Episode 3 Incredible Medicine: Dr Weston's Casebook


Episode 3

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We're discovering astonishing things about the human body all the time

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through people who are different from most.

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I'm Gabriel Weston.

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As a surgeon, I've spent years studying the human body.

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And the secrets of how it works are often revealed by the most

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rare and surprising of cases.

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So I've searched the world to find these extraordinary people

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and bring you their stories.

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This is my heart. I'm the only one that has this.

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I'm Jordy Cernick, and I can't feel fear.

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My name is Harnaam Kaur, and I'm a fabulous bearded lady.

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With the help of the doctors that treat them and some of the

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world's leading scientists,

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I'll be uncovering exactly what makes their bodies unique.

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'I'm going to show you the hidden

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'processes that make them exceptional.'

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Just look at that!

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I'll discover how they're leading us to the cures of the future.

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When we make a breakthrough like this, it is very exciting.

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And I'll use the latest technology to uncover the secrets of

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their bodies and reveal how all of these cases are giving us

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a new understanding of the most amazing natural machine on

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the planet - the human body.

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'There are some extraordinary people who have talents and

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'abilities that seem almost superhuman.

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'In this programme, we'll discover why this woman can see

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'millions of colours most of us can't...

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'..and why this man never forgets a face,

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'why this woman can smell disease before it happens

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'and why this man has never felt pain in his life.

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'I'll reveal how these remarkable people are helping us understand

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'the most complex and mysterious part of our body, the brain.'

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And I'm going to start with the strange case of

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a man who awoke one morning with an astonishing new talent.

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October 27th 2007 was a day

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that would change Derek Amato's life forever.

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I woke up and I could suddenly just play the piano.

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Derek had never touched a piano before,

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so how could he possibly become a virtuoso pianist overnight?

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It began with a misadventure that might have killed him.

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I was visiting some friends for a barbecue by the pool.

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We were throwing a little football around,

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and I just decided to go running along the pool and dive into the

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shallow end to catch the football.

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I struck the bottom of the pool with the upper left side of my head.

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There's a moment when you hit your head that you know that

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that noise is traumatic and you know something's definitely wrong.

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Derek was rushed to hospital,

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where he was treated for severe concussion.

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He was sent home and slept for five days.

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When he finally came round,

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he felt an uncontrollable urge to do something he'd never done before.

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My hands started basically understanding where they

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were supposed to go and my brain seeing these black and white

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squares telling my fingers what to do.

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And then my brain started seeming like I started racing, to the

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point where I was going...

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..and then I was on overload.

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What's so surprising is that Derek can't read a note of music.

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He'd messed around with a guitar and drums as a child,

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but he'd never actually learned to play an instrument.

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Now beautiful melodies were tumbling out of him.

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I had to play all the time, because it felt like if I didn't play,

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it was like it was building up,

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as almost if you were just pouring musical notes into my brain

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and it was just filling up.

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I just didn't know what to make of it.

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I didn't know if I should tell someone,

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because they're going to think I'm

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nuts anyway, because I already had a head injury and now I'm telling

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you I'm a piano player, so someone's going to have some questions for me.

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Derek's story seems unbelievable, but he's not alone.

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In 1988, Jon Sarkin, a chiropractor from New Jersey,

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suffered a massive stroke and went into a coma.

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When he awoke, he felt a sudden compulsion to draw and paint,

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and he's a prolific artist to this day.

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And in 2002, Jason Padgett, a furniture salesman from

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Washington, was attacked outside a bar and suffered severe concussion.

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When he recovered, he'd become a mathematical genius who saw

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the world in complex geometric shapes.

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It's a rare but recognised condition called acquired savant syndrome,

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and what happens in it is that after damage to the brain,

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an individual develops an ability that they never had before,

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and scientists are trying to figure out what's happening in the

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brains of these extraordinary people.

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Professor Berit Brogaard is a neuroscientist who studies

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acquired savant syndrome at the University of Miami.

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She's been using MRI scans to look deep inside Derek's brain.

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You see some white spots here, and there's

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a small white spot here that we are particularly interested in.

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These white spots are damage caused by Derek's concussion.

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One of them lies in a part of the brain called the prefrontal

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cortex that's involved in rational and logical thinking.

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With this part damaged,

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Derek now needs to use other parts of his brain.

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When you give up on logical thinking and rational decision making

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a little bit, you have the possibility of developing new

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talents or become more creative,

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more original, so original thinking,

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thinking that actually results from not using the prefrontal

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cortex but using other areas of the brain.

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Berit believes that Derek's brain has quite literally rewired itself.

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After brain damage, a powerful chemical called serotonin

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encourages nerve cells in the brain, or neurons, to make new connections.

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In Derek's case, these connections seem to have been established

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in a part of the brain involved with creative thinking...

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..which may explain his sudden musical talent.

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And Berit's research suggests we might also have hidden talents that

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could be unlocked by stimulating the brain in the right way.

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In the future, we can use magnetic stimulation

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or electric stimulation or perhaps a pill to unlock the abilities

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that we see unlocked in people with acquired savant syndrome.

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We can already improve people's drawing abilities and math

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abilities, and it will only be a matter of time before we can

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make people great musicians or great mathematicians.

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What I think is the greatest gift is human potential,

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and I think we have it in all of us.

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It's not just me. Maybe I hit my head just right,

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but I do believe that it's in all of us.

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Derek's case shows how a small change in the brain can have

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a huge impact on the person we are.

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And there's one case in the history of medicine that was the

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first to demonstrate just how much our brain controls.

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Phineas Gage was an American railroad construction foreman

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who, in 1848, defied the odds by surviving an accident in which

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a large metal rod was driven all the way through his head,

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nearly destroying his frontal lobe.

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Now, when people were done being amazed that he'd survived,

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what they noticed was that he was a different man.

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Previously reliable, he became unpredictable and impulsive,

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and he stayed that way for the last 12 years of his life.

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His was the first case to show that the brain affects the personality.

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Until then, it was believed that our

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personality and abilities were God-given.

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But this case demonstrated

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that they're actually determined by our brain.

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Nearly 200 years later,

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we're still only scratching the surface in understanding what

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all the different parts of our brain do and how they affect us.

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And to unlock some of its secrets,

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the next few cases I'm going to look at are remarkable people who

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see the world around them in extraordinary ways.

