Medications for daytime sleepiness in individuals with idiopathic hypersomnia.

BACKGROUND: Idiopathic hypersomnia is a disorder of excessive daytime sleepiness, often accompanied by long sleep times or pronounced difficulty in awakening, in the absence of a known cause. The optimal treatment strategy for idiopathic hypersomnia is currently unknown. OBJECTIVES: To assess the effects of medications for daytime sleepiness and related symptoms in individuals with idiopathic hypersomnia and, in particular, whether medications may: 1. reduce subjective measures of sleepiness; 2. reduce objective measures of sleepiness; 3. reduce symptoms of cognitive dysfunction; 4. improve quality of life; and 5. be associated with adverse events. SEARCH METHODS: We searched the following databases on 4 February 2021: Cochrane Register of Studies (CRS Web), MEDLINE (Ovid, 1946 to 1 February 2021), and reference lists of articles. CRS Web includes randomized or quasi-randomized controlled trials from PubMed, Embase, ClinicalTrials.gov, the World Health Organization International Clinical Trials Registry Platform (WHO ICTRP), the Cochrane Central Register of Controlled Trials (CENTRAL), and the specialized registers of Cochrane Review Groups, including the Cochrane Epilepsy Group. We previously searched the WHO ICTRP separately when loading of ICTRP records into CRS Web was temporarily suspended. SELECTION CRITERIA: Randomized studies comparing any medication to placebo, another medication, or a behavioral intervention. DATA COLLECTION AND ANALYSIS: Two review authors independently extracted data and assessed trial quality. We contacted study authors for additional data. We collected data on adverse events from the included trials. MAIN RESULTS: We included three trials, with a total of 112 participants. Risk of bias was low for the included studies. Two pharmaceutical company-sponsored trials compared modafinil with placebo, involving 102 participants, nearly all of whom had idiopathic hypersomnia without long sleep time. Modafinil significantly improved self-reported sleepiness on the Epworth Sleepiness Scale by 5.08 points more than placebo (95% confidence interval (CI) 3.01 to 7.16; 2 studies, 101 participants; high-certainty evidence). Modafinil also significantly improved disease severity on the Clinical Global Impression of Severity scale by 1.02 points (95% CI 0.11 to 1.93; 1 study, 30 participants; moderate-certainty evidence) and resulted in a greater proportion of participants who were "much improved" or "very much improved" on the Clinical Global Impression of Change (odds ratio (OR) for improvement 5.14, 95% CI 1.76 to 15.00; 1 study, 70 participants; moderate-certainty evidence). Ability to remain awake on the Maintenance of Wakefulness Test was significantly improved with modafinil, by 4.74 minutes more than with placebo (95% CI 2.46 to 7.01; 2 studies, 99 participants; high-certainty evidence). Ratings of exhaustion and effectiveness/performance were improved with modafinil compared to placebo in one study. Number of naps per week was no different between modafinil and placebo across two studies. Participants receiving modafinil experienced more side effects, although the difference did not reach statistical significance (OR 1.68, 95% CI 0.28 to 9.94; 2 studies, 102 participants; low-certainty evidence). One trial studying 20 participants with different disorders of sleepiness included 10 participants with idiopathic hypersomnia, with or without long sleep time, and compared clarithromycin to placebo. We only included the subset of trial data for those participants with idiopathic hypersomnia, per our protocol. There were no significant differences between clarithromycin and placebo for the Epworth Sleepiness Scale, psychomotor vigilance testing, sleep inertia, other subjective ratings, or side effects. AUTHORS' CONCLUSIONS: Modafinil is effective for the treatment of several aspects of idiopathic hypersomnia symptomatology, based on studies predominantly including participants with idiopathic hypersomnia without long sleep times, with low risk of bias, and evidence certainty ranging from high to low. There is insufficient evidence to conclude whether clarithromycin is effective for the treatment of idiopathic hypersomnia. There is a clear need for additional studies testing interventions for the treatment of idiopathic hypersomnia.

Images: Periodic Limb Movements During Sleep Noted on Ventral Thigh Surface Electromyography in an Above-the-Knee Amputated Stump.

None: Ventral thigh surface electromyography may be used to assess for periodic limb movements during sleep (PLMS) in a limb with an above-the-knee amputation. Presence of PLMS in the proximal portion of an amputated lower extremity supports theories of spinal and supraspinal mechanisms in PLMS generation, and demonstrates that intact distal motor efferent pathways and distal sensory afferent pathways are not absolutely necessary for the generation of periodic limb movements. CITATION: Chada A, Hoque R. Periodic limb movements during sleep noted on ventral thigh surface electromyography in an above-the-knee amputated stump. J Clin Sleep Med. 2019;15(8):1183-1184.

Legal Aspects of Sleep Medicine in the 21st Century.

