Rasmussen's encephalitis

Christina Santhouse isn't your average 15-year-old. She's a straight-A student, a high scorer in her bowling league, and a karate expert, working her way toward a black belt. Oh, and she has half a brain.

When Christina was 8 years old, she and her family took a summer trip from their home in Bristol, Pa., to the New Jersey shore. There, her mother, Lynne Catarro, noticed a tremor in Christina's foot. She took Christina to a local emergency room, where doctors rushed her to St. Christopher's Hospital for Children in Philadelphia. After several days of testing, doctors handed the family a frightening diagnosis: Rasmussen's encephalitis, a rare brain disease that strikes only children.

RADICAL SURGERY

Rasmussen's encephalitis attacks one hemisphere, or half, of the brain. It causes seizures, loss of motor skills, mental deterioration, and hemiparesis, or paralysis of one side of the body.

Within weeks of her diagnosis, Christina's tremors graduated to seizures. She began seeing John Freeman, a pediatric neurologist at Johns Hopkins Children's Center, in Baltimore. Freeman suggested a radical treatment--a hemispherectomy, or removal of the diseased right half of Christina's brain.

FLEXIBLE BRAIN

Removing half a brain is obviously a dramatic course of action. The brain controls all physical and mental activity in the body. Fortunately for hemispherectomy patients, much of that activity is controlled by both halves of the brain. After the surgery, "[the patients] have full retention of memory and intellect," said Freeman.

However, some mental and physical functions are mainly controlled by one hemisphere. The right hemisphere of the brain regulates movement of the left side of the body, and the left hemisphere regulates movement of the right side.

Hemispherectomy patients always have some paralysis on the side of the body opposite to that of the removed hemisphere.

Language is also controlled primarily by one hemisphere--the left. After a left hemispherectomy, patients often develop expressive aphasia. They understand language but have trouble remembering the correct words when they speak.

Children recover best from hemispherectomies because their brains are more adaptable than those of adults. The remaining half of a child's brain is better at taking over the functions from the side that was removed. In the case of expressive aphasia, left hemispherectomy patients eventually regain language abilities by using the right half of the brain, even in patients in their teens.

Luckily for Christina, she required a right hemispherectomy, so losing language wasn't as much of a concern. Still, site had to prepare herself for major side effects. "Since I wouldn't be able to use my left side, my mom wrapped it in bandages so I could get used to how it would feel," Christina told Current Science. The bandages limited her movements and prepared Christina for doing things with her right hand alone.

Five months after her diagnosis, Christina had the surgery. By then she was experiencing about 100 seizures a day. The operation took 14 hours. Afterward, Christina suffered an intense headache that lasted several days. She spent two weeks in the hospital and then two more in a rehabilitation center. "My soccer team made a video for me and sent it to the hospital, and it made me want to go back and see all my friends," she remembered. Four months after the surgery, she returned to school.

In the past few years, researchers have found an autoimmune link to Rasmussen's encephalitis. In an autoimmune disease, the immune system malfunctions, attacking the body's own tissues and cells as well as substances normally produced by the body. New therapies that can slow the progression of Rasmussen's are now being developed, but hemispherectomy is still the only cure.

CHRISTINA SPARKLES

Today, Christina takes no medication for her condition and hasn't had a seizure since the surgery. Occasionally, when site turns her head too quickly, she feels a sloshing sensation in the space where her right brain used to be. That space has since filled with cerebrospinal fluid, a substance that normally fills cavities in the brain and spinal cord. The fluid lubricates tissues and absorbs shocks.

Christina is still in therapy. Her left arm is partially paralyzed: site can move her left shoulder and elbow, but doesn't have normal sensation or muscle control in her left hand and fingers. She also wears an ankle brace to help support her left foot. "It took a long time to figure out how to do things with one hand that I used to do with two," she said.

But she hits learned--and excelled. "All [hemispherectomy patients] recover much as Christina has done," said Freeman, "although few sparkle as she does." Today, when she has a moment free from school, karate, bowling, and friends, Christina meets with other Rasmussen's patients to help them prepare for the surgery.

She maintains a positive outlook. "Take the challenges as an opportunity to make something better," she said. "Don't take anything for granted."

ROAD TO RECOVERY

Doctors studied scans of Christina Santhouse's brain before her surgery.

Eight-year-old Christina was readied in the hospital ...

... for the 14-hour operation, during which surgeons removed the right half of her brain.

After the operation, Christina spent two weeks recovering in the hospital ...

... and another two weeks in a rehabilitation center.

Christina is now a sophomore in high school, where she gets top grades ...

and scores big on her bowling team ...

and otherwise lives the life of a normal teenager.

Objectives

This feature about a girl who developed a severe brain disease describes how the body recuperates when half the brain is surgically removed.

Critical Thinking

* How might Rasmussen's encephalitis be cured?

* Name some other autoimmune diseases.

National Science Education Standards

* Personal and community health: illness

* Structure and function in living systems: organs and specialized cells

Internet Links

* Bucks County Courier Times: Christina's New Challenge: http://www.phillyburbs.com/couri ertimes/news/news/0906chris.htm

* Rosie: A Beautiful Mind: http://www.rosiemagazine.com/ kids/0208_amazingcase.html

1. Rasmussen's encephalitis is a rare disease in children that attacks one hemisphere of the brain. Symptoms include seizures, loss of motor skills, mental deterioration, and hemiparesis, or paralysis on one side of the body. 2. A hemispherectomy is radical surgery to remove one hemisphere of the brain. 3. Language is controlled mainly by the left hemisphere of the brain. Only those hemispherectomy patients who have had their left hemisphere removed experience difficulty with language. 4. An autoimmune disease is one in which the immune system malfunctions, attacking the body's own cells and tissues and other substances normally produced by the body. 5. Cerebrospinal fluid is a substance that fills cavities in the brain and the spinal cord. It lubricates tissues and acts as a shock absorber.

Short Answer

1. What is Rasmussen's encephalitis? What are its symptoms? --

2. What is a hemispherectomy? --

3. Why do only some patients who have had a hemispherectomy experience difficulties with language after the operation? --

4. What is an autoimmune disease? --

5. What is cerebrospinal fluid? What is its function? --

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