SUMMARYThe diagnosis, evaluation, and management of infantile spasms (IS) continue to pose significant challenges to the treating physician. Although an evidence-based practice guideline with full literature review was published in 2004, diversity in IS evaluation and treatment remains and highlights the need for further consensus to optimize outcomes in IS. For this purpose, a working group committed to the diagnosis, treatment, and establishment of a continuum of care for patients with IS and their families-the Infantile Spasms Working Group (ISWG)-was convened. The ISWG participated in a workshop for which the key objectives were to review the state of our understanding of IS, assess the scientific evidence regarding efficacy of currently available therapeutic options, and arrive at a consensus on protocols for diagnostic workup and management of IS that can serve as a guide to help specialists and general pediatricians optimally manage infants with IS. The overall goal of the workshop was to improve IS outcomes by assisting treating physicians with early recognition and diagnosis of IS, initiation of short duration therapy with a first-line treatment, timely electroencephalography (EEG) evaluation of treatment to evaluate effectiveness, and, if indicated, prompt treatment modification. Differences of opinion among ISWG members occurred in areas where data were lacking; however, this article represents a consensus of the U.S. approach to the diagnostic evaluation and treatment of IS.

Article abstract-Objective: The Quality Standards Subcommittee of the American Academy of Neurology develops practice parameters as strategies for patient management based on analysis of evidence. For this practice parameter, the authors reviewed available evidence on evaluation of the first nonfebrile seizure in children in order to make practice recommendations based on this available evidence. Methods: Multiple searches revealed relevant literature and each article was reviewed, abstracted, and classified. Recommendations were based on a three-tiered scheme of classification of the evidence. Results: Routine EEG as part of the diagnostic evaluation was recommended; other studies such as laboratory evaluations and neuroimaging studies were recommended as based on specific clinical circumstances. Conclusions: Further studies are needed using large, well-characterized samples and standardized data collection instruments. Collection of data regarding appropriate timing of evaluations would be important.

OXC adjunctive therapy administered in a dose range of 6 to 51 mg/kg/day (median 31.4 mg/kg/day) is safe, effective, and well tolerated in children with partial seizures.

Abstract-The Quality Standards Subcommittee of the American Academy of Neurology and the Practice Committee of the Child Neurology Society develop practice parameters as strategies for patient management based on analysis of evidence regarding risks and benefits. This parameter reviews published literature relevant to the decision to begin treatment after a child or adolescent experiences a first unprovoked seizure and presents evidence-based practice recommendations. Reasons why treatment may be considered are discussed. Evidence is reviewed concerning risk of recurrence as well as effect of treatment on prevention of recurrence and development of chronic epilepsy. Studies of side effects of anticonvulsants commonly used to treat seizures in children are also reviewed. Relevant articles are classified according to the Quality Standards Subcommittee classification scheme. Treatment after a first unprovoked seizure appears to decrease the risk of a second seizure, but there are few data from studies involving only children. There appears to be no benefit of treatment with regard to the prognosis for long-term seizure remission. Antiepileptic drugs (AED) carry risks of side effects that are particularly important in children. The decision as to whether or not to treat children and adolescents who have experienced a first unprovoked seizure must be based on a risk-benefit assessment that weighs the risk of having another seizure against the risk of chronic AED therapy. The decision should be individualized and take into account both medical issues and patient and family preference. NEUROLOGY 2003;60:166 -175 Population-based studies of the incidence of first unprovoked seizures suggest that there are between 25,000 and 40,000 children per year in the United States who experience a first unprovoked seizure. [1][2][3][4] Until relatively recently, it was common practice for physicians to begin long-term, daily antiepileptic drug (AED) therapy after a child or adolescent experienced a single seizure of any type. The rationale for this practice was based on the belief that all seizures were likely to recur and that seizures could be dangerous and cause brain damage. Furthermore, it was thought that if any recurrence were to take place, this would lead to progressively more seizures. It was also assumed that AED were safe, had few side effects, and were effective in prevention of seizure recurrences. These assumptions have undergone substantial modification over the last 20 years, leading to a more optimistic view about the nature of seizures and a more conservative approach to the use of treatment. However, no clear evidence-based guidelines have emerged regarding the initiation of treatment after a first unprovoked seizure in the pediatric population.Practice parameters are developed by the Quality

Summary:Purpose: We present three children who underwent right-sided vagus nerve stimulation (R-VNS). This treatment option for people with refractory epilepsy has not been described in children.Methods: We reviewed our database of >350 patients implanted with vagus nerve stimulators and now describe our experience in three patients with R-VNS for the treatment of intractable seizures. All three patients improved dramatically with left-sided vagus nerve stimulation (L-VNS), but the devices had to be removed because of infection. The patients were thought to be at high risk for nerve injury if they were reapproached for L-VNSs; therefore R-VNSs were implanted.Results: All three patients with an R-VNS had a reduction in seizures. Our first patient has had an R-VNS for 5 years; he has been seizure free for >2 years on R-VNS monotherapy. The second patient had an R-VNS for 8 months. His seizure control improved slightly, but not as dramatically as with L-VNS. The third child has had an R-VNS for >7 months and has cessation of his most disabling seizure type (generalized tonicclonic seizures). None of the patients had cardiac side effects from therapeutic R-VNS. However, two of the three patients had respiratory events with R-VNS.Conclusions: VNS is known to be an effective treatment in pharmacoresistant epilepsy. R-VNS should be considered if a patient has significant benefit from L-VNS but is unable to continue with L-VNS. R-VNS appears also to have antiepilepsy effects. Additionally, our case report suggests that in some patients, a differential response is found regarding seizure control with R-VNS or L-VNS, raising the question whether L-VNS failures should pursue a trial of R-VNS. Patients should be cautioned and monitored for reactive airway disease if they undergo R-VNS. More research is needed to compare the effects of rightand left-sided VNS on cardiac and pulmonary function in humans and to determine which has the best antiseizure effect. Key Words:Vagus nerve stimulation-Epilepsy-Intractable seizures-Cardiac effects-VNS-Right-sided.Nonpharmacologic treatment options should be investigated for medically refractory epilepsy patients (1,2). If patients do not qualify for epilepsy surgery or are not good candidates, vagus nerve stimulation (VNS) is an option (3). VNS is approved in the United States as adjunctive therapy in patients older than 12 years with refractory partial-onset seizures (4); however, it is effective in younger children with refractory epilepsy (5).Traditionally VNSs have been placed on the left side. The rationale for stimulating the L-VNS is based more on conventional wisdom than on sound scientific evidence. The VNS physician's manual states, "The NCP system is indicated for use only in stimulating the left vagus nerve in the neck area inside the carotid sheath," and "The safety and efficacy of the NCP System treatment have not been established for stimulation of the right vagus nerve" (6). We report therapeutic right-sided VNS in three children without cardiac side effects. The patient...