Extraordinary People - Page 3 of 4 - A My Multiple Sclerosis site

Neurofibromatosis A benign tumour that weighed 11 stone (70 kgs)

Stephen July 10, 2015 Extraordinary People

Lucica Bunghez - Megatumour

In the Romanian province of Transylvania, a woman is being eaten alive, consumed by a giant parasite on her back. Lucica Bunghez has a tumour that weighs an incredible eleven stone. Its so big and feeds off so much of her blood that she barely has the energy to move. She has been confined to her bed for the last three years. Doctors in Transylvania say that surgery to remove Lucica’s tumour would cause fatal bleeding. Inside, it is a tangled mass of arteries and viens. Cutting these, they say, world cause fatal bleeding. So, its been left to grow and grow.

Lucica’s heart is beginning to fail, she lies waiting to die! But, there is one surgeon who’s dared to operate on a tumour this size before and he’s going to try to make medical history again. But, will Lucica survive?

Brasov is in the heart of the Transylvanian mountains, and it was here that Lucica was born in 1957. She was a normal, healthy child who grew into an attractive young woman. She married and had a son. Lucica’s husband, Marian, still works in the same communist-era factory he has for decades. For the first eight years of marriage there was no sign that there was anything wrong with Lucica, until one day everything changed.

One evening, in the bath, she screamed and started banging on the wall. When Marian came to see what was the matter, the bath was full of blood. Marian phoned for an ambulance and she was taken to the district hospital in Brasov. The doctors told them that a blood vessel had burst in a patch of brown skin on her back, That brown patch was the tell-tale sign that Lucica had neurofibromatosis or NF.

NF is a genetic condition, a fault in a gene that regulates normal cell division means that cells start dividing out of control, It leads to the growth of benign tumours on nerves or nearofibromas. Mostly, nearofibromas remain quite small but, in Lucica’s case a tumour started to grow at an extraordinary rate over the next six years. The tumour was left to keep on growing by as much as 3lbs a month.

Lucica needed constant care, but Marian still had to go to work. The tumour didn’t get any better, it grew to an astonishing eleven stone. Whilst Marian worked, their son Marius had to spend all day, at home, caring for his mother. This seemed to be the life they were condemned to, Lucica bed-ridden with Marius caring for her by day and, by night, Marian nursing her to sleep. But, Lucica had one more chance!

On the other side of the world was a surgeon who had once before successfully removed another massive tumour but, as yet he was unaware of Lucica’s plight.

Four years ago, in Chicago, plastic surgeon McKay McKinnon successfully removed a massive tumour from a woman who, like Lucica Bunghez, had been told it was inoperable, Word reached him about Lucica’s predicament and her pleas for help. He responded “Knowing that our resources might be the only reasonable chance that she had, since she’s been turned down in more than one place, I wouldn’t be able to live easily with myself having turned her down”.

Dr Madelyn Kahana worked with Dr McKinnon on the previous giant tumour case. A specialist in anaesthetics and intensive care at the University of Chicago Hospital, it was to be her responsibility to keep Lucica alive during the lengthy operation.

McKinnon and Kahana set off for Romania, and with them went a hand-picked team of nurses. When Lucica’s family heard that the Americans were on their way, they drove her the hundred miles or so to from their home to the Romanian capital city of Bucharest. Lucica was admitted to the emergency hospital, one of Romania’s leading hospitals, where the operation was to be performed.

The American team were met, at the airport, by a large and lively contingent of the Romanian press.

McKay McKinnon and Professor Ioan Lascar, Romania’s leading surgeon, met for the first time and together began planning the operation. The idea was that Lascar’s skills and McKinnon’s experience of the previous operation would prove equal to this mammoth task.

Neurofibromatosis — Lucica and her Megatumour

The tumour had grown from the base of Lucica’s spine , enveloping her back and wrapping around her thighs. It was tightly packed with blood vessels, so cutting chunks out of it would have caused massive bleeding. The surgeons agreed upon a strategy to try and peel the tumour off whole from Lucica’s sides before turning her over and prising it off her back. Professor Lascar had over thirty years experience of removing tumours, but this was the first time he’d seen Lucica or a tumour this size.

Lucica’s giant tumour is not cancerous, but its size makes it a deadly parasite, so it has to be removed to save her life.

Professor Lascar understands that before surgery they need to locate each of the hundreds of blood vessels feeding the tumour. Accidentally severing any one of these could cause fatal bleeding. The operation is going to be a 12 hour ordeal. Professor Lascar and Dr McKinnon know surgery is Lucica’s only chance, but that chance is only around 50%

In the weeks leading up to the operation, the hospital blood-bank appealed to the Romanian public to donate blood. The public responded and the fridges are packed and ready. Hearing that surgery is about to begin, the world’s media descends on the hospital’ Lucica’s plight has become global news.

The fifteen strong team of surgeons begin work. Anaesthesiologist Madelyn Kahana, carefully monitors Lucica’s already struggling heart in case the anaesthetic causes it to fail. Bit by bit, the tumour is removed from Lucica’s front and side. She’s monitored continuously for signs of haemorrhaging or heart attack.

Surgical Team — Doctors McKinnon and Kahana and Professor Lascar

Now, its time for the riskiest moment yet. Lucica must be turned over before the surgeons can begin prising the tumour off her back. The tumour is heavy enough to tear away under its own weight, so her body and the tumour must ne lifted and turned as one. She has multiple intravenous lines and a breathing tube, critical to keeping her alive. Turning her could risk losing all of this.

Its a struggle, and for a moment the tumour almost gets the better of them. After the turn, the surgeons are able to pick up the pace. The Romanians and Americans are working well as a team. They’re approaching the final stage of the operation, they have to tie off and cut the big blood vessels anchoring the tumour at the base of Lucica’s spine, before attempting to lift it off her.

