Extraordinary People: Living with Half a Brain - Monday October 1

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Extraordinary People: Living with Half a Brain - Monday October 1

27 Sep five's blog | email this | 217 reads

extraordinary people: living with half a brain
21.00–22.00

Five’s acclaimed documentary strand continues with another batch of absorbing programmes exploring remarkable stories of human experience. Tonight’s programme follows the stories of two young sufferers of epilepsy as they undergo radical surgery to remove large sections of their brains.

At the age of just three, six-year-old Cameron Mott developed a devastating and progressive brain disorder called Rasmussen’s encephalitis. This rare disease attacks the right side of the sufferer’s brain, causing a rapid decline in mental faculties and –if left untreated –eventually leading to partial paralysis. Cameron’s condition has left her with extreme epilepsy. Her daily life is plagued by sudden and frequent fits forcing her to wear a protective helmet at all times. She is only free from the fits for a precious 30 minutes at the beginning of every day, before she collapses and falls victim once more to the relentless cycle of seizures.

This film follows the Mott family as they travel from their home in North Carolina to the Johns Hopkins Medical Institute in Baltimore for a radical treatment. Cameron is about to undergo a complex operation called a hemispherectomy, which is the last resort for doctors treating children with her condition.

During seven hours of surgery, led by Dr George Jallo, chunks of the right side of Cameron’s brain are painstakingly removed. Though the operation is incredibly delicate and difficult, time is of the essence, since the cavity left in Cameron’s head fills with cerebral-spinal fluid at a rate of a teaspoonful every five minutes. Once surgery is over, Cameron is immobilised –any movement could dislodge the remaining half of her brain. For 48 hours, Shelley and Casey Mott cannot hold or hug their little daughter.

Just eight days after her radical treatment, Cameron pedals down the hospital corridor on a tricycle, laughing as she cycles. Her parents believe it is nothing short of a miracle and even her neurosurgeon is amazed by the little girl’s rate of recovery. “I always think that these children... are going to be dependent on their parents for the rest of their lives,” admits Dr Jallo.

However, surviving the operation is only the first hurdle for Cameron –her real challenge is yet to come. The effects of the surgery are similar to the results of a major stroke, so there is a chance that the entire left side of the little girl’s body could be permanently paralysed.

Incredibly, it is also possible that Cameron will make an almost complete recovery. At her age, the brain has a remarkable capacity to reorganise itself – with one side of the brain effectively taking over the functions of the other. All Cameron’s parents can do for now is wait and hope, but they remain confident that their daughter’s determination will see her through.

Elsewhere, in London’s GreatOrmondStreetHospital, 14-year-old epileptic Sean Goldthorpe has his brain connected to a machine. As Sean reads aloud, neurologist professor Helen Cross sends electric charges into the part of his brain that controls language. Sean stumbles and is unable to read further, becoming anxious and frustrated. This disquieting session is part of an invasive monitoring programme being used by Professor Cross to pinpoint the part of Sean’s brain causing his fits. It is an exhausting ordeal for Sean, but he is willing to go through with it if it will give him an opportunity to be seizure-free.

Professor Cross eventually discovers that Sean’s fits are emanating from his hippocampus – an area deep within the brain responsible for emotion and memory. As he grows older, the effects of Sean’s seizures are spreading to the language area at the back of his brain via a lesion. To stop his fits, Sean will need to have both of these areas of his brain removed, but doctors will only go ahead with the operation if they are certain that his memory and speech will not be damaged irreversibly. Sean’s parents now face an anxious wait as their son’s future lies in the doctors’ hands.

Hemispherectomy

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Intervention:
Hemispherectomy
ICD-10code:
ICD-9code: / 01.52
Other codes:

Hemispherectomy is a surgical procedure where one cerebral hemisphere (half of the brain) is removed or disabled. This procedure is used to treat a variety of seizure disorders where the source of the epilepsy is localized to a broad area of a single hemisphere of the brain. It is solely reserved for extreme cases in which the seizures have not responded to medications and other less invasive surgeries.

Contents

[hide]

• 1History and changes
• 2Results
• 3In the Media
• 4References
• 5See Also
• 6External links
• 7Further reading

[edit]History and changes

Hemispherectomy was first tried on a dog in 1888 by Friedrich Goltz. The first such operation on humans was done by Walter Dandy in 1923. In the 1960s and early 1970s, hemispherectomy involved removing half of the brain, but this resulted in unacceptable complications and side effects in many cases, like filling of excessive body fluids in the skull and pressuring the remaining lobe (known as hydrocephalus). Today, the functional hemispherectomy has largely replaced this procedure, in which only the temporal lobe is removed; a procedure known as corpus callosotomy is performed; and the frontal and occipital lobes disconnected.

[edit]Results

All hemispherectomy patients suffer at least partial hemiplegia on the side of the body opposite the removed or disabled portion, and may suffer problems with their vision as well.

