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

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

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.

0006-4971
4587-4596
DeBaun, Michael R.
76559153-80c6-4642-bdf8-672a75570dfe
Armstrong, F. Daniel
a5b111f8-efe1-438e-a87a-5ce90703e625
McKinstry, Robert C.
6a775b1a-5925-45a2-8b14-fb108d313208
Ware, Russell E.
e805818e-bbf0-47d0-ac17-758c7c19dc60
Vichinsky, Elliot
96f0dd63-b632-456f-833d-47e5ffc49360
Kirkham, Fenella J.
1dfbc0d5-aebe-4439-9fb2-dac6503bcd58
DeBaun, Michael R.
76559153-80c6-4642-bdf8-672a75570dfe
Armstrong, F. Daniel
a5b111f8-efe1-438e-a87a-5ce90703e625
McKinstry, Robert C.
6a775b1a-5925-45a2-8b14-fb108d313208
Ware, Russell E.
e805818e-bbf0-47d0-ac17-758c7c19dc60
Vichinsky, Elliot
96f0dd63-b632-456f-833d-47e5ffc49360
Kirkham, Fenella J.
1dfbc0d5-aebe-4439-9fb2-dac6503bcd58

DeBaun, Michael R., Armstrong, F. Daniel, McKinstry, Robert C., Ware, Russell E., Vichinsky, Elliot and Kirkham, Fenella J. (2012) Silent cerebral infarcts: A review on a prevalent and progressive cause of neurologic injury in sickle cell anemia. Blood, 119 (20), 4587-4596. (doi:10.1182/blood-2011-02-272682).

Record type: Review

Abstract

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.

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Published date: 17 May 2012

Identifiers

Local EPrints ID: 429117
URI: http://eprints.soton.ac.uk/id/eprint/429117
ISSN: 0006-4971
PURE UUID: 73602655-84ea-4363-b02f-5b0e62cc4a53
ORCID for Fenella J. Kirkham: ORCID iD orcid.org/0000-0002-2443-7958

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Date deposited: 21 Mar 2019 17:30
Last modified: 20 Jul 2022 01:39

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Contributors

Author: Michael R. DeBaun
Author: F. Daniel Armstrong
Author: Robert C. McKinstry
Author: Russell E. Ware
Author: Elliot Vichinsky

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