Migrating partial seizures of infancy

Yoshimi Sogawa MD (Dr. Sogawa of the University of Pittsburgh has no relevant financial relationships to disclose.)
Solomon L Moshé MD (Dr. Moshe of Albert Einstein College of Medicine received honorariums from UCB as a member of a data and safety monitoring board.)
Jerome Engel Jr MD PhD, editor. (Dr. Engel of the David Geffen School of Medicine at the University of California, Los Angeles, has no relevant financial relationships to disclose.)
Originally released March 15, 1999; last updated July 2, 2014; expires July 2, 2017

Overview

Migrating partial seizures in infancy is a rare infantile epileptic encephalopathy of early onset characterized by normal development before seizure onset; seizure onset in the first 6 months of life; seizure focus that migrates from one area of the cortex to the other; rapidly progressive to nearly continuous seizures; and permanent deterioration of the psychomotor abilities. The majority of cases are sporadic. Recently, several gene mutations have been reported by different groups, ranging from SCN1A, PLCB1, and KCNT1. No effective treatment has been identified. In this article, the authors focus on advances in detecting gene mutations and treatment.

Key points

 

• Migrating partial seizures in infancy are a rare, intractable, early infantile epileptic encephalopathy.

 

• There are multifocal bilateral independent seizure foci.

 

• It is characterized by rapidly progressive to nearly continuous seizure discharges.

 

• It results in regression and severe global developmental delay, including acquired microcephaly.

 

SCN1A, PLCB1, KCNT1, and TBC1D24 mutations have recently been reported.

Historical note and terminology

A report in 1995 by Coppola and colleagues described 14 infants who developed migrating partial seizures (Coppola et al 1995). The first seizures had occurred at the mean age of 3 months, and the full pattern was developed between 1 month and 10 months of age. Patients regressed developmentally. Three of them died between 7 months and 8 years of age. Only 2 patients had seizures controlled by medication, and 3 patients resumed psychomotor development. Similar cases were subsequently reported in other countries such as Japan (Okuda et al 2000), European countries (Wilmshurst et al 2000; Veneselli et al 2001; Hahn et al 2007), Israel (Gross-Tsur et al 2004), and the United States (Marsh et al 2005).

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