Sleeping Beauty Syndrom

[shutterbug]

I first heard of this today on Good Morning America, but had to leave for work before I could watch the piece.

I think there's also something that's suppose to be on tonight around 9/10 p.m.

Anyway, they are calling "Sleeping Beauty" syndrom when a person sleeps most of a 24-hour period and are truely incapable of staying awake. They likened it to "hibernating in your home."

I personally have experienced this during both my major depressive episodes, with the last one being worse than the first go-round. I have been recovering from this last major depression for many months now and yet last Sunday I spent 23 hours in bed straight - but luckily, my energy returned in time for the work week ahead.

Just wondering if anyone else has heard of this being called "sleeping Beauty" syndrom or disorder or whatever? From everything I've read on depression and bipolar....my understanding has been that the extreme tiredness and over-sleeping is just another symptom that some major depressives go through?

Thoughts? Info?

[velvet]

My daughter is bipolar, crack and heroin user. She has always slept, with no problem before the drug use began. But now with the addiction, when she is using heroin, she sleeps for days and nights only getting up to use the restroom or grab something to drink. When she has the speed, obviously she is awake. Then when she cannot get either drug she really goes into sleep mode. The house could burn down around her and she would not wake or even care. It is unthinkable to me to be able to sleep that much. The depression causes alot of it, but to just keep sleeping....
I will try to catch that on tv tonight. Thanks for the thread.

[velvet]

I just checked the tv

It is on at 10, tonight...on Primetime.

[shutterbug]

thanks Velvet. I'm not an addict, but you bring to mind my ideas about WHY my body does all this uncontrollable sleeping -- MANIA. And the speed your daughter uses probably runs her body into the ground just like my mania.

I guess my theory is that the severe sleeping is a defense mechanism in my body to repair all that's lost from the months or years I've spent hypomanic. Our bodies can only take so much and then, perhaps like a worn out washing machine...we just loose the ability to function complete and then require major repairs.

It's like playing catch-up, I think. Sounds understandable to me that your daughter's body would be doing the same thing - just totally giving out when no more loads of stress can be handled.??

Just thoughts...

[velvet]

That sounds right. She sleeps when she is out of dope and money. She feels hopeless, but not ready to tackle the overwhelming thought of getting clean. She says she is miserable, but doesn't want to stop using.

[Nina Kay]

Velvet,
My son does the same thing after using speed. From what I've heard Shutterbug is right about why this happens. There body is playing catch-up.
((((((HUGS))))))))

[shutterbug]

I missed it, but found it here: http://abcnews.go.com/Primetime/Mine/story?id=1476380.

On the left side of the site, click on the link to video about "medical Mystery: Sleeping Beauty Disorder" and if you have sound on your computer you can watch the entire piece.

[shutterbug]

They are also calling it Kleine-Levin Syndrome and a foundation has been developed for the study of it.

That address is http://www.klsfoundation.org/site/kls/

They are currently looking for KLS volunteers:


IMPORTANT NEW KLS RESEARCH STUDY - VOLUNTEERS NEEDED

An important new KLS research study is being conducted to investigate a possible viral involvement in KLS. The KLS Foundation is assisting by identifying volunteers for this study. We are looking for active cases of KLS that are preceded by flu-like symptoms or an upper respiratory illness.

You will be asked to obtain a nasal sample with a sterile cotton swab immediately before or at the beginning of a suspected or actual KLS episode. The cotton swab is then shipped to the lab at Stanford University. Cotton swabs, shipping vial and container will be provided ahead of time to participating families.

This is an important study that requires samples to be collected from a number of individuals with KLS. If your KLS is active with frequent episodes and you are willing to participate in this study or have any questions, please contact the Foundation at facts@klsfoundation.org or Neal Farber, PhD at KLSResearch2@aol.com.

[shutterbug]

Doctors Familiar with KLS

DOCTORS FAMILIAR WITH KLEINE-LEVIN SYNDROME

This list is provided solely as an aid to those seeking physicians familiar with Kleine-Levin Syndrome. It does not imply an endorsement in any way by the KLS Foundation.

United States:

CALIFORNIA

Emmanuel Mignot, M.D., PhD
Stanford UniversityCenter of Narcolepsy
Dept. of Psychiatry
1201 Welch Road
Stanford, CA 94305-5485
Tel: 650-725-6517
Note: Dr. Mignot heads the KLS Foundation research program.

Dr. Milton Erman or Dr. Merrill Mitler
ScripsPacific Sleep Medical Services
9834 Genessee Avenue, #328
La Jolla, CA 92037858-657-0550


UCLA Sleep Disorder Center
anta Monica, CA
310-319-4062

Dr. Sydney C. Choslovsky
65 N. 14th St.San Jose, CA
Tel: 408-279-1400

Tracy Newkirk, M.D.
1099 D Street
San Rafael, California 94901
Tel: 415-258-1838

Christian Guilleminault, M.D.
Stanford Health Services
Sleep Disorder Clinic
401 Quarry Road – Suite 3301
Stanford, CA 94305
Tel: 650-723-6601

Ruth Noel

David Paltin, Ph.D.
Psychologist173-B
N. Glassell
Orange, CA 92866
714-796-1752


HAWAII

Dr. Ye Hsia (assistant: Janet Brumblay)
Kapiolani Genetics
319 Punahou Street – Suite 530
Honolulu, Hawaii 96826
Tel: 1-800-983-6472

