Burst suppression pattern on EEG in West syndrome in an infant with heterozygous variant in the CACNA1A gene

Abstract

West syndrome is a severe epilepsy syndrome characterized by the classical triad of infantile spasms, hypsarrhythmia on electroencephalography (EEG) and psychomotor retardation. The classical hypsarrhythmia pattern on EEG consists of a high amplitude, arrhythmic, disorganized background with multifocal spikes and slow and sharp waves. The burst suppression (BS) pattern on EEG is a less commonly described pattern in West syndrome. Among the genetic causes of West syndrome, mutations in the CACNA1A gene are rarely reported. We hereby report a BS pattern on EEG in an infant with West syndrome with heterozygous variant in the CACNA1A gene.

Keywords

• burst suppression pattern on eeg
• cacna1a gene
• modified hypsarrhythmia
• west syndrome