Multiple manifestations of sleep disorders may interact with the law, making it important to increase awareness of such interactions among clinicians. Patients with excessive sleepiness may have civil (and in some states criminal) liability if they fall asleep while driving and cause a motor vehicle accident. Employers may be held vicariously liable because of the actions of sleepy employees. Hence, awareness of causes of excessive sleepiness, such as sleep deprivation and OSA, is increasing among trucking, railroad, and other safety-sensitive occupations. Interestingly, litigation related to perioperative complications because of OSA is more frequent than nonoperative issues such as a failure to diagnose OSA. Parasomnia-associated sleep-related violence represents a challenge to clinicians because they may be asked to consider parasomnia as a possible contributing, mitigating, or exculpatory factor in criminal proceedings. Clinicians should also familiarize themselves with the legal and regulatory aspects of running an independent sleep laboratory. Sleep telemedicine practice using 21st century technology has opened novel and unique challenges to existing laws. In this review, we cover the most common interactions between sleep disorders and the law, including the challenges of excessive sleepiness and driving, other legal issues involving patients with OSA, and the liabilities associated with parasomnia disorder. We will also cover some practical legal aspects involving independent sleep laboratories and the field of sleep telemedicine.

Legal and Regulatory Aspects of Sleep Disorders.

Sleep disorders may interact with the law, making awareness important. Insufficient sleep and obstructive sleep apnea (OSA) are prevalent and associated with excessive sleepiness. Patients with excessive sleepiness may have civil or criminal liability if they fall asleep and cause a motor vehicle accident. Awareness of screening and treatment of OSA is increasing in certain industries. Parasomnia associated sleep-related violence represents a challenge to clinicians, who may be called on to consider parasomnia as a contributing, mitigating, or exculpatory factor in criminal proceedings. Improving access to sleep medicine care is an important aspect in reducing the consequences of sleep disorders.

Severe Postictal Hypoxemia-Associated Seizure in a 12-Year-Old Child.

ABSTRACT: A 9 year-old girl with developmental delay and seizure disorder presented for evaluation of witnessed breathing pauses during sleep that were not associated with snoring or gasping. Polysomnography showed a generalized seizure with post ictal central apnea associated with severe desaturation and a post central apnea brief seizure. This case report discusses the pathophysiology of post-ictal desaturations and breathing abnormalities during seizures and their possible association with sudden unexpected death in epilepsy. The case also demonstrates the utility of polysomnography for the diagnosis of breathing abnormalities associated with seizures.

Sleep-Disordered Breathing in Duchenne Muscular Dystrophy: An Assessment of the Literature.

STUDY OBJECTIVES: The aim of this review is to review the literature on sleep-disordered breathing in Duchenne muscular dystrophy (DMD). METHODS: PubMed was searched with an array of search terms, including "OSA," "obstructive sleep apnea," "sleep-disordered breathing," "muscular dystrophy," "neuromuscular," "Duchenne muscular dystrophy," "polysomnography," and "portable monitoring." All relevant articles were discussed. RESULTS: Eighteen research articles and 1 consensus statement were reviewed, and assessed with relevant data presented. Three early studies prior to 1990 assessed DMD associated obstructive sleep apnea. Five studies assessed positive airway pressure (PAP) ventilation and/or sleep in varying neuromuscular disorders, including a cohort with DMD. Six studies since 2000 include PSG data in exclusively DMD cohorts. Three studies involved portable monitoring (PM). CONCLUSIONS: PSG with transcutaneous CO2 capnography is an important part of the clinical care for those with DMD. The utility of PM in DMD is unclear with only 1 study to date comparing PSG to PM data. Initiation of PAP therapy using bilevel modality may prevent the need for device switching as the disease progresses.

Nocturnal Hypermotor Activity during Apnea-Related Arousals.

ABSTRACT: We present a case of a 50-year-old patient who exhibits nocturnal hypermotor activity occurring exclusively during apnea-related arousals consisting of repetitive lower extremity hip-flapping. This movement is unusual and reflects a new form of lower extremity movement associated with apnea-related arousals.

Continuous Positive Airway Pressure Device Time to Procurement in a Disadvantaged Population.

Introduction. The management of obstructive sleep apnea (OSA) in patients who cannot afford a continuous positive airway pressure (CPAP) device is challenging. In this study we compare time to CPAP procurement in three groups of patients diagnosed with OSA: uninsured subsidized by a humanitarian grant (Group 1), uninsured unsubsidized (Group 2), and those with Medicare or Medicaid (Group 3). We evaluate follow-up and adherence in Group 1. We hypothesize that additional factors, rather than just the ability to obtain CPAP, may uniquely affect follow-up and adherence in uninsured patients. Methods. 30 patients were in Groups 1 and 2, respectively. 12 patients were in Group 3. Time of CPAP procurement from OSA diagnosis to CPAP initiation was assessed in all groups. CPAP adherence data was collected for Group 1 patients at 1, 3, 6, and 9 months. Results. There were no significant differences between groups in gender, age, body mass index, or apnea hypopnea index. The mean time to procurement in Group 1 was shorter compared to Group 2 but not significant. Compared to both Group 1 and Group 2, Group 3 patients had significantly shorter times to device procurement. Conclusion. Time to procurement of CPAP was significantly shorter in those with Medicaid/Medicare insurance compared to the uninsured.