Have you ever seen your little one suddenly jerk, as if he was struck by lightning? His arms and legs suddenly fold, or he throws himself backwards. Even if this only lasts for a few seconds, if it happens several times in a row, it can be very scary for any parent who sees it. We are going to talk about a condition that is so scary, but it is very important to recognize it quickly. That is West Syndrome.
Simply put, what is West Syndrome?
West Syndrome is a rare form of epilepsy that affects young children, especially those under one year old . It is named after the doctor who first discovered it.
This has several names among doctors. You may hear the name `Infantile Spasms (IS)`. That means ``baby spasms.'' It is also called ``Epileptic Spasms.'' Although there are many names, they all refer to the same medical condition.
What are the symptoms of this condition?
The main feature of this is the jerking that we talked about earlier. These are called `spasms`. These jerks have several special characteristics:
- How the contractions come: The baby suddenly leans forward (like when having a stomachache) or throws back. The arms and legs suddenly stiffen and twitch.
- Time: One stroke lasts only a few seconds.
- Clusters: These contractions do not come once and stop. They come in a continuous, clustered pattern . One contraction is followed by another, and then another, and then another, up to 150 contractions in a cluster. There are babies who have up to 60 such clusters a day.
- Time: You can usually see these movements as soon as the baby wakes up from sleep.
In addition to these tremors, other symptoms may be seen.
| Symptom | Description |
|---|---|
| Constant crying/being angry (Crankiness) | The baby is constantly crying for no reason and is difficult to comfort. |
| Loss of appetite | Reduce or refuse to drink milk. |
| Sleep changes | Sleeping less at night, sleeping more during the day. |
| Growth arrest or regression | This is a very important characteristic. The baby, who used to do things like smile, play, and recognize his mother, gradually stops doing those things. He forgets the things he learned earlier. |
Why does this happen to babies?
West Syndrome is a very rare condition. It affects about 6 out of 10,000 babies. It most often affects babies between the ages of 4 and 8 months. Although the exact cause is not always known, several main causes have been identified.
- Structural brain problems: Any abnormalities that occur during the development or formation of the brain.
- Genetic changes: This condition can be caused by certain changes in the baby's genes.
- Brain damage: Due to lack of oxygen to the brain during birth or at some other time, such as an accident.
- Metabolic disorders: Due to some problems with the body's chemical processes.
- Brain infections: Infectious conditions affecting the brain.
- Tuberous Sclerosis Complex: A genetic condition in which noncancerous tumors develop in the body and brain.
How is the diagnosis made?
If you notice your baby having this kind of seizure, you should see a pediatrician as soon as possible .
Very important: Before you go to see your doctor, it is very helpful if you can videotape your baby's contractions. This can often be mistaken for colic, so it is a great help for the doctor to see the video to help diagnose the condition.
The doctor mainly refers to these tests:
- EEG (Electroencephalogram): This is the most important test. Simply put, it records the electrical activity in the brain as a pattern. A baby with West Syndrome will show a very irregular pattern called `Hypsarrhythmia` on the EEG. This is the main clue to the diagnosis of this condition.
- MRI or CT scan: These scans help to see if there are any problems, damage, or abnormalities in the structure of the brain.
- Blood, urine, and cerebrospinal fluid (CSF) tests: These tests help determine whether the cause of the tremor is a genetic or metabolic problem.
What are the treatments for this?
It is very important to start treatment as soon as possible after West Syndrome is diagnosed, because controlling the seizure helps minimize damage to the baby's brain development.
Medication
The most commonly used treatment for this is medication.
- ACTH: This is a hormone. It is given as an injection. It is very effective in controlling the heartbeat.
- Vigabatrin (Sabril): This is also a medication for epilepsy. It is especially effective in babies who have West Syndrome, a condition caused by Tuberous Sclerosis.
- Steroids: Steroids like `Prednisone` are also used.
These medications can have side effects such as decreased immunity, increased blood pressure, and vision problems. Therefore, the doctor always examines the baby and treats him/her based on the benefits and risks.
Ketogenic Diet
For some babies, if medication is not enough to control their diarrhea, the doctor may suggest this special diet. This is a high-fat, very low-carbohydrate diet.
Warning: Never try to do this at home alone. This is usually started in the hospital, with the help of a doctor and a nutritionist/dietitian.
Surgery
If the MRI scan clearly shows that the stroke is caused by a specific lesion in the brain, the doctor may suggest surgical removal of that area. This is not something everyone can do. It is decided by a medical team.
What can you say about the future? (Outlook)
While this is a difficult question to ask, it is important to know the truth. The future of West Syndrome will be determined by several key factors.
- Cause: What is the cause of the seizure? If it is caused by a serious brain injury, the results may be poor. If it is caused without an obvious cause (idiopathic), the results are relatively good.
- Time to start treatment: If treatment can be started within a month of diagnosis, the baby's development is much more likely to return to normal.
- Response to treatment: How quickly the seizure can be controlled.
These seizures usually stop by the age of 4. However, many children may later develop other types of epilepsy .
Many children have some level of learning disability or mental development problems. However, with the best treatment available early on, about 20%-25% of babies can live a normal life with normal intelligence. Therefore, taking early action is important.
Take-Home Message
- If you notice a sudden, clustered twitch in your little one (especially between 3-12 months), don't ignore it.
- If possible, videotape the contractions. It will be very helpful to the doctor.
- See a pediatrician immediately. Wasting time can increase the damage to the baby's brain.
- West Syndrome is a treatable condition. Early treatment can control the condition and give the baby the best chance for future development.
- This journey is difficult. But you are not alone. Talk to your doctor regularly and get the support you need.
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