Agenesis of the Corpus Callosum

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Abstract
Roentgen criteria for diagnosis of agenesis of the corpus callosum during life were established in 1934 by Davidoff and Dyke and Penfield and Hyndman. Reviews of the literature reveal that intra vitam diagnoses of this anomaly have been made in 47 cases by encephalography. The present study is based on 19 cases of agenesis of the corpus callosum seen at the Neurological Institute between 1933 and 1953. Two patients came to necropsy and confirmed the clinical (roentgen) diagnosis. Initial symptoms manifest by these patients became apparent prior to 3 years of age in 89 percent of the cases. Initial symptoms were focal or Jacksonian seizures in 37%, hydrocephalus in 31.5% and physical and/or mental retardation in 31.5% of the cases. Sixty-three percent of these patients had authenticated major seizures sometime during their illness. Such seizures were focal or Jacksonian in 75% of the cases. Eighty percent of the 15 patients studied by standard psychometric techniques were found to be mentally retarded or defective. Forty-two percent of the patients in this series were hydrocephalic. It has been postulated that the high incidence of focal or Jacksonian seizures in patients with agenesis of the corpus callosum may be due to deficient neopallial commissural fibers which inhibit the spread of the seizures to the opposite hemisphere.

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