West syndrome

West syndrome, also called Infantile Epileptic Spasms Syndrome (IESS), is a rare and severe form of epilepsy that starts in infancy. It is marked by infantile spasms, a chaotic brain activity pattern on EEG (hypsarrhythmia), and developmental delays or regression. Key characteristics Infantile spasms: Brief episodes of muscle stiffening or jerking, often in clusters. Movements may be mild twitches or stronger “jackknife” bends of the body. Hypsarrhythmia: Abnormal and disorganized brain activity seen on EEG. Developmental regression: Infants may stop progressing or lose skills like rolling, sitting, or babbling. Symptoms and onset Symptoms usually appear between 3 and 12 months of age. Spasms are brief but frequent, often happening after waking. Causes The condition can be linked to structural brain problems, genetic mutations, metabolic disorders, or infections. Diagnosis EEG is used to detect hypsarrhythmia. MRI can show brain abnormalities. Genetic and laboratory tests may reveal underlying causes. Treatment There is no cure. Treatment aims to stop spasms and support development. Medications: Vigabatrin, ACTH, and corticosteroids are commonly used. Diet therapy: The ketogenic diet may reduce seizures in some children. Surgery: Considered if seizures come from a specific brain area and don’t respond to other treatments. Rehabilitation: Psychomotor therapy helps address developmental delays.