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Background
The International Classification of Sleep Disorders (ICSD) describes primary hypersomnia as an
idiopathic disorder of presumed central nervous system (CNS) cause that is associated with excessive
sleepiness (ie, prolonged episodes of nonrapid eye movement [NREM] sleep). (See Etiology and
Presentation.)[1]
The Diagnostic and Statistical Manual of Mental Disorders, Fourth Edition, Text Revision (DSM-IV-TR)
defines primary (idiopathic) hypersomnia as excessive daytime sleepiness without narcolepsy or the
associated features of other sleep disorders.[2]
In 1966, William Dement proposed that patients with excessive daytime sleepiness, but without cataplexy,
sleep paralysis, or sleep-onset rapid eye movement (REM), should not be considered narcoleptic. [3] In
1972, Roth et al described a type of hypersomnia with sleep drunkenness that consists of difficulty
coming to complete wakefulness, confusion, disorientation, poor motor coordination, and slowness,
accompanied by deep and prolonged sleep.[4] The abrupt sleep attacks seen in classic narcolepsy are not
present in this disorder.
In comparison with narcolepsy, which is characterized by well-defined clinical, polysomnographic, and
immunogenetic features, primary hypersomnia is not well characterized.[5, 6] Moreover, while the DSM-IVTR and the ICSD each define 2 types of idiopathic/primary hypersomnia based on the sleep time duration
(ie, with or without long sleep), the presentation is often heterogeneous. (See Presentation, DDX, and
Workup.)[7]
Please note that while the ICSD prefers the diagnostic label of idiopathic hypersomnia, the DSM prefers
the diagnostic label of primary hypersomnia. As the terms are interchangeable, this article will refer to
them alternately.

Classification
Primary hypersomnia can be classified as monosymptomatic or polysymptomatic. Isolated excessive
daytime sleepiness that is not due to abnormal nocturnal awakenings characterizes the
monosymptomatic form. The polysymptomatic form consists of abnormally long nocturnal sleep and signs
of sleep drunkenness upon awakening.[8]
In the literature, 3 possible subgroups of idiopathic CNS hypersomnia have been suggested.
Subgroup I
These patients have a positive family history, and associated clinical symptoms suggest dysfunction of
the autonomic nervous system. These symptoms include headache, syncope, orthostatic hypotension,
and peripheral vasoconstriction (cold hands and feet).
Subgroup II
This group includes patients who had a viral infection associated with neurologic symptoms, such as
Guillain-Barr syndrome, infectious mononucleosis, or atypical viral pneumonia. Even after their
infectious disease resolves, these patients continue to require significantly more nocturnal sleep and
continue to feel very tired.
Although initially these patients are fatigued, they subsequently have difficulty differentiating fatigue from
sleepiness. To fight tiredness, these patients nap and eventually present with complaints of excessive
daytime sleepiness. Analysis of cerebral spinal fluid demonstrates moderate lymphocytosis (30-50
cells/L with mild to moderate elevation in protein).
Subgroup III
These patients do not have a positive family or viral infection history, and the cause of the disorder truly is
idiopathic.

Recurrent primary hypersomnia


Kleine-Levin syndrome (KLS) is a rare disorder that starts during adolescence and has a male gender
preference. The patients have recurrent episodes of hypersomnia, which are often associated with
compulsive overeating and hypersexuality.[9] The periods of hypersomnia occur for days to weeks at a
time and recur several times a year. In between the symptomatic periods, the patients have normal sleep
requirements and do not have excessive daytime sleepiness. Some patients may develop symptoms of
irritability, impulsive behavior, depersonalization, hallucinations, depression, and confusion. The etiology
of this disorder is not known.[10, 11]
The disorder mainly affects males (68%). The median age of onset is 15 years (range, 4-82y; 81% during
the second decade), and the syndrome may last up to 8 years. The episodes recur every 3-4 months and
may last up to 10 days, but they may last longer in women. (See Epidemiology.)
KLS may be precipitated by infections (38.2%), head trauma (9%), or alcohol consumption (5.4%).
Characteristic symptoms include the following[11] :

Hypersomnia - 100%
Cognitive changes - 96%, including a specific feeling of derealization
Eating disturbances - 80%
Hypersexuality - 43%
Compulsions - 29%
Depressed mood - 48%
Menstrual-related hypersomnia is diagnosed when excessive daytime sleepiness occurs on a periodic
basis over a few days preceding menstruation.[12] It is assumed that the symptoms follow hormonal
changes, but the etiology of the syndrome, as well as its prevalence and course, are virtually unknown.
The ICSD recurrent hypersomnia is classified separately to describe the recurrent form of primary
hypersomnia according to the DSM-IV-TR.

