Infantile spasm: an overview

Abstract

Infantile spasm (IS) is the archetypal catastrophic childhood epilepsy syndrome. It is catastrophic for two reasons: first, it is difficult to control and second, it is strongly associated with mental retardation. In some cases, profound mental retardation is an inevitable consequence of the underlying disease; for example, lissencephaly. In other cases, the developmental outcome may be improved by controlling the seizures. Thus, early diagnosis and treatment is important. IS has a characteristic clinical presentation with flexor spasms beginning in the first year of life. The diagnosis of IS is confirmed by the electroencephalogram (EEG), which typically demonstrated hypsarrhythmia, modified hypsarrhythmia or multifocal spikewave discharges. A normal EEG should raise the possibility of one of the benign infantile seizure disorders such as benign neonatal convulsions, benign infantile familial convulsions or benign infantile myoclonus. Having identified IS clinically and confirmed