Does your little one suddenly start to twitch? Is this what we mean by Infantile Spasms? Let's find out!

Have you ever noticed your little one suddenly twitching and stiffening up? This probably happens several times a day. You may be wondering if this is normal or if it is a specific condition that affects babies. That is what we are going to talk about today. This condition is called `(Infantile Spasms)`. Let's see what it is, why it happens, what are the symptoms, how to detect it, and what are the treatments.

What is this (Infantile Spasms)?

Simply put, `(Infantile Spasms)` is a type of epilepsy that occurs in babies under one year old, that is, under 12 months old . Epilepsy is a condition (`neurological disorders`) caused by an abnormal problem with the electrical activity of your baby's brain. When these `(Infantile Spasms)` occur, the baby's body, especially the abdomen, head, neck, arms, and legs, suddenly tighten and seem to jerk.

These contractions last for a very short time, about one or two seconds . But they can occur in a series, meaning one contraction comes and then another after 5 or 10 seconds, or in a `cluster`. Imagine a baby kicking several times in a row. Most of the time, these contractions come after babies wake up from sleep .

The sad thing is, many babies who suffer from this condition may already have developmental delays, or they may develop them later. That is, the baby is late in doing things that are appropriate for their age.

What is the difference between (Infantile Spasms) and (West Syndrome)?

Now you may be wondering what this ``West Syndrome'' is. Sometimes doctors also call it this. ``West Syndrome'' is a collection of several symptoms. That is:

• We have this ``Infantile Spasms'' that we're talking about.
• There is an abnormal pattern in the brain's electrical waveform called `(Hypsarrhythmia)`. (This is detected by an `(EEG)` test, we will talk about it later).
• There are developmental delays or the loss of previously existing abilities (`developmental regression`).

However, not every baby with `(Infantile Spasms)` necessarily has `(West Syndrome).` That is, it is possible to have `(Infantile Spasms)` without any developmental delays.

What is the difference between spasms and seizures?

Yes, this is an important question. `(Infantile Spasms)` is actually a type of seizure. A ``Seizure`` is a sudden, uncontrolled electrical activity between brain cells. This can cause temporary muscle stiffness, changes in movement, behavior, and unusual changes in sensations. Not all seizures are the same.

Compared to the tonic-clonic or grand mal seizures that we usually think of when we think of seizures, these infantile spasms are much shorter. Infantile spasms last about one or two seconds, but they come in clusters. Other types of seizures can last anywhere from 30 seconds to about two minutes.

What is the difference between Infantile Spasms and Startle Reflex?

This is also something that confuses many parents, because the way ``Infantile Spasms'' appear may seem similar to the way babies usually twitch.

Babies usually startle (also called the Moro reflex) when they hear a loud noise or when they are suddenly moved. The baby then throws their head back, spreads their arms and legs wide, and turns their head upwards. Sometimes they may cry, and then pull their arms and legs back to their body.

But in `(Infantile Spasms)` there is no such external cause . That is, the spasms do not occur because of a sound or something else. The other is that `(Infantile Spasms)` come in clusters . That is, one comes, then another, and another. A normal spasm does not last that long. Also, when the spasm occurs, the baby's eyes may roll up, or it may look like he is frowning . These are less common in normal spasms.

At what age do infantile spasms start?

According to doctors, 90% of `(Infantile Spasms)` are diagnosed in babies under 12 months, that is, in babies under one year old. It is most common between the ages of four and seven months . However, sometimes children over 12 months can also have this type of spasm. In that case, it is classified as `(Epileptic Spasms)`.

How common is this condition?

This is not a very common condition, but it is not non-existent either. It is estimated that approximately one in 2,000 to 4,000 babies may be affected by this condition (Infantile Spasms).

What are the symptoms of Infantile Spasms?

When these contractions come, your baby's body suddenly stiffens, or jerks. It only lasts a few seconds. The baby may arch their back, bend their arms, legs, and head forward. As we mentioned earlier, these contractions come in a cluster, one after the other, with a 5-10 second break.

After a kick or a few kicks, the baby may become agitated and cry. But that doesn't always happen.

Sometimes these `(Infantile Spasms)` come on very subtly . We may not even recognize them easily. Such subtle symptoms are:

• Rolling your eyes.
• Abdominal pain.
• Chin movements.
• Grimacing.
• Head nodding.

If the tremor is caused by an abnormality in the baby's brain, it may often affect only one side of the body. Or the head or eyes may appear to be pulled to one side.

