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Purpose: The aim of this study is to describe demographic data, semiology and etiology in a pediatric population with status epilepticus (SE) and refractory SE (RSE). Method: We retrospectively reviewed patients with the following inclusion criteria: i) age between two months and eighteen years; ii) SE diagnosis; iii) admission from January 2001 to December 2016; iv) available clinical data. Results: We enrolled 124 patients. Mean and median age was 4.6 ± 4.2 years and 3.3 [1.2-7.5] years respectively. SE had a “de novo” onset in 66.9%. Focal convulsive-SE was the most common semiology (50.8%) whilst generalised (32.3%) and nonconvulsive-SE (NCSE) (16.9%) were less represented. Some etiologies showed a different age distribution: febrile in youngest age (p = 0.002, phi 0.3) and idiopathic-cryptogenic in older children (p = 0.016, phi 0.2). A statistical significance correlation was detected between semiology and etiology (p < 0.001, Cramer's V 0.4), chemotherapy and NCSE (n = 6/21 vs 3/103, p < 0.001) as well as PRES and NCSE (n = 7/21 vs 5/103, p < 0.001). Only 17.7% had a RSE. No correlation was found in demographic and clinical data, but NCSE, acute and idiopathic-cryptogenic etiologies were more frequently associated to RSE. Encephalitis was the most common diagnosis in acute etiologies whereas unknown epilepsy in idiopathic-cryptogenic group. Conclusion: Most of our findings were previously described however we found a significant role of non-antiepileptic treatments (chemotherapy-dialysis) and comorbidity (PRES) determining acute etiology and NCSE. Acute (mostly encephalitis), idiopathic-cryptogenic (mainly unknown-epilepsy) and NCSE were frequently detected in RSE. In the above mentioned conditions a high level of suspicion was recommended.

PURPOSE: Epileptic seizures are frequent manifestations after hematopoietic stem cell transplantation (HSCT). In this retrospective single-center study we evaluated electroclinical features and analyzed etiologies and outcome of seizures after pediatric HSCT. METHODS: Of 261 children transplanted between 2000 and 2010, we identified and analyzed data of 28 patients with seizures within a year from HSCT. RESULTS: Most frequent etiologies were posterior reversible encephalopathy syndrome (PRES, 14 patients) and central nervous system (CNS) infections (4 patients). Seizures were the presentation of the underlying complications in 22 patients. Sixteen episodes of status epilepticus were identified. Seizures secondary to PRES were usually longer and associated with non-convulsive signs. Early neuroimaging and EEG monitoring proved to be crucial to diagnose and treat seizures and their causes. No patients developed epilepsy suggesting that chronic antiepileptic therapy is not necessary in these patients. Overall survival was 32.3% over 5 years in patients with seizures and 45.8% in patients without seizures (p

Lennox-Gastaut syndrome (LGS) is a severe epileptic encephalopathy with a prevalence of 1-2% of all patients with epilepsy. It is characterized by multiple pharmaco-resistant seizure types, including tonic, atypical absences and tonic or atonic drop attacks, and the presence of electroencephalographic abnormalities, such as slow-spike waves and paroxysmal fast rhythms. Intellectual disability, behavioural and psychiatric disorders are common comorbidities; these disturbances have a multi-factorial pathogenesis. The selection of the most appropriate drug must be tailored to each patient and guided by the prevalent seizure type. In this paper available pharmacological options are discussed and for each pharmacological agent, current evidence of efficacy and tolerability is provided. Valproic acid represents one of the first-line options in the treatment of LGS. Anyway, other antiepileptic drugs (AEDs) may be considered and added: lamotrigine, rufinamide, topiramate, clobazam can be efficacious. The use of felbamate must be carefully evaluated because of its adverse events. Perampanel, zonisamide, levetiracetam and fenfluramine have shown to be useful in the treatment of selected patients; nevertheless, the lack of RCTs does not allow to recommend their use in a systematic way. Recently, cannabidiol has provided high evidence of efficacy against LGS seizures; however, these data must be confirmed by long-term extensive studies and by trials comparing different AEDs, one to each other.

SummaryThe aim of this multicentric, prospective and uncontrolled study was to evaluate the efficacy and safety of levetiracetam in 110 children with refractory epilepsy, of whom 21 were less than 4 years old. After a median follow-up period of 7 months, levetiracetam administration was effective (responders with >50% decrease in seizure frequency) in 39% of children, of whom 10 (9%) became seizure-free. The efficacy was higher in patients with localization-related epilepsy (58% of responders) than in those with generalized epilepsy (37% of responders). Levetiracetam was well tolerated. The main side effects of somnolence and irritability occurred in 14% of patients. In one patient acute choreoathetosis occurred after few doses of levetiracetam. Overall, the adverse effects were not severe. Children younger than 4 years were particularly tolerant. In conclusion, the present study confirms that levetiracetam is effective and well tolerated as an add-on treatment in children with refractory epilepsy. Our preliminary data also indicate that levetiracetam may be a valid therapeutic option for epilepsy in infants and young children.

