“The expeditious diagnosis and treatment of infantile spasms is essential. To the extent that ongoing infantile spasms and hypsarrhythmia pose a significant threat to long‐term development, the greatest harm is believed to be sustained early …”*

Because infantile spasms can be catastrophic to development, resection or disconnection surgery should be considered as soon as possible for children with focal, including unilateral or unihemispheric, brain malformations.

If your child has failed frontline medical treatments (vigabatrin (Sabril) and adrenocorticotropphic hormone (ACTH) or prednisolone) – do not delay! It is time for a surgical evaluation.

Referral for a surgical evaluation should occur when:

Your child has failed first-line treatments

No longer considered a last resort, epilepsy surgery is a viable treatment option to stop seizures when the child’s infantile spasms are considered drug-resistant (also known as intractable or refractory).

The most recent practice guidelines from the American Academy of Neurology and the Child Neurology Society for the medical treatment of infantile spasms state that adrenocorticotropic hormone (ACTH, sold as Acthar gel) and Vigabatrin (also sold as Sabril) are the first line drugs for infantile spasms. There is also some evidence that high-dose oral prednisolone can be an effective treatment as well.

If a child has failed these drug combinations, the infantile spasms are considered drug resistant. To understand the meaning of drug resistance, we explain it in detail here.

One third of children presenting with infantile spasms before 36 months of age will be drug-resistant – and the largest group are children with infantile spasms. Children with focal or hemispheric lesions or malformations, such as hemimegalencephaly and cortical dysplasia (the children most often considered for surgery) also carry a high risk of drug resistance.

Your child has a focal cortical abnormality

The International League Against Epilepsy recommends a surgical evaluation for that infants with drug resistant epilepsy who have focal-onset seizures – particularly those with a unilateral structural brain abnormality such as hemimegalencephaly, or focal cortical abnormalities such as the cortical dysplasias, tuberous sclerosis, and porencephaly. A presurgical evaluation will attempt to map the cortical abnormality so that the surgeon knows what part of the brain to remove.

If the child does not have a focal abnormality, corpus callosotomy is also an option. In a recent research study of 56 children who had corpus callosotomy to stop infantile spasms, almost 43% were free of infantile spasms after surgery.

Your child does not have a metabolic or degenerative brain condition

Children with degenerative brain conditions or metabolic disorders which cause seizures typically are not candidates for resective epilepsy surgery.

Your child exhibits delay or developmental arrest

In order to be a candidate for surgery, your child must be progressing very slowly or development has “frozen in time”. Most children with drug-resistant infantile spasms exhibit significant developmental delay or development arrest.

There is no unacceptable loss of function if surgery is selected

Very difficult decisions must be made – and fast – for parents considering brain surgery to stop their child’s infantile spasms. The most sobering decision to make is how much function are you willing to lose in exchange for stopping the infantile spams?

When considering surgery, remember that ongoing infantile spasms will cause serious developmental regression or arrest and, in many cases, death.

The sooner the better

Research shows that surgery should be considered as soon as possible rather than waiting months or years. This is because seizures, especially in infancy, can be catastrophic – this means that they are strongly associated with intellectual disability(formerly known as mental retardation) and poor developmental outcome.

Infantile spasms are associated with an almost four-point IQ drop for every week they continue.

If your child has uncontrolled infantile spasms, you are already aware that your child will probably always face physical, developmental, and/or behavioral challenges regardless of the treatments that you choose.

Moderate or severe learning difficulties may be present in 70–90% of children with infantile spasms. Autism is present in up to 30% of children with epilepsy, especially in children who had seizures in the first two years of life.

Sadly, the premature death rate is reported between 5 – 33% in children with infantile spasms.

It is unlikely that your child will “grow out” of infantile spasms

You may be wondering if you should wait and see if your child’s infantile spasms go away on their own. It is true that the spasm seizure type stops by three years of age in approximately one-half of children with infantile spasms and rarely continues after age five; however, 50 to 90 percent of children with infantile spasms will go on to develop other seizure types.

