Abstracts

Rationale: Infantile spasms (IS) is a severe epileptic encephalopathy of infancy with a poor developmental outcome. The pathophysiologic process underlying IS is still not understood. They are characterized by a great diversity of many etiologies both acquired and genetic that all manifest with the same clinical presentation. Animal models that have been developed are not able to reproduce every aspect of IS. Therefore, studying the possible etiologic relationship between known acquired brain injuries and IS in human beings could provide new insights into the understanding and eventually the prevention of this disorder.Methods: We describe two cases of infantile spasms that developed several weeks after the infants had suffered from a non accidental head injury (NAHI) or shaken-baby syndrome .Results: Two previously normal male infants suffered from NAHI at the age of (1) one and (2) three months. During the acute stage, both of them developed multifocal seizures that were controlled with Phenobarbital and Phenytoin. Neuroimaging studies showed multiple subdural hematomas, areas of parenchymal contusions, subarachnoid and/or intraventricular hemorrhages and, subsequently, (1) encephaloclastic lesions or (2) diffuse brain atrophy. In patient No 1, Phenobarbital was continued till the age of four months and then, although the infant was seizure free, replaced with Clobazam as the EEG records were still capturing multifocal epileptic discharges. Patient No 2 continued on Phenobarbital for one month and had no recurrence of seizures upon tapering. Both infants developed IS at the age of (1) five and (2) six months. EEG records were characterized by typical hypsarrhythmia. In both cases, spasms were rapidly controlled with Vigabatrin. Both children were seizure free at (1) fourteen months and (2) five-years follow-up but were significantly developmentally delayed.Conclusions: Our two patients highlight the risk of young children suffering from NAHI to develop infantile spasms. These children seem to respond well to Vigabatrin as first line therapy. A better knowledge of the incidence of IS following NAHI could help us better understand the link between NAHI and IS as well as develop neuroprotective treatments to prevent the occurrence of this severe form of epilepsy.