💾 Archived View for gmi.noulin.net › mobileNews › 2695.gmi captured on 2021-12-03 at 14:04:38. Gemini links have been rewritten to link to archived content
-=-=-=-=-=-=-
By Frances Cronin BBC News
Trying to wake Louisa during one of her episodes is difficult
While most teenagers struggle to get out of bed in a morning, Louisa Ball might
take 10 days to fully wake from her slumber, due to a very rare neurological
disorder. So what's it like living with Kleine-Levin Syndrome?
Louisa has slept through holidays, friends' birthdays and half of her GCSEs.
In 2008, aged 14, she had been suffering from flu-like symptoms. She was at her
school in Sussex when she started nodding off in class and behaving strangely.
"I didn't know what I was doing, what I was saying, everyone thought 'hey this
isn't right,'" she recalls.
"I was hallucinating and after that I don't remember anything. All of a sudden
it just went blank and I just slept for 10 days. I woke up and I was fine
again."
Her parents Rick and Lottie watched their daughter becoming fidgety and with
unusual facial expressions as she sank into sleep. The first time was a
frightening experience for them, although Louisa herself says she wasn't scared
by the episode, more puzzled.
"It was really weird, no one knew what was wrong, we just thought it wasn't
going to happen again. And then four weeks later it happened again."
What is Kleine-Levin Syndrome?
KLS is a disease of adolescence, and sometimes will begin after infection or
illness, says Tom Rico of the Center for Narcolepsy & KLS Research at Stanford
University, California.
"An individual with KLS will have sleep episodes, typically lasting between one
and three weeks, with coinciding cognitive disturbance in the few hours of
wakefulness.
"During this time period, a patient will sleep anywhere between 16 to 22 hours
a day, every day, until the conclusion of the episode."
But the excessive sleeping is only half the problem, he says, because when
awake during the episode, patients experience what they describe as a
"dream-like state".
She was finally diagnosed with Kleine-Levin Syndrome (KLS). There is no known
cause or cure but Louisa says it was good to know what it was and that it
wasn't life threatening.
The average time it takes to diagnose the condition is four years, because
there is no test and so it requires a process of elimination of other
disorders.
The disease was named after Willi Kleine, a neurologist from Frankfurt, and Max
Levin, a psychiatrist from New York, who identified patients with similar
symptoms in 1925 and 1936.
Louisa is unusual as KLS usually affects teenage boys, who can also exhibit
hypersexuality and inappropriate behaviour.
As well as excessive sleeping, symptoms include behaviour changes,
irritability, feeling in a dream-like state and binge eating, symptoms that can
be mistaken for normal teenage behaviour. There are no drugs that have
conclusively shown to alleviate symptoms.
'No dreams'
Louisa Ball Louisa was relieved not to miss her school prom
People with the sleep disorder narcolepsy fall asleep immediately, but people
with KLS might sleep more and more over a number of days before falling into
sleep mode.
Louisa says she remembers very little when she wakes up from an episode: "It's
just blank - no dreams. Now I'll remember a lot more that's gone on. Before I
wouldn't remember anything at all. My dad thinks my brain is learning to cope
with it more."
So how do you deal with a disorder that takes over your life so much?
It nearly ruined Louisa's career ambitions, because she slept through most of
her GCSEs but her college allowed her to enrol and she is studying sport
performance and excellence, with dreams of being a dancer.
What happens after an episode?
"After these one to three weeks, the individual will experience a full recovery
from the symptoms upon conclusion of the episode," says Mr Rico.
"All of the symptoms go away completely, sleep habits return to normal, and
cognitive behaviour is no longer affected.
"Sometimes patients describe it like a light switch, where the symptoms will be
turned on or off almost immediately, with this dramatic transition over the
course of a few hours."
A recovered state can last between a month and a year, and symptoms last a
median of eight years, he says. The episodes become less frequent and less
severe, until they finally stop.
The recommended treatment is to allow the patient to sleep, rather than using
medication.
At first, her school teachers didn't understand, she says. "They'd give work to
my brother for me to do and when I went back to school they expected me to have
done it but I'd have slept for 10 days."
