Infantile spasms, or West syndrome, is a type of childhood epilepsy which typically presents in the first 4 to 8 months of life and is more common in male infants. They are often associated with a serious underlying condition and carry a poor prognosis

Features

• characteristic 'salaam' attacks: flexion of the head, trunk and arms followed by extension of the arms
• this lasts only 1-2 seconds but may be repeated up to 50 times
• progressive mental handicap

Investigation

• the EEG shows hypsarrhythmia in two-thirds of infants
• CT demonstrates diffuse or localised brain disease in 70% (e.g. tuberous sclerosis)

Management

• poor prognosis
• vigabatrin is now considered first-line therapy
• ACTH is also used