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Electroclinical signs of benign neonatal familial convulsions
1Service d'Epileptologie Clinique, Hôpitaux Universitaires de Strasbourg, France.
Insights
Benign neonatal familial convulsions are a type of epilepsy with genetic origins. Seizures present as generalized tonic-clonic events, often with asymmetrical features, despite their familial inheritance pattern.
Area of Science:
- Neurology
- Genetics
- Pediatrics
Background:
- Benign neonatal familial convulsions (BNFC) represent an autosomal inherited epileptic syndrome.
- The precise electroclinical characteristics of BNFC seizures remain incompletely understood.
Observation:
- Electroencephalographic (EEG)-video monitoring of 14 seizures in 3 children from two families with BNFC was performed.
- Seizures consistently initiated during sleep, following a brief arousal, with initial bilateral EEG flattening, apnea, and tonic activity.
Findings:
- EEG flattening was succeeded by prolonged bilateral spike-and-wave discharges, accompanied by vocalizations, chewing, and clonic activity.
- Motor and EEG abnormalities exhibited interictal and intra-individual asymmetry.
- Seizures concluded without postictal EEG or clinical depression.
Implications:
- These findings suggest BNFC seizures are a variant of generalized tonic-clonic seizures.
- The observed asymmetry may stem from immaturity of the corpus callosum or other seizure-synchronizing brain structures.
- This study refines the understanding of BNFC electroclinical manifestations.
Abstract:
Benign neonatal familial convulsions comprise a distinct epileptic syndrome with an autosomal mode of transmission. The electroclinical signs of seizures in this syndrome are not yet well defined. In 3 children from two families presenting with benign neonatal familial convulsions, 14 seizures were recorded during electroencephalographic (EEG)-video sessions. All seizures occurred during sleep, after a short arousal reaction. Seizures started with bilateral, symmetrical flattening of the EEG for 5 to 19 seconds; simultaneously there was apnea and tonic motor activity. The EEG flattening was followed by a long (1-2-minute) bilateral discharge of spikes and sharp waves; simultaneously, there were vocalizations, chewing, and focal or generalized clonic activity. The prominence of EEG and motor abnormalities varied between the left and the right from one seizure to the next in any given child. The seizures stopped without EEG or clinical postictal depression. These electroclinical observations suggest that the convulsions of benign neonatal familial convulsions are a form of generalized tonic-clonic seizure whose expression may be asymmetrical, probably because of the immaturity of the corpus callosum or other structures ensuring seizure synchronization.