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James Rabbett is a man with an astonishing talent.

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He can recognise just about every face he's ever seen,

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even one he's only glimpsed for a few seconds...

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..an ability that's beyond most of us.

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Sometimes, when I'm going to meet someone on the weekend, I say,

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"Let's meet at Waterloo station."

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Because my brain is middle-aged, I can find it quite an effort looking

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amongst all the people there to try and pick out my friend

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or my brother or whoever it is I'm going to meet.

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But James is different. He's what scientists call a super recogniser,

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and he's about to have that ability tested to its limit.

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Hello!

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Dr Josh Davis is a psychologist from the University of Greenwich

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who studies super recognisers.

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He's trying to discover the full extent of their powers of

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recognition and what can explain them.

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Waterloo station, with these large crowds of people.

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You've got to try and find my four actresses,

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who have hidden themselves away in the crowd or be wandering around.

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-It's really, really difficult.

-A big challenge, yeah?

-Yeah.

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-It's really busy.

-So, here you go. Here are four targets.

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What Dr Davis is asking him to do is have

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a quick look at four photos of actresses that he's never

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seen before and then look down on the concourse at Waterloo and

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pick out these individuals

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from in amongst thousands and thousands of other people,

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none of whom James has ever seen before.

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-I'm intrigued to see how you get on.

-It's going to be really challenging.

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This is probably one of the hardest tests I've done, I think.

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It's only recently that the very

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existence of super recognisers was discovered.

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Interestingly, James was working already as

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a detective in the Metropolitan Police at Scotland Yard

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and part of his job was to look through hours and hours of

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footage of the 2011 riots, and he was much, much better at

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picking individuals out from the footage than anyone else.

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And he's actually become part of a very small team of detectives who

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are known to be super recognisers and who are able to look at lots

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and lots of photos of suspects and crimes and pick out the right

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person in order that convictions can be secured.

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So, what is it that gives these super recognisers their

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extraordinary powers?

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Well, we've only known about them for a short time,

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so scientists are only just now beginning to uncover their secrets,

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and it seems like the answer lies in a particular part of the brain.

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Within our brain, this part, the temporal lobe,

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is involved in perception and memory.

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Deep inside it, scientists have identified a small area on

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each side that becomes active when we recognise a face.

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It's known as the fusiform gyrus, and it's thought to process visual

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information from our eyes and allow us to recognise individual faces.

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Scientists are now beginning to investigate whether the

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fusiform gyrus might be especially powerful in super recognisers.

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It may be that in super recognisers, this area may be working more

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effectively, or it may be just passing information across

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the brain more effectively.

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We don't really know, but this is the sort of research that we

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are interested in doing in the future.

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At Waterloo station, James is about to try and identify four people

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he's never seen before after just a brief glimpse of their photos.

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-Black leather jacket, blue jeans.

-Brilliant!

-Pink laces!

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Well done, because I thought she was the hardest.

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-Is it the lady in the black jumper, cream top and blue jeans?

-Brilliant!

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That was difficult.

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White top, blue jeans, sandals.

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Do you not think she looked so different in...?

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Yeah, they are very, very different.

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-This is her here with the handbag, blonde hair.

-Yeah.

-There she is.

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Brilliant, well done.

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-Congratulations.

-Thank you. Cheers!

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It's impressive to see James do what's utterly beyond most of

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us and correctly identify all four faces in a crowd.

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When you were looking at the photographs at the start,

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what were you trying to learn when you were looking at them?

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I knew that they weren't going to be made up as they would have

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been on the evenings when they were out having these photographs taken.

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It's trying to get as much of the stable facial features as you can.

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With yours, I had to take myself away from the fact that you

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might have red hair or blonde hair or brown hair or whichever

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colour hair you decided to have on the day,

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so I just had to really focus on the nose, the mouth, jawline,

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because I know that they were not going to change.

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Robbie's wearing glasses. She's not wearing glasses in the photos.

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-And again, you still see through that.

-Yeah.

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You've got a distinctive jawline, your nose, the mouth as well.

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You know, these features don't change.

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Do you not think that's quite impressive?

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-Could you have done it?

-No! I wouldn't have been able to do it.

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It looks like we've done all right!

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It's clear from the point of view of criminal justice why people

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with this amazing extra quality are so useful.

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But for scientists, working with super recognisers like James

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is bringing a new understanding of how we all recognise other people

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and of the complex and vital relationship between our eyes

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and our brain.

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It's that connection between our brain and our senses - vision,

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hearing, touch, taste,

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smell - that tells us everything we know about the world around us.

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And our senses are some of the most intricate and sophisticated

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systems in our anatomy.

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And just look at the eye.

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It really is one of the wonders of the human body.

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Now, this complex biological camera delivers all sorts of

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information to us in three dimensions and technicolour,

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but I've come across a case recently

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of someone who can see even more vividly than the rest of us.

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I'm Concetta Antico, and I can see colours that nobody else can.

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Like any artist, Concetta has an eye for colour,

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but while most of see the world in a palette of a million colours,

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Concetta can see up to one hundred million.

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I really enjoy painting.

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My eyes were always drawn to light and things of beauty,

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things that were brightly coloured, and I felt this passion to

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paint those images or those colours that I was seeing

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from a very early age.

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It affects me from the moment I wake up.

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The light that's coming in is affecting my mood.

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I walk down the street, and I'm not just walking down the street,

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I'm looking at the colour of the little stones in the

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concrete in the sidewalk.

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Life with an almost superhuman

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ability to see colour can be pretty intense.

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'Shopping, for me, is difficult. It's too much.'

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Can we go back to the old days, where there was just one

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bottle of, you know, tomato sauce and one can of beans?

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As an artist and art teacher, Concetta spent years

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describing colours to her students that they simply couldn't see.

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She had no idea why she was seeing colour differently until by chance

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she heard of a rare condition called tetrachromacy that

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affects the eyes.

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It's the structure of our eyes that allows us to see colour.

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If I dissect into it here, you can see just how complex it is.

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At the front, we've got the iris and the pupil, the lens at the back,

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and just here is the retina, where the image is formed.

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On the retina are special cells called colour receptors, or cones.

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These cones are triggered by different wavelengths of light,

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and they fire off signals to our brain,

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which combines them to produce all the colours we can see.

