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Background and Objective: Although there are several treatments for narcolepsy type 2 and idiopathic hypersomnia, studies that assess amphetamines, symptoms beyond sleepiness, and comparative effectiveness are needed. We performed a randomized, fully blinded, noninferiority trial of modafinil versus amphetamine-dextroamphetamine in these disorders., Methods: Forty-four adults were randomized to modafinil or amphetamine-dextroamphetamine, individually titrated to a maximum of modafinil 200 mg twice daily or amphetamine-dextroamphetamine 20 mg twice daily, for 12 weeks. Primary outcome was change in Epworth from baseline to week 12, with a noninferiority threshold of 2 points. Secondary outcomes were (1) patient global impression of change measures of disease severity, sleepiness, sleep inertia, and cognition; (2) change from baseline in Hypersomnia Severity Index; and (3) change from baseline in Sleep Inertia Questionnaire. Adverse events were compared between groups., Results: Epworth improved 5.0 [± standard deviation (SD) 2.7] points with modafinil and 4.4 (± SD 4.7) with amphetamine-dextroamphetamine; noninferiority of amphetamine-dextroamphetamine was not demonstrated (P = 0.11). Noninferiority of amphetamine-dextroamphetamine was demonstrated for change scores of severity, sleepiness, sleep inertia, Hypersomnia Severity Index, and Sleep Inertia Questionnaire. Dropouts due to adverse events were 31.8% for modafinil (including two severe events) and 9.1% for amphetamine-dextroamphetamine, P = 0.13. Anxiety was more common with modafinil and appetite suppression with amphetamine-dextroamphetamine., Conclusion: Noninferiority of amphetamine-dextroamphetamine to modafinil was not demonstrated for the primary outcome. However, amphetamine-dextroamphetamine was noninferior on multiple secondary measures of disease severity and symptomatology. These data may inform shared decision-making regarding treatment for idiopathic hypersomnia and narcolepsy type 2., Registration: Clinicaltrials.gov Registration (NCT03772314) 12/10/18. ., (© 2024. The Author(s), under exclusive licence to Springer Nature Switzerland AG.)

Competing Interests: Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper.

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., (Copyright © 2021 The Cochrane Collaboration. Published by John Wiley & Sons, Ltd.)

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., (© 2019 American Academy of Sleep Medicine.)

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., (Published by Elsevier Inc.)

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., (Copyright © 2016 Elsevier Inc. All rights reserved.)

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., (© 2016 American Academy of Sleep Medicine)

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., (© 2016 American Academy of Sleep Medicine.)

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., (© 2016 American Academy of Sleep Medicine.)

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.

The importance of the cerebellum in sleep disorders, and vice versa, is only beginning to be understood. Advanced neuroimaging modalities have revealed cerebellar changes in both common and rare sleep disorders. Sleep disorders in those with genetic cerebellar disease, such as spinocerebellar ataxia, Friedreich ataxia, Joubert syndrome, and ataxia-telangiectasia, include excessive daytime sleepiness, restless legs syndrome, periodic limb movements of sleep, obstructive apnea, central apnea, and rapid eye movement behavior disorder. Sleep medicine is an important and under-recognized part of the neurologic evaluation in those with cerebellar disease., (Copyright © 2014 Elsevier Inc. All rights reserved.)

Background: The suprachiasmatic nucleus (SCN) plays a critical role in maintaining melatonin and sleep-wake cycles., Methods/patient: We report a case of 38-year-old woman who, after gunshot wound to the right temple, developed a sleep complaint of multiple nocturnal awakenings and several naps throughout the day., Results: Computerized tomography and magnetic resonance imaging revealed bilateral optic nerve and optic chiasm damage. Diagnostic polysomnography and actigraphy revealed an irregular sleep wake rhythm., Conclusions: We speculate concurrent damage of the SCN and optic nerves bilaterally resulted in the posttraumatic irregular sleep-wake rhythm.

This is a case of paraneoplastic overlap of limbic encephalitis and opsoclonus myoclonus in a patient with non-small squamous cell lung carcinoma.

Background: Airway patency in both children and adults depends on the tonic and phasic activation of muscles of the tongue and pharynx supplied by the hypoglossal nerve arising at the medullary level., Methods/patient: We report a case of a 2-year-old who after resection of fourth ventricle anaplastic ependymoma developed severe sleep disordered breathing and tongue fasciculation., Results: Polysomnography showed severe obstructive sleep apnea with oxygen desaturation to 33%. Magnetic resonance imaging of the brain showed post-surgical effacement of the dorsal lateral medulla., Conclusions: We postulate that damage to the hypoglossal nerve at the level of the medulla contributed to the patient's severe obstructive sleep apnea. Patient was treated with tracheostomy.

