The value of immunotherapy in children with initial short-term frequent seizures
Yongheng Zhao1,2, Jun Li1, Liang Gao1,2
1Department of Pediatrics, Qilu Hospital of Shandong University, Jinan, China.
Insights
Even with negative autoimmune antibody tests, consider immunotherapy for children with frequent, acute-onset seizures unresponsive to antiseizure medicines. This treatment shows significant benefits in managing autoimmune epilepsy.
Area of Science:
- Pediatric Neurology
- Immunology
- Epileptology
Background:
- Autoimmune epilepsy is a growing concern in pediatric neurology.
- Identifying specific biomarkers and treatment responses is crucial for effective management.
- The role of immunotherapy in antibody-negative epilepsy requires further investigation.
Purpose of the Study:
- To analyze clinical features, treatment outcomes, and antibody prevalence in children with frequent, short-duration seizures.
- To evaluate the effectiveness of immunotherapy in antibody-negative pediatric epilepsy.
- To assess the utility of the Antibody Prevalence in Epilepsy (APE) score in this population.
Main Methods:
- Retrospective analysis of 9 pediatric patients with acute-onset epilepsy.
- Inclusion criteria: autoimmune-antibody negative, frequent seizures, poor response to antiseizure medicines (ASM).
- Data collected: clinical characteristics, imaging, electrophysiology, treatment response, and APE scores.
Main Results:
- All 9 patients presented with acute-onset, high-frequency, short-duration seizures.
- Standard ASM treatment was largely ineffective.
- Immunotherapy led to seizure freedom in 8 patients and significant reduction in 1 patient, regardless of APE score.
Conclusions:
- Autoimmune epilepsy should be suspected in children with specific seizure patterns and poor ASM response, even with negative antibody tests.
- Early immunotherapy initiation is recommended and yields favorable outcomes.
- Consider immunotherapy even if the Antibody Prevalence in Epilepsy (APE) score is below 4.
Abstract:
This study aimed to discuss clinical characteristics, therapy, and antibody prevalence in epilepsy (APE) score for short-term, frequent epileptic seizures in children who are autoimmune-antibody negative and respond well to immunotherapy. The clinical characteristics, imaging manifestations, electrophysiology, and effective treatment plan of 9 children who met the above criteria were retrospectively analyzed in the Pediatric Neurology Department of Qilu Hospital at Shandong University from June 2019 to December 2021. All 9 patients (6 boys, 3 girls; aged 13 months-11 years and 5 months, median 3.5 years) had acute-onset seizures within 3 months. All had previous normal growth/development with no family history of disease. Seizure types were focal motor seizures (6), generalized tonic-clonic seizures (2), and generalized secondary-to-focal (1); occurred >10 times/day; and lasted <1 min/episode. Formal treatment with ≥2 types of antiseizure medicine (ASM) achieved an unsatisfactory effect. Cranial magnetic resonance imaging showed an abnormal result in 1 case. The APE score was ≥4 in 3 cases and <4 in 6 cases. All patients experienced symptomatic relief with immunotherapy; subsequently, 8 patients were free of recurrence and 1 had significantly reduced seizure frequency. Autoimmune antibody screening is recommended for children who were previously well and have acute-onset epilepsy; high frequency, short-duration seizures; no good response to 2 types of ASM; and other etiologic factors excluded, even with APE score <4. Even with negative autoimmune antibody results, the possibility of autoimmune epilepsy should be considered for urgent initiation of immunotherapy, which can achieve good results.
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