ILLINOIS

Rosalind Cartwright, M.D.
680 Lake Shore Drive
Chicago, Illinois 60068
Tel: 312-642-1065


MASSACHUSETTS

Allan Ropper, M.D.
Joshua D. Katz, M.D.
St. Elizabeth's Medical Center
Department of Neurology
736 Cambridge Street
Boston, MA 02135-2997

Thomas Scammell, M.D.
Beth Israel Boston Sleep Disorder Center
330 Brookline Avenue
Boston, MA 02215
Tel: 617-667-0833/617-667-3237

Norman P. Spack, M.D.
Clinical Director, Endocrine Division
Children's Hospital Boston
Dept of Endocrinology
Harvard Medical School
300 Longwood Avenue
Boston, MA 02115

Joel Herskowitz, M.D.
New England Medical Center -
Floating HospitalDivision of Pediatric Neurology
750 Washington Street
Boston, MA 02111
Tel: 508 879-7532

MISSISSIPPI

South MS Psychiatry Group
Dr. Anthony Bianco
Diane Little
Hattiesburg, MS
601-288-8720

MISSOURI

St. Louis Sleep Center
Dr. Nau

NEW YORK

Orrin Devinsky, MD
Professor of Neurology, Neurosurgery, and Psychiatry
Department of Neurology
New York University School of Medicine
403 E 34th Street
New York, New York 10016
212 598-6412

Edwin H. Kolodny, M.D.
Professor and Chairman
Dept. of Neurology
New York University Medical Center
550 First Avenue
New York, NY 10016

Dr. Michael J. Thorpy M.D.
Montefiore Medical Center
The University Hospital for the Albert Einstein College of Medicine
111 East 210th Street
Bronx, NY 10467-2490
Tel: 718-920-4841

Stephen Balsam, M.D.
105 East 63rd St. – Suite 3A
New York, NY 10021
Tel: 212-751-9116

Dr. Gary Zammit
Sleep Disorders Institute
1090 Amsterdam Avenue17th Floor
New York, New York

OHIO

Dr. Derrick Lonsdale
Cleveland

PENNSYLVANIA

Dr. Ronald E. Dahl
University of Pittsburg Medical Center
412-624-1810

TENNESSEE

Dr. Schoumacher
Director of the Sleep Clinic for LeBonhaur Children’s Hospital
Memphis, TN
His assistant is Linda901-572-3337

TEXAS

Dr. Don Glaze
Houston
713-790-3105

Todd J. Swick, M.D, P. A.
F. A. A. N.
Diplomate, American Board of Sleep Medicine
Sleep Wake Disorders Neurology
8333 Freeway, Suite 102
Houston, TX 77024
713-465-9282
713-465-9248 - Fax

International:

CANADA

Brian Murray MD
Neurology and Sleep Medicine
Womens Colelge Campus
Burton Hall, Room 41860
Grofvenor Street
Toronto, Ontario
Canada M5F1B2
Telephone: 416-323-6486

BELGIUM

Professor Paul Linkowski
Hôpital ERASME
Unité métabolique de psychiâtrie
Laboratoire de sommeil
Route de Lennik, 808
B-1070 BRUSSELS
BELGIUM
tél : +32 2 555 32 21
fax : +32 2 555 69 55
e-mail : Paul.Linkowski@ulb.ac.
Website: www.ulb.ac.be/erasme/

Doctor Pierre Fossion
Hôpital CHU Brugmann
Psychiâtrie
Place Van Gehuchten, 4
B-1020 BRUSSELS
BELGIUM
tél : +32 2 477 22 78
e-mail : pierre.fossion@chu-brugmann.be
website : www.chu-brugmann.be

Professeur Olivier Van Reeth
Hôpital ERASME
Centre d'Etudes des Ryhtmes Biologiques (CERB)
ULB - Université Libre de Bruxelles
CP618, route de Lennik, 808
B-1070 BRUSSELS
BELGIUM
tél : +32 2 555 32 21
fax : +32 2 555 69 55
e-mail : ovanree@ulb.ac.be
www.ulb.ac.be/erasme/

ENGLAND

Professor David Nutt
Psychopharmacology Unit
School of Medical Science
University of Bristol
Bristol BS81TD, England
Tel: 011-44 117 928 9000

FRANCE

Isabelle Arnulf, MD, PhD
Fédération des pathologie du Sommeil
Hôpital Pitié-Salpêtrière
47-83 Boulevard de l'Hôpital
75013 Paris, France

ISRAEL

Nathan Gadot, M.D.
Meir Hospital
Department of Neurology
Stolberg Building 2nd FL
Kfar Saba, 44281
Tel: +972-9-747 2828/2555

Peretz Lavie, PhD
Andre Ballard Chair in Biological Psychiatry
The Bruce Rappaport Faculty of Medicine
Technion - Israel Institute of Technology
Haifa 31096
(collaborates with Dr. Gadot)

Yakov Sivan, MD
Director
Institute of Pediatric Pulmonology
Critical Care and Sleep Medicine
Dana Children's Hospital
Tel Aviv Medical Center
Tel: +972-3-697-4614

--------------------------------

LINKS:

http://www.diseasesconditions.com/

http://www.ninds.nih.gov/disorders/k...eine_levin.htm

http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?DB=pubmed

http://www.rarediseases.org/

http://home.tiscali.be/facewave/kls/

http://groups.msn.com/SyndromedeKlei...ntraideetdinfo

[ranae1221]

I did watch that last night, and I was amazed to hear that something like that exsists! I couldn't imagine what it would be like to sleep for months and then to wake up finding that so much has changed. For example, there was a girl who slept for months, and then woke up to find her grandfather had died. That would be so sad.

