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A Neuropsychological Profile for Agenesis of the Corpus Callosum? Cognitive, Academic, Executive, Social, and Behavioral Functioning in School-Age Children

Published online by Cambridge University Press:  07 March 2018

Vanessa Siffredi ,
Vicki Anderson ,
Alissandra McIlroy ,
Amanda G. Wood ,
Richard J. Leventer  and
Megan M. Spencer-Smith
Vanessa Siffredi
Affiliation:
Laboratory for Behavioral Neurology and Imaging of Cognition, University of Geneva, Switzerland School of Psychological Sciences, University of Melbourne, Melbourne, Australia
Vicki Anderson
Affiliation:
Laboratory for Behavioral Neurology and Imaging of Cognition, University of Geneva, Switzerland School of Psychological Sciences, University of Melbourne, Melbourne, Australia Department of Psychology, Royal Children’s Hospital, Melbourne, Australia
Alissandra McIlroy
Affiliation:
Laboratory for Behavioral Neurology and Imaging of Cognition, University of Geneva, Switzerland
Amanda G. Wood
Affiliation:
Laboratory for Behavioral Neurology and Imaging of Cognition, University of Geneva, Switzerland School of Life and Health Sciences, Aston University, United-Kingdom
Richard J. Leventer
Affiliation:
Department of Paediatrics, University of Melbourne, Melbourne, Australia Department of Neurology, Royal Children’s Hospital, Melbourne, Australia Neuroscience Research Group, Murdoch Children’s Research Institute, Melbourne, Australia
Megan M. Spencer-Smith*
Affiliation:
Laboratory for Behavioral Neurology and Imaging of Cognition, University of Geneva, Switzerland School of Psychological Sciences and Monash Institute of Cognitive and Clinical Neurosciences, Monash University, Melbourne, Australia
*
Correspondence and reprint requests to: Megan Spencer-Smith, School of Psychological Sciences and Monash Institute of Cognitive and Clinical Neurosciences, Monash University, 18 Innovation Walk, Clayton Campus, Clayton VIC 3800, Australia. E-mail: [email protected]
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Abstract

Objectives: Agenesis of the corpus callosum (AgCC), characterized by developmental absence of the corpus callosum, is one of the most common congenital brain malformations. To date, there are limited data on the neuropsychological consequences of AgCC and factors that modulate different outcomes, especially in children. This study aimed to describe general intellectual, academic, executive, social and behavioral functioning in a cohort of school-aged children presenting for clinical services to a hospital and diagnosed with AgCC. The influences of age, social risk and neurological factors were examined. Methods: Twenty-eight school-aged children (8 to 17 years) diagnosed with AgCC completed tests of general intelligence (IQ) and academic functioning. Executive, social and behavioral functioning in daily life, and social risk, were estimated from parent and teacher rated questionnaires. MRI findings reviewed by a pediatric neurologist confirmed diagnosis and identified brain characteristics. Clinical details including the presence of epilepsy and diagnosed genetic condition were obtained from medical records. Results: In our cohort, ~50% of children experienced general intellectual, academic, executive, social and/or behavioral difficulties and ~20% were functioning at a level comparable to typically developing children. Social risk was important for understanding variability in neuropsychological outcomes. Brain anomalies and complete AgCC were associated with lower mathematics performance and poorer executive functioning. Conclusions: This is the first comprehensive report of general intellectual, academic, executive social and behavioral consequences of AgCC in school-aged children. The findings have important clinical implications, suggesting that support to families and targeted intervention could promote positive neuropsychological functioning in children with AgCC who come to clinical attention. (JINS, 2018, 24, 445–455)

