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Agenesis of the corpus callosum, infantile spasms, ocular anomalies (Aicardi's syndrome)

Clinical and pathologic findings

From the Ophthalmogenetic Department of the Netherlands Ophthalmic Research Institute, Wilhelmina Gasthuis, Amsterdam; the Department of Ophthalmic Pathology, Erasmus University, Rotterdam; and the Department of Neuropathology, University of Nijmegen, and de Wever Ziekenhuis, Heerlen, The Netherlands.

A patient with Aicardi's syndrome had the complete clinical picture of mental subnormality, convulsions, electroencephalographic disturbances, ocular anomalies, female sex, and agenesis of the corpus callosum. A second patient had the features of the syndrome, with microphthalmia and one depigmented zone. In both cases and in Brihaye's case, no pineal gland was found. Since the epiphysis cerebri is important as a clock for sexual development, its absence may be incompatible with the development of a male fetus. The pathogenesis of the syndrome remains obscure. While an exogenous cause may be at work in some of the cases, the complex and stereotypical character of the symptoms plead for a hereditary origin.

Dr. de Jong's address is Netherlands Ophthalmic Research Institute, Wilhelmina Gasthuis, Eerste Helmersstraat 104, Amsterdam 1013, The Netherlands.

Received for publication December 1, 1975.

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