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Agenesis of the corpus callosum (ACC) is an infrequent congenital abnormality that has been diagnosed by necropsy, surgery, pneumoencephalography, computerized tomography (CT), and magnetic resonance imaging. The reported prevalence has varied as a function of disability status of the population studied and diagnostic technique. This report found 33 cases of ACC in a consecutive series of 1,447 CTs of developmentally disabled individuals. The prevalence, 2.3%, is consistent with studies using other techniques. However, the significantly higher (p < 0.01) rate from 1978 to 1979 suggestes an initial tendency of neuroradiologists to overdiagnose ACC, and CT data from the 1970s may need to be reexamined.

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This study examined 35 patients with developmental disabilities who were referred for diagnostic evaluation that later revealed agenesis of the corpus callosum (ACC) by computerized tomography (CT). Sixteen had partial ACC, six had complete ACC, and one had a hypoplastic corpus callosum. In the other twelve cases, ACC existed, but the degree of callosal defect was not specified. Other intracranial defects were frequently present. Clinically, 15 patients (43%) had a history of seizures, 28 (82%) were mentally retarded or developmentally delayed and an additional five patients (15%) possessed borderline intelligence, and 10 (29%) had cerebral palsy. Ocular, spinal, and orofacial abnormalities were often present. Detailed summaries of these findings are given in Table I. Although several genetic causes of ACC have been identified, in the vast majority, the etiology is assumed to be multifactorial. In our study, two patients had trisomy 8 mosaicism and 11 (35%) had a family history of developmental disability. A review of the literature on chromosomal abnormalities in acalossal patients revealed 81 additional cases, which are discussed and outlined in Table II.

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We prospectively studied 712 consecutive patients during a 1-year period who presented with amnesia or loss of consciousness after nonpenetrating head trauma and who had a perfect Glasgow Coma Scale score of 15. Of the 67 (9.4%) patients with acute traumatic lesions disclosed by computed tomography (CT) of the head, 2 required neurosurgical intervention and 1 died. Four factors were statistically correlated (P < 0.05) with abnormal CT findings: Older age, white race, signs of basilar skull fracture, and being either a pedestrian hit by a motor vehicle or a victim of an assault. Sex, length of antero- or retrograde amnesia, forward and reverse digit spans, object recall, focal abnormality on the general neurological exam, and subjective complaints were not statistically correlated with CT abnormality. Using step-wise discriminant function analysis, no single item or combination of items could be used to classify 95% of the patients into either the normal or abnormal CT group. Therefore, regardless of age, mechanism of injury, or clinical findings, intracranial lesions cannot be completely excluded clinically on head-trauma patients who have loss of consciousness or amnesia, even if the Glasgow Coma Scale score is 15. However, only two patients (0.3%) required neurosurgical intervention.

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