The SANTÉ study at 10 years of follow-up: Effectiveness, safety, and sudden unexpected death in epilepsy.

OBJECTIVE: We evaluated the efficacy and safety of deep brain anterior thalamus stimulation after 7 and 10 years, and report the incidence of sudden unexpected death in epilepsy (SUDEP) and overall mortality in adults in the Stimulation of the Anterior Nucleus of the Thalamus for Epilepsy (SANTÉ) study. METHODS: After the 3-month blinded and 9-month unblinded phases, subjects continued to be assessed during long-term follow-up (LTFU) and later a continued therapy access phase (CAP), to further characterize adverse events and the incidence of SUDEP. Stimulus parameter and medication changes were allowed. RESULTS: One hundred ten implanted subjects accumulated a total of 938 device-years of experience (69 subjects during the LTFU phase and 61 subjects in the CAP phase). Prior to study closure, 57 active subjects continued therapy at 14 study centers, with follow-up of at least 10 (maximum 14) years. At 7 years, median seizure frequency percent reduction from baseline was 75% (p < .001), with no outcome differences related to prior vagus nerve stimulation or resective surgery. The most severe seizure type, focal to bilateral tonic-clonic, was reduced by 71%. Adding new antiseizure medications did not impact the pattern of seizure reduction over time. There were no unanticipated serious adverse events in the study. The definite-plus-probable SUDEP rate, based on SANTÉ study experience (two deaths in 938 years) and previous pilot studies (0 deaths in 76 years), indicated a rate of 2.0 deaths for 1000 person-years. Overall mortality was 6.9 deaths per 1000 person-years. SIGNIFICANCE: The long-term efficacy and safety profiles of the deep brain stimulation (DBS) system for epilepsy are favorable and demonstrate stable outcomes. Improvement in frequency of the most severe seizure type may reduce SUDEP risk. The SUDEP rate with DBS (2.0) is comparable to other neuromodulation treatments (i.e., vagus nerve stimulation, responsive neurostimulation) for drug-resistant focal epilepsy.

Postictal generalized EEG suppression and respiratory dysfunction following generalized tonic-clonic seizures in sleep and wakefulness.

OBJECTIVE: Sudden unexpected death in epilepsy (SUDEP) is a common cause of death in epilepsy and frequently occurs following generalized tonic-clonic seizures (GTCS) in sleep. Postictal generalized electroencephalography (EEG) suppression (PGES), postictal immobility, and periictal respiratory dysfunction are potential risk factors for SUDEP. We sought to determine whether there was a difference in respiratory dysfunction, PGES, and postictal immobility for GTCS occurring during wakefulness or sleep. METHODS: We retrospectively analyzed video-EEG telemetry data in the epilepsy-monitoring unit. Patients' state at seizure onset and seizure characteristics were identified. Respiratory parameters and heart rate were recorded. Presence and duration of PGES and time to first postictal nonrespiratory movement were recorded. RESULTS: There were 165 seizures in 67 patients. There was no significant difference in the duration of postictal immobility in GTCS occurring out of wakefulness or sleep (p = 0.280). Oxygen desaturation nadir (p = 0.572) and duration of oxygen desaturation were not significantly different for GTCS starting during sleep or wakefulness (p = 0.992). PGES occurred more frequently when seizure onset was in sleep than in wakefulness (p = 0.004; odds ratio [OR] 2.760). There was no difference in the duration of PGES between the two groups. SIGNIFICANCE: PGES occurs more commonly after GTCS in sleep than in wakefulness but, in the epilepsy-monitoring unit (EMU), a patient's state at seizure onset does not affect the degree of respiratory dysfunction or duration of postictal immobility. In sleep, outside the hospital setting, GTCS are likely to go unnoticed. Postictal immobility in prone patients prevents head repositioning and unimpeded air exchange. A positive feedback cycle ensues with increasing respiratory distress, potentiating postictal immobility and PGES and eventually leading to asystole. Our findings suggest that the high incidence of nocturnal SUDEP may be related to the unsupervised environment during sleep rather than the severity of sleep-related respiratory dysfunction or PGES duration in the immediate postictal period.

