de Urgencias

IntroductionElectroencephalography (EEG) is an essential diagnostic tool in epilepsy. Its use in emergency departments (ED) is usually restricted to the diagnosis and management of non-convulsive status epilepticus (NCSE). However, EDs may also benefit from EEG in the context of other situations in epilepsy.MethodsWe conducted a retrospective observational study using the clinical histories of patients treated at our hospital's ED for epileptic seizures and suspicion of NCSE and undergoing EEG studies in 2015 and 2016. We collected a series of demographic and clinical variables.ResultsOur sample included 87 patients (mean age of 44 years). Epileptic seizures constituted the most common reason for consultation: 59.8% due to the first episode of epileptic seizures (FES), 27.6% due to recurrence, and 12.6% due to suspected NCSE. Interictal epileptiform discharges (IED) were observed in 38.4% of patients reporting FES and in 33.3% of those with a known diagnosis of epilepsy. NCSE was confirmed by EEG in 36.4% of all cases of suspected NCSE. Presence of IED led to administration of or changes in long-term treatment in 59.8% of the patients.ConclusionsEEG is a useful tool for seizure management in EDs, not only for severe, sudden-onset clinical situations such as NCSE but also for diagnosis in cases of non-affiliated epilepsy and in patients experiencing the first episode of epilepsy.     

Intravenous Lacosamide in refractory nonconvulsive status epilepticus

BackgroundMany patients present with refractory Status epilepticus (SE) despite multiple anti-epileptic drugs (AEDs). Lacosamide (LCM) was recently approved as an adjunct AED for partial-onset seizures. It has unique mechanism of modulating voltage-gated sodium channels by enhancing their slow inactivation. LCM has demonstrated efficacy in animal models of pharmacoresistant seizures. To date, there are isolated anecdotal reports of LCM use in SE.ObjectiveTo report a single center experience with IV Lacosamide in patients with NCSE.MethodsPharmacy records were reviewed to identify patients with SE who received IV LCM in our institution. Data on demographics, response to therapy and adverse effects/outcomes were analyzed. All patients had continuous EEG monitoring.Results10 patients (4 men, 6 women), age 16–90 years with refractory SE were given LCM. Eight patients were in focal non-convulsive SE (NCSE), 2 were in generalized non-convulsive SE. The etiologies included anoxic brain injury, idiopathic, encephalitis, tumor, posterior reversible encephalopathy syndrome (PRES), stroke, and AVM. IV LCM was added after traditional AEDs, including drug-induced coma in some, failed to control the SE. NCSE resolved in 7/10 patients whereas 1/10 patient showed partial response with cessation of NCSE but still frequent electrographic seizures and 2/10 patients were resistant to therapy.ConclusionsLCM is a useful adjunct in refractory NCSE. The IV formulation allows prompt administration in the intensive care unit setting. Response was seen especially in focal SE. Similar to other AEDs, response was poor in patients with postanoxic injury. Our data is limited by the small number of patients. Larger controlled studies are necessary to assess accurately the efficacy of IV LCM as an early treatment of SE.     

Patients' ability to react before complex partial seizures

ObjectiveThe purpose of the study described here was to investigate the pathophysiology of patients’ ability to react during the conscious (aura) phase of complex partial seizures (CPS) originating from the temporal lobe.MethodsWe reviewed video recordings of CPS experienced by 130 adult patients who had undergone epilepsy surgery for intractable medial temporal lobe epilepsy. All patients were instructed to push the alarm button when they felt an aura. We defined the preictal reactivity as the ability to push the alarm button before the complex partial (unconscious) phase of seizures.ResultsSeventy-seven patients (59%) pushed the alarm button before seizures. Patients with preictal reactivity were significantly younger, more often had lateralized EEG seizure patterns, and had a better postoperative outcome. Patients who did not push the alarm button had secondarily generalized seizures more often.ConclusionsAbility to react before CPS is associated with a circumscribed region involved at seizure onset and spread, and with a seizure-free postoperative outcome.     

