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What is idiopathic hypersomnia? How has it changed your life?
Idiopathic hypersomnia (IH) is a neurological disorder characterized by excessive sleepiness and excessive daytime sleepiness (EDS). The disorder was first described by Bedrich Roth in 1976 and can be divided into two forms: multisymptomatic and monosymptomatic. Typically, the condition becomes apparent in early adulthood, and most patients diagnosed with IH have had the disease for many years prior to diagnosis. According to information from August 2021, there is currently one IH drug approved by the US FDA - Xywav, which is an oral solution of calcium, magnesium, potassium and sodium obatin. In addition, there are several off-label therapies, primarily FDA-approved medications for narcolepsy.
Idiopathic hypersomnia, which may also be called IH, IHS, or primary hypersomnia, belongs to a group of sleep disorders called central hypersomnias.
Symptoms and symptoms
Common symptoms among IH patients include excessive daytime sleepiness, sleep inertia, brain fog, and long sleep durations. Excessive daytime sleepiness is characterized by persistent tiredness during the day and a lack of energy even after getting enough sleep at night. Patients often nap multiple times during the day and feel very sleepy while driving, working, eating, or talking to people.
Sleep inertia is a state of extreme difficulty in waking up, accompanied by an irresistible desire to fall asleep again.
Other symptoms of hypersomnia, such as difficulty concentrating, abnormal thought processes, and speech disturbances, are also common. People with IH may feel forgetful, confused, or unable to think clearly.
Discussion of causes
Unlike narcolepsy, which has a known cause (autoimmune destruction), the root cause of IH remains unknown. Researchers have identified some abnormalities associated with IH in experimental animal studies, which may help further clarify the cause in the future. IH has also been associated with dysfunction of the norepinephrine system and decreased histamine levels in the cerebrospinal fluid.
Diagnostic Process
Idiopathic hypersomnia lacks clearly defined biomarkers. Doctors will usually rule out other possible causes of excessive daytime sleepiness to accurately diagnose IH. In the diagnosis of IH, multiple tests are usually required to obtain objective data, including the multiple sleep latency test (MSLT). However, studies have shown that the validity of the MSLT test is questionable, and many patients with IH still show symptoms of IH even though their test results are normal.
Management and treatment
Because the underlying mechanisms of IH are not fully understood, treatment focuses primarily on symptom management. Xywav became the first FDA-approved IH treatment in August 2021. In addition, many of the stimulants used for narcolepsy are also used in the management of IH.
Central nervous system stimulants are the mainstay of treatment for excessive daytime sleepiness and their primary effect is to increase dopamine release.
However, stimulatory agents are generally less effective for IH and may be poorly tolerated. In addition to drug therapy, cognitive behavioral therapy (CBT) is also believed to be helpful in improving the emotional state and self-efficacy of IH patients.
Impact on quality of life
The impact of IH on work, education, and quality of life can be profound. Patients often need to adjust their lifestyle to avoid situations where sleepiness could be dangerous, such as high-risk work or driving. Research shows that people with IH face higher risks from these activities than people with sleep apnea or severe insomnia.
Epidemiological status
Symptoms of IH usually begin in adolescence or young adulthood and may worsen in the first few years but are usually stable at the time of diagnosis. According to epidemiological data, IH seems to have a more obvious female tendency (1.8/1), and the incidence of family cases ranges from 25% to 66%, but the inheritance pattern is not clear.
Future Research Directions
Currently, the treatment for IH is still in the exploratory stage, and many new research methods and therapies are underway, including the study of GABA receptors and the application of non-invasive brain stimulation technology. These studies have the potential to improve the quality of life of IH patients.
Although IH is considered a rare disease, the impact on patients and their families is profound. How should people treat these rare diseases?
