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Introduction  The absence of standardized reporting for sleep medicine exams across different laboratories can lead to misinterpretation, diagnostic inconsistencies, and suboptimal treatment strategies. This document seeks to establish guidelines for the development of sleep study reports, covering recordings from studies of types 1 to 4, and represents the official position of Associação Brasileira do Sono (ABS; Brazilian Sleep Association) on the standardization of polysomnography (PSG) and cardiorespiratory polygraphies. Materials and Methods  The recommendations for the items to be reported in PSG records were developed by means of a Delphi study, comprised of two voting rounds. In each round, participants had to vote regarding the appropriateness of items to be reported in type-1 to -4 sleep studies, rating them as recommended , optional , or not recommended . The consensus threshold was set at 66% in each voting round, or 75% for the combined responses of recommended and optional . Results  The panel was comprised of 29 experts. After 2 voting rounds and subsequent deliberations by the steering committee, 352 items were included in the final set of recommendations. Consensus was achieved for 339 items (96.3%), of which 145 (41.2%) were classified as recommended , 154 as optional (43.8%), 35 as not recommended (9.9%), and 5 as not allowed (1.4%). No consensus was reached for 13 items (3.7%). The items recommended in this consensus are detailed in the main text. Conclusion  These guidelines provide comprehensive recommendations for reporting diagnostic tests in sleep medicine., Competing Interests: Conflict of Interests GNP is a shareholder at SleepUp Tecnologia em Saúde Ltda. (São Caetano do Sul, SP, Brazil). The other authors have no conflict of interests to declare., (Brazilian Sleep Association. This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. ( https://creativecommons.org/licenses/by-nc-nd/4.0/ ).)

Study Objectives: We performed this study to describe the characteristics of sleep in children with congenital Zika syndrome through polysomnographic assessment., Methods: Polysomnography with neurological setup and capnography was performed. Respiratory events were scored according to American Academy of Sleep Medicine criteria. Children were classified based on neuroclinical examination as having corticospinal plus neuromuscular abnormalities or exclusively corticospinal abnormalities. Neuroradiological classification was based on imaging exams, with children classed as having supratentorial plus infratentorial abnormalities or exclusively supratentorial abnormalities., Results: Of 65 children diagnosed with congenital Zika syndrome, sleep apnea was present in 23 children (35.4%), desaturation in 26 (40%), and snoring in 13 (20%). The most prevalent apnea type was central in 15 children (65.2%), followed by obstructive apnea in 5 (21.7%) and mixed type in 3 (13%). The average of the lowest saturation recorded was slightly below normal (89.1 ± 4.9%) and the mean partial pressure of end-tidal carbon dioxide value was normal. Periodic leg movements were present in 48 of 65 children. Lower ferritin levels were observed in 84.6% of children. Palatine and pharyngeal tonsils (adenoids) were small in most children and not associated with the presence of obstructive apnea. Ventriculomegaly and subcortical and nucleus calcification were the most frequent neuroimaging findings. Supratentorial and infratentorial anomalies were present in 26.7% (16 of 60) and exclusively supratentorial changes in 73.3% (44 of 60). In the neuroclinical classification, isolated corticospinal changes were more frequent and the mean peak in capnography was lower in this group. There was no difference regarding the presence of apnea for children in the neuroclinical and neuroradiological classification groups., Conclusions: Sleep disorders were frequent in children with congenital Zika syndrome, with central sleep apnea being the main finding., Citation: Brandão Marquis V, de Oliveira Melo A, Pradella-Hallinan M, et al. Sleep in children from northeastern Brazil with congenital Zika syndrome: assessment using polysomnography. J Clin Sleep Med . 2023;19(10):1759-1767., (© 2023 American Academy of Sleep Medicine.)

Background: Sleep disorders are commonly observed in children with Down syndrome (DS) and can lead to significant behavioral and cognitive morbidities in these individuals., Objective: To perform a systematic review evaluating sleep disorders in individuals with DS., Methods: Search strategies were based on combinations of keywords: "Down syndrome"; "trisomy 21"; "sleep disorders"; "dyssomnias"; "sleep apnea"; "obstructive"; "sleeplessness"; "insomnia"; "parasomnias"; and "excessive daytime sleepiness". PubMed and Science Direct were used. Only original studies and retrospective reviews in English published between January 2011 and March 2021 were included., Results: 52 articles were included, most of them involving children and adolescents under 18 years of age. The main sleep disorder associated with DS was obstructive sleep apnea (OSA). Some studies reported the presence of cognitive dysfunction in patients with DS and sleep-disordered breathing, and few have been found about parasomnia, insomnia, and daytime sleepiness in these patients. Movement disorders and unusual postures during sleep may be related to disordered sleep breathing in DS. The main treatment options for OSA are continuous positive airway pressure therapy (CPAP), surgery, and weight control. Computational modeling associated with MRI has been used to plan surgical interventions in these patients., Conclusions: Individuals with DS are at high risk of developing sleep-related breathing disorders. The main sleep disorder associated with DS was OSA. The presence of sleep-disordered breathing contributes to a worsening of cognitive function in patients with DS.

