Brief CommunicationSilent brain infarcts in two patients with zeta chain-associated protein 70Â kDa (ZAP70) deficiency

•Herein, we presented two patients with ZAP70 deficiency.•Both of them had silent brain infarcts as a common feature which has not been report medical literature.•The first patient presented with recurrent infections and tuberculosis as in classic SCID•The second patient presented with congenital nephrotic syndrome due to cytomegalovirus infection and had novel mutation.

Zeta-chain associated protein 70 kDa deficiency (ZAP70) is a form of severe combined immunodeficiency (SCID). It is caused by defects in the signaling pathways associated with T-lymphocyte activation. ZAP70 deficiency is characterized by a marked reduction in peripheral CD8 + T-cells. In this report, we described two patients with ZAP70 deficiency who presented with recurrent infections, lung tuberculosis (TBC), congenital nephrotic syndrome (CNS), and silent brain infarcts (SBIs) as a common feature. The first patient initially presented with recurrent infections and TBC as in a classic SCID patient. At the age of 4, he was interned with febrile seizure. Cranial magnetic resonance imaging (MRI) showed SBIs. The second patient, an 8-month-old boy, presented with congenital nephrotic syndrome caused by cytomegalovirus (CMV) and he had also SBIs.