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Seizure frequency and characteristics in children with Down syndrome
H Goldberg-Stern1, R H Strawsburg, B Patterson
1Epilepsy Center, Schneider Children's Medical Center of Israel, Petah Tiqva, Israel.
Insights
Epileptic seizures affect 8% of children with Down syndrome, with infantile spasms being common. Poor neurodevelopmental outcomes were observed even with seizure control in this group.
Area of Science:
- Neurology
- Genetics
- Pediatrics
Background:
- Seizures are not typically recognized as a primary feature of Down syndrome.
- Understanding the prevalence and characteristics of seizures in individuals with Down syndrome is crucial for comprehensive care.
Purpose of the Study:
- To determine the prevalence of epileptic seizures in a cohort of children and adolescents with Down syndrome.
- To analyze seizure types and treatment outcomes, particularly for infantile spasms.
Main Methods:
- Retrospective analysis of 350 children and adolescents with Down syndrome evaluated between 1985 and 1997.
- Categorization of seizure types including partial, infantile spasms, and generalized tonic-clonic seizures.
- Evaluation of treatment response and neurodevelopmental outcomes.
Main Results:
- Eight percent (28/350) of patients experienced epileptic seizures.
- Infantile spasms were a significant seizure type (32%), with no clear correlation between EEG patterns, treatment type (valproic acid, adrenocorticotropic hormone), and seizure control or remission.
- Poor neurodevelopmental outcomes were noted in the infantile spasm group, irrespective of seizure control.
Conclusions:
- This study confirms an association between seizures and Down syndrome.
- Further prospective research, including national/international registries, is needed to emphasize developmental assessment and long-term follow-up for this population.
Abstract:
Seizures have not historically been considered a major component of Down syndrome. We examined the prevalence of epileptic seizures in 350 children and adolescents with Down syndrome evaluated at a regional center between 1985 and 1997. Results showed that 28 patients (8%) had epileptic seizures: 13 (47%) partial seizures; 9 (32%) infantile spasms, and 6 (21%) generalized tonic-clonic seizures. In the infantile spasm group, there was no relationship between the initial electroencephalogram (EEG) pattern and response to treatment or long-term seizure control, or between type of pharmacologic treatment (valproic acid, adrenocorticotropic hormone or both) and clinical remission, EEG normalization or long-term seizure control. Neurodevelopmental outcome was poor despite good seizure control in the infantile spasm group. This regional study reinforces the relative association of seizures and Down syndrome. A prospective study including a national/international registry with emphasis on developmental assessment and long-term follow up is warranted.