Silent brain infarcts in adult Kuwaiti sickle cell disease patients

Marouf, Rajaa; Gupta, Renu; Haider, M.Z.; Adekile, Adekunle

doi:10.1002/ajh.10376

Cited by 43 publications

(33 citation statements)

References 36 publications

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“…In this report, the authors did not study the association between brain lesions and large artery abnormalities, because neither MRA nor TCD was performed. 23 We showed that abnormalities in brain MRI were more frequent in patients with stenoses and intracranial arterial tortuosity, suggesting that large vessel disease is implicated in the pathophysiology of the silent lesions.…”

Section: Discussionmentioning

confidence: 91%

Brain Magnetic Resonance Imaging Abnormalities in Adult Patients With Sickle Cell Disease

Silva

Vicari

Figueiredo

et al. 2009

Stroke

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Background and Purpose-Brain imaging abnormalities were reported in up to 44% of children with sickle cell disease (SCD). The prevalence of neuroimaging abnormalities in adult patients with SCD and their relationship to transcranial Doppler is still unclear. Our objectives were to study the frequency of MRI and MR angiography abnormalities in adults with SCD and to define what transcranial Doppler velocities are associated with intracranial stenoses detected by MR angiography. Methods-We examined all adult patients (Ͼ16 years) with SCD followed in the hematology outpatient clinic at our university hospital with MRI, MR angiography, and transcranial Doppler. Results-We evaluated 50 patients. The overall prevalence of MRI abnormalities was 60%. Abnormal MRI findings were more frequent when vessel tortuosity or stenoses were present on MR angiography (PϽ0.01). Patients with intracranial stenoses had significantly higher time-averaged maximum mean velocities (Pϭ0.01). A time-averaged maximum mean velocity of 123.5 cm/s allowed the diagnosis of middle cerebral artery or internal carotid artery intracranial stenosis with sensitivity of 100% and specificity of 73% with an area under the receiver operator characteristic curve of 0.91 (CI, 0.79 to 1.00). Conclusions-The frequency of brain imaging abnormalities detected by MRI/MR angiography in adults with SCD was higher than that described for children. Transcranial Doppler velocities in adult patients with intracranial stenoses were lower than those described for the pediatric population with SCD.

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Section: Discussionmentioning

confidence: 91%

Brain Magnetic Resonance Imaging Abnormalities in Adult Patients With Sickle Cell Disease

Silva

Vicari

Figueiredo

et al. 2009

Stroke

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“…15 SCI is most common in patients with hemoglobin SS but may also be identified in individuals with heterozygous genotypes including sickle ␤-thalassemia and hemoglobin SC (HbSC) disease. Approximately 3% to 38% of patients with sickle ␤-thalassemia, 17,18,20,21 and 5% to 31% of those with HbSC disease, 15,22 will have SCI. However, limited sample size allows no significant inferences about the rate of progression or potential benefit of therapy.…”

Section: Clinical Significance Of Scismentioning

confidence: 99%

Silent cerebral infarcts: a review on a prevalent and progressive cause of neurologic injury in sickle cell anemia

