Hostname: page-component-55f67697df-xlmdk Total loading time: 0 Render date: 2025-05-11T07:27:54.203Z Has data issue: false hasContentIssue false
  • English
  • Français

The Neuropsychological Syndrome of Agenesis of the Corpus Callosum

Published online by Cambridge University Press:  29 January 2019

Warren S. Brown  and
Lynn K. Paul
Warren S. Brown*
Affiliation:
Fuller Graduate School of Psychology, Travis Research Institute, Pasadena, California
Lynn K. Paul
Affiliation:
California Institute of Technology, Division of Humanities and Social Sciences, Pasadena, California
*
Correspondence and reprint requests to: Warren S. Brown, Fuller Graduate School of Psychology 180 N. Oakland Avenue, Pasadena, CA 91101. E-mail: [email protected]
Get access Rights & Permissions [Opens in a new window]

Abstract

Background: Agenesis of the corpus callosum (AgCC) involves congenital absence of all or part of the corpus callosum. Because the disorder can only be firmly diagnosed via neuroradiology, it has a short research history, and only recently has the cognitive syndrome become clear. Purpose: Our purpose is to review the primary deficits in AgCC that constitute the core syndrome. Conclusions: The cores syndrome includes: (1) reduced interhemispheric transfer of sensory-motor information; (2) reduced cognitive processing speed; and (3) deficits in complex reasoning and novel problem-solving. These domains do not appear to reflect different neuroanatomical abnormalities, but rather different domains of expression of reduced interhemispheric communication from callosal absence. Implications: These core deficits are expressed across various domains of cognitive, behavioral, and social functioning. The impact of these deficits varies across development and may be moderated by individual factors such as co-occurrence of other neurodevelopmental conditions, general intellectual capacity, and environmental support. (JINS, 2019, 25, 324–330)

Keywords

Corpus callosumAgenesis of the corpus callosum
Type
Short Review
Information
Journal of the International Neuropsychological Society , Volume 25 , Issue 3 , March 2019 , pp. 324 - 330
Copyright
Copyright © The International Neuropsychological Society 2019 

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.)

