Symptomatic Etiology Research Articles

Electrical status epilepticus in sleep (ESES)/continuous spikes and waves during slow sleep (CSWS) syndrome in children: An electroclinical evaluation according to the EEG patterns.

The aim of this study was to describe the electroclinical spectrum in children with electrical status epilepticus in sleep (ESES)/continuous spikes and waves during slow sleep (CSWS) syndrome according to the EEG patterns. Clinical data of 44 patients with ESES/CSWS syndrome who were treated and followed at least two years were analyzed. Records of EEGs of patients were reevaluated to determine two aspects of the ESES pattern: (1) the spike-wave index (SWI) on the NREM sleep EEG (Group I: typical vs. atypical ESES pattern (33/11 patients)) and (2) the area of maximum amplitude of continuous epileptic activity (Group II: anterior vs. posterior ESES pattern (33/11 patients)). Symptomatic etiology was more defined in patients with the typical ESES pattern (40%) than the group with the atypical ESES pattern (9%) by a factor of four. All patients were receiving at least two antiepileptic drug (AED) treatments. Eighteen patients (41%) received AEDs plus ACTH therapy. Complete disappearance of the ESES pattern on the EEG was observed in 18 patients (41%), more than 50% reduction was observed in five patients (11%), less than 50% reduction was observed in eight patients (18%), and no response was observed in five patients (11%). No significant difference was found when comparing the groups in terms of reduction of seizures and the SWI. Seizure outcome at the two-year follow-up was similar between the group with ESES treated with AEDs plus ACTH and the group with ESES treated with AEDs without ACTH therapy. This study demonstrated that the rate of the SWI (typical vs. atypical ESES) and the maximum amplitude of the ESES pattern (anterior vs. posterior) have no significant correlation with seizure control and reduction of the SWI on the EEG in children with ESES syndrome.

Predictors of Poor Seizure Control in Children Managed at a Tertiary Care Hospital of Eastern Nepal.

Various factors have been claimed to predict outcome of afebrile seizures in children. This study was aimed to find out the predictors of poor seizure control in children at a resource limited setting. This prospective study was done from July 1st, 2009 to January 31st, 2012 at B.P. Koirala Institute of Health Sciences, Nepal. Children (1 month-20 yr of age) with afebrile seizures presenting to pediatric neurology clinic were studied. Significant predictors on bivariate analysis were further analyzed with binary logistic model to find out the true predictors. Positive predictive values (PPVs) and negative predictive values (NPVs) for the true predictors were calculated. Out of 256 patients (male: female ratio 3:2) with afebrile seizures followed up for median duration of 27 (IQR 12-50) months, seizure was poorly controlled in 20% patients. Three factors predicted poor seizure control. They were frequent (≥1 per month) seizures at onset (OR 12.76, 95% CI 1.44-112.73, PPV 25%, NPV 98%); remote symptomatic etiology (OR 3.56, 95% CI 1.04-12.17, PPV 36%, NPV 92%); and need of more than one anticonvulsant drug (polytherapy) (OR 12.83, 95% CI 5.50-29.9, PPV 56%, NPV 96%). The strongest predictor was need of polytherapy. When all three factors were present, PPV and NPV for prediction of poor seizure control were 70% and 90% respectively. Frequent seizures at onset, remote symptomatic etiology of seizure and need of polytherapy were associated with poor seizure control in children with afebrile seizures.

“Eating” epilepsy revisited- an electro-clinico-radiological study

This study aimed to evaluate the clinical, video electroencephalographic and MRI attributes of patients with eating epilepsy (EE). Consecutive patients who were diagnosed with EE and underwent potential pre-surgical work-up from 2003 to 2012 formed the study cohort. Their electro-clinico-radiological and seizure outcome data were obtained from our prospectively maintained medical records. Out of 7094 patients who underwent evaluation for refractory seizures, 47 patients satisfied the criteria for EE. Twenty-three (48.9%) had exclusive EE; the remainder had a combination of predominantly eating-induced and unprovoked seizures with no differences noted in timing of seizures in relation to meals. Lesional epilepsy was seen in 34% of patients, with posterior cortex (PC; posterior temporo-parieto-occipital) predominance. In MRI negative patients, PC interictal epileptiform discharges were present in 34.4% of patients and multifocal in 20.6% of patients compared to the MRI positive group with 12.5% and 6.5%, respectively (p=0.003). Among 24 patients (51.1%) with co-existent unprovoked seizures, developmental delay and PC ictal onset was more prevalent (p=0.013 and 0.029) as compared to exclusive EE. The seizure frequency and outcome did not significantly differ between patients with or without MRI abnormality. Two patients underwent anterior temporal lobectomy, with persistence of their eating seizures postoperatively. EE is a complex reflex epilepsy of cryptogenic and symptomatic etiology. As opposed to the traditionally implied temporo-limbic mechanisms behind epileptogenesis, a multilobar network originating from the PC receiving sensory and visual inputs linked to the limbic-opercular pathways represents a plausible mechanism. Surgical selection should be diligent and cautious in this group of patients.