Postictal immobility and generalized EEG suppression are associated with the severity of respiratory dysfunction.

OBJECTIVE: The pathophysiology of sudden unexpected death in epilepsy (SUDEP) remains undetermined. Seizures are accompanied by respiratory dysfunction (RD). Postictal generalized electroencephalography (EEG) suppression (PGES) may follow generalized tonic-clonic seizures (GTCS). Following GTCS patients have impaired arousal and may be motionless. Patients with SUDEP are usually prone. Postictal immobility (PI) may contribute to SUDEP by not permitting repositioning of the head to allow unimpeded ventilation. To determine whether RD and/or ictal characteristics are associated with PI, we analyzed patients with GTCS in the epilepsy monitoring unit. METHOD: We investigated for associations between PI duration and PGES, ictal/postictal oxygen saturation (SpO2 ), end-tidal CO2 (ETCO2 ), seizure localization, duration, and tonic and total convulsive phase duration. We investigated for linkage between PGES and these measures. RESULTS: Seventy patients with 181 GTCS and available SpO2 and/or ETCO2 data were studied. Simple linear regression analysis by seizures showed that PI duration was associated with peak periictal ETCO2 (p = 0.03), duration of oxygen desaturation (p = 0.005) and with SpO2 nadir (p = 0.02). PI duration was not associated with tonic, convulsive phase or total seizure duration. Analysis by patients also showed significant association of PI with RD. Duration of PI was longer following seizures with PGES (p < 0.001). PGES was not associated with the tonic, convulsive phase or total seizure duration. SpO2 nadir was lower in seizures with PGES (p = 0.046), ETCO2 peak change (p = 0.003) was higher, and duration of ETCO2 elevation (p = 0.03) was longer. Multivariable regression analysis showed that PGES and severe RD were associated with PI duration. SIGNIFICANCE: The duration of PI and presence of PGES are associated with periictal RD. The duration of PI is also associated with the presence of PGES. Seizure duration or duration of the convulsive phase is not associated with PI or PGES. Interventions aimed at reversing impaired arousal and PI may reduce SUDEP risk.

Who to target in sudden unexpected death in epilepsy prevention and how? Risk factors, biomarkers, and intervention study designs.

The risk of dying suddenly and unexpectedly is increased 24- to 28-fold among young people with epilepsy compared to the general population, but the incidence of sudden unexpected death in epilepsy (SUDEP) varies markedly depending on the epilepsy population. This article first reviews risk factors and biomarkers for SUDEP with the overall aim of enabling identification of epilepsy populations with different risk levels as a background for a discussion of possible intervention strategies. The by far most important clinical risk factor is frequency of generalized tonic-clonic seizures (GTCS), but nocturnal seizures, early age at onset, and long duration of epilepsy have been identified as additional risk factors. Lack of antiepileptic drug (AED) treatment or, in the context of clinical trials, adjunctive placebo versus active treatment is associated with increased risks. Despite considerable research, reliable electrophysiologic (electrocardiography [ECG] or electroencephalography [EEG]) biomarkers of SUDEP risk remain to be established. This is an important limitation for prevention strategies and intervention studies. There is a lack of biomarkers for SUDEP, and until validated biomarkers are found, the endpoint of interventions to prevent SUDEP must be SUDEP itself. These interventions, be they pharmacologic, seizure-detection devices, or nocturnal supervision, require large numbers. Possible methods for assessing prevention measures include public health community interventions, self-management, and more traditional (and much more expensive) randomized clinical trials.

Adult tonic-clonic convulsive status epilepticus over the last 11 years in a resource-poor country: a tertiary referral centre study from southern Thailand.