[shutterbug]

More stories I found about KLS:

Sleeping Their Lives Away
Mike Bederka

Advance NewsMagazines
Advance.com
---------------------------------------

Sleeping Their Lives Away
Since the age of 13, Steve Maier occasionally has had fortnight-long episodes where typical daily living all but stops. He sleeps more than double the average person, and a trance-like state greets visitors while he's 'awake.'

'You lose some of your motor functions, your ability to reason, your inhibitions,' said Maier, 27, ofSan Jose, Calif. 'A lot of things you see don't seem real.'

Maier's rare condition baffled doctors, and it took four episodes and several specialists for someone to finally diagnose his disorder as Kleine-Levin Syndrome (KLS).

'My Eyes Look Dead'
KLS is a nonfatal condition characterized by hypersomnolence (up to 20 hours a day), excessive food intake, and an abnormally uninhibited sexual drive, according to the National Organization for Rare Diseases (NORD). When awake, KLS sufferers may exhibit irritability, lethargy and/or apathy. They also may appear disoriented and experience hallucinations.

'My family and friends say my eyes look dead, like there's nothing behind them,' Maier describes.

He estimates he has had between 25 and 30 episodes over the past 14 years. They vary from 10 days to 16 days.

'Missing two weeks of your life every six months is obviously difficult, but it's manageable,' said Maier, who couldn't attend his high school prom because of his illness.

A typical KLS episode can last anywhere from 12 hours to a couple of weeks, with three to five days being the most common, said Muhammad Hamadeh, MD, FCCP, medical director of the Sleep Disorders Center at Advocate Christ Medical Center in Oak Lawn, Ill.

KLS primarily strikes adolescent boys, NORD reports, but Maier said he has been in contact with a growing number of female victims.

An Elusive Diagnosis
Doctors don't know why people get KLS. Some blame a malfunction in the hypothalamus, Dr. Hamadeh said. Overeating, uninhibited sexual drive and hypersomnolence are related to that particular region of the brain.

Viral infection, abnormal central nervous system chemistries and encephalitis have been explored as possible causes to the malfunction, Dr. Hamadeh said. Doctors suspect a bout with the flu may have triggered Maier's first episode.

KLS remains elusive because there isn't an abundance of patients to study. The medical literature on the subject only describes 100 to 120 cases, although Dr. Hamadeh and other professionals admit probably more cases go undiagnosed.

'It's a highly unusual disorder,' said Allen Boone, RPGST, co-director of the Institute of Sleep Medicine at Birmingham, Ala. 'I tell the students who come through here you'll be lucky if you ever even see it in the course of your career. Nevertheless, you have to be vigilant for it.'

KLS oftentimes is misdiagnosed as psychiatric problems or just as part of adolescence because the symptoms aren't specific, Dr. Hamadeh said. In Maier's case, physicians first hypothesized drug use, and later viral meningitis, as the reason for his sleep states.

'It took me two years to get diagnosed in Silicon Valley,' Maier said. 'So the person that's living in Timbuktu with the one town doctor will have a hard time getting diagnosed, unless the doctor has read something about it.'

Age the Only Reprieve
Medications like lithium, pemoline and melatonin have been used to treat KLS, but no drug has offered any definitive results. The long period between episodes, Dr. Hamadeh said, can trick doctors into thinking they have actually cured their patient.

'It's hard to assess the response to therapy because the symptoms are so variable, and they wax and wane,' he said. 'It might take a while for the treatment to start working, and by that time, the symptoms have abated anyway.'

Age seems to be the only reprieve, Dr. Hamadeh said. The natural history of the disease is that the time between episodes gets longer and longer until it finally subsides.

He doesn't expect to see many studies and pharmaceutical companies trying to find a cure because KLS is self-limiting.

Maier agrees, 'There's not an outpouring of money for this quirky syndrome that only affects a handful of people.'

A Helping Hand
Knowing that few resources are available, Maier and his family took it upon themselves to help out others with KLS. In 1998, Maier's mother created the Web site www.klsfoundation.org, and soon after its inception the Kleine-Levin Syndrome Foundation obtained nonprofit status. In addition to providing information about the disorder, the organization allows KLS sufferers and their families to share their experiences.

'The biggest benefit of the site is to connect the parents, let them know their child isn't going to die, and that there are other people out there they can call and talk to,' Maier said. 'It at least gives you a sense of belonging. You're not all by yourself.'

In his youth, Maier resented his illness, but the support of friends, family and people he has encountered through the Web site has helped him to overcome his anger. And age has brought him more than perspective.

Maier's episodes used to be six months apart. As he grows older, the attacks have become less frequent. After nearly two years of peace, Maier recently recovered from a two-week spell.

He remains hopeful that every time will be the last. 'It's something that's always hanging over you,' Maier said.

