West Syndrome: Questions Aplenty- Few Answers
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West syndrome is an infantile-onset epileptic encephalopathy, characterized by epileptic spasms, developmental delay or regression, and hypsarrythmia on the EEG . Though it can result from genetic or metabolic causes, a structural etiology is most commonly identified in Indian children . In addition to the epileptic spasms, affected children are at high risk for developmental disabilities, intellectual and behavioral abnormalities, and long-term epilepsy. Epileptic spasms are unique in that they are resistant to most conventional antiepileptic drugs and hormonal therapy with corticosteroids has proven to be the most effective treatment. The mechanism of action of corticosteroids on epileptic spasms is still unclear, but it is believed that it is due to inhibition of Corticotropin-releasing hormone (CRH) overexpression in these children. Though high-dose Adrenocorticotropic hormone (ACTH) had been the standard of treatment, based on limited evidence, international guidelines...
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