An audit of the predictors of outcome in status epilepticus from a resource-poor country: a comparison with developed countries.

Status epilepticus is a neurological emergency with significant morbidity and mortality. This study describes the clinical profile, treatment, and predictors of outcome of status epilepticus in a tertiary referral centre in a developing country and aims to highlight the similarities and differences from data available from the western world. A retrospective analysis of data of patients treated for status epilepticus was conducted from prospectively maintained records, between January2000 and September2010. The demographic data, clinical profile and investigations (including neuroimaging and EEG), aetiology, treatment, and outcomes were studied and compared with data available from the western world. The analysis included 108 events in 84 patients. A single episode of status epilepticus was treated in 72 patients (86%) and multiple status epilepticus events, ranging from two to six per patient, were managed in 12 patients (14%). Mean age was 24.1±20.3 years and 63% were males. The types of status epilepticus included convulsive status in 98 (90.7%), non-convulsive status in seven (6.5%), and myoclonic status in three (2.8%). The majority of events (60%) were remote symptomatic, 16% were acute symptomatic, 16% were of unexplained aetiology, and 8% were progressive symptomatic. In 85 events (79%), status epilepticus could be aborted with first and second-line drugs. The remaining 23 events (21%) progressed to refractory status epilepticus, among which, 13 (56%) were controlled with continuous intravenous midazolam infusion. Case fatality rate was 11%, neurological sequelae were reported in 22%, and 67% returned to baseline. Acute symptomatic status, older age, altered sensorium at the time of admission, and delayed hospitalisation were predictors of poor outcome. Aetiology was the most important determinant of outcome of status epilepticus, as in reports from the western world, with remote symptomatic aetiology secondary to gliosis being the most common. Treatment delay was frequent and adversely affected the outcome.

Refractory and super-refractory status epilepticus in adults: a 9-year cohort study.

While status epilepticus (SE) persisting after two antiseizure agents is called refractory (RSE), super-refractory status epilepticus (SRSE) defines SE continuing after general anaesthesia. Its prevalence and related clinical profiles have received limited attention, and most studies were restricted to intensive care facilities. We therefore aimed at describing RSE and SRSE frequencies and identifying associated clinical variables. Between 2006 and 2015, consecutive adult SE episodes were prospectively recorded in a registry. Occurrence of RSE and SRSE and their relationship to clinical variables of interest, including outcome, were analysed. Of 804 SE episodes, 268 (33.3%) were RSE and 33 (4%) SRSE. Coma induction for SE treatment occurred in 79 (9.8%) episodes. Severe consciousness impairment (OR 1.67; 95% CI 1.24-2.46; P = 0.001), increasing age (OR 1.01, 95% CI 1.01-1.02), and lack of remote symptomatic SE aetiology (OR 0.48; 95% CI 0.32-0.72) were independently associated with RSE, while severe consciousness impairment (OR 4.26; 95% CI 1.44-12.60) and younger age (OR 0.96; 95% CI 0.95-0.99) correlated with SRSE; however, most SRSE episodes were not predicted by these variables. Mortality was 15.5% overall, higher in RSE (24.5%) and SRSE (37.9%) than in non-refractory SE (9.8%) (P < 0.001). Super-refractory status epilepticus appears clearly less prevalent in this cohort than previously reported, probably as it is not restricted to intensive care unit. SRSE emerges in younger patients with marked consciousness impairment, pointing to the underlying severe clinical background, but these variables do not predict most SRSE developments. There is currently a knowledge gap for prediction of SRSE occurrence that needs to be filled.

