Migrating Partial Seizures in Infancy: A Malignant Disorder with Developmental Arrest

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.

De novo SCN1A mutations in migrating partial seizures of infancy

“…Refractory seizures migrate between regions. 2 Later, infantile spasms may occur. 2 Regression and severe global developmental delay are usual with cortical visual impairment and acquired microcephaly.…”

“…2 Later, infantile spasms may occur. 2 Regression and severe global developmental delay are usual with cortical visual impairment and acquired microcephaly. 3 MPSI is a diagnosis of exclusion.…”

“…Onset is typically from 40 days to 3 months (range 1 day-6 months). [1][2][3] MPSI is characterized by multifocal bilateral independent seizures rapidly becoming very frequent, if not continuous. Refractory seizures migrate between regions.…”

Epileptic Encephalopathies: New Genes and New Pathways

“…Recurrent seizures begin before the age of 6 months and commonly start within a few weeks of birth [51]. The seizures in MMPSI are described as partial (or focal) because the seizure activity is confined to focal brain regions.…”

Epileptic Encephalopathy Syndromes in Infancy

“…This represents the basic concept that frequent seizures and/or interictal discharges may significantly disrupt the function of neuronal networks involved in language, learning, memory, behavioral regulation, and other higher cortical functions, resulting in either transient or permanent deficits. For example, continuous abnormal discharges during sleep may cause disruption of hippocampal function and interfere with learning and memory while awake and memory consolidation in sleep (Coppola G, 1995. Moruzzi G, 1995.…”