Agenesis of the Corpus Callosum Associated with Multiple Developmental Anomalies of the Cerebral Arteries

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Abstract
According to Davidoff and Dyke (7), agenesis of the corpus callosum was first described in 1812 by Reil. The angiographic features were described by Mäurer in 1940 (23); in his 33-year-old patient with extrapyramidal syndrome, however, a diagnosis had been previously established by air study. In 1952, Zellweger (30) reported the angiographic findings in 5 cases of agenesis of the corpus callosum and stressed the diagnostic significance of an abnormal course of the anterior cerebral artery and its branches, which was observed in 4 of his 5 cases. Subsequently, other angiographic features have been described by Van Epps (29), Sheldon and Peyman (27), Holman and MacCarty (13), and several others (5, 10, 12, 21, 22, 24, 25). Two cases of agenesis of the corpus callosum have been examined by cerebral angiography. The diagnosis was verified by cerebral pneumography in each instance, but the angiographic features seemed specific enough to permit the correct diagnosis on the basis of the angiograms alone. In one of our cases, multiple developmental anomalies of the cerebral arteries were associated, and in addition there were early suture closure and deformity of the skull. From available information (8, 9, 14, 17, 19) it appears that each of the anomalies of the cerebral arteries found in this case is rare by itself, and the concurrence of the multiple arterial anomalies and agenesis of the corpus callosum prompted us to report the angiographic findings. Case Report A 19-month-old girl was admitted to the clinic because of deformity of the skull. Birth was normal and the early postnatal period uneventful, but, when she was three months old, her mother noticed abnormal flatness of the occiput. There was no history of febrile illness, visual disturbance, or vomiting. There were no feeding problems or sphincter disturbances and she could speak several words. The general physical examination was entirely within normal limits except for a cephalic deformity. The head was broad and short, and the occipital region was abnormally flat. The circumference of the head was not increased above normal and the fontanelles were closed. The patient was mentally retarded, but the neurologic examination revealed no abnormality. The electroencephalogram was interpreted as normal. Plain radiographs of the skull disclosed partial closure of the coronal suture and flattening of the occiput. There was no radiologic evidence of increased intracranial pressure. Pneumoencephalography was performed under general anesthesia (Fig. 1). The lateral ventricles were moderately and symmetrically dilated. The enlargement was particularly marked in the posterior portions of the bodies of the lateral ventricles. The dorsolateral margin of each lateral ventricle was pointed, and the lateral ventricles were separated from each other. The third ventricle was markedly enlarged, and the roof extended upward beyond its usual limits.

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