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Most of us are trichromats, meaning that we have three types of cone.

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But research suggests that some people are tetrachromats.

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They have an extra fourth type of cone,

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and this could allow them to see millions more colours.

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To find out if she might be a tetrachromat,

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Concetta began to contact scientists working in the field.

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Dr Kimberly Jameson at the University of California, Irvine,

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has been studying how we see colour for 20 years.

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She agreed to test Concetta.

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We're going to look at three colours from the same colour group,

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and what I want you to do is pick the odd one out.

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One of the three colours is very slightly different,

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but only a tetrachromat would be able to see it.

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-What number?

-21.

-21. OK. That was correct. That's the odd one out.

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Three more. Look at them carefully.

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Yep!

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Good job, got all three correct.

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Kimberly put Concetta through a series of different tests.

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Try to make a smooth colour continuum and do it as

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quickly as possible.

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-That's kind of pulling them into the zone.

-Mm-hm.

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-OK, I'm done.

-OK.

-Like that.

-Let's see how you did.

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18, 19, 20, 21.

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Good job!

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So Concetta is able to detect minute differences between shades of

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colour that look identical to most of us.

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Kimberly has established that she IS a tetrachromat.

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That fourth type of cone in her eye explains the overwhelming

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range of colours she's been experiencing all her life.

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And what's really fascinating

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is that this extraordinary colour vision may only exist in women,

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thanks to our DNA.

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DNA is arranged in our cells into structures called chromosomes.

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Men have one X chromosome but women have two.

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And it's an abnormality, or mutation, on one of

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those X chromosomes that causes the crucial change to the eye,

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producing the fourth type of cone in the retina.

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This is why it's thought only women can be tetrachromats.

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But it's possible that this superhuman vision might not

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be quite so unusual in the future.

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Human visual systems are still evolving.

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Here we are, perhaps on a cusp of human tetrachromacy

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sort of mutating and emerging and being in a pipeline,

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trying to understand where human vision and cognition is going

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or could potentially go in future generations.

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It is sort of like an amazing problem and an

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amazing set of things to look at.

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Perhaps one day many more women will have the ability to see an

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astounding world of colour, like Concetta, but for now,

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she remains exceptional.

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It is a mutation. I call it a gift.

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Will it become more expressed in our world,

0:23:180:23:21

and what does it all mean? I don't know!

0:23:210:23:24

It's going to be things that I'll never know. I'll be long gone.

0:23:240:23:29

But it's key to me and makes my life have meaning.

0:23:290:23:32

Concetta's eyes, with their remarkable colour vision,

0:23:370:23:40

give her brain a sensory overload,

0:23:400:23:44

but some people perceive the world around them in unusual ways

0:23:440:23:47

because a key part of the sensory system is missing.

0:23:470:23:51

All our senses have vital connections to the brain,

0:23:540:23:58

and I've come across a case recently that shows just how profound

0:23:580:24:02

the impact can be if one of these senses is severed.

0:24:020:24:06

There's one sensory experience that most of us do our best to avoid,

0:24:110:24:16

but Paul Waters has never felt it in his life.

0:24:160:24:20

'I don't feel pain. I feel everything else,

0:24:230:24:27

'I just don't feel pain.'

0:24:270:24:29

Come on, then!

0:24:290:24:30

Paul has never experienced an ache, throb or sting like the rest of us.

0:24:300:24:35

'I can feel pressure as opposed to pain.'

0:24:370:24:39

If I cut myself, for example, I would feel that as a...

0:24:410:24:47

..kind of...

0:24:480:24:50

I would say pins and needles would be the easiest way to describe it.

0:24:500:24:56

Paul's parents first realised something was wrong when

0:24:580:25:01

he was just nine months old.

0:25:010:25:03

There was an occasion where my dad had got in from work and

0:25:050:25:11

he trod on my arm, and my mum jumped immediately and said, you know,

0:25:110:25:16

"You're standing on Paul's arm."

0:25:160:25:18

They then sort of stood back and thought to themselves,

0:25:180:25:20

"Well, hang on a minute, Paul didn't flinch."

0:25:200:25:23

It was a kind of a sign that perhaps they should take me somewhere.

0:25:230:25:28

Doctors tested Paul and later his sisters and diagnosed them with

0:25:280:25:33

an extremely rare condition called

0:25:330:25:35

congenital insensitivity to pain, or CIP.

0:25:350:25:39

For most of us, if we cut our finger with a knife,

0:25:430:25:47

the cut activates nerve endings in the finger.

0:25:470:25:50

The nerve endings send an electrical signal through special cells

0:25:500:25:54

called pain neurons up to the brain,

0:25:540:25:57

and this is what we experience as pain.

0:25:570:26:01

But for some reason,

0:26:010:26:02

this system wasn't working in Paul and his sisters.

0:26:020:26:05

Growing up, me and my sisters would get up to a lot of mischief -

0:26:080:26:13

jumping off of high objects,

0:26:130:26:15

you know, at the risk of breaking a bone.

0:26:150:26:17

If a child breaks a bone, they're going to be in pain,

0:26:170:26:21

they're probably not going to do it again.

0:26:210:26:23

None of those negatives were present in me or my sisters,

0:26:230:26:28

so we used to do a lot of things

0:26:280:26:30

as children that other children wouldn't do.

0:26:300:26:33

An oven, to me, back then was a toy.

0:26:350:26:37

I'd put my hand in the hob just to hear it sizzle.

0:26:370:26:41

Now, it might sound like this condition is actually

0:26:410:26:44

protecting people like Paul from pain and harm...

0:26:440:26:47

..but our ability to sense pain is vital.

0:26:480:26:51

It alerts us to danger and helps us avoid it.

0:26:510:26:54

Not having it can be catastrophic.

0:26:540:26:58

One of Paul's sisters died as a result of an injury she didn't feel.

0:26:580:27:02

And Paul himself now lives with the consequences of

0:27:030:27:07

a lifetime of broken bones.

0:27:070:27:09

It's affected his height, and he needs frequent operations.

0:27:090:27:13

Ready? Scalpel...

0:27:150:27:18

Can we start?

0:27:180:27:20

So, why doesn't Paul experience pain, like the rest of us?

