Rasmussen's encephalitis

IF A CHILD'S FIERCE LOVE could cause a toy to speak, Abby Lampley's purple dinosaur Chomper would be a chatterbox. With his unusual skill, he'd doubtless tell stories about the incredible resilience of human beings.

That's because Chomper spent a year sleeping tucked under 5-year- old Abby's right arm as the seizures rumbling through her left side worsened, evidence of a brain disease found in fewer than 1,100 children nationwide.

Then Chomper followed Abby from her home in the Spokane Valley to California for a battery of tests, until he was eventually wheeled into a Los Angeles operating room right by her side. There he witnessed a surgery so unfathomable that recovery could only prove the strength of his friend's spirit.

Chomper saw Abby after therapy sessions at St. Luke's Rehabilitation Institute on the South Hill, as she slowly regained skills lost when doctors detached her brain's right hemisphere. Skills like walking and naming the color yellow.

Now Chomper is at Walt Disney World, celebrating Christmas in Florida with Abby and her family, thanks to the Make-A-Wish Foundation.

"It's bigger and bigger and bigger and bigger," Abby said, explaining her desire to go to Disney World. She visited Disneyland in August, days before her surgery.

Abby was granted her wish after her grandmother, Trish Beckham, called the foundation's Spokane office. But Abby is not dying. Like many of the children served by the worldwide organization, Abby is battling a life-threatening disease.

So far, it looks like she's winning.

No one knows exactly what lies ahead for Abby. Little is known about the disease, its cause or how those affected respond to such radical surgery.

But there is a good possibility that Abby will lead a normal and productive life, her doctors say. Beating a disease like Abby's depends on an uncertain combination of determination and luck. But with the strong will she's demonstrated so far, it's easy to think Abby's heading that way.

Theodore Rasmussen, a Canadian doctor, first identified the disease he called "chronic encephalitis" in 1955. He described it as a viruslike syndrome that develops in early childhood, causing swelling and seizures as it slowly damages one side of the brain.

Abby had her first seizure in September 2000, when she was 3. Within a year she was diagnosed with Rasmussen's syndrome.

Both the disease and the seizures affect a child's mental development and are not controllable with drugs. If left to run its course, Rasmussen's syndrome can cause paralysis, mental retardation and possibly death.

For those reasons, parents sometimes opt for a radical surgery called a hemispherotomy, where neurosurgeons detach and remove the diseased part of the brain. When successful, this procedure can stop the seizures and halt the course of the disease.

The procedure is only performed at three hospitals in the United States. At UCLA, they've averaged fewer than 10 hemispherotomies a year since they started in 1986, most of the procedures performed on children.

"Of course there's a point when you say, `No, not my child,'" said Abby's mother, Tammy Lampley, an Alabama native who moved to the Spokane Valley four years ago. "You can't cut into my daughter's brain."

But Tammy read every study, Web site, book and article she could get her hands on.

She learned that time is critical with Rasmussen's, since a young and developing brain is astoundingly pliant and able to recover from the drastic procedure.

"In the end, you just have to have faith," she said.

Abby underwent a hemispherotomy in August.

UCLA's Dr. Gary Mathern disconnected and removed the main portions of Abby's right hemisphere from her brain stem. He also removed her corpus callosum, the tissue that connects the two halves of the brain and delivers messages from one side to the other. He left intact her thalamus, which plays a role in pain sensation, attention and alertness, and a ring of disconnected tissue around the outer surface to prevent excessive blood loss.

After the surgery, Abby experienced no complications, her seizures stopped, and she was released from the hospital about two weeks later, earlier than expected.

Recovery from the procedure now requires Abby's brain to transfer movement, language and perception tasks once performed by the right side to the left. Doctors say the extent to which this transfer happens depends on the individual patient, though all patients have permanent loss of fine motor skills on their left side.

"(The transfer) is a phenomenon we can observe, but the mechanisms as to why it happens remain unclear," said Mathern.