Status epilepticus is a common condition in patients admitted to hospital in resource-poor countries and reports indicate that aetiology, factors of poor outcome, and treatment strategies are variable. To date, there is no report of a prospective study in Thai adults. Herein, we investigated the aetiology, clinical features, factors of predicted poor outcome, and treatment strategies in Thai adult patients who presented with convulsive status epilepticus. A total of 180 patients, whose ages ranged from 15 to 106 years, were included. Of these, 121 patients (67.2%) had acute symptomatic aetiology. The most common aetiology of status epilepticus was encephalitis (36.1%), followed by scarring of the cerebral hemisphere (15%). The median duration of status epilepticus before treatment was three hours. The rate of mortality in the study was 26.7%. Poor outcome was identified in 112 (62.2%) patients. For referral patients, all received only intravenous drugs before referral. The variables that correlated with poor outcome were aetiology and duration of status epilepticus. An approach to incorporate improved prevention of encephalitis, a more effective transportation system, and provision of the essential intravenous antiepileptic drugs would effectively increase the response to treatment.

Seizure Analysis Presented to Emergency Department in Saudi Arabia: New VS Chronic Cases.

Background: Epilepsy, characterized by recurrent unprovoked seizures, poses a significant global burden on individuals and healthcare systems. Accurate identification of underlying causes is vital for optimal intervention. However, studies reveal a lack of standardized approaches, potentially resulting in unnecessary investigations. Objective: We aimed to highlight the importance of avoiding unnecessary testing to minimize healthcare costs and resource waste. Methods: In the Emergency Department of King Fahd Hospital of the University (KFUH) in Alkhobar, a retrospective cross-sectional study encompassed 190 patients presenting with seizures from January 1, 2020, to December 31, 2022. The study aimed to elucidate the epidemiological profile and distinguish clinical and demographic factors between new onset seizures and known cases. Results: The study included 190 epilepsy cases, with 51.1% known and 48.9% new onset. Generalized tonic-clonic seizures were prominent (43.2%), and non-compliance (24.2%) was a leading cause. New onset seizures were associated with abnormal CT findings (p=0.025), drug use (74.2%), and intoxication (6.5%). Demographically, Saudis showed higher new onset prevalence (82.8%, p=0.001). Conclusion: The average length of stay was 5.93 hours, and the distribution of new vs. known cases was nearly equal among the 190 patients. Laboratory findings showed no significant associations with either group, mostly falling within the normal range. To optimize care further, we recommend continued refinement of protocols, emphasis on medication compliance.

Benign afebrile convulsions in the course of mild acute gastroenteritis: a study of 28 patients and a literature review.

OBJECTIVES: Since the description of afebrile convulsions in the course of mild acute gastroenteritis (AGE) in 1982 by Morooka in Japan, there have been few reports of further cases outside Asia. The aim of this study was to share our casuistry--from a non-Asian country. METHODS: This is a retrospective study of identified cases in our center from January 2002 to December 2007. RESULTS: A total of 28 patients were studied. All were previously healthy patients who experienced convulsions with mild AGE without dehydration and with normal blood analysis. The mean age was 17.25 months (range, 6-48 months), with 93% younger than 24 months. Seizures were generalized tonic-clonic (61%), followed by generalized tonic (31%), and hypotonic (5.2%), with 2 (2.6%) partial. Only 8 patients (28.6%) presented one convulsion, and in 13 patients (46%), the seizures were in clusters from 3 to 6. Eleven patients (39%) presented 2 different types of convulsion. The duration of the crises ranged from 30 seconds to 10 minutes, and all of them occurred within 24 hours of the first. Electroencephalograms, obtained for all patients, were normal. Rotavirus was the main infectious agent in the AGEs, found in 11 patients with 22 determinations. In one patient, Salmonella serotype Enteritidis was isolated. All of the patients developed favorably, with no sequelae or epilepsy during the follow-up period. CONCLUSIONS: Afebrile convulsion in the course of mild gastroenteritis exists in our environment. It is a banal symptom in the course of the disease with good prognosis. Recognition of this fact may help avoid needless explorations and treatment in patients of this kind.

[Oligoepilepsy: a real entity or the benign form of epileptic disorder?]. / Oligoepilepszia: onálló entitás vagy az epilepsziás készség benignus formája?

