Case Report: Infantile Ischemic Stroke and Antiphospholipid Antibodies, Description of Four Cases

Abstract

Antiphospholipid syndrome (APS) is a rare condition in childhood, but even more in the neonatal age. Most neonatal cases are considered a passively acquired autoimmune disease, due to a transplacental passage of maternal antiphospholipid antibodies (aPL) from mothers with primary or secondary APS or, more often, from asymptomatic aPL carriers. Exceedingly unusual is the neonatal de novo production of aPL. We present four infants with presumed perinatal stroke in presence of increased and persistent aPL levels, even after 6 months of life, opening the window on a gray zone related to the origin of these antibodies (maternal or neonatal) and on their role in the pathogenesis of stroke.

Neonatal IVC thrombosis in a baby born to an aPL positive mother – a case report

“…5 However, in rare cases, such as the present one, this can cause thrombosis, with potentially devastating consequences. In most cases of neonatal APS, the antibody positivity is transient, with aPLs becoming negative in the first few years of life, 6 and in cases where the patient survives, there does not seem to be a recurrence of thromboses, or development of autoimmune disease or manifestations of APS. 7 However, it would be of interest to follow up females with neonatal APS through their own future pregnancies, to determine whether they are at higher risk of APS-like complications.…”

Antiphospholipid antibodies and cerebrovascular thrombosis in the pediatric population: Few answers to many questions

“…Most of the cases noted a positive confirmation test. In the only case where aCL disappeared upon confirmation, 58 the infant developed long term neurological complications. However, this infant was also heterozygous for factor V Leiden mutation, which questions the role of the transient IgG aCL positivity.…”

“…Few case reports illustrated low titers of aCL, while only one case reported high aCL titer which eventually disappeared with time. 58 The prevalence of aCL among the studies is variable. When both IgG and IgM were assessed, IgG were more prevalent, even after confirmation which is similar to adult ischemic stroke.…”

“…10,42,45 In other cases however, the use of antiplatelet or anticoagulant was not related to future aPL titer or neurological outcomes. 58 Apart from aPL, the data on anticoagulation/antiplatelet and cerebral ischemic stroke in neonates and children is also still limited. In Boffa and Lachassinne review, 88 low-dose aspirin was given in one infant which led to resolution of thrombus, whereas the combination of heparin and aspirin did not prevent death of another infant.…”

“…10,42,45 In other cases however, the use of antiplatelet or anticoagulant was not related to future aPL titer or neurological outcomes. 58…”