Clinical profile and outcome of refractory convulsive status epilepticus in older children from a developing country

PurposeThe current study evaluates the etiology, clinical course and outcome of refractory convulsive status epilepticus (CSE) in older children. MethodsRetrospective analysis of data of 73 children with CSE, aged ≥2 and ≤12 years was performed. Odds ratios were calculated between variables for clinical course and outcome. Mortality of the group was analyzed using survival analysis. ResultsThirty three (45.2%) children progressed to refractory status epilepticus (RSE). The most common etiology for CSE was acute symptomatic in 44 (60.3%) of which 37 had presumed CNS infections. The odds of progressing to RSE were higher in children with acute symptomatic etiology (OR 2.62; CI – 95%; 0.99–7.14; p=0.041). Progression to RSE increased the chances of severe sepsis by six times (OR 6.08; CI – 95%; 1.19–31.02; p=0.036) and acidosis by nearly 15 times (OR 14.77; CI – 95%; 1.19–31.02; p=0.020). Overall mortality was 13.7%, higher in RSE (21.2% vs.7.5%). Amongst the 63 surviving children followed for 1 year from discharge, progression to RSE increased the odds of disability by seven times (OR 7.08; CI 29.31; p=0.004). ConclusionAcute symptomatic etiology was the commonest cause of CSE among older children from developing country and increased the odds of progressing to RSE. RSE was significantly associated with disability at 1 year from discharge.

Causes and outcomes of new onset status epilepticus and predictors of refractoriness to therapy

We aimed to evaluate the determinants of outcome in new onset refractory status epilepticus (SE). A retrospective analysis of patients with new onset SE admitted between May 2005 and October 2013 was performed. Regression analysis was used to determine factors that affect progression of new onset SE to refractory status epilepticus (RSE) and mortality. Among 114 patients with new onset SE, 52 patients progressed to RSE. Sixty seven (58.7%) were men. New onset RSE patients were younger than new onset SE patients (mean 35.9±standard deviation18.2 versus 28.7±20.2years; p=0.050). Cryptogenic aetiology was the most significant determinant of progression of new onset SE to RSE (Exp [β]=5.68; p=0.001). The overall mortality in the entire group was 23.7%, significantly higher in new onset RSE group (40.4% versus 9.7%; p<0.0001). New onset RSE patients with symptomatic and cryptogenic etiology did not differ for clinical characteristics and outcome. Acidosis was the strongest predictor of mortality in the entire cohort (Exp [β]=8.72; p=0.005). Nearly half of the patients with new onset SE progressed to RSE. While cryptogenic aetiology determined progression of new onset SE to RSE, acidosis was associated with mortality. The outcome was similar between symptomatic and cryptogenic new onset RSE.

Reappraisal of epileptic pain as a rare symptom of seizures.

To draw attention to epileptic pain which is a rare seizure symptom mostly causing wrong diagnosis and delayed treatment. We present nine patients in whom pain was a prominent initial or early ictal symptom. We reviewed the records of 4736 patients, and found nine patients who had pain as an aura or an early prominent symptom of their seizures. Seizure semiology, EEG, and cranial imaging features were evaluated retrospectively. Age at seizure onset ranged from 6 months to 50 years, and the mean age during the study was 37.7±11.9 years. Pain was predominantly peripherally localized in four patients, whereas cephalic pain was detected in three patients, and abdominal pain was detected in two patients. Electroencephalography (EEG) revealed epileptic abnormalities on the temporoparietal and frontotemporal regions in three patients each. Photosensitive generalized epileptic discharges were detected in one and diffuse background slowing in the remaining two other patients. Electroencephalography abnormalities were lateralized to the contralateral site of the pain in four patients with strictly localized pain. Three patients revealed no abnormality on the cranial MR imaging, whereas others showed different types of abnormality such as heterotopias (n:1), mesial temporal lobe atrophy (n:1), white and gray matter sequela lesions (n:1), diffuse white matter lesion (n:1), chronic encephalomalacia and gliosis (n:1), and perivascular space dilatation (n:1). Epileptic pain is a neglected, but important, semiologic symptom with localization and lateralization value in the patients with somatosensorial seizures of parietal as well as temporal lobe origin. It occurs mainly as peripherally localized, cephalic, or abdominal pain and is usually associated with a symptomatic etiology. Awareness of epileptic pain is important to avoid misdiagnosis and delayed treatment.