After 12 long hours, 28 units of blood and 30 units of plasma, comes the moment of victory, the tumour is finally separated from its host. It weighs a remarkable 70 kilos. Lucica, free from her parasite for the first time in years, can be bandaged up for recovery. Lucica has to be rushed into intensive care, right through the middle of the jostling press-pack.

Lucica is not yet out of danger. 40% of her body is now an open wound and over the next few weeks she will need many skin grafts to close it. Until then, her life will hang in the balance as she can catch infection and die at any time.

After nine months, finally she hopes her suffering is at an end. Professor Lascar has had to perform multiple skin grafts to close the giant wound on her back. Any infection during this time could have killed her. Lucica is finally ready to leave hospital and travel back to Brasov.

Lucica believes her recovery was an act of God, for her doctors it proves that neurofibroma tumours this size can be removed after all.

CREDITS: All of the above information came from the UK Channel 4 “Body Shock” Documentary Series

Sharon Parker The Woman with the Mysterious Brain

Stephen July 10, 2015 Extraordinary People

Hydrocephalus

Sharon Parker was born and bred in Barnsley, Yorkshire. She’s married with three children and is a qualified staff nurse. But, Sharon has almost no observable brain. Since she was a child, doctors have told her that she has no more than 10-15% of a normal brain.

Historically, it was a popular assumption that the size of the brain determined the level of a person’s intelligence. By that reckoning, Sharon should be a gibbering idiot.

Dr. Jonathan Cole, Consultant Neurophysiologist explains further: “For a long time, people have measured the size of the brain, in post mortem, and and they’ve measured the size of of heads in people and tried to relate the size of the brain to how bright the person is.

There are some people who are severely micro cephalic, with very small brains, and they have severe learning difficulties. But, on the whole, it’s very difficult to relate the size of the brain to how it functions. The function of the brain is not something that is related to how big the brain is or to it’s volume. Rather it’s how well the nerve cells function within that brain. In particular, how well they connect with other brain cells”.

Sharon’s mother Pat recalls: “We were told, so many times, that Sharon wasn’t going to live to adulthood”.

Sharon and Dave have been married for fifteen years. Dave has his own construction business. Not a master of new technology; he still writes his invoices out by hand. Sharon is the one who works them out and puts them on the computer.

Sharon is clearly competent, but just how intelligent is she? She was asked to take an IQ test marked by an educational psychologist. How does she compare with the rest of the population? Dave thinks that Sharon is normal, except for her untidiness. Sharon claims she sees past the untidiness and maybe that part of her brain is missing.

If Sharon has a hole where most people have brain, has she noticed anything else she has difficulty with? She tells us: “Sequences of numbers, telephone numbers, I find hard to remember”. The IQ test results that Sharon, far from being an idiot, has an IQ of 113 making her above average. 80% of the population would have a lower score.

Water on the Brain

Brain Scans — Normal Brain vs. Sharon’s Brain

So, how did Sharon come to have such an extraordinary brain? When she was eight months old, her parents were worried that her head was unusually large. They took her to see a specialist who diagnosed water on the brain. At nine months they found out she was hydrocephalic and needed surgery.

Traditionally, hydrocephalus or water on the brain was a killer. Occasionally, children survived, but as their heads swelled they became little more than monsters at a freak show.

Mr Cole explains “Hydrocephalus is a build up of fluid within the brain. We all have fluid in the brain, in areas called ventricles and that flows out to bathe the outside of the brain. If that fluid builds up within the ventricles it tends to expand and the brain is pushed out against the skull. In children the skull is not set and the squashing of the brain and th slightly raised pressure can actually lead to an enlargement of the skull itself”.

Sharon’s life was saved by a pioneering operation in which a valve was put into her brain to control the fluid. By the time her hydrocephalus was detected, the fluid had been building up for nine months, creating an enormous hole in the middle o her brain.

Brains that have developed abnormally are intriguing subjects of research for neuro specialists. Leading American Neurosurgeon Dr Mark Luciano believes there is much that such a brain can tell us.

Dr Luciano says: “For me, Sharon is most interesting because the hydrocephalus has developed so slowly that her brain has adapted very well to allow her to function to a high level. We want to know how the brain can do that”. So, Sharon and her family fly to Cleveland, Ohio to meet Dr Luciano, to find out more about what her brain is really like.

Armed with the latest technology and with new tests available, Dr Luciano is hopeful of finding out much more about Sharon’s brain than she ever knew before.

First, Sharon goes for an MRI scan, but this is no ordinary scanner. Dr Luciano explains “It is considered one of the fastest scanners in the world. It has a lot of channels and takes lots of pictures at one time. What it can do is not only a standard picture of the brain, it can also get a picture of the blood vessels and blood flow. Sharon’s brain scan takes over two hours. Doctors are looking at her brain from every angle, pinpointing functions and checking blood flow. It emerges that Sharon has had a lucky escape. As her brain pressed against her skull, it became dangerously thinned in the crucial frontal lobe.

The outer surface of the normal brain is ridged to give it more surface area, whereas Sharon’s brain has been stretched and the surface flattened, especially in the frontal lobe where functions like memory are located.

Phrenology

Back in the 18th century, there was a fashion for something called phrenology. It was believed that bumps on the skull were a guide to a person’s abilities. Phrenologists even mapped the skull to show where different human characteristics were, supposedly, located. These theories have long since been discredited but, in one respect, the phrenologists were not so far from the truth. We now know that different functions are located in specific areas of the brain.

It was only with memory that Sharon showed signs of a problem but, even there the doctors discovered that her brain had made every effort to compensate. Tests showed that she scored well above average on her long-term memory but, below average on her short-term memory. The amazing thing is that she seems, somehow, to combine the two in order to overcome some of her memory problems.

Dr Luciano summarises “Overall, I’m surprised that each test showed so much normalcy. I think she’s a very good example of how the brain can adapt to a very unusual situation and that if the forces against the brain occur slowly enough and early enough the brain can be extremely flexible”.

