It may of course be that some of the psychological issues have a basis in biology. Don has a tendency to compulsive behaviour, some sort of abnormal reward mechanism, an excessive ‘buzz’ from certain types of activity, of which the alcoholism is a feature. At one point, when I try to treat his periodic limb movement disorder with a dopa agonist, he develops a compulsion to flirt with women on the internet. This resolves as soon as the drug is stopped. And this dysfunction of the reward system seems to have been inherited from his mother, and passed on to his daughter. So, perhaps there is a heightened reward that Don gets from food.
I am drawn back to his description of his time at Harvard, when he gained 50lbs over a few short months, this happening way before the sleep-eating surfaced. Perhaps that excessive drive for food emerges at night, when asleep, due to the activation of his limbic system while in that non-REM parasomnia brain state, or when awakened by his periodic limb movement disorder. While during the day, he is so distended, so bloated by the vast volumes consumed at night, that he does not eat. Much to my chagrin, I find myself agreeing with the psychoanalysts: that the night-time may uncover ‘hidden desires’.
I cannot remember ever having seen anyone with such severe sleep-eating as Don, in terms both of intensity and difficulty in treatment. And I am not the only sleep physician who has tried to treat him. Standard treatments for non-REM parasomnias have failed, and I have tried to treat his sleep apnoea as well as his periodic limb movement disorder. Managing his medications has proved problematic. Many of the treatments for non-REM parasomnias can worsen his limb movements, and I have avoided many of the treatments for periodic limb movements because of their potential for addiction and dependency, of major concern in his case. The dopamine receptor agonist, the standard treatment for restless legs syndrome, triggered further compulsive behaviours. One treatment often used in sleep-related eating disorder is an anti-epileptic drug called topiramate, which acts to suppress appetite. It made no difference at all. At the moment we are trialling iron infusions, the aim being to dampen down the leg movements and address the sleep-related eating disorder and night-eating syndrome in that way. But I do not hold out much hope.
Don has learned to live with his nocturnal appetites. He controls it to some degree by limiting the food accessible to him at night. He has tried to put more healthy food in the fridge, like fruit, but even this can cause difficulties. ‘I was eating too many grapes, and waking up with diarrhoea,’ he shrugs. And, undoubtedly, his sleep-eating puts a strain on his relationship with his wife, and how he functions during the day. He is frustrated by how the disorder he deals with every night is perceived. ‘It is a serious problem that affects your life greatly. I have a very fine career, but that was in spite of the difficulty that I started each day with. I say that because I have more than once seen TV shows on sleep disorders, and each time they include sleep-eating as the comic relief.’
For me, Don’s case exemplifies many of the sleep issues that we see day-to-day in the clinic, perhaps not in its nature but in its origins. Sleep is so dependent on biological and psychological factors – your genes, your anatomy, your brain chemistry – but also the stresses and strains of life – your mood, your anxiety levels, your state of mind. It cannot be seen in isolation, only in the greater context of someone’s life. And if that is the case with sleep in general, it is certainly the case with disorders of sleep too.
12
A PECULIAR FAIRY TALE
Every few months, I sit in a clinic with my paediatric colleagues, seeing youngsters with conditions like narcolepsy or severe sleepwalking. The aim is to smooth the transition between the ‘touchy-feely’ world of the children’s hospital next door, with its brightly coloured, mural-covered walls and helter-skelter in the foyer, and the slightly harsher medical environment of adult medicine. Some of my paediatric colleagues wear lurid yellow-and-purple shirts, with a different shade of hair colour every clinic, and their ID lanyards are festooned with badges or keyring cuddly toys – not a look that my adult colleagues (or I) cultivate.
And with adulthood come new challenges as a patient. The life of a teenager is more complicated, socially, educationally and medically. For the patient, there is an increasing onus on managing one’s own health, and for the teenager and the parents alike, that shift of responsibility can be difficult.