Keywords

agenesis of the corpus callosumcongenital brain malformationneuropsychological outcomespediatricscognitive functionssocio-behavioral functions
Type
Research Articles
Information
Journal of the International Neuropsychological Society , Volume 24 , Issue 5 , May 2018 , pp. 445 - 455
Copyright
Copyright © The International Neuropsychological Society 2018 

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References

REFERENCES

Anderson, L.B., Paul, L.K., & Brown, W.S. (2017). Emotional intelligence in agenesis of the corpus callosum. Archives of Clinical Neuropsychology, 32(3), 267–279. doi: 10.1093/arclin/acx001 Google ScholarPubMed
Anderson, V., Spencer-Smith, M., Leventer, R., Coleman, L., Anderson, P., Williams, J., & Jacobs, R. (2009). Childhood brain insult: Can age at insult help us predict outcome? Brain, 132(Pt 1), 45–56. doi: 10.1093/brain/awn293 Google Scholar
Barr, M.S., & Corballis, M.C. (2002). The role of the anterior commissure in callosal agenesis. Neuropsychology, 16(4), 459–471. doi: 10.1037/0894-4105.16.4.459 CrossRefGoogle ScholarPubMed
Bedeschi, M.F., Bonaglia, M.C., Grasso, R., Pellegri, A., Garghentino, R.R., Battaglia, M.A., & Borgatti, R. (2006). Agenesis of the corpus callosum: Clinical and genetic study in 63 young patients. Pediatric Neurology, 34(3), 186–193.CrossRefGoogle ScholarPubMed
Booth, R., Wallace, G.L., & Happe, F. (2011). Connectivity and the corpus callosum in autism spectrum conditions: Insights from comparison of autism and callosal agenesis. Progress in Brain Research, 189, 303–317. doi: 10.1016/B978-0-444-53884-0.00031-2 Google Scholar
Brown, W.S., & Paul, L.K. (2000). Cognitive and psychosocial deficits in agenesis of the corpus callosum with normal intelligence. Cognitive Neuropsychiatry, 5(2), 135–157. doi: 10.1080/135468000395781 Google Scholar
Brown, W.S., Paul, L.K., Symington, M., & Dietrich, R. (2005). Comprehension of humor in primary agenesis of the corpus callosum. Neuropsychologia, 43(6), 906–916. doi: 10.1016/j.neuropsychologia.2004.09.008 Google Scholar
Caillé, S., Schiavetto, A., Andermann, F., Bastos, A., de Guise, E., & Lassonde, M. (1999). Interhemispheric transfer without forebrain commissures. Neurocase, 5(2), 109–118. doi: 10.1093/neucas/5.2.109 CrossRefGoogle Scholar
Chiarello, C. (1980). A house divided? Cognitive functioning with callosal agenesis. Brain and Language, 11(1), 128–158. doi: 10.1016/0093-934x(80)90116-9 Google Scholar
D’Antonio, F., Pagani, G., Familiari, A., Khalil, A., Sagies, T.L., Malinger, G., & Prefumo, F. (2016). Outcomes associated with isolated agenesis of the corpus callosum: A meta-analysis. Pediatrics, 138(3). doi: 10.1542/peds.2016-0445 Google Scholar
Dennis, M. (2000). Developmental plasticity in children: The role of biological risk, development, time, and reserve. Journal of Communication Disorders, 33(4), 321–331. quiz 332.CrossRefGoogle ScholarPubMed
Dennis, M., Yeates, K., Taylor, H., & Fletcher, J. (2006). Brain reserve capacity, cognitive reserve capacity, and age-based functional plasticity after congenital and acquired brain injury in children. London and New York: Taylor & Francis.Google Scholar
