One patient's bizarre behaviour almost defied human science. Photo / Getty Images
One patient's bizarre behaviour almost defied human science. Photo / Getty Images
During my career I have come across many strange stories of people who have tested my knowledge, often to its limits: a stressed school janitor who hallucinated a fairy-tale scene; a ballet dancer who couldn't stop falling; an office worker who lost her trust in the person she loved; and, this one, of a girl who kept running away.
August was and is a clever, rebellious woman, with a mind of her own. Her story begins in a school playground. She was 16 years old. She was standing at one end of the yard where all the teenagers gathered at lunchtime, her foot resting on a railing, talking with friends. It was an ordinary day. Then something unusual happened. August abruptly stopped talking. She unhooked her foot clumsily from the railing, almost causing herself to fall. Then she ran, tearing across the tarmac. She stopped only when she reached the fence that was the furthest boundary of the yard.
The friends August had been talking to assumed that something must have upset her. August herself had no sense of why she had suddenly taken off at such speed. It was attributed to the behaviour of a teenager looking for attention.
Unfortunately, soon after that August began to take off across the classroom or yard or playing fields in unpredictable but regular bursts. She would stand up suddenly in class and run from the room. During a sports lesson she ran around the field with no regard for anybody else.
The problem wasn't seen as a medical one in the first instance. It was assumed that August was acting out in the face of exam pressure. She was an ambitious girl with high expectations of herself. August had a series of meetings with the school counsellor.
The meetings quickly came to a stalemate and then to an end. August's tendency to abruptly stand up and run away did not come to an end, however. It got worse. Watching television, eating breakfast, mid-conversation, August randomly stood and ran from the room. August's mother soon realised that this was something more than an upset teenager. She took her reluctant daughter to see their doctor.
The doctor looked for stresses in August's life and found a few, so she was referred to a psychiatrist, who concluded that August showed no evidence of any psychiatric complaint. August and her family agreed. August had no idea what was happening to her. She started falling behind in lessons and it became harder and harder to go back to class. Eventually she stopped going to school.
The situation came to a head when she and her mother were walking down their local high street and August abruptly turned and ran out into the road in front of traffic. She narrowly missed being hit by a bus. August's mother and her older brother decided to take her to the casualty department, where they insisted that she be investigated. Some spark of an idea resulted in a phone call to a neurologist. As soon as he heard the story, the neurologist considered epilepsy a likely diagnosis. August had very little memory of the events. The running episodes were brief. In between them, August seemed quite well. All of this fit with self-remitting brainstorms. He arranged a series of tests.
Not unusually, they were normal. Since neurologists expect that tests can be entirely normal even in severe brain disease, he started August on treatment based on a clinical diagnosis of epilepsy. One year and three epilepsy drugs later, August was no better.
I HEARD August's story for the first time when she was in her 20s. Adele, the specialist epilepsy nurse I worked with, asked if I would admit August to hospital for video telemetry, to try to come to some sort of conclusion about the diagnosis. I introduced myself and asked her to describe the running attacks to me.
"I just start running around like a nutter," she said. "Do you know it's going to happen? Do you have any sense of what's happening while you are running?" I asked.
I could see that every new question irked her a little more. "I run. What more do you need to know? I've told you everything. Why don't we just start the video so you can see it for yourself?"
August's expression told me I might lose her completely if I didn't back off. I decided that was the best course of action. August would stay on the ward for a week. "If you think you can keep me in this room when I have a seizure, you're mad," she said.
The next day Adele called me to let me know that August had had a seizure. "Can you look at it? They couldn't keep her in the room. I think this might turn into a disaster," she said.
I looked through the video. August was sitting by her bed, watching television. A tray table sat to her right, between her and an open door leading to a corridor. There was no preamble. No march of symptoms. When she started moving it was so quick that she was only on camera for a second or two. She stood up and ran to her right. Just ran. Very fast. She pushed through the tray table like it wasn't even there. A plate crashed to the floor. I had only a video of an empty room to watch.
August was nearly through the double doors of the ward and heading for the staircase when they caught her.
We started again. The nurses were prepared for the second seizure. The attacks typically came in a cluster, so we anticipated that there would be another very soon. We moved August's armchair to the side of the bed furthest from the door. That way it would take her longer to leave the room. A nurse sat in with August.
She was there and ready when August jumped to her feet. The nurse was much closer to the door so got there first and shut them both in.
Suddenly August had nowhere to go. She swerved and collided with the wall. That didn't stop her. She bounced backwards, reversed direction and headed towards the window opposite.
Arriving there, she was blocked again and turned to the left and ran the short distance available in that direction. She came to a stop at the tray table and crouched under it. The nurse followed her but when she tried to get close August shook her arm threateningly. The nurse pressed the alarm and two senior nurses came to join her. By the time they arrived it was over. August had recovered. She stood up, dusted herself off, straightened her clothing, and went back to sit on her bed. It was as if waking up on the floor underneath a table was as normal as could be.