Relationship between severity of epilepsy and developmental outcome in Angelman syndrome

To clarify the relationship between the degree of developmental disturbance and the severity of epilepsy in Angelman syndrome, we investigated 11 patients and measured both clinical outcomes and EEG parameters. Seven patients were followed up until after 8 years of age. Eight patients were found to have 15q11-q13 deletions. All patients experienced epileptic seizures and all but one displayed non-convulsive status epilepticus (NCSE) during the period of observation. Epileptic seizures, including NCSE, disappeared by around 8 years of age. In addition, specific epileptic discharges, as measured by EEG, tended to subside with age. Although development seemed almost normal or only slightly delayed during the first 6 months of life, all patients eventually developed severe retardation. Two patients displayed very severe retardation and were unable to comprehend language or walk independently at the last follow-up. Only one patient was able to speak a few meaningful words. In one of the most severely affected patients, who showed the earliest onset of seizures and NCSE, it is possible that the repetitive bouts of NCSE might be responsible for the severe developmental outcome. However, the other patient with particularly severe retardation did not experience NCSE, while the patient with the most favorable outcome had repetitive episodes of NCSE. Therefore, we conclude that the severity of developmental disturbance in Angelman syndrome is not necessarily related to the degree of epilepsy. However, intensive therapy for NCSE might still be justified because there are some patients in whom NCSE results in a transient and sometimes permanent decline in mental and motor functioning.     

Absence seizures during sleep in childhood absence epilepsy: A sign of drug resistance?

BackgroundChildhood absence epilepsy (CAE) is a common pediatric epilepsy syndrome. It is characterized by typical absence seizures and a highly recognizable electroencephalography (EEG) pattern. But little is known about absence seizures during sleep.Case reportA 7-year-old female presented with frequent typical absence seizures with 3 Hz generalized spike and wave discharges on EEG. Based on electro-clinical features she was diagnosed with CAE. When she was 8 years old, absence seizures occurred during sleep. She had refractory absence seizures even with valproic acid, lamotrigine, levetiracetam, and perampanel.ConclusionAbsence seizures can occur during sleep in CAE. Absence seizures should be considered, especially when 3 Hz generalized spike and wave discharges last >2 s on EEG during sleep. It may be a sign of drug resistance and poor prognosis.     

Tiagabine-induced generalised non convulsive status epilepticus in patients with lesional focal epilepsy.

PURPOSE: To report 3 cases with focal lesional epilepsy that had non-convulsive status epilepticus (NCSE) induced by treatment with tiagabine (TGB) and review the previously published cases. Drugs that enhance GABAnergic transmission are recognised to promote absence seizures in patients with generalised epilepsy syndromes and may on occasions even induce NCSE. However, that TGB can also induce NCSE in focal lesional epilepsy is not widely recognised in clinical practice. METHOD: The clinical history, EEG and MRI findings were reviewed in 3 patients with lesional focal epilepsy who presented to our epilepsy programs over a 12 month period with TGB-induced NCSE. All previously reported cases in the English medical literature were reviewed. RESULTS: The three patients had longstanding complex partial and secondarily generalised seizures refractory to multiple different anti-epileptic drugs. In two cases, MRI demonstrated a focal malformation of cortical development in the left parieto-occipital region and in the third left mesial temporal sclerosis. Following commencement of TGB in one patient and dose escalation in two, prolonged episodes of confusion and poor responsiveness were noted. Prolonged EEG monitoring demonstrated continuous high amplitude, generalised, 2-4 Hz delta activity with intermingled spikes during the episodes of unresponsiveness, consistent with NCSE. The clinical and EEG activity normalised following the administration of IV clonazepam followed by dose reduction or withdrawal of the TGB. Eleven previously reported cases of patients with partial epilepsy and a focal underlying lesion on MRI were identified, all of whom had similar features to that seen in our cases. CONCLUSIONS: These cases illustrate that TGB may induce generalised NCSE in patients with focal lesional epilepsy, in addition to those with generalised syndromes. We hypothesise that patients may have developed an acquired alteration in the sensitivity of their thalamocortical circuitry that renders them more sensitive to the effects of drugs that enhance GABAnergic activity.     