The most important thing is that this kick occurs most often after the baby wakes up from sleep . It occurs very rarely while the baby is sleeping.

Not long after these kicks start, you will be able to notice other changes in your baby:

• Developmental milestones such as rolling over, sitting up, kneeling, and babbling are lost.
• Social interactions with others decrease, and laughter decreases .
• Always nervous, or always silent.

If possible, videotape your baby when they have these spasms. You can then show them to your pediatrician. It is important to recognize these ``Infantile Spasms'' early. If you think your baby is having these spasms, see a doctor as soon as possible.

How often can infantile spasms occur?

A baby can have up to 100 seizures a day. But each baby is affected differently. So, if you notice your baby having seizures, even if it's just once or twice a day, it's best to talk to a doctor as soon as possible .

What are the causes of Infantile Spasms?

There are many possible causes of infantile spasms. Scientists have identified more than 200 health conditions that can cause them. However, in about a third of cases, doctors cannot find the exact cause.

This stroke may be caused by an abnormality in a small part of the baby's brain, or it may be caused by a problem throughout the brain.

Some of the main reasons are:

• Brain damage or infection: This stroke can be caused by any type of brain injury. The main causes are `(Hypoxic-ischemic encephalopathy)` (which means the brain does not get enough oxygen or blood for a long time), `(Meningitis)` (meningitis), and `(Perinatal stroke)` (which means a stroke that occurs during the middle of the baby's pregnancy and within the first month after birth).
• Brain Development Problems: This can be caused by a number of abnormalities in the central nervous system (brain and spinal cord) that occur while the baby is developing in the womb. For example, conditions like `(Focal cortical dysplasia)`, `(Lissencephaly)`, `(Holoprosencephaly)` and `(Aicardi syndrome)`. It can also be caused by abnormal blood vessels in the baby's brain (`(Arteriovenous malformations)`).
• Gene mutations: Scientists are now linking many gene mutations to infantile spasms. Some examples include Trisomy 21 (Down syndrome), Tuberous sclerosis, Miller-Dieker syndrome, and CDKL5 deficiency disorder.
• Metabolic conditions: Inborn errors of metabolism (IEM) can also cause this condition. These are genetic conditions that block the metabolic pathways that break down the food we eat and produce energy. Very rarely, vitamin B6 deficiency can also cause this condition.

How is Infantile Spasms diagnosed?

Your baby's doctor will ask you about your baby's symptoms and medical history, and will also perform a physical exam.

Your baby may then be referred to a pediatric neurologist or epileptologist. They will perform an electroencephalogram (EEG) to confirm whether the baby is having infantile spasms. They may also do other tests to find the cause.

Since there can be hundreds of causes for `Infantile Spasms`, it can be a bit difficult for doctors to find the cause.

What are the diagnostic tests?

The doctor mainly performs an ``Electroencephalogram - EEG'' test to confirm whether ``Infantile Spasms'' are present.

An EEG measures and records the electrical signals in your baby's brain. When an EEG is performed, a technician places small metal plates (electrodes) on the baby's scalp. These electrodes are connected to a machine. The machine gives the doctor information about the baby's brain activity. It is best if the baby has a seizure during the EEG and the time between seizures can be recorded. Doctors look for a specific EEG pattern called hypsarrhythmia. This pattern is very specific. This is why it is often easy for doctors to accurately diagnose infantile spasms.

The doctor may order further tests to find the cause of this tremor:

• Magnetic resonance imaging (MRI) scan of the brain: An MRI scan is a painless test that can make very clear pictures of the tissues and structures in your baby's brain. An MRI uses a large magnet, radio waves, and a computer to create these detailed pictures. It does not use X-rays. An MRI can help your doctor find out what's causing your baby's seizures.
• Genetic and metabolic tests: If your baby's MRI is normal, your doctor will often do other tests, such as genetic and metabolic tests, to try to find the cause of the seizures. These tests usually require a sample of blood, urine, or spinal fluid.

How are Infantile Spasms treated?

There are two main goals of treatment. One is to stop or control the stroke . The other is to treat the cause of the stroke , if possible.

