[Clinical and prognostic heterogeneity in Aicardi's syndrome: a description of two cases].
BACKGROUND
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.


METHODS
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.


CONCLUSIONS
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.
Cruz-Velarde
J A
JA
Servicio de Neurología, Hospital Universitario Gregorio Marañón, Madrid, España.
Garzo
C
C
García-Muñoz
S
S
Gil
R
R
Muñoz
L
L
D061085:Agenesis of Corpus Callosum; D002658:Developmental Disabilities / Q000175:diagnosis; D004831:Epilepsies, Myoclonic / Q000175:diagnosis*; D005128:Eye Diseases / Q000175:diagnosis*; D005260:Female; D006801:Humans; D007223:Infant; D008607:Intellectual Disability; D008279:Magnetic Resonance Imaging; D011379:Prognosis; D013036:Spasms, Infantile / Q000175:diagnosis*; D013577:Syndrome
Heterogeneidad clínica y pronóstica en el síndrome de Aicardi: a propósito de dos casos.