This transitional clinic aims to facilitate the slow weaning of parental involvement, the appropriate handover of care to a new team, and to begin the discussion of the impact of their condition on driving, their social life, alcohol and drugs, and moving away from home. Sometimes, these patients fulfil the stereotype of teenagers – grumpy, monosyllabic, slumped in the chair, devolving responsibility to their understandably overbearing parents who talk for them. But Jamie is about as far away from that teenage stereotype as one could imagine.
Now seventeen years old, he is articulate, thoughtful and conscientious. He is clearly academic and very sporty. He and his entire family are rugby-crazed. He plays at a very high level, for his school and county; his father coaches; and his mother and his younger brothers are also involved in the sport. He harbours ambitions to study medicine at Imperial or Oxford. The prospect of a career in orthopaedics and sports medicine represents a confluence of his love of sport and his academic potential. But, despite his obvious talents, Jamie has other problems to contend with.
‘It started just after my fourteenth birthday,’ he tells me.
I was on a school skiing trip to Serre Chevalier [a French ski resort near the Italian border]. We had a nightmare journey out there. We were caught in a massive snowstorm. The coach was stuck for six or seven hours on the motorway. No food, no drink; forty or fifty of us. No communication. We got to the hotel at two in the morning. Everyone was shattered. But we were there to ski, so at eight o’clock the next morning we got up and went skiing.
The next few days were incredibly strenuous. Jamie and his friends were skiing, ice skating, sledging and staying up late at night. He recalls: ‘One afternoon I started feeling really drowsy, so I took the afternoon off skiing. I thought I was dehydrated, as I hadn’t been drinking a lot of water. I was drinking a lot of Coca-Cola, which obviously wasn’t doing enough. I felt really tired so I fell asleep.’
At that time, he was not on his own. Several other boys were feeling the effects of overdoing it, and some also chose to rest in their rooms that afternoon. But, despite his rest, Jamie continued to feel sleepy, and while his friends went out that night to go ice skating, he elected to stay in bed. ‘And two hours later, once they [his roommates] got back from the ice skating, I woke up with the room full of people. They had played a prank. It was something to do with whipped cream, some silly prank that fourteen-year-old boys do. I woke up and I was really disorientated. I felt quite aggressive towards the boys that had done it. I remember just being in a complete daze.’
But, despite not feeling well, Jamie continued to ski the following day. ‘I vividly remember going up the ski lift thinking, Something’s not right, and falling asleep on the lift.’ Jamie continues: ‘It was a really tricky few days, not feeling well. I just thought that it was some sort of ailment, just some sort of a bug. So I slept in the coach all the way back to the airport. I was really disorientated in the airport, [and] got lost from the group. I didn’t know where I was, I didn’t know what was happening.’
On Jamie’s return home, he remembers being ravenously hungry. ‘I sat down and started eating dinner. And I was eating really, really quickly. I remember my family saying, “Just calm down!” They were asking me about the trip, and they were cross with me because they had paid a lot of money for me to go on this trip and I was just silent. I wasn’t saying anything.’
At this point, Jok, Jamie’s father, takes up the story. ‘We went off to rugby as normal on Sunday. We left him at home. We got back in the afternoon and he was still asleep. Jamie was still not rising. We thought maybe he was still asleep because of dehydration; maybe he had bumped h
is head and had a bit of concussion. But we knew something wasn’t right.’ Oriele, his mother, describes what he was like: ‘Vacant. Big pupils. Just staring, and nothing was getting through, no communication.’ It was at this point that Jamie’s parents thought it was time to take him to hospital.
In the emergency department, after initial tests all returned normal, the doctors presumed that Jamie was suffering from a psychiatric condition. Oriele recalls them being interrogated by the doctors. ‘They were really grilling him about what drugs he had taken. And eventually he said: “I did take a Nurofen because I had a headache,’ ” she laughs. ‘He managed to say that. But then the doctor there said that in the nine years he had been working there, the only time he had ever seen a child like this was when they had been sexually abused.’
Jok still recalls this moment with horror. ‘So of course at this point alarm bells were ringing. We were really concerned.’