Predictor factors for relapse after remission of seizure in adults with remote symptomatic epilepsy

Background: Several studies have examined remission in individuals with epilepsy and relied on factors that may affect seizure outcome. However, the information on those patients with remote symptomatic epilepsy who are prone to present remission is quite less. Objective: To evaluate the remission rate and relapse in adult population with remote symptomatic epilepsy. Materials and Methods: Between 2012 and 2013, we performed a retrospective study of epilepsy patients seen in 2007 at the King Abdulaziz Hospital, Jeddah, Saudi Arabia, and followed up subsequently. To be eligible for our study, potential subjects had to meet all the following criteria of symptomatic epilepsy: adult patients 12 years and above with seizures that were the result of one or more identifiable neurological insults of the brain, people with seizure-free period of 2 years and more and followed up by stopping antiepileptic drugs (AEDs), and people with follow-up of at least 2 years after the discontinuation of AED at the first visit in 2001 in which they met the inclusion criteria. Result: The study included 145 patients with remote symptomatic epilepsy. Among most of the patients (80.6%), the type of the seizures was generalized symptomatic epilepsy, while both the focal and generalized types were reported among 16.6% of them. Patients with number of seizures ranged between three and five were significantly less likely to present remission when compared with those with one or two seizures [crude odds ratio (OR) = 0.39; 95% confidence interval (CI) = 0.18–0.83]. Patients who reported history of status epilepticus were significantly less likely to show remission when compared with those with no history of status epilepticus (crude OR = 0.33; 95%CI = 0.13–0.83). Patients who showed abnormal magnetic resonance imaging (MRI) were 57% less likely to exhibit remission when compared with those who showed normal MRI (crude OR = 0.43; 95%CI = 0.23–0.99). Conclusion: Among the acquired causes of symptomatic etiology, the traumatic brain injury is found to be associated with a high rate of remission and good seizure outcome when compared with mesial temporal sclerosis, central nervous system infection, and vascular causes, while the malignant tumor was associated with a high rate of relapse and drug refractory epilepsy.

Refractory Convulsive Status Epilepticus in Children: Etiology, Associated Risk Factors and Outcome.

Refractory status epilepticus (RSE) is a life-threatening disease in children wherein the patient's convulsive seizures do not respond to adequate initial anticonvulsants. RSE is associated with high rate of mortality and morbidity. This study was aimed to survey the risk factors leading status epilepticus (SE) to RSE in children, and their early outcome. Patients with SE hospitalized in Tabriz Children's Hospital, Iran were studied during the years 2007 and 2008 with regard to their clinical profile, etiology, the treatment methods available to them and their outcome upon release from the hospital. Among 132 patients with SE, 53 patients (40.15%) suffered from RSE. Acute symptomatic etiology was a risk factor responsible for developing RSE in the patient (P=0.004). Encephalitis was the most common etiology of acute symptomatic SE. There was no significant relationship observed between RSE and the patients' age, gender, date of initial drug intake and type of seizure. The mortality rate was 8.3% and a new neurological deficit occurred in 25.7% of cases. None of RSE with encephalitis returned to the baseline status. Mortality and morbidity rates were significantly higher in children with RSE than in those with SE (P=0.006). Etiology of SE significantly influenced prognosis of it with significant incidence of RSE in acute symptomatic group. Because acute neurological insult such as encephalitis and meningitis are common causes of RSE in children, properly management of them is necessary to avoid permanent brain damage.

The natural history and prognosis of epilepsy.

Epilepsy is a brain condition characterized by the recurrence of unprovoked seizures. Generally, prognosis refers to the probability of attaining seizure freedom on treatment and little is known about the natural history of the untreated condition. Here, we summarize aspects of the prognosis and prognostic predictors of treated and untreated epilepsy and of its different syndromes. Usually, epilepsy is a fairly benign condition. Most epilepsies have a good prognosis for full seizure control and eventual discontinuation of AEDs, but epilepsy syndromes have differing outcomes and responses to treatment. Prognostic factors include aetiology, EEG abnormalities, type of seizures and the number of seizures experienced before treatment onset, and poor early effects of drugs. Early response to treatment is an important positive predictor of long-term prognosis, while the history of a high number of seizures at the time of diagnosis, intellectual disability, and symptomatic aetiology are negative predictors. Different prognostic patterns can be identified, suggesting that the epileptogenic process is not static. Epilepsy carries a greater than expected risk of premature death. Aetiology is the single most important risk factor for premature death.

A one-year prospective study of refractory status epilepticus in Modena, Italy.

