Posterior Reversible Encephalopathy Syndrome with Stroke in Puerperal Woman with High Titer of Anti-Phospholipid IgM Antibody

Abstract

Posterior reversible encephalopathy syndrome with stroke is very rare in puerperal women. A 36-year-old nulliparous woman with both rheumatoid arthritis and recurrent pregnancy loss, probably due to a high titer of anti-phospholipid IgM antibody, was referred at 10 weeks of gestation. Low-dose aspirin at 100 mg/day and heparin calcium subcutaneous injection at 10,000 units/day were started before pregnancy and stopped at 35+6 and 40+2 weeks, respectively. She transabdominally delivered a male infant weighing 3,344 g at 40+5 weeks. A tonic-clonic seizure abruptly occurred without either hypertension or proteinuria 5 days after delivery. Intracerebral hemorrhage involving an area of 2 cm in diameter in the right frontal lobe and subarachnoid hemorrhage with PRES were confirmed. Seizure recurred 2 days after the initial episode. She showed severe headache and mild disturbance of consciousness but no neurological findings. We suggested that a high titer of anti-phospholipid IgM antibody might be associated with stroke.

Anti-phospholipid syndrome leading to digital ischaemia and rare organ complications in systemic sclerosis and related disorders

“…Our final case of SSc with secondary APS resulted in the rare PRES (also known as reversible posterior leukoencephalopathy syndrome (RPLS)). There have only been a small number of reported cases of PRES in association with primary and secondary APS, often in association with high titre ACL IgM, as was found in our case (40)(41)(42)(43). To our knowledge this is the first reported case of PRES occurring in the context of SSc and secondary APS.…”