0:27:220:27:27

Well, his condition's very rare, so for years it's been a medical

0:27:270:27:31

mystery, but now one scientist is trying to get to the bottom of it.

0:27:310:27:36

Geoff Woods is a professor of medical genetics at the

0:27:380:27:41

University of Cambridge.

0:27:410:27:43

He had seen that CIP tended to occur in families, like with Paul and his

0:27:430:27:48

sisters, and so he knew it was most likely caused by a faulty gene.

0:27:480:27:53

It was such a rare condition that no-one had worked on it,

0:27:550:27:59

really, no-one saw any potential benefits to the condition,

0:27:590:28:03

and in some ways it was just forgotten about.

0:28:030:28:06

It was that sort of realisation that we could do this,

0:28:060:28:09

it could be a genetic disease.

0:28:090:28:11

Geoff knew that if he could find this gene,

0:28:130:28:15

then he might find the cause of CIP and perhaps even a cure.

0:28:150:28:20

His team began examining the genes of families with the condition,

0:28:220:28:26

searching for a mutation that they all had in common.

0:28:260:28:30

After ten years of painstaking work,

0:28:320:28:35

he eventually found what he was looking for,

0:28:350:28:37

a mutation in one particular gene that all the families shared.

0:28:370:28:43

He knew this had to be a key gene for controlling pain.

0:28:430:28:46

It was called SCN9A.

0:28:460:28:49

The day we sequenced SCN9A, my technician was looking

0:28:520:28:56

through the sequence results,

0:28:560:28:58

and all three families we used to map where the gene was,

0:28:580:29:01

we'd found mutations in them, and so we thought,

0:29:010:29:05

"Gosh, this could really exciting."

0:29:050:29:07

I think people had not expected that a single gene would be able

0:29:090:29:13

to control all pain sense in humans.

0:29:130:29:16

When our nerve endings sense something painful, they

0:29:180:29:21

trigger a surge of charged particles to flow into the pain neurons.

0:29:210:29:26

This stimulates the electrical signal to be fired

0:29:260:29:29

and sent up to the brain.

0:29:290:29:31

The SCN9A gene controls this flow of charged particles, but in Paul

0:29:310:29:37

the gene is faulty, and so the pain signal is never sent to his brain.

0:29:370:29:42

Geoff had finally found the reason why Paul and others with CIP

0:29:420:29:46

couldn't feel any pain.

0:29:460:29:49

With our patients who can't cause that electrical stimulus to be

0:29:510:29:55

fired, they've got their pain neurons sitting there but

0:29:550:29:59

they just can't respond.

0:29:590:30:02

Knowing the cause of CIP provides hope of

0:30:020:30:05

a cure for future generations of people like Paul.

0:30:050:30:08

But Geoff and his team realised that the discovery of

0:30:080:30:12

a gene for pain has wider implications for all of us.

0:30:120:30:16

If scientists can find a way to block the gene temporarily,

0:30:180:30:22

then they'll be able to produce more effective painkillers in the future,

0:30:220:30:26

essentially providing total pain relief for patients who need it.

0:30:260:30:31

It surely should be a goal that pain is a controllable problem.

0:30:330:30:38

The way we've made such huge strides in the understanding and the

0:30:380:30:42

treatment of cancer, that must be possible, also, for pain.

0:30:420:30:45

I would hope that,

0:30:470:30:48

as a result of the research being done into my condition,

0:30:480:30:52

that that can be used somehow to create something that would

0:30:520:30:57

act as a block to pain to someone who suffers from too much of it.

0:30:570:31:01

So use someone who doesn't feel pain as a way of helping someone who

0:31:020:31:07

feels too much pain.

0:31:070:31:08

Extraordinary people like Concetta and Paul are giving crucial

0:31:130:31:17

insights into how our senses work, but every now and again an

0:31:170:31:22

individual is discovered who has a sensory superpower with the

0:31:220:31:26

potential to change the course of medicine,

0:31:260:31:29

and I've come across a case recently that could provide

0:31:290:31:33

a breakthrough in diagnosing one of the most devastating diseases

0:31:330:31:38

of the modern age.

0:31:380:31:39

Joy Milne can smell things that other people can't.

0:31:420:31:46

As a nurse, I found I could smell a lot of things.

0:31:470:31:52

Blood has a definite smell.

0:31:520:31:54

I would say, "Oh, there's an awful smell in here," but I didn't

0:31:540:31:59

realise other people didn't have that sense of smell.

0:31:590:32:02

But even she was shocked to discover the true power of her nose.

0:32:020:32:07

It began with her husband, Les.

0:32:070:32:10

When Les was in his middle thirties,

0:32:110:32:14

I began to nag a little about his smell.

0:32:140:32:17

I just said to him he wasn't showering enough,

0:32:170:32:19

and then I had to say he wasn't brushing his teeth well enough,

0:32:190:32:22

and he was adamant he was doing both.

0:32:220:32:24

At the time, Joy didn't think much of it, but a few years later,

0:32:250:32:29

she noticed other changes in Les.

0:32:290:32:32

He....would miss things.

0:32:340:32:36

We were playing darts with the boys one night,

0:32:370:32:40

and he let the dart literally go through the front of his shoe.

0:32:400:32:43

Les was diagnosed with Parkinson's, a progressive neurological

0:32:450:32:49

disease that causes tremors and difficulties with movement

0:32:490:32:53

and can lead to dementia.

0:32:530:32:55

As they began to encounter other Parkinson's patients,

0:32:550:32:58

Joy noticed something extraordinary.

0:32:580:33:01

It wasn't until we went to our first Parkinson's group that I came home

0:33:060:33:10

and I said to Les, "They smell the same as you."

0:33:100:33:13

And he sort of... He said, "Are you sure?"

0:33:130:33:16

I said, "Yes. It has a smell.

0:33:160:33:18

"It definitely has a smell. These people smell like you."

0:33:180:33:22

'On the face of it, it seemed completely bizarre.

0:33:230:33:27

'Was it really possible that Parkinson's had an odour and

0:33:270:33:30

'that Joy could smell it?

0:33:300:33:33

'She was determined to find the answer.

0:33:340:33:36

'In 2012, she attended a talk given by Dr Tilo Kunath,

0:33:380:33:42

'a Parkinson's expert at the University of Edinburgh.