On the video, August was like a silver ball in a pinball machine, bouncing from target to target. I looked at her brainwaves. She was running so frantically that the insulated leads hanging down her back were bouncing around, pulling on the electrodes on her scalp. This created movement that obscured the recording. If there was a seizure discharge anywhere in August's brain tracing I couldn't see it. As soon as the running was over and she relaxed, the brainwaves were clear and normal again but by then she was better.
To suggest that you can tell everything the brain is doing by sticking metal discs on the scalp is something one would have been wise to be sceptical about. After all, skull, hair and muscle lie between the electrodes and the brain. Muscles themselves produce electrical activity that creates distracting "noise" in the brainwave pattern. If a patient was to tap their tongue rhythmically in their mouth it might register in the brainwaves to look something like a seizure.
The electroencephalogram (EEG) electrodes cannot tell if the electrical activity they record comes directly from the brain or somewhere else. It is up to the doctor to differentiate a tapping tongue from a seizure. Other times, the electrical activity of a seizure is in some hidden part of the brain that scalp electrodes just cannot "see". I still believed that August had epilepsy even though the EEG had failed to prove it.
Neurologist Suzanne O'Sullivan.
Since her seizures had started, August had not only dropped out of school, she was also unable to work, dependent on her family and largely confined to her home.
I didn't know what to do next. August had tried three epilepsy medications. Any additional drug I tried had only a minuscule chance of making her seizure-free.
"Am I stuck like this?"
That was the start of a long, arduous journey for August and me. I began to experiment with new combinations of medication. Almost every drug resulted in some negative consequences. Her weight began to fluctuate wildly.
August is statuesque. She stands proud, broad-shouldered, with a striking sort of beauty. Until, that is, she turned up in my clinic having lost kilos in weight. The tablets had made her seizures a little better but had also caused her to become dangerously underweight. Skin hung from her bones, with nothing in between. Her face had become angular and hard.
"I look terrible," she said coldly. I could see she was holding back the tears. "Do I have to keep taking them?"
I quickly withdrew the drug and replaced it with another. A year later she had regained the weight. Six months later she was overweight. Another drug, another side effect.
Once she was on a bus when she had an attack. Her only memory is of getting on the bus and taking a seat on the upper deck. Moments later, transported as if by dark magic, she was sitting in a strange living room, surrounded by a large multi-generational Asian family.
At one point I admitted her to hospital for reassessment. I was so worried about her and desperately looking for new solutions. August's EEG remained normal. Her MRI, however, became abnormal — not because her disease had changed but rather because technology had improved. The scan showed scattered grey dots buried deep in the white matter, somewhere they should not be. These were consistent with a disorder called a neuronal migration disorder.
In the adult brain, the cell bodies of neurons make up the cortical layer. Underneath the cortex is the white matter of the brain, which contains the axons that connect one part of the nervous system to the rest. But our brains don't start life that way. They develop almost inside out.
The grey matter or neuronal bodies that eventually make the outer layer of the brain start on the inside as neuroblasts. In the first two months after conception, as the fetus is developing, neuroblasts must migrate to their final destination in the outer cortical layer of the brain where they become neurons. Sometimes that migration fails and islands of grey matter are stranded in the wrong place. In some people it does no harm at all. They live their whole lives not knowing about it. In others it can lead to severe physical and mental disability. Some babies with this problem don't survive. In others it causes epilepsy.
It was bad news. Neuronal migration disorders are genetic conditions. People affected by them can have difficulty sustaining a successful pregnancy and their offspring are at risk of having severe developmental problems. It was another complication to August's life.
It is now more than 10 years since I first met August and five years since we found a definitive cause for her running attacks. She cannot have surgery because there are so many stray grey areas in her brain that it would be impossible to say which was responsible for her seizures. She is stuck with medications and they are still proving unhelpful.
But August keeps moving forward with her life. Just a different sort of life. One confined within four walls. "I am starting a cake-making business," she told me recently. "I've enrolled in a cake-making class. But my mum has to come with me to all the classes."
She could not risk going alone. Her mother couldn't afford to pay to join the class herself, so she sat on the sidelines and watched.
"I think they all thought we were both a bit mad, me with my mum following me everywhere," August told me. "Then I had a seizure and ran out the door. Mum said their mouths were all hanging open. They never saw anything like it. Mum followed me and brought me back. Then she just said to the rest of them, 'That's why I'm here.' That shut them up."
"Did you explain to the class why you'd run?" I asked August. "No. Why should I?"
Edited extract from Brainstorm: Detective Stories from the World of Neurology, by Suzanne O'Sullivan (Chatto & Windus, $48).