BACKGROUND AND PURPOSE: Although oligoepilepsy (OLE) is a used term in many protocols, guidelines and the everyday routine, it is found practically nowhere in the scientific literature. The aim of our study is to investigate and evaluate of the main characteristics of his subcategory of epilepsy. We try to find answer to the basic question of not only theoretical but also great practical importance whether the OLE does really exist, is it an independent entity of epilepsy or only its general benign clinical presentation. METHODS: We considered OLE if the patients had two seizures maximally in the last year of their course. We counted only the two most severe clinical types, the generalized tonic-clonic and the complex partial seizures. We divided the OLE into two subtypes: those patients who had OLE from the beginning of their epilepsy (OLE1) and those in whom the OLE was the result of the treatment (OLE2). We retrospectively analysed the data of 817 "OLE-suspicious" patients taken from our EPIMED database. RESULTS: We found 47 patients met the inclusion criteria (OLE1 = 34, OLE2 = 13). OLE patients did not differ from the general epileptic population according to the age and gender, the type of seizures, the electro-clinical diagnosis and the possible cause of their first seizure. But we found statistically significant differences in two measures. In OLE, far less seizure provoking factors were found in the sporadic seizures. Concerning the social conditions: while the range of employees was equal, the vast majority of OLE patients were able to work at their level of education. CONCLUSION: We found that more than 5% of people with epilepsy can belong to the OLE category. In the majority of OLE patients the seizure frequency is low from the beginning of the disease. The sporadic seizures in OLE are generally not provoked. The chances of OLE patients in the world of job are better for the OLE patients. In our opinion OLE rather seems to be an independent entity among epilepsy. Therefore larger prospective studies are needed to the exact description of OLE and to establish the special standards for the everyday medical practice.

Initiating an epilepsy surgery program with limited resources in Indonesia.

To share the experiences of organizing the epilepsy surgery program in Indonesia. This study was divided into two periods based on the presurgical evaluation method: the first period (1999-2004), when interictal electroencephalogram (EEG) and magnetic resonance imaging (MRI) were used mainly for confirmation, and the second period (2005-2017), when long-term non-invasive and invasive video-EEG was involved in the evaluation. Long-term outcomes were recorded up to December 2019 based on the Engel scale. All 65 surgical recruits in the first period possessed temporal lobe epilepsy (TLE), while 524 patients were treated in the second period. In the first period, 76.8%, 16.1%, and 7.1% of patients with TLE achieved Classes I, II, and III, respectively, and in the second period, 89.4%, 5.5%, and 4.9% achieved Classes I, II, and III, respectively, alongside Class IV, at 0.3%. The overall median survival times for patients with focal impaired awareness seizures (FIAS), focal to bilateral tonic-clonic seizures and generalized tonic-clonic seizures were 9, 11 and 11 years (95% CI: 8.170-9.830, 10.170-11.830, and 7.265-14.735), respectively, with p = 0.04. The utilization of stringent and selective criteria to reserve surgeries is important for a successful epilepsy program with limited resources.

Focal clinical and electroencephalographic features in patients with juvenile myoclonic epilepsy.

OBJECTIVE: To identify prevalence and factors associated with occurrence of focal clinical and electroencephalogram (EEG) abnormalities in patients with juvenile myoclonic epilepsy (JME). MATERIALS AND METHODS: Clinical asymmetries in the seizures and focal EEG abnormalities were analyzed in 266 patients with JME. RESULTS: All the patients had myoclonic jerks (MJ) and generalized tonic-clonic seizures (GTCS); 56 (21%) had absence seizures. Asymmetry in clinical seizures was reported in 45 (16.9%) and focal EEG abnormalities were noted in 92 (45.5%) patients. Amplitude asymmetry or focal onset of generalized discharges was noted in 41 (44.6%) and independent focal EEG abnormalities in 30 (32.6%) patients. A statistically significant association was seen with the presence of GTCS and MJ (P = 0.007), a family history of epilepsy (P = 0.001) and drug resistance (P = 0.04) and the occurrence of focal EEG abnormalities. CONCLUSION: Patients with JME showed focal clinical and EEG features. These features should not be misinterpreted as indicative of partial epilepsy.

Outcome predictors for surgical treatment of temporal lobe epilepsy with hippocampal sclerosis.