Eventually, after several hours, Jamie’s parents were allowed to take him home for the night, but he was readmitted the following day. Jamie recalls lying in a room on a bed, watching the planes flying into Heathrow overhead. ‘I was put on suicide watch. I remember a rather eccentric man looking after me. I was there for a while, eating really horrible food and watching lots of TV, not really doing anything. I don’t personally remember sleeping that much. I just remember my family coming in and me manically eating Jaffa Cakes.’
Over the course of the next few days, Jamie had a lumbar puncture, brain scan and an EEG. He also remembers being separated from the family for a psychiatric assessment. ‘That for me was really scary,’ he recalls. ‘Some of the things I said were really uncharacteristic. I was saying I was suicidal and I had thought about killing myself with an aerosol can. I wasn’t thinking about what I was saying.’ But it was shortly after this that Jamie and his family were given a diagnosis.
The opinion was that Jamie had developed a condition called PANDAS, a rare autoimmune neurological disorder triggered by streptococcal sore throat. Antibodies directed against the streptococcus bacterium attack a part of the brain called the basal ganglia, and cause psychiatric symptoms as well as some abnormal movements. Jamie and his family remember the diagnosis as a source of relief. He was treated with intravenous antibiotics, and after a few days he gradually returned to normal. After a couple of weeks, he was back at school.
Jok says: ‘Obviously the PANDAS diagnosis wasn’t 100 per cent clear. We could tell the doctors weren’t convinced. But that’s what seemed to fit the bill at the time. But by then he was out and he was better and we thought, Phew! We had our Jamie back.’
Life for Jamie and his family quickly returned to normal, and they soon put this episode behind them. Jamie continued to study hard and play hard, now playing rugby for a local club as well as everything else in his already busy life. Normality resumed.
A year and a month after his first episode, however, Jamie went to a party with his friends. Rugby players are not renowned for their abstinence from alcohol, and I ask if he had been drinking. ‘I have never drunk excessively, but I had had a bit of alcohol, been at this party, and got home quite late. The next morning it kind of hit me again, so I slept all day in a darkened room.’ It was immediately obvious to his parents when they got home that something was not right. He was again very sleepy. Oriele says: ‘It was so similar to before. It was that vacant stare again. So we took him straight back into hospital. It is strange walking into an A&E department with a fully grown young man – physically fine, but you’re trying to explain he is not communicating, not functioning.’
This admission was no less traumatic than the first. He was kept in a room with doctors walking in and out. ‘One doctor, a child psychiatrist, was so rude,’ Jok recalls. ‘He just walked in, said: “Acute psychosis!”, and walked straight back out again.’
For the next ten days, Jamie remained in hospital. His behaviour was strikingly abnormal. ‘[He was] manically eating,’ says Jok. ‘We couldn’t leave him with a packet of biscuits. CAMHS [child and adolescent mental health services] came in to see him again and he had a packet of biscuits. He ate the whole packet in one fell swoop, which they thought was extraordinary.’ Jamie has some memories of this episode. ‘I got quite stroppy when my grandparents came to visit. I didn’t like anyone unfamiliar. I couldn’t speak. I remember speaking to my mum, using Notes [the app] on her phone. I was swearing a lot. They asked me what I’d had to eat or drink, and I just said, “Fucking water!’ ”
Oriele confirms this: ‘Lots of swearing, which is so unlike him. Things like “Bitch!”, which he would never say, or at least I hope not!’ His parents tell me the psychiatrists soon decided that this was not a mental health issue – ‘We are off, we have done our job, we have checked him out, it is not mental.’
It was not until the following day that Jamie began to show signs of other alarming behaviour. Oriele reports: ‘Jamie was different, doing strange things around me. It was totally out of character.’ Jok chips in: ‘It was inappropriate suggestions and things you wouldn’t do really around any woman, but especially around your mum.’ At this point, I see Jamie shifting uncomfortably in his chair, and throwing a look across to his girlfriend Megan, who by this time has joined us. I can see the colour rising in his cheeks, and feel sorry for his embarrassment. Oriele continues, in a very matter-of-fact way, to describe Jamie’s highly inappropriate actions. Jamie winces at hearing the details, and she apologises to him for causing him discomfort. ‘I then realised that I needed to keep my distance and not get too close to him.’ Jok says: ‘So we became quite protective of him and we made sure that invariably I was there, especially when the nurses were coming in.’