For Sharon, the most important discovery the American doctors made concerned the size of her brain. Something that only the very latest technology allowed them to measure. Having been told, all her life, that she has only 10-15% of a normal brain the result came as a welcome surprise, but it was something of a shock for Dave. While the volume of Dave’s brain was 1300cc Sharon took great delight in telling him that hers was 2300cc.

The American doctors had discovered that although Sharon’s ventricles expanded hugely because of her hydrocephalus, it was not at the expense of brain size. Part of the brain mass was pushed to the bottom rear of her skull and because her infant head swelled slightly her brain is actually occupying a larger space.

Lori Hoogewind 14st Neurofibromatosis Tumour

Stephen July 10, 2015 Extraordinary People

Lori and Gary Hoogewind, Michigan

Lori Hoogewind

Lori and Gary Hoogewind led a perfectly normal life in Michigan. They were married in 1984 and adopted a little girl 13 years later in 1997. All was well until the winter of 1998 when Lori’s life was about to be turned upside down.

When she was born her mother noticed a small lump and a fairly large birthmark on her back. Nothing much was thought of it at the time, but it was to prove very significant.

Neurofibromatosis is caused by the mutation of a single gene which distorts the CNS (Central Nervous System) and leads to the spontaneous development of small, usually benign, tumours all over the body

Birthmark

By the time Lori was married she had undergone several operations to remove these small tumours. At this time, doctors were afraid to operate on the original lump on her back. In 1984 a malignant tumour was found on Lori’s body, for which she underwent radiotherapy which stopped the cancer but had a catastrophic side-effect.

The radiotherapy, rather than shrinking the residual tumour, caused an explosion of growth in the benign tissue. Within a matter of months, the tumour had grown to immense proportions and was consuming vast quantities of her blood supply. The tumour ballooned rapidly to 14 stone, double her original body weight, and wrapped around from her back to her stomach.

At this point Lori and Gary Hoogewind could not find a doctor willing to treat a tumour of this size, and the local health services began counselling for, what they believed to be, her imminent demise. A lucky break put them in touch with Dr. Priscilla Short who introduced them to Dr. McKinnon.

Neurofibromatosis Tumour

Dr. McKay McKinnon Clinical Assistant Professor of Plastic Surgery began to assemble his team of experts, including Dr. Madelyn Kahana, Medical Director of Paediatric Intensive Care and a leading anaesthetist, and Dr. David Song, Chief Resident of Plastic Surgery who would plan the complex operation. The size of the tumour presented particular problems, not least of which was arranging some form of support to hold such a massive growth.

The removal of the biggest tumour in medical history would take many hours and would require every one of the many blood vessels contained within the tumour to be cut and sealed one at a time to prevent huge haemorrhage which would almost certainly cause Lori to bleed to death.

During the second half of the operation, major problems set in. Lori began to bleed and Dr. McKinnon had a critical decision to make. To stop the operation and Lori would die, but to continue may have the same outcome. Finally he decided to continue but cut around a particularly bloody portion of the tumour located close to the spine and leave this for later removal.

After 18 hours of surgery and 50 pints of blood the tumour was removed. This left Lori with a 40% skin loss which over the next 8 weeks would require 5 major skin grafts. Ultimately, the operation was a success and Dr. McKinnon and his team made medical history. Lori Hoogewind recovered fully and has returned home to her husband and daughter.

The World’s Tallest Man Leonid Stepanovych Stadnyk

Stephen July 9, 2015 Extraordinary People

Pituitary Giantism, Gigantism

Leonid Stadnyk

Leonid Stadnyk is a giant, he’s eight foot four inches tall, but he doesn’t want a place in the record books, he wants help. He’s tall because of a huge tumour under his brain. A tumour which, if it carries on growing, will kill him. One of the world’s leading specialists is going to meet Leonid to see if he can save the life of the tallest man in the world.

The Ukraine is a newly independent country that used to be part of the Soviet Union. 200km from the capital Kiev is a little town known, in Ukrainian, as Place of Wonders. Just outside this town is a village so tiny it’s not on any map and in this very small village is a very small house and in this very small house is the tallest man in the world; Leonid Stadnyk. Leonid is 36 years old, but as a child he was average height.

Far from Leonid’s isolated existence, there are now, in the West, tall clubs where some of the world’s most vertiginous people can gather and look one another in the eye, To be a member of Tall Clubs International a man must be over 6′ 2″ and a woman must be over 5′ 10″.

There’s a woman from Shelbyville, Indiana who towers over all the other members of the tall club. Sandy Allen has, for years, held the record of the world’s tallest woman at seven foot seven inches. The reason Sandy is so tall is a frightening one, because to get this tall, chances are you are seriously ill. She had a tumour in her pituitary gland which has been surgically removed.

Chris Greene

Like Sandy, Leonid is a giant because of a pituitary tumour, but unlike Sandy, Leonid has never had surgery to remove the tumour so his life is in danger.

One of the world’s leading experts in the treatment of pituitary giantism is the Harley Street specialist Professor Michael Besser “Pituitary giantism is due to excessive growth hormone secretion from the pituitary gland”. The pituitary gland might be tiny but, its vitally important. It manufactures most of the hormones that keep the body functioning normally, including growth hormone.

When a tumour grows on the pituitary gland, because of the proximity to the optic nerve, it can cause blindness and in some cases brain damage. In cases if pituitary giantism the gland responds by pumping out more and more growth hormone.

If you grow to a height of seven feet or more, the entire body begins to fail under the strain. The knees and spine begin to crumble and the internal organs begin to struggle with the increased workload. Many untreated patients have died of heart failure. Pituitary giantism can be treated, but the treatments are not available everywhere.

Robert Wadler

The first line of treatment is surgery, a procedure called a transphenoidal operation in which surgeons gain access to the pituitary by going through the nose. They break through into the space beneath the brain and, avoiding the optic nerve, scoop out the offending tumour. If the tumour is small there’s and 80% chance of success, when the tumour is large that reduces to 20%. It then rquires medication to replace or balance the hormone production.

