Hostname: page-component-76fb5796d-dfsvx Total loading time: 0 Render date: 2024-04-25T09:50:37.050Z Has data issue: false hasContentIssue false
  • English
  • Français

Cerebral blood flow velocity and language functioning in pediatric sickle cell disease

Published online by Cambridge University Press:  03 February 2010

CARMEN E. SANCHEZ ,
JEFFREY SCHATZ  and
CARLA W. ROBERTS
CARMEN E. SANCHEZ*
Affiliation:
Department of Psychology, University of South Carolina, Columbia, South Carolina
JEFFREY SCHATZ
Affiliation:
Department of Psychology, University of South Carolina, Columbia, South Carolina
CARLA W. ROBERTS
Affiliation:
Department of Pediatrics, University of South Carolina, Columbia, South Carolina
*
*Correspondence and reprint requests to: Carmen E. Sanchez, Department of Psychology, University of South Carolina, Columbia, SC, 29208. E-mail: sanchece@mailbox.sc.edu
Get access Rights & Permissions [Opens in a new window]

Abstract

We investigated the association of increased cerebral blood flow velocity with specific language abilities in children with sickle cell disease (SCD). Thirty-nine children ages 5 to 8 years old with high-risk genotypes of SCD underwent cognitive testing, which included tests of language skills, visual motor skills, and attention/working memory as part of a routine hematology health-maintenance visit. Transcranial Doppler (TCD) velocities were obtained from review of medical records, with the velocities that were in closest temporal proximity to the cognitive assessment used in the analysis. TCD velocities predicted scores on tests of syntactical skills, even when controlling for anemia severity. Semantic and phonological ability and other cognitive skills were not strongly related to TCD velocities. Elevated blood flow velocities in children with high-risk SCD may contribute to a specific language impairment or to a broader dysfunction of short-term and/or working memory. This study underscores the need for clinicians to monitor language skills of children with SCD who have elevated TCD velocities, as these cognitive abilities might be particularly sensitive to cerebrovascular disruption related to their disease. (JINS, 2010, 16, 326–334.)

Keywords

AnemiaSickle CellNeurobehavioral manifestationsChildUltrasonographyDopplerTranscranialCerebrovascular disordersNeuropsychological test
Type
Research Articles
Information
Journal of the International Neuropsychological Society , Volume 16 , Issue 2 , March 2010 , pp. 326 - 334
Copyright
Copyright © The International Neuropsychological Society 2010