PURPOSE: To study long-term postoperative course and identify predictors for postoperative seizure control in patients with medically intractable temporal lobe epilepsy (TLE) associated with hippocampal sclerosis (HS), diagnosed by magnetic resonance imaging (MRI), and ascertained histopathologically. To compare patients becoming seizure-free (i.e., cured from epilepsy) and patients experiencing prolonged seizure-free periods interposed with recurring seizures. METHODS: One hundred thirty-five patients (74 women) underwent complete evaluation for epilepsy surgery. The predictive value of duration of epilepsy, age at onset, age at surgery, gender, febrile convulsion history, ictal dystonic posturing, unilateral interictal electroencephalography (EEG) discharges (IED), preoperative secondarily generalized tonic-clonic seizures (SGTCS), and preoperative seizure frequency for short- and long-term postoperative seizure control were evaluated with two classification systems: Classification 1 (seizure-freedom with or without auras during 12-months before observation points) and the stringent classification 2 [International League Against Epilepsy (ILAE) Ia; absolute absence of seizures and auras after operation]. RESULTS: Unilateral IED at year 1 and 2 (p = 0.037 and p = 0.034), male gender and low seizure frequency at year 2 (p = 0.013 and p = 0.046) were significant predictors for seizure freedom using classification 1. All variables (except male gender at year 2; p = 0.035) lost their predictive power, applying classification 2. The proportion of seizure-free patients remained stable between 70% to 79% with classification 1, but decreased from 64.4% at year 1 to 45.8% at year 5 with classification 2. DISCUSSION: Positive predictors of short-term outcome do not predict long-term outcome in patients with TLE associated with HS. Absolute freedom of seizures and auras cannot be predicted by conventional preoperative variables.

Clinical description of seizures in patients with systemic lupus erythematosus.

OBJECTIVE: To evaluate the frequency of and risk factors for epileptic seizures in patients with systemic lupus erythematosus (SLE) in a large cohort series. METHODS: One thousand two hundred patients with SLE were analyzed. The type and frequency of risk factors for seizures associated with SLE were studied and compared with two other series reported in the literature. RESULTS: One hundred and forty-two patients had seizures. Seventy-five patients were studied with a mean follow-up of 5 years from the first seizure episode. Fifty-eight (77%) patients had tonic-clonic seizures, 9 (12%) complex partial seizures (PS), 5 (7%) simple partial motor seizures and 3 (4%) secondary tonic-clonic seizures. In 41 (54%) patients, the seizures occurred within the first year of the SLE diagnosis. Recurrence occurred in 40 (53%) patients, and was associated with PS in 14 (35%; p = 0.006) and time of seizures with SLE onset in 5 (12.5%; p = 0.05). Less than one third of the patients had positive antiphospholipid antibodies. A concurrent infection was present in 16 (21%) patients. CONCLUSIONS: Epileptic seizures were more common during the first year after SLE diagnosis. Neither infection nor antiphospholipid syndrome was associated with the occurrence of seizures.

[How do temporal lobe seizures change by age?]. / Hogyan változnak a temporalislebeny-rohamok az életkorral?

Seizure semiology describes different - motor, sensory, autonomic, etc. - aspects of epileptic seizures. Several semiological studies showed already that different epilepsies - especially temporal lobe epilepsy - contain age-dependent features. In our researches, we tried to assess these subjective aspects with as objective methods as possible. We gave a comprehensive (preictal, ictal, and postictal) description of seizure semiology in patients fulfilling the gold standard criteria of semiological studies: being seizure free after temporal lobe resections. Our studies based on a large population, assess epileptic features at different levels of brain maturation. They help to understand why certain semiological axes show special characteristics at different ages. In this review, I summarize the most important results of our seizure semiology studies in temporal lobe epilepsy.

Sudden unexpected death in epilepsy patients: Risk factors. A systematic review.

