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Kleine-Leven Syndrome - The "Sleeping Beauty" Disease

This article was written as a response to the question: I want to know about klein leven syndrome
An ailment that may last throughout the patient's entire life time, and is characterized by excessive periods of eating and sleeping for as many as eighteen hours a day, the rare neurological sleeping disorder known as Klein-Leven Syndrome generally affects teenage boys more than any other group of people making the patient sleep most of the day and night, appear childlike, dioriented, confused, lethargic, irritable, aggressive, and full of apathy to the point that they can not attend school, go to work, or even care for themselves. Many of these patients also suffer from hallucinations, become uncommunicative, bedridden, and hypersensitive to light and noise.

Klein-Leven Syndrome:

Characterized by excessive periods of eating and sleeping for as many as eighteen hours a day, the rare neurological sleeping disorder known as the Kleine-Leven Syndrome, or the "Sleeping Beauty" Disease,  generally affects teenage boys more than any other group of people making the patient sleep most of the day and night, only waking up to eat and go to the bathroom.  The ailment can change the patient's demeanors when they are awake, frequently make them appear childlike, disoriented, confused, lethargic, irritable, aggressive, and fill them with apathy to the point they can not attend school, go to work, or even care for themselves.  Many of these patients also suffer from hallucinations. The Kleine-Leven Syndrome can render them uncommunicative, bedridden, unable to focus, and hypersensitive to light and noise.  The syndrome maylast for months, or even years, without any symptoms at all before the patient experiences another episode, that typically reappears with very little warning, and for most patients the syndrome may last throughout their entire life time.

Recurrent Hypersomnia:

Generally diagnosed if the patient experiences symptoms that significantly impact their lives for at least a month or longer, and present in all patients suffering from the Kleine-Leven Syndrome, recurrent hypersomnia may produce excessive sleepiness, especially during the day, that usually provides no relief from symptoms that may include anxiety, decreased energy levels, losses of appetite, slowed speech, slowed thinking, memory problems, and hallucinations.  Recurrent Hypersomnia also has many causes including clinical depression, brain damage, uremia, kidney failure, fibromyalgia, the autoimmune small intestine disorder known as Celiac Disease that causes genetically predisposed patients fatigue, failure to thrive, and diarrhea.  Recurrent Hypersomnia may also be caused by such things as the Restless Leg Syndrome, sleep apnea, narcolepsy, disturbed night time sleep, the Periodic Limb Movement Disorder, adverse reactions to taking particular medications such as psychotropics, drug and alcohol abuse, withdrawing off certain medicines, genetics, being overweight, influenza, and mononucleosis. 

Causes:

There is no known cause for the Kleine-Leven Syndrome and it may be attributable to an autoimmune disorder, or to genetic predisposition, with a malfunction in the hypothalamus, that is located just above the brain stem, and is responsible for certain metabolic autonomic nervous system activities that help control hunger, thirst, sleep, fatigue, and the circadium rhythm cycles.  The hypothalamus is responsive to such things as olfactory stimulus, pheromones that impact behavior, corticosteroids, autonomic inputs, invading microorganisms, neutrally transmitted information from the stomach, heart, and reproductive tract, blood-borne stimulations including insulin, leptin protein hormones that regulate energy intakes and expenditures affecting metabolism and appetite, pituitary hormones, glucose plasma concentrations, angiotensin peptide hormones that constrict blood vessels and raise blood pressures, cytokine protein molecules used in intercellular communications, osmolarity plasma concentrations, the length of day, light, the physiological reaction known as photoperiodism, nucleus of the solitary tract inputs that affect taste, locus coeruleus nucleus pons that produce physiological responses to panic and stress, oxytocin mammalian hormones that are the brain's neuromoderators, and vasopressin neurokypophysical hormones that increase water absorption in the kidneys collecting ducts nephrons.  The hypothalamus also regulates blood pressures, body temperatures, and heart rates.

Symptoms:

Symptoms of the Kleine-Leven Syndrome are cyclical between episodes that may last for days, weeks, or even months and periods of the patient being symptom-free.  These symptoms may include hyposomnia, altered states of mind while the episodes occur, cognitive impairments, amnesia, delusions, hallucinations, changes in eating behaviors during the time episodes happen, megaphagia, excessive hunger, atypical hypersexual behaviors during the episodes, a variety of obsessive compulsions, abnormal speech, depression, excessive body rocking, the excessive desire to start fires, chewing the lips, altered perceptions, and paranoia.

Diagnosis:

Often misdiagnosed as a psychiatric disorder, a manic bipolar disorder, temporal lobe epilepsy, which is a chronic neurological condition that produces recurrent seizures coming from the temporal lobes of the brain, narcolepsy, defined as a chronic disorder of the central nervous system demonstrated by excessive sleepiness at inappropriate times, Idiopathic Recurrent Stupor caused by endodiazepine or benzodiazepine sleep inducing, anti-anxiety, anticonvulsant, muscle relaxing, amnesia causing tranquilizors, metabolic encephalopathic brain diseases, hyperammonemia, that is metabolic disturbances caused by excessive blood ammonia, hypothyroidism, multiple sclerosis, severe depression, and the Kluver-Bucy Behavior Syndrome caused when both the left and right medial temporal lobes of the brain malfunction, particularly in the almond-shaped nuclei groups of the amygdala, that memorize and process emotional reactions, the Kleine-Leven Syndrome is a diagnosis of exclusion and is entirely clinical, with recurrent hyposomnia being the major criteria for establishing the ailment, and it must be combined with cognitive or mood disturbances during episodes, compulsive eating during episodes, hypersexuality during episodes, or abnormal odd behaviors during episodes, to be determined as the Kleine-Leven Syndrome.  Other diagnostic tools that may be used to help identify the Kleine-Leven Syndrome include EEGs, MRIs, polysomnographic studies, brain computerized tomographies, cerebrospinal Fluid Analysis, and Single Photon Emission Computed Tomography radiological investigations.

Treatment Options:

There are currently no defining treatment options available for the Kleine-Leven Syndrome, however, methylphenidate psychostimulant drugs, modafinil analeptic drugs, amphetamines, and other stimulants taken orally may be used to treat the patient's sleepiness, while lithium and carbamazepine mood-stabilizing drugs may be useful in shortening episodes.  Other medications that may also be used for treating symptoms of the ailment with limited success include phenytoin sodium, an antiepileptic that suppresses abnormal brain activities, phenobarbital, and valprorate anticonvulsants.  Therefore, education and family understanding remain two key factors in supporting patients suffering from the Kleine-Leven Syndrome.

Prognosis:

The Kleine-Leven Syndrome possesses a benign clinical course, and the unique feature that in many cases the ailment disappears just as mysteriously as it first appears, with as many as ninety percent of patients afflicted by the syndrome outgrowing it.  The Kleine-Leven Syndrome may also last as long as twenty years or more for patients who also have hypersexuality along with the ailment, or who contract the disease as an adult, and women tend to experience the Kleine-Leven Syndrome longer than male patients do.

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