That could be a specific sleep disorder (such as narcolepsy or idiopathic hypersomnia), a medical condition (such as sleep apnea), or a medication (such as antihistamines or beta-blockers).
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Since the underlying disease mechanism is not yet fully understood, treatment efforts have usually focused on symptom management. There are no FDA-approved medicines for idiopathic hypersomnia. The wake-promoting medications used in narcolepsy are also commonly used off-label to help manage the excessive daytime sleepiness of idiopathic hypersomnia. However, the medications currently used for idiopathic hypersomnia are far from satisfactory.
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Daytime naps are generally very long (up to several hours) and are also unrefreshing. Some studies have shown increased frequencies of other symptoms in patients with idiopathic hypersomnia, although it is not clear whether these symptoms are caused by the idiopathic hypersomnia. These symptoms include palpitations, digestive problems, difficulty with body temperature regulation, and cognitive problems, especially deficits in memory, attention, and concentration. Anxiety and depression are often increased in idiopathic hypersomnia, most likely as a response to chronic illness.
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The true primary hypersomnias include these: narcolepsy (with and without cataplexy); idiopathic hypersomnia; and recurrent hypersomnias (like Klein-Levin syndrome).
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There is a very low level of public awareness of idiopathic hypersomnia, which often leads to stigma for those who suffer from it. There is currently no cure, but there are several off-label treatments, which are primarily FDA-approved narcolepsy medications. In the medical literature, idiopathic hypersomnia may also be referred to as IH, IHS, primary hypersomnia, central hypersomnia, or hypersomnia of brain origin. The Diagnostic and Statistical Manual of Mental Disorders, Fourth Edition (DSM-IV) defines idiopathic hypersomnia as EDS without narcolepsy or the associated features of other sleep disorders.
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Idiopathic hypersomnia is a rarity in the public eye and has a very low level of public awareness. Because of this low awareness, patients with idiopathic hypersomnia often need significant support because they are at risk of being isolated and misunderstood. Therefore, the education of relatives, friends, and colleagues helps the patient to function much better with this incurable disease.
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A case series in 2010 found that peripheral vascular symptoms, such as cold hands and feet (Raynaud's-type phenomena) were more common in people with idiopathic hypersomnia than in controls. In addition to difficulty with temperature regulation and Raynaud's type symptoms, other symptoms associated with autonomic dysfunction were noted to occur in idiopathic hypersomnia. These included: fainting episodes (syncope); dizziness upon arising (orthostatic hypotension); and headaches (possibly migrainous in quality). Food cravings and impotence have also been reported.
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For one patient, daily administration of flumazenil by sublingual lozenge and topical cream has proven effective for several years. A 2014 case report also showed improvement in idiopathic hypersomnia symptoms after treatment with a continuous subcutaneous flumazenil infusion. Clarithromycin, an antibiotic approved by the FDA for the treatment of infections, was found to return the function of the GABA system to normal in patients with idiopathic hypersomnia. In the pilot study, clarithromycin improved subjective sleepiness in GABA-related hypersomnia.
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In fact, "the most severe cases of daytime somnolence are found in patients affected by narcolepsy or idiopathic hypersomnia." Surprisingly, excessive daytime sleepiness is even more handicapping than the cataplectic attacks of narcolepsy. Due to the consequences of their profound EDS, both idiopathic hypersomnia and narcolepsy can often result in unemployment. Several studies have shown a high rate of unemployment in narcoleptics (from 30–59%), which was felt to be related to the severe symptoms of their illness.
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Unlike narcolepsy with cataplexy, which has a known cause (autoimmune destruction of hypocretin-producing neurons), the cause of idiopathic hypersomnia has, until recently, been largely unknown, hence its name. However, researchers have identified a few abnormalities associated with IH, which with further study may help to clarify the etiology. Destruction of noradrenergic neurons has produced hypersomnia in experimental animal studies, and injury to adrenergic neurons has also been shown to lead to hypersomnia. Idiopathic hypersomnia has also been associated with a malfunction of the norepinephrine system and decreased cerebrospinal fluid (CSF) histamine levels.
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Given the possible role of hyper-active GABAA receptors in the primary hypersomnias (narcolepsy and idiopathic hypersomnia), medications that could counteract this activity are being studied to test their potential to improve sleepiness. These currently include clarithromycin and flumazenil.
