Infantile Spasms: Symptoms, Causes & Treatment

Last updated: 12-06-2020

Read original article here

Infantile Spasms: Symptoms, Causes & Treatment

Infantile Spasms: Symptoms, Causes & Treatment
Page Content
​​​​​​​​Watching your baby's every movement typically brings parents so much joy. But when a baby shows signs and symptoms of a serious neurological condition such as infantile spasms (IS), that joy can quickly turn into concern.
Infantile spasms are little seizures with big consequences. Early recognition leads to better outcomes; waiting to seek treatment can increase your baby's risk of permanent brain injury.
What are infantile spasms?
Infantile spasms, first described with a group of symptoms known as West syndrome, is a form of epilepsy ​ that occurs in 1 in 2,000 children. It typically begins between 2-12 months of age and peaks between 4-8 months of age.
What do infantile spasms look like?
​While these seizures may last only a second or two, they often happen close together with each spasm occurring every 5-10 seconds in a series. During a spasm, the body stiffens suddenly, the back may arch, and the arms, legs, and head may bend forward. However, infantile spasms can sometimes be hard to notice—perhaps only the eyes roll up or there is a small tummy crunch. They are most common just after a baby wakes up and rarely occur during sleep.
Soon after the spasms begin, parents may notice a number of changes in their baby:
Loss of developmental milestones​ previously learned (e.g., rolling over, sitting, crawling, babbling)​​
Loss of social interactions and smiles
Increased fussiness or silence
Don't hesitate to talk with your pediatrician if your child is no longer meeting developmental milestones. Trust your instincts—​​you know your child best!
​Parents and childcare providers, or anyone wanting to educate a friend or relative about infantile spasms: watch this video, below. We see a baby experiencing a cluster of individual spasms. Each spasm is less than 1 second; the child exhibits a look of surprise, a brief stare, and elevation/extension of both arms. In between each spasm, the baby appears to be fine—very typical of infantile spasms.

Contin​uing the Fight for Adelaide
​By Kelly Cervantes
​Infantile spasms...It sounds so innocuous right? Like a shiver or a small jerk - if only. The truth is that infantile spasms is an emergency. Our daughter, Adelaide, was diagnosed with infantile spasms when she was 9 months old. I was out of town when my husband called to tell me that Adelaide was doing a weird repetitive head drop and seemed to be losing consciousness each time it happened. We decided he should take her to the emergency room out of an abundance of caution.
I'll never forget walking into Adelaide's hospital room, having taken a cab straight from the airport, and seeing a shadow of the daughter I had left behind. I'll never forget the month we spent in the hospital trying to gain control of her chaotic brain waves.
​There are a couple powerful frontline treatments that may relieve hypsarrhythmia (the devastating brain wave pattern associated with IS) and even the seizures altogether. The side effects can be terrifying, but they are nothing compared to the damage of IS. In the best-case scenario, babies can get seizure control and ​live nearly normal lives with maybe just a few delays or learning disabilities . Children with the best odds are those developmentally typical prior to IS. Adelaide, unfortunately, was not. She was already diagnosed with a global developmental delay, hypotonia, and generalized epilepsy when we added infantile spasms to the list.
Our fierce warrior passed away five days shy of her fourth birthday from a neurodegenerative disorder after having battled and overcome hypsarrhythmia and infantile spasms three times in her short life. Each time Adelaide regressed to a developmentally fetal state and never fully worked her way back to where she was before the IS struck. One step forward, two steps back.
Hopefully, there is a “true" diagnosis that can point doctors toward the best treatment plan available. Infantile spasms is a diagnosis of a seizure type, but is not a diagnosis in and of itself. IS is a symptom of some larger problem and, like all epilepsies, results from a genetic or acquired condition. Fight for every scan, genetic test and second opinion available--be it through your neurologist or an outside research study.
Infantile spasms is devastating, but there is hope as organizations like CURE Epilepsy​ fund research that could one day stop or prevent infantile spasms from ever wreaking havoc on our babies' brains. Science couldn't catch up to Adelaide, but I promised her that I wouldn't give up the fight.
Above, epilepsy advocate Kelly Cervantes with daughter Adelaide, son Jacks​​on and husband Miguel Cervantes. Adelaide was diagnosed with infantile spasms when their family moved to Chicago where Miguel starred in "Hamilton: An American Musical." Read more about Adelaide's journey and the ongoing fight to end infantile spasms on Kelly's blog Inchstones at  www.kellycervantes.com . Photo credit: Lori Sapio​​
Early diagnosis is key
It is very important that infantile spasms are diagnosed early. If you suspect your baby may be having infantile spasms, talk to your pediatrician right away. Your child may need to be seen by a pediatric neurologist . If you can, try to video any episodes of your baby having a spasm and show this to your child's doctors.
You may be told to go to the emergency room so a pediatric neurologist can examine your child. Your child may have to be admitted to the hospital to have a test called video-electroencephalogram (EEG) monitoring. Video-EEG is needed to verify specific brain-wave patterns during the spasms and to document specific patterns in between the spasms. Pediatric neurologists look for a distinct EEG pattern called hypsarrhythmia. The seizures' appearance and EEG are so distinct that the diagnosis of infantile spasms can be made with certainty in most cases. Determining the cause of the spasms may be more difficult.
Causes of infantile spasms
There are many causes of infantile spasms; almost any brain abnormality or brain injury can cause them. More than 50 genetic/metabolic diseases are associated with infantile spasms, and many patients have other disorders that cause developmental delays (e.g., cerebral palsy , Down syndrome , tuberous sclerosis, etc.) prior to the onset of the spasms.
Determining the cause of infantile spasms is very important, because it affects treatment and prognosis.
Know the signs to ‘STOP’ Infantile Spasms.
Keep this easily remembered acronym in mind: 'STOP' Infantile Spasms.

See the signs: Clusters of sudden, repeated, uncontrolled movements like head bobs or body crunching.
Take a video: Record the symptoms and talk to your doctor immediately.
Obtain diagnosis: Confirm an irregular brain wave pattern with an EEG test.
Prioritize treatment: End spasms to minimize developmental delays
Treatment op​​tions
The American Academy of Neurology and the Child Neurology Society recommend adrenocorticotropic hormone (ACTH) as the first line of therapy for infantile spasms. This medication is given as a shot. The first doses are given in the hospital so your child can be closely watched for any side effects. Since your child will need to continue to get ACTH shots for about 6 weeks, you will be instructed on how to give your child the injections at home. The goals for this medication are to
Completely stop the infantile spasms.
Improve the abnormal EEG.
In some cases, pediatric neurologists prescribe the seizure medication vigabatrin. Both drugs work well, but your child's doctor will talk with you about which medicine may be the better choice for your child.
What is the outlook for children with infantile spasms?
Even if the infantile spasms stop, many children develop other kinds of epilepsy and have intellectual or other developmental disabilities later in life. If the treatment is prompt and successful, the outlook is brighter. Normal development is possible for those who were developing on track before the spasms started.
More Information


Read the rest of this article here