Introduction: Aicardi's syndrome is characterized by infantile spasms, agenesis of the corpus callosum and ocular lesions. Clinically it presents as severe mental retardation, severe limitation of motor development and of language, with a prognosis of survival for only a few months or years. We present two new cases of this uncommon syndrome and describe the heterogeneity of its clinical and prognostic severity.

Clinical cases: Case 1. A ten-month old patient had flexion spasms of the limbs at the age of 4 months, bilateral corioretinal lesions and generalized hypoplasia of the corpus callosum. During the clinical course of the disorder, the epileptic crises were controlled, there was mental retardation, the head was held steady and the baby could sit. Case 2. A nine year old patient had had flexion spasms when aged 2 months, had bilateral retinal lesions and generalized hypoplasia of the corpus callosum. During his clinical course the epileptic crises were controlled, there was severe mental retardation, the patient could pay attention and collaborate, articulate single words, walk on his own and manipulate objects.

Conclusion: Aicardi's syndrome should be considered to be a syndrome in which the clinical findings and prognosis are heterogeneous, as seen from new cases with less clinical and functional limitation than the patients first described.

Introducción El síndrome de Aicardi se caracteriza por espasmos infantiles, agenesia de cuerpo calloso y lesiones oculares características. Se manifiesta clínicamente con intenso retraso mental, grandes limitaciones en el desarrollo motor y del lenguaje, con pronóstico vital y funcional limitado a meses o a los primeros años de vida. Se presentan dos nuevos casos de este infrecuente síndrome, y se expone la heterogeneidad de su gravedad clínica y pronóstica.

Casos clínicos Caso 1. Paciente de 10 meses de edad con espasmos en flexión de extremidades a la edad de 4 meses, lesiones coriorretinianas bilaterales e hipoplasia generalizada de cuerpo calloso. Evolución con crisis epilépticas controladas, retraso psíquico, sostén cefálico y sedestación. Caso 2. Paciente de 9 años, espasmos en flexión a los 2 meses de edad, con lesiones retinianas bilaterales e hipoplasia generalizada de cuerpo calloso. Evolución con crisis epilépticas controladas, intenso retraso mental, atención y colaboración conservadas, articulación de palabras aisladas y marcha independiente con capacidad para manipular objetos.

Conclusión Se debe considerar al síndrome de Aicardi como un síndrome con heterogeneidad clínica y pronóstica a la luz de nuevos casos con menores limitaciones clínicas y funcionales en relación con los primeros pacientes descritos