Mike Bederka is editorial assistant for ADVANCEforSleep.com
----------------------------------

The Woman Who Couldn't Wake Up
Cosmopolitan Magazine, September 2000 issue To order a copy contact the Back Issue Dept. 1-800-925-0485
--------------------------------

I Fell Asleep for 5 Months
Visit www.ipc.co.uk
-----------------------------

Kleine Levin Syndrome featured on CNN
Kleine Levin Syndrome (KLS) was featured on the nationally televised CNN show “Paula Zahn Now” and on CNN Headline News on December 29, 2005. The segment featured Eric, a very brave 15 year old, as he struggles with KLS. In addition, the segment featured Dr. Mignot fromStanford University who stated that he is interested in researching possible genetic factors associated with KLS. He further said that more research is needed to determine a definite cause for Kleine-Levin Syndrome.
Here is a transcript of that show.

TRANSCRIPT OF PAUL ZAHN SHOW DECEMBER 2005

COLLINS: Tonight, after a long day of running around and getting things done, we are all looking forward to a good night's sleep. In fact, most of us just can't get enough of it. Three-quarters of us have trouble sleeping, according to the National Sleep Foundation.

But you're about to meet a teenager whose life is a nonstop battle against sleep.

Here's Kareen Wynter now, looking into one of the mysteries of the mind.

(BEGIN VIDEOTAPE)
KAREEN WYNTER, CNN CORRESPONDENT (voice-over): He's only 15 years old, but he's in a race against time. Eric (ph) Haller seizes every moment on the basketball court and at home with friends. Simple things other people take for granted are precious to Eric (ph). He knows it's just a matter of time before he loses control.

UNIDENTIFIED MALE: I'm pretty freaked out about that.
WYNTER: Before he has to sleep again.
UNIDENTIFIED MALE: It's pretty stressful. Sometimes, it's depressing.
WYNTER: This vibrant, outgoing teenager slips into an altered state, in which he sleeps, and sleeps, and sleeps, sometimes for up to 20 hours at a time, day after day, buried beneath a blanket, getting up only to use the bathroom or for a quick bite.

Eric's (ph) biggest fear was getting sick and missing Christmas, just like last year. And, this year, it happened again. UNIDENTIFIED MALE: Do you remember the last time you woke up?

UNIDENTIFIED MALE: No. I just want to sleep.
WYNTER: Most medical researchers have never heard of this rare disorder. It's called Kleine-Levin syndrome. And it's a mystery. No one yet has found the cause. It's marked by episodes of excessive sleep, combative and childlike behavior. Each episode can last for weeks, even months, with patients literally sleeping their lives away. Eric (ph) has missed school, holidays, a large part of his childhood.

Loerry Haller says her son usually falls into an episode twice a month. His sleeping spells can last a week or longer.

LOERRY HALLER, MOTHER: He's going through so much agony right now, and -- in this little hell right now that he's in.
WYNTER: Loerry's life is also on hold.
HALLER: Our life stops, because Eric's (ph) life changes drastically. He cries, and asks, mom, when am I going to be better?
WYNTER: It's 8:00 at night, day nine. Eric (ph) has slept 18 hours today. The next morning, he wakes up, briefly, to use the bathroom.
HALLER: This is day 10, so he has been sleeping for 10 days.
WYNTER: But Eric (ph) goes right back to bed. A few hours go by. Loerry is concerned. Watch what happens when she tries to wake him up in the middle of the afternoon.
HALLER: Aren't you hungry now? You haven't eaten in a long time.
UNIDENTIFIED MALE: No. Get out.
HALLER: Eric (ph), do you feel like maybe you're coming out of it?
UNIDENTIFIED MALE: No. I just said get the (EXPLETIVE DELETED) out.
WYNTER: It wasn't always like this. Loerry says her son began to get sick in the sixth grade. She took him to specialists and psychologists, who invariably told her Eric (ph) was either depressed or on drugs, or even faking his condition. It took two frustrating years until one doctor finally reached a diagnosis, Dr. David Palton. He stumbled on the answer in a 20-year-old textbook.

DAVID PALTON, PSYCHOLOGIST: It talked about a case of a 17-year- old young lady who would go to sleep for -- for a couple of weeks at a time, and talked about her regression in personality. And then, you know, I knew that that was something close to what I was seeing in Eric (ph). WYNTER (on camera): Kleine-Levin syndrome. Finally, the Hallers had a name for Eric's (ph) problem. There are only 500 documented cases worldwide, but this new knowledge was a mixed blessing.

PALTON: Both had a big sigh of relief. It was -- it was bittersweet. It was good and bad news, of course.
WYNTER (voice-over): Dr. Palton says there has been almost no research into KLS. No one has come up with a cure. Each case is different. If they're awakened, some patients might try to stay up in a confused, foggy state. But they quickly go back to sleep.

DR. EMMANUEL MIGNOT, STANFORD UNIVERSITY: There's actually even a few cases where people have died of suffocating from eating and overeating during these episodes.

