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Blood, 1 July 2006, Vol. 108, No. 1, pp. 379-381.
Prepublished online as a Blood First Edition Paper on March 14, 2006; DOI 10.1182/blood-2005-10-4029.


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Submitted October 11, 2005
Accepted February 17, 2006

Inverse correlation between cerebral blood flow measured by continuous arterial spin-labeling (CASL) MRI and neurocognitive function in children with sickle cell anemia (SCA)

John J Strouse*, Christiane S Cox, Elias R Melhem, Hanzhang Lu, Michael A Kraut, Alexander Razumovsky, Kaleb Yohay, Peter C van Zijl, and James F Casella

Department of Pediatric Hematology, Johns Hopkins University School of Medicine, Baltimore, MD, USA
Department of Developmental and Cognitive Neurology, Kennedy-Krieger Institute, Baltimore, MD, USA
Department of Radiology, Johns Hopkins University School of Medicine, Baltimore, MD, USA
F. M. Kirby Research Center for Functional Imaging, Kennedy-Krieger Institute, Baltimore, MD, USA
Neurocirculatory Physiology Laboratory, Departments of Neurology, Anesthesia, and Critical Care Medicine, Johns Hopkins University School of Medicine, Baltimore, MD, USA
Department of Neurology, Johns Hopkins University School of Medicine, Baltimore, MD, USA
Department of Radiology, Johns Hopkins University School of Medicine, Baltimore, MD, USA; F. M. Kirby Research Center for Functional Imaging, Kennedy-Krieger Institute, Baltimore, MD, USA

* Corresponding author; email: jstrous1{at}jhmi.edu.

Overt stroke, clinically "silent" cerebral infarct, and neurocognitive impairment are frequent complications of sickle cell anemia (SCA). Current imaging techniques have limited sensitivity and specificity to identify children at risk for neurocognitive impairment. We prospectively evaluated 24 children with SCA with a neurological exam, complete blood count, transcranial Doppler ultrasound, measurement of intelligence quotient (IQ), and magnetic resonance imaging (MRI) with measurement of cerebral blood flow (CBF) using continuous arterial spin-labeling (CASL) MRI. Average CBF to gray matter was 112 ± 36 ml/100g/min. We identified a strong inverse relationship between performance IQ and CBF (-1.5 points per 10 ml/100 g/min increase in CBF, p = 0.013). Elevated steady-state white blood cell count (≥ 14,000/ul) was associated with lower full scale IQ (86 ± 9 vs. 99 ± 10, p = 0.005). CASL MRI may identify children with neurocognitive impairment, before damage is evident by structural MRI or TCD.


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