Article purchase

Temporarily unavailable

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 Scholar
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. doi:10.1016/j.pediatrneurol.2005.08.008 Google Scholar
Bogen, J., & Frederiks, J. (1985). Split-brain syndromes. Handbook of clinical neurology (Vol. 45, pp. 99–106). Amsterdam, Netherlands: Elsevier Science Publishing Co.Google Scholar
Bridgman, M. W., Brown, W. S., Spezio, M. L., Leonard, M. K., Adolphs, R., & Paul, L. K. (2014). Facial emotion recognition in agenesis of the corpus callosum. Journal of Neurodevelopmental Disorders, 6, 32. doi:10.1186/1866-1955-6-32 Google Scholar
Brown, W. S. (1991). The Bimanual Coordination Test: Version 1. The Travis Research Institute Papers.Google Scholar
Brown, W. S., Anderson, L. B., Symington, M. F., & Paul, L. K. (2012). Decision-making in individuals with agenesis of the corpus callosum: Expectancy-valence in the Iowa Gambling Task. Archives of Clinical Neuropsychology, 27(5), 532–544. doi:10.1093/arclin/acs052 Google Scholar
Brown, W. S., Jeeves, M. A., Dietrich, R., & Burnison, D. S. (1999). Bilateral field advantage and evoked potential interhemispheric transmission in commissurotomy and callosal agenesis. Neuropsychologia, 37(10), 1165–1180.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.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, 906–916.Google Scholar
Brown, W. S., Symington, M., VanLancker, D., Dietrich, R. & Paul, L. K. (2005). Paralinguistic processing in children with callosal agenesis: Emergence of neurolinguistic deficits. Brain and Language, 93, 135–139. doi:10.1016/j.neuropsychologia.2004.09.008 Google Scholar
Bryden, M. P., & Zurif, E. B. (1970). Dichotic listening performance in a case of agenesis of the corpus callosum. Neuropsychologia, 8(3), 371–377.Google Scholar
Buchanan, D. C., Waterhouse, G. J., & West, S. C. Jr. (1980). A proposed neurophysiological basis of alexithymia. Psychotherapy and Psychosomatics, 34, 248–255.Google Scholar
Cherbuin, N., & Brinkman, C. (2005). Practice makes two hemispheres almost perfect. Cognitive Brain Research, 24(3), 413–422.Google Scholar
Chiarello, C. (1980). A house divided? Cognitive functioning with callosal agenesis. Brain and Language, 11, 128–158.Google Scholar
David, A. S., Wacharasindhu, A., & Lishman, W. A. (1993). Severe psychiatric disturbance and abnormalities of the corpus callosum: review and case series. Journal of Neurology, Neurosurgery, and Psychiatry, 56, 85–93.Google Scholar
Dunn, C., Paul, L., Schieffer, B., & Brown, W. (2000). Spatial tactile interhemispheric transfer and task complexity in agenesis of the corpus callosum [Abstract]. Presented at the Twenty-Eighth Annual International Neuropsychological Society Conference: February 9–12, 2000 Denver, Colorado. Journal of the International Neuropsychological Society, 6(2), 165.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, 1579–1613. doi:10.1093/brain/awt358 Google Scholar
Eliassen, J. C., Baynes, K., & Gazzaniga, M. S. (2000). Anterior and posterior callosal contributions to simultaneous bimanual movements of the hands and fingers. Brain, 123(Pt 12), 2501–2511.Google Scholar
Erickson, R. L., Paul, L. K., & Brown, W. S. (2014). Verbal learning and memory in agenesis of the corpus callosum. Neuropsychologia, 60, 121–130. doi:10.1016/j.neuropsychologia.2014.06.003 Google Scholar
Erickson, R. L., Young, C., Paul, L. K., & Brown, W. S. (2013). WAIS-III index scores in individuals with agenesis of the corpus callosum [Abstract]. Presented at the Forty First Annual Meeting International Neuropsychological Society February 6–9, 2013 Waikoloa, Hawaii. Journal of the International Neuropsychological Society, 19(S1), 224.Google Scholar
Fischer, M., Ryan, S. B., & Dobyns, W. B. (1992). Mechanisms of interhemispheric transfer and patterns of cognitive function in acallosal patients of normal intelligence. Archives of Neurology, 49(3), 271–277.Google Scholar
Garrels, S. R., Paul, L. K., Schieffer, B. M., Florendo, E. V., Fox, M. M., Turk, A. A., & Brown, W. S. (2001). Abstract problem solving in children with callosal agenesis [Abstract]. Presented at the Twenty-Ninth Annual International Neuropsychological Society Conference, February 14–17, 2001 Chicago, Illinois. Journal of the International Neuropsychological Society, 7(2), 258.Google Scholar