INTRODUCTION: Several risk factors for sudden unexplained death in epilepsy patients (SUDEP) have been proposed, but subsequent work has yielded conflicting data. The relative importance of various risk factors for SUDEP was never explored. The aim of this study is to review systematically risk factors for SUDEP and also to determine their relevance for SUDEP by calculating relative risk factor ratios. METHODS AND MATERIALS: Authors performed a literature-search on "SUDEP" in Medline, the Cochrane Library and EMBASE. Studies with unknown number of SUDEP cases or with less than five SUDEP cases and reviews were excluded from further analysis. The value of each paper was assessed, based on the quality of the study and the reliability of the diagnosis of SUDEP. This value ranged from 1 (low quality) to 10 (high quality). Papers with a value below 7 were eliminated for further analysis. For each analysed factor, a risk factor ratio was determined, with a higher ratio for a stronger risk factor. RESULTS: A number of strong risk factors for SUDEP: young age, early onset of seizures, the presence of generalized tonic clonic seizures, male sex and being in bed. Weak risk factors for SUDEP: prone position, one or more subtherapeutic bloodlevels, being in the bedroom, a strucural brain lesion and sleeping. CONCLUSIONS: In this study, authors have designed a quality scale to select papers. The relative importance of risk factors for SUDEP is demonstrated.

Treatment of pediatric epilepsy: European expert opinion, 2007.

BACKGROUND: Childhood epilepsies are a heterogeneous group of conditions that differ in diagnostic criteria and management and have dramatically different outcomes. Despite increasing data on treatment of epilepsy, research findings on childhood epilepsy are more limited and many clinical questions remain unanswered, so that clinicians must often rely on clinical judgment. In such clinical situations, expert opinion can be especially helpful. METHODS: A survey on pediatric epilepsy and seizures (33 questions and approximately 650 treatment options) was sent to 57 European physicians specializing in pediatric epilepsy, 42 (74%) of whom completed it. In some questions, the experts were asked to recommend overall treatment approaches for specific syndromes (the order in which they would use certain strategies). Most of the questions asked the experts to rate options using a modified version of the RAND 9-point scale for medical appropriateness. Consensus was defined as a non-random distribution of scores by chi-square test, with ratings used to assign a categorical rank (first line/usually appropriate, second line/equivocal, and third line/usually not appropriate) to each option. RESULTS: Valproate was treatment of choice for symptomatic myoclonic and generalized tonic-clonic seizures. For initial monotherapy for complex partial seizures, carbamazepine and oxcarbazepine were treatments of choice, with valproate also first line. As initial therapy for infantile spasms caused by tuberous sclerosis, viagabatrin was treatment of choice. As initial therapy for infantile spasms that are symptomatic in etiology, vigabatrin was also treatment of choice, with adrenocorticotropic hormone (ACTH) and prednisone other first-line options. As initial therapy for Lennox-Gastaut syndrome, valproate was treatment of choice. For acute treatment of a prolonged febrile seizure or cluster of seizures, rectal diazepam was treatment of choice. Valproate was treatment of choice as preventive therapy for febrile seizures. For benign childhood epilepsy with centro-temporal spikes, valproate was treatment of choice. For childhood and juvenile absence epilepsy, valproate was treatment of choice, with lamotrigine another first-line option (ethosuximide was another first-line option for childhood absence epilepsy). For juvenile myoclonic epilepsy in adolescent males, valproate was treatment of choice, with lamotrigine another first-line option; for juvenile myoclonic epilepsy in adolescent females, lamotrigine was treatment of choice, with valproate another firstline option. As initial therapy for neonatal status epilepticus, intravenous (IV) phenobarbital was treatment of choice. As initial therapy for all types of pediatric status epilepticus, IV diazepam was treatment of choice. For generalized tonic-clonic status epilepticus, rectal diazepam and IV lorazepam were also treatments of choice; for complex partial status epilepticus, IV lorazepam was another first-line option. CONCLUSION: The expert panel reached consensus on many treatment options. Within the limits of expert opinion and with the understanding that new research data may take precedence, the experts' recommendations provide helpful guidance in situations where the medical literature is scant or lacking. The information in this report should be evaluated in conjunction with evidence-based findings.

Outcome of children with juvenile absence epilepsy.