Objectives: Restless legs syndrome (RLS) is a frequent comorbid condition associated with distinct unrelated diseases. While the incidence of RLS has not been definitively confirmed, RLS-like symptoms have been reported in a section of Asian population who also had hyperthyroidism. The prevalence of RLS is generally low in Asian populations. Under these circumstances, we hypothesized that in a population where RLS is common, such as in Brazil, RLS could manifest as a comorbid ailment alongside Graves' disease, a common hyperthyroid condition., Methods: In a cross-sectional survey, 108 patients who presented with Graves' disease were analyzed for restless legs or associated symptoms., Results: Twelve patients (11.1%) displayed symptoms of RLS prior to the incidence of Graves' disease. These patients experienced worsening of the symptoms during their hyperthyroid state. Six patients (5.6%) developed RLS, consequent upon the incidence of Graves' disease as per the consensus of the panel of the experts. Fifteen patients (13.9%) also presented with RLS-like symptoms without any discernible circadian feature of the syndrome., Conclusion: Our findings confirm that Graves' disease might trigger restless legs-like symptoms, while the condition of hyperthyroidism could also be complicated by definite RLS.

Objectives: Pediatric obesity and sleep-disordered breathing (SDB) are strongly associated, and both promote metabolic impairments. However, the effects of a lifestyle intervention on the overall metabolic syndrome (MetS) are unknown. The objectives were i) to evaluate the effects of a lifestyle intervention on cardiometabolic risk (CMR), assessed with a dichotomous (MetS) and a continuous (MetScore FM ) instrument, in obese adolescents with and without SDB and ii) to compare the post-intervention cardiometabolic responses between adolescents with persistent (apnea-hypopnea index; AHI≥2) or normalized-SDB (AHI<2)., Methods: Seventy-six adolescents with obesity recruited from two specialized institutions underwent a 9-12month diet and exercise intervention. Sleep and SDB (AHI≥2) were studied by polysomnography. Anthropometric parameters, fat mass (FM), glucose, insulin, lipid and leptin profiles, blood pressure (BP), MetScore FM and MetS were assessed pre- and post-intervention. We performed comparisons between Non-SDB and SDB groups and between Normalized-SDB and Persistent-SDB subgroups., Results: Fifty participants completed the study. Pre-intervention, twenty youth had SDB (40%) with higher insulin concentrations and systolic BP than Non-SDB participants (p < 0.01), for a similar degree of obesity. Post-intervention, MetScore FM (p < 0.001) and MetS prevalence (p < 0.05) were decreased in both groups. Eleven participants (55%) normalized SDB along with a decrease in insulin concentrations and BP (p < 0.05). Triglycerides, total cholesterol and LDL-cholesterol concentrations (p < 0.01) improved equally in the Normalized and Persistent-SDB subgroups., Conclusion: SDB was associated with lower insulin sensitivity and higher BP but did not affect the lipid profile. A diet and exercise lifestyle intervention is effective in decreasing the CMR whether or not SDB was normalized in obese adolescents., (Copyright © 2020 Elsevier B.V. All rights reserved.)