Refractory status epilepticus (RSE) is a particular critical condition characterized by seizures that continue despite the use of first- and second-line therapies and by high mortality. To date, only one prospective study investigated clinical features and prognostic factors in RSE. In this study, we performed a one-year prospective survey to identify clinical features, outcomes, and variables associated with the development of RSE in the adolescent and adult population of Modena, northern Italy. We observed 83 episodes of SE in 83 patients. In 31% of the cases, third-line therapy (anesthetic drug) was needed. Among this group, 14% resolved and were classified as RSE, while, in 17%, seizures recurred at withdrawal of anesthetics and were classified as super-RSE. The development of RSE/super-RSE was associated with a stuporous/comatose state at presentation and with the absence of a previous history of epilepsy. Refractory status epilepticus/super-refractory status epilepticus showed a worse outcome compared with responsive SE: 54% versus 21% for 30-day mortality; 19% versus 56% for a return to baseline condition. This prospective study confirms stupor/coma at onset as a relevant clinical factor associated with SE refractoriness. We observed a rate of RSE comparable with previous reports, with high mortality and morbidity. Mortality in the observed RSE was higher than in previous studies; this result is probably related to the low rate of a previous epilepsy history in our population that reflects a high incidence of acute symptomatic etiologies, especially the inclusion of patients with postanoxic SE who have a bad prognosis per se. This article is part of a Special Issue entitled "Status Epilepticus".

The demise of Archbishop Wolf Dietrich--A historical note on a fatal status epilepticus documented at Salzburg in 1617.

Wolf Dietrich of Raitenau (WD) ruled the archiepiscopal Salzburg from March 2nd 1587 to December 17th 1611. He was condemned by his successor Archbishop Markus Sittikus of Hohenems to spend his last years imprisoned at the Fortress Hohensalzburg, where he died on January 16th 1617. This historical note describes the causes of his death. The original Latin handwriting, including the detailed medical history and the autopsy of the Archbishop's body performed by his personal physician, was analyzed in conjunction with historical handwritings provided by St. Peter's Abbey, Salzburg handwriting assigned to Markus Sittikus. Wolf Dietrich of Raitenau had his first well-documented left hemispheric stroke in winter 1604/05. He had palsy of his right arm, was unable to write, and, therefore, used a stamp instead of his signature until October 1605. After another stroke, right hemispheric in origin with persisting palsy of his left arm ["leva corporis pars iam pridem simili ex apoplectico assultu in paralysin resoluta"], he developed symptomatic epilepsy with recurring seizures ["epileptico insultu quo etiam alias correptus est"]. On January 15th 1617, he suffered from a secondarily generalized convulsive status epilepticus ["toto corpore convellitur epileptico insultu"] with stertorous breathing and distortion of his face ["spuma stertore insigni faciei perversione"] and was unconscious for 8h. He recovered from coma and showed dysphagia, buccofacial apraxia ["abolitam diglutiendi facultatem"], reversible speech disturbance ["accisa etiam verba loqui"], and left-sided hemiplegia ["leva corporis pars… immobilis prorsus est reddita"]. The following day, he had speech disturbances, and he died at noon. His autopsy showed large but intact liver ["hepar magnum sanum"] and heart ["cor magnum in quo lapsus nullus"]. There was intrapulmonal mucus ["pituita imbutus"], and part of the lungs adhered to its pleura. He had five kidney stones and a partly cirrhotic spleen. The cause of his death was assumed to be intracerebral ["causa mortis in capite requienda fuisset"]. The terminal suffering of Wolf Dietrich of Raitenau is the first witnessed case report on a fatal status epilepticus in Salzburg. Most likely, he suffered from vascular epilepsy due to a right hemispheric stroke, leading to status epilepticus with left-sided Todd's palsy and speech disturbances. An acute symptomatic etiology of this disease cannot be ruled out, as for religious reasons, the Archbishop's brain was not autopsied. Meticulous medical reporting including autopsy was already available in Salzburg in 1617, and the symptomatic etiology of epilepsy was diagnosed correctly. This article is part of a Special Issue entitled "Status Epilepticus".

Predictors of Outcome of Convulsive Status Epilepticus Among an Egyptian Pediatric Tertiary Hospital.

