Skip to main content Accessibility help

Neuropsychological Profiles of Children with Aqueductal Stenosis and Spina Bifida Myelomeningocele

  • Lyla E. Hampton (a1), Jack M. Fletcher (a1), Paul Cirino (a1), Susan Blaser (a2), Larry A. Kramer (a3) and Maureen Dennis (a4) (a5)...

We compared neuropsychological profiles in children with shunted hydrocephalus secondary to aqueductal stenosis (AS), a rare form of congenital hydrocephalus, and spina bifida myelomeningocele (SBM), a common form of congenital hydrocephalus. Participants were 180 children with shunted hydrocephalus grouped according to etiology: SBM (n = 151), AS (n = 29), and typically developing (TD; n = 60) individuals. The group with AS performed below the TD group on all tasks except for reading, and their overall performance was higher than the group with SBM, who had the lowest performance in the sample. Both clinical groups significantly differed from the TD group on tasks of spatial function, concept formation, motor function, and memory. Performance of the subgroup of AS children with normal cerebellum status approximated that of the TD group, while those with cerebellar anomalies performed lower than others with AS. Cerebellar abnormalities (present in the whole SBM group and in a subset of the AD group) are associated with more compromise of cognitive as well as motor function. (JINS, 2012, 18, 1–10)

Corresponding author
Correspondence and reprint requests to: Lyla Hampton, Department of Psychology, University of Houston Texas Medical Center Annex, 2151 W. Holcombe Blvd., Suite 222, Houston TX 77204-5053. E-mail:
Hide All
Barkovich, A.J. (2005). Congenital anomalies of the spine. Philadelphia: Lippincott Williams and Wilkins.
Barnes, M., Dennis, M. (1998). Discourse after early-onset hydrocephalus: Core deficits in children of average intelligence. Brain and Language, 61, 309334.
Boulet, S., Gambrell, D., Shin, M., Mathews, T., Honein, M. (2009). Racial/Ethnic Differences in the Birth Prevalence of Spina Bifida-United States, 1995–2005. Morbidity and Mortality and Weekly Report (MMWR), 57, 14091413.
Brewer, V., Fletcher, J., Hiscock, M., Davidson, K. (2001). Attention processes in children with shunted hydrocephalus versus attention deficit/hyperactivity disorder. Neuropsychology, 15, 185198.
Brookshire, B., Fletcher, J., Bohan, T., Landry, S., Davidson, K., Francis, D. (1995). Specific language deficiencies in children with early onset hydrocephalus. Child Neuropsychology, 1, 106117.
Burmeister, R., Hannay, H.J., Copeland, K., Fletcher, J.M., Boudousquie, A., Dennis, M. (2005). Attention problems and executive functions in children with spina bifida and hydrocephalus. Child Neuropsychology, 113, 265283.
Charney, E. (1992). Neural tube defects: Spina bifida and myelomeningocele. In M. L. Batshaw & Y. M. Perret (Eds.), Children with disabilities: A medical primer. Baltimore: Brookes.
Cohen, J. (1960). A coefficient of agreement for nominal scales. Educational and Psychological Measurement, 20, 3746.
Del Bigio, M.R. (2010). Neuropathology and structural changes in hydrocephalus. Developmental Disabilities Research Reviews, 16, 1622.
Del Bigio, M.R., Wilson, M.J., Enno, T. (2003). Chronic hydrocephalus in rats and humans: White matter loss and behavior changes. Annals of Neurology, 53, 337346.
Delis, D., Kramer, J.H., Kaplan, E., Ober, B. (1994). California Verbal Learning Test – Children's version. San Antonio, TX: San Antonio Psychological Corporation.
Dennis, M., Barnes, M. (2010). The cognitive phenotype of spina bifida meningomyelocele. Developmental Disabilities Research Reviews, 16, 3139.
Dennis, M., Fitz, C.R., Netley, C.T., Sugar, J., Harwood-Nash, D.C., Hendrick, E.B., Humphreys, R.P. (1981). The intelligence of hydrocephalic children. Archives of Neurology, 38(10), 607615.
Dennis, M., Landry, S.H., Barnes, M., Fletcher, J.M. (2006). A model of neurocognitive function in spina bifida over the life span. Journal of the International Neuropsychological Society, 12, 285296.
Diamond, A. (2000). Close interrelation of motor development and cognitive development and of the cerebellum and prefrontal cortex. Child Development, 71, 4456.
Fletcher, J., Dennis, M. (2010). Spina bifida and hydrocephalus. In K. O. Yeates, D. M. Ris, H. G. Taylor, & B. F. Pennington (Eds.), Pediatric Neuropsychology: Research, Theory and Practice. New York: Guilford.
Fletcher, J.M., Brookshire, B.L., Bohan, T.P., Brandt, M.E., Davidson, K.C. (1995). Early Hydrocephalus. In B. P. Rourke (Ed.), Syndrome of nonverbal learning disabilities: Neurodevelopmental manifestations. New York: Guilford Press.