Background and Aims: Pediatric obesity and sleep-disordered breathing (SDB) are associated with cardiometabolic risk (CMR), but the degree of severity at which SDB affects cardiometabolic health is unknown. We assessed the relationship between the CMR and the apnea-hypopnea index (AHI), to identify a threshold of AHI from which an increase in the CMR is observed, in adolescents with obesity. We also compared the clinical, cardiometabolic and sleep characteristics between adolescents presenting a high (CMR+) and low CMR (CMR-), according to the threshold of AHI., Methods and Results: 114 adolescents with obesity were recruited from three institutions specialized in obesity management. Sleep and SDB as assessed by polysomnography, anthropometric parameters, fat mass (FM), glucose and lipid profiles, and blood pressure (BP) were measured at admission. Continuous (MetScore FM ) and dichotomous (metabolic syndrome, MetS) CMR were determined. Associations between MetScore FM and AHI adjusted for BMI, sex and age were assessed by multivariable analyses. Data of 82 adolescents were analyzed. Multivariable analyses enabled us to identify a threshold of AHI = 2 above which we observed a strong and significant association between CMR and AHI (Cohen's d effect-size = 0.57 [0.11; 1.02] p = 0.02). Adolescents with CMR+ exhibited higher MetScore FM (p < 0.05), insulin resistance (p < 0.05), systolic BP (p < 0.001), sleep fragmentation (p < 0.01) and intermittent hypoxia than CMR- group (p < 0.0001). MetS was found in 90.9% of adolescents with CMR+, versus 69.4% in the CMR- group (p < 0.05)., Conclusions: The identification of a threshold of AHI ≥ 2 corresponding to the cardiometabolic alterations highlights the need for the early management of SDB and obesity in adolescents, to prevent cardiometabolic diseases., Clinical Trials: NCT03466359, NCT02588469 and NCT01358773., (Copyright © 2019 The Italian Society of Diabetology, the Italian Society for the Study of Atherosclerosis, the Italian Society of Human Nutrition, and the Department of Clinical Medicine and Surgery, Federico II University. Published by Elsevier B.V. All rights reserved.)

Introduction: Sleep is essential for human beings, especially children. Insufficient sleep is linked to somatic and psychological problems. This study aims to describe nocturnal sleep patterns in children aged 7 to 13 years and investigate if sex or weekdays influence sleep habits. It also analyses factors associated with sleep length and the difference between sleep habits on weekends and weekdays., Methods: A retrospective cross-sectional study with questionnaires from children with sleep complaints referred to our service (December 2003 to June 2009) in Sao Paulo City, Brazil. Median of sleep hours, time going to bed, waking up, and the difference in amount of sleep during weekends and weekdays were calculated. A generalized linear model was used to find associations between covariates and a) sleep hours, and b) sleep weekend minus - weekdays., Results: We analyzed 577 children (median 9.5 y, 61% boys). Median bedtime was 22h. Median wake up time was 7h on weekdays and 9h on weekends. Median sleep duration was 9.5h during weekdays and 10h on weekends. The median difference in the amount of sleep during weekends and weekdays was 0.5h (IQR=1.5). Shorter sleep duration was associated with age and school schedule. Higher difference weekend - weekdays was associated with older children, girls, and school schedule., Conclusion: Children 7 to 13 years usually sleep more on weekends. Age, morning and full-time classes are associated with shorter sleep duration on weekdays and higher weekend-weekdays; girls sleep more during weekends.

Sleep is essential for children's learning, memory processes, school performance, and general well-being. The prevalence of sleepiness in children is approximately 4%. Reductions of sleep duration have daytime consequences, including sleepiness, behavior problems, cognitive deficits, poor school performance, inflammation, and metabolic dysfunction. Chronic pain, movement disorders, and sleep-disordered breathing also may lead to daytime somnolence, inattention, hyperactivity, oppositional behaviors, and mood dysregulation. Parent-report questionnaires are useful tools to assess subjective sleepiness in children. Sleepiness in children may be secondary to a sleep problem, such as narcolepsy, central hypersomnia, Kleine-Levin disease, or circadian rhythm disorder., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Objective: Intermittent hypoxia caused by obstructive sleep apnea syndrome (OSAS) may lead to damage in brain areas associated to auditory processing. The aim of this study was to compare children with OSAS or primary snoring (PS) to children without sleep-disordered breathing with regard to their performance on the Gaps-in-Noise (GIN) test and the Scale of Auditory Behaviors (SAB) questionnaire., Methods: Thirty-seven children (6-12 years old) were submitted to sleep anamnesis and in-lab night-long polysomnography. Three groups were organized according to clinical criteria: OSAS group (13 children), PS group (13 children), and control group (11 children). They were submitted to the GIN test and parents answered SAB questionnaire. The Kruskal-Wallis statistical test was used to compare the groups; p < 0.05 was considered statistically significant., Results: The OSAS group performed significantly worse than PS (p = 0.011) and Control (p = 0.029) groups on gap detection percentage, while PS and Control groups showed no significant differences. The three groups showed similar gap detection thresholds. Regarding SAB questionnaire, PS group had significantly worse scores when compared to Control (p = 0.011), but not to OSAS (p = 0.101) groups. No statistical difference between OSAS and Control groups were found., Conclusion: Children with OSAS showed worse performance on GIN test in comparison to children with PS and children without sleep-disordered breathing. PS negatively affected auditory behavior in children. These findings suggest that sleep-disordered breathing may lead to auditory behavior impairment., (Copyright © 2017 Elsevier B.V. All rights reserved.)

