Aicardi's Syndrome: Are Corticosteroids Teratogens?

To the Editor.—  Aicardi's syndrome is an easily recognizable entity that is characterized by infantile spasms, agenesis of corpus callosum, chorioretinal lacunae, mental subnormality, vertebral abnormalities, and poor life expectancy.^1-3 It occurs only in females. Its cause eludes us. Influenza⁴ and cytomegalovirus infection⁵ have been implicated. X-chromosomal-dominant inheritance with manifestation in the heterozygote, the gene being male-lethal,¹ or a multiple gene defect,^1,6 have been proposed. I studied a case in which corticosteroids may have been a factor.

Report of a Case.—  A 12-month-old girl was admitted to the hospital with multiple craniofacial and vertebral anomalies. She was born to a gravida 5, para 4, abortus 1, 34-year-old nurse who has multiple sclerosis. At eight weeks, the pregnancy was complicated by a relapse of multiple sclerosis, for which 80 units of adrenocorticotropic hormone was given daily for ten days. The rest of the pregnancy was uneventful.