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Acute silent cerebral ischemic events in children with sickle cell anemia

Acute silent cerebral ischemic events in children with sickle cell anemia
Acute silent cerebral ischemic events in children with sickle cell anemia

Background: Irregular, sporadic episodes of ischemic brain injury are known to occur in sickle cell anemia (SCA), resulting in overt stroke and silent cerebral infarction. Ongoing ischemia in other organs is common in SCA but has never been documented in the brain. Objective: To test the hypothesis that acute silent cerebral ischemic events (ASCIEs) are frequent and potentially transient. Design: Cross-sectional and cohort study of children with SCA screened by magnetic resonance imaging (MRI) of the brain for a randomized clinical trial. Setting: Clinical trial setting in tertiary care centers. Patients: Asymptomatic children with SCA without known stroke, neurologic injury, or epilepsy not receiving treatment with transfusions or hydroxyurea. Main Outcome Measure: Incidence of ASCIEs calculated using single diffusion-weighted MRI scans (acute ischemic events that occurred within 10 days of the MRI). Results: Acute silent cerebral ischemic events were detected on 1.3% of MRIs (10 of 771) in 652 children (mean age, 10.0 years), with an incidence of 47.3 events per 100 patient-years (95% CI, 22.7-87.2). Two of 10 children with ASCIEs had follow-up MRIs of the brain; only 1 had silent cerebral infarction in the same location as the previously detected ASCIE. Conclusions: Children with SCA experience ongoing (chronic, intermittent) cerebral ischemia, sometimes reversible, far more frequently than previously recognized. The brain in SCA is at constant threat of ischemia.

0003-9942
58-65
Quinn, Charles T.
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Robert, C. McKinstry
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Dowling, Michael M.
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Ball, William S.
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Kraut, Michael A.
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Casella, James F.
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Dlamini, Nomazulu
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Ichord, Rebecca N.
d3cb64eb-0dab-4e05-bbb6-f0ff9bbdcea8
Jordan, Lori C.
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Kirkham, Fenella J.
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Noetzel, Michael J.
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Steve Roach, E.
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Strouse, John J.
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Kwiatkowski, Janet L.
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Hirtz, Deborah
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DeBaun, Michael R.
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Quinn, Charles T.
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Robert, C. McKinstry
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Dowling, Michael M.
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Ball, William S.
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Kraut, Michael A.
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Casella, James F.
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Dlamini, Nomazulu
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Ichord, Rebecca N.
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Jordan, Lori C.
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Kirkham, Fenella J.
1dfbc0d5-aebe-4439-9fb2-dac6503bcd58
Noetzel, Michael J.
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Steve Roach, E.
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Strouse, John J.
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Kwiatkowski, Janet L.
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Hirtz, Deborah
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DeBaun, Michael R.
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Quinn, Charles T., Robert, C. McKinstry, Dowling, Michael M., Ball, William S., Kraut, Michael A., Casella, James F., Dlamini, Nomazulu, Ichord, Rebecca N., Jordan, Lori C., Kirkham, Fenella J., Noetzel, Michael J., Steve Roach, E., Strouse, John J., Kwiatkowski, Janet L., Hirtz, Deborah and DeBaun, Michael R. (2013) Acute silent cerebral ischemic events in children with sickle cell anemia. Archives of Neurology, 70 (1), 58-65. (doi:10.1001/jamaneurol.2013.576).

Record type: Article

Abstract

Background: Irregular, sporadic episodes of ischemic brain injury are known to occur in sickle cell anemia (SCA), resulting in overt stroke and silent cerebral infarction. Ongoing ischemia in other organs is common in SCA but has never been documented in the brain. Objective: To test the hypothesis that acute silent cerebral ischemic events (ASCIEs) are frequent and potentially transient. Design: Cross-sectional and cohort study of children with SCA screened by magnetic resonance imaging (MRI) of the brain for a randomized clinical trial. Setting: Clinical trial setting in tertiary care centers. Patients: Asymptomatic children with SCA without known stroke, neurologic injury, or epilepsy not receiving treatment with transfusions or hydroxyurea. Main Outcome Measure: Incidence of ASCIEs calculated using single diffusion-weighted MRI scans (acute ischemic events that occurred within 10 days of the MRI). Results: Acute silent cerebral ischemic events were detected on 1.3% of MRIs (10 of 771) in 652 children (mean age, 10.0 years), with an incidence of 47.3 events per 100 patient-years (95% CI, 22.7-87.2). Two of 10 children with ASCIEs had follow-up MRIs of the brain; only 1 had silent cerebral infarction in the same location as the previously detected ASCIE. Conclusions: Children with SCA experience ongoing (chronic, intermittent) cerebral ischemia, sometimes reversible, far more frequently than previously recognized. The brain in SCA is at constant threat of ischemia.

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Published date: January 2013

Identifiers

Local EPrints ID: 424272
URI: http://eprints.soton.ac.uk/id/eprint/424272
ISSN: 0003-9942
PURE UUID: d87f6940-5e42-4a9b-be10-14c2b0490bbf
ORCID for Fenella J. Kirkham: ORCID iD orcid.org/0000-0002-2443-7958

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Date deposited: 05 Oct 2018 11:35
Last modified: 16 Mar 2024 03:22

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Contributors

Author: Charles T. Quinn
Author: C. McKinstry Robert
Author: Michael M. Dowling
Author: William S. Ball
Author: Michael A. Kraut
Author: James F. Casella
Author: Nomazulu Dlamini
Author: Rebecca N. Ichord
Author: Lori C. Jordan
Author: Michael J. Noetzel
Author: E. Steve Roach
Author: John J. Strouse
Author: Janet L. Kwiatkowski
Author: Deborah Hirtz
Author: Michael R. DeBaun

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