Objective: To describe the total sleep time, stages of sleep, and wakefulness of preterm newborns and correlate them to levels of sound pressure, light, temperature, relative air humidity, and handling inside incubators., Design: Observational, correlational study., Setting: A neonatal intermediate care unit., Participants: Twelve preterm newborns, who were 32.2 ± 4.2 weeks gestational age and weighed 1,606 ± 317 g., Methods: Sleep records were assessed by polysomnograph. Environmental variables were measured with a noise dosimeter, light meter, and thermohygrometer. To record time and frequency of handling, a video camera was used. All recordings were made for an uninterrupted 24-hour period., Results: Mean total sleep time in 24 hours was 899 ± 71.8 minutes (daytime = 446 ± 45.3 and nighttime = 448 ± 60.2). Mean wakefulness was 552 ± 94.0 minutes. The predominant stage was quiet sleep. A significant correlation was identified only between the levels of light and wakefulness (r = 0.65 and p = .041)., Conclusion: The environmental conditions and care provided to hospitalized preterm newborns did not influence sleep except for high light levels, which increased wakefulness. Nurses in clinical practice should implement strategies to promote and protect sleep by decreasing newborns' exposure to excessive light., (Copyright © 2017 AWHONN, the Association of Women’s Health, Obstetric and Neonatal Nurses. Published by Elsevier Inc. All rights reserved.)

Changes in sleep patterns negatively influence some emotional responses, but their effects on facial expressiveness identification are unclear. To investigate these effects, 21 young, healthy, male volunteers of intermediate chronotype evaluated emotional expressiveness of faces depicting 6 basic emotions in 5 emotional gradients every 4 h over 36 h of continuous wakefulness. To measure attention and mood we used the Psychomotor Vigilance test and the Positive and Negative Affect Schedule Expanded, respectively. We found effects of emotional gradient for all types of emotions (100% > 80% > 60% > 40% > 20%) during all tested periods, with no indications of circadian effects. The only emotional rating to be affected was disgust, which was progressively blunted throughout the experiment. This effect did not parallel homeostatic and circadian changes in mood, alertness or attention. We conclude that identifying disgust on facial photographs is particularly sensitive to lack of sleep irrespective of sleep-induced changes in mood and attention in males.

Seasonal affective disorder is defined as recurrent episodes of major depression, mania, or hypomania with seasonal onset and remission. In this class of mood disturbances, a unipolar major depressive disorder known as winter depression is common in populations living in northern latitudes far from the equator. Winter depression repeatedly occurs in the autumn or winter and remits in the spring or summer, and its etiopathogenesis is currently unknown. However, one can surmise that excessive melatonin production during the reduced duration of daily sunlight in the autumn and winter plays a role in its pathophysiology. Melatonin is synthesized from tryptophan within the pineal gland, which is located outside the blood-brain barrier, and overproduction of melatonin may lead to augmented consumption of tryptophan, from which serotonin is synthesized. As tryptophan is captured from the blood and excessively utilized by the pineal gland, tryptophan blood levels may decline; as such, it is more difficult for tryptophan to pass through the blood-brain barrier and reach the serotonergic neurons as the ratio of tryptophan to the other amino acids that compete for the same transporter to enter the brain is diminished. As such, less tryptophan is available for serotonin synthesis. Moreover, melatonin is known to modulate thyrotropin expression in the thyrotrophic cells of the pars tuberalis of the pituitary gland, and overproduction of melatonin in the autumn or winter months may cause excessive signaling in the pars tuberalis, diminishing its release of thyrotropin and resulting in central hypothyroidism. Both conditions reduced serotonin production and central hypothyroidism may cause depression. Furthermore, the excessive synthesis of melatonin during the autumn and winter may negatively affect the expression of neuromedin U in the pars tuberalis, causing an increased appetite, which is common in winter depression patients. The hypersomnia common in winter depressive patients can be ascribed to excessive circulating melatonin, a hormone that increases the propensity for sleep. Furthermore, central hypothyroidism may also increase sleepiness, as it is known that hypothyroid patients usually experience excessive somnolence. In this theoretical article, we also propose studies to evaluate winter depression patients with regard to the necessity, or not, of offering them an increased amount of tryptophan in their diets during the autumn and winter. We also suggest that the administration of triiodothyronine to winter depressive patients may mitigate their central hypothyroidism., (Copyright © 2016 Elsevier Ltd. All rights reserved.)

