Vagus nerve stimulation (VNS) is effective in treating catastrophic 1 epilepsy in very young children

Zamponi, Nelia; Rychlicki, F.; Corpaci, Ludovica; Cesaroni, Elisabetta; Trignani, Roberto

doi:10.1007/s10143-008-0134-8

Cited by 74 publications

(43 citation statements)

References 20 publications

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“…Data related to infants are sparse, and limited to either small proportions of larger case series, or small case reports of up to six infants. [22][23][24][25][26] Because epileptic spasms are the more common single seizure type to occur in infancy, this condition was assessed in isolation. West syndrome is characterized by an electroclinical triad of (1) epileptic spasms, (2) hypsarrhythmia on EEG study, and (3) developmental stagnation or regression.…”

Section: Approach To Therapy and Interventions In Infantile-onset Seimentioning

confidence: 99%

Summary of recommendations for the management of infantile seizures: TaskForceReport for theILAE Commission ofPediatrics

et al. 2015

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Summary Evidence‐based guidelines, or recommendations, for the management of infants with seizures are lacking. A Task Force of the Commission of Pediatrics developed a consensus document addressing diagnostic markers, management interventions, and outcome measures for infants with seizures. Levels of evidence to support recommendations and statements were assessed using the American Academy of Neurology Guidelines and the Grading of Recommendations Assessment, Development and Evaluation (GRADE) system. The report contains recommendations for different levels of care, noting which would be regarded as standard care, compared to optimal care, or “state of the art” interventions. The incidence of epilepsy in the infantile period is the highest of all age groups (strong evidence), with epileptic spasms the largest single subgroup and, in the first 2 years of life, febrile seizures are the most commonly occurring seizures. Acute intervention at the time of a febrile seizure does not alter the risk for subsequent epilepsy (class 1 evidence). The use of antipyretic agents does not alter the recurrence rate (class 1 evidence), and there is no evidence to support initiation of regular antiepileptic drugs for simple febrile seizures (class 1 evidence). Infants with abnormal movements whose routine electroencephalography (EEG) study is not diagnostic, would benefit from video‐EEG analysis, or home video to capture events (expert opinion, level U recommendation). Neuroimaging is recommended at all levels of care for infants presenting with epilepsy, with magnetic resonance imaging (MRI) recommended as the standard investigation at tertiary level (level A recommendation). Genetic screening should not be undertaken at primary or secondary level care (expert opinion). Standard care should permit genetic counseling by trained personal at all levels of care (expert opinion). Genetic evaluation for Dravet syndrome, and other infantile‐onset epileptic encephalopathies, should be available in tertiary care (weak evidence, level C recommendation). Patients should be referred from primary or secondary to tertiary level care after failure of one antiepileptic drug (standard care) and optimal care equates to referral of all infants after presentation with a seizure (expert opinion, level U evidence). Infants with recurrent seizures warrant urgent assessment for initiation of antiepileptic drugs (expert opinion, level U recommendation). Infantile encephalopathies should have rapid introduction and increment of antiepileptic drug dosage (expert opinion, level U recommendation). There is no high level evidence to support any particular current agents for use in infants with seizures. For focal seizures, levetiracetam is effective (strong evidence); for generalized seizures, weak evidence supports levetiracetam, valproate, lamotrigine, topiramate, and clobazam; for Dravet syndrome, strong evidence supports that stiripentol is effective (in combination with valproate and clobazam), whereas weak evidence supports that topiramate, zonisamide,...

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Section: Approach To Therapy and Interventions In Infantile-onset Seimentioning

confidence: 99%

Summary of recommendations for the management of infantile seizures: TaskForceReport for theILAE Commission ofPediatrics

et al. 2015

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“…4 Many children with catastrophic epilepsies also have seizures as a result of clinically important underlying brain abnormalities 21,22 and are very pharmacoresistant. 23,24 The results suggest that infants are a particularly treatment-resistant patient population. By contrast, seizures associated with epilepsies of later onset in patients older than 2 years, which usually have different underlying causes, are more readily treatable with AEDs.…”

