Aicardi Syndrome: A case report.
- Author:
Jeong Lim MOON
1
;
Kyung Heui JUNG
;
Sae Yoon KANG
Author Information
1. Department of Rehabilitation Medicine, School of Medicine, The Catholic University of Korea.
- Publication Type:Case Report
- Keywords:
Aicardi syndrome;
Infantile spasm;
Coupus callosum agenesis;
Chorioretinal lacunae
- MeSH:
Agenesis of Corpus Callosum;
Aicardi Syndrome*;
Atrophy;
Brain;
Choroid;
Coloboma;
Corpus Callosum;
Female;
Humans;
Infant;
Infant, Newborn;
Magnetic Resonance Imaging;
Microcephaly;
Periventricular Nodular Heterotopia;
Prognosis;
Quadriplegia;
Seizures;
Spasms, Infantile
- From:Journal of the Korean Academy of Rehabilitation Medicine
2000;24(3):576-581
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
Aicardi syndrome is defined by the clinical triad infantile spasms, agenesis of the corpus callosum, and pathognomonic chorioretinal lacunae. Infantile spasm begins at early infancy and tends to be controlled poorly. The prognosis is poor in the patient with severe developmental delay and intractable seizures being common. We present a case of Aicardi syndrome in the 9-month-old female infant with infantile spasm, spastic tetraplegia and microcephaly. Her brain MRI revealed corpus callosum agenesis, atrophy of left hemisphere and periventricular heterotopia. She showed bilateral choroidal and optic disc coloboma. We report this case with the review of literatures.