The tallest man who ever lived was Robert Pershing Wadlow of London. He was a pituitary giant of eight feet eleven inches, He died at the age of 22 from heart failure.

After he left school, Leonid Stadnyk studied and qualified as a veterinary surgeon and worked on the local collective farm. As he grew taller it became more difficult and as his condition worsened he could no longer travel to work.

While his mother was in hospital, Leonid was persuaded to give a blood sample and this along with his medical record was sent to Professor Besser. What the records reveal is that when he was 12 years old, his Ukrainian doctors did discover that he had a pituitary tumour but, they were less interested in the effect of the growth hormone levels than they were in the damage it was doing to the brain.

A large tumour can cause hydrocephalus. The tumour blocks the circulatory movement of fluid around the brain. Surgeons did insert a shunt to allow the fluid to drain, but they never touched the tumour. The reports are confusing, they appear to indicate that Leonid has had the tumour removed but, there’s no record of any surgery.

Professor Besser visited the Medical Centre in Kiev where Leonid had originally been operated on and where doctors had more recently taken blood samples and carried out an MRI scan. Professor Besser was amazed to discover thet the growth hormone levels were normal and whre the tumour should have been on the scan was only a small smudge, The tumour had died off on its own which was very fortunate as the growth, as it had been, would have been inoperable.

CREDITS: All of the above information came from the UK television’s Channel 5 Extraordinary People series

Lawrence Koomson The Boy Who Never Grew Up

Stephen July 9, 2015 Extraordinary People

Male, 33, Seeks Puberty

Lawrence Koomson

Lawrence Koomson, a doctor living and working in London, is frequently asked: “Doctor, how old are you?”. He has never had acne, has never had bodily hair, and has never had an erection. But, this is about to change, as over the next six months he will go through puberty - at the age of 33.

Dr. Pierre Bouloux, an endocrinologist, has tested Lawrence’s blood testosterone level and finds it is less than 0.5. The reference testosterone level for a men of his age is between 9.9 and 27.8. Lawrence has the testosterone level of a one-year-old.

Lawrence Koomson has a condition known as Kallman’s Syndrome where the hypothalamus, an area in the brain, is not functioning correctly, causing a hormonal imbalance. Kallman’s Syndrome predominantly affects men, but can also affect women.

Dr. Piierre Bouloux

Dr. Bouloux proposes a treatment to boost his testosterone levels. The treatment will consist of six small transdermal implants in the buttocks, which will release 200mg of testosterone over the next six months. Te implants can be placed in the abdomen, it is a matter of patient choice.

As a teenager in Ghana, Lawrence was quiet and withdrawn, and did not show the usual teenage interest in rough and tumble sports or in girls. Instead, he stuck to his studies, at which he excelled and qualified as a doctor. He moved to London 2 years ago, where he works in a local hospital.

His lack of testosterone mean that his muscles and sexual organs have not developed fully, and he has no sexual urges or fantasies. He has had a girlfriend in the past. Ten years ago he was seeing Ruby, but it was very much a platonic affair and was destined for failure for this reason. Lawrence did not confide in Ruby about his condition.

Gerry Koomson

Three days after the testosterone implant treatment he awoke to his very first erection. He was ecstatic, but scared as he had never experienced anything like it before. He talks about getting voice training to lower his child’s voice and brother Gerry jokes about him ending up sounding like Barry White. Gerry is only three years older than Lawrence, but they look more like father and son.

Lawrence is encouraged to begin exercising to help build up his muscles, so he begins jogging and joins the local gym. Unfortunately, this does not last long. In typical pubescent teenage style, his mind is elsewhere, he rebels at being told what to do, and he cannot concentrate on anything for long.

Within a few weeks, he is having erections seven or eight times a day, and he is suddenly aware of women. He is very unsure of himself, an intelligent, well-educated, medically qualified man, he cannot understand the physical responses in him, or the mental turmoil he is going through.

Lawrence meets with Dr. Bouloux again to discuss his feelings and the inconvenience of the perpetual erections. The doctor suggests taking a cold shower or masturbation to relieve the erection. Lawrence is not in favour of masturbating as his upbringing makes him think it is wrong. Dr Bouloux explains that 99% of men masturbate, even those who claim they do not.

It is now six months since he received the testosterone implants and Dr. Bouloux examines him and finds considerable growth in his penis and scrotum. His blood testosterone level has risen to 37.3, way above the average. Lawrence is delighted, he thinks he is now a full-grown man.

Ruby the Girlfriend

Shortly after this, Lawrence is ready to tell Ruby the truth about his Kallman’s Syndrome. Ruby’s reaction is to take him under her wing and find him a girlfriend. She takes him shopping for clothes and after-shave, albeit he isn’t actually shaving yet. But still Lawrence seems to be unable to take the plunge.

After a few weeks, Lawrence has moved out of his hospital digs and moved in with Ruby. Lawrence is much happier with the new living arrangement, but they are still, very much, “Just Good Friends”.

As we leave him, Lawrence is still a virgin. He is not yet ready for sex and is worried about what his performance will be like if and when it should happen.

CREDITS: All of the above information came from the UK television’s BBC series entitled “One Life”

George Widener | Prodigious Savant

Stephen July 9, 2015May 11, 2016 Extraordinary People

George Widener - Prodigious Savant

George Widener

Give George Widener any date in history and he can, within seconds, tell you what day of the week it was. At his home in Asheville, North Carolina, George Widener works into the night drawing elaborate calendars. Before bed he reads, not a novel but, printed sheets of population figures. Numbers follow George wherever he goes, he finds comfort in them, he considers them his friends. George says no-one ever taught him to calculate calendars. In fact, he insists he’s not calculating at all.

Date Genius

George’s skill is phenomenal, but it comes at a cost. He suffers from a form of autism, a brain disorder that leads to awkwardness and social isolation.

