About Infantile Spasms
Infantile spasms are seizures commonly associated with West syndrome—a severe infantile developmental and epileptic encephalopathy often accompanied by poor developmental and epilepsy outcomes. However, early treatment can improve outcomes. Babies with infantile spasms have epilepsy. There are three cases of infantile spasms per 10,000 live births in the U.S. each year. Greater than 90 percent of these seizures begin between 3 and 12 months of age with peak onset at 6 months. Although they often involve sudden vigorous muscle contractions of the neck, arms, legs and trunk, they can also present as mild contractions of the abdominal muscles or subtle movements of the lower lip, eyes, head or shoulders. The clustering of these seizures, often after waking from sleep, is often the key to diagnosis. Infantile spasms result from a brain abnormality with many different associated conditions that can be structural, metabolic, genetic or of unknown cause. It is not known how such varied conditions lead to infantile spasms.
The Infantile Spasms Program
An integral part of our Level-Four Epilepsy Center, the Infantile Spasms Program at Nationwide Children’s Hospital is a multidisciplinary team consisting of pediatric epileptologists, nurse practitioner, social worker, developmental psychologist and registered nurses. John R. Mytinger, MD, is a specialist in the management of infantile spasms and is the Director of the Infantile Spasms Program.
When it comes to making a timely diagnosis of infantile spasms, caregivers and primary care physicians play a critical role. Sudden onset jerks that can repeat in a series (“cluster”) often cause caregivers to pursue a medical evaluation. Clinicians should consider infantile spasms in any infant with spells, especially if they cluster. A home video of the spells can be very helpful. Your primary care physician may pursue an EEG (electroencephalogram, “brain wave test”) or an urgent neurological evaluation. This neurological evaluation may take place in the clinic or emergency room. If infantile spasms are suspected by the pediatric neurologist, an urgent EEG is performed to confirm the diagnosis. A longer EEG (one or more days) may be needed. If so, it will be performed during a hospital admission.
We have established a standardized management protocol for the diagnosis, treatment and follow-up of infantile spams that is consistent with best available evidence and national expert recommendations. The protocol emphasizes the rapid diagnosis and treatment of infantile spasms, the use of first-line therapy and early changes if the initial treatment is deemed ineffective.
Some treatments require a several day hospital admission whereas other treatments can be started at home. While medications are typically tried first, some children are best treated with brain surgery or the ketogenic diet. The goals of treatment are to:
- Completely stop the infantile spasms
- Improve the abnormal EEG
Although we are not able to achieve these goals in all children, the infantile spasms team will do everything possible to improve your child’s developmental and epilepsy outcome.
If the chosen medication appears to be effective, we will obtain an EEG in 10-14 days after the start of treatment. This initial EEG includes about one hour of recording. Given the importance of capturing sleep, your child should not sleep on the way to the EEG. You may need to adjust your child’s schedule (wake him or her up earlier for example) in order to assure that sleep is obtained. If this EEG appears to be improved, then we may pursue an overnight EEG to assure that the EEG is persistently improved and that there are no subtle infantile spasms. On the other hand, if the chosen medication does not stop the spasms or does not improve the EEG, then a new treatment will be necessary.
Physician Referral Information
The Neurosciences Center at Nationwide Children’s Hospital accepts referrals from primary care providers and specialists from across the U.S. and internationally. To make a referral, there are three options: