Sharon’s world is regularly reversed by a rare brain malfunction. Now neurologists, and Wonder Woman, have come to the rescue

In 1952, when she was a child, Sharon was playing in the front garden. She was blindfolded while her friends ran around her, laughing, trying not to be caught in a game of blind man’s buff. Sharon grabbed hold of someone’s sleeve and whipped off the scarf that covered her eyes. “You’re it!” she shouted.

Then she blinked and looked around her. She panicked. The house and the street looked different. She had no idea where she was. Sharon ran into the back garden and discovered her mother sitting in a lawn chair.

“What are you doing here?” Sharon asked. “Whose back yard is this? Where am I?”

Her mother looked at her, puzzled. “What’s wrong with you?” she asked her daughter. “This is our house!”

Her mum pointed a finger at Sharon, aged five: ‘Don’t tell anyone about this. They’ll say you’re a witch and burn you’

Sharon told her mother that everything around her looked different. Her mother looked irritated. Sharon didn’t understand: why wasn’t her mother helping her?

“I don’t know where this place is, it all looks wrong,” she said. “I’m so confused.”

Her mum looked her in the eye, and pointed a finger at her face.

“Don’t ever tell anybody about this,” she said. “Because they’ll say you’re a witch and burn you.”

***

“I can remember that moment as if it were yesterday,” Sharon says, more than 60 years later. “I was five years old.”

Sharon woke up the next morning knowing that something weird had happened again. It was as though her walls had moved in her sleep. She was in her bedroom but things didn’t look as if they were in the right place. Her door was on the wrong side. “I knew it had to be my bedroom,” she says, “and bits of the room were familiar, but it was all wrong at the same time. Nothing was where I thought it should be.”

Sharon’s disorientation began to occur more frequently, until it became constant. It made finding her way around her neighbourhood and her school impossible. She never mentioned her problem to anyone. Instead, she used her sense of humour and intelligence to complete her education, make friends and get married, without anyone ever knowing she was almost permanently lost.

“I hid it for 25 years,” she says.

***

I meet Sharon at her home in Denver, Colorado. Even here, she can get lost walking between her bathroom and her kitchen.

Sharon has flaming-copper hair, swept into a stylish crop set off by a bright pink blouse. The colours complement her deep-red lipstick. Outside her front door sits a giant metal lobster with a faded ‘Welcome’ sign written across his rusty belly. Inside, her house is open plan and as neat as a pin. Stuck on to her fridge door are pictures of friends, phone numbers, notes from grandchildren, a picture of Wonder Woman and a large photo of a handsome young Italian. It is held up with a magnet that says: “A true friend knows everything about you… and likes you anyway.” A smaller photo, of Sharon and the same man together, arms round each other’s shoulders and smiling at the camera, is pinned above it.

Facebook Twitter Pinterest Dr Giuseppe Iaria who diagnosed Sharon, and Wonder Woman who inspired her solution, both feature on her fridge. Photograph: Benjamin Rasmussen/The Guardian

“That’s Giuseppe,” says Sharon. “Isn’t he cute? He’s such a gentle and compassionate man. He changed my life.”

As a young post-doc, Giuseppe Iaria was fascinated by navigation. While working at the University of British Columbia, he investigated why some healthy people have a better sense of direction than others. One day, a middle-aged woman, Claire, showed up at his lab complaining of a peculiar problem: she was constantly lost.

Iaria suspected that Claire’s disorientation was the result of another condition. He began ruling out possible options one by one. He knew that inner-ear infections can damage a delicate structure called the labyrinth, causing the sensation that your world is moving around you. Brain tumours, lesions and dementia can damage the hippocampus, which is involved in many types of memory. Or maybe it was epilepsy, sudden bursts of uncontrolled electrical activity in the brain, that was stopping her from being able to memorise directions. It took Iaria and a colleague two years to eliminate all the potential problems. But, as far as their tests showed, Claire was in perfect health.

Claire told Iaria that she hadn’t lost the ability to orientate herself; she’d just never learned it in the first place. She recalled that, from the age of six, she would panic at the supermarket each time her mother disappeared from view. She never left home by herself, because she got lost each time she tried.