The main treatments given to stop or manage seizures are:

• Adrenocorticotropic hormone (ACTH): ACTH is a hormone produced by your pituitary gland. It plays a major role in how your body responds to stress. When ACTH is released, the adrenal glands are stimulated to produce the "stress hormone" cortisol. ACTH is a synthetic hormone used to treat seizures. It is given as an injection. The first few doses are given in the hospital. The doctor will then watch you closely for any possible side effects. Since ACTH injections will continue for about six weeks, your doctor will instruct you on how to give your baby the injection at home.
• Oral prednisolone: ​​This medication is a synthetic form of a corticosteroid hormone produced by the adrenal glands. It works well to treat infantile spasms. It can be used when ACTH is difficult to obtain.
• Vigabatrin (`Vigabatrin - Sabril®`): This is an anticonvulsant medication. It is given by mouth as a pill or liquid.

If your baby does not respond to these hormonal treatments or Vigabatrin, there are other treatment options:

• The ketogenic diet.
• Other anticonvulsant medications.
• If the cause of `Infantile Spasms` is a problem in one place in the brain (`focalized lesion`), epilepsy surgery is recommended.

Infantile Spasms can sometimes be difficult to treat. Some children may need to repeat a course of treatment or a second course of treatment. Throughout the treatment, the baby's brain activity may need to be monitored by repeated EEG tests to see if the treatment is working.

If the doctor can find the cause of the baby's fussiness, they should treat that cause if possible. This may include surgery or targeted therapy for an underlying genetic or metabolic condition.

Developmental delays and regression are common with Infantile Spasms. The baby's medical team will suggest therapies that will help with development as much as possible.

What are the side effects of infantile spasms treatment?

There are several possible side effects of adrenocorticotropic hormone (ACTH) treatment:

• High blood pressure (Hypertension).
• Weakening of the immune system (`(Immunosuppression)`).
• Swelling (often in the face).
• Restlessness and irritability.
• Stomach and/or intestinal ulcers.
• Excretion of glucose in the urine.

Possible side effects of `Vigabatrin` treatment:

• Irreversible narrowing of the field of vision.
• Anger.

Because of these side effects, your baby's doctor will talk to you to determine the best treatment option. They will also monitor your baby closely to see if these side effects occur.

What is the prognosis of Infantile Spasms?

The future of this condition, or the `prognosis`, depends on several factors:

• The cause of `(Infantile Spasms)`.
• Can the cause be treated?
• How quickly the baby was diagnosed and treatment started.
• Whether the baby has reached developmental milestones before the kicks start.

About 50% of children with infantile spasms may have long-term developmental and neurological problems. Even if the spasms stop, many children may develop other types of seizures and epilepsy.

However, if diagnosed and treated quickly and successfully, the overall outlook can be good . Children who met developmental milestones before the onset of seizures can develop normally.

How do I take care of my child with infantile spasms?

If your baby has ``Infantile Spasms'', you should follow the doctor's instructions to help him:

• Give all medications as prescribed.
• Refer to developmental assessments and therapies.
• Go to all follow-up medical visits.

Infantile Spasms can cause cognitive, neurologic, and/or sensory problems. Many children with Infantile Spasms have developmental problems and may need help with daily activities throughout their lives.

Your baby's medical team can answer your questions and provide you with support. They may also be able to direct you to support groups in your area or online.

When should I see my child's doctor about infantile spasms?

If your child has been diagnosed with Infantile Spasms, they will need to see their medical team regularly to make sure the treatment is working. They will also need to have electroencephalogram (EEG) tests to monitor brain activity throughout the treatment.

If you think your baby has Infantile Spasms, but have not yet been diagnosed, it is very important to see a doctor as soon as possible .

It's normal to feel worried when your baby isn't acting normally or isn't meeting developmental milestones. You know your baby best . If you think your baby is having a spasm - a sudden, stiffening, twitching sensation, often in clusters - talk to a doctor as soon as possible. If possible, take a video of the spasm. You can show it to the doctor. Early recognition of ``Infantile Spasms'' can lead to better outcomes .

Things we should remember from this story (Take-Home Message)

Okay, so here are a few things to keep in mind from what we've talked about:

• Infantile Spasms is a type of severe epilepsy that occurs in babies under 12 months of age.
• If your baby suddenly twitches and stiffens, especially in clusters after waking up , don't take it lightly .
• This is different from the baby's normal startle reflex.
• If possible, videotape the contractions and show them to the doctor .
• Early diagnosis and treatment can have a major impact on the baby's future development.
• If you notice any changes in your baby's behavior, smile, or development, talk to your doctor about that as well.
• This can be a very stressful experience for both parents. So, while following the doctor's instructions exactly, get support for yourself. Don't worry, the doctors will help you.

` Infantile Spasms, epilepsy, baby twitching, convulsions, West Syndrome, infantile epilepsy, EEG, MRI