Hostname: page-component-5cf477f64f-xc2pj Total loading time: 0 Render date: 2025-04-03T08:00:01.790Z Has data issue: false hasContentIssue false
  • English
  • Français

EXAMINER Executive Function Battery and Neurologic Morbidity in Pediatric Sickle Cell Disease

Published online by Cambridge University Press:  27 November 2013

Jeffrey Schatz ,
Melita Stancil ,
Tal Katz  and
Carmen E. Sanchez
Jeffrey Schatz*
Affiliation:
Department of Psychology, University of South Carolina, Columbia, South Carolina
Melita Stancil
Affiliation:
Department of Psychology, University of South Carolina, Columbia, South Carolina
Tal Katz
Affiliation:
Department of Psychology, University of South Carolina, Columbia, South Carolina
Carmen E. Sanchez
Affiliation:
Department of Psychology, University of South Carolina, Columbia, South Carolina
*
Correspondence and reprint requests to: Jeffrey Schatz, Department of Psychology, University of South Carolina, 1512 Pendleton Street, Columbia, SC 29205. E-mail: schatz@sc.edu
Get access Rights & Permissions [Opens in a new window]

Abstract

Sickle cell disease (SCD) is blood disorder with a high risk for cerebral vascular morbidities that impact neurocognitive functioning. Specific cognitive abilities are known to be more sensitive to neurologic effects of SCD than IQ scores, yet there is little consensus about which measures to use to assess neurocognitive functioning. We evaluated the ability of the Executive Abilities: Methods and Instruments for Neurobehavioral Evaluation and Research (EXAMINER) Battery to detect neurologic effects in SCD. Thirty-two youth with SCD and sixty demographically-matched comparison youth completed the EXAMINER Battery and selected tests from the Woodcock-Johnson Tests of Cognitive Ability, 3rd edition (WJ-III). Neurologic severity was examined via clinical history for morbidities and midsagittal corpus callosum (CC) area. Results indicated cognitive performance decreased with increasing neurologic morbidity across all cognitive measures; two of four EXAMINER factors were related to CC area. The association with clinical history and midsagittal CC area appeared at least as large for the Examiner Battery scores as for the WJ-III measures. The Examiner Battery showed sensitivity to neurologic history and white matter effects in SCD; this new measure compares favorably to established measures of disease-related neurocognitive effects, but would benefit from further development. (JINS, 2014, 1, 1–12)

Keywords

AnemiaSickle cellChildAdolescentCerebrovascular disordersNeuropsychologic testsImagingMagnetic resonance
Type
Special Series
Information
Journal of the International Neuropsychological Society , Volume 20 , Issue 1 , January 2014 , pp. 29 - 40
Copyright
Copyright © The International Neuropsychological Society 2013 

