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Myoclonic epilepsy in infancy with preceding or concurrent afebrile generalized tonic-clonic seizures in Chinese
Zhixian Yang1, Hui Li1, Jiao Xue1
1Department of Pediatrics, Peking University First Hospital, Beijing, China.
Insights
Myoclonic epilepsy in infancy (MEI) with or without afebrile generalized tonic-clonic seizures (GTCS) showed similar outcomes. Afebrile GTCS may indicate stronger cortical hyperexcitability in MEI patients.
Area of Science:
- Neurology
- Pediatric Epilepsy
Background:
- Myoclonic epilepsy in infancy (MEI) is a severe form of childhood epilepsy.
- Understanding MEI subtypes, particularly those with afebrile generalized tonic-clonic seizures (GTCS), is crucial for diagnosis and management.
Purpose of the Study:
- To investigate the clinical characteristics and classification of MEI.
- To compare MEI patients with and without afebrile GTCS.
Main Methods:
- Retrospective analysis of 33 children with MEI confirmed by video-electroencephalogram (VEEG).
- Patients were categorized based on the occurrence of afebrile GTCS relative to myoclonic seizures (MS).
- Clinical data, VEEG features, and patient outcomes were analyzed.
Main Results:
- No significant differences in demographics, seizure history, age at onset/control, MS duration, or psychomotor development were found among the three groups (typical MEI, MEI with preceding GTCS, MEI with concurrent GTCS).
- Patients experiencing GTCS (groups 2 and 3) often required multiple antiepileptic drugs for seizure control compared to typical MEI.
- Afebrile GTCS was linked to increased cortical hyperexcitability.
Conclusions:
- MEI with or without afebrile GTCS exhibits similar clinical and EEG features and outcomes.
- The presence of afebrile GTCS suggests heightened cortical hyperexcitability.
- Consideration should be given to classifying MEI with preceding or concurrent afebrile GTCS as distinct subgroups or epileptic syndromes.
Objective:
To investigate the general characteristics and the category of myoclonic epilepsy in infancy (MEI) with or without afebrile generalized tonic-clonic seizures (GTCS).
Methods:
Thirty-three children were retrospectively recruited from approximately 42,814 video-electroencephalogram (VEEG) recordings monitored in our department over last nearly 10years. Myoclonic seizures (MS) must be identified by VEEG in all patients. The clinical, EEG features and outcome were analyzed among these patients.
Results:
The 33 patients (25 boys and 8 girls) were divided into three groups: 11 patients with typical MEI; 16 patients with MEI experienced afebrile GTCS before MS onset; and 6 patients with MEI presented afebrile GTCS occurring concurrently with MS. No significant differences were found among the three groups, including gender distribution, family history, personal history of febrile seizures, the age at seizure onset and control, the duration of MS, the interval between age at onset and seizure control, the age at EEG normalization, the interval between seizure onset age and EEG normalization age and normal psychomotor development at the end of follow-up. More patients in group two and group three were controlled by two or three kinds of antiepileptic drugs compared with those in group one.
Conclusions:
In this study, three groups of patients had similar clinical, EEG features and outcome. Afebrile GTCS was associated with a stronger cortical hyperexcitability. It was worth considering whether MEI with preceding or concurrent afebrile GTCS should be recognized as subgroups or different epileptic syndromes independent of MEI.
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