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Cerebrovascular Disease in Sickle Cell Disease Is About
Cerebrovascular Disease in Sickle Cell Disease Is About
He had lived independently until moving in with his trophoresis showed HbC decreased to 27% and HbS to 32.9%.
brother because of progressive cognitive problems. He was discharged to a skilled nursing facility.
On presentation, he appeared mildly confused, with mild Unfortunately, 3 months later, the patient passed away
bilateral proximal weakness, and scored a 3 on the National because of urosepsis. His serum electrophoresis showed an
Institutes of Health Stroke Scale. HbS of 37%.
Laboratories Discussion
Hemoglobin was 12.9, and peripheral blood smear showed SCD is an inherited hemoglobinopathy where there is a quali-
occasional sickle cells. Serum electrophoresis demon- tative disorder in either the α or the β globin chain. HbS is
strated 52% hemoglobin S (HbS) and 44.7% hemoglobin the most important inherited hemoglobinopathy in the United
C (HbC). States followed by HbC. β-Globin gene can be homozy-
gous (HbSS), commonly referred to as sickle cell anemia ,
Imaging or heterozygous sickle cell trait, HbSC and HbS-thalassemia.
Magnetic resonance imaging (MRI) brain revealed multifo- Stroke is a major complication of SCD and most frequently
cal punctate areas of restricted diffusion of acute infarction seen in HbSS in up to 25% in these patients followed by the
affecting the deep white matter and the cortex. These were thalassemias and HbC.1,2 Understanding the mechanism of
superimposed on diffuse volume loss and multifocal areas of stroke is crucial to prevent their recurrence. Several risk fac-
old lacunar infarction (Figure 1). Computer topographic angi- tors increase the likelihood of strokes in patients with SCD,
ography showed severe stenosis in the right superior division including cerebral vasculopathy, elevated TCD velocities,
of the middle cerebral artery and moderate stenosis in the dis- anemia, leukocytosis, evidence of silent infarcts, and tradi-
tal aspect of bilateral anterior, middle, and posterior cerebral tional cardiovascular risk factors.2
arteries (Figure 2). His transesophageal echocardiogram was Our patient’s presentation of a progressive cognitive
normal, and transcranial Doppler (TCD) showed no evidence decline is consistent with the severe leukoaraiosis seen on
of vasospasm or elevated velocities. MRI. The lateralizing findings on his neurological examination
corresponded to his acute multifocal strokes. The presentation
Clinical Course of multifocal recurrent strokes within 2 weeks of maximal
He appeared stable with no obvious changes on clini- medical management did not fit the typical pattern observed in
cal examination. The hematology team was consulted, but intracranial atherosclerosis. Although his leukoaraiosis could
they did not think he was a candidate for exchange transfu- be partially attributed to his one known vascular risk factor
sion given his normal HgB, normal MCV, few peripheral (hypertension), his symptom progression and history of HbSC
sickle cells, and most importantly, because of compound suggested the alternative pathogenesis of symptomatic SCD.
heterozygosity with HbC. Given the uncertainty of stroke Underlying pathogenesis of cerebrovascular disease in
pathogenesis and lack of treatment consensus, repeat SCD involves both large vessel as well as penetrating (small)
Received February 7, 2018; final revision received March 21, 2018; accepted March 26, 2018.
From the Departments of Neurology (S.J., A.A., A.M.) and Hematology and Oncology (J.K.), West Virginia University, Morgantown.
Correspondence to Sneha Jacob, MD, Department of Neurology, West Virginia University, 1 Medical Center Dr, Suite 7500, PO Box 9180, Morgantown,
WV 26506. E-mail snjacob@hsc.wvu.edu
(Stroke. 2018;49:e224-e227. DOI: 10.1161/STROKEAHA.118.021057.)
© 2018 American Heart Association, Inc.
Stroke is available at http://stroke.ahajournals.org DOI: 10.1161/STROKEAHA.118.021057
e224
Jacob et al Stroke and Sickle Cell Disease e225
Figure 3. Axial diffusion-weighted imaging (DWI; left) at initial presentation demonstrating scattered restricted diffusion consistent with
acute ischemia. Axial DWI (right) obtained 1 wk later illustrates new areas of restricted diffusion in the left lentiform nuclei.
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9. Adams RJ, Cox M, Ozark SD, Kanter J, Schulte PJ, Xian Y, et al. Coexistent KEY WORDS: anemia, sickle cell ◼ cognitive dysfunction ◼ hemoglobinopathies
sickle cell disease has no impact on the safety or outcome of lytic ◼ magnetic resonance imaging ◼ stroke
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More Than Meets the Eye: Cerebrovascular Disease in Sickle Cell Disease Is About More
Than Sickling
Sneha Jacob, Amelia Adcock, Ann Murray and Joanna Kolodney
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