Treatment  
 
 
"There are a number of different strategies that have been used over the years to try to treat these spasms. Treatment includes medications, surgical interventions, such as taking out abnormal pieces of brain that might have developed aberrantly during development,Jong Rho, MD or other non-pharmacologic treatments, such as the ketogenic diet, which is a non-drug form that can be very effective for treating some patients with spasms as well. No single therapeutic intervention has proven to be universally effective, so really the clinicians have to look at all of the treatment options and try to tailor them as much as possible to the particular patient with spasms."
Jong Rho, MD
Associate Director, Child Neurology
Barrow Neurologic Institute

In infantile spasms, the goal of treatment is to eliminate the spasms and hypsarrhythmia as quickly as possible. Awareness and proper identification can result in the selection of appropriate therapy that can improve a patient’s developmental outcomes. Click here to read the AAN (American Academy of Neurology) treatment guidelines on infantile spasms

Physician's Role

Parents often come to their pediatricians with concerns about their infant's development or odd movements; sometimes it is the health professional who first suspects a serious problem.

Once a proper diagnosis is made, treatment often includes the involvement of child neurologists, neurosurgeons, and other professionals including dieticians who specialize in epilepsy care.

Medication

Hormonal therapy: Hormonal therapy for infantile spasms usually consists of intramuscular injections of adrenocorticotropic hormone therapy (ACTH ) or oral prednisone.

Seizure medication: Anti-convulsants are drugs that can stop seizures by reducing abnormal electrical excitability in the brain. Seizure medications with the best evidence for treating infantile spasms include vigabatrin, valproate and topiramate.

Overall, the data supports the first line treatment of adrenocorticotropic hormone and vigabatrin as most effectiveness in treating infantile spasms . Vigabatrin appears to be most effective in treating infantile spasms caused by tuberous sclerosis (a genetic disorder often associated with involvement of brain, skin, heart, and kidneys).

 
Dr Jong Rho, MD "Among child neurologists who deal with patients with infantile spasms, many now consider ACTH or vigabatrin as the two primary drugs of choice, mainly because of the empirical evidence that they work, combined with a number of clinical studies that these drugs are effective against different forms of infantile spasms."
Jong Rho, MD
Associate Director, Child Neurology
Barrow Neurologic Institute

Note: H.P. Acthar® Gel (repository corticotropin injection) has not been approved by the Food and Drug Administration (FDA) for use in infantile spasms.

Diet and Non-Medication Treatment Options

Ketogenic diet: This form of therapy for IS has been around since the 1920s. The ketogenic diet is a high fat, low carbohydrate diet with sufficient protein for growth. Recent reports show that a ketogenic diet may help to control spasms in children who don’t respond to other medical therapies.

High-dose IV immunoglobulin: This form of treatment has been used to treat a number of seizure disorders and there is some evidence that it can be effective in treating cryptogenic (unknown cause) infantile spasms. Because of the limited available data in treating IS, this treatment option is usually considered only after other medical therapies have failed.

Surgery: When medication treatment options fail, cortical resection (removal of the brain abnormality) can potentially control seizures and improve developmental outcomes if a localized area of brain abnormality can be identified and safely removed without causing additional deficit.

 
"If I have a patient with tuberous sclerosis, no question in my mind that the first drug is going to be vigabatrin."
Don Shields, M.D.
Professor of Neurology
and Pediatrics
UCLA School of Medicine