Purpose: To evaluate the frequency of preterm infant positions during sleep, and to investigate the association among positions, arousals, sleep patterns, and time of day., Design and Methods: This observational study was conducted in a neonatal unit with 10 preterm infants. Polysomnographic and video recordings during 24 hr identified sleep positions, arousals, sleep patterns, and time of day., Results: Preterm infants were placed most frequently in the supine position (58.4%), followed by right side (24.9%), left side (15.5%), and prone (1.2%). The longest amount of time spent sleeping, and the most frequent number of arousals, occurred in the supine position, followed by prone, left-side, and right-side positions. After controlling for length of time spent in each position, the number of arousals per hour was the greatest in the supine position (13.562 ± 0.732) and least in the prone position (11.56 ± 4.754; p < .001). There were no significant differences in arousals according to position and sleep pattern (indeterminate, quiet, active sleep) or position and time of day (morning, afternoon, twilight, night)., Practice Implications: Nurses should evaluate the frequency of preterm infant arousals in each position, and use more often those positions that lead to a lower frequency of arousals and better sleep quality., (© 2016, Wiley Periodicals, Inc.)

Background: Duchenne muscular dystrophy (DMD) causes serious health consequences that include impairment of the respiratory system and sleep. The aim of our study is to investigate the sleep architecture and respiratory profile during sleep of symptomatic patients with DMD without ventilatory support., Methods: We evaluated polysomnography (PSG) of boys with DMD (n = 44) and a control group (n = 79) with sleep complaints that was matched in age but without neuromuscular disease., Results: DMD patients presented sleep impairments when compared with the control group in terms of decreased sleep efficiency (72.4 ± 1.9 vs 80.3 ± 1.4 %, P = 0.002) and increased apnea-hypopnea index (AHI) during nonrapid eye movement (NREM) sleep (1.6 ± 0.3 vs 0.3 ± 0.2/h, P = 0.003). The main changes were observed during rapid eye movement (REM) sleep: an increase in REM sleep latency (202.2 ± 11.8 vs 152.3 ± 8.6 min, P < 0.001), a reduced percentage of REM sleep (13.1 ± 0.9 vs 17.9 ± 0.7 %, P = 0.001), and exacerbation of AHI (8.7 ± 1.5 vs 1.0 ± 1.1 events/h, P = 0.001). There was an increase in the total number of apneas, especially obstructive apneas (6.8 ± 1.9 vs 0.8 ± 1.3, P = 0.013)., Conclusions: The sleep and respiratory profile during sleep of patients with DMD are compromised. The results suggest that these changes reflect the muscle weakness inherent in DMD and are demonstrated mainly during REM sleep. Thus, the use of PSG is important to identify sleep-disordered breathing at an early stage, before deciding when to introduce noninvasive respiratory support for prevention of respiratory complications.

Obesity is considered a chronic subinflammatory disease and is a risk factor for many diseases such as sleep-disordered breathing (SDB). Although the interaction between obesity and sleep has been explored, not much is known about SDB in the adolescent population. Thus, the aims of this study were, first, to verify the effect of 1 year of interdisciplinary therapy on inflammatory markers in SDB and without SDB and, second, to investigate the influence of SDB on the result of the therapy by comparing these groups. A total of 36 obese adolescents were enrolled; however, only 24 completed the therapy (SDB group, n=12; non-SDB obese group, n=12). Sleep, anthropometric, metabolic, and inflammatory profiles were evaluated at baseline and after the treatment. In both groups, the therapy was able to improve all anthropometric variables. Metabolic parameters such as insulin, homeostasis model assessment (HOMA), and quantitative insulin sensitivity check index (QUICKI) were significantly improved only in non-SDB group. In both groups, the inflammatory state was significantly improved by the reduction in the leptin/adiponectin ratio. After the intervention, both groups no longer presented the hyperleptinemic state, favoring not only the inflammatory state, but also neuroendocrine regulation. Regarding the sleep parameter, the SDB group improved significantly in all respiratory events, and after therapy only four patients remained with SDB. Furthermore, there was an increase in sleep time. The lifestyle intervention was able to improve anthropometric, metabolic, and inflammatory parameters in both groups; however, the presence of SDB impaired better results. The data supported that the inclusion of SDB in the metabolic syndrome because of the link shown between them.

