CHILDHOOD STROKE ASSOCIATED WITH FAMILIAL PROTEIN-S DEFICIENCY

Cerebral infarction is a rare pathology among children and its etiolog y can be identified in almost two-thirds of cases. The remaining one-t hird are considered idiopathic. Recently, inherited disorders of blood coagulation predisposing to thrombosis have been taken into account a s a possible cause of childhood stroke. We describe here a case of a 6 -year old child presenting with ischemic stroke and protein S (PS) def ect. The family study suggested inheritance of the defect. The immunol ogical characterization of PS in the affected family members was consi stent with a defect mainly in the free form of PS. In the case here re ported no associated predisposing condition to stroke could be identif ied but familial PS defect was found. No therapy was administered. Nev ertheless symptoms disappeared spontaneously and there were no recurre nces at the 1 year follow-up. Diagnostic imaging techniques demonstrat ed that a reduction in the cerebral ischemic area had occurred 2 month s after the stroke.