This procedure is almost exclusively performed in children, since their brains generally display more neuroplasticity, allowing neurons from the remaining hemisphere to take over the tasks from the lost hemisphere. This likely occurs by strengthening neural connections which already exist on the unaffected side but which would have otherwise remained small in a normally functioning, uninjured brain.[1] One case, demonstrated by Smith & Sugar, 1975; A. Smith 1987, showed that one patient with this procedure had completed college, had attended graduate school and scored above average on intelligence tests. Studies have found no significant long-term effects on memory, personality, or humour after the procedure[2], and minimal changes in cognitive function overall.[3]

[edit]In the Media

A hemispherectomy is performed on a patient played by Dave Matthews in the Season 3 episode of House, M.D., "Half Wit." His right hemisphere was severely damaged in a car accident when he was 10 years old.

In the season 1 episode called "The Self-Destruct Button" on Grey's Anatomy, Dr. Shepherd performs a hemispherectomy on a 3 year old girl with a seizure disorder.

[edit]References

Why would you remove half a brain? The outcome of 58 children after hemispherectomy-the Johns Hopkins experience: 1968 to 1996.

Vining EP, Freeman JM, Pillas DJ, Uematsu S, Carson BS, Brandt J, Boatman D, Pulsifer MB, Zuckerberg A.

Pediatric EpilepsyCenter, Johns Hopkins Medical Institutions, Baltimore, Maryland, USA.

PURPOSE: To report the outcomes of the 58 hemispherectomies performed at Johns Hopkins between 1968 and January 1996. METHODS: Charts were reviewed of the 58 hemispherectomies performed at Johns Hopkins Medical Institutions by the Pediatric Epilepsy Group during the years 1968 to 1996. Twenty-seven operations were done for Rasmussen's syndrome, 24 operations for cortical dysplasias/hemimegalencephalies, and 7 for Sturge-Weber syndrome or other congenital vascular problems. Seizure control alone did not seem to adequately describe the outcomes of the procedure. Therefore, a score was constructed that included seizure frequency, motor disability, and intellectual handicap. This burden of illness score better described the child's handicap before and after surgery. RESULTS: Perioperative death occurred in 4 out of 58 children. Of the 54 surviving children, 54% (29/54) are seizure-free, 24% (13/54) have nonhandicapping seizures, and 23% (12/54) have residual seizures that interfere to some extent with function. Reduction in seizures was related to the etiology of the unilateral epilepsy. Eighty-nine percent of children with Rasmussen's, 67% of those with dysplasias, and 67% of the vascular group are seizure-free, or have occasional, nonhandicapping seizures. All operations were considered by the parents and the physicians to have been successful in decreasing the burden of illness. In 44 the procedure was very successful, in 7 it was moderately successful, and in 3 it was minimally successful. Success was related to the etiology, and early surgery was preferable. CONCLUSION: Hemispherectomy can be a valuable procedure for relieving the burden of seizures, the burden of medication, and the general dysfunction in children with severe or progressive unilateral cortical disease. Early hemispherectomy, although increasing the hemiparesis in children with Rasmussen's syndrome, relieves the burden of constant seizures and allows the child to return to a more normal life. In children with dysplasias, early surgery can allow the resumption of more normal development.

PMID: 9240794 [PubMed - indexed for MEDLINE

This is truly, truly amazing. I remember reading somewhere before (in an article called “Is Your Brain Really Necessary” by somebody I can’t remember) on the condition of hydrocephalia which sometimes is discovered only late in life. Someteimes the patients have less than half of their brain tissue left, the most being taken over by brain fluid the uptake of which has for some reason been blocked. Nevertheless, in some cases the patients have been living normal, happy lives, engaged in highly intellectual and social activities.
And now there is this story. In some severe cases doctors have found that the only cure is to surgically remove hal of the patients brain, the left or the right hemisphere, as the case may be. If this is done in early childhood, thge patients may make an almost full recovery, both in terms of physical ability (therer may be a period of left/right paralysis), linguistic skills (even if you remove the right hemipshere) and social and cognitive abilities.
So what does this suggest to me? On one hand that that surgery is an amazing handicraft. Or what do you make of the fact that one surgeon performing the hemispheroctomies describes his expertise in a tactile way: “sick brain feels like mushy apple, healthy brain like very soft boiled egg”. On the other hand, that despite all the extravagant claims made in terms of physical correlates of consciousness or certain cognitive functions, we actually do know very little. This is proven by the simple fact that normally functioning hydrocephalics or patients after hemispherectomies simply do not possess the parts of brain that are supposed to be the “correlates” of this or that mental function. We do know that you need to have a brain in order to have a mind, but after that there is a long long stretch of unknowledge, and the neuroscientific claims coming after that immense unknowledge are not much better in illuminating philosophical questions than LaMetrrie’s description of the brain as a collection of self-winding springs in the 17th century.