Clinical implications of scalp ictal EEG pattern in patients with temporal lobe epilepsy

ObjectiveTo determine the clinical implications of scalp ictal EEG pattern in patients with temporal lobe epilepsy (TLE).MethodsScalp EEG ictal patterns were retrospectively determined in 27 consecutive patients with medically refractory temporal lobe epilepsy who underwent phase-1 scalp video-EEG and phase-2 simultaneous scalp and intracranial video-EEG recordings for pre-surgical evaluation.ResultsOf the 192 temporal lobe seizures recorded during phase-1 and phase-2 scalp video-EEG studies, 124 (65%) seizures were associated with theta/alpha (5–9 Hz) ictal onset pattern, and 68 (35%) seizures were associated with delta (2–5 Hz) ictal onset pattern. Fourteen (52%) patients had exclusively theta/alpha ictal onset, 3 (11%) patients had exclusively delta ictal onset, and 10 (37%) patients had mixed theta/alpha and delta ictal onsets. MTLE was observed in 26 patients who had 124 seizures with theta/alpha ictal onset and 59 seizures with delta ictal onset. LTLE was observed in one patient who had 9 seizures with delta ictal onset. Scalp ictal EEG pattern was not significantly correlated with postsurgical seizure outcomes.ConclusionsBoth scalp delta and theta/alpha ictal onset patterns can be commonly found in patients with MTLE.SignificanceScalp delta ictal onset is not a unique EEG pattern for LTLE as commonly believed.     

Seizure outcomes of posterior reversible encephalopathy syndrome and correlations with electroencephalographic changes

RationaleSeizures are among the most common clinical presentations of posterior reversible encephalopathy syndrome (PRES). This syndrome has rarely been reported to cause chronic epilepsy or persistent cortical dysfunction. The prognostic value of EEG findings during PRES is unknown. We retrospectively evaluated EEG characteristics in patients with PRES in a single medical center. We also evaluated the long-term outcome regarding seizure occurrence beyond the acute phase in these patients.MethodsWe searched a radiology database at the University of Minnesota from 1997 to 2012 to identify patients with clinically and radiologically diagnosed PRES. Among the patients with PRES, we reviewed MRI images, EEG findings, clinical manifestations including seizure occurrences, and clinical outcomes beyond the acute phase.ResultsSeventy-five patients were included in the study. Fifty-eight out of seventy-five (77.3%) patients with PRES had seizures. A total of 48 EEG studies were performed in 38 patients. Generalized slowing was the most common EEG pattern. Among the 38 patients who had EEGs, 37 (97.3%) patients had diffuse or focal slowing of the background, and 11 (28.9%) patients had IEDs. Four out of seventy-five (5.3%) patients had seizures later than one month beyond their hospitalization for PRES. None of these 4 patients had seizures before the episode of PRES. Two patients developed chronic epilepsy, with seizures occurring later than one year after the PRES.ConclusionMost patients who had seizures or who had epileptiform activities in EEG during PRES did not subsequently develop chronic epilepsy. No patient developed chronic epilepsy in the absence of clinical seizures during PRES. Posterior reversible encephalopathy syndrome may infrequently be associated with subsequent development of symptomatic epilepsy.     