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Furthermore, unlike the polysomnography, it is less expensive and non-invasive. An actigraphy over several days can show longer sleep periods, which are characteristic for idiopathic hypersomnia. Actigraphy is also helpful in ruling out other sleep disorders, especially circadian disorders, leading to an excess of sleepiness during the day, too.
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The MSLT measures, by several nap opportunities in one day, how long it takes a person to fall asleep. It also determines whether REM sleep appears upon falling asleep. It is usually performed immediately after an overnight study. This test is the standard to test for narcolepsy and idiopathic hypersomnia.
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Excessive daytime sleepiness is characterized by persistent sleepiness and often a general lack of energy, even during the day after apparently adequate or even prolonged nighttime sleep. EDS can be considered as a broad condition encompassing several sleep disorders where increased sleep is a symptom, or as a symptom of another underlying disorder like narcolepsy, circadian rhythm sleep disorder, sleep apnea or idiopathic hypersomnia. Some persons with EDS, including those with hypersomnias like narcolepsy and idiopathic hypersomnia, are compelled to nap repeatedly during the day; fighting off increasingly strong urges to sleep during inappropriate times such as while driving, while at work, during a meal, or in conversations. As the compulsion to sleep intensifies, the ability to complete tasks sharply diminishes, often mimicking the appearance of intoxication.
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Even with medication, patients may struggle with these activities. Many patients are chronically tardy to work, school or social engagements and, over time, may lose the ability to function in family, social, occupational or other settings altogether. Idiopathic hypersomnia profoundly affects work, education, and quality of life. Patients often need to drastically adapt their lifestyle after diagnosis.
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The Epworth Sleepiness Scale has been validated primarily in obstructive sleep apnea, though it has also shown success in detecting narcolepsy and idiopathic hypersomnia. It is used to measure excessive daytime sleepiness and is repeated after the administration of treatment (e.g., CPAP) to document improvement of symptoms. In narcolepsy, the Epworth Sleepiness Scale has both a high specificity (100%) and sensitivity (93.5%).
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It may also be effective in reducing excessive daytime sleepiness while improving vigilance in primary hypersomnias, such as idiopathic hypersomnia. The drug has also been used in hepatic encephalopathy. It may have beneficial short‐term effects in people with cirrhosis, but there is no evidence for long-term benefits. The onset of action is rapid, and effects are usually seen within one to two minutes.
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Sodium oxybate is an orphan drug which was designed specifically for the treatment of narcolepsy. Common side effects include nausea, dizziness, and hallucinations. A 2016 study by Leu-Semenescu et al. found sodium oxybate improved daytime sleepiness in idiopathic hypersomnia to the same degree as in patients with narcolepsy type 1, and the drug improved severe sleep inertia in 71% of the hypersomnia patients.
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There are many neurological disorders that may mimic the primary hypersomnias, narcolepsy and idiopathic hypersomnia: brain tumors; stroke- provoking lesions; and dysfunction in the thalamus, hypothalamus, or brainstem. Also, neurodegenerative conditions such as Alzheimer's disease, Parkinson's disease, or multiple system atrophy are frequently associated with primary hypersomnia. However, in these cases, one must still rule out other secondary causes. Early hydrocephalus can also cause severe excessive daytime sleepiness.
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A 1999 study found that sleep deprivation resulted in reduced cortisol secretion the next day, driven by increased subsequent slow-wave sleep. Sleep deprivation was found to enhance activity on the hypothalamic-pituitary-adrenal axis (which controls reactions to stress and regulates body functions such as digestion, the immune system, mood, sex, or energy usage) while suppressing growth hormones. The results supported previous studies, which observed adrenal insufficiency in idiopathic hypersomnia.
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Idiopathic hypersomnia is a neurological disorder which is characterized primarily by excessive sleep and excessive daytime sleepiness (EDS).Narcolepsy and hypersomnia: review and classification of 642 personally observed cases. Roth B. Schweiz Arch Neurol Neurochir Psychiatr. 1976;119(1):31-4 It has historically been rarely diagnosed and is often very difficult to diagnose at an early stage; it is usually a lifelong chronic disease, which is often debilitating.
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" "Planned naps are unhelpful, as they are both long and unrefreshing." Although behavioral approaches have not been shown to improve EDS, the goal, as in CBT (cognitive behavioral therapy), is often to help patients learn to reduce their negative emotional responses (e.g. frustration, anger, depression) to their disease symptoms. Furthermore, because idiopathic hypersomnia "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.
