1147. Moyamoya-Like Vasculopathy in a Woman with Recurrent HELLP Syndrome in Pregnancy

Authors: Adam Benjamin Lipson, MD; Christiana Stark, BS; Alex Michael, MD; Casey Muehle, MD (Springfield, IL)

Introduction :Characterized by bilateral stenosis of the terminal portion of the internal carotid and anterior circulation arteries, Moyamoya-like vasculopathy develops in association with, and as a consequence of, various systemic conditions. The development of peripartum Moyamoya syndrome is an intriguing clinical entity, whose pathophysiology is incompletely understood. Methods :We present the case of a 40-year old female with a recurrent history of syndromic hemolysis, elevated liver enzymes, and low platelet count (HELLP) in three prior pregnancies, who was found to have a Moyamoya-like vasculopathy on vessel imaging following an ischemic stroke. In addition, we review the current literature on Moyamoya disease and HELLP syndrome, and discuss the natural history of these conditions and their putative association in pregnancy. Results :An otherwise healthy 40-year old female with no known personal or family history of neurologic or cerebrovascular disease, presented to our institution with an acute episode of diplopia, dizziness, and right-sided weakness and dysesthesia. She noted that in all three of her prior pregnancies, she had been diagnosed with HELLP syndrome. Radiographic work-up including cerebral angiography revealed symmetric mild narrowing of the supraclinoid segments of the internal carotid arteries, severe bilateral stenosis at the takeoff of the middle cerebral arteries, and a peripheral flow pattern of the middle cerebral artery branches. Following management of ischemic stroke, the patient was placed on antiplatelet agents, and later underwent surgical revascularization. On initial follow-up, she was found to have regained significant strength in the right hemi-body. Conclusion : While the pathogenesis of Moyamoya is still largely unclear, its association with inflammatory and hypercoaguable states has been recognized. Here, we describe the presumed association of multiple episodes of HELLP syndrome and Moyamoya pathology, and suggest that a deeper understanding of these conditions may allow for identification of an additional population at risk.