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

References

Abboud, M.R., Cure, J., Granger, S., Gallagher, D., Hsu, L., Wang, W., the STOP study. (2004). Magnetic resonance angiography in children with sickle cell disease and abnormal transcranial Doppler ultrasonography findings enrolled in the STOP study. Blood, 103, 2822–2826.CrossRefGoogle ScholarPubMed
Adams, R.J. (2005). TCD in sickle cell disease: An important and useful test. Pediatric Radiology, 35, 229–234.CrossRefGoogle ScholarPubMed
Adams, R.J., Ohene-Frempong, K., Wang, W. (2001). Sickle cell and the brain. Hematology: American Society of Hematology Education Program, 1, 31–46.CrossRefGoogle Scholar
American Academy of Pediatrics. Section on Pediatric Pulmonology, Subcommittee on Obstructive Sleep Apnea Syndrome. (2002). Clinical practice guideline: Diagnosis and management of childhood obstructive sleep apnea syndrome. Pediatrics, 109, 704–712.CrossRefGoogle Scholar
Armstrong, F.D., Thompson, R.J., Wang, W. Jr., Zimmerman, R., Pegelow, C.H., Miller, S., Vass, K. (1996). Cognitive functioning and brain magnetic resonance imaging in children with sickle Cell disease. Neuropsychology Committee of the Cooperative Study of Sickle Cell Disease. Pediatrics, 97, 864–870.CrossRefGoogle ScholarPubMed
Baldeweg, T., Hogan, A.M., Saunders, D.E., Telfer, P., Gadian, D.G., Vargha-Khadem, F., Kirkham, F.J. (2006). Detecting white matter injury in sickle cell disease using voxel-based morphometry. Annals of Neurology, 59, 662–672.CrossRefGoogle ScholarPubMed
Berkelhammer, L.D., Williamson, A.L., Sanford, S.D., Dirksen, C.L., Sharp, W.G., Margulies, A.S., Prengler, R.A. (2007). Neurocognitive sequelae of pediatric sickle cell disease: A review of the literature. Child Neuropsychology, 13, 120–131.CrossRefGoogle ScholarPubMed
Bernaudin, F., Verlhac, S., Fréard, F., Roudot-Thoraval, F., Benkerrou, M., Thuret, I., Brugières, P. (2000). Multicenter prospective study of children with sickle cell disease: Radiographic and psychometric correlation. Journal of Child Neurology, 15, 333–343.CrossRefGoogle ScholarPubMed
Buchanan, I.D., James-Herry, A., Osunkwo, I. (2013). The other side of abnormal: A case series of low transcranial Doppler velocities associated with stroke in children with sickle cell disease. Journal of Pediatric Hematology Oncology, 35, 543–546.CrossRefGoogle ScholarPubMed
Casella, J.F., King, A.A., Barton, B., White, D.A., Noetzel, M.J., Ichord, R.N., Debaun, M.R. (2010). Design of the silent cerebral infarct transfusion (SIT) trial. Pediatric Hematology Oncology, 27, 69–89.CrossRefGoogle ScholarPubMed
Cohen, J. (1988). Statistical power analysis for the behavioral sciences. Hillsdale, NJ: Lawrence Erlbaum Associates.Google Scholar
DeBaun, M., Schatz, J., Koby, M., Craft, S., Resar, L., Chu, J.Y., Noetzel, M. (1998). Cognitive screening examinations for silent cerebral infarcts in sickle cell disease. Neurology, 50, 1678–1682.CrossRefGoogle ScholarPubMed
Giedd, J.N., Blumenthal, J., Jeffries, N.O., Rajapakse, J.C., Vaituzis, C., Liu, H., Castellanos, F.X. (1999). Development of the human corpus callosum during childhood and adolescence: A longitudinal MRI study. Progress in Neuro-Psychopharmacology and Biological Psychiatry, 23, 571–588.Google ScholarPubMed
Goldstein, N.A., Keller, R., Rey, K., Rao, S., Weedon, J., Dastgir, G., Miller, S.T. (2011). Sleep-disordered breathing and transcranial Dopplers in sickle cell disease. Archives of Otolaryngology Head and Neck Surgery, 137(12), 1263–1268.CrossRefGoogle ScholarPubMed
Hanley, J.A., McNeil, B.J. (1982). The meaning and use of the area under a receiver operating characteristic (ROC) curve. Radiology, 143, 29–36.CrossRefGoogle Scholar
