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.