Vagus nerve stimulation: Clinical experience in drug-resistant pediatric epileptic patients

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Vagal nerve stimulation in intractable epilepsy: clinical experience on 100 patients and review of the literature

Introduction: Vagus Nerve Stimulation (VNS) is an effective alternative treatment for patients with refractory epilepsy. Nevertheless, information regarding VNS is still limited. Materials and Methods: In the present non randomized, prospectic study we report our clinical safety and effectiveness of VNS in 100 patients (52 Males and 48 Females) with drug resistant epilepsy. Patient’s age at implant ranged from 0,64 to 51,04 years (mean age 15.3 years). The mean follow-up time was 54,8 months ( range 2 to 108,3 months). Seventeen patients suffered from Lennox-Gastaut Syndrome, 34 patients suffered from partial epilepsy with drop attacks and secondary bysinchronism on the EEG (Lennox Gastaut-like) and 49 patients had Partial Epilepsy without drop attacks. Data collection forms were designed for prospectively gathering data on each patient’s history, seizures, drug therapy, implant device settings and side effects. Patients were assessed prior the implant and 3, 12 and 24 months after surgery. Results: Seventy-eight patients completed the 24 months follow-up session. VNS produced a mean seizure rate reduction of 32% at 3 months, 41% at 12 months, and 45% at 24 months. At 24 months, only the Partial Epilepsy patients showed a seizures reduction of 50%, which is considered clinically significant. Moreover both the age at implant and epilepsy duration were inversely correlated with the percentage of seizure reduction at 24 months. Side effects were minor and transient; the most common were voice alteration and coughing during stimulation. In 7 patients electrode breakage occurred three years after the surgical procedure. Conclusion: In our study, clinical effectiveness is higher in younger children implanted before than 12 years with shorter epilepsy duration suggesting a precocious useful role of VNS. Patients with Lennox Gastaut Syndrome show a worse clinical response rather than other epileptic syndromes

Lacosamide in children with refractory status epilepticus. A multicenter Italian experience

Abstract OBJECTIVE: Status epilepticus (SE) is considered a life-threatening medical emergency. First-line treatment with antiepileptic drugs (AEDs) consists of intravenous benzodiazepines followed by phenytoin. SE is considered refractory (RSE) when unresponsive to standard doses of the first two AEDs. Scarce evidence is available to support specific guidelines for the management of RSE in either adults or children. This study aimed to assess the efficacy and tolerability of intravenous (iv) lacosamide (LCM) in children affected by RSE. METHOD: Children with RSE who were treated with ivLCM were included in the study. Efficacy was defined as the cessation of seizures after administration of ivLCM, with no need for any further antiepileptic drug. All patients had been unsuccessfully treated following standard protocols before ivLCM was administered. RESULTS: Eleven children entered the study (mean age: 9.4 years). Etiology was symptomatic in 7 patients (63%). RSE was convulsive (focal or generalized) in 6 patients and nonconvulsive in 5. The mean initial bolus dose of LCM was 8.6 mg/kg. The drug, which was used as a fourth or later option, was effective in stopping RSE in 45% of patients, with seizures terminating within 12 h in three children. No serious adverse events attributable to LCM were reported. CONCLUSIONS: LCM might be an effective and well-tolerated AED in children with RSE

Long-term seizure and behavioral outcomes after corpus callosotomy

Outcomes of corpus callosotomy (CC) have been mainly focused on seizures. The present study aimed to evaluate the long-term effects of CC on adaptive behaviors and caregivers' satisfaction in addition to seizures and to identify clinical predictors of postsurgical outcomes. Medical records of 26 patients (mean age at study time: 40 years, mean follow-up: 14 years) with childhood-onset epilepsy who underwent anterior or 2-stage complete CC were reviewed. A structured questionnaire was submitted to caregivers asking about relative changes in different seizure types, behavioral functions, and satisfaction with the postoperative outcomes. Formal neuropsychological assessment was carried out in a subgroup of patients. Selected clinical variables including age at surgery, extent of callosal section, length of follow-up, epilepsy syndrome, and presurgical cognitive level were submitted to multiple regression analysis. At the last follow-up visit, a reduction greater than 50% was observed mainly for drop attacks (65% of patients), followed by generalized tonic-clonic seizures (53%), and complex partial seizures (50%). No presurgical variables were significantly associated with seizure outcome. After surgery, more than half of patients showed attention enhancement, which was related to drop seizure improvement. Early age at surgery was associated with better behavioral regulation; complete CC slightly worsened language abilities. Satisfaction with surgery outcomes was expressed by 73% of caregivers and was dependent on drop seizure reduction and improvements in activities of daily living. A long-term positive psychosocial outcome is likely after CC also in severely disabled patients, especially if surgery is performed early

Vaccination and occurrence of seizures in SCN1A mutation-positive patients: A multicenter Italian study