Streptococcal infections are suggested as a risk factor for narcolepsy. This hypothesis is supported by the presence of anti-streptolysin antibodies in 65% of patients with narcolepsy. These infections are associated with the activation of general immunity and concomitant increased permeability of blood-brain barrier after T cell activation during inflammation and fever. It has also been shown a significant association between birth order and narcolepsy in genetically susceptible patients, with positivity for HLA-DQB1⁎0602 allele. Watson and colleagues showed a significant association between birth order and narcolepsy in genetically susceptible patients, with positivity for HLA-DQB1⁎0602 allele. In that study, the disease was predominant in young children cases compared to controls. We report here the case of a child diagnosed with narcolepsy with cataplexy, positivity for the HLA-DQB1⁎0602 and previous history of streptococcal infection.

Introduction: the evolution of snoring and OSAS in children is not well established since few studies of patients without surgical treatment have been published., Objective: to evaluate the evolution of sleep disordered breathing in children who had not been submitted to upper airway surgery., Method: twenty-six children with snoring who had not undergone upper airway surgery were evaluated prospectively. Patients were evaluated by full physical examination and nocturnal polysomnography, after which they were divided into 2 groups: apnea (16 children) and snoring (10 children). After 6 months following the initial evaluation, patients were submitted to a new nocturnal polysomnography, and all data were compared to those of the first examination., Results: the groups did not show any differences regarding age, weight, height and airway physical examination. After 6 months of follow-up, the apnea index did not change, but the respiratory disturbance index increased in the snoring group and the number of hypopneas decreased in the group apnea., Conclusion: there was an increase in the percentage of N1 sleep stage and the respiratory disturbance index in the patients with primary snore. The AHI did not show significant alteration in both groups, but the number of hypopneas decreased in patients with SAOS., (Copyright © 2014 Associação Brasileira de Otorrinolaringologia e Cirurgia Cérvico-Facial. Published by Elsevier Editora Ltda. All rights reserved.)

The Kleine-Levin Syndrome is a differential diagnosis for patients with diurnal excessive sleepiness and a suspicion of narcolepsy. It is characterized by paroxysmal attacks of diurnal excessive sleepiness, associated with one or more symptoms of hyperphagia, hypersexuality, coprolalia and copropraxia. During crisis intervals, there are no symptoms. This pathology predominantly manifests itself in teenagers, being more frequent among males. The course of this disease is unpredictable, with variable duration and frequency. The most accepted physiopathology is that of a hypothalamic dysfunction, although and recently, there has appeared a hypothesis of a post-infectious autoimmune disorder. These patients show an elevated body mass index, which can predispose to association with comorbidities such as the sleep obstructive apnea syndrome. Treatment involves medications with different effects, but there is no specific and effective therapy. Our article shows a classic case of Kleine-Levin Syndrome associated with sleep obstructive apnea syndrome, a rare association in the literature.

We are reporting a case of a 29 year-old female with diagnosis of myotonic dystrophy type 1 (Steinert's disease) with excessive daytime sleepiness, muscle fatigue, snoring, frequent arousals, non-restorative sleep, and witnessed apneas. Pulmonary function tests revealed a mild decrease of forced vital capacity. Nocturnal polysomnography showed an increase of apnea/hypopnea index (85.9 events/h), mainly of central type (236), minimal oxygen saturation of 72%, and end-tidal carbon dioxide values that varied from 45 to 53 mmHg. Bi-level positive airway pressure titration was initiated at an inspiratory pressure (IPAP) of 8 and an expiratory pressure (EPAP) of 4 cm H2O. IPAP was then gradually increased to eliminate respiratory events and improve oxygen saturation. An IPAP of 12cm H20 and an EPAP of 4cm H2O eliminated all respiratory events, and the oxygen saturation remained above 90%. Bi-level positive airway pressure treatment at spontaneous/timed mode showed an improvement in snoring, apneas, and Epworth sleepiness scale decreased from 20 to 10. This case illustrates the beneficial effects of Bi-level positive airway pressure support in central sleep apnea syndrome of a patient with myotonic dystrophy type 1.

