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Benign pediatric localization-related epilepsies. Part I. Syndromes in infancy
Lama M Chahine1, Mohamad A Mikati
1American University of Beirut Medical Center, P.O. Box 11-0236/B52, Beirut, Lebanon.
Insights
Pediatric benign epilepsy syndromes are increasingly recognized, but long-term follow-up is crucial to confirm their benign nature. Some cases challenge the definition, necessitating careful diagnosis and potential use of "possible" or "probable" syndrome classifications.
Area of Science:
- Neurology
- Pediatrics
- Epileptology
Background:
- Growing interest in classifying pediatric benign epilepsy syndromes.
- Benign epilepsy syndromes are typically defined by normal development, good treatment response, and no lasting effects.
- The concept of benign epilepsy is challenged by a minority of patients with persistent seizures or neuropsychological issues.
Purpose of the Study:
- To review the concept and diagnosis of pediatric benign epilepsy syndromes.
- To discuss the epidemiology, clinical features, EEG, diagnosis, and management of specific benign epilepsy syndromes.
- To highlight the importance of long-term follow-up for confirming benign epilepsy diagnoses.
Main Methods:
- Literature review of pediatric benign localization-related epilepsy syndromes.
- Analysis of diagnostic criteria, differential diagnoses, genetics, and prognosis.
- Examination of specific syndromes including benign infantile familial convulsions and various benign partial epilepsies.
Main Results:
- The definition of benign epilepsy is complex, with some cases not following a benign course.
- Long-term follow-up is essential for definitive diagnosis of benign epilepsy syndromes.
- Suggests using "possible" or "probable" for syndrome classification until confirmed by long-term outcomes.
Conclusions:
- Accurate diagnosis of pediatric benign epilepsy requires careful consideration of long-term outcomes.
- The classification of benign epilepsy syndromes should be approached with caution due to variable patient trajectories.
- Further research and standardized long-term follow-up protocols are needed for precise classification.
Abstract:
There is currently increasing interest in identifying and classifying pediatric benign epilepsy syndromes and recently several new syndromes have been recognized. Benign epilepsy syndromes, by definition, occur in children with normal developmental history, respond well to therapy, and remit without sequelae. The large majority of children with benign epilepsy syndromes follow a truly benign course. The concept of benign epilepsy syndromes has, however, been challenged by the minority of patients who continue to have seizures despite therapy, develop new seizures after initial remission, or exhibit neuropsychological abnormalities. Without long-term follow-up, benignity can not be truly ascertained a priori. Thus it may be preferable to use the terms possible and probable before the name of a specific syndrome until such time that the diagnosis of a definite benign syndrome is confirmed on long-term follow-up. In this review of the pediatric benign localization-related epilepsy syndromes, we address the concept of benignity and the process of diagnosis of a benign epilepsy syndrome. In addition we review the epidemiology, clinical manifestations, EEG findings, work-up, diagnostic criteria, differential diagnosis, genetics, management and prognosis of benign infantile familial convulsions, benign partial epilepsy in infancy with complex partial seizures, benign partial epilepsy in infancy with secondarily generalized seizures, benign infantile convulsions associated with mild gastroenteritis, and benign infantile focal epilepsy with midline spikes and waves during sleep.
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