DeBaun

Armstrong

McKinstry

et al. 2012

Blood

289

292

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Silent cerebral infarct (SCI) is the most common form of neurologic disease in children with sickle cell anemia (SCA). SCI is defined as abnormal magnetic resonance imaging (MRI) of the brain in the setting of a normal neurologic examination without a history or physical findings associated with an overt stroke. SCI occurs in 27% of this population before their sixth, and 37% by their 14th birthdays. In adults with SCA, the clinical history of SCI is poorly defined, although recent evidence suggests that they too may have ongoing risk of progressive injury. Risk factors for SCI include male sex, lower baseline hemoglobin concentration, higher baseline systolic blood pressure, and previous seizures. Specific morbidity associated with SCI includes a decrement in general intellectual abilities, poor academic achievement, progression to overt stroke, and progressive SCI. In addition, children with previous stroke continue to have both overt strokes and new SCI despite receiving regular blood transfusion therapy for secondary stroke prevention. Studies that only include overt stroke as a measure of CNS injury significantly underestimate the total cerebral injury burden in this population. In this review, we describe the epidemiology, natural history, morbidity, medical management, and potential therapeutic options for SCI in SCA. IntroductionOne of the most devastating medical complications of sickle cell anemia (SCA) is cerebral injury, which limits the full potential of the developing child and adult. The most common neurologic injury in SCA is silent cerebral infarct (SCI). Although there are few studies, SCI occurs in approximately one-quarter of children with SCA before their sixth birthday 1 and approximately one-third before their 14th birthday. 2 The first evidence that SCI had clinical significance was published in a cross-sectional computed tomography (CT) scan study of 25 patients. Five had cerebral infarcts but only 4 had a focal neurologic deficit (overt stroke). 3 The investigators referred to the fifth patient as having a covert stroke, 3 a term also used by others. 4 The term SCI or silent stroke has gained widespread use to describe this condition because the Cooperative Study of Sickle Cell Disease (CSSCD) used standard clinical magnetic resonance imaging (MRI) to define the radiologic parameters. 5 Subsequently, only a few studies of SCI have been completed. Until recently, the clinical importance of identifying SCI did not seem to justify the expense of MRI requiring sedation or anesthesia in preschool children, particularly in the absence of an effective intervention. Despite being the most common type of cerebral injury among children and adults with SCA, relatively little is known about the cause and optimal therapy of SCI. We will review the current literature on definition, epidemiology, natural history, clinical significance, medical management, and potential therapeutic options for the treatment of SCI in SCA. Definition of SCI in children with SCAThe largest longitudinal cohort study to a...

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“…Few studies in patients with HbS-b thalassemia have been reported and the results are inconclusive [21][22][23][24]. The effectiveness of HU for the prevention of silent cerebral infarction has not been reported.…”

Section: Introductionmentioning

confidence: 99%

Cerebrovascular events in sickle cell‐beta thalassemia treated with hydroxyurea: A single center prospective survey in adult Italians

et al. 2013

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Stroke is a common cause of morbidity and mortality in sickle cell disease (SCD) and silent cerebral infarction is the most common form of neurologic injury. The frequency and risk factors for new silent cerebral infarction are incompletely understood. Moreover, no recommended treatment has been established. Although hydroxyurea (HU) is recommended for SCD, concerns remain regarding its role in the prevention of cerebrovascular events, including silent cerebral infarction. A single center population of 104 Italian patients with HbS-ß thalassemia treated with HU has been followed for a mean of 11 years. Clinical evaluation and brain imaging by Magnetic Resonance Imaging were done before and during HU treatment. During follow-up, the number of sickle cell crises (86%, 7.8 6 6.9 vs. 1.2 6 0.5 per year, P < 0.0001), hospitalizations (2.5 6 2.9 vs. 0.3 6 1.5 per year, P < 0.0001), and days in the hospital (22.4 6 21.9 vs. 0.361.5 per year, P < 0.0001) decreased significantly and HbF increased from a mean of 8-20.8%. Cerebral infarcts occurred in 37.5% of patients. Among these, 6.7% had overt strokes, while 30% had new or progressive silent cerebral infarction. Stroke and silent cerebral infarction were not related to clinical hematologic or HbF response to HU. These findings suggest that in adults, HU treatment does not prevent new cerebrovascular events or the progression of existent silent cerebral infarcts in HbS-b thalassemia. A major benefit of HU is the increase in HbF; the association of high HbF and reduced cerebrovascular disease has been weak. New treatment strategies should be developed for the prevention of sickle cerebrovascular disease. Am. J. Hematol. 88:E261-E264,

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Silent brain infarcts in adult Kuwaiti sickle cell disease patients

Cited by 43 publications

References 36 publications

Brain Magnetic Resonance Imaging Abnormalities in Adult Patients With Sickle Cell Disease

Brain Magnetic Resonance Imaging Abnormalities in Adult Patients With Sickle Cell Disease

Silent cerebral infarcts: a review on a prevalent and progressive cause of neurologic injury in sickle cell anemia

Cerebrovascular events in sickle cell‐beta thalassemia treated with hydroxyurea: A single center prospective survey in adult Italians

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