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Electromagnetic Source Imaging in Presurgical Evaluation of Children with Drug-Resistant Epilepsy
Published on: September 20, 2024
[Epilepsy onset between one month and three months of life: our 11 years experience]
R Pérez Delgado1, M Lafuente Hidalgo, J López Pisón
1Grupo de Investigación Neurometabólico, Instituto Aragónes de Ciencias de la Salud, España.
Insights
Early onset epilepsy (1-3 months) indicates a poor prognosis, with many cases showing severe developmental delays or death. Prompt diagnosis and treatment are crucial for potentially treatable conditions like metabolic disorders.
Area of Science:
- Pediatric Neurology
- Epileptology
- Clinical Genetics
Context:
- Epilepsy prognosis is largely determined by its cause.
- Early seizure onset in infants is linked to poorer outcomes.
- Understanding early-onset epilepsy is critical for intervention.
Purpose:
- To review the outcomes of epilepsy in infants with first seizures between 1 and 3 months of age.
- To highlight the etiological spectrum and prognosis in this cohort.
- To emphasize the need for a structured diagnostic-therapeutic approach.
Summary:
- Eighteen infants with epilepsy and onset between 1-3 months were analyzed.
- Causes included Dravet syndrome spectrum, cryptogenic epilepsy, inborn errors of metabolism, infections, and prenatal encephalopathy.
- Fifty percent experienced severe psychomotor delay, and two died, underscoring a poor prognosis.
Impact:
- Corroborates the poor prognosis of early-onset epileptic seizures (1-3 months).
- Stresses the importance of a diagnostic-therapeutic strategy to address etiological diversity and improve outcomes.
- Recommends specific protocols, including vitamin therapy for unexplained convulsions, similar to neonatal cases.
Introduction:
The prognosis of epilepsy is basically determined by its aetiology. Early onset of seizures is generally associated with poor progress.
Material And Methods:
We review our experience in epilepsy with children born after 1 January 1997 and who had their first seizure between 1 and 3 months of age before January 2008.
Results:
Eighteen cases diagnosed with epilepsy and a first seizure between 1 and 3 months of age were included. One case was within the Dravet syndrome spectrum with the c829 T>G c277G heterozygous mutation of the SCN1A gene. Four were cryptogenic epilepsies and thirteen were asymptomatic: 2 were inborn errors of metabolism (biotinidase deficiency with a response to biotin and Leigh's syndrome); 2 were of infectious origin and the remaining nine prenatal encephalopathy. Nine (50%) currently have a severe psychomotor delay and 2 died. The cryptogenic cases had a relatively poor progress.
Conclusions:
Our experience corroborates the poor prognosis associated with early onset, between 1 and 3 months, of epileptic seizures. Given the wide aetiological range and the poor prognosis in the absence of specific treatment, an appropriate diagnostic-therapeutic strategy is required to avoid diagnostic uncertainties and can identify potentially treatable cases, such as some inborn errors of metabolism. In this age group, the protocol for convulsions of unknown cause must be the same as that for neonatal convulsions, including treatment with a vitamin cocktail, after collecting biological samples.
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