As a child, school was a place of fear and dread, but a chance encounter at his grandmother’s house when he was seven changed his life. He met another child who was fascinated with his grandmother’s calendar. Today, George is perhaps the world’s greatest calendar calculator, but he is not unique. He belongs to a select group of people known as savants.

Psychologist Darold Treffert has studied savants for over forty years. He says: “A savant, by definition, is somebody who has a mental handicap of some sort with, what I call, an island of genius”. Savant skills come in a spectrum:

Splinter Skills those gifted in memorising licence plates and sports trivia
Talented Savant those with a conspicuous skill in relation to their handicap
Prodigious Savant those whose skill would be spectacular even if seen in a normal person

The achievements of prodigious savants are all the more remarkable given the handicap they often carry. Kim Peek is physically and mentally disabled but, he can read 500 pages a minute and has memorised 9,000 books. Leslie Lemke and Derek Paravicini are both blind and mentally disabled but can play back a piece of music after hearing it only once. Daniel Tammet can perform incredibly complex mathematics in his head and can learn new languages in a very short period of time.

How do prodigious savants like George Widener do what they do? As recently as a decade ago, scientists could only guess. But now, the latest technology allows them to probe more deeply.

Top neurologist, Joy Hirsch, is going to try and discover which regions of George’s brain are activated when he does his extraordinary maths. While scanning his brain, Hirsch will present him with a new question every four seconds. Some he must read from a monitor, other he will listen to on headphones; the experiment lasts an hour.

Incredibly, Hirsch discovers that George’s brain, while structurally normal, is mysteriously ablaze with activity in unexpected areas while he’s calculating dates. Three of these areas are normally used to recognise words, shapes and to initiates movement. Why George Widener uses the motor areas of his brain to do his maths is unclear, the results hint that George Widener has an unusually organised brain, but Hitsch needs more proof before she can conclude that he is, indeed, wired differently.

Since the average person cannot do what George can do, Hirsch devises a second test that, pretty much, anyone can do, to see exactly where his brain is different. She asks him to name pictures of everyday objects. This time, what Hirsch discovers is extraordinary.

Language and Vision Centres

These are the language and vision areas of the brain that most people would use to describe everyday objects. Shockingly, George’s brain shows no activity in these areas.

If Hirsch’s results are any indication, then savants like George, do indeed, have brains that are wired differently. But, her significant discovery poses a new question. How do the brains of savants become mis-wired in the first place? Some believe that savants are born with their astonishing skills.

Darold Treffert

For Darold Treffert, the story of how savants brains emerge begins inutero as the brains two hemispheres battle for supremacy. The hemispheres generally perform different functions. The left id considered dominant in language and logical thinking, the right in art, maths and musical abilities; the domain of savants. In the foetal brain, the right hemisphere will often finish its development ahead of the left. This leaves the left susceptible to a host of agents that can disrupt brain function. The lead culprit, Treffert believes, is the male hormone testosterone. He tells us: “In the developing foetus, the levels of testosterone reaches the same levels as an adult male”. Treffert believes that in rare cases the flood of testosterone can interfere with the assembly of brain’s neural circuitry resulting in a tangle of mis-wired connections.

With the left hemisphere compromised, the right compensates. In this way, the savant is free from the normally dominant logical influence of the left giving freer reign to the creative right. In keeping with this theory, George Widener possesses other unusual abilities associated with the right hemisphere. He is an exceptional artist, he has an enhanced appreciation of detail and a draughtsman’s ability to record it.

It’s not just brain impairment at birth that brings about savant skills. There’s powerful evidence that brain damage can trigger the release of hidden talents much later in life. Tommy McHugh is one such case.

External Links

George Widener, Savant - Wikipedia Page

CREDITS: The above information came from the UK Channel 5 “My Brilliant Brain” documentary series.

Kim Peek - Savant, The Original Rain Man

Stephen July 9, 2015 Extraordinary People

Speed Reader and Immense Memory

Preceding the savant Daniel Tammet by several years, Kim Peek is the real life Rain Man whom the Dustin Hoffman character was based in the movie. Described as a confounding mixture of disability and brilliance, Kim is in love with knowledge. Kim Peek is probably the world’s most famous savant. Kim was diagnosed as being mentally retarded at birth, but with father Fran’s unflagging support he has developed a memory that is without equal.

Born and raised in Salt Lake City, Utah. Kim has lived here all of his life. The local library is Kim’s favourite place in the world. Kim devours books on anything and everything, as many as eight in a day. He reads at a phenomenal rate, a page that may take you or I three minutes will take Kim about 10 seconds. He reads the left page with his left eye and the right page with his right eye and will retain about 98% of it. He has 15 subject areas but about the only thing he can’t do is, he can’t reason out mathematical problems.

In 1951, doctors wanted to place Kim in a mental hospital named American Fork. Fran was determined that Kim would not go to an institution, he would be raised at home as a normal child.

Fran and Kim’s mother parted in 1975, leaving Fran as Kim’s sole carer. Fran is now almost 80 years old. Kim says “I wasn’t supposed to make it past about 14, and yet here I am at 54, a celebrity!”.

As a child, Kim was very introverted. It wasn’t until he was 37 when he met Dustin Hoffman, that he could look into someone’s face. Fran relates that “Dustin Hoffman said to me, you have to promise me one thing about this guy, share him with the world. And pretty soon it got so that nobody was a stranger to him, they were people, and so was he”. Barry Morrow, the Rain Man screenwriter says of Kim: “I love the way he’s flowered, it belies the myth that people don’t change, especially people with developmental disabilities”.

Fran took Dustin Hoffman’s advice to share Kim with the world. The once introverted Kim has now appeared in front of more than 2 million people, all eager to test his genius with obscure questions.