Doherty, D., Tu, S., Schilmoeller, K., & Schilmoeller, G. (2006). Health-related issues in individuals with agenesis of the corpus callosum. Child: Care, Health And Development, 32(3), 333–342.Google Scholar
Edwards, T.J., Sherr, E.H., Barkovich, A.J., & Richards, L.J. (2014). Clinical, genetic and imaging findings identify new causes for corpus callosum development syndromes. Brain, 137(Pt 6), 1579–1613. doi: 10.1093/brain/awt358 Google Scholar
Farah, M.J., Shera, D.M., Savage, J.H., Betancourt, L., Giannetta, J.M., Brodsky, N.L., & Hurt, H. (2006). Childhood poverty: Specific associations with neurocognitive development. Brain Research, 1110(1), 166–174. doi: 10.1016/j.brainres.2006.06.072 CrossRefGoogle ScholarPubMed
Field, A.P. (2013). Discovering statistics using IBM SPSS statistics: And sex and drugs and rock ‘n’ roll (4th ed.), London: Sage.Google Scholar
Gioia, G.A., Isquith, P.K., Guy, S.C., & Kenworthy, L. (2000). Behavior rating inventory of executive function. Odessa, FL: Psychological Assessment Resources.Google Scholar
Glass, H.C., Shaw, G.M., Ma, C., & Sherr, E.H. (2008). Agenesis of the corpus callosum in California 1983-2003: A population-based study. American Journal of Medical Genetics. Part A, 146A(19), 2495–2500.Google Scholar
Goodman, R. (1997). The strengths and difficulties questionnaire: A research note. Journal of Child Psychology and PSYCHIATRY, 38(5), 581–586.Google Scholar
Graham, J.M. Jr., Visootsak, J., Dykens, E., Huddleston, L., Clark, R.D., Jones, K.L., & Stevenson, R.E. (2008). Behavior of 10 patients with FG syndrome (Opitz-Kaveggia syndrome) and the p.R961W mutation in the MED12 gene. American Journal of Medical Genetics. Part A, 146A(23), 3011–3017.CrossRefGoogle ScholarPubMed
Graham, J.M. Jr., Wheeler, P., Tackels-Horne, D., Lin, A.E., Hall, B.D., May, M., & Cox, T.C. (2003). A new X-linked syndrome with agenesis of the corpus callosum, mental retardation, coloboma, micrognathia, and a mutation in the Alpha 4 gene at Xq13. American Journal of Medical Genetics. Part A, 123A(1), 37–44.Google Scholar
Gresham, F.M., & Elliott, S.N. (2008). Social Skills Improvement System Rating Scales manual. Minneapolis, MN: NCS Pearson.Google Scholar
Groen, M.A., Whitehouse, A.J., Badcock, N.A., & Bishop, D.V. (2012). Does cerebral lateralization develop? A study using functional transcranial Doppler ultrasound assessing lateralization for language production and visuospatial memory. Brain and Behavior, 2(3), 256–269. doi: 10.1002/brb3.56.Google Scholar
Hackman, D.A., & Farah, M.J. (2009). Socioeconomic status and the developing brain. Trends in Cognitive Sciences, 13(2), 65–73. doi: 10.1016/j.tics.2008.11.003 Google Scholar
Hannay, H.J., Dennis, M., Kramer, L., Blaser, S., & Fletcher, J.M. (2009). Partial agenesis of the corpus callosum in spina bifida meningomyelocele and potential compensatory mechanisms. Journal of Clinical and Experimental Neuropsychology, 31(2), 180–194.CrossRefGoogle ScholarPubMed
Hetts, S.W., Sherr, E.H., Chao, S., Gobuty, S., & Barkovich, A.J. (2006). Anomalies of the corpus callosum: An MR analysis of the phenotypic spectrum of associated malformations. AJR American Journal of Roentgenology, 187, 1343–1348.CrossRefGoogle Scholar
Huber-Okrainec, J., Blaser, S.E., & Dennis, M. (2005). Idiom comprehension deficits in relation to corpus callosum agenesis and hypoplasia in children with spina bifida meningomyelocele. Brain and Language, 93(3), 349–368.Google Scholar