Incidence of recurrent seizures following hospital discharge in patients with LPDs (PLEDs) and nonconvulsive seizures recorded on continuous EEG in the critical care setting

PurposeContinuous EEG (cEEG) has helped to identify nonconvulsive seizures (NCS) and nonconvulsive status epilepticus (NCSE) along with lateralized periodic patterns (LPDs or PLEDs) in ICU patients with much higher frequency than previously appreciated, but understanding their implications may be more complex. The aim of this study was to investigate the incidence of recurrent seizures after hospital discharge and their associated factors in patients with PLEDs and NCS in the critical care setting.MethodsAfter IRB approval, we used our EEG reporting database to find 200 consecutive patients who had PLEDs and/or NCSs on cEEG. Patients with less than 3 months of follow-up were excluded. Remaining patients were divided into three groups: PLEDs + Seizure (NCS/NCSE), PLEDs only, and Seizures (NCS/NCSE) only. Medical records were reviewed to gather demographical and clinical details. Univariate data analysis was done using JMP 9.0 (Marlow, Buckinghamshire, UK).ResultsThere were 51 patients in ‘PLEDs + Seizure’ group, 45 in ‘PLEDs only’ group, and 22 in ‘Seizure only’ group. Ischemic stroke, hemorrhage, and tumors were the top three etiologies. Nearly 47% of our study population had postdischarge seizures during a mean follow-up period of 11.9 (+/− 6) months. We found that 24.4% of patients in the PLEDs only group had seizures after discharge, which increased to 60.7% if they had seizures as well during their ICU stay. Slightly more than 52% of patients had a postdischarge EEG, of which, 59% was in the form of inpatient cEEG during a rehospitalization, accounting for 30.5% of the total study population. It was an indicator of high readmission rates in this population.ConclusionAlmost every other patient with PLEDs and/or NCS on cEEG had seizures after ICU discharge. A quarter of patients on cEEG in the ICU with PLEDs alone had seizures after discharge, and after excluding prior epilepsy, 17% of patients with PLEDs had seizures on follow-up. This was dramatically increased with the recording of PLEDs with NCS, with 60% of patients having seizures after discharge from the ICU and 48% of patients after excluding prior epilepsy. Patients with NCS on cEEG alone had 63% chance of seizure recurrence that dropped to 38% with exclusion of prior epilepsy. Future studies are needed to define the postdischarge outcomes including seizure recurrence in this patient population.This article is part of a Special Issue entitled “Status Epilepticus”.     

Nonconvulsive status epilepticus in children: clinical and EEG characteristics

BACKGROUND: Nonconvulsive status epilepticus (NCSE) is a highly heterogeneous clinical condition that is understudied in the pediatric population. OBJECTIVE: To analyze the epidemiological, clinical, and electroencephalograpic features in pediatric patients with NCSE. METHODS: We identified 19 pediatric patients with NCSE from the epilepsy database of the Comprehensive Epilepsy Center at, Columbia University between June 2000 and December 2003. Continuous electroencephalographic (EEG) monitoring was analyzed and chart review was performed. RESULTS: The patients ranged from 1 month old to 17 years of age. Five patients developed NCSE following convulsive status epilepticus (CSE), and a further 12 patients developed NCSE after brief convulsions. Two developed NCSE as the first manifestation during a comatose state following hypoxic events. Acute hypoxic-ischemic injury was the most frequent etiology of NCSE in our population (5 of 19; 26%), followed by exacerbation of underlying neurometabolic disease (4 of 19; 21%), acute infection (3 of 19; 16%), change in antiepileptic drug regimen (3 of 19;16%), refractory epilepsy (2 of 19; 11%) and intracranial hemorrhage (2 of 19; 11%). Six patients had associated periodic lateralized epileptiform discharges (PLEDs), one had generalized periodic epileptiform discharges (GPEDs). Five (5 of 19; 26%) patients died of the underlying acute medical illness. Periodic discharges were associated with worse outcome. CONCLUSION: The majority of our patients with NCSE had preceding seizures in the acute setting prior to the diagnosis of NCSE, though most of these seizures were brief, isolated convulsions (12 patients) rather than CSE (five patients). Prolonged EEG monitoring to exclude NCSE may be warranted in pediatric patients even after brief convulsive seizures. Prompt recognition and treatment may be necessary to improve neurological outcome.     