This is a retrospective review of PSG data from 8 narcolepsy patients and 8 idiopathic hypersomnia (IH) patients, evaluating electrophysiologic differences between these two central hypersomnias. Spindles were identified according to the AASM Manual for the Scoring of Sleep and Associated Events; and counted per epoch in the first 50 epochs of N2 sleep and the last 50 epochs of N2 sleep in each patient's PSG. Spindle count data (mean ± standard deviation) per 30 second-epoch (spindle index) in the 8 narcolepsy patients was as follows: 0.37 ± 0.73 for the first 50 epochs of N2; 0.65 ± 1.09 for the last 50 epochs of N2; and 0.51 ± 0.93 for all 100 epochs of N2. Spindle index data in the 8 IH patients was as follows: 2.31 ± 2.23 for the first 50 epochs of N2; 2.84 ± 2.43 for the last 50 epochs of N2; and 2.57 ± 2.35 for all 100 epochs of N2. Intergroup differences in spindle count in the first 50 N2 epochs, the last 50 N2 epochs, and all 100 epochs of scored N2 were significant (P < 0.01) as were the intragroup differences between the first 50 N2 epochs and the last 50 N2 epochs.

Introduction: The AASM Manual for the Scoring of Sleep and Associated Events (Manual) has provided standardized definitions for tonic and phasic REM sleep without atonia (RSWA). This study used Manual criteria to characterize REM sleep in patients with narcolepsy and idiopathic hypersomnia (IH)., Methods: A retrospective review of PSG data from ICSD-2 defined patients with narcolepsy or IH, performed by two board certified sleep medicine physicians. Data compiled included REM sleep epochs and the presence in REM sleep of epochs scored as sustained muscle activity (tonic), and excessive transient muscle activity (phasic) as defined by Manual criteria., Results: PSG data from 8 narcolepsy patients (mean age: 27.5 years; age range: 11-55) showed mean ± standard deviation values for: total REM sleep epochs 205 ± 46.1; RSWA/ phasic epochs 56.1 ± 25.4; and RSWA/tonic epochs 15.0 ± 10.7. PSG data from 8 IH patients (mean age: 33.1 years; age range: 20-57) showed mean ± standard deviation values of total REM sleep epochs 163.8 ± 67.9; RSWA/phasic epochs 6.2 ± 3.5; and RSWA/tonic epochs 0.2 ± 0.4. Comparison revealed intergroup differences in phasic REM sleep (p < 0.01) and tonic REM sleep (p < 0.01) were significantly increased in narcoleptics compared to IH., Conclusion: Our retrospective analysis showed that RSWA phasic activity and RSWA tonic activity are significantly increased in patients meeting ICSD-2 criteria for narcolepsy compared to patients meeting ICSD-2 criteria for IH. This robust difference, with further validation, could be useful as electrophysiological criteria differentiating the two disorders and understanding the physiological differences.

Sarcoidosis is a multisystemic granulomatous disease, which uncommonly affects nervous system. However, when present, it may affect both central and peripheral nervous systems and potentially mimics other chronic diseases of the nervous system. Pathogenesis of neurosarcoidosis remains largely unknown, and its diagnosis and management pose serious challenges to clinicians. Early diagnosis and aggressive treatment of neurosarcoidosis are necessary to produce satisfactory clinical outcomes. This review discusses clinical manifestations, current diagnostic studies, and currently available modalities for management of neurosarcoidosis.

Introduction: Excessive fragmentary myoclonus (EFM) consists of brief, asynchronous, twitch-like movements appearing asymmetrically in sleep. The new AASM Manual for the Scoring of Sleep and Associated Events identifies some EFM scoring criteria but does not provide amplitude criteria for scoring EFM. Older observational series have used 50 μVs. We report data from various amplitude criteria using blinded comparisons., Methods: EFMs were analyzed on the polysomnograms of 8 patients (7 men and 1 woman, mean age 57 years, range: 47-79) using a standardized protocol for sensitivity, tonus threshold, impedance, amplitude measurements, and sleep stage. The first 20 minutes each of wake, Stage 1-2, SWS, and REM were analyzed. EFMs ≥ 25, ≥ 40, and ≥ 50 microvolts (μVs) in negative deflection above the baseline were counted in tibialis anterior muscle electromyography (EMG) channels bilaterally., Results: The mean EFM index per minute for wake, regardless of impedance, was: 7.19 ± 5.90 for ≥ 25 μV amplitude; 2.43 ± 2.02 for ≥ 40 μVs; and 2.08 ± 2.23 for ≥ 50 μVs. For sleep stages, the EFM index by stage and amplitude criteria used for measurements were: Stage 1-2: 7.38 ± 5.79 for ≥ 25 μVs; 3.13 ± 3.33 for ≥ 40 μVs; and 2.36 ± 2.66 for ≥ 50 μVs; SWS: 10.05 ± 8.04 for ≥ 25 μVs; 2.71 ± 3.13 for ≥ 40 μVs; and 1.38 ± 1.92 for ≥ 50 μVs; Total REM: 15.96 ± 11.32 for ≥ 25 μVs; 6.32 ± 4.25 for ≥ 40 μVs; and 3.94 ± 3.73 for ≥ 50 μVs; Phasic REM: 19.69 ± 15.45 for ≥ 25 μVs; 8.63 ± 7.06 for ≥ 40 μVs; and 5.52 ± 6.44 for ≥ 50 μVs; Non-phasic REM: 13.93 ± 11.31 for ≥ 25 μVs; 5.16 ± 3.57 for ≥ 40 μVs; and 3.20 ± 2.92 for ≥ 50 μVs., Conclusion: EFM rates increase with SWS and total REM with the highest EFM rates occurring during phasic REM. EFM rates were increased across all sleep stages when impedance was > 30 KΩ.