WYNTER: Dr. Emmanuel doctor is a researcher at Stanford University's Sleep Disorder Clinic.
MIGNOT: We are finding that there's probably a genetic factor that's important in predisposing to Kleine-Levin syndrome.
WYNTER: Dr. Mignot says researchers are still far from a cure. Until then, patients like Eric (ph) Haller will live as much of their lives as they can, in those precious moments of reality, before they have to sleep again.
Kareen Wynter, CNN, Placentia, California.
(END VIDEOTAPE)
COLLINS: And one more thing. Kleine-Levin syndrome first appears during adolescence, and mostly in boys.
------------------------------------------------
The Montel Williams Show

March 14, 2001, THE MONTEL WILIAMS SHOW
-----------------------------------

The "Rip Van Winkle" Sleep Disorder
May 11, 2000, EXTRA
http://extratv.warnerbros.com/cmp/sp...000/05_11b.htm
-----------------------------------------

Sleeping Beauties
October 2, 2000, INSIDE EDITION
------------------------------------

The Sleeping Princess
October 17, 2000, NBC DATELINE
-----------------------------------

Scientific Research Program
A KLS research program has been established at Stanford University under the direction of Dr. Emmanuel Mignot MD, PhD and co-investigator Dr. Isabelle Arnulf. Studies will focus on possible immune, infectious, neurological and genetic bases for the syndrome. The program will use a range of medical and basic research methods, working together with a multidisciplinary team studying hypersomnia and other sleep disorders at Stanford. The research program has begun establishing a comprehensive medical information database about KLS and the first large scale KLS blood sample collection. While the vast majority of cases are sporadic and cases have been reported in all continents, a few very intriguing cases of co-affected family relationships within this rare disease population have been identified. The first investigations will examine HLA and possible other genetic factors predisposing to KLS. This study is part of a larger Stanford study of HLA predisposing genes in various disorders of excessive daytime sleepiness

Objective of the Research Program
Information about KLS is scattered among approximately 100+ published case reports. To obtain a better understanding of the disease, a comprehensive review article is in preparation and a questionnaire has been prepared to accumulate patient data to better characterize KLS symptoms and look for commonalities among patients.A blood sample collection of up to 200 KLS patients and families (with age, sex and ethnic-matched controls) is being established so that various hypotheses about the possible causes of KLS can be readily tested. This blood sample collection will also be a resource available for other investigations and will hopefully foster further research interest in KLS by clinicians and scientists. A consent form has been approved by the Panel for Human Subjects in Medical Research at Stanford which participants will be required to complete to provide samples to the program.

How to Participate in the Research Program
The first step in participating in the program is to contact Stanford University Research Program directly at Arnulf@Stanford.edu. Participants will first be asked to complete a questionnaire.

The questionnaire is designed to collect information about:
· Personal and family medical history, including ethnic information
· History of KLS – symptoms, precipitating factors, kinetics of episodes
· Results of treatments tried, and medical and lab tests taken
After completion of the questionnaire, participants (typically the KLS patient and their parents) willing to provide blood samples will be required to complete a consent form allowing Stanford to use the samples and the data obtained from the samples. Once the form is complete blood sampling kits (with pre-paid FedEx return) will be sent.

Foundation’s Role
In addition to helping fund this research, the KLS Foundation plays an important role in the program by providing contact information on KLS patients and in motivating compliance.

Researcher’s Background
Dr. Mignot is an Associate Professor of Medicine, Stanford Medical School , Professor of Psychiatry and Behavioral Sciences, Director of the Stanford Center for Narcolepsy, and a Howard Hughes Institute investigator, who is very experienced in clinical and basic research in the area of sleep disorders medicine and is internationally recognized as having discovered the cause of narcolepsy. He has received numerous honors, is the co-author of more than 100 original scientific publications, and serves on the editorial board of scientific journals in the field of sleep disorders research.

Research Program FAQ’s
Please use the following link for questions on the Research project:
http://med.stanford.edu/school/Psych...lepsy/KLS.html

Stanford University KLS Research Project Progress Report: June 2005
The KLS research program underway at Stanford University is progressing at a very impressive pace.

Stanford University KLS Research Project
The KLS Foundation has partnered with sleep experts at the Stanford University Sleep Center to establish a basic and clinical research program on KLS. This project began in the fall of 2004 in the laboratory of Emmanuel Mignot, MD, PhD, under the direction of Isabelle Arnulf, MD, PhD, working with a small support team.

The research program began with a thorough review of nearly 200 cases of KLS reported in the literature and a complete analysis of the symptoms, risk factors and treatment responses of patients. This comprehensive review has been submitted for publication.Two valuable resources for KLS medical research have been established1) the first large scale KLS blood sample collection for laboratory investigations(2) a comprehensive questionnaire of KLS patients and parents to create an information database on KLS.

Status of blood sample collection and questionnaire

Compliance has been excellent. To date, a total of 114 patients with KLS who meet the ICSD-2 Revised criteria have taken part in the study by submitting blood samples and completing a detailed questionnaire. In addition to the patients, 164 parents took part in the study, so complete sets of many patients with both parents are available for genetic and other studies. A collection of age, sex and ethnic matched controls of 115 subjects is complete. The patient database also contains families with multiple occurrences of KLS (siblings, father and son, first cousins).

Responses from the questionnaires have been entered in a large confidential Excel database, stored with restricted access at Stanford University. All data entered have been double-checked and cleaned. The questionnaire collected data about personal & family medical history, history of KLS (symptoms, risk factors, kinetics of episodes, many sleep related questions) and results of treatments tried and medical & lab tests taken.

Statistical analysis on KLS vs. controls questionnaire data has begun. Already a higher incident rate in one ethnic group and a correlation with one risk factor have been identified.

Investigations of the blood samples
From the hundreds of blood kits returned, samples of serum, plasma, and buffy coat cells have been prepared, DNA has been extracted and all stored in a blood bank. First studies are designed to look for possible genetic factors predisposing individuals to KLS.

