On last Thursday’s episode of Grey’s Anatomy (titled “Poker Face”), new mom Mary has a brain tumor called “butterfly tumor” (or Astrocytoma) and only 6 months left to live. Meredith thinks differently and tries to change the course of Mary’s disease.

Danger Level: High

What is it?

Astrocytoma is a type of brain tumor which tends to spread inside the brain ignoring anatomical borders. That causes it to be present on both sides of the brain (and sometimes look life a butterfly – hence the name).

Who gets it?

Astrocytomas are rare – in the United States, every year there are 5.4 new cases per 100,000 people. They are a little more common in men, and most cases appear around ages 20-45.

What causes it?

Astrocytes are star-shaped brain cells located between nerve cells (astro=star, cyte=cell). They are actually part of a larger group of cells in the brain called glia cells – which support the nerve cells and glue them together.

Photo by Neurorocker

When astrocytes start dividing without control, they create a tumor called astrocytoma. These tumors come in different grades (1 to 4) – low grade tumors are slow-growing while high-grade tumors can be very aggressive (on the Grey’s Anatomy episode, Mary had a grade-4 tumor which is the most aggressive type).

The reason for astrocytoma is unknown, but scientists have found that it can be related to radiation to the head at young age, and there are several genes that are also related to this tumor.

How does it feel?

The symptoms of astrocytoma are related to its location – almost any neurological symptom is possible, including changes in mental status, seizures, and movement or sensory problems. These symptoms appear because the tumor puts pressure on brain tissue or ruins it while growing. Symptoms like these are called focal neurological symptoms.

Besides that, astrocytoma (and any other brain tumor) can increase the pressure inside the skull (called intra-cranial pressure) – this causes symptoms like headaches, nausea and vomiting as well as decreased alertness.

How is it discovered?

When someone comes to the doctor with the symptoms described above, the doctor will order a scan – a CT or an MRI (MRI is the best option). Sometimes doctors can guess the type of the tumor according to the way it looks on MRI – but final diagnosis can be made only when checking the tumor under a microscope after it’s removed in surgery.

This is a part of a brain with a grade 4 astrocytoma which spread to both sides of the brain.

How is it treated?

Aggressive astyrocytomas are treated with surgery to remove the tumor, radiation and chemotherapy. Steroids sometimes help with the symptoms, and so do anti-epileptic drugs (for patients with seizures).

What happens after treatment?

Even with treatment, astrocytoma can be deadly. Patients with low-grade tumors can survive for an average of 6-8 years, but with high-grade aggressive disease, average survival is less that 1 year.

The story of Jodi Fenton who recovered from grade-3 astrocytoma

The bottom line – how do I avoid it

Unfortunately, there is no known way to avoid astrocytoma.

Related posts:

1. As Seen on Grey’s Anatomy – Tay-Sachs Disease – Fat Accumulating in The Brain
2. Nesidioblastoma – A Tumor of the Pancreas That Can Secrete Hormones (As Seen on House MD)
3. As Seen on Grey’s Anatomy – Normal Pressure Hydrocephalus (NPH)

On last night’s episode of Grey’s Anatomy, a cystic fibrosis (CF) patient named Ricky is about to undergo lung transplantation when it is found that he is in a relationship with another CF patient. They are forbidden to continue dating each other in order for him to be able to get the lung.

Why do CF patients have to keep away from one another? To understand that, first here’s a reminder about what cystic fibrosis is all about. As you can read in our article, CF is a genetic disease in which various organs in the body get damaged due to a genetic defect. An example of such an organ are the lungs. In CF, secretions in the airways are more thick, leading to their clogging. Germs multiply in the clogged airways, causing repeated infections.

Photo by Tanya Dawn

People with cystic fibrosis can pass on infections from one another easily, as both are more prone to infections than the general population. There are certain bacteria that are more common in CF patients than in other people. One of those is called Burkholderia cepacia, and another is pseudomonas aeruginosa. A person with CF can easily pass those on to another person with CF through close personal contact or by coughing near them. This is known as cross-infection.

The solution: Antibiotics. But, as more and more people with CF become infected with these bacteria, the bacteria may become resistant to antibiotic treatment.

Since people with CF are more likely to pick up strains from each other than from the environment, it is sadly recommended that people with CF do not come in close contact with each other. This includes no physical contact (like kissing, or perhaps even hand-shaking), no prolonged close contact (no playing together for young children, no long car rides together), no holiday parties for groups of CF families, etc.