0:33:420:33:45

'At the end, she raised her hand to ask a question.'

0:33:480:33:52

I just said, "Why aren't we using the smell of Parkinson's?"

0:33:530:33:57

That was a pretty unusual question.

0:33:570:33:59

I've never had that question posed before, and I have to admit, yeah,

0:33:590:34:03

I was confused and I didn't know what you were getting at

0:34:030:34:06

-at the time.

-You were!

-Yeah.

0:34:060:34:09

Intrigued, Tilo contacted Joy after the talk

0:34:090:34:13

and was shocked to hear how she had noticed the change in Les's

0:34:130:34:16

scent before he was even diagnosed.

0:34:160:34:20

-Hi!

-Hello!

0:34:200:34:22

And I asked you questions like "Can you describe the smell in words?"

0:34:220:34:26

-Nasty.

-And woody?

-Yes, a heavy, musky smell.

-Yeah.

0:34:260:34:31

Tilo designed an experiment

0:34:310:34:33

to put Joy's remarkable claims to the test.

0:34:330:34:36

Could she really smell Parkinson's disease?

0:34:360:34:39

He recruited 12 volunteers, six with Parkinson's and six without,

0:34:410:34:46

and asked Joy to identify them based on the scent of their T-shirts.

0:34:460:34:51

So, she told us who all the Parkinson's patients were,

0:34:540:34:58

and then, of the six controls, she got five of them right.

0:34:580:35:01

And we thought, "Pretty good, 11 out of 12, that's quite a good score."

0:35:010:35:04

But nine months later, the patient that Joy had seemed to identify

0:35:040:35:08

incorrectly as having Parkinson's

0:35:080:35:11

was diagnosed with the disease. She'd been right all along.

0:35:110:35:15

My jaw just dropped, and I couldn't believe that she could predict

0:35:170:35:20

someone that was in the early stages of Parkinson's.

0:35:200:35:23

The tests show that Joy really can smell Parkinson's and, more

0:35:250:35:31

than that, she can detect it before a patient has any symptoms.

0:35:310:35:36

So, how is this possible, and what exactly is she smelling?

0:35:360:35:41

Every smell is a unique collection of odour molecules that

0:35:440:35:48

travel through the air.

0:35:480:35:50

These are picked up by chemical receptors at the back of our

0:35:510:35:54

nose which then send a pattern

0:35:540:35:56

of signals to a part of the brain called the olfactory bulb.

0:35:560:36:01

It's this pattern of signals that we interpret as a smell.

0:36:010:36:05

It could be possible that Joy's odour receptors are highly

0:36:110:36:14

sensitive compared to the rest of us, but more pressing for

0:36:140:36:18

scientists is to discover what exactly it is that she's smelling.

0:36:180:36:22

Professor Perdita Barran

0:36:250:36:26

is a a chemist at the University of Manchester.

0:36:260:36:29

Tilo asked her to analyse the T-shirts from the experiments.

0:36:310:36:35

But first, Perdita needed to know which part of them Joy had smelt.

0:36:350:36:40

Contrary to what we had assumed,

0:36:420:36:44

that the smell would be in sweat and therefore perhaps strongest

0:36:440:36:47

in the armpit areas of the T-shirts, it wasn't,

0:36:470:36:51

it was strongest in the middle of the back.

0:36:510:36:53

The middle of the back contains a large concentration of an

0:36:550:36:58

oily substance called sebum that protects the skin.

0:36:580:37:02

Perdita knew that there must be a molecule in sebum that Joy

0:37:020:37:06

could smell that was unique in people with Parkinson's disease.

0:37:060:37:11

She analysed the sebum from the T-shirts and

0:37:110:37:13

found 9,000 possible molecules.

0:37:130:37:17

What we are really wanting to find out is what is the difference

0:37:180:37:22

between 9,000 molecules in one person and 9,000 molecules in

0:37:220:37:26

a Parkinson's sufferer,

0:37:260:37:29

and potentially one or two or three molecules will be very different.

0:37:290:37:33

So it is a needle-in-a-haystack situation.

0:37:330:37:35

To identify the few molecules in 9,000 that are specific to

0:37:370:37:42

Parkinson's disease, Perdita needs a much larger number of samples.

0:37:420:37:47

So she's started a clinical trial involving sebum samples

0:37:490:37:53

from 100 people who have Parkinson's disease and 100 people who don't.

0:37:530:37:57

Perdita hopes that by identifying the molecule that Joy is smelling,

0:37:590:38:03

it could transform the way doctors diagnose and treat Parkinson's.

0:38:030:38:09

What we hope is that we will be able to develop a diagnostic test.

0:38:090:38:13

That's our primary aim.

0:38:130:38:15

We really hope that that will help to diagnose Parkinson's at an

0:38:150:38:18

earlier stage than currently available,

0:38:180:38:20

and at the moment there really isn't a test.

0:38:200:38:22

And all of that is thanks to Joy.

0:38:220:38:25

The possibility of an early diagnostic test for Parkinson's

0:38:270:38:31

disease could improve the lives of millions of people around the world,

0:38:310:38:36

all thanks to one woman and her very keen nose.

0:38:360:38:40

Joy's acute sense of smell gives her a special understanding of

0:38:420:38:46

other people, but, in fact, in all of us smell is inherently

0:38:460:38:50

associated with the formation of memory and emotions.

0:38:500:38:54

You might know this if a smell's ever reminded you of something.

0:38:540:38:57

And if you look at the anatomy, it's clear why.

0:38:570:39:01

Smell enters through the nose, and it comes up and is processed in

0:39:010:39:06

the olfactory bulb before sending signals to parts of the brain

0:39:060:39:10

called the amygdala and the hippocampus.

0:39:100:39:14

These two areas of the brain are crucial in the formation of

0:39:140:39:18

emotions and memory.

0:39:180:39:20

How memory works is one of the deepest secrets of the brain...

0:39:220:39:26

..one that scientists are unravelling thanks to

0:39:280:39:31

a few exceptional individuals.

0:39:310:39:33

This is Tracy Fitzgerald. Tracy lives in Boston, Massachusetts.

0:39:380:39:44

She's a student advisor, which would seem to be a normal job.

0:39:440:39:48

But Tracy has a completely extraordinary ability.

