Abstract
A case of 5-month-old female infant with Aicardi's syndrome is presented. The main clinical features were severe developmental retardation and intractable epileptic seizures. Ophthalmoscopic examination revealed pathognomonic chorioretinopathy. Ultrasonic examination of the brain detected agenesis of the corpus callosum, whereas CT showed a coexisting malformation of the brain, i.e. cortical heterotopia of the gray matter. Agenesis of the corpus callosum is an entity well-recognized by sonography. However ultrasonography is an insufficient modality for the visualization of cortical heterotopia which is common to all cases of Aicardi's syndrome. Therefore, in cases of suspected Aicardi's syndrome CT is recommended, as it enables the diagnosis of cortical heterotopia.
Similar content being viewed by others
References
Aicardi J, Lefebre J, Lerique-Koechlin A (1965) A new syndrome: spasm in flexion, callosal agenesis, ocular abnormalities. Electroencephalogr Clin Neurophysiol 19: 609
Aicardi J, Chevrie JJ, Rousselie F (1969) Le syndrome spasme en flexion, agenesie calleuse, anomalies chorio-retinenns. Arch Fr Pediatr 26: 1103
Jong YGY, Delleman JW, Houben M, Manschot WA, de Minjer A, Mol J, Slooff JL (1976) Agenesis of the corpus callosum, infantile spasms, ocular anomalies (Aicardi's syndrome): clinical and pathological findings. Neurology 26: 1152
Dennis J, Bower BD (1972) The Aicardi's syndrome. Dev Med Child Neurol 14: 382
Fariello RG, Chun RWM, Doro JM, Buncic JR, Prichard JS (1977) EEG recognition of Aicardi's syndrome. Arch Neurol 34: 563–566
Bergeron RT (1969) Radiographic demonstration of cortical heterotopia. Acta Radiol (Stockh) 9: 135–139
Phillips HE, Carter AP, Kennedy JL, Rosman NP, O'Connor JF (1978) Aicardi's syndrome. Radiologic manifestations. Radiology 127: 453
Kotlarek F, Schütz E (1985) Diagnostische Maßnahmen bei epileptischen Anfallsformen. Sozialpädiatrie 7: 241
Couture A, Ferran JL (1983) The use of ultrasound in cerebral malformations. Ann Radiol (Paris) 26: 99
Babcock DS (1984) The normal, absent, and abnormal corpus callosum: sonographic findings. Radiology 151: 449
Babcock DS (1986) Sonography of congenital malformations of the brain. Neuroradiology 28: 428
Cao A, Cianetti C, Signorini E, Loi M, Sanna G, De Virgilis S (1977) Agenesis of the corpus callosum, infantile spasms, spastic quadriplegia, microcephaly and severe mental retardation in three siblings. Clin Genet 12: 290
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Bešenski, N., Bošnjak, V., Ligutić, I. et al. Cortical heterotopia in Aicardi's syndrome — CT findings. Pediatr Radiol 18, 391–393 (1988). https://doi.org/10.1007/BF02388043
Accepted:
Issue Date:
DOI: https://doi.org/10.1007/BF02388043