Order Number 9201784 Agenesis of the corpus callosum in preschool children Wuebbens, Stephanie Ann, Ph.D. The Ohio State University, 1991 Copyright ©1991 by Wuebbens, Stephanie Ann. All rights reserved. UMI 300 N. Zeeb Rd. Ann Arbor, MI 48106 AGENESIS OF THE CORPUS CALLOSUM IN PRESCHOOL CHILDREN DISSERTATION Presented in Partial Fulfillment of the Requirements for the Degree Doctor of Philosophy in the Graduate School of the Ohio State University By Stephanie Ann Wuebbens, B.A., M.S ***** The Ohio State University 1991 Dissertation Committee: Approved by H. Leland D. Hammer yAdvidviser G. Berntson Departme j?ft o f Psychology Copyright by Stephanie Ann Wuebbens 1991 ACKNOWLEDGMENTS My sincere appreciation to Dr. Henry Leland for his guidance, encouragement, and insight during the course of this research. Many thanks to Dr. David Hammer and Dr. Gary Berntson for their suggestions and guidance. Gratitude is expressed to Dr. Keith Yates and Dr. Eric Handler for their assistance and to Dr. Edward Kosnik for his medical expertise. Thank you to Dr. Donald T. Campbell for his thoughtful input on methodology and design. To the parents of the children in this study, who were so willing to cooperate in the research and so eager for the information obtained, my thanks. To the agencies involved with the children's education, and to the teachers for their interest and assistance, my appreciation. A special thanks to my friends and colleagues who kept a sharp eye out for potential subjects. To Greg, my love and continuing appreciation for the patience, understanding, and support through all the peaks and valleys. VITA August 14, 195 9 ..... Born - Detroit, Michican 1981................... B.A., Capital University, Columbus, Ohio 1981-1984............. Case Manager, CompDrug VITA Youth Services, Columbus, Ohio 1984................... M.S., The Ohio State University, Columbus, Ohio 1984-198 5 ............. Psychology Intern, Midwest Neuropsychological Institute, Columbus, Ohio 1985-Present ......... Psychology Intern, Franklin County Board MR/DD, Early Childhood Education Center, Columbus, Ohio FIELDS OF STUDY Major Field: Psychology Studies in Mental Retardation and Developmental Disabilities. Professors Henry Leland, David Hammer. Studies in Developmental Psychology. Professors Dorothy Jackson, Dale Blythe, Felicisima Serafica, Gerald Winer, John Gibbs. Studies in the Neurological Bases of Behavior. Professors Gary Berntson, Ronald Mervis. Studies in Family Relations and Human Development. Professors Barbara Newman, Joseph Mullan. TABLE OF CONTENTS ACKNOWLEDGEMENTS.......................................... ii VITA....................................................... iii LIST OF TABLES............................................ vii LIST OF FIGURES........................................... ix CHAPTER PAGE I. INTRODUCTION...................................... 1 Statement of the problem......................... 10 Objective.......................................... 11 Research questions................................ 11 Definition of terms.............................. 12 II. REVIEW OF THE LITERATURE......................... 13 Development of the cerebral commissures........ 13 Callosal agenesis................................. 16 Associated anomalies............................. 17 Probst's bundles............................... 17 Other structural anomalies.................... 18 Associated neurological anomalies............ 21 Shapiro syndrome............................... 22 Andermann syndrome............................. 23 Holoprosencephaly 2 3 Prevalence........................................ 25 Etiology........................................... 26 Clinical features................................. 29 Cognitive development.......................... 30 Language development........................... 32 Visual functioning............................. 37 Visual-motor integration and bimanual coordination.................................... 40 Bimanual transfer of learning................. 44 Kinesthetic funtioning 4 5 Summary............................................ 46 III. METHODOLOGY...................................... 4 8 Subjects and setting............................. 48 iv Procedure.......................................... 50 Measures........................................... 51 Adaptive Behavior............................... 51 Cognitive and Developmental................... 52 Language Development........................... 53 Visual-motor and Bimanual...................... 5 3 Statistical analyses.............................. 54 IV. DATA ANALYSIS...................................... 56 Overview of the chapter.......................... 5 6 Background data................................... 57 Current functioning............................... 64 Developmental trends 7 6 Partial agenesis of the corpus callosum........ 88 Summary............................................ 93 V. CASE STUDIES....................................... 94 Subjects with Agenesis of the Corpus Callosum........................................... 94 Case 1 .............................................. 94 Previous Assessments........................... 96 Tests Administered.............................. 97 McCarthy Scales........... 97 ABSI........................................... 99 Language Tests................................ 101 Perceptual-Performance and Bimanual Tasks.. 101 Case 2 .............................................. 103 Previous Assessments........................... 105 Tests Administered.............................. 105 McCarthy Scales............................... 105 ABSI........................................... 108 Language Tests................................ 110 Perceptual-Performance and Bimanual Tasks.. Ill Case 3 .............................................. 114 Previous Assessments........................... 115 Tests Administered.............................. 116 Bayley Scales................................. 116 ABSI........................................... 118 Language Tests................................ 121 Case 4 ............................................. 121 Tests Administered.............................. 123 Bayley Scales................................. 123 ABSI........................................... 125 Language Tests................................ 127 Case 5 ............................................. 127 Tests Administered.............................. 128 McCarthy Scales............................... 128 ABSI........................................... 130 Language Tests................................ 131 v Perceptual-Performance and Bimanual Tasks.. 132 Case 6 ............................................... 135 Tests Administered.............................. 136 Bayley Scales.................................. 136 ABSI............................................ 138 Subjects with Partial Agenesis................... 140 Case P7.............................................. 140 Previous Assessments.............................. 141 Tests Administered............ 142 McCarthy Scales.................................. 142 Bayley Scales.................................... 143 ABSI............................................... 144 Language Tests................................... 146 Perceptual-Performance and Bimanual Tests.... 146 Case P8 .............................................. 148 Previous Assessments.............................. 149 Tests Administered................................. 150 Bayley Scales.................................... 152 ABSI............................................... 153 Language Tests................................... 155 VI. DISCUSSION.......................................... 156 General Findings................................... 156 Patterns of Developmental Delay.................. 157 Specific Developmental Delays.................... 160 Visual-motor functioning....................... 161 Language skills.................................. 162 Age and Developmental Delay...................... 163 Implications of the Study......................... 165 Implications for Education of Young Acallosal Children............... 167 Limitations of the Study.......................... 168 Future Research.................................... 17 0 Conclusions......................................... 171 LIST OF REFERENCES......................................... 173 vi LIST OF TABLES TABLE PAGE 1. Subject characteristics....................... 49 2. Age at diagnosis and symptoms................ 58 3. Complications at birth........................ 60 4. Seizure type and age at onset................ 61 5. Congenital anomalies as notedin medical records........................................ 63 6. McCarthy results.............................. 65 7. Bayley results,................................ 66 8. Adaptive behavior results.................... 6 8 9. Verbal measures............................... 72 10. Visual-motor tasks............................ 7 3 11. Case 2: Previous developmental levels....... 85 12. Case 3: Previous developmental levels....... 87 13. Comparison of functioning levels: subjects with agenesis and partial agenesis matched by age.............. .......................... 8 9 14. Case 1: Summary of results................... 102 15. Case 2: Summary of results................... 113 16 Case 3: Summary of results................... 120 17 Case 4: Summary of results................... 126 18 Case 5: Summary of results..................