Related Experiment Video
Updated: Aug 7, 2026

Electromagnetic Source Imaging in Presurgical Evaluation of Children with Drug-Resistant Epilepsy
Published on: September 20, 2024
The spectrum of benign infantile seizures
Nicola Specchio1, Federico Vigevano
1Department of Neuroscience, Bambino Gesù Children's Hospital, Piazza Sant'Onofrio 4, 00165 Roma, Italy.
Insights
Benign epilepsies in infancy, including benign familial infantile seizures (BFIS), require clinical and nosological clarification. This review details various benign infantile epilepsy syndromes, their genetics, and diagnostic criteria for better understanding.
Area of Science:
- Pediatric Neurology
- Clinical Epilepsy Research
- Medical Genetics
Background:
- Infantile epilepsies present diagnostic challenges, with historical reports highlighting benign outcomes.
- Key syndromes like benign familial infantile seizures (BFIS) and benign familial neonatal-infantile seizures (BFNIS) have been identified.
- Genetic loci and clinical associations, including choreoathetosis and hemiplegic migraine, have been linked to these conditions.
Purpose of the Study:
- To review and clarify various benign epilepsy syndromes occurring in infancy.
- To discuss the clinical and nosological aspects of these conditions.
- To differentiate these syndromes from other benign epilepsies like benign neonatal seizures and later-onset childhood epilepsies.
Main Methods:
- Literature review of historical and recent studies on infantile epilepsy syndromes.
- Analysis of clinical presentations, genetic findings, and outcomes.
- Comparison with established epilepsy classifications, such as the International League Against Epilepsy (ILAE) classification.
Main Results:
- Several distinct benign infantile epilepsy syndromes have been described, including BFIS, BFNIS, benign infantile seizures with mild gastroenteritis (BIS with MG), and benign infantile focal epilepsy with midline spikes and waves during sleep (BIMSE).
- Genetic studies have identified specific chromosomal loci associated with BFIS.
- These syndromes are increasingly being classified, with distinctions made between familial and non-familial forms.
Conclusions:
- A comprehensive understanding of benign infantile epilepsies is crucial for accurate diagnosis and management.
- Further research is needed to refine nosological classifications and understand the genetic underpinnings.
- Distinguishing these syndromes from other benign epilepsies ensures appropriate clinical care and prognosis.
Abstract:
Benign epilepsies during infancy are a wide topic, which needs both clinical and nosological clarifications. Already in 1963 Fukuyama reported patients with seizures during infancy with a benign outcome. In the late 80s and early 90s, Watanabe reported series of infants with complex partial seizures or partial seizures with secondary generalization, with a normal development before onset and a benign outcome. In the same years Vigevano focused on familial cases: he described several families with seizures with onset around the 6-month of age, and autosomal dominant mode of inheritance. To define this condition, he coined the term "benign familial infantile seizures" (BFIS). Afterwards, studying families with this phenotype, loci on chromosomes 19, 16 and 2 responsible for BFIS were detected. Similar loci were found in families affected by BFIS and subsequent choreoathetosis, and BFIS associated with familial hemiplegic migraine. In most recent years a new form of benign epilepsy has been proposed, with an intermediate onset between the neonatal and infantile age, which was defined with the term benign familial neonatal-infantile seizures (BFNIS). This condition could have some clinical and genetic features overlapping with BFIS. Seizures with a benign outcome have been reported also in infants during episode of mild gastroenteritis (BIS with MG) frequently with positive Rotavirus antigen. Lastly, sleep EEG abnormalities have been reported in children with a peculiar form of epilepsy by Capovilla, who defined this condition as benign infantile focal epilepsy with midline spikes and waves during sleep (BIMSE). Some of these entities have been included in the last classification proposed by the ILAE and have been differentiated in familial and non-familial forms. The aim of this review is to describe these entities, discuss their nosological aspects, pointing out the similarities and differences with benign neonatal seizures and benign focal epilepsies appearing later in life such as early-onset benign occipital seizure susceptibility syndrome (EBOSS), or benign epilepsy of childhood with centro-temporal spikes (BECTS).
Related Concept Videos
Seizures: Classification
Seizures are typically classified into two main categories: focal and generalized seizures.
Focal Seizures
Focal seizures originate from specific regions of the brain. These seizures are further sub-classified into two types:
Seizures l: Introduction
Seizures ll: Types
Epilepsy ll: Types
Epilepsy and Seizures: Overview
Various factors can trigger epilepsy, including genetic factors, brain damage, metabolic causes, and unknown etiology. Diagnosis of epilepsy involves electroencephalography (EEG), which...
Antiepileptic Drugs: GABAergic Pathway Potentiators
The key GABA pathway potentiators used in epilepsy management are as follows.
Benzodiazepines are a well-known class of drugs used for their...