Section: Resultsmentioning

confidence: 96%

Randomized trial of adjunctive topiramate therapy in infants with refractory partial seizures

et al. 2010

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Objective: To evaluate the efficacy and safety of adjunctive topiramate (sprinkle capsules or oral liquid) in reducing daily rates of partial-onset seizures (POS) in infants with refractory POS. Methods:In this double-blind, placebo-controlled, parallel-group, international study, infants (n ϭ 149) with clinical or EEG evidence of refractory POS were randomly allocated (1:1:1:1) to receive adjunctive topiramate 5, 15, or 25 mg/kg/d or placebo for 20 days. The primary variable was the median percentage reductions in daily POS rate from baseline to final assessment as recorded on a 48-hour video-EEG. Results:Of the 149 infants (mean age 12 months) included in the intent-to-treat analysis set, 130 completed the study. Median percentage reduction from baseline in daily POS rate was not significantly different (p ϭ 0.97) between topiramate 25 mg/kg (20.4%) and placebo (13.1%). Lower doses were not formally tested, but nominal p values for comparisons with placebo were not significant (15-mg/kg/d dose: p ϭ 0.97; 5-mg/kg/d dose: p ϭ 0.91). Treatment-emergent fever, diarrhea, vomiting, anorexia, weight decrease, somnolence, and viral infection occurred more frequently (Ն10% difference) with topiramate than with placebo. The incidence of newly diagnosed epilepsy is highest in the first year of life and includes many syndromes that are unique and are difficult to diagnose and classify. Conclusion:1,2 Also, seizure types and epilepsy syndromes evolve with age, making accurate diagnoses difficult or retrospectively incorrect in up to 25% of cases. 3,4 Contributing to the treatment challenges, many randomized controlled trials of newer antiepileptic drugs (AEDs) did not assess efficacy in children, and most available AEDs have not been rigorously investigated in infants, leaving physicians with incomplete data on which to base treatment decisions.5 A sizeable number of epilepsy syndromes are significantly different from those in adults. Therefore, clinical trial design in this population requires careful evaluation and must be different from adult trials.6 Drug dosing is also more difficult in children and often requires adjustment as the child matures. e-Pub ahead of print on January 20, 2010, at www.neurology.org. Investigators and institutions at which trials were performed are listed in appendix e-1 on the Neurology ® Web site at www.neurology.org.

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“…Data related to the efficacy and safety of VNS in infants are sparse, limited to class 4 studies, and respondents were limited in their approach to use of this device in infants. [37][38][39][40][41] No randomized controlled trials were identified for infants with seizures and epilepsy surgery. There is expert consensus with regard to referral and assessment of children for epilepsy surgery that recognizes infants as a high risk but important group, and as such, all should be assessed early in their natural history.…”

Section: Discussionmentioning

confidence: 99%

Treatment of infants with epilepsy: Common practices around the world

et al. 2015

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SUMMARYObjectives: High quality data to guide recommendations for infants with epilepsy are lacking. This study aimed to develop an understanding of common practice and regional variations in the treatment interventions of infants with epilepsy, and also to identify areas for further study and to highlight where common practice occurs without sound evidence. Method: A survey addressed clinical treatment practice for infants with epilepsy. Alternative interventions were included. Results: The survey found that most regions had similar practice for first-line interventions, except for North America, where more levetiracetam was prescribed. There was a preference for valproate as first-line therapy for generalized seizures, myoclonic seizures, and Dravet syndrome; only Oceania differed for generalized and myoclonic seizures. Phenobarbital was used for generalized and focal seizures in resource-poor and resource-equipped regions. Carbamazepine and oxcarbazepine were the preferred agents for focal seizures from all regions except North America, which uses more levetiracetam. For second-and third-line interventions, the range of choices was diverse, often with little correlation across regions. The ketogenic diet, vagus nerve stimulation, and epilepsy surgery were considered viable choices in most settings, but usually only once seizures were considered medically refractory. The survey highlighted the marked discrepancy in Africa, the one region that consistently confirmed a lack of access to these alternative interventions and to the newer antiepileptic drugs. Significance: More randomized controlled trials in infants with seizures are needed to permit useful recommendations. The survey identified widespread use of levetiracetam in North America, which may be the result of effective marketing or based on good clinical practice. The widespread use of valproate may have safety implications. The lack of access to care in the African region highlighted the need for more sustained resources. Although the survey was not evidence based, the findings could be useful to support additional well-designed studies.

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Vagus nerve stimulation (VNS) is effective in treating catastrophic 1 epilepsy in very young children

Cited by 74 publications

References 20 publications

Summary of recommendations for the management of infantile seizures: TaskForceReport for theILAE Commission ofPediatrics

Summary of recommendations for the management of infantile seizures: TaskForceReport for theILAE Commission ofPediatrics

Randomized trial of adjunctive topiramate therapy in infants with refractory partial seizures

Treatment of infants with epilepsy: Common practices around the world

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