Kim’s gifts come at a price. Like all savants, Kim is an acutely different man, sometimes understanding Kim can be a challenge. Kim can become very agitated when trying to understand a new concept, but Fran has phenomenal patience and will calm Kim by gently changing the subject to something Kim is happier with.

Looking after Kim is more than a full-time job, Fran jokes that he needs 30 hours a day and 10 days a week. Kim knows how essential his dad is to him: “My dad and I share the same shadow!”

This year is a landmark year for Fran and Kim, a fact that Kim is unusually aware of. He says “Things will really heat up this winter, with my dad approaching 80. Only one other member of my dad’s immediate family has ever reached that mark, it’s my Grandma, which makes this year so important to us”. So this year the Peek’s have set themselves a mission to find out everything they can about Kim’s brain. They are going to California to meet some of world’s foremost neuro-scientists to find some answers while they’re still together.

Most savants have only one dominating interest, Kim seems to soak up everything. His interests range from boxing, to politics, to the British Monarchy.

Dr. Darold Treffert is an authority on Savant Syndrome and consulted on the Rain Man movie. “About once a century comes along a truly stellar savant, an already rare condition, and Kim is in that category” explains Dr. Treffert. “Kim’s memory is not only deep, it is also very wide which is very unique among savants” he continues.

What is it that makes Kim’s brain work in such an extraordinary way, and do his disabilities, in some way, enhance his memory? In California, Kim has been invited to take part in a study at the University of California.

Neurologist Dr. Elliott Sherr begins by going over Kim’s background. This is something of a sore point as the first neurologist Kim saw, when he was only a baby, took 5 minutes to dismiss him as a hopeless case who should institutionalised. Dr. Sherr was to discover that Kim had learned to read by the age of two. While this was not a formal neuropsychological assessment, it was plain that Kim had difficulty following directions.

Psychologist Dr. Rita Jeremy gives him a standardised intelligence test to see where he stands in relation to the norm. She discovers that Kim has trouble with tasks that require new thinking and for which he can’t call upon facts from his memory. His results are wildly erratic but Dr. Jeremy is philosophical and decides that a standardised test cannot apply as Kim is most definitely not standard.

The following day, Neuroradiologist Dr. Pratik Mukherjee will use Diffusion Tensor Imaging, a ground-breaking way of mapping how the brain functions, to look inside Kim’s head. This reveals that the two halves of his brain are not joined in the normal manner, a condition known as agenesis of the Corpus Callosum.

The fibres that should have made this connection are travelling in different, peculiar directions. Has the way Kim’s brain rewired itself to compensate for the missing Corpus Callosum account for his incredible memory?

Next, Professor V.S. Ramachandran wants to test a long-standing theory that savants are not capable of conceptual thinking. Professor Ramachandran and his students run a number of word association tests which appear to prove that Kim simply accepts words at face value without processing them in any way. He takes metaphors literally. All of which seems to back up the theory.

Kim Peek may be the greatest savant we have ever known, he is certainly the best known and more than entitled to be referred to as a mega-savant.

He can recall thousands of zip codes, phone codes, dates and countless facts and figures. He has a truly awesome memory. Is he autistic? Probably, but like Daniel Tammett, not in the conventional sense. He was originally diagnosed with autism but this was later changed.

Tommy McHugh An Accidental Genius

Stephen July 9, 2015 Extraordinary People

A Fortunate Accident?

Following the story of the savant George Widener, whose incredible skills result from brain damage at birth, we consider the evidence that accidental brain damage can trigger the release of hidden talents much later in life. One such case is Tommy McHugh. Until five years ago, this Liverpudlian builder had never picked up an artist’s brush but, ever since a near-fatal brain injury Tommy has been consumed by a powerful compulsion to paint.

One morning, Tommy was sitting on the toilet when he was rudely interrupted. A knock at the door prompted him to try too hard to evacuate his bowel resulting in a sudden increase in blood pressure that caused two arteries to rupture in his brain, one on either side haemorrhaging a large quantity of blood.

Surgeons worked quickly to save Tommy’s life. They inserted a wire coil to plug the leak on the right side of his brain and applied a metal clip to the left to prevent further injury.

When he was released from hospital, he couldn’t walk, he couldn’t eat, he didn’t know that he needed to eat. Once he started to walk, his head became full of notions, things he wanted to say, things he wanted to get out, so he began writing poetry - obsessively. He recalls: “The more I wrote, the more I wanted to write, it was like a drug”. But, Tommy’s poetry was just a prelude to his madness to come.

Tommy’s wife, Jan, was in the kitchen when she got her next shock. Tommy was drawing hundreds of alien-like faces all with screaming, agape mouths. This was his inner torment being expressed.

Tommy’s artistic mania has threatened to overtake his surroundings and no-one can accuse him of not making the most of the available space. He has paintings in every room, on every surface, even the ceilings. His talent might fall short of Michelangelo’s but he makes up for it with his manic drive. It was this drive that, ultimately, became too much for Jan.

It all came to a head when Jan, a houseproud woman, realised that the house was filling with smoke. She went upstairs to investigate and found Tommy melting wax candles, blackening himself, the room and the house. Tommy’s response was “You don’t know who I am, do you?”.

What had happened to Tommy McHugh? Nobody seemed to know. Alone and desperate, he sent off nearly sixty cries for help, written in rhyme, to doctors around the world. Unknown to Tommy, nearly 3,000 miles away, a Harvard neurologist would understand exactly what Tommy was going through.

Alice Flaherty, a leading expert on the effects of brain injury on behaviour, had discovered from first-hand experience how unpredictable the brain can be. She had suffered the still-birth of her twins after a complicated and traumatic pregnancy. She immediately felt very sad at her loss but, after about ten days she was overcome with the compulsion to write everything down. She believes that the traumatic birth led to a biochemical change in her brain that resulted in her manic compulsion to write.

Alice turned to psychiatric drugs for help. Eventually, she found the ones that curbed her mania. As word of her experience spread, writers and artists with similar conditions started flocking to her door.