Improvement of epilepsy with lacosamide in a patient with ring chromosome 20 syndrome

BackgroundRing chromosome 20 syndrome is a rare chromosomal disorder characterized by refractory seizure, mental retardation, and behavioral problems. Although there are reports of the effective treatment of patients with antiepileptic drugs (AEDs), no study has reported the effects of lacosamide(LCM) in children with this syndrome. We report a 7-year-old boy with this syndrome whose refractory and behavioral abnormalities have been remarkably improved by treatment with LCM.Case presentationThe patient was a 7-year-old boy with no medical or family history of epilepsy. He developed epilepsy with cessation of movement and derivation of the eyes followed by hyperkinetic seizures that made him squeak strangely and cling to his parents. The seizures lasted for less than a minute and were frequent (they occurred more than 30 times a day), particularly at night. Behavioral abnormalities such as hyperactivity also presented. Brain magnetic resonance imaging revealed no structural abnormalities, but an interictal electroencephalogram (EEG) indicated spikes and waves in the frontal lobe dominantly, and ictal single-photon emission computed tomography (SPECT) revealed a blood flow increase in the bilateral orbital frontal area in comparison to interictal SPECT. After chromosome examination, we diagnosed the patient with ring chromosome 20 syndrome (4/30 mosaic). Carbamazepine was ineffective, and seizures were exacerbated with levetiracetam (LEV). LCM was added to the treatment regimen with valproic acid (VPA) and lamotrigine (LTG); consequently, the seizures disappeared, and EEG results also improved. The patient’s behavioral disorders, such as hyperactivity, were improved, and he was able to return to elementary school.ConclusionAlthough VPA and LTG are generally effective for the treatment of ring chromosome 20 syndrome, they do not completely suppress seizures. LCM can be considered an effective option for seizure control in patients with this syndrome.     

Non-convulsive status epilepticus; the rate of occurrence in a general hospital

BackgroundNon-convulsive status epilepticus (NCSE) has been increasingly recognized as a cause of impaired level of consciousness in the ICU and emergency rooms. The diagnosis can be easily missed without an electroencephalogram (EEG) given the paucity of overt clinical signs in this condition. Recently few published data estimated the prevalence to be between 3% and 8%.ObjectiveTo assess the rate of occurrence of NCSE among patients with various degrees of impaired consciousness referred to the Neurophysiology Laboratory at Vancouver General Hospital.MethodWe conducted a retrospective analysis of 451 adult patients (>16 years of age) with a question of NCSE or with an unknown cause of impaired level of consciousness between the years 2002 and 2004. NCSE was defined according to the Young's criteria of electrographic status epilepticus. NCSE was categorized into focal and generalized epileptic activity based on the continuous EEG monitoring (CEEG). Further analysis of age, gender and etiology was performed.ResultsOf 451 patients, EEG demonstrated electrographic status epilepticus with no overt clinical signs in 42 patients (9.3%). Median age was 61.8 years (range 21–94). According to etiology, 38.1% of patients with NCSE had hypoxic–anoxic injury, 19% had intracerebral hemorrhage (including trauma), 11.9% had the diagnosis of idiopathic or cryptogenic epilepsy, 7.1% had ischemic stroke, 4.8% were secondary to tumors and 4.8% to viral encephalitis.ConclusionThe rate of occurrence of NCSE in patients with decreased level of consciousness was 9.3%. The cohort represented a group of patients who were comatose and required assisted ventilation or had altered level of consciousness. Hypoxic brain injury was the most responsible etiology of NCSE in the cohort studied.     

