Do you have a little fear or doubt about your little one's development? Sometimes we get even more worried when we hear new names from doctors, don't we? Well, today we are going to talk about a rare brain condition that you may not have heard of, but is very worth knowing about. This is called Schizencephaly.
What is Schizencephaly?
Simply put, schizencephaly is a brain abnormality that occurs at birth, that is, when a baby comes into this world. Our brain has two parts called cerebral hemispheres , to be precise, the right side and the left side. These are the parts that control our motor skills like speaking and walking, our way of thinking, that is, cognitive functioning , our emotions , hearing , and vision , and many other important things. In schizencephaly, a cleft or hole (cleft) forms in these cerebral hemispheres, that is, in these sides of the brain. This cleft can be on only one side of the brain (`unilateral`), or it can be on both sides (`bilateral`).
Imagine, there is a liquid called cerebrospinal fluid that protects and nourishes our brain. Also, there is a type of tissue called gray matter . This helps control our movement, memory, and emotions. Now, inside that gap, this cerebrospinal fluid and gray matter are filled. However, if these accumulate too much, problems arise, that is, symptoms begin to appear. For example, the baby's development may be delayed , paralysis may occur, or the head size may be small .
What are the main types of schizencephaly?
There are two main types of this condition, Schizencephaly.
- Open-lip schizencephaly: In this case, the cleft extends from the outside of the brain all the way into the ventricles , the chambers inside the brain that are filled with cerebrospinal fluid. This means the cleft is a bit larger and deeper.
- Closed-lip schizencephaly: In this type, the cleft is a little shorter. It does not extend all the way to the ventricles. Sometimes, this type may be asymptomatic.
How common is this condition?
Schizencephaly is actually a very rare condition. It's not something that happens to many people. Statistically, it affects about 1.5 out of every 100,000 babies born in the United States. In the United Kingdom, it affects about 1.48 out of every 100,000 babies. So you can see how rare this is.
What are the symptoms of Schizencephaly?
The symptoms of this condition can vary from person to person, depending on the size and location of the brain lesion. Here are some common symptoms:
- Head size smaller than normal.
- Muscle weakness or loss of strength (hemiparesis). It feels like the limb is paralyzed.
- Muscle stiffness or rigidity (`(spasticity)`).
- Paralysis : This means that part or all of the body is paralyzed.
- Epileptic conditions , that is, seizures .
- An excess of cerebrospinal fluid (CSF) builds up inside the brain. This is called hydrocephalus .
- A change in the position of the eyes , such as strabismus .
In addition, schizencephaly can cause developmental delays in children . This means that these children take a little longer to learn and do things that a child would normally start doing at a certain age.
- Move around on your own, touch things (gross and fine motor skills).
- Speak and communicate with others (speech and language skills).
- Learn and remember new things (cognitive skills).
- Play and get along with others (social and emotional skills).
But remember, some children with the previously mentioned closed-lip schizencephaly may not show any symptoms .
What are the causes of Schizencephaly?
To be honest, the exact cause of Schizencephaly is still unknown . However, research suggests that it may be caused by exposure to certain things during fetal development .
- Some medications , for example, blood thinners like Warfarin.
- Some drugs , for example `(Cocaine)`.
- Some viral infections , for example `(Zika virus)` or `(Cytomegalovirus)`.
- It may also be due to some complications that occur during a test called ``Amniocentesis'' performed during pregnancy.
In addition, genetic changes (mutations) can also affect the way a fetus's brain develops. Genetic changes are changes in our DNA. These can often occur randomly, meaning that no one in the family has had the condition before, but they can occur suddenly. Very rarely, the condition can be passed down from generation to generation.
Who is most at risk for this condition?
If certain things happen during pregnancy, the risk of having a baby with schizencephaly may slightly increase.
- Substance use disorder.
- Developing a viral infection (e.g., Cytomegalovirus or Zika virus).
- Low oxygen levels in the blood (hypoxia).
- Amniocentesis test .
- Experiencing some shock or injury (Trauma) .
- Using the medicine ``(Warfarin)`` prescribed by a doctor.
It is also said that the mother's age, being under 25 , adds some risk to this condition.
Can other conditions occur with schizencephaly?