Spinning fixes the problem. ‘I go into a cubicle, close my eyes and spin around. It's my Wonder Woman impression’

As an adult, Claire had figured out how to get to work by taking a particular bus, memorising the stop and a prominent landmark near her office. But her employer was moving to an unfamiliar area, and she had decided it was time to get some professional help.

Iaria routinely encountered disorientation as a symptom of other conditions, but never as a developmental disorder – one that occurs as you grow up. He took Claire for a short walk around the local area. He then handed her detailed directions so she could repeat the route by herself. Claire followed the directions without any mistakes. However, when Iaria asked her to draw a map of the route she had just walked, or of the town in which she lived, she found it impossible. She said she did not have “in my mind a map to report”.

Iaria called her Patient One and named the condition “developmental topographical disorientation”: the inability to generate, and therefore use, a mental map of your surroundings, despite an absence of any brain damage.

Over time, Iaria found others with the same condition. One person told him: “No matter how long I live in the same building, I can never picture in my mind where the bathroom is.”

At 60, Sharon was Iaria’s case number four.

***

Sharon tells me that she was not permanently lost from the age of five. “Some of the time, my world looked perfectly normal and I could navigate perfectly well. But then all of a sudden my world would flip, and I’d become completely disoriented.”

And she never told anyone? “No. Instead I was the class clown. I thought if I could stand up and make the class laugh, they wouldn’t know my secret.” No one ever noticed that most of the time she was lost. “I would follow my friends when we walked to school, and if it happened during class, I’d spend the rest of the lesson trying to memorise the way the room looked so I’d know where everything was the next time it happened.”

One day, when Sharon was still a young girl, she discovered a solution. She was at a friend’s party, and next to play pin the tail on the donkey. “After I spun around, I knew something was horribly wrong. I felt like I was walking in completely the wrong direction.” When she took off the blindfold, she thought, “I know I’m at my friend’s house, but this doesn’t look like my friend’s house.”’

They said I needed to see a psychiatrist – they thought I was crazy. I wanted to die

But when it was her turn to be blindfolded and spun around for a second time, Sharon’s world flipped back to normal. “That’s when I learned that spinning could cause the disorientation. But that it also fixed it.”

“These days I usually try to find the nearest bathroom,” Sharon says. “I go into a cubicle, close my eyes and spin around. When I open my eyes, my world is recognisable again.”

She chuckles and points towards the picture pinned to her fridge. “I call it my Wonder Woman impression.”

Why does she do it in the bathroom?

“Well, what would you think if you saw an old woman standing by her car spinning around in circles with her eyes closed? I always did it in secret because I was humiliated by it.”

***

For most of us, navigating feels easy. But many of Iaria’s patients feel as if they live in a constant “first day”. No matter how much time they spend somewhere, their surroundings never become familiar. Like Claire, many have learned to navigate the most important routes in their life by remembering a specific sequence of turns. But to remember all your journeys this way would place a huge strain on your memory. Instead, we use a dynamic tool, a cognitive map, a kind of internal representation of our surroundings that becomes familiar.

Our cognitive map is created by a number of different cells. There are those that fire only when we pass through a specific location. A nearby cell fires at a different location. Then there are cells that fire only when our head is facing a particular direction, and others that are responsible for where we are in relation to walls and boundaries. One might fire, say, when there’s a wall to the south or when we’re near the edge of a cliff.

We also fill our mental maps with permanent landmarks that are meaningful to us, like the post box at the end of our road, or the bus stop outside our office. There is a part of the brain dedicated to this task – the retrosplenial cortex. The current theory is that the combination of all these cells’ activity creates a map of our world, which is constantly updated to help us find our way around. But when one or more of these regions isn’t working correctly, things can get very confusing.

Sharon drives us to a nearby restaurant for lunch, insisting she knows the way. From her condo we drive around a couple of roundabouts, pass through a set of traffic lights and indicate left then right without a hitch. We turn on to a small highway that runs through the town, the Rocky Mountains dominating the landscape to the west. Sharon tells me that sometimes she’ll be driving into town when she suddenly realises that the mountains are to the north and she’ll know that her world has flipped. Then we fly past the entrance.