The incidence and natural history of childhood absence epilepsy are well documented, but those of juvenile absence epilepsy are poorly delineated. We conducted a retrospective chart study to evaluate the incidence and outcome of patients with juvenile absence epilepsy by retrieving the medical records of consecutive patients with juvenile absence epilepsy who were evaluated in three pediatric neurology outpatient clinics in Israel. Inclusion criteria included the onset of epilepsy after the age of 10 years and follow-up until at least 15 years of age. The patients with an electroencephalogram (EEG) suggestive of myoclonic epilepsy (polyspike and wave) were excluded from the study. Seventeen patients (10 female and 7 male) fulfilled the inclusion criteria for juvenile absence epilepsy. They presented with epilepsy at a mean age of 11.94 years (range 10-16.5 years). The mean duration of follow-up was 6.05 years (range 2-12 years). Five patients (29.4%) had a family history of epilepsy. All 17 patients had a normal neurodevelopmental status. Eight patients (47%) experienced generalized tonic-clonic seizures. At follow-up, eight patients (43.7%) were seizure free. Only three (37.5%) of the patients who experienced generalized tonic-clonic seizures were seizure free during follow-up compared with five (55.5%) patients without generalized tonic-clonic seizures. Our results indicate that the outcome of patients with juvenile absence epilepsy is less favorable than children with childhood absence epilepsy and that the presence of generalized tonic-clonic seizures is a predictor for poorer outcome.

Predicting seizure frequency after epilepsy surgery.

OBJECTIVE: To identify clinical features related to seizure frequency after epilepsy surgery in patients with recurrent seizures. BACKGROUND: No studies have examined the differences between patients who have rare seizures and patients who experience frequent seizures after epilepsy surgery. Since seizure frequency correlates with morbidity and quality of life, it is desirable to know which preoperative clinical features predict postoperative seizure frequency. METHODS: Patients with recurrent seizures were placed in two categories: rare postoperative seizures (< or =2 per year) and frequent postoperative seizures (> or =12 per year) using seizure frequency in the second postoperative year. Variables included preoperative seizure frequency, age of first risk, age at first seizure, epilepsy duration, age at surgery, history of febrile convulsions, tonic-clonic seizures, status epilepticus, or family history, IQ, magnetic resonance imaging (MRI), and positron emission tomography (PET). Variables were analyzed using non-parametric tests to assess relationship to postoperative seizure frequency. RESULTS: Of 475 patients who had epilepsy surgery, 111 had rare or frequent seizures in the second postoperative year. After anterior temporal lobectomy (ATL), age of first risk< or =5 years and presence of mesial temporal sclerosis on MRI were associated with rare seizures (66% of patients), whereas lack of these risk factors was associated with frequent seizures (75% of patients) (p<0.03). For non-ATL operations, preoperative seizure frequency of > or =20 seizures per month was associated with frequent postoperative seizures (p=0.03). No other variables influenced outcome. CONCLUSIONS: Some preoperative clinical features correlate with postoperative seizure frequency in patients with recurrent seizures after epilepsy surgery. This has implications for the surgical decision making process and early postoperative management.

Generalized tonic-clonic status epilepticus in the elderly in China.

The proportion of elderly people in China is projected to increase rapidly but there is limited information on status epilepticus (SE) in this population. We evaluated retrospectively the etiology, response to treatment, outcome and predictors of mortality in a group of elderly patients with generalized tonic-clonic SE in Hong Kong, China. Factors for increased mortality were analyzed using a logistic regression model. Of the 80 acute admissions for SE from two large urban hospitals over a seven-year period, 1996-2002, the two leading causes were attributed to cerebral infarct (n=28, 35%) and cerebral haemorrhage (n=14, 17.5%). The mean age was 74.2 years (range 60-93 years). At six months from the onset of seizures, 26 patients (32.5%) had made a good recovery but another 28 (35%) had died. Results showed that mortality was associated with increasing age (OR 1.08, 95% CI 1.01-1.16) and SE due to an acute symptomatic disturbance (OR 4.90, 95% CI 1.17-13.67). SE is associated with significant morbidity and mortality in this age group.

Long-term outcomes of generalized tonic-clonic seizures in a childhood absence epilepsy trial.

