More Than Meets the Eye

Abstract

A 56-year-old male with a known history of sickle cell disease (SCD) with HbSC and progressive deafness presented to the hospital with increased left-sided weakness accompanied by worsening confusion for the past 5 days. He experienced a multiorgan crisis requiring plasmapheresis 10 years prior. He had lived independently until moving in with his brother because of progressive cognitive problems. On presentation, he appeared mildly confused, with mild bilateral proximal weakness, and scored a 3 on the National Institutes of Health Stroke Scale. Hemoglobin was 12.9, and peripheral blood smear showed occasional sickle cells. Serum electrophoresis demonstrated 52% hemoglobin S (HbS) and 44.7% hemoglobin C (HbC). Magnetic resonance imaging (MRI) brain revealed multifocal punctate areas of restricted diffusion of acute infarction affecting the deep white matter and the cortex. These were superimposed on diffuse volume loss and multifocal areas of old lacunar infarction (Figure 1). Computer topographic angiography showed severe stenosis in the right superior division of the middle cerebral artery and moderate stenosis in the distal aspect of bilateral anterior, middle, and posterior cerebral arteries (Figure 2). His transesophageal echocardiogram was normal, and transcranial Doppler (TCD) showed no evidence of vasospasm or elevated velocities. Figure 1. Axial diffusion-weighted imaging showing multiple areas of restricted diffusion consistent with multifocal acute ischemia. Figure 2. Computed tomographic angiogram showing multifocal stenosis in different vascular territories most pronounced in the left internal carotid artery terminus. He appeared stable with no obvious changes on clinical examination. The hematology team was consulted, but they did not think he was a candidate for exchange transfusion given his normal HgB, normal MCV, few peripheral sickle cells, and most importantly, because of compound heterozygosity with HbC. Given the uncertainty of stroke pathogenesis and lack of treatment consensus, repeat brain imaging was performed demonstrating new punctate areas of restricted diffusion (Figure 3). Updated TCDs …