West Syndrome in an Infant with Complete Corpus Callosal Agenesis

Abstract

West syndrome is a severe form of epilepsy with onset in infancy and early childhood. The characteristic triad described includes infantile spasms (IS), an abnormal electroencephalogram (EEG) pattern referred to as hypsarrhythmia, and developmental regression. It comprises 2% of childhood epilepsies. Etiologically, West syndrome can be classified into symptomatic with known etiology and cryptogenic with unknown etiology. Hypsarrhythmia is defined as chaotic, asynchronous, disorganized slow background activity with high-voltage spike activity. Burst-suppression pattern is a rarely described pattern of modified hypsarrhythmia, which denotes severe disease. Hereby, we describe the case of a female infant who presented with IS; EEG showed a modified hypsarrhythmia pattern in the form of burst suppression, and magnetic resonance imaging brain showed complete corpus callosal agenesis. She was initiated on injection of adrenocorticotropic hormone and showed marked improvement in epileptic spasms.

Keywords

• adrenocorticotropic hormone
• burst suppression pattern
• corpus callosal agenesis
• modified hypsarrhythmia
• west syndrome