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A case of malignant migrating partial seizures in infancy as a continuum of infantile epileptic encephalopathy
Eun Hye Lee1, Mi-Sun Yum, Min-Hee Jeong
1Department of Pediatrics, College of Medicine, Kyung Hee University, Seoul, Republic of Korea.
Insights
Malignant migrating partial seizures in infancy (MMPSI) can evolve into West syndrome. This rare condition presents with early-onset, drug-resistant seizures and developmental delays, suggesting a continuum of infantile epileptic encephalopathy.
Area of Science:
- Pediatric Neurology
- Epileptology
- Developmental Neuroscience
Background:
- Malignant migrating partial seizures in infancy (MMPSI) is a severe early-onset epileptic encephalopathy.
- Characterized by continuous, multifocal seizures and poor neurodevelopmental outcomes.
- Its relationship with other infantile epileptic syndromes remains an area of investigation.
Observation:
- A case report details an infant diagnosed with MMPSI at 2 months old.
- The infant presented with refractory multifocal partial seizures and normal neuroimaging.
- Electroencephalogram (EEG) showed migrating epileptiform activities.
Findings:
- The infant's condition progressed, with seizures remaining refractory to multiple antiepileptic drugs and ketogenic diet.
- At 9 months, the infant developed epileptic spasms and hypsarrhythmia on EEG, consistent with West syndrome.
- Severe developmental delay was noted throughout the observation period.
Implications:
- MMPSI may represent a precursor or a continuum of infantile epileptic encephalopathies.
- The evolution to West syndrome highlights the potential for progression in certain severe infantile epilepsies.
- Understanding this progression can inform early diagnosis and management strategies for infantile epileptic encephalopathies.
Abstract:
The syndrome of malignant migrating partial seizures in infancy (MMPSI) is characterized by onset before the age of 6 months, nearly continuous electrographic seizures involving multiple independent areas of onset in both hemispheres, and poor developmental outcome. This report presents a case involving a patient with MMPSI, who later developed West syndrome. At the age of 2 months old, he showed multifocal partial seizures, which were refractory to antiepileptic drugs. His electroencephalogram (EEG) revealed characteristic migrating multifocal epileptiform activities and neuroimaging finding was normal. The focal seizures were refractory to antiepileptic drugs and ketogenic diet. When he was 9 months old, epilepic spasms were observed with hypsarrhythmia on EEG. He also showed severe developmental delay. MMPSI may be a continuum of infantile epileptic encephalpathy and could evolve to West syndrome.
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