Summary of recommendations for the management of infantile seizures: Task F orce R eport for the ILAE C ommission of P ediatrics
Open accessAbstract
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, valproate, bromide, and the ketogenic diet are possibly effective; and for Ohtahara syndrome, there is weak evidence that most antiepileptic drugs are poorly effective. For epileptic spasms, clinical suspicion remains central to the diagnosis and is supported by EEG, which ideally is prolonged (level C recommendation). Adrenocorticotropic hormone (ACTH) is preferred for short‐term control of epileptic spasms (level B recommendation), oral steroids are probably effective in short‐term control of spasms (level C recommendation), and a shorter interval from the onset of spasms to treatment initiation may improve long‐term neurodevelopmental outcome (level C recommendation). The ketogenic diet is the treatment of choice for epilepsy related to glucose transporter 1 deficiency syndrome and pyruvate dehydrogenase deficiency (expert opinion, level U recommendation). The identification of patients as potential candidates for epilepsy surgery should be part of standard practice at primary and secondary level care. Tertiary care facilities with experience in epilepsy surgery should undertake the screening for epilepsy surgical candidates (level U recommendation). There is insufficient evidence to conclude if there is benefit from vagus nerve stimulation (level U recommendation). The key recommendations are summarized into an executive summary. The full report is available as Supporting Information. This report provides a comprehensive foundation of an approach to infants with seizures, while identifying where there are inadequate data to support recommended practice, and where further data collection is needed to address these deficits.
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For seizures starting between 3 and 12 months, SCN2A-specific data are limited, so standard AED guidelines are recommended.
“For seizures starting between 3 months and 12 months there are limited SCN2A-specific data, so standard AED guidelines are recommended [53].”
From Progress in Understanding and Treating SCN2A-Mediated Disorders (2018) · cites this paper as [53]
The Approach to therapy and interventions section recommends early AED treatment for infants, and the Discussion says evidence for specific treatments in infants is limited. Neither passage addresses SCN2A-specific data or singles out seizures beginning between 3 and 12 months; the report offers general infantile-seizure recommendations rather than the stated SCN2A-specific rationale. (LLM, conf=0.86)
Experimental — accuracy not yet measuredThere are ASM protocols published for specific DEE syndromes based on expert consensus.
“There are ASM protocols published for specific DEE syndromes based on expert consensus 181,182 .”
From Developmental and epileptic encephalopathies (2024) · cites this paper as [182]
The effectiveness of sodium channel blockers in early-onset SCN2A seizures is opposite to current guidelines for empiric neonatal seizure treatment and is a compelling reason for rapid genetic testing.
“Of note, this is the opposite of current guidelines for empiric neonatal seizure treatment [53] and is a compelling reason for rapid genetic testing for this condition [54,55].”
From Progress in Understanding and Treating SCN2A-Mediated Disorders (2018) · cites this paper as [53]
The cited paper’s Summary and Table 6 (page not available in our copy) discuss treatment of infantile seizures, but do not give an empiric neonatal treatment recommendation to compare with sodium-channel blockers. A search for neonatal treatment and SCN2A found no passage establishing the claimed opposition; the separate rapid-testing rationale is cited to other sources [54,55]. (LLM, conf=0.74)
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Sourbron, J., Klinkenberg, S., van Kuijk, S. M. J., Lagae, L., Lambrechts, D., Braakman, H. M. H., & Majoie, M. (2020) · Child's Nervous System · doi:10.1007/s00381-020-04578-7 · Cited by 111 · see citing works
Epileptic spasms: A South African overview of aetiologies, interventions, and outcomes
Raga, S. V., Essajee, F., Solomons, R., Van Toorn, R., & Wilmshurst, J. M. (2023) · Developmental Medicine & Child Neurology · doi:10.1111/dmcn.15433 · Cited by 10 · see citing works
Self-citation
- Authors
- Wilmshurst, J. M., Gaillard, W. D., Vinayan, K. P., Tsuchida, T. N., Plouin, P., Van Bogaert, P., Carrizosa, J., Elia, M., Craiu, D., Jovic, N. J., Nordli, D., Hirtz, D., Wong, V., Glauser, T., Mizrahi, E. M., & Cross, J. H.