A 66-year-old man with hypertension presented with complaints of excessive daytime sleepiness (Epworth Sleepiness Score 14/24), dyspnea upon exertion, and episodes of noninjurious dream-enacting behavior. He reported tongue biting when sleeping in the right lateral decubitus position. Medications included atenolol 12.5 mg, lovastatin 20 mg, doxazosin 2 mg, amlodipine 5 mg, isosorbide mononitrate 60 mg, and aspirin 81 mg. He denied headaches, visual changes, dysarthria, dysphagia, or localized weakness. He denied use of alcohol, tobacco, or drugs.

Background: Pharmacologically induced/exacerbated restless legs syndrome (RLS), periodic limb movements in sleep (PLMS), and REM behavior disorder/REM sleep without atonia (RSWA) are increasingly recognized in clinical sleep medicine. A scoring system to evaluate the literature was created and implemented. The aim was to identify the evidence with the least amount of confound, allowing for more reliable determinations of iatrogenic etiology., Methods: Points were provided for the following criteria: manuscript type (abstract, peer-reviewed paper); population size studied (large retrospective study, small case series, case report); explicitly stated dosage timing; identification of peak symptoms related to time of medication administration (i.e., medication was ingested in the evening or at bedtime); initiation of a treatment plan; symptoms subsided or ceased with decreased dosage or drug discontinuation (for RLS articles only); negative personal history for RLS prior to use of the medication; exclusion of tobacco/alcohol/excessive caffeine use; exclusion of sleep disordered breathing by polysomnography (PSG); and PSG documentation of presence or absence of PLMS. For RLS and PLMS articles were also given points for the following criteria: each 2003 National Institutes of Health (NIH) RLS criteria met; exclusion of low serum ferritin; and exclusion of peripheral neuropathy by neurological examination., Results: Thirty-two articles on drug-induced RLS, 6 articles on drug-induced PLMS, and 15 articles on drug-induced RBD/ RSWA were analyzed., Conclusion: Based on scores < or = 10 and trials of medication reduction/cessation, the strongest evidence available for drug induced RLS are for the following drugs: escitalopram; fluoxetine; L-dopa/carbidopa and pergolide; L-thyroxine; mianserin; mirtazapine; olanzapine; and tramadol. Since none of the PLMS articles assessed PLMI in trials of medication reduction/cessation, the strongest evidence based on scores > or = 10 are for the following drugs: bupropion, citalopram, fluoxetine, paroxetine, sertraline, and venlafaxine. Based on scores > or = 10 and/or trials of medication cessation, the strongest evidence for drug induced RBD/ RSWA is for the following drugs: clomipramine, selegiline, and phenelzine.

Zolpidem is a hypnotic which acts at the GABAA receptor and is indicated for short-term insomnia. Sleep related disorders including somnambulism, sleep related eating and sleep-driving have been reported with zolpidem. A 51-year-old insomniac who used zolpidem 10 mg nightly starting at 44 years of age is described. A few weeks after starting zolpidem she began walking, eating, and had one episode of driving while asleep. Episodes of sleep related eating, sleepwalking, and sleeptalking occurred 3 nights per week, 1 to 2 h after sleep onset. After her evaluation, the patient's zolpidem was gradually discontinued, and all sleep related activities immediately ceased. An 18F-FDG-PET was obtained 2 months after discontinuation of zolpidem. The following day, FDG was administered 1 h after oral administration of 10 mg zolpidem, and then a second PET was performed. We report the results and a review of the literature regarding other unintended effects seen with zolpidem use.

Objectives: Patients presenting to the emergency room with an acute or subacute onset of focal neurological deficits are evaluated initially by non-contrast computed tomogram (CT) of the brain. This is primarily carried out to differentiate an ischemic from hemorrhagic stroke. However, other neurological conditions may have a similar clinical presentation as well as only hypodensities on CT scan, thus mimicking ischemic stroke. This review focuses on the advanced neuroimaging modalities that help differentiate these other conditions from a cerebral infarction., Methods: The literature was reviewed in order to ascertain what conditions would clinically and by CT mimic an acute/subacute ischemic infarction, and what advanced neuroimaging techniques would be most useful in differentiating these conditions., Results: Several infectious, inflammatory, metabolic and vascular diseases were found with clinical presentations identical to subacute/acute ischemic cerebral infarction, which also could demonstrate only hypodensities on a non-enhanced CT scan. However, advanced neuroimaging techniques could readily differentiate these conditions from ischemic infarction., Conclusions: As presented in this review, although several diseases initially present a diagnostic dilemma upon presentation because of their clinical and non-enhanced CT similarities to cerebral infarction, advanced diagnostic neuroimaging readily establishes their unique pathologies.