Various autoimmune diseases are tightly linked to a specific human leukocyte antigen (HLA) type. For example, in narcolepsy, over 90% of the patients carry one specific HLA allele.

A first assay of 220 samples of KLS parents and patients has already been typed for a specific HLA allele and the percent positive is not different from that found in the normal population. These samples have been sent to the Stanford University HLA laboratory for full class-II genotyping.

In an additional project, the serum of approximately 100 KLS patients and controls has been prepared for measuring blood protein disease markers. Already, one serum protein, a marker of acute inflammation, has been shown to be twice as high in KLS patients than in controls.

We are also interested in examining a possible viral role in KLS pathology. To maximize the chance of finding viruses or viral markers, blood samples which were collected during a KLS episode or had clear “infectious signs” at an KLS episode onset have been selected for analysis.

Future
In addition to exploring possible hypothesis for the cause of Kleine-Levin Syndrome, these resources will be available for other KLS investigations and will hopefully foster further research interest by clinicians and scientists in the field of sleep medicine.

------------------------------

[shutterbug]

SDRAB Reports
Report to the NIH Sleep Disorders Research Advisory Board (SDRAB) December 2005

Name of Organization: Kleine-Levin Syndrome Foundation
Person Reporting: Neal M. Farber, Ph.D., Co-President
E-mail: facts@klsfoundation.org
Web site: www.klsfoundation.org
Kleine-Levin Syndrome (“KLS’) explained
KLS is an uncommon sleep disorder characterized by recurrent and unusually long episodes (lasting weeks to months) of hypersomnia, associated with behavioral/cognitive disturbances and often megaphagia. KLS primarily strikes adolescents and the cessation of episodes typically occurs in early adult life.

The cause of KLS is unknown. All diagnostic test results on KLS patients to date have been normal. However, nearly half of the patients experience a non-specific, flu-like infectious disease a few days before the onset of their first KLS episode. There are a few reports of co-affected familial relationships.

KLS Foundation
The Kleine-Levin Syndrome Foundation, Inc., established in 1999, has become an important network to identify and support individuals and families affected by KLS. The Foundation exchanges information with patients and the medical community to help in the diagnosis and care of those affected by KLS.

The Foundation continues to raise awareness of KLS. Fox TV News affiliate in Los Angeles aired a show on KLS on November 15th. Fox News came to the house of a 15 year-old boy while he was in a KLS hypersomnia episode and then to his football practice two days after his episode was over for interviews and filming. It was a well-done piece demonstrating a dramatic contrast in sleep state (see http://www.fox11.com/sections/news/onair.asp/). This story has already sparked interest by other TV network news on KLS and sleep disorders.

KLS Research Program Progress
The KLS Foundation has partnered with the Stanford University Sleep Center to establish a basic and clinical research program on KLS under the direction of Dr. Mignot.

A comprehensive review article on KLS has just been published (I. Arnulf, et al, Kleine-Levin syndrome: a systematic review of 186 cases in the literature, Brain, October 17, 2005, http://brain.oxfordjournals.org/cgi/...ract/awh620v1).

In KLS, the association of an infection before the onset of disease, young age at onset, recurrence of symptoms and possible genetic predisposition is suggestive of a possible autoimmune component. Ongoing studies are designed to look for the involvement of HLA, genetic or viral factors in KLS pathobiology.

To pursue this line of research, Stanford University has conducted a systematic study of new KLS cases. Blood samples and extensive clinical data were collected from 108 patients with KLS, most identified through the KLS Foundation. Parent samples were also collected (totaling 80 sets of patient plus both parent, termed “trios”), together with age-match controls. Diagnostic interviews by phone or in person were conducted in all cases.

While this data is still being analyzed, several new findings have already been made, including an increased ethnic predisposition of KLS: 15% of U.S. patients were of Jewish Ashkenazi heritage, a highly significant difference when compared to the 2% population frequency for this group in the United States(p<0.001). This strongly suggests a genetic founder effect in the Jewish population.

Preliminary HLA typing results of the samples has lead to a possible linkage effect in an HLA allele using transmission disequilibrium analysis of the family trios.

These results have spawned further research interests from groups in France and Israel.

Stanford continues to collect KLS blood samples (see http://med.stanford.edu/school/Psych...epsy/KLS.html).

Two KLS medical research resources have been established: (1) A large blood sample collection of KLS patients and parents for laboratory investigations and (2) a comprehensive database of KLS patients and parents, based on the collated questionnaire information. These resources are now available for KLS investigations and will hopefully foster further research interest by clinicians and scientists in the field of sleep medicine.

A scientific session on KLS is being organized for the June 2006 meeting of the Associated Professional Sleep Societies.

Report to the NIH Sleep Disorders Research Advisory Board (SDRAB) June 2005
Name of Organization: Kleine-Levin Syndrome Foundation
Person Reporting: Neal M. Farber, Ph.D., Co-President
E-mail: facts@klsfoundation.org
Web site: www.klsfoundation.org

The Kleine-Levin Syndrome Foundation is pleased to submit this third report to the NIH SDRAB, with a progress report on the KLS research program underway at Stanford University.

Kleine-Levin Syndrome (“KLS”) is a sleep disorder characterized by recurrent and unusually long episodes of hypersomnia, associated with behavioral/cognitive disturbances, megaphagia and rarely sexual disinhibition. These serious episodes last weeks to months and alternate with asymptomatic periods of normal sleep and behavior lasting months to years. KLS primarily strikes adolescents and the cessation of episodes typically occurs in early adult life.