Related posts:

1. Cystic Fibrosis – A Dangerous Inherited Disease Causing Thick Secretions
2. As Seen on Grey’s Anatomy – EX-Vivo Lung Transplant – A New Hope For Patients
3. As Seen on Grey’s Anatomy – Posttraumatic Stress Disorder – It Can Happen To Anyone

On Thursday’s episode of Grey’s Anatomy, a girl with no cheek bones needed a surgery so that she could start playing sports without the fear of getting her face harmed. The girl had a syndrome called Treacher-Collins syndrome, explained below.

Danger: Medium

What is it?

Treacher-Collins Syndrome (TCS for short), also called mandibulofacial dysostosis, is a rare genetic disease causing deformities in the facial bones of our skull, such as absent cheek bones.

Who gets it?

TCS is rare. It happens in 1 out of 40-70,000 births.

It is genetic, and in 40% of cases runs in the family, meaning that the child inherits the defective gene from one of the parents in 40% of cases. The other 60% don’t depend on the parent, and the defective gene appears for the first time in the child.

What causes it?

When a fetus develops, its organs have many phases along the way to becoming adult organs. An example for that are the pharyngeal arches, grooves, and pouches. These are all structures that exist only in the fetus, while it’s developing in the womb. They develop slowly during pregnancy into adult organs.

For example – there are 6 pharyngeal arches, each developing into different organs or organ parts in our body.

A developing fetus. Three of the pharyngeal arches are labeled below (mandibular, hyoid, third).

In TCS, there’s a problem with growth of organs that derive from the 1st and 2nd arches (and grooves, and pouches) which are responsible for growth of structures in our face.

How does it feel?

Most people with TCS have underdeveloped facial bones, especially cheek bones, and a small jaw and chin (this is called micrognathia).

Sometimes an opening in the roof of the mouth can occur, called a cleft palate.

In severe cases, it may impair the baby’s airways and be life threatening.

In terms of appearance, people with TCS have eyes that slant downward, sparse eyelashes, and a coloboma, which is a notch in the lower eyelids. There can be other eye abnormalities, causing loss of vision.

The ears can also be affected and be absent, small, or have an unusual shape. This can also lead to hearing problems.

Here’s an example – this video is from a girl with TCS -

How is it discovered?

The looks of a child with TCS help diagnose the disease. The set of features unique to the condition help the doctor come at the correct diagnosis. Also, genetic testing can be done to see if the child has the defective gene.

If the doctor is in doubt, an facial image using x-ray or a CT scan can be used to help with the diagnosis.

How is it treated?

Plastic surgery can be used to treat certain deformities in the face (on Grey’s Anatomy, a bone graft was used to give the girl cheek bones).

Other things are also done to aid other problems that come with the disease, such as hearing aids for hearing loss, or help with breathing if the disease involves the airways.

What happens after treatment?

Children with this syndrome usually grow to become normally functioning adults. It’s important to address any hearing problems, so that the child can perform normally in school.

If there is a history of TCS in the family, genetic counseling may prevent the birth of a child with the condition.

You can learn more about the condition by reading the Reflections on Treacher-Collins Syndrome blog, written by Amie.

Related posts:

1. As Seen on Grey’s Anatomy – Tethered Spinal Cord Syndrome
2. Superior Canal Dehiscence Syndrome – Hearing Sounds Inside Your Own Body (As Seen on Grey’s Anatomy)
3. Osteoporosis – How Your Bones Can Get Thinner With Age And What You Can Do To Avoid It

On Thursday’s episode of Grey’s Anatomy (titled “Shiny Happy People”), a teenage patient called Hayley is thought to have schizophrenia after trying to claw her own eyes out. Not unlike a House episode we recently covered, the diagnosis turns out wrong and instead she is found to have a condition called superior canal dehiscence syndrome.

Photo by ABC

Danger level: Medium

What is it?

Superior canal dehiscence syndrome (SCDS) is a rare condition affecting the ear which causes the person having it to hear sounds inside their body, among other things. It was first described in 1998.

Who gets it?

Since the condition was first described only in 1998, not much is known in that area. A study conducted on cadavers (dead bodies) found ears matching the condition in about 0.5% of bodies checked.

It appears to affect women and men alike. The condition usually affects people around the age of 40, although it may start earlier or later.

