Screening for Infantile Spasms

Image Citation Source by Kourtlyn Lott used under CC by-nd 2.0
West’s syndrome is a childhood epilepsy syndrome. It leads to drug-resistant seizures known as infantile spasms, abnormal brain activity (referred to as hypsarrhythmia), and in most children, intellectual disability. West’s syndrome with infantile spasms usually starts within the first year of life, and is more likely to occur in children who have pre-existing issues within the brain, such as brain tumours from tuberous sclerosis complex or scarring from a brain injury.

One way to screen children at risk for developing West’s Syndrome with infantile spasms is to use electroencephalography (EEG), which records brain waves. Dr. Miguel Cortez and his team at the Hospital for Sick Children believe that hypsarrhythmia begins to appear in an EEG recording many weeks before the infantile spasms start and can be used to predict their onset.

Dr. Cortez and his team will conduct regular EEGs in infants at risk for West’s syndrome (e.g. children with tuberous sclerosis complex) and treat them with an approved drug therapy before the spasms develop. The hope is that if treatment is given at the first signs of hypsarrhythmia, the infantile spasms – and also the intellectual disability – may never occur in these children.

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