The cause of KLS is unknown. All diagnostic test results on KLS patients to date, including brain imaging, EEG, serum virus titers and CSF examination, have been normal. However, nearly half of the patients experience a non-specific, mainly flu-like infectious disease a few days before the onset of their first KLS episode. While the vast majority of cases are sporadic and cases appear on all continents, there are however a few reports of co-affected familial relationships. In KLS, the association of an infection before the onset of disease, young age at onset, recurrence of symptoms and possible genetic predisposition is suggestive of a possible autoimmune component.

Stanford University KLS Research Project
The KLS Foundation has partnered with sleep experts at the Stanford University Sleep Center to establish a basic and clinical research program on KLS. This began in the fall of 2004 in the lab of Emmanuel Mignot, MD, PhD (mignot@stanford.edu, 650-725-6517) under the direction of Isabelle Arnulf, MD, PhD, (arnulf@stanford.edu, 650 724-8839) working with a small support team.

Progress Report
The research program began with a thorough review of 186 cases of KLS reported in the literature and a meta-analysis of the symptoms, risk factors and treatment responses in patients. This review has been submitted for publication.

Two medical KLS research resources have been established: (1) The first large scale KLS blood sample collection for laboratory investigations and (2) a comprehensive questionnaire of KLS patients and parents to create an information database on KLS.

Blood collection and questionnaire

Compliance has been excellent. To date, a total of 105 patients with KLS who meet the ICSD-2 Revised criteria have taken part in the study by submitting blood samples and a detailed questionnaire. The mean age of the KLS group was 26.1± 11.1 years (range 8 - 66 years) and 81 (77%) patients were male. Reported ethnicities include 88 Caucasian, 14 Jewish, 1 African (French Antilla), 1 Asian (Korean) and 1 Hispanic patient. In addition to the patients, 164 parents took part to the study, so the rate of trios is 78%. A collection of age-, sex- and ethnic-matched controls of 111 subjects is almost complete. The current KLS patient database contains 4 cases with the following family relationships: 2 siblings affected (n=2), father and son affected (n= 1) and first cousins affected (n= 1).< /FONT>

Responses from the questionnaires have been all entered in a large confidential Excel database, stored with restricted access at Stanford University. All data entered have been double-checked and cleaned. We are waiting for the last 2 controls to start statistics on KLS vs. controls questionnaire data.

DNA has been extracted from of all blood samples. Samples of serum, plasma, and buffy coat cells have been prepared. The blood sample bank is stored at –80°C.

Investigations of the blood samples

First studies are designed to look for HLA and possible other genetic factors predisposing to KLS.

Various autoimmune diseases are tightly linked to a specific human leukocyte antigen (HLA) type. For example, in narcolepsy, 88-98% of the patients carry one specific HLA allele (HLA-DQB1*0602), while the corresponding percentages in control populations vary from 12 to 38%. The HLA system has been poorly studied in KLS patients, however a study by Dr. Dauvilliers and Tafti in a limited number of KLS patients suggested a higher than control frequency of another HLA allele (HLA-DQB1*201).

A first assay of 220 samples of KLS parents and patients has already been phenotyped for HLA DQB1*602 and the percent positive is not different from that found in the normal population. These samples have been sent to the Stanford University HLA laboratory for full class-II genotyping. We will try to replicate the reported HLA finding of DQB1*02 association in KLS using a sample collection 3-4 times larger.

The serum of approximately 100 KLS patients and controls have been prepared for measuring blood protein disease markers.
We are also interested in examining a possible viral role in KLS pathology. To maximize the chance of finding viruses or viral markers, blood samples have been selected for analysis which were collected during a KLS episode, closed to the KLS onset and had clear “infectious signs” at KLS onset.

Funding, future
In addition to exploring possible hypothesis for the cause of Kleine-Levin Syndrome, these resources will be available for other KLS investigations and will hopefully foster further research interest by clinicians and scientists in the field of sleep medicine.

This pilot project has been funded by a small grant from the KLS Foundation and we are fortunate that Dr Isabelle Arnulf, the major investigator coordinating this project is on paid sabbatical from Europe. Our research goal has been to gather preliminary data for subsequent outside support in this area. NIH has not funded KLS research, despite the disabling nature of this disease. Although characterized as a rare sleep disorder, its real prevalence is unknown and the success of the Stanford recruitment indicates it may be more prevalent than conventionally thought.
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[shutterbug]

Originally Posted by ranae1221

I did watch that last night, and I was amazed to hear that something like that exsists! I couldn't imagine what it would be like to sleep for months and then to wake up finding that so much has changed. For example, there was a girl who slept for months, and then woke up to find her grandfather had died. That would be so sad.

Yes, it is very sad and dibilitating. I remember very, very little from my 2-year "breakdown" or whatever...and I still tried to work as much as possible, but my boss made life worse. When I attempted to come back from short term leave he suddenly said I had to work a set schedule everyday! I had NEVER had that at the paper before. And my first week back, no matter how hard I tried to get there by his 10 a.m. time...I mostly failed (even though I had people ringing my phone off the hook and banging on my doors and windows most every morning to try and wake me.

