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Year : 2006  |  Volume : 54  |  Issue : 1  |  Page : 91--93

Aicardi syndrome: A report of five Indian cases

1 National Neurosciences Centre, Kolkata, India
2 Shishu Sanjivan, Kolkata, India
3 B. R. Singh Hospital, Kolkata, India

Correspondence Address:
T K Banerjee
National Neurosciences Centre Kolkata, Peerless Hospital Campus, 360 Panchasayar, Garia, Kolkata - 700094
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0028-3886.24719

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Five Indian cases of Aicardi syndrome, aged 1-13 years and all with the classic triad of infantile spasm, corpus callosal agenesis and chorioretinal lacunae, were presented. The purpose of this report was to demonstrate the clinical, electroencephalographic (EEG) and radiological spectrum and the treatment outcome of this disorder among the Indians. None of the patient had any family history of similar illness. All had profound psychomotor impairment with no meaningful speech development. There were microphthalmia in 2, optic disc coloboma in 1, interhemispheric cyst in 1, periventricular heterotopia in 2 and thoracolumbar kyphoscoliosis in 2 cases. They all had early onset epilepsy and there were multiple types of seizures. Two cases (40%) had the characteristic dissociated burst-suppression pattern in EEG. Two cases whose antiepileptic medications included vigabatrin had complete control of seizure.


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Online since 20th March '04
Published by Wolters Kluwer - Medknow