Later, when we get there, we sit down and Sharon explains what she sees when her world flips. She tells me to think about a busy street. “Imagine you’ve had a day shopping,” she says. “You come out of the last shop and head left towards the station. All of a sudden, you realise the station is actually on your right, because you were in a shop on the opposite side of the street to where you thought you were. In that split second, you feel momentarily disoriented because the station that you thought should be east is now west. Your world hasn’t flipped but your perception of it has.”

Most people’s brains are surprisingly forgiving. As soon as it gets confused, the brain spins everything around and reorients itself within milliseconds. But that split second in which your mental map doesn’t match with where things actually are is how Sharon feels when her world is flipped.

The doctor looked at me like I was telling a made-up story. She asked how I correct it and I told her I spin around

“I just can’t flip my world back around like you can,” says Sharon. “Unless I do my Wonder Woman impression.”

I ask why we missed the entrance to the restaurant. Sharon explains that it was on a curved road, and that they make her world flip. It has made finding work difficult. Every time she had an interview, she would have to work out in advance where the building was and whether it sat on a curved road. If there were a lot of winding passages in the building itself, she would have to turn down the job.

Was it not possible for her to recognise enough of her environment to work out which way to turn? “Think about standing in front of a bathroom cabinet with a mirrored door,” she says. “Open that door and look at the rest of the room through it and you’ll know it’s your bathroom, but everything’s kind of in the wrong place. Plus you’re stressed because everything looks different. It makes it hard.”

When Sharon has to get up in the night to go to the toilet, or if she’s in a rush in the morning and doesn’t have time to do her Wonder Woman impression, she says she feels like she is in a different condo. When she had young children, she would have to follow their cries to find their room in the night.

Sharon was almost 30 before her secret came out. Her brother had phoned her, asking to be taken to the hospital. He had Crohn’s disease and was feeling unwell. Sharon rushed out of the house in a panic, got into the car and set off on the short journey to his house. But on the way, her world flipped and she got completely lost. She pulled into a petrol station to call him. “I can’t find your house,” she said, and described the petrol station. Her brother was confused. He said, “You’re two blocks away from me – how do you not know where you are?” After the two of them had returned from the hospital, her brother asked her what was going on.

“It was so emotional for me, I could hardly say the words.” It was the first time Sharon had talked about her condition since she was five. Sharon’s brother told his doctor, and the doctor set up a meeting with a neurologist. He told her it sounded like a benign tumour or epilepsy. He organised a barrage of tests, but Sharon’s brain looked healthy.

“They said I needed to see a psychiatrist – they thought I was crazy.” She suffered a bout of severe depression. “I wanted to die. I’d just had my hopes raised, thinking that the doctors would find something that could be fixed.”

Sharon saw a psychologist for more than a year, and although he helped her work through her depression, he was unable to fix her disorientation. He told her to keep checking in with a neurologist every few years, to see if the research community had discovered anything new.

Facebook Twitter Pinterest ‘I’ve always been silly and funny because it misdirected people away from the things I was hiding,’ says Sharon. Photograph: Benjamin Rasmussen/The Guardian

When Sharon turned 40 she decided to see another doctor. But as soon as she sat down, she felt uncomfortable. “She looked at me like I was telling her a made-up story. She asked me how I correct it and I told her I spin around and it fixes it. She said, ‘Let me see you do it.’” Sharon had never spun in front of anyone before. She winces at the memory. “I swallowed my pride, stood up and closed my eyes. I spun around in circles until I knew the world had flipped.”

The doctor asked Sharon what she saw. “I said, ‘Well, I’m in a different room now. I know logically I’m not, but this doesn’t look like the same room.’”

Sharon spun around again and sat back down. The doctor put down her pen and pad and said: “Has anyone ever suggested the possibility that you have a multiple personality disorder?” Sharon was mortified. “I felt like I was being told I was crazy again. I just couldn’t go through that again. I left.”

It was another decade before Sharon made a further attempt to understand what was wrong with her brain. A friend had read some books by the neurologist Oliver Sacks and recommended that Sharon write to him. Sacks replied, apologising that he had not heard of such a condition. But he said the problem might be similar to another condition called prosopagnosia, in which people are unable to recognise familiar faces.

Sharon Googled “prosopagnosia” and found a website that tested how good you are at recognising faces. After the test, there was a questionnaire. One of the questions hit a nerve: “Have you ever been in an environment that you know you should recognise but doesn’t look familiar?”