OBJECTIVE: To determine incidence and early predictors of generalized tonic-clonic seizures (GTCs) in children with childhood absence epilepsy (CAE). METHODS: Occurrence of GTCs was determined in 446 children with CAE who participated in a randomized clinical trial comparing ethosuximide, lamotrigine, and valproate as initial therapy for CAE. RESULTS: As of June 2014, the cohort had been followed for a median of 7.0 years since enrollment and 12% (53) have experienced at least one GTC. The median time to develop GTCs from initial therapy was 4.7 years. The median age at first GTC was 13.1 years. Fifteen (28%) were not on medications at the time of their first GTC. On univariate analysis, older age at enrollment was associated with a higher risk of GTCs (p=-0.0009), as was the duration of the shortest burst on the baseline EEG (p=0.037). Failure to respond to initial treatment (p<0.001) but not treatment assignment was associated with a higher rate of GTCs. Among patients initially assigned to ethosuximide, 94% (15/16) with GTCs experienced initial therapy failure (p<0.0001). A similar but more modest effect was noted in those initially treated with valproate (p=0.017) and not seen in those initially treated with lamotrigine. CONCLUSIONS: The occurrence of GTCs in a well-characterized cohort of children with CAE appears lower than previously reported. GTCs tend to occur late in the course of the disorder. Children initially treated with ethosuximide who are responders have a particularly low risk of developing subsequent GTCs.

Análisis del impacto familiar y necesidades del síndrome de Dravet en España / Analysis of the family impact and needs of Dravet's syndrome in Spain

Introducción: El síndrome de Dravet (SD) es una encefalopatía epiléptica, iniciada en la infancia, con un gran impacto en la vida de los pacientes y los familiares. Actualmente se necesitan mejoras en su diagnóstico y tratamiento: existen dos fármacos aprobados para el tratamiento del SD en Europa, aunque hay nuevos tratamientos en desarrollo o en vías de comercialización próximamente. Objetivos: Comprender la situación del SD en España e identificar las oportunidades de mejora. Sujetos y métodos: Análisis de los datos de una macroencuesta europea en la que los cuidadores de pacientes con SD manifestaron su experiencia con la enfermedad. Resultados: Datos de 57 familias con hijos con SD (edad media: 9 años). El tiempo hasta el diagnóstico, generalmente tras otro erróneo (80%), se incrementa en los pacientes de mayor edad (el 80% de los adultos: retraso diagnóstico > 4 años). La demora induce un mayor uso de fármacos antiepilépticos contraindicados. Las crisis (87% de los casos; las más frecuentes, tonicoclónicas: 90%) y las hospitalizaciones (60% de los casos) continúan hasta la edad adulta. La gravedad de la enfermedad y el número de hospitalizaciones se correlacionan con el impacto en los cuidadores y la familia. La eficacia de los tratamientos y el futuro de los pacientes son las mayores preocupaciones. Conclusiones: Para mejorar el manejo y la calidad de vida de los pacientes con SD y los familiares, es necesario un diagnóstico temprano y la incorporación de nuevos tratamientos que ayuden al control de las crisis epilépticas y de las comorbilidades de la enfermedad

Introduction: Dravet's syndrome (DS) is an epileptic encephalopathy that starts in infancy and has an important impact on the lives of patients and their relatives. There is currently a need for improvement in diagnosis and treatment: two drugs have been approved for the treatment of DS in Europe, although new treatments are under development or are scheduled for commercialisation soon. AIMS. To understand the situation of DS in Spain and to identify opportunities for improvement. Subjects and methods: The study will involve an analysis of data from a European macro-survey in which carers of patients with DS expressed their experience with the disease. Results: Data from 57 families with children with DS (mean age: 9 years). The time to diagnosis, usually after another misdiagnosis (80%), increases in older patients (80% of adults: diagnostic delay > 4 years). The delay induces an increased use of contraindicated antiepileptic drugs. Seizures (87% of cases; the most frequent, tonic-clonic: 90%) and hospitalisations (60% of cases) continue into adulthood. The severity of the illness and the number of hospitalisations correlate with impact on caregivers and family. The effectiveness of treatments and the future of patients are the greatest concerns. Conclusions: In order to improve the management and quality of life of patients with DS and their families, it is necessary to have an early diagnosis and to incorporate new treatments that help to control the epileptic seizures and the comorbidities of the disease