Hijmans, C.T., Fijnvandraat, K., Grootenhuis, M.A., van Geloven, N., Heijboer, H., Peters, M., Oosterlaan, J. (2011). Neurocognitive deficits in children with sickle cell disease: A comprehensive profile. Pediatric Blood and Cancer, 56, 783–788.CrossRefGoogle ScholarPubMed
Hogan, A.M., Kirkham, F.J., Isaacs, E.B., Wade, A.M., Vargha-Khadem, F. (2005). Intellectual decline in children with moyamoya and sickle cell anaemia. Developmental Medicine and Child Neurology, 47, 824–829.CrossRefGoogle ScholarPubMed
King, A.A., DeBaun, M.R., White, D.A. (2008). Need for cognitive rehabilitation for children with sickle cell disease and strokes. Expert Review of Neurotherapeutics, 8, 291–296.CrossRefGoogle ScholarPubMed
Kral, M.C., Brown, R.T., Nietert, P.J., Abboud, M.R., Jackson, S.M., Hynd, G.W. (2003). Transcranial Doppler ultrasonography and neurocognitive functioning in children with sickle cell disease. Pediatrics, 112, 324–331.CrossRefGoogle ScholarPubMed
Kramer, J.H., Mungas, D., Possin, K.L., Rankin, K.P., Boxer, A.L., Rosen, H.J., Widmeyer, M. (2013). NIH EXAMINER: Conceptualization and development of an executive function battery. Journal of the International Neuropsychological Society, 1–9.Google ScholarPubMed
Lal, C., Strange, C., Bachman, D. (2012). Neurocognitive impairment in obstructive sleep apnea. Chest, 141(6), 1601–1610.CrossRefGoogle ScholarPubMed
Macey, P.M., Henderson, L.A., Macey, K.E., Alger, J.R., Frysinger, R.C., Woo, M.A., Harper, R.M. (2002). Brain morphology associated with obstructive sleep apnea. American Journal of Respiratory and Critical Care Medicine, 166(10), 1382–1387.CrossRefGoogle ScholarPubMed
Miyake, A., Friedman, N.P., Emerson, M.J., Witzki, A.H., Howerter, A., Wager, T.D. (2000). The unity and diversity of executive functions and their contributions to complex “Frontal Lobe” tasks: A latent variable analysis. Cognitive Psychology, 41, 49–100.CrossRefGoogle ScholarPubMed
Morrell, M.J., McRobbie, D.W., Quest, R.A., Cummin, A.R., Ghiassi, R., Corfield, D.R. (2003). Changes in brain morphology associated with obstructive sleep apnea. Sleep Medicine, 4(5), 451–454.CrossRefGoogle ScholarPubMed
Murphy, J.M., Berwick, D.M., Weinstein, M.C., Borus, J.F., Budman, S.H., Klerman, G.L. (1987). Performance of screening and diagnostic tests: Application of receiver operating characteristic analysis. Archives of General Psychiatry, 44, 550.CrossRefGoogle ScholarPubMed
Ohene-Frempong, K., Weiner, S.J., Sleeper, L.A., Miller, S.T., Embury, S., Moohr, J.W., Gill, F.M. (1998). Cerebrovascular accidents in sickle cell disease: Rates and risk factors. Blood, 91, 288–294.Google ScholarPubMed
Pegelow, C.H., Macklin, E.A., Moser, F.G., Wang, W.C., Bello, J.A., Miller, S.T., Kinney, T.R. (2002). Longitudinal changes in brain magnetic resonance imaging findings in children with sickle cell disease. Blood, 99, 3014–3018.CrossRefGoogle ScholarPubMed
Sanchez, C.E., Schatz, J., McClellan, C.B., Roberts, C.W. (2010). Cerebral blood flow velocity and language functioning in pediatric sickle cell disease. Journal of the International Neuropsychological Society, 16, 326–334.CrossRefGoogle ScholarPubMed
Schatz, J., Brown, R.T., Pascual, J.M., Hsu, L., DeBaun, M.R. (2001). Poor school and cognitive functioning with silent cerebral infarction and sickle cell disease. Neurology, 56, 1109–1111.CrossRefGoogle ScholarPubMed
Schatz, J., Buzan, R.F. (2006). Decreased corpus callosum size in sickle cell disease: Relationship with cerebral infarcts and cognitive functioning. Journal of the International Neuropsychological Society, 12, 17–23.CrossRefGoogle ScholarPubMed
Schatz, J., Finke, R.L., Kellet, J.M., Kramer, J.H. (2002). Cognitive functioning in children with sickle cell disease: A meta-analysis. Journal of Pediatric Psychology, 27, 739–748.CrossRefGoogle ScholarPubMed