Luckily for Tommy McHugh, one of the dozens of letters he’d sent off landed on Alice Flaherty’s desk. She immediately understood, and felt a bond with Tommy. She wanted to meet Tommy as she believed she could help him better understand what had happened to his brain.

Tommy has a criminal record which prevents him entering The United States, but touched by his letters Alice travelled to England for their first ever meeting. To Alice, the full spectacle of Tommy’s mania isn’t so unfamiliar. The Harvard neurologist quickly finds herself becoming immersed in Tommy’s strange world.

In anticipation of Alice’s visit, Tommy has asked his surgeon for his medical records to show her. After studying his scans, Alice believes that the bleed from Tommy’s aneurysm produced his overwhelming urge to create by putting pressure on two critical areas of his brain; the frontal lobe which generates ideas and the temporal lobe which controls their orderly release into the world. In Tommy’s brain the delicate balance these two areas has been upset causing a manic outpouring of ideas. Alice explains: “This balance is now unstable in him and gives mood fluctuations, gives creativity, gives suffering and sometimes it’s hard to tease them apart. Typically, when someone has a big burst of output after a tragedy, it will die away. With Tommy it’s still going strong after five years and I don’t think this is ever going to stop”.

For Tommy his horror story has a happy ending. He acquired his savant like ability through a near fatal trauma but he is, in his own slightly-crazy way, content.

CREDITS: The above information came from the UK Channel 5 “My Brilliant Brain” documentary series.

Foetal Theft | She Stole my Foetus | Unborn baby Abduction

Stephen July 9, 2015May 11, 2016 Extraordinary People

Foetal Theft - Stealing Unborn Babies

Deborah Evans and Children

Foetal theft or the abduction of unborn babies, sounds bizarre, but the horrific reality is that it is happening.

On the 16th of November 1995, in Addison, Illinois near Chicago, Deborah Evans, a 28 year old pregnant mother and her 10 year old daughter and 8 year old son were found murdered at their home. Jordan, her 19 month old son was found unharmed at her side, having lain alone for six hours before his discovery. Deborah’s father, Sam Evans, said: “She was killed for her baby. They actually cut her unborn baby from her womb as she was dying.”

Targeting Pregnant Women

Police launched a massive search for the child cut from Deborah’s womb. She had already named him: Elijah. 2 days later the Police received a tip-off about a woman showing off a new-born baby who it was suspected was not the baby’s mother.

Elijah was found alive and unharmed, in the arms of Jacqueline Annette Williams. Jospeh E. Birkett the Attorney General of DuPage County described the crime: “It’s horrible, it’s as bad as the human condition can get.”

Fedell Caffey, Jacqueline Williams and Levern Ward

Investigators discovered that Deborah knew her killer. Jacqueline had befriended her claiming that she too was expecting her fourth child. She had, in fact, conducted an elaborate charade to convince all around her that she was pregnant. Her crime was months in the planning and showed Jacqueline as a cold, manipulative con woman.

Boyfriend Fedell Caffey protested his innocence, but the evidence said otherwise. Police also arrested Jacqueline’s cousin, Levern Ward who, it transpired, was Deborah’s ex-boyfriend and father of both Jordan and Elijah. Jacqueline Annette Williams will now spend the rest of her life in prison.

4 months later in Tuscaloosa, Alabama another woman’s body was found with horrific abdominal injuries. She too had been 9 months pregnant. The abducted child was found with Felicia Scott in what was almost a copy cat scenario from Chicago. Psychologists and Psychiatrists began to see a pattern in the character and the behaviour of women who kill other women for their babies.

Psychiatrist Professor Michael Welner

Psychiatrist Professor Michael Welner of NYU School of Medicine is undertaking a study to build a profile of offenders and the dynamics of their foetal theft crimes. he believes that a key element is a past or recent relationship which has broken down or is endangered.

Psychologists who evaluated Felicia determined that she was not insane but had a personality disorder, a narcissism so extreme she could not appreciate the suffering she had inflicted upon her victim. These narcissistic tendencies mean the world revolves around the individual. Everything is about them, about what is best for them.

Professor Katherine Ramsland is one of America’s leading forensic psychologists. She specialises in the workings of the criminal mind and has written over 25 books on the subject. She believes that these crimes are narcissistic in nature, where victims are moved around like pawns and the perpetrator is likely to feel victimised themselves when things go wrong.

Since Felicia Scott was sentenced to life imprisonment, there have been 5 other cases of ceasarian homicide. In every case the woman refuses to admit any responsibility or any remorse.

Until June 2004, all recorded case of foetal abduction were in the United States. This changed with Luzmilla Fierra in Hirondelle, Colombia near the capital Bogota. She is in prison on remand accused of foetal theft. She is also the first woman to talk on camera about the incident. Although, in her mind, she is the victim not the culprit.

Luzmilla Fierra

It began in the local hospital when she noticed a pregnant woman in the waiting room. The woman started talking to her, alleges Luzmilla, she said that she had lots of problems with her husband and her husband hit her. Then she said: “she wanted me to do a favour, and the favour was she wanted me to take out her baby”.

This case is different from the others as here the victim survived. It is believed that burundanga, a plant-extract sedative often used in date-rape crimes, was given to the young mother. Her name is Solangela Cartagena and she tells a very different story.

She remembers going to the hospital for an appointment she says: “I went to get a glass of water for my daughter and for myself. I left it on the table while I went to the toilet. When I came back I drank the water and after that I don’t remember anything. I lost all my memory”.

Solangela Cartagena

Police believe this was the moment when Solangela was slipped a drug. This occurred around noon. Her next memory is 6 hours later when she awoke in the woods. At her side was her 2 year old daughter Nicole. Her daughter woke her with cries of “Mum, Mum the lady left running with the baby”. It was Nicole’s shouts that roused Solangela and enabled her to stumble for help. She was losing a lot of blood and was fortunate to survive.