- Affiliations
- University of Cape Town (ZA)
- George Washington University (US)
- Amrita Institute of Medical Sciences and Research Centre (IN)
- Children's National (US)
- Inserm (FR)
- Paediatric Neurology Department of Pediatrics Erasme Hospital Université Libre de Bruxelles Brussels Belgium
- Universidad de Antioquia (CO)
- Oasi Maria SS (IT)
- Carol Davila University of Medicine and Pharmacy (RO)
- Neurology Clinic of Neurology and Psychiatry for Children and Youth Faculty of Medicine University of Belgrade Belgrade Serbia
- Lurie Children's Hospital (US)
- National Institutes of Health (US)
- Queen Mary Hospital (CN)
- Cincinnati Children's Hospital Medical Center (US)
- Baylor College of Medicine (US)
- Great Ormond Street Hospital (GB)
- Corresponding author
- Wilmshurst, Jo M.
- ORCID
- Venue
- Epilepsia
- Publisher
- Wiley
- Published
- 2015-06-30
- Volume
- 56
- Issue
- 8
- Pages
- 1185-1197
- ISSN
- 0013-9580 (ISSN-L)
- 1528-1167
- OpenAlex
- W1940036272
- Cited by
- 418
- References
- 80
- Citations by year
- 2026: 22 · 2025: 29 · 2024: 31 · 2023: 36 · 2022: 47 · 2021: 57 · 2020: 69 · 2019: 38 · 2018: 33 · 2017: 25 · 2016: 25 · 2015: 6
- FWCI
- 4.08
- Type
- journal article
- Language
- en
- Open access
- bronze
- doi.org
- Locations
- Epilepsia (publishedVersion)
- PubMed (publishedVersion)
- Institutional Repository University of Antioquia (University of Antioquia) (submittedVersion, cc-by-nc-nd)
- Dépôt institutionnel de l'Université libre de Bruxelles (Université Libre de Bruxelles) (submittedVersion)
- UCL Discovery (University College London) (submittedVersion)
- Funders
- Epilepsy Foundation (1P30HD40677-01, 2K12NS052159-06A1, RO1 MH084961, 1R21MH092615, 095998, 1UO1DP003255, DOD/USAMRAA, PCORI)
- Citizens United for Research in Epilepsy (CURE)
- Vitaflo
- Action Medical Research
- Epilepsy Research United Kingdom
- Sparks - Children's Medical Research Charity
- National Institute for Health and Research
- Great Ormond Street Hospital Children's Charity
- National Institute of Neurological Disorders and Stroke (NINDS
- National Institute of Mental Health (NIMH
- National Science Foundation (NSF
- Centers for Disease Control and Prevention (CDC
- Department of Defense/United States Army Medical Research Acquisitions Activity (W81XWH-11-2-0198)
- Patient-Centered Outcomes Research Institute
- American Epilepsy Society
- Infantile Epilepsy Research Foundation
- Topics
- Health Sciences › Medicine › Psychiatry and Mental health › Epilepsy research and treatment
- Health Sciences › Medicine › Psychiatry and Mental health › Cerebral Palsy and Movement Disorders
- Health Sciences › Medicine › Physiology › Diet and metabolism studies
- Keywords
- infantile seizures, infantile epilepsy, febrile seizures, epileptic spasms, antiepileptic drugs, levetiracetam, anticonvulsant adverse effects, antiepileptic drug resistance, Dravet syndrome, genetic counseling, video-EEG monitoring, neuroimaging
- MeSH terms
- Anticonvulsants, Seizures, Febrile, Electroencephalography, Epilepsy, Humans, Infant, Infant, Newborn, Spasms, Infantile, Practice Guidelines as Topic, Disease Management, Advisory Committees, Neuroimaging
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