Convulsive status epilepticus is a common neurologic emergency in pediatrics. We aimed to study the etiology, clinical features, and prognostic factors among pediatric patients with convulsive status epilepticus. Seventy patients were included in this cohort study from pediatric emergency department of the specialized Children Hospital of Cairo University. The outcome was evaluated using the Glasgow Outcome Score. Acute symptomatic etiology was the most common cause of convulsive status epilepticus. Refractory convulsive status epilepticus was observed more significantly in cases caused by acute symptomatic etiologies. The outcome was mortality in 26 (37.1%) patients, severe disability in 15 (21.4%), moderate disability in 17 (24.3%), and good recovery in 12 (17.1%) patients. The significant predictor of mortality was lower modified Glasgow Coma Scale score on admission, whereas lower modified Glasgow Coma Scale score on admission and refractory convulsive status epilepticus were the significant predictors for disability and mortality.

Aetiology and outcome of generalized convulsive status epilepticus in elderly

PurposeConvulsive status epilepticus (CSE) is a common neurologic emergency in elderly people. The current study elaborates the clinical characteristics and outcome of CSE in elderly patients. MethodsAnalysis of data of generalized CSE patients, aged 60 years and above admitted at the neurointensive care unit (NICU) was performed. The primary outcome for analysis was in-hospital mortality. The study population was divided into groups based on progression of CSE and mortality to analyze difference in study variables. Mortality of the group was analyzed using life tables. ResultsA total of 33 patients satisfied the inclusion criteria from medical records of 212 patients with CSE. Mean age of the study population was 67.0±6.8 years; 69.7% were men. Acute symptomatic aetiology was the commonest cause of CSE (60.6%); nine (27.3%) patients progressed to refractory status epilepticus (RSE) of which five patients had prolonged RSE. The overall mortality was 18.2%. Complications of mechanical ventilation and mean age were higher in patients who died. Though vascular aetiology was the leading cause of CSE (39.3%), it was not associated with progression to RSE or mortality. Acute symptomatic aetiology accounted for five out of the six deaths in the entire cohort. ConclusionLess than one-third of elderly patients with CSE progressed to RSE. Vascular aetiology, the leading cause of generalized CSE in elderly, was not associated with progression to RSE and mortality. Acute symptomatic aetiology was associated with high mortality.

Induction of burst suppression or coma using intravenous anesthetics in refractory status epilepticus

General anesthetic-induced coma therapy has been recommended for the treatment of refractory status epilepticus (RSE). However, the influence of electroencephalographic (EEG) burst suppression (BS) on outcomes still remains unclear. This study investigated the impact of intravenous anesthetic-induced BS on the prognosis of RSE using a retrospective analysis of all consecutive adult patients who received intravenous anesthetic treatment for RSE at the Seoul National University Hospital between January 2006 and June 2011. Twenty-two of the 111 episodes of RSE were enrolled in this study. Of the 22 RSE patients, 12 (54.5%) were women and 18 (81.4%) exhibited generalized convulsive status epilepticus. Sixteen patients (72.7%) were classified as having acute symptomatic etiology, including three patients with anoxic encephalopathy, and others with remote symptomatic etiology. Only two patients (9.1%) had a favorable Status Epilepticus Severity Score (0–2) at admission. All patients received midazolam (MDZ) as a primary intravenous anesthetic drug for RSE treatment; three (13.6%) received MDZ and propofol, and one (4.5%) received MDZ and pentobarbital. The rates of mortality and poor outcome at discharge were 13.6% (n=3) and 54.5% (n=12), respectively. While BS was achieved in six (27.5%) patients, it was not associated with mortality or poor outcome. Induced BS was associated with prolonged hospital stay in subgroup analysis when excluding anoxic encephalopathy. Our results suggest that induction of BS for treating RSE did not affect mortality or outcome at discharge and may lead to an increased length of hospital stay.