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

REFERENCES

Abboud, M.R., Cure, J., Granger, S., Gallagher, D., Hsu, L., Wang, W., et al. . (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., Brambilla, D.J., Granger, S., Gallagher, D., Vichinsky, E., Abboud, M.R., et al. . (2004). Stroke and conversion to high risk in children screened with transcranial doppler ultrasound during the stop study. Blood, 103(10), 3689–3694.CrossRefGoogle ScholarPubMed
Adams, R.J., McKie, V.C., Brambilla, D., Carl, E., Gallagher, D., Nichols, F.T., et al. . (1998a). Stroke prevention trial in sickle cell anemia. Controlled Clinical Trials, 19, 110–129.CrossRefGoogle ScholarPubMed
Adams, R.J., McKie, V.C., Carl, E.M., Nichols, F.T., Perry, R., Brock, K., et al. . (1997). Long-term stroke risk in children with sickle cell disease screened with transcranial Doppler. Annals of Neurology, 42, 699–704.CrossRefGoogle ScholarPubMed
Adams, R.J., McKie, V.C., Hsu, L., Files, B., Vichinsky, E., Pegelow, C., et al. . (1998b). Prevention of a first stroke by transfusions in children with sickle cell anemia and abnormal results on transcranial Doppler ultrasonography. New England Journal of Medicine, 339, 5–11.CrossRefGoogle ScholarPubMed
Adams, R.J., McKie, V.C., Nichols, F., Carl, E., Zhang, D.L., McKie, K., et al. . (1992). The use of transcranial ultrasonography to predict stroke in sickle-cell disease. New England Journal of Medicine, 326, 605–610.Google Scholar
Adams, R.J., Ohene-Frempong, K., & Wang, W. (2001). Sickle cell and the brain. Hematology, 31–45.CrossRefGoogle ScholarPubMed
Baddeley, A.D. (1986). Working memory. Oxford: Oxford University Press.Google ScholarPubMed
Baddeley, A.D. (2000). The episodic buffer: A new component of working memory? Trends in Cognitive Sciences, 4, 417–423.CrossRefGoogle ScholarPubMed
Baddeley, A.D., & Hitch, G. (1974). Working memory. In Bower, G.A. (Ed.), Recent advances in learning and motivation (Vol. 8, pp. 47–90). Burlington, MA: Academic Press.Google Scholar
Baldeweg, T., Hogan, A.M., Saunders, D.E., Telfer, P., Gadian, D.G., Vargha-Khadem, R., et al. . (2006). Detecting white matter injury in sickle cell disease using voxel-based morphometry. Annals of Neurology, 59, 662–672.Google Scholar
Beery, K.E., Buktenica, N.A., & Beery, N.A. (2004). The Beery–Buktenica developmental test of visual-motor integration: Administration, scoring and teaching manual. Minneapolis, MN: NCS Pearson.Google Scholar
Berkelhammer, L.D., Williamson, A.L., Sanford, S.D., Dirksen, C.L., Sharp, W.G., Margulies, A.S., et al. . (2007). Neurocognitive sequelae of pediatric sickle cell disease: A review of the literature. Child Neuropsychology, 13, 120–131.CrossRefGoogle ScholarPubMed
Bernaudin, F., Verlhac, S., Freard, F., Roudot-Thoravel, F., Benkerrou, M., Thuret, I., et al. . (2000). Multicenter prospective study of children with sickle cell disease: Radiographic and psychometric correlation. Journal of Child Neurology, 15, 333–343.Google Scholar
Bhutta, A.T., Cleves, M.A., Casey, P.H., Cradock, M.M., & Anand, K.J.S. (2002). Cognitive and behavioral outcomes of school-aged children who were born preterm – A meta-analysis. JAMA – Journal of the American Medical Association, 288, 728–737.Google Scholar
Brambilla, D.J., Miller, S.T., & Adams, R.J. (2007). Intra-individual variation in blood flow velocities in cerebral arteries of children with sickle cell disease. Pediatric Blood & Cancer, 49, 318–322.CrossRefGoogle ScholarPubMed
Charache, S., Lubin, B., & Reid, C.D. (Eds.). (2002). Management and therapy of sickle cell disease. Washington, DC: U.S. Government Printing Office.Google Scholar
Cohen, P., & Cohen, J. (1984). The clinician’s illusion. Archives of General Psychiatry, 41, 1178–1182.CrossRefGoogle ScholarPubMed
Gaston, M., Smith, J., Gallagher, D., Flournoygill, Z., West, S., Bellevue, R., et al. . (1987). Recruitment in the cooperative study of sickle-cell disease (CSSCD). Controlled Clinical Trials, 8, S131–S140.Google Scholar
Gathercole, S.E. (1999). Cognitive approaches to the development of short-term memory. Trends in Cognitive Sciences, 3, 410–419.CrossRefGoogle Scholar
Hankins, J.S., Fortner, G.L., McCarville, M.B., Smeltzer, M.P., Wang, W.C., Li, C.S., et al. . (2008). The natural history of conditional transcranial Doppler flow velocities in children with sickle cell anaemia. British Journal of Haematology, 142, 94–99.Google Scholar
Hill, C.M., Hogan, A.M., Onugha, N., Harrison, D., Cooper, S., McGrigor, V.J., et al. . (2006). Increased cerebral blood flow velocity in children with mild sleep-disordered breathing: A possible association with abnormal neuropsychological function. Pediatrics, 118, E1100–E1108.CrossRefGoogle ScholarPubMed
Kral, M.C., & Brown, R.T. (2004). Transcranial Doppler ultrasonography and executive dysfunction in children with sickle cell disease. Journal of Pediatric Psychology, 29, 185–195.CrossRefGoogle ScholarPubMed
Kral, M.C., Brown, R.T., Connelly, M., Cure, J.K., Besenski, N., Jackson, S.M., et al. . (2006). Radiographic predictors of neurocognitive functioning in pediatric sickle cell disease. Journal of Child Neurology, 21, 37–44.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
Laws, G. (2004). Contributions of phonological memory, language comprehension and hearing to the expressive language of adolescents and young adults with Down syndrome. Journal of Child Psychology and Psychiatry, 45, 1085–1095.Google Scholar