Seizure semiology and electroencephalography in young children with lesional temporal lobe epilepsy

PurposeThis study aimed to discuss the clinical features of seizure semiology and electroencephalography (EEG) in young children with lesional temporal lobe epilepsy (TLE).MethodChildren with lesional TLE received presurgical evaluation for intractable epilepsy. They were followed up for more than one year after temporal lobectomy. We reviewed the medical history and video-EEG monitoring of children with TLE to analyze the semiology of seizures and EEG findings and compared the semiology of seizures and EEG findings of childhood TLE and adult TLE.ResultsA total of 84 seizures were analyzed in 11 children (aged 23–108 months). The age of seizure onset was from 1 month to 26 months (a mean of 17.6 months). All of the patients exhibited prominent motor manifestations including epileptic spasm, tonic seizure, and unilateral clonic seizure. Seven children manifested behavioral arrest similar to an automotor seizure in adult TLE but with a shorter duration and higher frequency. The automatisms were typically orofacial, whereas manual automatisms were rarely observed. The EEG recordings revealed that diffuse discharge patterns were more common in younger children, whereas focal or unilateral patterns were more typical in older children. All of the patients were seizure-free after temporal lobectomy with more than one-year follow-up. All of the children had a mental development delay or regression; however, there was improvement after surgery, especially in those with surgery performed early.ConclusionIn contrast to TLE in adults, young children with lesional TLE probably represent a distinct nosological and probably less homogeneous syndrome. Although they had generalized clinical and electrographic features, resective epilepsy surgery should be considered as early as possible to obtain seizure control and improvement in mental development.     

Subacute encephalopathy with seizures in alcoholics (SESA syndrome) revisited

PurposeThe aim of this paper is to describe two additional cases of subacute encephalopathy with seizures in alcoholics (SESA syndrome), and to propose that this entity now should be considered as a subtype of nonconvulsive status epilepticus (NCSE).MethodsWe retrospectively analyzed the clinical characteristics, electroencephalography (EEG), neuroimaging data, and prognosis of these two further cases of SESA syndrome. In addition, we compare our findings with the cases previously described in the English literature in order to propose new diagnostic criteria.ResultsTwo adults with history of chronic alcohol abuse were admitted because of confusion and seizures. A routine EEG showed frequent periodic lateralized epileptiform discharges (PLEDs) localized over the right temporal regions. In one case, we captured two complex partial seizures (CPSs) arising from the right hemisphere. Neuroimaging studies revealed subjacent chronic vascular pathology. Following transfer to the intensive care unit (ICU), both improved to antiepileptic treatment and were discharged with full recovery.ConclusionOn the basis of our findings and a review of the literature, we suggest that SESA syndrome represents a subtype of partial or localization-related NCSE given its particular clinical, electroencephalographic, neuroimaging and prognostic characteristics.     

Non-convulsive status epilepticus associated with tiagabine therapy in children

A case of a 7-year-old male with epilepsy who developed non-convulsive status epilepticus (NCSE) with electroclinical features consistent with those of atypical absence seizures after adjunctive antiepileptic therapy of tiagabine (TGB) is reported. The patient had frequent generalised and rare partial seizures with generalised epileptic discharges on prior electroencephalogram (EEG) recordings. NCSE was developed when rapid dosage increase and high dose of TGB was given. This case emphasises the need for close monitoring of children with epilepsy taking TGB for exacerbation of seizures or development of NCSE.     

Epileptiform activity in the electroencephalogram of 6-year-old children of women with epilepsy

Purpose:To study the epileptiform discharges (EDs) in the electroencephalogram (EEG) of 6-8-year-old children of women with epilepsy (WWE).Results:Of the 185 children examined, 37 (20%) children (19 males, 18 females) had ED in their EEG. The EDs were generalized in 7 children, and focal in 30 children. The EDs were present in the sleep record only of 16 (43%) children and in the awake record only of 6 (16%) children. Out of the 94 children for whom seizure history was available, 7 children (7.4%) had seizures (neonatal seizures: 4, febrile seizure: 1, and single nonfebrile seizure: 2) and none had history of epilepsy or recurrent nonfebrile seizures. The odds ratio (OR) for occurrence of ED in the EEG was significantly higher for children of WWE [OR = 3.5, 95% confidence interval (CI) 2.3-6.0] when compared to the published data for age-matched children of mothers without epilepsy. There was no association between the occurrence of ED and the children''s maternal characteristics [epilepsy syndrome, seizures during pregnancy, maternal intelligence quotient (IQ)] or the children''s characteristics [antenatal exposure to specific antiepileptic drugs (AEDs), birth weight, malformations, IQ].Conclusion:Children of WWE have a higher risk of epileptiform activity in their EEG when compared to healthy children in the community though none had recurrent seizures.     