Jordan, N.C., & Levine, S.C. (2009). Socioeconomic variation, number competence, and mathematics learning difficulties in young children. Developmental Disabilities Research Reviews, 15(1), 60–68. doi: 10.1002/ddrr.46 Google Scholar
Lábadi, B., & Beke, A.M. (2017). Mental State Understanding in Children with Agenesis of the Corpus Callosum. Frontiers in Psychology, 8, 94. doi: 10.3389/fpsyg.2017.00094 Google Scholar
Lassonde, M., & Jeeves, M.A. (1994). Callosal agenesis: A natural split brain?. New York, NY: Plenum Press.CrossRefGoogle Scholar
Lassonde, M., Sauerwein, H., Chicoine, A.J., & Geoffroy, G. (1991). Absence of disconnexion syndrome in callosal agenesis and early callosotomy: Brain reorganization or lack of structural specificity during ontogeny? Neuropsychologia, 29(6), 481–495.Google Scholar
Lee, S.W., Kim, K.S., Cho, S.M., & Lee, S.J. (2004). An atypical case of Aicardi syndrome with favorable outcome. Korean Journal Of Ophthalmology, 18(1), 79–83.Google Scholar
Leventer, R.J., Phelan, E.M., Coleman, L.T., Kean, M.J., Jackson, G.D., & Harvey, A.S. (1999). Clinical and imaging features of cortical malformations in childhood. Neurology, 53(4), 715–722.Google Scholar
Loeser, J.D., & Alvord, E.C. Jr. (1968). Clinicopathological correlations in agenesis of the corpus callosum. Neurology, 18(8), 745–756.Google Scholar
Marsh, A.P., Heron, D., Edwards, T.J., Quartier, A., Galea, C., Nava, C., & Depienne, C. (2017). Mutations in DCC cause isolated agenesis of the corpus callosum with incomplete penetrance. Nature Genetics, 49(4), 511–514. doi: 10.1038/ng.3794 Google Scholar
Mellor, D. (2005). Normative data for the Strengths and Difficulties Questionnaire in Australia. Australian Psychologist, 40(3), 215–222. doi: 10.1080/00050060500243475 Google Scholar
Moutard, M.L., Kieffer, V., Feingold, J., Kieffer, F., Lewin, F., Adamsbaum, C., & Ponsot, G. (2003). Agenesis of corpus callosum: Prenatal diagnosis and prognosis. Child’s Nervous System, 19(7-8), 471–476.CrossRefGoogle ScholarPubMed
Oldfield, R.C. (1971). The assessment and analysis of handedness: The Edinburgh inventory. Neuropsychologia, 9(1), 97–113.Google Scholar
Palmer, E.E. & Mowat, D. (2014). Agenesis of the corpus callosum: A clinical approach to diagnosis. Am J Med Genet C Semin Med Genet, 166C(2), 184–197. doi: 10.1002/ajmg.c.31405.Google Scholar
Paul, L.K. (2011). Developmental malformation of the corpus callosum: A review of typical callosal development and examples of developmental disorders with callosal involvement. Journal of Neurodevelopmental Disorders, 3(1), 3–27. doi: 10.1007/s11689-010-9059-y Google Scholar
Paul, L.K., Brown, W.S., Adolphs, R., Tyszka, J.M., Richards, L.J., Mukherjee, P., &&Sherr, E.H. (2007). Agenesis of the corpus callosum: Genetic, developmental and functional aspects of connectivity. Nature Reviews. Neuroscience, 8(4), 287–299.Google Scholar
Paul, L.K., Corsello, C., Kennedy, D.P., & Adolphs, R. (2014). Agenesis of the corpus callosum and autism: A comprehensive comparison. Brain, 137(Pt 6), 1813–1829. doi: 10.1093/brain/awu070 Google Scholar
Paul, L.K., Schieffer, B., & Brown, W.S. (2004). Social processing deficits in agenesis of the corpus callosum: Narratives from the Thematic Appreciation Test. Archives of Clinical Neuropsychology, 19(2), 215–225.Google Scholar
Paul, L.K., Van Lancker-Sidtis, D., Schieffer, B., Dietrich, R., & Brown, W.S. (2003). Communicative deficits in agenesis of the corpus callosum: Nonliteral language and affective prosody. Brain and Language, 85(2), 313–324.Google Scholar