Fletcher, J.M., Copeland, K., Frederick, J.A., Blaser, S.E., Kramer, L.A., Northrup, H., Dennis, M. (2005). Spinal lesion level in spina bifida: A source of neural and cognitive heterogeneity. Journal of Neurosurgery, 102, 268279.
Fletcher, J.M., Francis, D., Thompson, N., Brookshire, B., Bohan, T., Landry, S., et al (1992). Verbal and nonverbal skill discrepancies in hydrocephalic children. Journal of Clinical and Experimental Neuropsychology, 14, 593609.
Fletcher, J.M., Shaywitz, S.E., Shankweiler, D.P., Katz, L., Liberman, I.Y., Stuebing, K.K., et al (1994). Cognitive profiles of reading disability: Comparisons of discrepancy and low achievement definitions. Journal of Educational Psychology, 86, 623.
Hampton, L.E., Fletcher, J.M., Cirino, P.T., Blaser, S., Kramer, L.A., Drake, J., Dennis, M. (2011). Hydrocephalus status in spina bifida: An evaluation of variations in neuropsychological outcomes. Journal of Neurosurgery. Pediatrics, 8, 289298.
Hannay, H.J., Walker, A., Dennis, M., Kramer, L., Blaser, S., Fletcher, J. (2008). Auditory interhemispheric transfer in relation to patterns of partial agenesis and hypoplasia of the corpus callosum in spina bifida myelomeningocele. Journal of the International Neuropsychological Society, 14, 771781.
Hollingshead, A.B. (1975). Four factor index of social status. Unpublished manuscript. New Haven, Connecticut: Yale University.
Hommet, C., Billard, C., Gillet, P., Barthez, M., Lourmiere, J.M., Santini, J.J., Autret, A. (1999). Neuropsychological and adaptive functioning in adolescents and young adults shunted for congenital hydrocephalus. Journal of Child Neurology, 14, 144150.
Juranek, J., Salman, M.S. (2010). Anomalous development of brain structure and function in spina bifida myelomeningocele. Developmental Disabilities Research Reviews, 16, 2330.
Limperopoulos, C., du Plessis, A.J. (2006). Disorders of cerebellar growth and development. Current Opinion in Pediatrics, 18, 621627.
Lindgren, S.D., Benton, A.L. (1980). Developmental patterns of visuospatial judgment. Journal of Pediatric Psychology, 5, 217225.
Matson, M.A., Mahone, E.M., Zabel, T.A. (2005). Serial neuropsychological assessment and evidence of shunt malfunction in spina bifida: A longitudinal case study. Child Neuropsychology, 11, 315332.
McMillan, J.J., Williams, B. (1977). Aqueduct stenosis. Case review and discussion. Journal of Neurology, Neurosurgery, and Psychiatry, 40, 521532.
Moffitt, K.B., Abiri, O.O., Scheuerle, A.E., Langlois, P.H. (2011). Descriptive epidemiology of selected heritable birth defects in Texas. Birth Defects Research, 91, 990994.
Raimondi, A.J. (1994). A unifying theory for the definition and classification of hydrocephalus. Child's Nervous System, 10, 212.
Schmahmann, J.D. (2004). Disorders of the cerebellum: Ataxia, dysmetria of thoughts, and the cerebellar cognitive affective syndrome. Journal of Neuropsychiatry and Clinical Neuroscience, 16, 367378.
Scott, M.A., Fletcher, J.M., Brookshire, B.L., Davidson, K.C., Landry, S.H., Bohan, T.C., Francis, D.J. (1998). Memory functions in children with early hydrocephalus. Neuropsychology, 12, 578589.
Swartwout, M.D., Garnaat, S.L., Myszka, K.A., Fletcher, J.M., Dennis, M. (2010). Associations of ethnicity and SES with IQ and achievement in spina bifida meningomyelocele. Journal of Pediatric Psychology, 35, 927936.
Tew, B., Laurence, K. (1975). The effects of hydrocephalus on intelligence, visual perception, and school attainment. Developmental Medicine and Child Neurology, 16, 186188.
Thorndike, R.L., Hagen, E.P., Sattler, J.M. (1986). The Stanford-Binet Intelligence Scale: Technical manual (4th ed.). Chicago: Riverside Publishing.
Tiffin, J. (1968). The Purdue Pegboard. Chicago, IL: Research Associates, Inc.
Woodcock, R.W., Johnson, M.B. (1989, 1990). Woodcock-Johnson Psycho-Educational Battery-Revised. Itasca, IL: Riverside Publishing.
Woodcock, R.W., Mather, N. (1989, 1990). WJ-R tests of cognitive ability – standard and supplemental batteries: examiners manual. In R. W. Woodcock & M. B. Johnson (Eds.), Woodcock-Johnson psycho-educational battery-revised. Itasca, IL: Riverside Publishing.
Recommend this journal

Email your librarian or administrator to recommend adding this journal to your organisation's collection.

Journal of the International Neuropsychological Society
  • ISSN: 1355-6177
  • EISSN: 1469-7661
  • URL: /core/journals/journal-of-the-international-neuropsychological-society
Please enter your name
Please enter a valid email address
Who would you like to send this to? *



Full text views

Total number of HTML views: 0
Total number of PDF views: 0 *
Loading metrics...

Abstract views

Total abstract views: 0 *
Loading metrics...

* Views captured on Cambridge Core between <date>. This data will be updated every 24 hours.

Usage data cannot currently be displayed