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Lives That Are All but Whole on Half a Brain : Rare Surgery Dramatically Aids Victims of Progressive Seizure Disorders

November 26, 1987|NANCY SHULINS | Associated Press

BALTIMORE — Maranda Francisco stands at the center of a crowded conference room. All eyes and all cameras are on the fidgety 6-year-old, from the tips of her light-brown curls to the toes of her new party shoes.

For a few self-conscious seconds, she stares back. Then, with a throat-clearing giggle, on a single intake of breath: "Humpty-Dumpty-sat-on-a-wall," she whispers. "Humpty-Dumpty-had-a-great-fall."

Had she quoted Shakespeare rather than a child's nursery rhyme, the performance by the little girl from Denver couldn't have been more dazzling to her audience of doctors, nurses, parents and reporters.

Half of Maranda's brain, her dominant left hemisphere, the half that is verbal and analytical, is preserved in glass jars in a pathologist's freezer, removed two years ago in a heroic feat of surgery known as "hemispherectomy."

Seizure Disorders

It is rare surgery for rare neurological disease: progressive seizure disorders that affect only one hemisphere of the brain and that don't respond to any other treatment. Such disorders can be the result of a stroke, a congenital abnormality, a low-grade tumor, perhaps even a virus.

Maranda, along with seven other "hemis" and their families, is back at Johns Hopkins Hospital for four days of mutual support and testing. It is the first of several planned reunions of these premier patients, who may one day lead to better understanding of these ailments.

But beyond the mystery of the disease looms a greater mystery: that of the cure. How can a surgeon remove half the human brain, yet excise none of the human spirit?

The 10-hour operation that took half of Maranda's brain has given her two years without seizures. Now a kindergartner who swims and takes dancing lessons, who prefers purple to green, who strains at her mother's hand like an exuberant puppy on too short a leash, Maranda thinks the clouds on her flight from Denver looked like "big, puffy pillows." And says so.

Julie Klingelhofer, 20, of Baltimore, poised and ladylike in her summery print dress, smiles knowingly as she watches Maranda's performance.

Love of Children

Four years ago, Julie lost the left frontal, parietal, occipital and temporal lobes of her brain. She did not lose her love of children or her desire to work with them.

Today, Julie Klingelhofer has a certificate of competence from a community college, a steady boyfriend named Wayne and an IQ of 77, a full 10 points higher than it was before her radical surgery.

She also has a job in a day-care center, a job she would have been unable to hold before her 1983 hemispherectomy. "I walk more normally now," she says, glancing shyly down at her white heels. "I'm also more confident."

Six-year-old Jennie McCameron of Kokomo, Ind., underwent the removal of the right side of her brain, the side that governs art and music awareness, the realm of insight and imagination.

Less than a year later, over breakfast, Jennie puts down her goblet of orange juice to sing: "I'm a little teapot, short and stout."

A few minutes later, her mind, minus its right hemisphere, takes an insightful turn. "Every day, in every way," she says solemnly, "I'm getting better and better."

Medical Assessment

Which is not just a reflection of her mother's belief in positive thinking; it's also a medical assessment the doctors can endorse--for Jennie, and for the other hemis who are back at the hospital's Children's Center.

The agenda for their four-day visit includes a full slate of medical and mental tests, as well as a picnic, a pizza party and a news briefing.

The news briefing is, by necessity, brief.

For every question the doctors can answer, more crop up that they can't--about the neurological disorders that crippled these children; about the process by which the remaining, healthy portions of their brains have taken over functions lost to surgery or disease; about the consequences that may one day result from an operation their neurologist, Dr. John Freeman, describes as "horrendous."

Beyond that, the questions broaden: How can half of the brain be lost without any discernible loss of its byproducts--generosity, humor, insight, and optimism?

How can a human being with half a brain still worry, plan, invent, imagine and reason?

How is it that 8-year-old Beth Usher of Storrs, Conn., can lose her left hemisphere, yet retain her large repertoire of knock-knock jokes?

Memory Survives Coma

Beth's memories survived not just the loss of brain tissue, but also the 32 days that she spent in a coma, the result of some brain stem swelling that occurred in response to the trauma of surgery.

Shortly after Beth regained consciousness, her father began quizzing her about people and places from her past. Brian Usher didn't get very far. "Dad," Beth interrupted, with a trace of impatience. "I remember everything."

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