Leonard, L.B. (1998). Children with specific language impairment. Cambridge, MA: MIT Press.Google ScholarPubMed
Lowe, L.H., & Bulas, D.I. (2005). Transcranial Doppler imaging in children: Sickle cell screening and beyond. Pediatric Radiology, 35, 54–65.CrossRefGoogle ScholarPubMed
McGrew, K.S., & Woodcock, R.W. (2001). Woodcock–Johnson III, technical manual. Itasca, IL: Riverside Publishing.Google Scholar
Newcomer, P.L., & Hammil, D.D. (1997). Examiner’s manual, Test of Language Development Primary: Third edition. Austin, TX: Pro-Ed.Google Scholar
Noll, R.B., Stith, L., Gartstein, M.A., Ris, M.D., Grueneich, R., Vannatta, K., et al. . (2001). Neuropsychological functioning of youths with sickle cell disease: Non-chronically ill peers. Journal of Pediatric Psychology, 26, 69–78.CrossRefGoogle ScholarPubMed
Ohene-Frempong, K., Weiner, S.J., Sleeper, L.A., Miller, S.T., Embury, S., Moohr, J.W., et al. . (1998). Cerebrovascular accidents in sickle cell disease: Rates and risk factors. Blood, 91, 288–294.Google ScholarPubMed
Peterson, B.S., Vohr, B., Staib, L.H., Cannistraci, C.J., Dolberg, A., Schneider, K.C., et al. . (2000). Regional brain volume abnormalities and long-term cognitive outcome in preterm infants. JAMA – Journal of the American Medical Association, 284, 1939–1947.CrossRefGoogle ScholarPubMed
Pickett, J.L., Theberge, D.C., Brown, W.S., Schweitzer, S.U., & Nissenson, A.R. (1999). Normalizing hematocrit in dialysis patients improves brain function. American Journal of Kidney Diseases, 33(6), 1122–1130.CrossRefGoogle ScholarPubMed
Prengler, M., Pavlakis, S.G., Prohovnik, I., & Adams, R.J. (2002). Sickle cell disease: The neurological complications. Annals of Neurology, 51, 543–552.CrossRefGoogle ScholarPubMed
Puffer, E., Schatz, J., & Roberts, C.W. (2007). The association of oral hydroxyurea therapy with improved cognitive functioning in sickle cell disease. Child Neuropsychology, 13, 142–154.Google Scholar
Schatz, J., Craft, S., Koby, M., Siegel, M.J., Resar, L., Lee, R.R., et al. . (1999). Neuropsychological deficits in children with sickle cell disease and cerebral infarction: Role of lesion site and volume. Child Neuropsychology, 5, 92–103.Google Scholar
Schatz, J., Finke, R.L., Kellett, 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., Roberts, C.W. (2004). Interactions of biomedical and environmental risk factors for cognitive development: A preliminary study of sickle cell disease. Journal of Developmental and Behavioral Pediatrics, 25, 303–310.CrossRefGoogle ScholarPubMed
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
Steen, R.G., Fineberg-Buchner, C., Hankins, G., Weiss, L., Prifitera, A., & Mulhern, R.K. (2005). Cognitive deficits in children with sickle cell disease. Journal of Child Neurology, 20, 102–107.Google Scholar
Steen, R.G., Miles, M.A., Helton, K.J., Strawn, S., Wang, W., Xiong, X.P., et al. . (2003). Cognitive impairment in children with hemoglobin SS sickle cell disease: Relationship to MR imaging findings and hematrocrit. American Journal of Neuroradiology, 24, 382–389.Google Scholar
Steen, R.G., Xiong, X.P., Mulhern, R.K., Langston, J.W., & Wang, W.C. (1999). Subtle brain abnormalities in children with sickle cell disease: Relationship to blood hematocrit. Annals of Neurology, 45, 279–286.3.0.CO;2-7>CrossRefGoogle ScholarPubMed
Steinberg, M.H. (1984). Review – The sickle hemoglobinopathies:Genetic analyses of common phenocopies and new molecular approaches to treatment. American Journal of the Medical Sciences, 288, 169–174.CrossRefGoogle ScholarPubMed
Stivelman, J.C. (2000). Benefits of anaemia treatment on cognitive function. Nephrology Dialysis Transplantation, 15, 29–35.CrossRefGoogle ScholarPubMed
Strouse, J.J., Cox, C.S., Melhem, E.R., Lu, H.Z., Kraut, M.A., Razumovsky, A. et al. (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). Blood, 108(1), 379–381.CrossRefGoogle ScholarPubMed
Toppelberg, C.O., & Shapiro, T. (2000). Language disorders: A 10-year research update review. Journal of the American Academy of Child and Adolescent Psychiatry, 39, 143–152.CrossRefGoogle ScholarPubMed
Tucker, J., & McGuire, W. (2004). ABC of preterm birth – Epidemiology of preterm birth. British Medical Journal, 329, 675–678.CrossRefGoogle Scholar
Uylings, H.B.M., Malofeeva, L.I.Bogolepova, I.N., Amunts, K., & Zilles, K. (1999). Broca’s language area from a neuroanatomical and developmental perspective. In Brown, C.M. & Hagoort, P. (Eds.), The neurocognition of language (pp. 319–336). Oxford: Oxford University Press.Google Scholar
Venketasubramanian, N., Prohovnik, I., Hurlet, A., Mohr, J.P., & Piomelli, S. (1994). Middle cerebral-artery velocity changes during transfusion in sickle-cell-anemia. Stroke, 25, 2153–2158.Google Scholar
Wang, W., Enos, L., Gallagher, D., Thompson, R., Guarini, L., Vichinsky, E., et al. . (2001). Neuropsychologic performance in school-aged children with sickle cell disease: A report from the Cooperative Study of Sickle Cell Disease. Journal of Pediatrics, 139, 391–397.CrossRefGoogle ScholarPubMed
Wang, W., Gallagher, D.M., Pegelow, C.H., Wright, E.C., Vichinsky, E.P., Abboud, M.R., et al. . (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.Google Scholar
Zimmerman, S.A., Schultz, W.H., Burgett, S., Mortier, N.A., & Ware, R.E. (2007). Hydroxyurea therapy lowers transcranial Doppler flow velocities in children with sickle cell anemia. Blood, 110, 1043–1047.Google Scholar