Etiology
Primary hypersomnia is an idiopathic disorder. Although head injury or viral infections can cause a
disorder resembling primary hypersomnia, the true causes for most cases remain unknown. No genetic,
environmental, or other predisposition has been identified.[6]
Excessive daytime sleepiness has been described in a subset of patients following viral illnesses such as
Guillain-Barr syndrome, hepatitis, mononucleosis, and atypical viral pneumonia. Familial cases
associated with HLA-Cw2 and HLA-DR11 genotypes have also been reported.[13] However, the majority of
the patients diagnosed with idiopathic hypersomnia have neither a positive family history nor a past
medical history of viral illnesses.
In experimental animal studies, destruction of the nonadrenergic neurons of the rostral third of the locus
ceruleus complex has produced hypersomnia. While trauma has been associated with excessive daytime
sleepiness in a case series, cerebrospinal fluid (CSF) analysis for specific neurotransmitter metabolites
did not differentiate patients with posttraumatic excessive daytime sleepiness from patients with
narcolepsy or other patients with excessive daytime sleepiness. [14]Injury to the adrenergic neurons at the
bundle of isthmus has led to hypersomnia associated with a proportional increase of both NREM and
REM sleep.[15]
Evidence suggests that a dopamine system dysfunction may occur in narcolepsy, while a similar
malfunction of the norepinephrine system may occur in idiopathic hypersomnia. Decreased CSF
histamine levels have been reported in primary hypersomnia, as well as in narcolepsy, but not in nonCNS hypersomnias, suggesting that histamine may be an indicator of a central (versus a peripheral)
origin for hypersomnias.[16]
A major advance in the understanding of the pathology of narcolepsy, a disorder closely related to
primary hypersomnia, was made after the discovery of narcolepsy-associated genes in animals; ie, genes

involved in the pathology of the hypocretin/orexin ligand and its receptor. [17, 18] Low CSF concentrations of
hypocretin-1 and hypocretin-2 in HLA DQB1*0602 were also found in primary hypersomnia, and a
generalized defect in hcrt-2 transmission may be present in this disorder. As hypocretin peptides excite
the histaminergic system by the hypocretin receptor 2,[19] hypocretin deficiency may result in excessive
daytime sleepiness via decreased histaminergic function.[16]

Epidemiology
Occurrence in the United States
While the rates of excessive daytime sleepiness complaints in the general population are between 0.5-5%
of adults (in surveys without a specific consideration of causes/diagnoses), idiopathic hypersomnia is
diagnosed in about 5-10% of individuals who are self referred to a sleep clinic with a chief complaint of
daytime sleepiness.[1] A precise estimation of idiopathic hypersomnia prevalence is complicated by a lack
of clear biologic markers or unambiguous diagnostic criteria.
A study by Ohayon et al suggested that excessive sleepiness is more prevalent than previously
estimated. The study found that with 27.8% of 15,929 individuals from 15 US states reported excessive
sleepiness. Even when using restrictive criteria of frequency at least 3 times per week for at least 3
months despite normal sleep duration, the prevalence was 4.7%.[20]

Sex- and age-related demographics


Gender ratio for idiopathic hypersomnia is unknown. Kleine-Levin syndrome affects males approximately
3 times more often than females.[1]
As with narcolepsy and Klein-Levin syndrome, onset of primary hypersomnia is most common during
adolescence and rare in people older than 30 years. The diagnosis of idiopathic hypersomnia is
complicated by the fact that differentiating between excessive versus long sleep or normal versus
abnormal wakefulness is often difficult in this population.

