Update on the Treatment of Idiopathic Hypersomnia

Abstract

Purpose of Review Idiopathic hypersomnia is an incapacitating disorder with a profound impact on daytime performance and quality of life. The most commonly used treatment modalities are lifestyle advice and pharmacological therapy. We present an update on the evidence concerning treatment options for idiopathic hypersomnia. Recent Findings Evidence for non-pharmacological interventions is lacking; improvement in symptoms on introducing these interventions is often less pronounced than in narcolepsy. Additional pharmacological treatment is therefore usually initiated. The few treatment studies that have been performed are hampered by small sample sizes and the use of variable and often insufficiently validated outcome parameters for the whole spectrum of idiopathic hypersomnia symptoms. Conclusion Evidence on treatment is scarce. Since the efficacy of modafinil is consistently described and there is much experience with this substance, it is reasonable to start with modafinil as a first choice treatment. Methylphenidate and dexamphetamine are good alternatives. In the future, newer drugs such as sodium oxybate, pitolisant, and solriamfetol might be authorized for use in idiopathic hypersomnia.

Efficacy and Safety of Lower-Sodium Oxybate in an Open-Label Titration Period of a Phase 3 Clinical Study in Adults with Idiopathic Hypersomnia

“…12 , 14 In clinical practice prior to the approval of LXB for the treatment of idiopathic hypersomnia in the United States, patients with idiopathic hypersomnia may have taken more than 1 off-label medication to manage their idiopathic hypersomnia symptoms, 45 or cycled through multiple off-label treatments sequentially due to lack of efficacy. 22 , 23 In the present study, similar efficacy was seen between participants entering and maintaining concomitant treatment with alerting agents (traditional stimulants or wake-promoting agents) and participants who were treatment naive, indicating that LXB may be useful as first-line therapy as a single agent, or in combination with alerting agents.…”

“…Even physicians who may have experience using SXB or LXB in patients with narcolepsy likely had less familiarity with treatments for idiopathic hypersomnia, as there were no FDA-approved therapies for idiopathic hypersomnia at the time of the study. 22 , 23 Finally, it has been demonstrated previously that while many participants with narcolepsy showed a clinically meaningful response to SXB within 2 months, on average, a period of over 3 months is required to reach the maximum therapeutic response to SXB in terms of EDS. 24 …”

“…Even physicians who may have experience using SXB or LXB in patients with narcolepsy likely had less familiarity with treatments for idiopathic hypersomnia, as there were no FDA-approved therapies for idiopathic hypersomnia at the time of the study. 22,23 Finally, it has been demonstrated previously that while many participants with narcolepsy showed a clinically meaningful response to SXB within 2 months, on average, a period of over 3 months is required to reach the maximum therapeutic response to SXB in terms of EDS. 24 Both ESS and IHSS total scores decreased with open-label LXB treatment during OLT and SDP, whether participants entered the study taking alerting agents or were treatment naive (the 6 participants who entered taking SXB maintained scores in the published range for healthy controls), 18,19 and improvements seen on the IHSS mirrored those observed on the FOSQ-10 and WPAI:SHP.…”

Symptom Severity and Treatment Satisfaction in Patients with Idiopathic Hypersomnia: The Real World Idiopathic Hypersomnia Outcomes Study (ARISE)

“…31 However, these symptom management options do not appear to be as effective as they are in people with narcolepsy; for example, intentional napping during the day is typically unrefreshing for people with idiopathic hypersomnia. 1,19,31,33 Collectively, these results underscore that there was an unmet need for therapies with better efficacy. At the time of this study, there were no approved treatments for idiopathic hypersomnia.…”

The Role of Sodium Oxybate in Idiopathic Hypersomnia: A Case Report Showing Improvement of Excessive Daytime Sleepiness and Reduced Symptoms

“…Non-pharmacological interventions include avoiding even slight sleep deprivation, keeping a light/dark cycle, maintaining a regular nocturnal sleep schedule, general self-care, and sleep hygiene. However, up to now, these measures have not shown scientific evidence of benefit [ 10 ]. Regardless, a study survey conducted in 2017 found that 96% of participants with self-reported IH used non-pharmacological strategies, including caffeine (82%), daytime naps (81%), scheduled nighttime sleep (75%), exercise (58%), temperature control (47%), and diet (40%) [ 11 ].…”