When I got there at 10:15 or (God-forbid) 10:30...that resulted in me in his office getting yelled at most of the day and crying my eyes out the rest of the time while working at my desk.

but I was a zombie....pure and simple. No amount of will power could have changed what was going on with me. And at times it was so bad that if my mom had not come over to pour me a glass of water then I would have dehydrated.

And, of course, people like my former boss...just think it's laziness.

[ranae1221]

I went through a simular expeirence, however it was not for 2 years. It was about 6 months before my "breakdown", I dragged myself to work every day. I was working as a receptionist, the most boring job I ever had (i had "quit" my job there in social work because it was just too much at the time), and I could barely do it. I left work at 5pm and went straight to bed. I didn't cook, I didn't do any grocery shopping, didn't do laundry, nothing for about 6 months. I was with my husband at the time and he had to do it all. Course he did while telling me how crazy and messed up I was and needed help.

After 6 months, I was hospitalzed the first time. After the 4 hospitalizations and 1st overdose, I literly slept the first 2 weeks I was there. Got up to eat and that was it. I don't remember much of it either. I took a medical leave for 3 months and then had to quit work and went on disability for awhile.

I was very fortunate though that I worked with a great group of people. They were very supportive, and when I handed in my official resignation, they told me I would always have a job there. Eventually I was able to go back to work there doing what I loved- social work.

[shutterbug]

wow...that's so awsome that your job was so supportive and even let you come back after recovering. Unfortunately, that is rare, but wonderful when employers and co-workers can see that it's an illness, just like anything else (rather than looking down on us as if we are horrible, lazy human beings).

[Lilya]

Interesting!
I wish I had access to American TV, but I haven´t right now. I would have wanted to watch it.

I have had a few periods like that. After a drug binge when I was using. It seems to be common.

I can relate to your story, Ranae and to yours, Shutterbug. What happened to you happened to me just after I lost my fiancé few weeks before we were supposed to be married. It was like a living death. I just slept, got up for a shower, had dinner, slept and hated waking up. I did this for almost a year. I was doing my University thesis in Paris at the time and had student loan, so I could live like this. I had to get a doctor´s certificate to redo the year. I really lost one year.

I also did it for a few months when I had troubles in my second marriage. My husband did everything, just like your´s, Ranae, and he told me also what a disgusting wast of everything I was. I was living with him in London.

I went to Paris to seek help. Eventually I moved from London and divorced my husband. It took a long time to feel awake again.

Sometimes sleep is a way of grief. Sleep is a great healer and it restores the body and mind, particularly after great stress. But it can also become a drug. I felt I was addicted to sleep for a while.

Love and light,

[upanddownjj]

Hi Shutterbug - WOW - Thank you !! Can't figure out why I'm so tired all the time when I'm not REALLY depressed..I am rapid cycling thru-out the day..If I go out - I HAVE to be home by 3pm - or I crash...I'm in & out of bed fm then on...I didn't realize that the hypomania could tire me out like mania can...I've never really had heavy duty mania (other than workaholism before my first crash about 10 yrs ago - I was in bed for 4 yrs)..Saw that show - not mental health sleepin - genetic disorder apparantly only found in Jewish families...wow - that would be awful..At least I got up & down more than that..Can you PM me with your experience with Hypomania ?? Have you ever had electro Shock ?? It has been suggested that it may benefit me and help me off or almost off meds....thx Janni

[shutterbug]

Lilya...the wonder of the internet is that you can watch the episode at this link I missed it, but found it here: http://abcnews.go.com/Primetime/Mine/story?id=1476380.

[shutterbug]

Originally Posted by up&downjj

...I didn't realize that the hypomania could tire me out like mania can...I've never really had heavy duty mania (other than workaholism before my first crash about 10 yrs ago - I was in bed for 4 yrs)..Saw that show - not mental health sleepin - genetic disorder apparantly only found in Jewish families...wow - that would be awful..At least I got up & down more than that..Can you PM me with your experience with Hypomania ?? Have you ever had electro Shock ?? It has been suggested that it may benefit me and help me off or almost off meds....thx Janni

up and down: yes hypos can do the same, just not to the same extent as the full-manias. Basically -- "Everything that goes up must come down."

My hypos are just like yours...in fact, that's how I judge the degree of mine now - is by how much "extra" time I'm spending at work.

yes - the show talked about the Jewish pre-disposition and I don't doubt that, but my thoughts are this: they have only just begun to study this and I don't see how there would NOT be a connection to the type of sleeping I've dealt with (and many of you) and the kind they have found a Jewish pre-dispostion to. And for all I know, I could easily have some Jewish ancestry in my blood...my family is much of a Heinz 57 variety. Call it what you like, a major depressive symptom, KLS or whatever....if it quacks like a duck and walks like a duck and has feathers like a duck -- then it's probably a duck.

Electro Shock -- this is something that only you can decide on, but me personally -- if I could have done it when I was majorly depressed and the meds weren't helping then I would have. Now that I have insurance again...it will continue to remain a possiblity for me (once my 1-year pre-existing clause has passed). There are some small risks involved, but nothing like the old days and the benefits for many are near miraculous. Read up on everything you can about it and don't rush into it. And also be sure and research the doctors who perform this with keen eye....make sure you can trust the person who will be performing such a procedure on you.

I don't know your history, but I'm not aware of ECT allowing people to go off meds completly - especially if you are bipolar (as your name implies).

Hugs,
Jenna