0:39:480:39:54

'I'm able to remember every day as if it were yesterday.'

0:39:540:39:58

She can remember precise events and the date they occurred going

0:39:580:40:02

back decades.

0:40:020:40:04

-INTERVIEWER:

-Can you remember what might have happened

0:40:040:40:06

-on February 11th 1990?

-Yes.

0:40:060:40:09

February 11th 1990 was the day that Nelson Mandela was released

0:40:090:40:13

from prison.

0:40:130:40:14

16th of October 1978.

0:40:140:40:17

The 16th of October 1978, Pope John Paul was named Pope.

0:40:170:40:22

November 9th 1989 was the day that the Berlin Wall officially

0:40:220:40:26

came down.

0:40:260:40:28

Do you happen to know the date that Diana and Charles got married?

0:40:280:40:31

July 29th 1981. It was a Wednesday.

0:40:310:40:34

Tracy doesn't learn these details by rote.

0:40:360:40:39

Her extraordinary recall goes way beyond the day's headlines.

0:40:390:40:43

I'll go back to 2010 and think of March 4th.

0:40:450:40:48

It's almost instantaneous. It's kind of like a DVD

0:40:480:40:52

is being put in and then set to play, and I'm recalling.

0:40:520:40:57

A large portion of information will immediately hit,

0:40:570:40:59

and then the rest kind of comes in like snow,

0:40:590:41:02

almost like a game of Tetris, starting to fall into place.

0:41:020:41:05

That year, on March 4th,

0:41:070:41:09

members from my office decided to go have an impromptu after-work

0:41:090:41:13

gathering at the local pub,

0:41:130:41:14

so now I've just remembered that before I was able to go to that,

0:41:140:41:18

I also had to go to a bank and make a credit-card payment, because

0:41:180:41:21

my credit-card payment was due on March 4th, and I think

0:41:210:41:24

I made the minimum, like, 85 payment that day

0:41:240:41:27

and was able to take out some money, 20, to be able to go to the bar

0:41:270:41:32

and join my friends that afternoon, so that information's coming in.

0:41:320:41:36

Tracy can remember this kind of precise detail from almost

0:41:390:41:43

every day of her life for the past 40 years.

0:41:430:41:46

It's a condition called highly superior autobiographical

0:41:460:41:50

memory, or HSAM.

0:41:500:41:53

For someone with a normal memory,

0:41:550:41:57

and certainly someone of my age who, I don't know,

0:41:570:42:00

I feel like I'm losing memory and details all over the place,

0:42:000:42:04

you watch her doing this and it's just completely astonishing.

0:42:040:42:08

Sometimes I begin my day or I end my day thinking about that date

0:42:080:42:13

and then going back in time to see if I can identify what I was

0:42:130:42:16

doing for the past year or so.

0:42:160:42:18

Those are some of the typical exercises that I do just for fun.

0:42:180:42:23

'Tracy's memory skills are incredibly rare,

0:42:260:42:30

'so what is it that enables her to have this exceptional recall?'

0:42:300:42:36

One scientist is trying to unlock the secrets of what's happening

0:42:360:42:40

inside the brains of people with this extraordinary condition.

0:42:400:42:44

'My name is James McGaugh, and I'm a professor of neurobiology and

0:42:460:42:50

'behaviour at the University of California, Irvine.'

0:42:500:42:53

Over the past 15 years, James has studied over 60 people with HSAM.

0:42:550:43:00

It's not that they have a very strong memory to begin with.

0:43:020:43:06

As a matter of fact, we are as good as they are for 24 hours.

0:43:060:43:10

So if you think about it, their autobiographical memory is just like

0:43:100:43:14

ours for a period of time, then we forget and they tend not to forget,

0:43:140:43:20

and that's the difference.

0:43:200:43:22

It's not just a strong memory, it's a very severe inability to forget.

0:43:220:43:28

To discover how this is possible,

0:43:290:43:31

James's colleague Dr Mike Yassa has been using an MRI scanner to

0:43:310:43:36

look inside the brains of people with HSAM.

0:43:360:43:39

He's comparing the structure of their brains with those of people

0:43:390:43:42

with normal memories, and he's found three intriguing differences.

0:43:420:43:46

This area here, called the striatum,

0:43:500:43:51

which is about the middle of the brain,

0:43:510:43:54

this is a region that's been implicated in habit formation

0:43:540:43:57

and habit learning, and that region seems to be slightly enlarged

0:43:570:44:02

in individuals with this ability.

0:44:020:44:04

There's a second area that's enlarged in people with HSAM,

0:44:040:44:08

the parahippocampal gyrus, involved in learning and memory, and there's

0:44:080:44:13

a pathway known to be involved in transferring information

0:44:130:44:17

around the brain that's also more pronounced.

0:44:170:44:20

So the scans have revealed what's different about the brains of

0:44:250:44:29

people with HSAM.

0:44:290:44:31

But to find out why these differences exist,

0:44:320:44:35

James has begun an exciting new phase of research.

0:44:350:44:40

'My name is Tyler. I am 13 years old,

0:44:410:44:44

'and I have highly superior autobiographical memory.'

0:44:440:44:47

Tyler is one of the youngest members

0:44:490:44:51

of the group being studied by James McGaugh.

0:44:510:44:54

He displays the same abilities as Tracy.

0:44:540:44:58

What was Thanksgiving in 2014? That was two years ago.

0:44:580:45:04

-The 27th.

-Correct.

0:45:040:45:06

Scientists think that Tyler might finally bring

0:45:080:45:12

a breakthrough in understanding HSAM,

0:45:120:45:16

not only because he's 14 and they can watch the condition develop

0:45:160:45:18

throughout his life.

0:45:180:45:20

Tyler brings a double opportunity...

0:45:200:45:22

..because this is Chad...

0:45:240:45:26

..Tyler's genetically identical twin brother.

0:45:280:45:31

And he doesn't have HSAM.

0:45:310:45:33

It's kinda cool, cos, like, if I don't remember a date for a certain

0:45:330:45:37

thing, say my parents ask me, he always remembers it and can tell me.

0:45:370:45:41

So, yeah, that's kinda cool.

0:45:410:45:43

In 2013, what was the day of the week before Halloween?

0:45:440:45:50

-Thursday.

-Correct.

