Intracranial and Extracranial Vascular Stenosis as Risk Factors for Stroke in Sickle Cell Disease.

Abstract:

BACKGROUND:Prevalence and contribution of intracranial and extracranial arterial stenosis to stroke risk were assessed prospectively in children and young adults with sickle cell disease. METHODS:In this cross-sectional study, children and young adults (mean = 19.4 years) with sickle cell disease underwent neurological examination, brain MRI, and magnetic resonance angiography of the head and neck. Two neuroradiologists independently recorded infarcts and arterial stenosis. Clinical features and stroke outcomes were compared between participants with and without stenosis and between children and young adults. Logistic regression analysis assessed the association of variables of interest with overt stroke and silent cerebral infarct. RESULTS:Of 167 participants (79 children and 88 young adults), 20 (12.0%) had intracranial stenosis, all in the anterior circulation, and nine had concurrent extracranial stenosis. No participants had isolated extracranial stenosis. Participants with intracranial stenosis were more likely than those without stenosis to have an overt stroke (70% vs 5%, P < 0.001) or silent cerebral infarct (95% vs 35%, P < 0.001). Logistic regression analysis indicated that intracranial stenosis was strongly associated with overt stroke when compared with participants with silent cerebral infarct alone and strongly associated with silent cerebral infarct when compared with participants with normal brain MRI; male sex and age were also significant predictors of silent cerebral infarct. CONCLUSIONS:Intracranial stenosis was strongly associated with both overt stroke and silent cerebral infarct; prevalence of intracranial stenosis was similar to prior estimates in sickle cell disease. Extracranial stenosis without concurrent intracranial stenosis did not occur and thus could not be evaluated as an independent risk factor for stroke.

journal_name

Pediatr Neurol

journal_title

Pediatric neurology

authors

Schlotman AA,Donahue MJ,Kassim AA,Lee CA,Waddle SL,Pruthi S,Davis LT,Rodeghier M,DeBaun MR,Jordan LC

doi

10.1016/j.pediatrneurol.2020.10.006

subject

Has Abstract

pub_date

2021-01-01 00:00:00

pages

29-34

eissn

0887-8994

issn

1873-5150

pii

S0887-8994(20)30340-4

journal_volume

114

pub_type

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