Geffen, G., Nilsson, J., Quinn, K., & Teng, E. L. (1985). The effects of lesions of the corpus callosum on finger localization. Neuropsychologia, 23, 497–514.Google Scholar
Giedd, J. N., Castellanos, F. X., Casey, B. J., Kozuch, P., King, A. C., Hamburger, S. D., & Rapoport, J. L. (1994). Quantitative morphology of the corpus callosum in attention deficit hyperactivity disorder. American Journal of Psychiatry, 151(5), 665–669.Google Scholar
Glass, H., Shaw, G., Ma, C., & Sherr, E. H. (2008). Agenesis of the corpus callosum in California 1983-2003: a population-based study. American Journal of Medical Genetics, 146A(19), 2495–2500. doi:10.1002/ajmg.a.32418 Google Scholar
Gooijers, J., Caeyenberghs, K., Sisti, H. M., Geurts, M., Heitger, M. H., Leemans, A., & Swinnen, S. P. (2013). Diffusion tensor imaging metrics of the corpus callosum in relation to bimanual coordination: effect of task complexity and sensory feedback. Human Brain Mapping, 34(1), 241–245. doi:10.1002/hbm.21429 Google Scholar
Gott, P. S., & Saul, R. E. (1978). Agenesis of the corpus callosum: Limits of functional compensation. Neurology, 28(12), 1272–1279.Google Scholar
Guillem, P., Fabre, B., Cans, C., Robert-Gnansia, E., & Jouk, P. S. (2003). Trends in elective terminations of pregnancy between 1989 and 2000 in a French county (the Isère). Prenatal Diagnosis, 23(11), 877–883. doi:10.1002/pd.711 Google Scholar
Hanna, S. D. (2018). Academic functioning in individuals with agenesis of the corpus callosum (Unpublished doctoral dissertation). Fuller Graduate School of Psychology, Pasadena, California.Google Scholar
Imamura, T., Yamadori, A., Shiga, Y., Sahara, M., & Abiko, H. (1994). Is disturbed transfer of learning in callosal agenesis due to a disconnection syndrome? Behavioural Neurology, 7, 43–48.Google Scholar
Jeeves, M. A. (1979). Some limits to interhemispheric integration in cases of callosal agenesis and partial commissurotomy. In I. S. Russell, M. W. Van Hof, & G. Berlucchi (Eds.), Structure and function of the cerebral commissures (pp. 449–474). London: McMillan.Google Scholar
Jeeves, M. A., & Ettlinger, E. G. (1965). Psychological studies of the three cases of congenital agenesis of the corpus callosum. Functions of the Corpus Callosum: CIBA Foundations Study Groups (Vol. 20, pp. 73–94).Google Scholar
Jeeves, M. A., & Silver, P. H. (1988). Interhemispheric transfer of spatial tactile information in callosal agenesis and partial commissurotomy. Cortex, 24, 601–604.Google Scholar
Jeeves, M. A., Silver, P. H., & Jacobson, I. (1988). Bimanual co-ordination in callosal agenesis and partial commissurotomy. Neuropsychologia, 26, 833–850.Google Scholar
Jeeves, M. A., Silver, P. H., & Milner, A. D. (1988). Role of the corpus callosum in the development of a bimanual skills. Developmental Neuropsychology, 4, 305–323.Google Scholar
Kang, K. H., Paul, L. K., Castelli, F., & Brown, W. S. (2009). Theory of mind in agenesis of the corpus callosum compared to high functioning autism [Abstract]. Poster presented at the Thirty-Seventh Annual Meeting International Neuropsychological Society: February 11–14, 2009 Atlanta, Georgia. Journal of the International Neuropsychological Society, 15(S1), 168. doi:10.1017/S1355617709090420 Google Scholar
Kaplan, J. M., Brown, W. S., Adolphs, R., & Paul, L. K. (2012). Psychological profile in agenesis of the corpus callosum [Abstract]. Poster presented at the Society for Neuroscience Annual Meeting: October 13–17, 2012 New Orleans, Louisiana. 198.19.Google Scholar
Kourtidou, P., McCauley, S. R., Bigler, E. D., Traipe, E., Wu, T. C., Chu, Z. D., . . . Wilde, E. A. (2013). Centrum semiovale and corpus callosum integrity in relation to information processing speed in patients with severe traumatic brain injury. Journal of Head Trauma Rehabilitation, 28(6), 433–441. doi:10.1097/HTR.0b013e3182585d06 Google Scholar
Koivisto, M. (2000). Interhemispheric interaction in semantic categorization of pictures. Cognitive Brain Research, 9(1), 45–51. PII: S0926-6410(99)00042-7.Google 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
Maertens, M., & Pollmann, S. (2005). Interhemispheric resource sharing: Decreasing benefit with increasing processing efficiency. Brain and Cognition, 58(2), 183–192. doi:10.1016/j.bandc.2004.11.002 Google Scholar
Mangum, R. (2018). Self-understanding of executive function in individuals with agenesis of the corpus callosum. (Unpublished doctoral dissertation). Fuller Graduate School of Psychology, Pasadena, California.Google Scholar
