Acute silent cerebral ischemic events in children with sickle cell anemia

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

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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.

Original languageEnglish (US)
Pages (from-to)58-65
Number of pages8
JournalArchives of Neurology
Volume70
Issue number1
DOIs
StatePublished - Jan 2013

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Sickle Cell Anemia
Cerebral Infarction
Brain
Ischemia
Stroke
Magnetic Resonance Imaging
Nervous System Trauma
Diffusion Magnetic Resonance Imaging
Hydroxyurea
Incidence
Brain Ischemia
Tertiary Care Centers
Brain Injuries
Sickles
Cells
Anemia
Epilepsy
Cohort Studies
Randomized Controlled Trials
Cross-Sectional Studies

ASJC Scopus subject areas

  • Clinical Neurology
  • Arts and Humanities (miscellaneous)

Cite this

Quinn, C. T., Robert, C. M., Dowling, M. M., Ball, W. S., Kraut, M. A., Casella, J. F., ... DeBaun, M. R. (2013). Acute silent cerebral ischemic events in children with sickle cell anemia. Archives of Neurology, 70(1), 58-65. https://doi.org/10.1001/jamaneurol.2013.576

Acute silent cerebral ischemic events in children with sickle cell anemia. / 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; DeBaun, Michael R.

In: Archives of Neurology, Vol. 70, No. 1, 01.2013, p. 58-65.

Research output: Contribution to journalArticle

Quinn, CT, Robert, CM, Dowling, MM, Ball, WS, Kraut, MA, Casella, JF, Dlamini, N, Ichord, RN, Jordan, LC, Kirkham, FJ, Noetzel, MJ, Steve Roach, E, Strouse, JJ, Kwiatkowski, JL, Hirtz, D & DeBaun, MR 2013, 'Acute silent cerebral ischemic events in children with sickle cell anemia', Archives of Neurology, vol. 70, no. 1, pp. 58-65. https://doi.org/10.1001/jamaneurol.2013.576
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 ; DeBaun, Michael R. / Acute silent cerebral ischemic events in children with sickle cell anemia. In: Archives of Neurology. 2013 ; Vol. 70, No. 1. pp. 58-65.
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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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AU - Ball, William S.

AU - Kraut, Michael A.

AU - Casella, James F.

AU - Dlamini, Nomazulu

AU - Ichord, Rebecca N.

AU - Jordan, Lori C.

AU - Kirkham, Fenella J.

AU - Noetzel, Michael J.

AU - Steve Roach, E.

AU - Strouse, John J.

AU - Kwiatkowski, Janet L.

AU - Hirtz, Deborah

AU - DeBaun, Michael R.

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N2 - 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.

AB - 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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