Again, it was a tip-off that led police to Luzmilla where they discovered she was already a mother of six children aged between six and fifteen.

Professor Katherine Ramsland explains that this is not about having a baby. The fixation is control. Controlling a situation or a person around you. The psychological instabilities may take the shape of getting a baby but most likely point to a deeper issue.

In common with the Chicago cases, psychiatrists believe the key element behind foetal theft is a fragile relationship in the woman’s life.

External Links

Foetal Theft or Abduction - Wikipedia Page

Dystonia - Bodies out of Control

Stephen July 8, 2015May 11, 2016 Extraordinary People

Bodies out of Control - Dystonia

Channel Five continue their Extraordinary People series with “Bodies out of Control”, a very apt title for the thought-provoking and disconcerting look at Dystonia.

Malfunction of the basal ganglia

Channel Five describe Dystonia as a rare condition, but there are thought to be 40,000 sufferers in the UK. Dystonia is thought to be caused by a malfunction in the part of the brain called the basal ganglia, this affects the way sensory signals are interpreted and motor signals are instigated. It causes the muscles to spasm involuntarily leading to twitching, shaking and the contorting of the face, neck and limbs is the most horrific fashion. Dystonia can cause the eyes to blink uncontrollably and even snap shut leaving the patient without sight. It can cramp the muscles of the larynx leaving the patient voiceless.

In the documentary we meet Valerie Cross, Janet Hunter, Joy, Martin and Amy Westall, Bridget Davies and Jacky Lewis, Ann Tonge and Daniel Rabone.

Each has their own story and each have their own way of dealing with this intrusive and debilitating condition.

Janet Hunter

Janet Hunter is a single mother of four who somehow manages to run a household and ensure her children are fed, clothed and sent to school each morning despite being unable to control her limbs and becoming exhausted after a few minutes work. She was, prior to dystonia, a brilliant linguist, speaking French, Arabic and Swahili amongst others. She has tried various treatments but none has worked. She consulted with a Dr. Choudhuri (pronounced Chowdra) who believes her condition is, at least in part, stress related. Janet has discovered that playing the flute allows her to focus her mind on reading the sheet music and playing the instrument. This allows the muscles to relax and gives some respite from the spasms. However, as soon as she stops playing the spasms return worse than ever.

Joy Westall succumbed to Dystonia at an early age. Her first signs of Dystonia began when she was just 9 years old. Despite this she went on to marry Martin her childhood sweetheart. After speaking with specialists and being assured that Dystonia was not hereditary, they decided to try for a family. In 1985 she had a baby girl, Amy, who was quite healthy. Sadly, this was not to stay. By the time Amy was eight years old, she like her mother began to show the initial tell-tale signs of dystonia. By the time Amy was eleven she had full, clear signs of dystonia. Ironically, about this time the specialists changed their position stating that Dystonia was hereditary and could be passed on through the genes.

In 2003 Joy and Amy learned of a revolutionary new operation being pioneered at National College Hospital in London. Known as Deep Brain Stimulation, this involved inserting 2 electrodes into the brain coupled to a battery pack located under the skin of the chest. This is a risky operation and, to date, less than 150 patients have undergone the procedure. Amy’s operation was a success, and almost immediately, her spasms stopped and the pain went. She is now swimming, taking dance classes and learning to drive. This operation had been reported here as being done at King’s College Hospital, however this was corrected after I was contacted by the administrator of www.parkinsonsappeal.com on behalf of Joy and Amy Westall.

Following Amy’s success Joy decided that she too would have the operation which, also, was a success and may mean that Joy will be able to put her foot flat on the ground for the first time in thirty years.

Bridget Davis and Jacky Lewis

Bridget Davis & Jacky Lewis

Bridget Davis and Jacky Lewis became best of friends after meeting at a dystonia society meeting and discovering that they only lived a short distance apart. They both suffer from blepharospasm affecting the eye muscles. They often spend time walking where they take it in turns to be the other’s eyes. Bridget and Jacky have received botulinum toxin (Botox) injections around their eyes to ease the symptoms.

Ann Tonge had been a keen singer and a self-confessed chatterbox before the laryngeal dystonia reduced her ability to speak, let alone sing. She also receives regular Botox injections in her throat and this has restored her voice and she can sing again, albeit not as well as before.

Daniel Rabone also finds comfort in focussing the mind on a single task. He carries a full mug of liquid with him at all times as he finds this relaxes the muscle spasms in his shoulder. Daniel has had the Deep Brain Stimulation operation, but it did not work. Nevertheless he manages to remain good humoured and continues with his coffee cup.

Forms of the condition

Cervical Dystonia, Spasmodic Torticollic, Antecollis or Retrocollis. This form targets the neck area.
Blepharospasm affects the eye muscles.
Oromandibular Dystonia affects the mouth or jaw
Laryngeal Dystonia affects the throat or larynx
Segmental or Generalise Dystonia (dystonia muscolorum defoemans) affects many parts of the body

Some forms of dystonia may also be referred to as: Meige’s Syndrome, Breughel’s Syndrome or Segawa’s Disease.

External Links

About Dystonia - Neurological Movement Society

Lucica Bunghez - Megatumour

Further Reading

Hydrocephalus

Water on the Brain

Phrenology

Further Reading

Lori and Gary Hoogewind, Michigan

Pituitary Giantism, Gigantism

Further Reading

Male, 33, Seeks Puberty

George Widener - Prodigious Savant

Date Genius

Darold Treffert

External Links

Further Reading

Speed Reader and Immense Memory

A Fortunate Accident?

Further Reading

Foetal Theft - Stealing Unborn Babies

Targeting Pregnant Women

Psychiatrist Professor Michael Welner

Luzmilla Fierra

Solangela Cartagena

External Links

Bodies out of Control - Dystonia

Malfunction of the basal ganglia

Janet Hunter

Bridget Davis and Jacky Lewis

Forms of the condition

External Links