Kleine Levin Syndrome Foundation
2001 article from Advance for Sleep.com, regardng Steve Maier and his experience with KLS.
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.