Treatment of pediatric epilepsy in Poland

BackgroundThe many types of childhood epilepsies make the diagnosis and treatment difficult and the outcomes frequently poor. Furthermore, there are few clinical trials in pediatric epilepsy that provide useful results to guide daily practice. Therefore for pediatric neurologists expert opinion may be useful. AimsTo provide an overview of current practice in Poland and compare results with European and US clinical guidelines. MethodsPolish specialists in pediatric neurology were asked to participate in a survey about pediatric epilepsy. The focus of the questions was on the overall strategy and treatment options for different syndromic diagnoses. The survey was developed and performed according to a previous European survey (Wheless et al., 2007). ResultsFifty-one Polish specialists, working in academic or clinical settings, completed the questionnaire. They limited combination therapy to two or three antiepileptic drugs. Valproate was the treatment of choice for myoclonic, generalized tonic-clonic seizures and Lennox-Gastaut syndrome. For infantile spasms caused by tuberous sclerosis and of symptomatic etiology, vigabatrin was treatment of choice; valproate and ACTH were other first line options. Valproate and ethosuximide were chosen for childhood absence epilepsy and valproate for juvenile absence epilepsy. Carbamazepine was the first-line treatment option for benign partial epilepsy of childhood with centrotemporal spikes and complex partial seizures. In the treatment of juvenile myoclonic epilepsy for males valproate, for females lamotrigine were chosen. ConclusionPolish pediatric neurologists agreed on the majority of questions. Their views reflect the clinical utility and availability of treatment options in Poland. Results may provide direction for clinicians.

Long-term outcomes in patients with West syndrome: An outpatient clinical study

PurposeNearly half of all patients with seizure onset in the first year of life suffer from West syndrome (WS). The prognosis of epilepsy and psychosocial outcomes in children with WS are variable. This study was performed to examine the factors influencing the outcome of this patient population. MethodsA total of 109 patients with WS followed up regularly for at least 3 years were included in the study. Relevant clinical, laboratory, and imaging data were collected. ResultsThe male/female ratio was 65/44 (59.6%/40.4%). The mean age at onset of infantile spasm (IS) was 6±6 (1–36) months. With regard to neuro-developmental and social conditions during the final evaluation, 29.4% of the patients were socially dependent on caregivers, 61.8% needed assistance, and 8.8% were normal. Among the patients, 5.9% were free of epilepsy and antiepileptic drugs (AED) for at least 2 years, 49.0% had no seizures with AEDs, and 45.1% had uncontrollable seizures. Parameters with significant negative effects on the long-term outcomes included symptomatic etiology, presence of developmental retardation before the onset of IS, persistence of active epilepsy, and male gender. ConclusionIn this study, 37 (33.9%) patients had severe consequences as a result of WS. The majority of the rest could cope with daily life with varying degrees of assistance. Eight percent of the patients had a normal development. These results draw attention to the two-thirds of patients with WS who have the chance of an acceptable quality of life (QoL) with early diagnosis and therapeutic measures.

Sudden death in Brazil: epilepsy should be in horizon.