What causes it?

Our ear, as we described here before, is divided into 3 parts: The outer ear, middle ear, and inner ear. Our inner ear is the innermost part, and it’s responsible for hearing and balance.

The inner ear.

Our inner ear is composed of 3 canals filled with fluid that sense our position in 3D, which helps us keep our balance. Just like a gyroscope that tells our brain the position of our head in real time. The superior canal is one of those canals.

Overlying the superior canal is one of our bones, called the temporal bone. In SCDS that bone is thinner than usual since it hasn’t developed properly.

Through a mechanism too complicated for this article, the symptoms of SCDS develop due to this bone defect.

How does it feel?

There are typical symptoms people with SCDS may experience:

1. Autophony – This means the person can hear their own speech or other noises inside their body (such as the heartbeat, eye movements, chewing, etc.) very loudly.
2. Dizziness/vertigo – Problems with balance, since, as mentioned above, our inner ear is responsible for keeping our balance. (You can learn about head spinning in our article published a while ago).
3. Tullio phenomenon – In this case, sound can cause the patient to lose balance. Sound can also cause rapid movement of the eyes, called a nystagmus.

These are the main symptoms, although there are others as well. This fascinating video shows a true story:

httpv://www.youtube.com/watch?v=f6vAkdGw8T4

How is it discovered?

One way is doing a CT scan of the temporal bone, which will show its thinning (or dehiscence).

There are other ways. Among them is the method shown in the Grey’s Anatomy episode, called a videonystagmography (VNG). In this method, hi-tech video goggles with infrared cameras are worn while doing things that can provoke rapid eye movements in the condition.

How is it treated?

The condition can be treated by a surgery, which repairs the gap in the temporal bone. Another surgical method is plugging the superior canal.

What happens after treatment?

The success rate of the operation is quite high. Most patients don’t have their old symptoms after the operation.

The bottom line – How do I avoid it?

There is no known way to prevent the condition.

If you happen to have SCDS, there’s a support group for you.

Related posts:

1. Meniere’s Disease – From Head Spinning To Hearing Loss
2. Treacher-Collins Syndrome – Being Born Without Cheek Bones (As Seen on Grey’s Anatomy)
3. As Seen on Grey’s Anatomy – Tethered Spinal Cord Syndrome

On yesterday’s episode of Grey’s Anatomy, a young patient who needed a lung transplant was also a lousy candidate for one, since he had too many risks. Instead of foregoing the whole thing, an ex-vivo lung transplant was performed on him instead.

Why does someone need a lung transplant, anyway?

There are some diseases that cause such damage to the lungs that a new lung is necessary. Such diseases include (don’t fret the confusing names – we will cover them at one time or another here on A Disease A Day) chronic obstructive pulmonary disease (COPD), idiopathic pulmonary fibrosis, cystic fibrosis, idiopathic pulmonary hypertension and others.

So why not give everyone the lungs they need?

There aren’t that many lungs that can be donated to begin with – only about 15-20% of donor lungs are acceptable for transplantation, since lungs are more susceptible to injuries than other donated organs (such as the heart).

Even when we have found a suitable lung for transplantation, not everyone waiting for a lung will get one. Each person waiting for a lung transplant has a score, based on the lung allocation score. For example, people who have a more immediate need for the lung come before those who can wait. Other factors, such as the illness the patient has, other diseases they have, etc. determine their score.

This means that many patients, like the one on the Grey’s Anatomy episode, are left far behind in the list, with a very low chance of ever getting a lung before dying.

What’s an ex-vivo transplant and how does it help things?

This technique was invented in 2008 by a team of lung transplant surgeons led by Dr. Shaf Keshavjee at Toronto General Hospital.

What it does is perfuse a special solution into an injured lung. It lets doctors “treat” the injured lungs while they are outside the body to make them suitable for transplantation. The technique can double the number of lungs fitting for donation.

How is it done?

After the donor lungs are removed from a deceased donor, they are transferred to a protective, transparent bubble-like chamber (like the one seen on the show). Then a series of steps is taken, in which the lungs are connected to a circuit composed of a pump, ventilator and filters through which oxygen, nutrients and a special solution are injected to the lung.

The bubble-like chamber with the lungs in it. Image courtesy of University Health Network

And on a final note – why is it called an ex-vivo transplantation?