Background: Parents of children with migraine have described a higher prevalence of sleep bruxism and other sleep disturbances in their children. The objective of this study was to use polysomnography to investigate the prevalence of bruxism during sleep in children with episodic migraine relative to controls., Findings: Controls and patients were matched by sex, age, years of formal education, presence of snoring, arousals per hour, and respiratory events per hour.A total of 20 controls, between 6 and 12 years old, with no history of headache, recruited from public schools in Sao Paulo between 2009 and 2012, and 20 patients with episodic migraine recruited from the Headache Clinic at the Federal University of Sao Paulo between 2009 and 2012 underwent polysomnography.No intervention was performed before sleep studies.Among migraine patients, 27.5% experienced aura prior to migraine onset. The sleep efficiency, sleep latency, REM sleep latency, arousals per hour, percentage of sleep stages, and breathing events per hour were similar between groups. Five children (25%) with episodic migraine exhibited bruxism during the sleep study while this finding was not observed in any control (p = 0.045)., Conclusions: Our data demonstrate that bruxism during sleep is more prevalent in children with episodic migraine. Further prospective studies will help elucidate the underlying shared pathogenesis between bruxism and episodic migraine in children.

This is a series of cases describing the use of citalopram for the treatment of cataplexy in patients with narcolepsy. Cataplexy is the most specific symptom of narcolepsy, being characterized by a sudden and temporary loss of muscle tonus, triggered by episodes of emotion during vigil. Some antidepressants, besides gamma-hydroxybutyrate, are used for the control of cataplexy. As gamma-hydroxybutyrate is not available in Brazil, local treatment is usually done by the use of antidepressants. Citalopram is a selective inhibitor of serotonin reuptake, with reasonable price and with fewer side effects when compared with other drugs of the same type. In this study, we report a series of cases with patients with narcolepsy and cataplexy, treated with citalopram for the control of cataplexy.

Studies have pointed out that approximately 50-60% of narcolepsy patients may demonstrate higher prevalence of periodical leg movements. However, we highlight that the prevalence studies and the effects of periodical leg movements in patients with narcolepsy are limited and with conflicting results. The objective of this study was that of describing and discussing the prevalence of periodical leg movements in patients with narcolepsy in the outpatient facility of diurnal excessive sleepiness of the Federal University of São Paulo, Brazil. We revised 59 files of patients with the clinical and electrophysiological diagnosis of narcolepsy according to the American Academy of Sleep Medicine. Of these 59 cases of patients with narcolepsy, 12 (20.3%) demonstrated periodical leg movements. Thirty five patients (59.3%) had history of cataplexy and 38 patients (64.4%) had the presence of the allele HLA-DQB1⁎0602. There was a higher prevalence of periodical leg movements in patients with cataplexy (p<0.0001) and in patients with the presence of the allele HLA-DQB1⁎0602 (p<0.0001). Our study characterized the higher prevalence of periodical leg movement in patients with narcolepsy, mainly in patients with cataplexy and with the presence of the allele HLA-DQB1⁎0602.

Narcolepsy is a chronic neurological disease characterized by diurnal excessive sleepiness and catapleaxy. It affects 1 in every 2000 to 4000 individuals with personal, social and familiar significant repercussions. The treatment of narcolepsy is mainly based on the use of stimulants for the control of the diurnal excessive sleepiness, in conjunction with behavioral measures and sleep hygiene. Among the stimulants, modafinil has presently been the drug of choice for the treatment of the diurnal excessive sleepiness in patients with narcolepsy. In the worldwide experience, its use is better tolerated and the majority of its side effects is considered light or moderate. However, the clinical use in Brazil was initiated at the end of 2008, with little experience on the narcolepsy population of this country. In this context, the objective of this study was the evaluation of the use of modafinil, verifying the indication of use, causes for discontinuation, daily dosage, efficiency of the treatment in a patient sample of narcoleptics consulted in a specialized center in Brazil. In this study, modafinil was effective for the control of the symptoms related do narcolepsy in 66% of the studied patients. The side effects such as headache, parestesias and diarrhea were the main reasons for the discontinuation of treatment with modafinil. It is important to clinically follow up the patients for a long period to evaluate symptomatology, control of use, tolerability and re-evaluation of the more effective therapeutic dosage able to control narcolepsy. Due to its high cost and clinical benefits, this drug should be on the government׳s list of free drugs for the treatment of these patients.