To date, a considerable amount of valuable information about the problem of sudden cardiac death (SCD) has been described. The incidence of SCD in the United States ranges between 180000-400000 cases per year1. Martinelli et al demonstrated an incidence of 21270 cases of SCD per year in the Metropolitan Area of Sao Paulo2. Recently, Braggion-Santos et al.3 described the characteristics of SCD in Ribeirao Preto, Brazil, according to autopsy reports3. Revising 4501 autopsies, they identified 899 cases of SCD (20%); the rate was 30/100000 residents/year3. The vast majority of SCD cases involved coronary artery disease (64%). Based on available scientific knowledge related to SCD, it is extremely important to identify new areas of research that might improve understanding of this problem and to establish effective preventive measures to minimize or even control the occurrence of SCD. Although studies have shown that the increase in the number of SCD caused by a combination of factors2,3, an equally important risk factor for SCD which is not reported and not explored in cardiologic research is epilepsy. Indeed, a series of data could be put forward to explain it. Epilepsy affects approximately 65 million individuals worldwide and is one of the most common, chronic and severe neurological diseases4-7. In developing and poor countries, the incidence of epilepsy is higher when compared with that of developed countries4-7. The prognostic evolution has clearly shown that seizures are successfully controlled with currently available antiepileptic drugs in approximately two-thirds of individuals with epilepsy, which results in one-third with refractory epilepsy4,8. For these patients with uncontrolled seizures, epilepsy should be considered a malignant condition, as it carries a mortality rate that is 2‑3 times higher than that in the general population9. Therefore, sudden unexpected death in epilepsy (SUDEP) is the most frequent cause of epilepsy-related death9-12. By definition, SUDEP is a sudden, unexpected, witnessed or unwitnessed, non‑traumatic and non-drowning death in individuals with epilepsy, with or without evidence of seizures, in which post-mortem examination does not reveal a toxicological or anatomical cause of death13. Epidemiological studies indicate that SUDEP is responsible for 7.5% to 17% of all deaths in epilepsy and has an incidence among adults between 1:500 and 1:1000 patient/year14. The main risk factors for SUDEP include the number of generalized tonic-clonic seizures, nocturnal seizures, young age at epilepsy onset, longer duration of epilepsy, dementia, absence of cerebrovascular disease, asthma, male gender, symptomatic etiology of epilepsy and alcohol abuse12,15. The cause or causes of SUDEP are still unknown, but one of the main proposed mechanisms is related to autonomic dysregulation, promoting cardiac abnormalities during and between seizures16-18. In this line of reasoning, our experimental data clarified some possibilities. Using the pilocarpine model of temporal lobe epilepsy, we evaluated heart rate in rats with epilepsy in vivo and in an isolated ex vivo preparation (Langendorff preparation)17. Baseline heart rate in vivo in animals with chronic epilepsy (346 ± 7 bpm) was higher than in control rats (307 ± 9 bpm)17. Incidentally, no difference was observed in the isolated ex vivo situation (control animals: 175 ± 7 bpm; chronic epilepsy: 176 ± 6 bpm), suggesting that autonomic modulation of the heart is altered in epileptic animals, explaining the maintenance of an increased basal heart rate in these animals17. In addition, we also evaluated heart rate responses during stage 5 of amygdala kindling model, the phase when animals develop generalized seizures18,19. Animals did not show significant differences in basal heart rate; however, basal heart rate was higher during stage 5 of kindling, possibly resulting from sympathetic activation caused by the chronic epileptic condition18,19. As demonstrated in previous studies20, intense bradycardia at the beginning of seizure was followed by rebound tachycardia18,19. Moreover, the intensity of tachycardia was directly related to the number of generalized seizures, suggesting that repeated generalized tonic-clonic seizures affect sympathetic outflow18,19. For that reason, a plausible explanation is that continuous and intermittent sympathetic activation due to uncontrolled seizures is capable of maintaining cardiac rhythm, modulating the heart in accelerated-state permanently. Considering all these translational information, it is clear that epilepsy-related mortality, particularly SCD, is a significant public health concern. Thus, it is crucial that a concerted and collaborative approach be implemented to solve this problem. In order to do so, it is extremely necessary to attain a real convergence between cardiologists and neurologists to carefully evaluate and discuss the electroencephalographic and electrocardiographic recordings, the cardiac and cerebral imaging findings and refined histopathological studies in order to detect or prevent the occurrence of a tragic fatal event among individuals with epilepsy.

Long-term outcome in children with infantile spasms treated with vigabatrin: a cohort of 180 patients.

Evaluation of efficacy of vigabatrin as the first drug in infants with previously untreated infantile spasms (IS) and reporting the long-term outcome. We analyzed a cohort of 180 infants with infantile spasms treated with vigabatrin as the first drug. Following initial evaluation and a 48-h basal period for counting the spasms, vigabatrin was administered using the same protocol in all. After 14 days all infants were assessed for therapeutic response (primary outcome). Psychomotor development was evaluated by a psychologist and neurologist prior to the initiation of treatment and during the follow-up. Seizure outcomes were followed prospectively, by seizure types and epilepsy syndromes. Long-term (secondary) outcomes included neurologic status, occurrence of late epilepsy, and developmental/cognitive status. Vigabatrin terminated the spasms in 101 patients (56.9%) at a mean period of 5 days. Patients with normal psychomotor development prior to the onset of spasms responded best. After follow-up of 2.4 to 18.9 years (mean 10.64; standard deviation [SD] 4.40), 38.1% of responders, treated with vigabatrin, had severe neurologic dysfunction, 42% had epilepsy, and 42.2% had unfavorable intellectual outcome. The group with symptomatic etiology and abnormal neurologic status at presentation demonstrated a significantly worse prognosis and a more unfavorable outcome than cryptogenic or idiopathic cases (85.1% and 81.6% versus 14.9% and 0%-p = 0.001). Idiopathic patients treated with vigabatrin were all intellectually normal, except the youngest patient who had borderline cognitive function. The most important prognostic factors were the underlying etiology and preexisting developmental profile. Long-term outcome in the patients treated with vigabatrin was similar to the outcome in patients treated with adrenocorticotropic hormone (ACTH) or corticosteroids, as reported in earlier studies. The long-term prognosis of idiopathic cases treated with vigabatrin was favorable.