Yes, sometimes when doctors diagnose schizencephaly, they also look for other conditions. Here are a few examples:
- Cerebral Palsy (`(Cerebral Palsy)`)
- Agenesis of the corpus callosum ( a bridge-like part of the brain that connects the two halves of the brain)
- Septo-optic dysplasia ( a condition that causes vision and hormonal problems)
- Arachnoid cysts ( fluid-filled cysts between the membranes covering the brain)
How to diagnose this disease?
Often, doctors can see signs of schizencephaly during prenatal ultrasound scans after 20 weeks of pregnancy. However, the exact diagnosis is made after the baby is born . This is done using either an MRI (Magnetic Resonance Imaging) scan or a CT (Computed Tomography) scan . These scans can clearly show the size of the brain defect and what type it is.
In addition, your child's doctor may also order a genetic test . This will look for any changes in the DNA that could be causing the condition.
What are the treatments for Schizencephaly?
The main focus of treatment for schizencephaly is to control and reduce symptoms , as it is not a completely curable condition. Treatment options include:
- Giving medications to prevent epileptic seizures .
- The condition called hydrocephalus , which is when fluid builds up in the brain, requires surgery to insert a shunt or tube . This diverts the excess fluid.
- Surgery is sometimes performed to reduce pressure caused by extra fluid in the brain.
- Treatments such as physical therapy , occupational therapy, and/or speech therapy can be very helpful in improving the child's abilities.
- They participate in educational programs supported by the school.
Are there any side effects of the treatment?
It's best to talk to your doctor about the side effects of schizencephaly treatment. There are a variety of side effects that can occur with both surgery and medication. These will vary depending on the type of treatment you or your child receives.
What is the outlook for someone with schizencephaly?
The outlook for someone with schizencephaly varies greatly depending on the size and location of the brain lesions . Some people may have only a small lesion, which can cause no symptoms and lead a normal life. However, others may have a larger lesion, which can cause more symptoms and may require lifelong support .
What about life span?
Schizencephaly does not directly affect your lifespan . That is, it does not shorten your life expectancy. However, some complications of the condition, such as uncontrolled seizures or hydrocephalus, can be life-threatening .
Can this situation be prevented?
In fact, there is no way to always prevent schizencephaly. Because some causes are beyond our control. However, by taking good care of yourself during pregnancy , you can reduce the risk of your baby developing this condition to some extent.
- See the doctor regularly and get checked out.
- Take necessary steps to protect yourself from infections .
- Talk to your doctor about the medications you are currently taking. Some medications are not suitable during pregnancy.
- Completely avoid things that are harmful to the fetus, such as drugs.
- It would also be good if you could meet with a genetic counselor to learn about the risk of a child having a genetic disease.
When should you see a doctor?
If you or your child has any of these symptoms, see a doctor immediately:
- Change in muscle tone (tightness or weakness).
- If you are unable to move a part of your body (such as paralysis).
- If the child is late in reaching developmental milestones appropriate for their age.
Most importantly: If you or your child has an epileptic seizure, immediately call the 1990 Suwaseriya Ambulance Service or go to the nearest hospital.
What questions should I ask my doctor?
When you find out that your child has Schizencephaly, you may have many questions. It's a good idea to ask your doctor things like:
- What type of schizencephaly does my child have?
- How should I support my child?
- What complications should we be especially aware of?
- Does my child need surgery?
- What treatment options do you recommend?
- Are there any side effects of the treatment?
- Are there support groups for parents caring for children like this?
Things to remember in brief
It's normal to feel overwhelmed when you learn about this rare neurological condition, schizencephaly, and whether you, your child, or someone you love has it. Schizencephaly and how it affects you varies depending on the size of the brain's brain. You may have no symptoms at all, or you may need some support throughout your life. The condition doesn't directly affect your lifespan, and it doesn't interfere with your ability to accomplish your daily goals. However, some things can be more challenging than others. Your medical team can help you learn more about the condition and discuss treatment options that can help you feel better. Don't feel alone, and get the help you need.
` Schizencephaly, brain fissure, brain abnormalities, congenital diseases, childhood diseases, developmental delay, epilepsy, hydrocephalus, genetic diseases


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