0:45:500:45:51

-A Saturday? No, wait, Friday. Yeah.

-Close. Not quite.

0:45:510:45:56

Oh...

0:45:560:45:57

Two people of the same age, same background, same family,

0:45:570:46:02

and we'll find out, hopefully,

0:46:020:46:04

why one of them is able to have it and one does not.

0:46:040:46:07

Here's a couple of twins who share a genetic code who are going to

0:46:100:46:16

provide Dr McGaugh with a remarkable opportunity to try

0:46:160:46:21

and identify what it is that makes people with HSAM different

0:46:210:46:24

from people without HSAM.

0:46:240:46:26

If we can learn something about how their brains work and how

0:46:280:46:32

their brain working differs

0:46:320:46:34

from those of people who do not, that will

0:46:340:46:36

be a new chapter in understanding the neurobiology of memory.

0:46:360:46:40

Every waking moment,

0:46:450:46:47

our brains are taking in a massive amount of information from

0:46:470:46:51

our senses and adding it to the memories that are already

0:46:510:46:54

stored there,

0:46:540:46:56

and it's this wealth of information that enables us to survive

0:46:560:47:00

and navigate through our lives.

0:47:000:47:02

These extraordinary cases that we've been looking at are enabling

0:47:020:47:07

scientists to work out just how the brain can do all this.

0:47:070:47:12

But sometimes science can learn the most when the power of the

0:47:120:47:17

brain is taken away, as our final case shows.

0:47:170:47:21

'I remember just seeing complete black.

0:47:240:47:27

'I guess my eyes weren't connected to my brain yet.

0:47:290:47:32

'I felt like I was a prisoner in my own body.

0:47:330:47:36

'This event changed my life forever.'

0:47:370:47:40

Three-and-a-half years ago,

0:47:410:47:43

Juan Torres was trapped in a seemingly vegetative state,

0:47:430:47:47

awake but unable to communicate with the outside world.

0:47:470:47:51

No-one expected him to recover.

0:47:510:47:54

The very fact he's talking to us now is truly astonishing.

0:47:540:47:59

CHATTER ECHOES

0:47:590:48:01

I remember going to a party.

0:48:020:48:05

And then I remember coming home.

0:48:050:48:07

Juan has no memory of the events that followed.

0:48:100:48:13

Mysteriously, something happened in the night that led to him

0:48:130:48:17

having a respiratory arrest. He stopped breathing.

0:48:170:48:20

And his mother, Margarita, obviously called the ambulance,

0:48:200:48:23

where he was rushed to hospital and doctors attempted to save him.

0:48:230:48:28

'They were struggling to keep him alive.'

0:48:280:48:31

But there was a moment where they told us,

0:48:320:48:36

"He's leaving, basically he's leaving. You can say goodbye."

0:48:360:48:40

But Juan survived.

0:48:410:48:43

When the doctors managed to get him through that period of time

0:48:440:48:49

where they thought he might not live,

0:48:490:48:52

the best that they were able to come to Margarita and tell

0:48:520:48:55

her was that he was still alive but that he was in

0:48:550:48:58

a vegetative state and would not recover.

0:48:580:49:01

As his mother was being told that story,

0:49:030:49:06

Juan was in the room hearing everything that was being said,

0:49:060:49:10

recognising the world around him and knowing that

0:49:100:49:13

he was still conscious, but in his case, he didn't even have the

0:49:130:49:17

ability to blink his eyes to let someone know that that was so.

0:49:170:49:21

It felt horrible.

0:49:230:49:25

I couldn't even cry...

0:49:250:49:27

..because I guess my brain hadn't got reconnected to my tear ducts.

0:49:290:49:33

For over two desperate months, Juan remained in what's known as

0:49:360:49:40

a locked-in state, conscious but unable to communicate.

0:49:400:49:44

Margarita is clearly an amazing woman,

0:49:460:49:50

and even in the face of doctors saying to her,

0:49:500:49:54

"We're really sorry, we've done the best we can by your son,

0:49:540:49:58

"he's alive and breathing but he's not there any more,"

0:49:580:50:02

she just chose not to believe that.

0:50:020:50:05

Sometimes, it was hard to stay positive because there would

0:50:070:50:11

be many days and hours where you don't see a glimpse of anything.

0:50:110:50:14

I love you, baby.

0:50:160:50:18

Do you see the nice place?

0:50:180:50:20

They have a beautiful garden outside, and park.

0:50:200:50:24

And then, one day, without warning,

0:50:250:50:27

for reasons that aren't understood, something truly remarkable happened.

0:50:270:50:32

We took him with my husband, and it was a sunny day, beautiful day.

0:50:330:50:37

That was the first day that he came out of the room.

0:50:370:50:40

I remember being outside.

0:50:410:50:44

And my mum said something like, "Come on,

0:50:440:50:48

"you're always my little Snow White." And then I chuckled.

0:50:480:50:51

And that's when they knew.

0:50:510:50:54

Oh, my God. It's very...!

0:50:540:50:57

'We were laughing. And so he started to laugh.'

0:50:570:51:01

Sleeping Beauty? Yeah.

0:51:010:51:04

'I remember feeling really happy.'

0:51:040:51:08

That was to me a very specific sign that he was going to come back.

0:51:080:51:15

Juan had finally been able to let the people around him know

0:51:150:51:19

that he was still conscious.

0:51:190:51:22

They were ecstatic.

0:51:220:51:24

I felt joy.

0:51:240:51:26

I remember I was just...kept telling myself that...

0:51:270:51:31

I'm going to walk again. That's what drove me.

0:51:310:51:34

The fact that I had it all,

0:51:340:51:37

and I was going to return to having it all.

0:51:370:51:40

THEY SPEAK IN SPANISH AND LAUGH

0:51:400:51:44

Why he was suddenly able to start communicating like this is

0:51:440:51:49

something that science can't completely explain.

0:51:490:51:52

And how and why this happens in some patients and what triggers it

0:51:520:51:57

are some of medicine's greatest unknowns.

0:51:570:52:01

The difficulty for us doctors is in trying to establish exactly

0:52:010:52:05

which patients are in a truly vegetative state,

0:52:050:52:10

and which are aware of what's going on but unable to communicate.

0:52:100:52:14

In short, which patients will get better.