The Deepest Cut

by Christine Kenneally

At nine o’clock on July 28th last year, Wendy Nissley carried her two-year-old daughter, Lacy, into O.R. 12 at JohnsHopkinsHospital to have half of her brain removed. Lacy suffers from a rare malformation of the brain, known as hemimegalencephaly, in which one hemisphere grows larger than the other. The condition causes seizures, and Lacy was having so many—up to forty in a day—that, at an age when other toddlers were trying out sentences, she could produce only a few language-like sounds. As long as Lacy’s malformed right hemisphere was attached to the rest of her brain, it would prevent her left hemisphere from functioning normally. So Lacy’s parents had brought her to Johns Hopkins for a hemispherectomy, which is probably the most radical procedure in neurosurgery.

Wendy laid her daughter on the operating table. Because Lacy was so small, it took the anesthesiologist almost ninety minutes to insert her intravenous lines. George Jallo, the attending neurosurgeon, spent a long time arranging her head on gel padding and then drew “Cut here” markings on her shaved scalp. The rest of Lacy’s head, including her face, was covered with a sterile drape. Jallo made one long cut across the top of her head from the front to the back, and another at right angles to the first, which started midway along it and stopped just in front of her right ear. He folded back the scalp and made small holes in her skull with a power drill, outlining a rough semicircle. Then he used the drill to connect the dots and removed a portion of the skull. He cut another T in the dura, a thin, leathery membrane covering the brain. Gently, he peeled back two large flaps.

By half past one, Jallo and a resident had already removed the right frontal lobe. David Lieberman, the pediatric neurologist who had examined Lacy when she first came to Johns Hopkins, looked on, shaking his head in wonderment. “It’s so open,” he said, turning to me. “Normally, with brain surgery, you make a hole about this big”—he curled his thumb and index finger into a circle.

After removing the frontal lobe, Jallo embarked on the parietal lobe. In case complications put a sudden stop to the surgery, it was important to take out the seizure hot spots first, gradually working through the hemisphere in descending order of priority: after the parietal lobe would come a small section of the occipital lobe, then the temporal lobe, then the rest of the occipital. Finally, Jallo would cut the corpus callosum, the bundle of fibres that connect the two hemispheres of the brain. The surgeons slowly worked around each side of the parietal lobe, making tiny pinches in the brain with electric cauterizing forceps. There was a slight smell of burning in the bright, noisy operating room. As the cut became deeper and wider, the tissue on either side browned and blackened, and the lobe started to move back and forth. At the bottom of the parietal wedge, the clean white of nerve fibres was visible; as the lobe was severed, they came apart like string cheese. A surgical technician bent toward Jallo with a small plastic bowl in his hands. Jallo picked the lobe out of the skull—it was the size of an infant’s fist—and dropped it into the container.

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As she led me out of the O.R., Eileen Vining, the attending neurologist, said, “Did you see how rigid it was? Normal brain sags in your hands.” Vining talked quickly, moving from one complicated idea to the next, punctuating each with “O.K.?” and an expectant nod. She had been in and out of the operating room all morning, and now she was off to find the Nissleys and tell them how Lacy was doing.

Four hours later, Vining took me back into the O.R. Lacy’s right hemisphere was gone, and her cranium looked like a wide, uneven bowl. I could see the deep cavity where the frontal and parietal lobes had been, and the white-pink color inside the base of the skull. In the middle of the remaining brain was a shallow mound where Jallo had left a layer of nerve fibres to protect the ventricle, a fluid-filled pocket that cushions the brain and the spinal cord. The white matter there was now gray-black. Jallo and his resident lightly touched their forceps to it, and the cauterizers fizzed, sealing the brain to prevent microhemorrhages. Hemorrhaging is a constant concern in brain surgery, and at one point in the operation Jallo decided to leave in a small piece of the right occipital lobe which threatened to bleed dangerously. Jallo glanced at Vining and Lieberman, and the doctors stretched forward to look at the severed corpus callosum. Over and over, the surgical technician poured in saline, and Jallo and his resident drew it out again with a loud suction pump.

When he had finished removing brain tissue, Jallo tipped in small packets of Surgicel, a feathery white substance that helps blood to clot. It melted onto the surface of the brain. “That was good. There was not a lot of bleeding,” Vining said. “You never know what you are going to get until you open it up. Sometimes you just go in there and you hold your breath and pray.”

In the final hour, Jallo sutured Lacy’s dura, which had shrunk slightly from dehydration, and filled the right side of her head with saline. The technician then brought the missing section of the skull back to Jallo, carrying it in a wide right angle over surgical carts rather than risk moving it over the floor. Jallo reattached it, using four tiny dissolvable plates made of a sugarlike substance. He then closed the scalp incision with a staple gun, leaving seventy-eight aluminum staples in Lacy’s skin. The hemispherectomy had taken nine hours. The resident bandaged Lacy’s head, gently turned her onto her right side, and stuck a piece of tape on her head that said “This side up.”