Ex-vivo in Latin means “out of the living”. In this case, the healing process for the lungs occurs outside of a human body, inside this bubble-like chamber.

Here’s a video showing the repaired lung in action -

Related posts:

1. Can Cystic Fibrosis Patients Date Each Other? (As Seen on Grey’s Anatomy)
2. Lung Cancer – Why You May Be at Risk (As Seen on House MD)
3. As Seen on Grey’s Anatomy – IL-2 treatment, Anterograde Amnesia

On the last episode of Grey’s Anatomy (which, we know, was two weeks ago – sorry for the delay) Dr Bailey recalls a case in her intern year that helped her become the doctor she is today.

The patient presented in the episode had chronic pain and she’s been through many surgeries, including having her ovaries removed, but nothing worked. That led Dr Bailey (then called Mandy) to the diagnosis of porphyria.

Danger level: Medium

What is it?

Porphyria is a group of diseases resulting in the buildup in the body of materials called porphyrins and related materials, leading to abdominal pain, constipation and muscle weakness.

There are 2 types: Acute porphyria and cutaneous porphyria. We will discuss acute porphyria here.

Who gets it?

Acute porphyria is quite rare – it happens to 1-5 people out of 100,000 in the US. Outside the US it can be much higher: in Sweden, for example, it happens to 60-100 people out of 100,000.

It affects women 1.5-2 times more than it affects men. It usually happens around ages 18-40.

What causes it?

Our body produces a substance called heme. It can be found in all of our body, but mostly in red blood cells, our bone marrow and our liver.

Heme is created in our body out of materials called porphyrins. In the body, the machines that are responsible for converting materials from one type to another are called enzymes. In acute porphyria, one of these enzymes is defective, meaning that instead of converting to heme, porphyrins accumulate in the body.

The problem with the enzyme is usually genetic, meaning you inherit it from your parents.

Having the defective enzyme isn’t always enough. People with the defect might not have any symptoms, a condition which is called latent porphyria. Sometimes, though, there are triggers in the environment which call our body to produce more heme – meaning it will need extra-action from the enzyme. In this case, the symptoms may begin.

Examples of such triggers:

• Certain drugs – Such as some forms of antibiotics, but even birth control pills.
• Surgery
• Stress
• Smoking
• Dieting/fasting
• Infections
• Alcohol use
• Sun exposure
• Excess iron in the body

How does it feel?

Acute porphyria mainly affects our nervous system (we mentioned above another type – cutaneous porphyria – this type affects mainly the skin).

What does a person with acute porphyria feel?

• Abdominal pain
• Constipation
• Vomiting
• Diarrhea
• Pain in the arms, legs or back
• Muscle pain, tingling, numbness or paralysis
• Seizures
• Fever
• Confusion
• Hallucincations
• Disorientation
• Paranoia
• Red urine
• High blood pressure

How is it discovered?

There are several ways to discover when someone has porphyria:

1. Urine test: In it, the high level of porphyrins in the body will be shown.
2. Stool test: Sometimes porphyrins can be detected in the feces better than in the blood.

How is it treated?

There are several ways to treat this condition:

1. Hemin or hematin: This is a form of heme that the doctor injects to your body. This way the body will have enough heme and not try to create more of it (which in this disease will lead to more porphyrins accumulating).
2. Stopping any triggers: Such as medications that may have led to the condition, or an infection.
3. Intravenous sugar (glucose) – Can also inhibit the body’s need to produce heme.
4. A high-carbohydrate diet – Forget Atkins. This is true only for attacks of the disease, not between them.
5. Pain killers 

What happens after treatment?

Porphyria is a life-long disease with symptoms that come and go, more in some people, and less in others. Avoidance of triggers can help reduce the time between attacks.

The bottom line – How do I avoid it?

You can’t prevent having the disease itself (it’s genetic). But if you know you have it, taking the following steps may help avoid attacks:

1. Avoid medications known as triggers (consult your doctor about this)
2. Don’t use alcohol or illicit drugs
3. Avoid fasting or dieting too heavily
4. Don’t smoke
5. Minimize sun exposure. When outdoors, wear protective clothing and use sunscreen.
6. Treat infections promptly.
7. Reduce stress

Family members of people with porphyria are advised to undergo genetic testing, to discover if they have the defect themselves.

