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Idiopathic hypersomnia

Clinical Presentation and Diagnosis: – Signs and symptoms of idiopathic hypersomnia include excessive daytime sleepiness, sleep inertia, brain fog, and long sleep periods. – Individuals […]

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Clinical Presentation and Diagnosis:
– Signs and symptoms of idiopathic hypersomnia include excessive daytime sleepiness, sleep inertia, brain fog, and long sleep periods.
– Individuals with IH may experience persistent sleepiness throughout the day, strong urges to nap, and clouding of consciousness affecting cognitive functions.
– Excessive sleep without feeling refreshed and unrefreshing daytime naps are key symptoms.
– Diagnosis lacks a clear biological marker, requiring exclusion of other causes of excessive daytime sleepiness through subjective scales and objective tests like polysomnography.
– Reports of sleepiness in IH may relate more to mental fatigue than physiological sleepiness.

Causes and Mechanisms:
– The cause of IH is largely unknown, with associations to destruction of noradrenergic neurons, malfunction of the norepinephrine system, abnormal hypersensitivity to GABA, and decreased cerebrospinal fluid histamine levels.
– An unidentified substance in the CSF may lead to increased sedation in IH patients.
– Decreased cerebrospinal fluid histamine levels have been linked to IH.

Treatment and Management:
– Treatment options for IH include FDA-approved medications like Xywav, off-label narcolepsy medications, and cognitive-behavioral therapy.
– Lifestyle changes, such as maintaining a consistent sleep schedule, regular exercise, and a healthy diet, can improve symptoms.
– Management focuses on symptom control, with low-sodium oxybate (Xywav) being the first FDA-approved treatment.
– Stimulant medications, lifestyle adaptations, and cognitive-behavioral therapy are essential for managing IH symptoms.

Medication Options:
– Stimulants like Methylphenidate and Dextroamphetamine are commonly used for excessive daytime sleepiness, increasing dopamine release and causing side effects like insomnia.
– Non-stimulant wake-promoting medications like Solriamfetol and Pitolisant are used, with interactions with low-dose contraceptives noted.
– Sleep-promoting medications like Sodium oxybate have shown benefits in reducing daytime sleepiness in IH patients.

Research, Impact, and Challenges:
– Ongoing research focuses on understanding the mechanisms of IH and exploring new treatment options.
– The impact of IH on daily functioning, education, and employment is significant, with challenges related to lack of awareness and understanding of the disorder.
– Collaborative efforts, clinical trials, and support from organizations like the Hypersomnia Foundation aim to improve diagnosis and treatment outcomes for individuals living with IH.

Idiopathic hypersomnia (Wikipedia)

Idiopathic hypersomnia (IH) is a neurological disorder which is characterized primarily by excessive sleep and excessive daytime sleepiness (EDS). Idiopathic hypersomnia was first described by Bedrich Roth in 1976, and it can be divided into two forms: polysymptomatic and monosymptomatic. The condition typically becomes evident in early adulthood and most patients diagnosed with IH will have had the disorder for many years prior to their diagnosis. As of August 2021, an FDA-approved medication exists for IH called Xywav, which is oral solution of calcium, magnesium, potassium, and sodium oxybates; in addition to several off-label treatments (primarily FDA-approved narcolepsy medications).

Idiopathic Hypersomnia
SpecialtySleep medicine, Neurology, Psychiatry

Idiopathic hypersomnia may also be referred to as IH, IHS, or primary hypersomnia and belongs to a group of sleeping disorders known as central hypersomnias, central disorders of hypersomnolence, or hypersomnia of brain origin. 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. It occurs in the absence of medical problems or sleep disruptions, such as sleep apnea, that can cause secondary hypersomnia.

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