Background The relation between epileptic seizures and vaccinations is sometimes debated. In the present work, the impact of vaccination on seizure onset and clinical outcome of SCN1A mutation-positive patients is addressed. Methods Seventy-two patients diagnosed with Dravet syndrome or generalized epilepsy with febrile seizure plus, carrying SCN1A mutations or not, were included. Details on vaccination type, temporal relationship between vaccination and seizure occurrence, seizure type at onset and during development, cognitive functioning, and vaccination completion was obtained by reviewing clinical records. Patients were divided into two groups based on the temporal window between vaccination and seizure onset (proximate group: 48 hours). Results Vaccination-related seizures occurred in 25% of patients with SCN1A mutation and 18% of patients without the mutation (no significant difference). The proximate group showed an earlier age at seizure onset and a higher frequency of status epilepticus during development than did the distant group. No other significant differences were found. Subsequent vaccinations did not significantly alter the evolution of the disease. Conclusions Results from this relatively small series provide evidence that vaccinations do not significantly affect clinical and cognitive evolution of Dravet syndrome and generalized epilepsy with febrile seizure plus patients even if they carry SCN1A mutations. © 2014 Elsevier Inc. All rights reserved

Rufinamide for refractory focal seizures. An open-label, multicenter European study

Purpose: The present study aimed to assess the efficacy and tolerability of rufinamide as adjunctive drug for the treatment of a large series of children, adolescents and adults with refractory cryptogenic or symptomatic focal epilepsy. Methods: Patients were recruited in a prospective, add-on, open-label treatment study from six Italian and one German centers for pediatric and adolescent epilepsy care. Inclusion criteria were: (1) age 3 years or more; (2) diagnosis of cryptogenic or symptomatic focal epilepsy refractory to at least three previous antiepileptic drugs (AEDs), alone or in combination; (3) more than one seizure per month in the last 6 months; (4) use of at least one other AED, but no more than three, at baseline; (5) informed consent from parents and/or caregivers. Results: Sixty-eight patients (40 males, 28 females), aged between 3 and 63 years (mean 19.9 years, median 16.0) ± SD 12.58, with cryptogenic (28 pts, 41.2%) or symptomatic focal epilepsy (40 pts, 58.8%), were recruited in the study. After a mean follow-up period of 10.4 ± 10.29 months, twenty-two patients (32.3%) had a 50-99% seizure reduction, and none became seizure-free. Twelve patients (17.6%) had a 25-49% seizure decrease, while in 30 (44.1%) seizure frequency was unchanged. A seizure worsening was reported in 5 patients (7.3%). A better response to rufinamide occurred in frontal lobe seizures (51.6%) and secondary generalized tonic-clonic seizures (50%). Conclusion: Rufinamide was effective against focal-onset seizures, particularly in the treatment of secondary generalized frontal lobe seizures

Microsurgical endoscopy-assisted anterior corpus callosotomy for drug-resistant epilepsy in an adult unresponsive to vagus nerve stimulation

Because most of the corpus callosotomy (CC) series available in literature were published before the advent of vagus nerve stimulation (VNS), the efficacy of CC in patients with inadequate response to VNS remains unclear, especially in adult patients. We present the case of a 21-year-old female with medically refractory drop attacks that began at the age of 8 years, which resulted in the patient being progressively unresponsive to vagus nerve stimulation implanted at the age of 14 years. Corpus callosotomy was recommended to reduce the number of drop attacks. However, the patient had only mild cognitive impairments and no neurological deficits. For this reason, we were forced to plan a surgical approach able to maximize the disconnection for good seizure control while, at the same time, minimizing sequelae from disconnection syndromes and neurosurgical complications because in such cases of long-lasting epilepsy the gyri cinguli and the arteries can be tenaciously adherent and dislocated with all the normal anatomy altered. In this scenario, we opted for a microsurgical endoscopy-assisted anterior two-thirds corpus callosotomy. The endoscopic minimally invasive approach proved to be quite adequate in this technically demanding case and confirmed that CC may offer advantages, with good results, even in adult patients with drop attacks who have had inadequate response to VNS

Long term outcome in children affected by absence epilepsy with onset before the age of three years

Objective: The goal of this study was to define the long-term outcome of absence epilepsy presenting before the age of 3 years. Methods: We retrospectively studied the medical records of 40 children from eight neuropediatric centers in Italy with respect to the personal and family histories of epilepsy or febrile seizures, time of follow-up, cognitive functions, treatment, and outcome. Results: Forty patients were enrolled in this study. They all fulfilled the criteria for absence epilepsy with 3-Hz spike-wave complexes on the EEG, normal neurological examination, and no other seizures types. Seizure onset occurred between 24.1 and 36.0 months. There was a family history of epilepsy in 28%, and of febrile seizures in 13%. Thirty-three patients were treated with valproic acid (VPA), mostly used in monotherapy (26 patients) or in association with ethosuximide. At final follow-up, 33 patients were seizure free and 29 had normal EEGs. Thirty-four patients had a normal intelligence quotient (IQ), whereas 6 had a decreased IQ mainly associated with poor control of seizures. Conclusion: In our series, absence seizures presenting before the age of 3 appeared to have quite a good long-term clinical prognosis; the neuropsychological outcome was comparable to that of childhood epilepsy presenting after 3 years of age

Vagal nerve stimulation for drug-resistant epilepsies in different age, aetiology and duration

The aim of the study was to compare the outcome with respect to age of implant, aetiology and duration of epilepsy