Related posts:

1. Acute Intermittent Porphyria Revisited (As Seen on House MD)
2. Acute Pancreatitis – A Dangerous Condition Which Can Be Caused by Drinking Alcohol
3. Acute Pericarditis – Inflammation of Your Heart’s Covering

On Thursday’s episode of Grey’s Anatomy, a patient woke up during her own surgery. She was able to smell her own flesh burning during the procedure (heat is used during surgery to burn down bleeding blood vessels), able to see her abdomen open and to see the surgeons operating on her. The only thing she wasn’t able to do was move. Not even her mouth or vocal cords – meaning she couldn’t even scream.

Sounds like science fiction, right? Unfortunately, this condition, called anesthesia awareness, happens to around 30,000 people a year in the US alone.

Danger level: High

What is it?

Anesthesia awareness is the condition in which a person is awake during surgery, being aware of the surgery, even though they are supposed to be asleep.

Imagine being cut down while awake. Photo by Brit.

Who gets it?

It occurs in about 1-2 out of every 1,000 surgical patients. Probably more in children.

What causes it?

When undergoing surgery, a patient is usually put to sleep using anesthesia. But that is not the only drug given to them. In order to insert a tube down a patient’s throat to ventilate them, and in order to cut through muscle, the patient’s muscles have to be paralyzed. A drug that causes that is given prior to surgery.

When the anesthesia doesn’t work, for any reason, the patient is left with the paralyzing drugs working – meaning they can’t move, speak, blink the eyes or otherwise respond to the pain, if they feel it.

So when does anesthesia not work? There can be a number of reasons:

1. Not using the full dose of anesthesia – In some high-risk surgeries, such as trauma, cardiac surgery, emergency c-sections, or when the patient’s condition is unstable, using the usual dose of anesthetic could harm the patient. In these situations awareness may not be completely avoidable.
2. Patient physiology – Some people (like the woman on the Grey’s Anatomy episode) may be more resistant to anesthetics than others. This can happen due to a genetic condition. Other things, such as other drugs, may interfere with the action of the anesthetic drug and may require using a higher dose of it in order for it to work effectively.
3. Human error – Sometimes a drug dose which is too low may be the cause. Also inadequate monitoring during surgery may be the cause, when there is a need for an increase in drug dosage, but no one has noticed.

How does it feel?

Most people don’t feel the pain of surgery. But even without pain, the experience can be traumatic. The patient can recall the details of their own surgery. Some describe it as being trapped inside a corpse.

This video shows the story of former patients who experienced the condition -

The bottom line – how do I avoid it?

Before surgery, you should meet with your anesthesiologist to discuss anesthesia options. You should share with them any problems experienced with previous anesthetics. You should discuss all prescription and over-the-counter medications you’re taking.

During surgery, the anesthesiologist monitors your vital signs, such as your heart rate, breathing rate and blood pressure. When you are awake when you shouldn’t be, these will change, indicating the need for more anesthesia.

There are also new tools being studied to assess how awake you are during surgery. It is still unclear how effective they are. No monitor exists yet that can completely guarantee you will not experience awareness during surgery.

Related posts:

1. As Seen on Grey’s Anatomy – Posttraumatic Stress Disorder – It Can Happen To Anyone
2. As Seen on Grey’s Anatomy – Tethered Spinal Cord Syndrome
3. Can Cystic Fibrosis Patients Date Each Other? (As Seen on Grey’s Anatomy)

On last night’s episode, Izzie Stevens brings her former biology teacher to Seattle Grace (+Mercy West) hospital as he has been having memory problems. Turns out her teacher has a condition called NPH, which we will cover today.

Danger Level: High

What is it?

Normal Pressure Hydrocephalus (NPH) is a condition in which there is excess “brain fluid” inside the brain.

Who gets it?

NPH usually occurs in elderly people (usually over 60). It can happen to men and women alike.

What causes it?

Our brain and our spinal cord are connected to each other. They are both a part of our central nervous system. It doesn’t take a doctor to know that damage to them can lead to severe consequences.

To prevent damage to them, they are protected by fluid which floats around them, cushioning them. This fluid also provides them with nutrients and gets rid of waste products they produce. This fluid is called CSF – or cerebrospinal fluid.

This fluid is produced within our brain and circulates inside the brain and along our spinal cord.

 

The CSF is produced inside the brain and protects the brain and spine.

Our brain isn’t a solid structure. It has “holes” in it, called ventricles. Inside them there is an area called choroid plexus, in which the CSF is produced.

