This Article Full Text (PDF) References Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Alert me to new issues of the journal Add to Saved Citations Download to citation manager Request Permissions Request Reprints Add to My Marked Citations Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Citing Articles via Scopus Google Scholar Articles by Shevell, M. I. Search for Related Content PubMed Articles by Shevell, M. I. Social Bookmarking                   What's this?

Clinical and Diagnostic Profile of Agenesis of the Corpus Callosum

Departments of Neurology/Neurosurgery and Pediatrics, McGill University, and the Division of Pediatric Neurology, Montreal Children's Hospital-McGill University Health Centre, Montreal, PQ, michael.shevell{at}muhc.mcgill.ca

This study reports the clinical profile, etiologies identified, and outcomes for a consecutive series of children with partial or complete agenesis of the corpus callosum. Children with agenesis of the corpus callosum were identified in a comprehensive computerized database of all patients seen in a single pediatric neurology practice over an 11-year interval. Medical records were then systematically reviewed. Twenty-four children with agenesis of the corpus callosum were identified of a total of 6911 children in the database (0.35%). Fifteen were male (62.5%); 9 (37.5%) had presented antenatally, 6 (25%) neonatally, and 9 (37.5%) postneonatally. Eight (33.3%) were microcephalic, 12 (50%) were dysmorphic, 11 (45.8%) had coexisting epilepsy, and 9 (37.5%) had a cerebral palsy variant. Investigations revealed an etiology in 11 (45.8%): 3 chromosomal abnormality, 3 metabolic disorder, 3 cerebral dysgenesis, and 2 genetic syndromes (Aicardi, Andermann). Outcomes identified included normal or mild developmental delay in 7 (29.2%) and moderate-severe developmental delay in the remaining 17 (70.8%). Factors predictive of successful etiologic determination on bivariate analysis included moderate-severe developmental delay or associated cerebral dysgenesis. Factors predictive of eventual developmental outcome included microcephaly, coexisting epilepsy, cerebral palsy, or cerebral dysgenesis. A spectrum of clinical presentations, underlying etiology, and developmental outcome is thus apparent in children with agenesis of the corpus callosum. An underlying etiology can be identified in slightly less than half of cases, and a normal or mildly delayed outcome is apparent in slightly less than a third. Factors predictive of identifying an underlying etiology or eventual outcome can be identified. (J Child Neurol 2002;17:895—899).

Journal of Child Neurology, Vol. 17, No. 12, 895-899 (2002)
DOI: 10.1177/08830738020170122601


CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this?

This article has been cited by other articles:

				W. Kulak, W. Sobaniec, B. Kubas, and J. Walecki Corpus Callosum Size in Children With Spastic Cerebral Palsy: Relationship to Clinical Outcome J Child Neurol, April 1, 2007; 22(4): 371 - 374. [Abstract] [PDF]