In general, children with infantile spasms caused by stroke, brain malformations, or other known cause more likely to develop other seizure types than those with infantile spasms with no known cause.

Approximately 27 to 50 percent of patients with infantile spasms develop a severe form of epilepsy known as Lennox-Gastaut syndrome.

Reading about the long-term consequences of infantile spasms is disheartening for any parent. However, patients with infantile spasms who are also epilepsy surgery candidates are offered a unique opportunity to permanently stop or reduce spasms and future seizure types and lower the number of anti-epileptic medications that they need to take. Seizure and medication reduction can lead to improved development and behavior that can change your child’s and your family’s quality of life for the better.

Epilepsy Surgery for Infantile Spasms

This special webinar in our series “Let Talk About Epilepsy Surgery” introduces you to epilepsy surgery to stop infantile spasms. Our Executive Director Monika Jones is joined by Dr. Aria Fallah, Director of the Pediatric Neurosurgery Program and Dr. Shaun Hussain, Director of the Infantile Spasms Program, at Mattel Children’s Hospital (UCLA) who provide an overview of infantile spasms as well the criteria for resection surgery, corpus callosotomy, and vagus nerve stimulation.

Infantile spasms are an emergency

This public service announcement produced on behalf of the Infantile Spasms Action Network as part its Infantile Spasms Awareness Week’s 2018 campaign. It sends a clear and sober message to parents and clinical providers that infantile spasms should be considered a medical emergency, requiring immediate diagnosis and treatment.

Infantile spasms and hemispherectomy surgery

W. Donald Shields, M.D., ret’d, Emeritus Professor of Neurology and Pediatrics, David Geffen School of Medicine at the University of California at Los Angeles, discusses infantile spasms, hemispherectomy, as well as the underlying etiologies that often require removing or disconnecting half the brain to stop catastrophic epilepsy. This segment provides an excellent introduction to infantile spasms, their catastrophic consequences if unabated, and hemispherectomy surgery to stop them.

Evidence-Based Research

Infantilespasmsproject.org: The Infantile Spasms Project is a program of the Division of Pediatric Neurology and Department of Pediatrics at the David Geffen School of Medicine and Mattel Children’s Hospital at the University of California, Los Angeles (UCLA). The information presented is intended as a straightforward and organized guide to parents and caregivers of children with infantile spasms, as well as health care professionals.

Child Neurology Foundation: The Child Neurology Foundation connects partners from all areas of the child neurology community so those navigating the journey of disease diagnosis, management, and care have the ongoing support of those dedicated to treatments and cures. Their website contains accurate, research-based information about infantile spasms and treatment, and they provide grants to families living with infantile spasms.

PubMed: PubMed is a service of the US National Library of Medicine® that: Provides free access to MEDLINE®, the NLM® database of indexed citations and abstracts to medical journal articles.

UpToDate: UpToDate® is an evidence-based, physician-authored resource for physicians and patients. Some content is subscription-based while others are available for a fee.

International League Against Epilepsy

The ILAE was founded in 1909 and has more than 100 chapters worldwide. It is an association of physicians and other health professionals working toward a world where no person’s life is limited by epilepsy. ILAE’s mission is to ensure that health professionals, patients, and their care providers, governments, and the public world-wide have the educational and research resources that are essential in understanding, diagnosing and treating persons with epilepsy.

Connect with other parents

There are several websites and social media groups created by parents, for parents, that focus on infantile spasms and brain surgery, including:

Sources

*Hussain, S.A. (2018), Treatment of infantile spasms. Epilepsia Open, 3: 143-154.

Pellock JM, Hrachovy R, Shinnar S. Infantile spasms: a U.S. consensus report. Epilepsia. 2010 Oct;51(10):2175-89.

Shields WD. Infantile spasms: little seizures, BIG consequences. Epilepsy Curr. 2006;6(3):63-69.