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Long-term course of childhood epilepsy with intractable grand mal seizures
T Fujiwara1, M Watanabe, Y Takahashi
1National Epilepsy Center, Shizuoka Higashi Hospital, Japan.
Insights
This study followed 29 children with severe epilepsy for over 5 years. Results show generalized tonic-clonic seizures persisted, impacting mental development, with a shift from waking to sleep seizures as children aged.
Area of Science:
- Pediatric Neurology
- Epileptology
- Clinical Neuroscience
Background:
- Childhood epilepsy often presents with frequent generalized tonic-clonic seizures, even with maximum antiepileptic drug dosages.
- Early onset (before 1 year) and persistent seizures pose significant challenges in managing pediatric epilepsy.
Purpose of the Study:
- To investigate the long-term seizure patterns and developmental outcomes in children with severe early-onset epilepsy.
- To differentiate between severe myoclonic epilepsy in infancy (SME) and intractable childhood epilepsy with generalized tonic-clonic seizures (GTC).
Main Methods:
- Longitudinal follow-up of 29 children with severe epilepsy for over 5 years.
- Classification of patients into three groups: SME, no SME, and intractable childhood epilepsy with GTC.
- Analysis of seizure types, seizure timing (waking vs. sleep), and mental development prognosis.
Main Results:
- Generalized tonic-clonic seizures persisted across all groups, while other seizure types decreased with age.
- A majority of patients experienced a shift from waking to sleep-related generalized tonic-clonic seizures.
- Poor prognosis for mental development was observed in the majority of cases.
- Monozygotic twins with SME suggested a potential genetic component.
Conclusions:
- Childhood epilepsy with persistent generalized tonic-clonic seizures has a poor developmental prognosis.
- Seizure patterns evolve with age, often transitioning to sleep-related occurrences.
- Intractable childhood epilepsy with GTC lacks distinct syndrome characteristics, necessitating further research into pathogenesis and treatment.
Abstract:
Twenty-nine children with childhood epilepsy characterized by frequent grand mal (generalized tonic-clonic) seizures in spite of maximal doses of antiepileptic drugs and by an early onset of seizures (before 1 year of age) were followed up for more than 5 years. The children were divided into 3 groups: severe myoclonic epilepsy in infancy (SME), no SME, and intractable childhood epilepsy with generalized tonic-clonic seizures (GTC). In all the 3 groups, the grand mal seizures persisted, whereas the other types of seizures tended to disappear as the patients aged, and the prognosis for mental development was poor. In the majority of cases in all the 3 groups, the waking grand mal seizures altered to sleep grand mal seizures with aging. Two pairs of monozygotic twins with SME suggested that genetic factors play a role in this epileptic syndrome. Intractable childhood epilepsy with GTC is distinguished by the absence of other types of generalized seizures. It cannot be regarded as an epileptic syndrome, but its pathogenesis and treatment require further studies.