Normally, CSF circulates in the ventricles and on to the spinal cord and when an excess is produced it is absorbed. In NPH the system which drains and absorbs extra CSF doesn’t work like it should and there is too much CSF inside the ventricles. This causes the ventricles to enlarge and press on different parts of the brain, causing the pressure inside our skull.

NPH can be caused because of a head injury, bleeding around the brain, stroke, meningitis, or a brain tumor.

How does it feel?

NPH normally causes three major things:

1. Dementia: This is a decline in our mental abilities, meaning our ability to remember things, solve problems, concentrate and so on. It is the same thing that’s seen in people with Alzheimer’s disease.

2. Urinary incontinence: This is an inability to hold urine.

3. Abnormal gait: This means difficulty walking.

Other things which can be seen in NPH are headaches, nausea and difficulty focusing the eyes.

How is it discovered?

NPH can sometimes, but not always, be seen on CT scans or MRI scans. In them the brain’s ventricles might look bigger than normal.

A better way to diagnose NPH is to perform a spinal tap (or lumbar puncture) – in it a syringe is inserted to an area around the spinal cord and CSF is removed. This fluid is than analyzed for possible abnormalities. The interesting thing about this procedure is that when fluid is removed, the pressure on the brain subsides, making the symptoms go away.

How is it treated?

If the cause (such as a brain tumor) for NPH is known, a surgery to correct the problem is performed.

If it isn’t (which happens in many cases) a shunt operation is performed. In it, a tube is implanted in the brain’s ventricles, and travels under the skin to the belly, to where the fluid is drained.

What happens after treatment?

The shunt operation should relieve future pressure from the brain and prevent recurrence of the symptoms. Without treatment, symptoms often get worse and could lead to death.

It’s important to note that surgical treatment improves symptoms in about 50% of cases.

The bottom line – How do I avoid it?

Treating disorders that may lead to NPH (such as a brain tumor or a stroke) may prevent it from developing.

This video sums it all up:

Related posts:

1. As Seen on Grey’s Anatomy – Tethered Spinal Cord Syndrome
2. Astrocytoma (Butterfly Tumor)–A Brain Tumor That Spreads To Both Sides (As Seen On Grey’s Anatomy)
3. Want Your Newborn To Have a Normal Weight? Exercise

Yesterday Grey’s Anatomy came back for another season, and so does this column, where we teach you about a disease featured on each episode. This time – tethered spinal cord syndrome.

Danger level: High

Health forums category: Children’s Diseases

What is it?

Tethered cord syndrome (TCS)  is a condition which limits the movement of the spinal cord within the spinal column.

Who gets it?

TCS is a rare disease – it happens in about 0.05-0.25 of 1000 births.

There is, however, a more common condition called spina bifida, which happens to 1-2 people out of 1000. About 20-50% of children born with spina bifida have TCS. Spina bifida, in case you’re wondering, is a condition in which the bones of the spine (the vertebrae) do not form properly during pregnancy.

What causes it?

To understand TCS, you have to know how our spine is built.

In our back there is a row of bones (vertebrae), which together form our spine. Inside these bones travels a cord, called the spinal cord. The spinal cord is responsible for sending messages from our brain (where it starts) to the rest of our body, and by this it controls movement and sensation in the body.

The lower tip of the spinal cord is called the conus medullaris. During development in the womb, the spinal cord moves up in the spine. In TCS, it stops in the middle of the way, so that the conus medullaris is lower in the spinal cord than it should be. In this case, it is tethered, and doesn’t float freely.

When a child that has tethering of his lower spinal cord starts growing, the spinal cord, which is still tethered, starts stretching. This can cause damage to the spine and interfere with the blood supply to it.

How does it feel?

There are several things which TCS can cause:

1. Back pain – It’s usually worsened by activity and relieved by rest.
2. Leg pain – Especially in the back of the legs.
3. Leg numbness or tingling
4. Changes in the strength of the legs
5. Difficulty riding a bicycle, playing sports and falling while running.
6. Muscle contractions
7. Tenderness along the spine
8. Scoliosis – This is a condition in which the spine grows crooked.
9. Problems with urinating

And others…

How is it discovered?

MRI – This is a technique in which an x-ray image of the spine is taken. This is the best way to detect TCS.

How is it treated?