“I was like, ‘Holy shit!’” says Sharon, who wrote everything about her condition in the notes section. “Within a week, I received a call from Brad Duchaine, a researcher at University College London.” Duchaine had created the online test. “He was so sweet,” says Sharon. “He believed everything I said, and assured me that at some point there would be someone doing research on my problem.”

Two years later, in 2008, Duchaine emailed her, saying he had good news. There was an Italian researcher moving to Vancouver to start researching the condition she had described. That man was Giuseppe Iaria.

“The first time Giuseppe called, I told him everything. He was such a gentle man. He nearly cried when I told him about the witch thing.”

Iaria told Sharon that he thought there might be a problem with the way in which the different navigational cells in her brain communicate with each other. Over the next five years, he began to test this theory.

Sharon’s brain looks anatomically normal, but several of the areas involved in navigation don’t communicate properly

He started by scanning the brains of healthy people, looking at how different brain regions known to be important for orientation and navigation communicate with each other. His team concluded that the best navigators were those with higher levels of communication.

This concept is called network theory and it’s an idea that underlies many human behaviours – that the connections between different regions of the brain may be more important than how well the regions function by themselves. It’s like having a quartet of the world’s best brass players who individually make wonderful sounds. But if they’re not playing in time with one another, that music turns into mayhem.

Iaria’s team then scanned the brains of a group of people with Sharon’s disorder. They noted a difference in the activity of their right hippocampus, an area involved in memory, and parts of their frontal cortex, an area that allows us to draw all the information about navigation together. It’s also an area involved in reasoning and general intelligence.

As Iaria’s patients had no problem with their memory, or reasoning, he concluded that the condition must be a result of ineffective communication between the two regions. “It’s not enough for the individual parts of the brain to be able to speak,” he tells me. “They have to have good conversational abilities, too.”

Since then, Iaria’s team has discovered that, just like Claire’s, Sharon’s brain looks anatomically normal; but several of the areas involved in navigation don’t communicate properly. Still, how could Sharon sometimes navigate perfectly well and then suddenly flip?

“Some people don’t actually lack the skill of forming a mental map,” Iaria explains, “but somewhere in the process of collecting all the pieces of the puzzle, errors accumulate, information gets lost, and suddenly the map shifts.”

To Iaria’s knowledge, Sharon’s spinning technique is unique. “I have to admit I have no idea why it works,” he says. “There’s nothing wrong with her vestibular system – she doesn’t get nauseous or have problems with her balance – but somehow shaking this system resets her mental map.” He sighs. “I can scan her brain, but I can’t enter her mind.”

Facebook Twitter Pinterest Louie the lobster, the ornament that tells Sharon she’s home. Photograph: Benjamin Rasmussen/The Guardian

Recently, Iaria has been testing his theory that developmental topographical disorientation has a genetic link. Of all the people he has identified with the condition – almost 200 of them – about 30% have at least one other family member affected. His team identified a handful of potential genes that might be causing the problem. While it’s unlikely that they will be able to replace the broken genes any time soon, it might be possible to intervene – using brain-training exercises that help children with these genes use other parts of their brain to navigate.

I ask Sharon whether her daughter, son or grandchildren have any signs of the condition. “No, thank goodness – they are all really good at navigating,” she says. Did Sharon’s condition appear spontaneously, I wonder, or might it have been inherited?

“My mum?” Sharon guesses. “Yes, I think she must have had it. Looking back, it all makes sense. She never told my dad about my condition, probably because she’d never told him about hers. She never walked us to school or picked us up from anywhere, unless there were other people with us. She never went anywhere by herself.”

Knowing there are people out there trying to understand her condition has helped. “I’ve always been silly and funny because it misdirected people away from the things I was hiding. Everyone said, ‘You’re always in such a good mood.’ They didn’t know that I would go home at night and cry. Before meeting Giuseppe, I was still a scared little girl. I don’t think I grew up and became a woman until the last 10 years, really. I’m happy now. I realised that in order to be fulfilled I needed to learn to like myself and accept who I am.”

As I leave, I catch another glimpse of her lobster lawn ornament. “I know he’s awful,” says Sharon, as she walks me to my car, “but I call him Louie.” She looks back at the house. “If I’m lost and I see Louie, I know I’m home.”