Some people with KLS have complained they have lost their friends because they
suddenly disappear for weeks on end but Louisa has a close knit group of
girlfriends. Some even visit her when she's sleeping, just to check she's ok.
When she wakes up, it takes her a few days to fully come round, and her body is
quite stiff so her dancing is affected for while.
"I've never really got upset about it but I sometimes do think 'why me',
because I've always been a normal healthy person. But all of a sudden it
happened and there's no reason why it happened and that sometimes frustrates
me.
"But I've got used to it now and learnt to live with it. I'm a special kid."
The change in behaviour before and during a sleep episode is one of the most
upsetting things for Louisa's parents, who take it in turns to remain with her.
Doctors have told the family it's crucial to wake Louisa once a day to feed her
and get her to the bathroom.
Rick and Lottie Ball Parents Rick and Lottie took Louisa to France to find out
more about the condition
But Lottie admits it can take a while to get her to come round. "I've tried
before to literally force her to wake up but she just starts swearing and gets
so agitated and aggressive."
After watching a video the family made of her while sleeping, Louisa says: "I
look scary, it doesn't look like me, it's like I'm on drugs."
Frustrated by the lack of information in the UK, Louisa was taken by her
parents to the Hospital Piti -Salp tri re in Paris, where researchers are
looking into whether it is caused by a defective gene.
Many sufferers have abnormalities in their temporal lobe, the area of the brain
involved in behaviour and memory. A scan of Louisa's brain function revealed
she does have abnormalities in her frontal lobe but there are no signs that
this has affected her behaviour or memory.
The good news is the disease can also disappear just as suddenly as it came on.
This normally happens after 10 to 15 years.
How rare is it?
It is too rare or too under-reported to give an accurate figure to illustrate
how rare it is, says Mr Rico. Because so many cases go undiagnosed or
unreported, it is impossible to estimate the prevalence, which is why you won't
find that number listed in any literature.
But Louisa is currently going through a good period. She was out doing
Christmas shopping with her best friend this week and has not had an episode in
13 weeks. A few weeks ago she won yet another dance competition.
"It's almost as if I've forgotten about it because I haven't had one in so
long."
Louisa's parents, however, are still watching her constantly for signs she
could be heading into a sleep state.
"It's weird - now I've left school I haven't actually had an episode, they
probably think I was faking it," she jokes.
Comments
All posts are pre-moderated
and must obey the house rules
5 Hours Ago
I suffer with KLS myself and the sleep is only part of it. To be honest, it's
the easiest part for me. It's the other symptoms that, by far, make my life
hell during episodes. I turn child-like, anxious, and have severe memory
problems. I cannot watch television, read, nor hold a conversation. I had a 1.5
year break and now they're not so severe but it stills puts everything in my
life on hold.
8th december 2010 - 23:09
Actually, narcoleptics don't "fall asleep right away". That can happen, but it
doesn't come close to explaining narcolepsy and sleep. Frequently there are
hallucinations--visual, auditory, and tactile--and falling asleep can take a
long time. (Narcolepsy is confusing, and the average time to diagnosis is 14
years.)
I can't imagine going through this as a teen. Louisa is handling it admirably.
8th december 2010 - 19:20
I have possible KLS combined with neurocardiogenic syncope. To those ignorant
enough to consider this a lifestyle choice of 'laziness' need to define a
distinct difference between choosing to sleep and/or a need to sleep.
I used to lay in on weekends and at every opportunity, since my condition I
relish the chance I get to be awake.
Once you lose control of your sleep, the novelty wears off.
8th december 2010 - 18:14
I have had KLS for almost 11 years and it brings your life to a stand still. I
have experienced both the 16-20 hours of sleep a day for up to 2 weeks and
extreme exhaustion for up to 6 months. One is a brief coma the other is a coma
that you are awake for. You can't do anything while awake. Medications didn't
work. I went to a sleep specialist to find help. Strict sleeping schedules help
some.
8th december 2010 - 14:06
I think its great that Louisa doesnt let this get in the way of her life, or
get her down. I think its a bit worrying that people may call her lazy,
teenagers can be lazy but sleeping for 10 days is not normal. Imagine any of us
just being hit with this sleep disorder, just stop and think of how damaging it
could be to your life, if you have children for instance, or a career! Have
some sympathy!!