Antiphospholipid syndrome (APS) is an autoimmune thrombophilia that rarely presents with progressive intracranial large-artery stenosis or occlusion, making early recognition difficult when vascular lesions mimic atherosclerotic disease. A 74-year-old female receiving edoxaban for atrial fibrillation presented with progressive left hemiparesis due to severe right internal carotid artery (ICA) stenosis. Angioplasty improved cerebral blood flow, but recurrent ischemic stroke occurred 3 months later due to restenosis, which was treated with repeat angioplasty and Wingspan stenting. Over the next year, she developed progressive right middle cerebral artery (MCA) stenosis that eventually led to occlusion, requiring superficial temporal artery-MCA bypass. After perioperative interruption of edoxaban, she developed in-stent thrombosis and bypass graft thrombosis. Laboratory testing revealed triple-positive antiphospholipid antibodies, confirming primary APS. Systemic heparinization followed by apixaban resulted in complete resolution of in-stent thrombosis, improved bypass flow, and partial MCA recanalization. This case illustrates that APS can present as a dramatically changing and potentially reversible intracranial arteriopathy, emphasizing the need to suspect APS in patients with recurrent or unexplained intracranial thrombosis.
Nakajima et al. (Thu,) studied this question.