Marco, E. J., Harrell, K. M., Brown, W. S., Hill, S. S., Jeremy, R. J., Kramer, J. H., . . . Paul, L. K. (2012). Processing speed delays contribute to executive function deficits in individuals with agenesis of the corpus callosum. Journal of the International Neuropsychological Society, 18(3), 521–529. doi:10.1017/s1355617712000045 Google Scholar
Marion, S. D., Kilian, S. C., Naramor, T. L., & Brown, W. S. (2003). Normal development of bimanual coordination: Visuomotor and interhemispheric contributions. Developmental Neuropsychology, 23(3), 399–421. doi:10.1207/s15326942dn2303_6 Google Scholar
Mathias, J. L., Bigler, E. D., Jones, N. R., Bowden, S. C., Barrett-Woodbridge, M., Brown, G. C., & Taylor, D. J. (2004) Neuropsychological and information processing performance and its relationship to white matter changes following moderate and severe traumatic brain injury: A preliminary study, Applied Neuropsychology, 11(3), 134–152. doi:10.1207/s15324826an1103_2 Google Scholar
Miller, J. S., Su, J. J., Paul, L. K., & Brown, W. S. (2018). Adaptive behavior in agenesis of the corpus callosum: Self and informant reports. [Abstract]. Presented at the Forty First Annual Meeting International Neuropsychological Society February 14–17, 2018 Washington, DC. Journal of the International Neuropsychological Society, 24(S1), 195.Google Scholar
Mueller, K. L. O., Marion, S. D., Paul, L. K., & Brown, W. S. (2009). Bimanual motor coordination in agenesis of the corpus callosum. Behavioral Neuroscience, 123(5), 1000–1011. doi:10.1037/a0016868 Google Scholar
Norman, W., Jeeves, M. A., Milne, A., & Ludwig, T. (1992). Hemispheric interactions: the bilateral advantage and task difficulty. Cortex, 28(4), 623–642.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. doi:10.1038/nrn2107 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, 1813–1829. doi:10.1093/brain/awu070 Google Scholar
Paul, L. K., Erickson, R. L., Hartman, J. A., & Brown, W. S. (2016). Learning and memory in individuals with agenesis of the corpus callosum. Neuropsychologia, 86, 183–192. doi:10.1016/j.neuropsychologia.2016.04.013 Google Scholar
Paul, L. K., Lautzenhiser, A., Brown, W. S., Hart, A., Neumann, D., Spezio, M., & Adolphs, R. (2006). Emotional arousal in agenesis of the corpus callosum. International Journal of Psychophysiology, 61(1), 47–56. doi:10.1016/j.ijpsycho.2005.10.017 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. doi:10.1016/S0887-6177(03)00024-6 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. doi:10.1016/s0093- 934x(03)00062-2 Google Scholar
Pazienza, S. R., Brown, W. S., & Paul, L. K. (2011). Emotional expressiveness and somatization in agenesis of the corpus callosum. [Abstract]. Poster presented at the Thirty-Ninth Annual Meeting International Neuropsychological Society: February 2–5, 2011 Boston, Massachusetts USA. Journal of the International Neuropsychological Society, 17(S1), 2. doi:10.1017/S1355617711000415 Google Scholar
Preilowski, B. F. (1972). Possible contribution of the anterior forebrain commissures to bilateral motor coordination. Neuropsychologia, 10(3), 267–277.Google Scholar
Raven, J. C. (1960). Guide to the standard progressive matrices. London: H.K. Lewis.Google Scholar
Raven, J. C. (1965). Guide to using the colored progressive matrices. London: H.K. Lewis.Google Scholar
Rehmel, J. L., Brown, W. S., & Paul, L. K. (2016). Proverb comprehension in individuals with agenesis of the corpus callosum. Brain and Language, 160, 21–29. doi:10.1016/j.bandl.2016.07.00 1 Google Scholar
Reuter-Lorenz, P. A., Stanczak, L., & Miller, A. C. (1999). Neural recruitment and cognitive aging: Two hemispheres are better than one, especially as you age. Psychological Science, 10(6), 494–500.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
Sauerwein, H. C., Lassonde, M., Cardu, B., & Geoffroy, G. (1981). Interhemispheric integration of sensory and motor functions in callosal agenesis. Neuropsychologia, 19, 445–454.Google Scholar
Saul, R. E., & Sperry, R. W. (1968). Absence of commissurotomy symptoms with agenesis of the corpus callosum. Neurology, 18, 307.Google Scholar
Schell-Apacik, C. C., Wagner, K., Bihler, M., Ertl-Wagner, B., Heinrich, U., Klopocki, E., . . . von Voss, H. (2008). Agenesis and dysgenesis of the corpus callosum: clinical, genetic and neuroimaging findings in a series of 41 patients. American Journal of Medical Genetics, 146A(19), 2501–2511. doi:10.1002/ajmg.a.32476 Google Scholar