Prognosis
After a typical onset between the ages 15-30 years, untreated primary hypersomnia presents a chronic,
but stable, course. Idiopathic hypersomnia is a lifelong disorder with no tendency to remit spontaneously.
Consequences of this disease are mostly social and professional in nature.
Daytime sleepiness can lead to depression. Of note, in children, daytime sleepiness can present as
hyperactivity.[1]

Patient Education
While treating patients with primary hypersomnia, the patient's close family should be involved in the
overall education and decision-making process.
Because these disorders may lead to marriage breakdown, extensive counseling for the patient's
partners, educating them about the symptomatology and treatment options, must be part of a
comprehensive management plan.
Patients with primary hypersomnia often need significant support because they are at risk of being
misunderstood as being incompetent or slothful. Therefore, education of relatives, friends, and colleagues
helps the patient to function much better with this incurable disease.
For patient education information, see the Sleep Disorders Center, as well asDisorders That Disrupt
Sleep (Parasomnias) and Narcolepsy.
Medline Plus/National Institutes of Health (NIH) provides concise and to-the-point summaries of the
diagnosis and recommendations for patients and families dealing with primary hypersomnia and KleineLevine syndrome.

The Mayo clinic offers an additional, more comprehensive patient resource onidiopathic/primary
hypersomnia.

Hypersomnia Overview
Hypersomnia is characterized by recurrent episodes of excessive daytime sleepiness or prolonged
nighttime sleep.

Hypersomnia Symptoms
Different from feeling tired due to lack of or interrupted sleep at night, persons with hypersomnia are
compelled to nap repeatedly during the day, often at inappropriate times such as at work, during a meal,
or in conversation. These daytime naps usually provide no relief from symptoms. Patients often have
difficulty waking from a long sleep, and may feel disoriented.
Other symptoms may include:

anxiety,

increased irritation,

decreased energy,

restlessness,

slow thinking,

slow speech,

loss of appetite,

hallucinations, and

memory difficulty.
Some patients lose the ability to function in family, social, occupational, or other settings.

Hypersomnia Causes

Hypersomnia may be caused by another sleep disorder (such as narcolepsy or sleep apnea),
dysfunction of the autonomic nervous system, or drug or alcohol abuse.

In some cases it results from a physical problem, such as a tumor, head trauma, or injury to the central
nervous system.

Certain medications, or medicine withdrawal, may also cause hypersomnia.

Medical conditions including multiple sclerosis, depression, encephalitis,epilepsy, or obesity may


contribute to the disorder.

Some people appear to have a genetic predisposition to hypersomnia; in others, there is no known
cause.

Typically, hypersomnia is first recognized in adolescence or young adulthood.

Hypersomnia Treatment
Treatment is symptomatic in nature.
Changes in behavior (for example avoiding night work and social activities that delay bed time) and diet
may offer some relief. Patients should avoid alcohol and caffeine.

Medications
Stimulants such as the following may be prescribed:

amphetamine,

methylphenidate (Concerta, Metadate CD, Metadate ER, Methylin, Methylin ER, Ritalin, Ritalin LA,
Ritalin-SR), and

modafinil (Provigil).
Other drugs used to treat hypersomnia include:

clonidine (Catapres),

levodopa (Larodopa),

bromocriptine (Parlodel),

antidepressants, and

monoamine oxidase inhibitors.

Outlook
The prognosis for persons with hypersomnia depends on the cause of the disorder. While the disorder
itself is not life threatening, it can have serious consequences, such as automobile accidents caused by
falling asleep while driving. The attacks usually continue indefinitely.

Research on Hypersomnia
The NINDS supports and conducts research on sleep disorders such as hypersomnia. The goal of this
research is to increase scientific understanding of the condition, find improved methods of diagnosing and
treating it, and discover ways to prevent it.

Hypersomnia Topic Guide

Hypersomnia: Hypersomnia is a condition characterized by episodes of prolonged nighttime sleep or

excessive daytime sleepiness. Symptoms include anxiety, increased irritation, decreased energy, slow
thinking, slow speech, loss of appetite, and more. Causes of hypersomnia include other sleep disorders,
physical problems such as tumor or head trauma, medications, medical conditions such as multiple
sclerosis, depression, epilepsy, obesity, and encephalitis, and genetic predisposition. Treatment for
hypersomnia is generally with medication and changes in behavior.