Infantile Spasms Treatment

 
 
 
Larry Brown "The Child Neurology Foundation is very pleased that the FDA has approved Acthar as a treatment for infantile spasms," said Lawrence W. Brown, MD, president of the Child Neurology Foundation, associate professor of neurology and pediatrics and director of the pediatric neuropsychiatry program at Children's Hospital of Philadelphia. "This is good news for children with this rare form of childhood epilepsy, as well as for parents and families. The FDA approval will also help standardize care for IS by providing specific guidelines for physicians on prescribing Acthar, the drug that has been the most widely-accepted initial approach for IS for more than 50 years."
Lawrence Brown, MD
Associate Professor of Neurology and Pediatrics
Children's Hospital of Philadelphia
President of the Child Neurology Foundation

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

Click here to read the CNS (Child Neurology Society) treatment guideline for infantile spasms

The American Academy of Neurology and Child Neurology Society specify that successful treatment must stop both spasms and hypsarrhythmia.

Physician's Role in Infantile Spasms

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.

FDA Approved Infantile Spasms Medication

There is no consensus on the best initial approach for infantile spasms. Many experts recommend a short course of hormonal therapy (ACTHar) but others use oral antiepileptic drugs (vigabatrin or topiramate) or oral steroids (prednisolone). H.P. ACTHar® Gel and Sabril® vigabatrin are the only FDA approved treatments for infantile spasms.

Hormonal Therapy:

ACTH (Acthar)
Acthar has a direct effect on the brain and may work by helping the body produce natural hormones with the goal of providing rapid resolution of spasms with clinically significant improvement in the chaotic EEG pattern (hypsarrhythmia) often times within days to weeks at approved dose.

Acthar is proven to:
  • Stop spasms
  • Stop hypsarrhythmia
In one clinical study, 87% of children had no spasms and no hypsarrhythmia by week 2. Some parents reported that their children's spasms disappeared as early as day 1 following the start of treatment. On average, children responded to treatment at day 3.

How is Acthar given?
Acthar is given by an intramuscular injection (into the muscle). Your doctor or nurse will provide detailed instructions about how and when to give Acthar to your child.

Potential Acthar side effects?
Potential side effects include infection, hypertension, irritability, and changes in appetite.

When was Acthar FDA approved?
Acthar was approved by the FDA in October 2010 for the treatment of infantile spasms. For more information about Acthar and assistance with obtaining Acthar, go to www.acthar.com or call 1-888-435-2284.

Anticonvulsant Therapy:

Vigabatrin (Sabril)
Sabril is a prescription medicine used to treat babies 1 month to 2 years old who have infantile spasms. Sabril works by increasing the availability of the main inhibitory neurotransmitter in the brain. Vigabatrin is especially effective in children with tuberous sclerosis.

How is Sabril given?
Sabril comes in a powder form that gets mixed with water. Sabril is then taken by mouth. Your doctor will have instructions on dosing how to mix it.

Potential Sabril side effects?
While relatively safe and effective, there is a risk of permanent vision loss when taking Sabril. Because of this risk, Sabril is used in babies with infantile spasms only when the possible benefits of Sabril are more important than the risks. The most common side effects include sleepiness, ear infection and irritability. When was Sabril FDA approved?

Sabril was approved for use in the USA by the FDA in 2009 for the treatment of infantile spasms. For more information about Sabril and assistance with obtaining Sabril, go to www.Sabril.net or call 1-888-457-4273.

How do I know when Infantile Spasms treatment is working?

Successful treatment must stop both the spasms and hypsarrhythmia as explained here by Dr. W. (Donald) Shields, incoming president of the Child Neurology Foundation.

"I think the concept is clear that treatment of infantile spasms requires both resolution of spasms and resolution of the encephalopathic EEG, however, some practitioners who don't commonly treat IS patients may not be fully aware of this. There is a reference to that in the practice parameter for infantile spasms from the AAN and CNS. It should be noted that Hypsarrhythmia or modified hypsarrhythmia occurs in most infantile spasms patients but not all. Some have other patterns but all indicate major problems, hence my reference to "encephalopathic EEG". All experts in the treatment of infantile spasms agree that the diagnosis requires confirmation by EEG and successful treatment has to be confirmed by EEG if it appears that the clinical spasms have resolved."

~ Dr. W. (Donald) Shields

Diet and Non-Medication Treatment for Infantile Spasms

Ketogenic diet: This form of therapy for infantile spasms 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 infantile spasms, 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.

 
"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
Head, Division of Pediatric Neurology
Alberta Children's Hospital

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 Emeritus of Neurology and Pediatrics
David Geffen School of Medicine at UCLA