Schieffer, B., Paul, L. K., & Brown, W. S. (2000). Deficits in complex concept formation in agenesis of the corpus callosum [Abstract]. Poster presented at the Twenty-Eighth Annual International Neuropsychological Society Conference: February 9–12, 2000 Denver, Colorado . Journal of the International Neuropsychological Society, 6(2), 164.Google Scholar
Schieffer, B., Paul, L. K., Schilmoeller, K., & Brown, W. S. (2000). Components of intelligence and basic achievement in agenesis of the corpus callosum [Abstract]. Poster presented at the Twenty-Eighth Annual International Neuropsychological Society Conference: February 9–12, 2000 Denver, Colorado. Journal of the International Neuropsychological Society, 6(2), 164.Google Scholar
Solmaz, B., Tunc, B., Parker, D., Whyte, J., Hart, T., Rabinowitz, A., . . . Verma, R. (2017). Assessing connectivity related injury burden in diffuse traumatic brain injury. Human Brain Mapping, 38(6), 2913–2922. doi:10.1002/hbm.23561 Google Scholar
Solursh, L. P., Margulies, A. I., Ashem, B., & Stasiak, E. A. (1965). The relationships of agenesis of the corpus callosum to perception and learning. Journal of Nervous and Mental Disease, 141(2), 180–189.Google Scholar
Sperry, R. W. (1968). Hemisphere deconnection and unity in conscious awareness. American Psychologist, 23(10), 723–733.Google Scholar
Sperry, R. W., Gazzaniga, M., Bogen, J., Vinken, P. J., & Bruyn, G. W. (1969). Interhemispheric relationships: the neocortical commissures; syndromes of hemisphere disconnection. Handbook of Clinical Neurology, 4, 273–290).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
Tang, P. H., Bartha, A. I., Norton, M. E., Barkovich, A. J., Sherr, E. H., & Glenn, O. A. (2009). Agenesis of the corpus callosum: an MR imaging analysis of associated abnormalities in the fetus. AJNR American Journal of Neuroradiology, 30(2), 257–263. doi:10.3174/ajnr.A1331Google Scholar
Turk, A., Brown, W. S., Symingtion, M., & Paul, L. K. (2010). Social narratives in agenesis of the corpus callosum: linguistic analysis of the Thematic Apperception Test. Neuropsychologia, 48, 43–50. doi:10.1016/j.neuropsychologia.2009.08.009 Google Scholar
Ubukata, S., Ueda, K., Sugihara, G., Yassin, W., Aso, T., Fukuyama, H., & Murai, T. (2016). Corpus callosum pathology as a potential surrogate marker of cognitive impairment in diffuse axonal injury. Journal of Neuropsychiatry and Clinical Neuroscience, 28(2), 97–103. doi:10.1176/appi.neuropsych.15070159 Google Scholar
Wahl, M., Strominger, Z., Jeremy, R. J., Barkovich, A. J., Wakahiro, I., Sherr, E. H., & Mukherjee, P. (2009). Variability of homotopic and heterotopic callosal connectivity in partial agenesis of the corpus callosum: a 3T diffusion tensor imaging and q-ball tractography study. AJNR American Journal of Neuroradiology, 30(2), 282–289. doi:10.3174/ajnr.A1361 Google Scholar
Wang, L. W., Huang, C. C., & Yeh, T. F. (2004). Major brain lesions detected on sonographic screening of apparently normal term neonates. Neuroradiology, 46(5), 368–373. doi:10.1007/s00234-003-1160-4 Google Scholar
Weissman, D. H., & Banich, M. T. (2000). The cerebral hemispherescooperate to perform complex but not simple tasks. Neuropsychology, 14(1), 41–59.Google Scholar
Weissman, D. H., & Compton, R. J. (2003). Practice makes a hemisphere perfect: The advantage of interhemispheric recruitment is eliminated with practice. Laterality: Asymmetries of Body, Brain, and Cognition, 8(4), 361–375.Google Scholar
Yakovlev, P. I., Lecours, A., & Minkovski, A. (1967). The myelogenetic cycles of regional maturation of the brain. Regional Development of the Brain in Early Life (pp. 3–65).Google Scholar
Young, C. M., Erickson, R. L., Paul, L. K., & Brown, W. S. (2013). Academic achievement in children and adults with agenesis of the corpus callosum. Poster presented at the Forty First Annual Meeting International Neuropsychological Society February 6–9, 2013 Waikoloa, Hawaii. Journal of the International Neuropsychological Society, 19(S1), 44. doi:10.1017/S1355617713000362 Google Scholar
Young, C. M., Folsom, R. C., Paul, L. K., Su, J., Mangum, R., & Brown, W. S. (in press). Awareness of consequences in agenesis of the corpus callosum: semantic analysis of responses.Google Scholar