Purpose To better define the convulsive status epilepticus (CSE) as a possible manifestation at the onset of Panayiotopoulos syndrome (PS) and to assess its prognostic value in these children. Methods Children with CSE and diagnostic criteria of PS were identified, followed clinically and compared with a group of patients with PS without CSE from 1993 to 2012. Results We identified 37 patients with CSE at the onset of PS. During the same period we identified 72 children with autonomic symptoms of PS without CSE. The first episode of CSE occurred at a mean age of 6.5 years. Generalized clonic seizures were the most common ictal event and one-third of the patients required admission to Intensive Care Units. Interictal EEGs showed occipital spike activity in 31 (83.7%) subjects. Only 14 (37.8%) patients were treated with valproic acid and for two of them (5.40%) it was necessary to administer other drugs. There were no intractable cases. The overall prognosis was excellent. After the first event, 15 subjects (40.54%) experienced at least another typical PS seizure, but all patients were seizure free at the last follow-up. Conclusion CSE is not uncommon in PS and it may occur at the onset of benign childhood epilepsy, without leading to a poor prognosis.

SummaryPurposeTo evaluate the long-term efficacy, tolerability, and safety of oxcarbazepine (OXC) in children with epilepsy.MethodsWe enrolled 36 patients (median age 7.75) with new diagnosis of partial epilepsy in an open prospective study. All type of epilepsy were included: 25 patients were affected by idiopathic epilepsy, eight by symptomatic epilepsy and three by cryptogenic epilepsy. Patients were then scheduled to come back for controls at 3 months (T1), 12 months (T2) and 24 months (T3) after the beginning of OXC-monotherapy (T0). At each control we evaluated patients through their seizure diary, a questionnaire on side effects, their level of 10-monohydroxy (MHD) metabolite and laboratory analysis.ResultsAt T1, 21/36 patients (58.3%) were seizure-free, 3/36 patients (8.3%) showed an improvement higher than 50%, 3/36 (8.3%) lower than 50%, while 2/36 worsened (5.6%). In 7/36 (19.5%) patients, no improvement was reported. At T2 13/18 patients (72.2%) were seizure-free, 1/18 showed a response to therapy higher than 50% while 2/18 worsened (11%). In two patients no improvement was reported. A correspondence between MHD plasmatic levels and clinical response (r=0.49; p

Purpose: To examine fever as a precipitating factor for focal seizures in patients with Panayiotopoulos syndrome (PS) and evaluate the role of SCN1A in PS patients with seizures triggered by fever. Methods: From January 2000 to June 2008, we identified patients referred for seizures who fulfilled the criteria of PS. Patients were divided into two groups, according to the presence (group A) or the absence (group B) of seizures triggered by fever. Electroclinical features of the two groups were compared. In addition, an analysis of SCN1A in patients of group A was performed. Results: Thirty patients fulfilled the inclusion criteria. Eleven patients (36%) had at least one focal autonomic seizure triggered by fever (group A). In group A, 7/11 patients (63.5%) had the first focal autonomic seizure during a febrile illness. Two of these 7 patients were misdiagnosed at the onset of PS. The median age at the onset of PS was slightly lower in group A than in group B (p =.050). Moreover, patients in group A more frequently had a positive familial history of febrile seizures (FS) (p =.047). No mutations of SCN1A were found in any of the 10 patients screened. Conclusion: Fever is a common trigger for focal autonomic seizures in PS. Knowing that an autonomic manifestation during fever can be an epileptic seizure could facilitate diagnosis and prevent unnecessary investigations and erroneous treatments. Moreover, our data show that SCN1A gene does not contribute significantly to susceptibility to autonomic seizures during fever in patients with PS. © 2011 British Epilepsy Association.

Alberto Verrotti *, Giulia Loiacono , Antonella Pizzolorusso , Pasquale Parisi , Oliviero Bruni , Anna Luchetti , Nelia Zamponi , Silvia Cappanera , Salvatore Grosso , Gerhard Kluger , Christine Janello , Emilio Franzoni , Maurizio Elia , Alberto Spalice , Giangennaro Coppola , Pasquale Striano , Piero Pavone , Salvatore Savasta , Maurizio Viri , Antonino Romeo, Paolo Aloisi , Giuseppe Gobbi , Alessandro Ferretti , Raffaella Cusmai , Paolo Curatolo q