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Published on: September 20, 2024
[Clinical polymorphism of malignant epilepsy of infancy with migrating multifocal seizures (8 cases)]
Abstract:
Malignant migrating partial seizures in infancy are rare epilepsy syndrome that begins in the first 6 months of life and characterized by multiple continuous electroencephalographic and electroclinical focal ictal patterns which involved different independent areas of both hemispheres with arrest of psychomotor development. The present detailed review is based on the personal observation of 8 patients newly diagnosed at the Russian Children Clinical hospital, Moscow, Russia. At least three ictal patterns recorded from different independent areas of both hemispheres were fixed by video-EEG-monitoring in all patients. The high polymorphism and very frequent seizures (not less than five types at every child) were observed. The cases were pharmacoresistant, with the absence of reaction to antiepileptic therapy and progressive deterioration in 4 (50%) patients. Decreasing of seizure frequency by 50% was achieved in 3 (37.5%) patients treated with the combination of valproates, benzodiazepines and barbiturates and by 75% in 1 (12.5%) patient case treated with valproates, benzodiazepines and levetyracetam (keppra). The authors proposed a definition of this epileptic syndrome as: "malignant epilepsy of infancy with migrating multifocal seizures".
Insights
Malignant epilepsy of infancy with migrating multifocal seizures is a rare, severe epilepsy syndrome starting in early infancy. This condition is characterized by frequent, diverse seizures and developmental arrest, often proving resistant to standard treatments.
Area of Science:
- Neurology
- Pediatric Neurology
- Epileptology
Background:
- Malignant migrating partial seizures in infancy (MMPSI) is a rare epilepsy syndrome.
- It begins within the first six months of life.
- Characterized by continuous electroencephalographic (EEG) and electroclinical focal ictal patterns involving independent areas of both hemispheres, leading to psychomotor development arrest.
Observation:
- This review details observations of 8 newly diagnosed patients at a Moscow hospital.
- All patients exhibited at least three distinct ictal patterns from independent hemispheric areas via video-EEG monitoring.
- High seizure polymorphism (≥5 types per child) and frequency were noted.
Findings:
- The observed cases were largely pharmacoresistant to antiepileptic therapies.
- Progressive deterioration occurred in 50% of patients.
- A combination of valproates, benzodiazepines, and barbiturates reduced seizure frequency by 50% in 37.5% of patients; valproates, benzodiazepines, and levetyracetam achieved 75% reduction in one case.
Implications:
- The study proposes a definition: "malignant epilepsy of infancy with migrating multifocal seizures".
- Highlights the need for specialized treatment approaches for this severe infantile epilepsy.
- Emphasizes the challenges in managing pharmacoresistant epilepsy in infants and the potential role of specific drug combinations.
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