The two sides of the human brain perform specialized tasks. Each side of the brain controls movement on the opposite side of the body. The left brain is often considered the center of logic and language, while the right side is thought to house space perception and creativity.

Abby has been working with speech, occupational and physical therapists at St. Luke's Rehabilitation Institute to facilitate this remarkable and mysterious process.

When Abby left the hospital in Los Angeles three months ago, her left side was effectively paralyzed since she'd lost the part of her brain that controlled its movements.

After about a month of therapy, she was able to walk unassisted. Though she will always have a crooked gait and no use of her left hand, fingers and toes, Abby should be able to run and jump like any other child.

During those first weeks, Abby also struggled to speak and say the names of colors she recognized. Though the brain's left side lords over the major elements of language, higher level skills such as understanding context and metaphor are housed in the right hemisphere. Whether Abby will experience some deficit in those areas is unclear, since detailed studies of the cognitive development of children like her have not yet begun.

One of the most striking challenges Abby faces is in regaining her ability to perceive objects on her left side.

During a September occupational therapy session, therapist Heidi Anderson was helping Abby regain strength and language skills.

Anderson gave Abby a piece of waxy red putty. She asked her to form it into a disk, like a cookie. She then had Abby select small beads from a jar, name their color and press them into the putty, as if adding chips to her cookie.

One by one Abby filled the disk with beads.

"Purple," she called out.

"Yellow," she said with a little prompting.

After a while, Anderson asked Abby a question.

"What about this side of the cookie?" she asked.

Abby turned her head slightly and suddenly noticed an entire crescent slice of the cookie was bare. She immediately started filling it in with beads.

Abby is experiencing "hemineglect," common among people who experience right hemisphere brain damage.

If a person with hemineglect is standing in a town square opposite a church, and they're asked to list all the buildings they see, they'll describe only the buildings on the right side of the square. But if they're asked to imagine themselves standing on the church steps and to describe the scene again, they'll list only the buildings they previously left out.

Abby's physical therapist, Dave Clark, describes the fear he saw in her eyes the first time he asked her to roll over from her stomach to her left side.

"In her mind, there's nothing there," Clark said. "It's like she's rolling into the unknown. She literally has to learn to see the left side of the world."

Abby may not fully understand the challenges she faces, but she's been strong enough to remain active and courageous throughout her ordeal - an attitude crucial to continual improvement.

Before the surgery one of her favorite games was hopscotch, despite severe seizures that sometimes caused her to fall while walking.

As an inpatient, she showed initiative, preferring assisted walking to the wheelchair as she made her way to therapy.

Today Abby will tell you riding the tricycle is her favorite part of physical therapy, a task that requires complex coordination between muscles and perception.

Of course, it's not always that simple. Abby is comfortable within her normal routine but is scared when entering the unknown. Accompanying mom to the grocery store is fine, but starting out in group therapy was terrifying.

Abby celebrated her 5th birthday on Halloween, just two months after the surgery. Her father, Chris, planned to take her to a party with a dozen other children. At first she was afraid to go, refusing even to put on her costume.

But she was persuaded to go, and before long she was drawn into the party by the other kids.

"I bobbed for apples," Abby squealed a month later.

Abby still has developmental progress to make, and her seizures could return in a few years if the disease spreads to the other side of her brain - a rare but possible occurrence. Nonetheless, her parents hope she can begin kindergarten on time next year.

It's hard to imagine any less from a girl with Abby's determination.

When the Make-A-Wish Foundation wish granter asked Abby to describe her favorite activities, she had a curious answer: spinning.

She said she was especially eager to spin in the teacup ride at Disney World.

If she does spin, she may find it to be a good way to see life on the left side of the world. Which would be yet another step toward recovery.

Copyright 2001 Cowles Publishing Company
Provided by ProQuest Information and Learning Company. All rights Reserved.