Last modified: 2018-09-09
Abstract
Introduction The aim of our study was to explore the clinical and neurophysiologic characteristics of epilepsy of infancy with migrating focal seizures (EIMFS).
Method We searched the database of all children dignosed as EIMFS in our hospital from January 2010 to May 2018, including clinical manifestation, video-EEG and other examinations.
Results 12 patients were identified, 8 girls and 4 boys. The age at seizure onset ranged from 22 days to 6 months. The seizures manifested with kinds of motor symptoms, such as deviation of head and eyes, blinking, unilateral tonic or clonic convulsions of limbs. Meanwhile, autonomic symptoms could be combined, like cyanosis, chewing, salivation, et al. 9 had status epilepticus. Interictal-EEG showed numerous multiple spikes with 2 cases presenting bust-suppression and 4 hypsarrhythimia. Ictal-EEG represented discharges from multiple independent sites moved from one area to another of the same or the other hemisphere in consecutive zeizures, with the next seizure starting before the end of the previous one. MRI: 5 cases with subarachnoid space broadening, 3 white matter dysplasia and 4 normal. Gene detection: 4 cases with KCNT1 mutation, 2 KCN1A mutation, 1 PCDH19 mutiation, and 1 KMT2D mutation. All the patients received anti-epileptic treatments. 3 cases acqiured seizure-control for 5-8 months, 2 not controlled, 5 died and 2 lost follow-up.
Conclusion EIMFS is a devastating syndrome with severe mental retardation and early onset of nearly continuous multifocal seizures arising independengtly from muiltiple regions of both hemispheres.