0:52:140:52:18

One man is trying to find a way to answer this question.

0:52:200:52:24

Adrian Owen is a neuroscientist from the University of Western Ontario.

0:52:240:52:29

He tested Juan when he seemed to be in a vegetative state.

0:52:330:52:36

Down, roll over!

0:52:360:52:39

And Juan's recovery offered a rare opportunity to discover how

0:52:390:52:43

much he'd actually been aware of at that time.

0:52:430:52:47

Once we heard he'd recovered, we very quickly brought him back

0:52:470:52:51

here to London, Ontario, and we put together a memory test.

0:52:510:52:54

We asked him things like which of these two people do you recognise?

0:52:540:52:58

Of course, he could identify my research assistant who'd

0:52:580:53:01

tested him on that day.

0:53:010:53:03

What we have to remember is that, at the time, Juan appeared to be

0:53:030:53:07

in a vegetative state. He was entirely non-responsive.

0:53:070:53:11

His eyes were open but there was

0:53:110:53:12

no evidence he was aware at all, yet he could remember people.

0:53:120:53:17

He could remember places.

0:53:170:53:19

And he could remember things we'd done to him.

0:53:190:53:22

Dr Owen was only able to find out that Juan had been aware of

0:53:240:53:28

what was going on after he'd already recovered.

0:53:280:53:31

He needed a way to find out which patients were truly vegetative

0:53:310:53:36

and which patients were locked in and had a chance to recover.

0:53:360:53:41

He decided to look inside their brains.

0:53:410:53:44

So, what Dr Owen's been doing is putting all of his patients,

0:53:460:53:51

who are in a vegetative state, into what's called

0:53:510:53:55

a functional MRI scanner,

0:53:550:53:57

which is a machine that looks at the brain and,

0:53:570:54:01

in response to certain questions, shows different levels of

0:54:010:54:05

blood flow to try and identify that there's some function there.

0:54:050:54:09

When you hear the instruction to begin,

0:54:100:54:12

please imagine playing a game of tennis.

0:54:120:54:16

We know that when people imagine playing tennis,

0:54:160:54:18

a part of the brain, known as the premotor cortex, lights up.

0:54:180:54:22

It's the part of the brain involved in moving your arms around,

0:54:220:54:25

as if you were playing a game of tennis.

0:54:250:54:27

If I say imagine playing tennis to you and your premotor cortex

0:54:270:54:30

lights up, I know that you've understood the instruction

0:54:300:54:34

and you've followed the command.

0:54:340:54:36

By scanning patients' brains in this way,

0:54:380:54:40

Dr Owen has found that nearly 20% of people who appear to be in

0:54:400:54:45

a vegetative state are in fact conscious and aware.

0:54:450:54:50

And now, thanks to this research,

0:54:510:54:53

scientists are closer to understanding how more

0:54:530:54:56

patients like Juan could be brought out of their locked-in state.

0:54:560:55:01

20 years ago, when I started working in this area,

0:55:050:55:07

nobody was interested in the vegetative state

0:55:070:55:10

because it was thought to be a hopeless condition.

0:55:100:55:12

Well, now we know that in some cases there is hope.

0:55:140:55:17

Now we know that in one in five cases there is awareness when

0:55:170:55:21

everybody else thinks there isn't.

0:55:210:55:23

One in five is a staggeringly high number.

0:55:250:55:28

But it's never going to be possible to take every patient

0:55:280:55:32

in such a critical condition

0:55:320:55:34

to an fMRI scanner to test their brain responses.

0:55:340:55:38

And this could mean that not every locked-in patient is identified.

0:55:380:55:42

The difficulty with fMRI scanning all of these patients

0:55:420:55:47

is MRI scanners are very heavy and not movable.

0:55:470:55:51

So, Dr Owen's come up with a brilliant solution to this problem.

0:55:520:55:57

This is the EEJeep. It allows him to take his testing on the road.

0:55:570:56:02

He uses a different technology to measure brain activity

0:56:030:56:07

so that critically ill patients don't have to be transported to him.

0:56:070:56:11

He can go to them.

0:56:110:56:12

There are 129 electrodes,

0:56:160:56:19

and each one of these will pick up a little bit of the electrical

0:56:190:56:23

activity that your brain naturally and continuously produces.

0:56:230:56:27

The way fMRI works is by measuring or by monitoring

0:56:280:56:32

how blood moves around the brain.

0:56:320:56:35

EEG works in a completely different way,

0:56:350:56:38

which is that it detects the electrical signals coming

0:56:380:56:41

from the activity of neurons in the brain.

0:56:410:56:45

It's actually a different way of looking at the same thing,

0:56:450:56:48

which is, essentially, which areas of the brain are active right now.

0:56:480:56:52

Dr Owen's research is bringing us closer to understanding why

0:56:540:56:58

patients like Juan recover and how we can identify others like him

0:56:580:57:02

so that we can bring more people back from being locked in.

0:57:020:57:06

Three years on, Juan is studying life sciences at university.

0:57:060:57:11

And learning to walk again.

0:57:110:57:13

You've got to put one foot in front of the other. That's how you learn.

0:57:160:57:21

I'm going to be walking in a year.

0:57:210:57:22

Exactly a year from now.

0:57:220:57:25

Or less.

0:57:250:57:27

I feel that we all appreciate life in a totally different...

0:57:270:57:33

manner than we used to.

0:57:330:57:35

It is amazing.

0:57:350:57:37

Juan was brought back from the edge of oblivion,

0:57:390:57:41

a terrifying experience.

0:57:410:57:44

But from this and all the other cases we've explored comes a better

0:57:440:57:49

understanding of the extraordinary power of the human brain.

0:57:490:57:53

It reminds us of the super abilities that our brains give us

0:57:530:57:58

and how they can sometimes recover, even in the most extreme cases.

0:57:580:58:04

Next time, I'll discover why this man can run for three days straight.

0:58:050:58:11

Some of the races I've run have been 200 miles.

0:58:110:58:14

Why this woman has to battle the whole world.

0:58:140:58:17

I'm allergic to everything.

0:58:170:58:19

And why this woman once had two hearts.

0:58:190:58:22

I always think of all the doctors, all the surgeons.

0:58:220:58:25

I wouldn't really be here without them.

0:58:250:58:27

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