TCS is usually treated with surgery, in which the cord is released so that it is no longer tethered.

This video shows the part in the surgery where the cord is released:

What happens after treatment?

With treatment, the problems should disappear.

Without surgery, though, the condition can get worse. As the child keeps growing and the spine keeps stretching, the blood vessels supplying it get damaged, and less blood is transferred to the cord. This can lead to further damage to the cord and deterioration of the condition.

As mentioned above, TCS often coexist with a condition called spina bifida. There are certain things you can do to lower your baby’s risk for having spina bifida, and by this lowering its risks for TCS:

1. Take folic acid during pregnancy. Foods rich in folic acid contain avocados, black beans, and asparagus. The best way to obtain it, though, is by taking a pill supplement. Consult your doctor for the recommended dosage.
2. If you take drugs to treat seizures or acne, you should consult your doctor before getting pregnant, as those can cause birth defects.
3. Avoid getting too hot in the first weeks of pregnancy (such as in a sauna, a very hot bath, or when you have high fever) – The heat may raise the risk for spina bifida.

Related posts:

1. Syringomyelia – Fluid Inside Your Spinal Cord (As Seen On House MD)
2. Treacher-Collins Syndrome – Being Born Without Cheek Bones (As Seen on Grey’s Anatomy)
3. As Seen on Grey’s Anatomy – Normal Pressure Hydrocephalus (NPH)

Anyone who watched last night’s season finale of Grey’s Anatomy has two different questions on their mind right now (and to not spoil it for anyone who didn’t, neither of them involves either IL-2 or anterograde amnesia). But tradition is tradition, and so you get to learn about two subjects for the price of one today.

IL-2

What is it?

IL-2 (or by its long name interleukin-2) is a chemotherapy used to treat melanomas and kidney cancer.

How does it work?

Interleukins are molecules in our immune system. They help in our body’s response to infections.

The cells that fight infections in our body are the white blood cells. The interleukins attach to these cells by connecting to a special receptor on them, and by this they activate them.

The good news is that the white blood cells can kill not only bacteria and viruses, but also cancer cells. IL-2 causes the body to make more of the immune system cells and makes the cells more effective.

The IL-2 molecule.

When is it used?

IL-2 is used to treat advanced stages of kidney cancer (called renal cell carcinoma) or melanoma, when these tumors metastasize (spread to other parts of the body).

How successful is it?

About 10-20% of people treated with IL-2 respond to the treatment. In a study done in 1999, 270 people with metastatic melanoma were given the treatment. 16% of them (43 people) improved with the treatment. Another study was done in 2000, in which again 16% of people improved.

It’s important to understand, though, that only 6% of these people were completely cured with this drug, while in the others it only helped partially.

Danger level: Medium

What is it?

Amnesia  is a Greek word which means forgetfulness or loss of memory. Anterograde amnesia is the inability to remember new facts.

Who gets it?

There are two types of things which can cause anterograde amnesia:

1. Drugs – No, not the ones that make you high. A group of drugs called benzodiazepines (which are usually used for anxiety or to induce sleep) can cause you to forget things. This can be seen in people receiving them before undergoing surgeries, who can’t recollect the minutes around taking the drug.
2. Physical damage to the brain – The damage can be the result of an injury (such as after an accident) or after a surgery to the brain. Such surgeries may be needed in some types of seizures, or when a tumor is in that part of the brain.

What causes it?

The area in our brain responsible for storing memories is called the hippocampus. Damage to the area of the hippocampus can cause a problem with learning new things and retaining them.

The hippocampus. Lose it and you’ll stop remembering new things.

How does it feel?

People with anterograde amnesia lose the ability to learn new facts. It’s important to understand, though, that they can learn how to do new things, such as talk on the phone. They won’t, whoever, remember who they talked to.

If the amnesia was caused by a drug, it usually goes away after a short time. If the amnesia is a result of damage to the brain, however, the chances of gaining the remembering ability back are much slimmer.

This video is a good example. It’s a part of a documentary about Clive Wearing, who suffers from anterograde amnesia:

You can see more examples by watching the (fictional) movies Memento and 50 First Dates.

How is it treated?

Unfortunately, once an area of the brain is damaged, there is currently no known way to recover that area.

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3. Astrocytoma (Butterfly Tumor)–A Brain Tumor That Spreads To Both Sides (As Seen On Grey’s Anatomy)