Schatz, J., Finke, R.L., Roberts, C.W. (2004). Interactions among biomedical and environmental factors in cognitive development: A preliminary study of sickle cell disease. Journal of Developmental and Behavioral Pediatrics, 25, 303–310.CrossRefGoogle ScholarPubMed
Schatz, J., Hale, S., Myerson, J. (1998). Cerebellar contribution to linguistic processing efficiency revealed through focal damage. Journal of the International Neuropsychological Society, 4, 491–501.CrossRefGoogle ScholarPubMed
Schatz, J., McClellan, C.B. (2006). Sickle cell disease as a neurodevelopmental disorder. Mental Retardation and Developmental Disabilities Research Reviews, 12, 200–207.CrossRefGoogle ScholarPubMed
Schatz, J., Puffer, E. (2006). Neuropsychological aspects of sickle cell disease. In R. T. Brown (Ed), Comprehensive handbook of childhood cancer and sickle cell disease (pp. 449–470). New York NY: Oxford University Press.Google Scholar
Schatz, J., Puffer, E.S., Sanchez, C., Stancil, M., Roberts, C.W. (2009). Language processing deficits in sickle cell disease in young school-age children. Developmental Neuropsychology, 34, 122–136.CrossRefGoogle ScholarPubMed
Schatz, J., Roberts, C.W. (2005). Short-term memory in children with sickle cell disease: Executive versus modality specific processing deficits. Archives of Clinical Neuropsychology, 20, 1073–1085.CrossRefGoogle ScholarPubMed
Schatz, J., Roberts, C.W. (2007). Neurobehavioral impact of sickle cell anemia in early childhood. Journal of the International Neuropsychological Society, 13, 933–943.CrossRefGoogle ScholarPubMed
Schmidt, E.A., Piechnik, S.K., Smielewski, P., Raabe, A., Matta, B.F., Czosnyka, M. (2003). Symmetry of cerebral hemodynamic indices derived from bilateral transcranial Doppler. Journal of Neuroimaging, 13, 248–254.CrossRefGoogle ScholarPubMed
Schneider, C.A., Rasband, W.S., Eliceiri, K.W. (2012). NIH image to ImageJ: 25 years of image analysis. Nature Methods, 9, 671–675.CrossRefGoogle ScholarPubMed
Serjeant, G.R. (1995). Natural history and determinants of clinical severity of sickle cell disease. Current Opinion in Hematology, 2, 103–108.CrossRefGoogle ScholarPubMed
Sforza, E., Roche, F. (2012). Sleep apnea syndrome and cognition. Frontiers in Neurology, 3, 87.CrossRefGoogle ScholarPubMed
Strauss, T., Sin, S., Marcus, C.L., Mason, T.B., McDonough, J.M., Allen, J.L., Arens, R. (2012). Upper airway lymphoid tissue size in children with sickle cell disease. Chest, 142, 94–100.CrossRefGoogle ScholarPubMed
Testa, R., Bennett, P., Ponsford, J. (2012). Factor analysis of nineteen executive function tests in a healthy adult population. Archives Of Clinical Neuropsychology, 27, 213–224.CrossRefGoogle Scholar
Wang, W.C., Gallagher, D.M., Pegelow, C.H., Wright, E.C., Vichinsky, E.P., Abboud, M.R., Adams, R.J. (2000). Multicenter comparison of magnetic resonance imaging and transcranial Doppler ultrasonography in the evaluation of the central nervous system in children with sickle cell disease. Journal of Pediatric Hematology Oncology, 22, 335–339.CrossRefGoogle ScholarPubMed
Wang, W.C., Ware, R.E., Miller, S.T., Iyer, R.V., Casella, J.F., Minniti, C.P., Thompson, B.W. (2011). Hydroxycarbamide in very young children with sickle-cell anaemia: A multicentre, randomized, controlled trial (BABY HUG). The Lancet, 377, 1663–1672.CrossRefGoogle ScholarPubMed
White, D.A., Saloria, C.F., Schatz, J., DeBaun, M.R. (2000). Preliminary study of working memory in children with stroke related to sickle cell disease. Journal of Clinical and Experimental Neuropsychology, 22, 257–264.CrossRefGoogle ScholarPubMed
Witelson, S.F. (1989). Hand and sex differences in the isthmus and genu of the human corpus callosum: A postmortem morphological study. Brain, 112, 799–835.CrossRefGoogle ScholarPubMed
Woodcock, R.W., McGrew, K.S., Mather, N. (2001). Woodcock-Johnson III tests of cognitive abilities. Itasca, IL: Riverside Publishing.Google Scholar