* * *
In the strange world of sleep disorders, there is no condition quite as extraordinary as Kleine–Levin syndrome, or as recognisable. Jamie’s episodes bear all the hallmarks of this condition, known as KLS, and on our first meeting, just after his sixteenth birthday, I tell him and his family that I am pretty certain that this is what he has. In fact, his parents already have an inkling as to the diagnosis. One of the psychologists during his second admission had said to them that he was interested in neurology, and had read about KLS. He had raised it as a possible explanation, but when Jamie’s family raised it with his medical team, the suggestion was met with short shrift.
‘We mentioned it to the doctors, but they dismissed it,’ Jok tells me. ‘But we began reading about it. There were an awful lot of boxes being ticked as we read about the symptoms. The sleeping, the excessive eating, the sexual behaviour, guarded behaviour, derealisation.’
I ask Jamie more about this. ‘It feels like I’m drunk. I’m not in my body, like I am almost watching myself. This is really tricky to describe. Like I was in a tunnel and I could see everything that was happening, but I could have no impact on what was outside me. I couldn’t feel my hands, my legs, and my feet. I couldn’t speak. I could think but I couldn’t speak.’
There are very few conditions in modern medicine that we have almost no inkling as to their cause. KLS is one of these disorders. We do know it is rare – exceedingly so. It is thought to affect between one and five in every million people, although it is probably more common in certain ethnicities, such as Ashkenazi Jews. We know what it looks like, and in fact it has been described for centuries. The first probable case in the medical literature dates back to 1705, in a paper unimaginatively entitled, ‘Relation of an extraordinary sleepy person at Tinbury near Bath’. But it was not until the mid-twentieth century that this syndrome – a syndrome being an association of symptoms rather than a disease – was better described.
The earliest modern descriptions, including those by Willi Kleine and Max Levin, physicians in Germany and Baltimore respectively, were of young men struck down by episodes of profound sleepiness, associated with ‘morbid hunger’ and marked hypersexuality. Since its original description, our understanding of the spectrum of symptoms that constitutes this syndrome has progres
sed a little. Whereas previously thought to be a condition almost exclusive to males, it is likely that the hypersexuality exhibited in boys is more immediately recognisable and leads to a quicker diagnosis. Nevertheless, KLS is still thought to affect more males than females. It can start at any age, but usually appears in adolescence. Occasionally, first episodes can start in childhood, and the oldest reported patient had his first attack in his eighties, although this is highly unusual. Jamie’s age of onset is very typical, as are his episodes.
Patients usually have bouts of behavioural change lasting days or weeks, in rare cases up to several months, recurring every few months. As with Jamie, the usual picture is one of profound sleepiness, so irresistible that patients will sleep anywhere. I have read of patients crawling under cars and being found asleep when someone has driven away, or, as Jamie describes, on a ski lift. One of my patients has gone to sleep on the concrete floor of an airport terminal, surrounded by hordes of passengers. Sleep may constitute up to twenty-two hours of the day, and the degree of sleepiness can be so severe that sufferers may only get up to eat or go to the toilet. I can recall at least one patient whose parents would leave bottles of fizzy drinks next to the bed. The only evidence of him having risen would be that the bottles of cola would be replaced with bottles of urine. When challenged to get up, the young person will often be incredibly difficult to rouse, and when woken will become very aggressive, with violent outbursts of swearing. These episodes of sleepiness have led to KLS being described as ‘Sleeping Beauty Syndrome’, a headline much loved by the tabloid newspapers, whenever a story on KLS is featured. Patients and their families hate this title with a passion, however, believing that it belittles the effects of the condition on people’s lives, and romanticises KLS.
The Nocturnal Brain Page 22