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Idiopathic hypersomnia is a lifelong disorder (with only rare spontaneous remissions) whose symptoms typically begin in adolescence or young adulthood. It is initially progressive, but may stabilize, and its main consequences are professional and social. The disorder is chronic, and the symptoms can be relentless. If an effective medication to control symptoms cannot be found, it can be extremely difficult for people with IH to hold down jobs, remain in school, maintain marriages, and fully engage with their family and friends.
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Avoiding situations that might be dangerous while sleepy, such as driving. It is not safe to drive a car unless the symptoms are under control with medication. Patients are often too sleepy to work or attend school regularly, and they are predisposed "to develop serious performance decrements in multiple areas of function as well as to potentially life- threatening domestic, work-related and driving accidents." Furthermore, these risks are higher for idiopathic hypersomnia patients than for those with sleep apnea or severe insomnia.
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Given the possible role of hyperactive GABAA receptors in idiopathic hypersomnia, medications that could counteract this activity are being studied to test their potential to improve sleepiness. These currently include clarithromycin and flumazenil. Flumazenil, a GABAA receptor antagonist is approved by the FDA for use in anesthesia reversal and benzodiazepine overdose. Research has shown that flumazenil provides relief for most patients whose CSF contains the unknown "somnogen" that enhances the function of GABAA receptors, making them more susceptible to the sleep-inducing effect of GABA.
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Eugeroics (originally, "eugrégorique" or "eugregoric"), also known as wakefulness-promoting agents and wakefulness-promoting drugs, are a class of drugs that promote wakefulness and alertness. They are medically indicated for the treatment of certain sleep disorders including excessive daytime sleepiness (EDS) in narcolepsy or obstructive sleep apnea (OSA). They generally have a very low addictive potential. Eugeroics are also often prescribed off-label for the treatment of EDS in idiopathic hypersomnia, a rare and often debilitating sleep disorder which currently has no official treatments approved by the Food and Drug Administration (FDA).
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Typically, the symptoms of idiopathic hypersomnia begin in adolescence or young adulthood, although they can begin at a later age. After onset, hypersomnia often worsens over several years, but it is often stable by the time of diagnosis and appears to be a lifelong condition. Spontaneous remission is only seen in 10–15% of patients. According to the limited epidemiological data that exists, IH "has more of a female preponderance (1.8/1)." Family cases are frequent, in a range from 25% to 66% without any clear mode of inheritance.
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Researchers have recently found an abnormal hypersensitivity to GABA (the major brain chemical responsible for sedation) in a subset of patients with central hypersomnia i.e.: idiopathic hypersomnia, narcolepsy without cataplexy and long sleepers. They have identified a small (500 to 3000 daltons) naturally occurring bioactive substance (most likely a peptide as it is trypsin-sensitive) in the CSF of afflicted patients. Although this substance requires further identification of its chemical structure, it is currently referred to as a "somnogen" because it has been shown to cause hyper-reactivity of GABAA receptors, which leads to increased sedation or somnolence.
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Those who suffer from idiopathic hypersomnia seems to have some common symtoms like Excessive daytime sleepiness, which is characterized by persistent sleepiness throughout the day and often a general lack of energy, even during the day after apparently adequate or even prolonged nighttime sleep. People with EDS are compelled to nap repeatedly during the day; fighting off increasingly strong urges to sleep during inappropriate times such as while driving, while at work, during a meal, or in conversations. Sleep inertia (also known as sleep drunkeness), which is characterized by having extreme difficulty waking up and feeling an uncontrollable desire to go back to sleep. Clouding of consciousness (also known as brain fog or mental fog), which is characterized by inattention, thought process abnormalities, comprehension abnormalities, and language abnormalities.
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Competence in sleep medicine requires an understanding of a plethora of very diverse disorders, many of which present with similar symptoms such as excessive daytime sleepiness, which, in the absence of volitional sleep deprivation, "is almost inevitably caused by an identifiable and treatable sleep disorder," such as sleep apnea, narcolepsy, idiopathic hypersomnia, Kleine-Levin syndrome, menstrual-related hypersomnia, idiopathic recurrent stupor, or circadian rhythm disturbances. Another common complaint is insomnia, a set of symptoms that can have many causes, physical and mental. Management in the varying situations differs greatly and cannot be undertaken without a correct diagnosis. ICSD, The International Classification of Sleep Disorders, was restructured in 1990, in relation to its predecessor, to include only one code for each diagnostic entry and to classify disorders by pathophysiologic mechanism, as far as possible, rather than by primary complaint.
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