Simultaneous cerebral venous and arterial involvement in the postpartum period: Rare presentation of Antiphospholipid syndrome

Abstract

Background: Reversible cerebral vasoconstriction syndrome (RCVS) and cerebral venous sinus thrombosis (CVST) are two dreadful causes of postpartum neurological complications and occurrence of concomitant RCVS and CVST is a rarity. Here, we report a rare case of postpartum stroke caused by concomitant RCVS and CVT tiggered by catastrophic antiphospholipid syndrome.

Case description: Our patient presented with postpartum seizure followed by prolonged unconsciousness with no prior history of headache. Her initial neuroimaging revealed extensive haemorrhagic infarcts involving brain, brainstem and cerebellum. Magnetic resonance angiography and venography revealed constriction of all large vessels of Circle of Willis with filling defects in superior sagittal sinus, junction of left transverse and sigmoid sinus and complete non-visualisation of left transverse sinus. Workup for vasculitis was negative but detailed evaluation of prothrombotic state was significant for presence of very high titres of lupus anti-coagulant antibody and anti beta-2 glycoprotein antibody which continued to remain high at 3 months follow up. Patient was treated with intravenous methylprednisolone, heparin, nimodipine infusion and anticonvulsants. After 1 month, patient improved remarkably with radiological resolution of parenchymal lesions, arterial narrowing and filling defects of cerebral venous sinuses.

Conclusion: Anti-phospholipid syndrome can trigger systemic prothrombotic state which involves both arterial and venous system; risk is even higher in peripartum period due to fluctuating oestrogen levels. Even in cases with extensive central nervous system involvement like our patient, considerable recovery is possible with aggressive medical management.