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Migrating partial seizures in infancy: a malignant disorder with developmental arrest
Insights
A novel epileptic condition in infants presents with continuous multifocal seizures and developmental regression. This severe neurological disorder, with unknown etiology, leads to significant disability and mortality in affected children.
Area of Science:
- Pediatric Neurology
- Epileptology
- Clinical Neuroscience
Background:
- A previously unreported epileptic condition in infants is described.
- The condition is characterized by nearly continuous multifocal seizures starting around 3 months of age without identifiable risk factors.
Purpose of the Study:
- To characterize a novel epileptic syndrome in infants.
- To investigate the clinical, electroencephalographic (EEG), and neuropathological features of this condition.
- To explore potential etiologies and outcomes.
Main Methods:
- Clinical observation of 14 infants with the condition.
- Electroencephalography (EEG) to analyze seizure patterns.
- Neuropathological examination of brain tissue in two cases.
Main Results:
- Seizures were multifocal, frequent, and involved random cortical areas with a consistent alpha or theta rhythmic EEG pattern.
- Infants experienced developmental regression, quadriplegia, and severe hypotonia.
- Three patients died, and seizure control was achieved in only two; psychomotor development resumed in only three.
Conclusions:
- This study defines a new, severe infantile epileptic syndrome with poor prognosis.
- The etiology remains unknown, with no familial recurrence observed.
- Neuropathology revealed significant hippocampal neuronal loss and gliosis.
Abstract:
Fourteen infants of both sexes had a previously unreported epileptic condition characterized by nearly continuous multifocal seizures. The first seizures occurred at a mean age of 3 months, without antecedent risk factors. At 1 to 10 months, the seizures became very frequent. They were partial with variable clinical expression, and the EEG showed that the discharges randomly involved multiple independent sites, moving from one cortical area to another in consecutive seizures. Although their topography varied, the EEG ictal pattern of each seizure was very similar. It consisted of rhythmic alpha or theta activity which spread to involve an increasing area of the cortical surface. Patients regressed developmentally and became quadriplegic with severe axial hypotonia. Three patients died at age 7 months and at age 7 and 8 years, respectively. Seizures were controlled in only 2 patients, and only 3 children resumed psychomotor development. Extensive investigation failed to determine an etiology, and there was no familial recurrence. Neuropathological examination of the brain in two cases showed only severe hippocampal neuronal loss and accompanying gliosis.