Persistent Nonconvulsive Status Epilepticus After the Control of Convulsive Status Epilepticus

Summary: Purpose: Convulsive status epilepticus (CSE) is a major medical and neurological emergency that is associated with significant morbidity and mortality. Despite this high morbidity and mortality, most acute care facilities in the United States cannot evaluate patients with EEG monitoring during or immediately after SE. The present study was initiated to determine whether control of CSE by standard treatment protocols was sufficient to terminate electrographic seizures. Methods: One hundred sixty-four prospective patients were evaluated at the Medical College of Virginia/VCU Status Epilepticus Program. Continuous EEG monitoring was performed for a minimum of 24 h after clinical control of CSE. SE and seizure types were defined as described previously. A standardized data form entry system was compiled for each patient and used to evaluate the data collected. Results: After CSE was controlled, continuous EEG monitoring demonstrated that 52% of the patients had no after-SE ictal discharges (ASIDS) and manifested EEG patterns of generalized slowing, attenuation, periodic lateralizing epileptiform discharges (PLEDS), focal slowing, and/or burst suppression. The remaining 48% demonstrated persistent electrographic seizures. More than 14% of the patients manifested nonconvulsive SE (NCSE) predominantly of the complex partial NCSE seizure (CPS) type (2). These patients were comatose and showed no overt clinical signs of convulsive activity. Clinical detection of NCSE in these patients would not have been possible with routine neurological evaluations without use of EEG monitoring. The clinical presentation, mortality, morbidity, and demographic information on this population are reported. Conclusions: Our results demonstrate that EEG monitoring after treatment of CSE is essential to recognition of persistent electrographic seizures and NCSE unresponsive to routine therapeutic management of CSE. These findings also suggest that EEG monitoring immediately after control of CSE is an important diagnostic test to guide treatment plans and to evaluate prognosis in the management of SE.     

Impact of stigma on the quality of life of patients with refractory epilepsy

PurposeTo assess the impact of perceived stigma on the quality of life of Bulgarian patients with refractory epilepsy.MethodsWe studied 70 adult patients with refractory epilepsy, without cognitive impairment, progressive somatic, neurological disease or recent seizures, and 70 patients with pharmacosensitive epilepsy. All participants completed a 3-item stigma scale, the patients with refractory epilepsy also completed a Health Related Quality of Life measure (the QOLIE-89).ResultsThe patients with refractory epilepsy had a mean disease duration 25.1 ± 1.3 years. 40.0% of patients (±5.9) had symptomatic epilepsy. Seventeen patients (24.2% ± 5.1) had partial seizures, 16 (22.8% ± 5.0) had generalized seizures and 37 (52.9 ± 6.0) had a mixture of partial and generalized seizures. Most participants had several seizures per week (45.7%) or month (30.0%) despite the fact that 90% were taking combination antiepileptic drug treatment. We found perceived stigma in 43.6% of patients with refractory epilepsy, and 28.7% self-reported severe stigmatization. Only 4 (5.7%) patients with pharmacosensitive epilepsy reported stigmatization which was mild or moderate in all cases. Perceived stigma had a negative impact on the overall score of the QOLIE-89 (T-score 47.8), as well as on all subscales of QOLIE-89, with the exception of “change in health” and “sexual relations”. Patients with refractory epilepsy reporting stigmatization most commonly had very low and low scores on the subscales “health perceptions” (82.9%), “emotional well-being” (71.5%), “memory” (63.4%) and “health discouragement” (62.5%). There was a negative correlation of all QOLIE-89 subscales with perceived stigma severity.ConclusionAll aspects of the quality of life of Bulgarian patients with refractory epilepsy correlate negatively with the severity of perceived stigma.