Neurology India, Vol. 58, No. 2, March-April, 2010, pp. 322-323
Letter To Editor
Infantile spasms associated with lissencephaly pachygyria in a female twin
Roshan Koul1, Rajeev Jain2, Amna Alfutais1
1 Department of child health (Neurology), College of Medicine and Health Sciences, Muscat, Oman
Correspondence Address: Roshan Koul, Department of child health (Neurology), College of Medicine and Health Sciences, Muscat, Oman, firstname.lastname@example.org, email@example.com
Date of Acceptance: 30-Nov-2009
Code Number: ni10083
Infantile spasms are one of the common and catastrophic seizure disorder in infancy. The common age group is between three months and one year. Psychomotor delay is seen often in these children, related to the underlying brain insult.  Symptomatic infantile spasms is the most common type. Brain malformations, are often associated with symptomatic infantile spasms.  Association of lissencephaly and infantile spasms in a twin is very rare. This has been reported once only previously.  We report a female twin with lissencephaly-pachygyria, who presented with infantile spasms.
The salient clinical features of the twins are given in [Table - 1]. In family there was no history of seizures or any neurologic disorder. The parents were consanguineous.
Infantile spasms are the drug resistant seizures of the infancy. Of the etiology, symptomatic group accounts for the majority of infantile spasms. Several underlying disorders have been associated with the infantile spasms. Any type of neuronal migration anomaly can be associated with seizures.  Lissencephaly may be one of the anomalies. The presentation of lissencephaly in a twin with infantile spasms is very rare. Only one study of a twin with infantile spasms and lissencephaly was reported from Japan.  The twins had equinovarus foot deformity, microcephaly, infantile spasms and severe psychomotor delay. Neuroimaging had simplified gyral pattern in bilateral frontotemporal areas, and a thin corpus callosum in both. The twins described in the present report had identical lissencephaly-pachygyria [Figure - 1],[Figure - 2], [Figure - 3] and [Figure - 4]. Mild or minimal brain malformations may be missed on neuroimaging in infants with infantile spasms.  In a series of six infants with infantile spasms, three infants were considered to be idiopathic as the neuroimaging was essentially normal. However autopsy revealed heterotopias, cortical dyslamination and other malformations in all the six infants. Vigabatrin is used mainly in children with infantile spasms associated with tuberous sclerosis and the response is usually good.  This report suggests possible another condition (Lissencephaly-pacchygyria in association with infantile spasms), wherein vigabatrin could be useful and effective in controlling the spasms.
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