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Intractable epilepsy in a population-based series of mentally retarded children
U Steffenburg1, A Hedström, A Lindroth
1Department of Pediatrics, Göteborg University, Sweden.
Insights
Intractable epilepsy is common in children with mental retardation (MR), often starting early and linked to severe MR and other neurological issues. Focal EEG changes are frequent, even with generalized brain lesions.
Area of Science:
- Pediatric Neurology
- Epileptology
- Neurodevelopmental Disorders
Background:
- Active epilepsy affects children with mental retardation (MR).
- Intractable epilepsy poses significant challenges in this population.
- Understanding characteristics is crucial for management.
Purpose of the Study:
- To analyze the characteristics of intractable epilepsy in children with MR.
- To identify predictive factors for intractable seizures in this cohort.
Main Methods:
- Population-based study utilizing diagnostic, EEG, and special education registers.
- Review of medical files, clinical examinations, and parent/caregiver interviews.
- Reevaluation of EEG, CT, and MRI scans of the central nervous system (CNS).
Main Results:
- 45% of children with MR and active epilepsy had intractable seizures (daily/weekly).
- Earlier age of onset (0.8 years) predicted intractable epilepsy.
- Severe MR, multiple seizure types, status epilepticus, and tonic seizures were predictive factors.
- 91% showed epileptiform EEG activity; 65% had focal activity.
- 70% had brain lesions on CT/MRI (60% generalized).
- Infantile spasms (IS) and Lennox-Gastaut syndrome (LGS) prevalence were 18% and 7% respectively.
Conclusions:
- Children with MR and intractable epilepsy frequently exhibit severe MR and major neuroimpairments.
- Focal EEG abnormalities are common, even when neuroradiology reveals generalized brain lesions.
Purpose:
The characteristics of intractable epilepsy were analyzed in a population-based study of active epilepsy in mentally retarded children aged 6-13 years.
Methods:
Diagnostic registers, EEG laboratory registers, and registers for the Education of the Subnormal were searched. Medical files were scrutinized. Clinical examinations and interviews with parents and caregivers or both were performed. EEG recordings, computed tomography (CT) and magnetic resonance imaging (MRI) of the CNS were reevaluated.
Results:
Forty-five percent (44 of 98) of the children with mental retardation (MR) and active epilepsy had intractable seizures, defined as one or more seizures every day or week. The median age at onset was 0.8 years, as compared with 3.0 years for those with controlled epilepsy. Predictive factors for frequent seizures were the number of seizure types, severe MR, status epilepticus (SE) and tonic seizures. Epileptiform EEG activity was present in 91%, and focal activity in 65%. Brain lesions were detected on CT and MRI in 70%, with generalized lesions in 60%. Concurrent focal epileptiform activity and focal brain lesions on CT/MRI were detected in 26%. The percentages and prevalence rates for infantile spasms (IS) and Lennox-Gastaut syndrome (LGS) were 18% (0.25 in 1,000) and 7% (0.06 in 1,000), respectively. One of 8 children with IS had had previous neonatal seizures, 3 had SE and 1 later developed LGS.
Conclusions:
Children with MR and intractable epilepsy have a high frequency of severe MR and additional major neuroimpairments. EEG recordings frequently showed focal changes despite generalized lesions in neuroradiology.