Pilu, G., Sandri, F., Perolo, A., Pittalis, M.C., Grisolia, G., Cocchi, G., & Bovicelli, L. (1993). Sonography of fetal agenesis of the corpus callosum: A survey of 35 cases. Ultrasound in Obstetrics & Gynecologogy, 3(5), 318–329. doi: 10.1046/j.1469-0705.1993.03050318.x Google Scholar
Pisani, F., Bianchi, M.E., Piantelli, G., Gramellini, D., & Bevilacqua, G. (2006). Prenatal diagnosis of agenesis of corpus callosum: What is the neurodevelopmental outcome? Pediatrics International, 48(3), 298–304.Google Scholar
Roberts, G., Howard, K., Spittle, A.J., Brown, N.C., Anderson, P.J., & Doyle, L.W. (2008). Rates of early intervention services in very preterm children with developmental disabilities at age 2 years. Journal of Paediatrics and Child Health, 44(5), 276–280. doi: 10.1111/j.1440-1754.2007.01251.x Google Scholar
Sarsour, K., Sheridan, M., Jutte, D., Nuru-Jeter, A., Hinshaw, S., & Boyce, W.T. (2011). Family socioeconomic status and child executive functions: The roles of language, home environment, and single parenthood. Journal of the International Neuropsychological Society, 17(1), 120–132. doi: 10.1017/S1355617710001335 Google Scholar
Sauerwein, H.C., & Lassonde, M. (1994). Cognitive and sensori-motor functioning in the absence of the corpus callosum: Neuropsychological studies in callosal agenesis and callosotomized patients. Behavioural Brain Research, 64(1-2), 229–240.Google Scholar
Shevell, M.I. (2002). Clinical and diagnostic profile of agenesis of the corpus callosum. Journal of Child Neurology, 17(12), 896–900.CrossRefGoogle ScholarPubMed
Siffredi, V., Anderson, V., Leventer, R.J., & Spencer-Smith, M.M. (2013). Neuropsychological profile of agenesis of the corpus callosum: A systematic review. Developmental Neuropsychology, 38(1), 36–57. doi: 10.1080/87565641.2012.721421 CrossRefGoogle ScholarPubMed
Siffredi, V., Spencer-Smith, M.M., Barrouillet, P., Vaessen, M.J., Leventer, R.J., Anderson, V., && Vuilleumier, P. (2017). Neural correlates of working memory in children and adolescents with agenesis of the corpus callosum: An fMRI study. Neuropsychologia, 106, 71–82. doi: 10.1016/j.neuropsychologia.2017.09.008 Google Scholar
Sirin, S.R. (2005). Socioeconomic status and academic achievement: A meta-analytic review of research. Review of Educational Research, 75(3), 417–453. doi: 10.3102/00346543075003417 Google Scholar
Symington, S.H., Paul, L.K., Symington, M.F., Ono, M., & Brown, W.S. (2010). Social cognition in individuals with agenesis of the corpus callosum. Social Neuroscience, 5(3), 296–308. doi: 10.1080/17470910903462419 Google Scholar
Turk, A.A., Brown, W.S., Symington, M., & Paul, L.K. (2010). Social narratives in agenesis of the corpus callosum: Linguistic analysis of the Thematic Apperception Test. Neuropsychologia, 48(1), 43–50. doi: 10.1016/j.neuropsychologia.2009.08.009 Google Scholar
Vergani, P., Ghidini, A., Strobelt, N., Locatelli, A., Mariani, S., Bertalero, C., && Cavallone, M. (1994). Prognostic indicators in the prenatal diagnosis of agenesis of corpus callosum. American Journal of Obstetrics and Gynecology, 170(3), 753–758.Google Scholar
Wechsler, D. (1999). Wechsler Abbreviated Scale of Intelligence (WASI). San Antonio, TX: Psychological Corporation.Google Scholar
Wechsler, D. (2003). Manual for the Wechsler Intelligence Scale for Children-IV. New York: Psychological Corporation.Google Scholar
Wilkinson, G.S., & Robertson, G.J. (2006). Wide Range Achievement Test 4, Professional manual. Lutz, FL: Psychological Assessment Resources.Google Scholar
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