Narcolepsy is a disease in which there is diurnal excessive sleepiness with sleep attacks and a prevalence in the general population of 1/4000 individuals. Classically, it is characterized by cataplexy, sleep paralysis, hypnagogic hallucinations and fragmented sleep. The use of modafinil in the treatment of narcolepsy is the first option of treatment for diurnal excessive sleepiness. Although considered a safe drug for use in patients with narcolepsy, being utilized for more than 20 years, modafinil possesses a series of side effects, some of them still not fully researched or described. Side effects such as headache, nausea, anxiety, insomnia, lumbago, diarrhea, dyspepsia, rhinitis and vertigo are the most frequent. However, the clinical follow-up of patients under treatment with modafinil must be intensive and the side effects ought to be noted and evaluated. The under-response to treatment or the unexpected side effects must always be directed to differential diagnostics. The objective of this article is to describe an unexpected side effect of the use of modafinil in a patient with incorrect diagnosis of narcolepsy.

Objective: It was to evaluate the degree of fatigue in patients with paralytic poliomyelitis (PP) and with post-polio syndrome (PPS), and correlate it with parameters of sleep and the circadian cycle., Methods: Thirty patients, 17 female (56.7%), participated in the study: they answered the Revised Piper Fatigue Scale and performed a nocturnal polysomnographic study. Eleven had PP (mean age±standard deviation of 47.9±6.4 years), and 19 had PPS (mean age±standard deviation of 46.4±5.6 years)., Results: Our study showed that fatigue was worse in the afternoon in the PP Group and had a progressive increase throughout the day in the PPS Group. We also observed compromised quality of sleep in both groups, but no statically significant difference was found in the sleep parameters measured by polysomnography., Conclusion: Fatigue has a well-defined circadian variation, especially in PPS Group. Poor sleep quality is associated with fatigue and, therefore, sleep disturbances should be evaluated and treated in this group of PPS.

Objective: Certain drug classes alleviate the symptoms of Willis-Ekbom's disease, whereas others aggravate them. The pharmacological profiles of these drugs suggest that drugs that alleviate Willis-Ekbom's disease inhibit thyroid hormone activity, whereas drugs that aggravate Willis-Ekbom's disease increase thyroid hormone activity. These different effects may be secondary to the opposing actions that drugs have on the CYP4503A4 enzyme isoform. Drugs that worsen the symptoms of the Willis-Ekbom's disease inhibit the CYP4503A4 isoform, and drugs that ameliorate the symptoms induce CYP4503A4. The aim of this study is to determine whether Saint John's wort, as an inducer of the CYP4503A4 isoform, diminishes the severity of Willis-Ekbom's disease symptoms by increasing the metabolism of thyroid hormone in treated patients., Methods: In an open-label pilot trial, we treated 21 Willis-Ekbom's disease patients with a concentrated extract of Saint John's wort at a daily dose of 300 mg over the course of three months., Results: Saint John's wort reduced the severity of Willis-Ekbom's disease symptoms in 17 of the 21 patients., Conclusion: Results of this trial suggest that Saint John's wort may benefit some Willis-Ekbom's disease patients. However, as this trial was not placebo-controlled, the extent to which Saint John's wort is effective as a Willis-Ekbom's disease treatment will depend on future, blinded placebo-controlled studies.

Willis-Ekbom's disease (WED), formerly called restless legs syndrome, is more common in pregnant than in non-pregnant women, implying that the physiological and biochemical changes during pregnancy influence its development. During pregnancy, many hormone levels undergo significant changes, and some hormones significantly increase in activity and can interfere with other hormones. For example, the steroid hormone estradiol interferes with the neuroendocrine hormone dopamine. During pregnancy, the activity of the thyroid axis is enhanced to meet the increased demand for thyroid hormones during this state. Dopamine is a neuroendocrine hormone that diminishes the levels of thyrotropin and consequently of thyroxine, and one of the roles of the dopaminergic system is to counteract the activity of thyroid hormones. When the activity of dopamine is not sufficient to modulate thyroid hormones, WED may occur. Robust evidence in the medical literature suggests that an imbalance between thyroid hormones and the dopaminergic system underpins WED pathophysiology. In this article, we present evidence that this imbalance may also mediate transient WED during pregnancy. It is possible that the main hormonal alteration responsible for transient WED of pregnancy is the excessive modulation of dopamine release in the pituitary stalk by estradiol. The reduced quantities of dopamine then cause decreased modulation of thyrotropin, leading to enhanced thyroid axis activity and subsequent WED symptoms. Iron deficiency may also be a predisposing factor for WED during pregnancy, as it can both diminish dopamine and increase thyroid hormone., (Copyright © 2012 Elsevier Ltd. All rights reserved.)

Unlabelled: Children may present sleep-disordered breathing (SDB) and suffer with adverse effects upon their quality of life., Objective: This study assessed the quality of life of children with SDB, compared subjects with obstructive sleep apnea syndrome (OSAS) and primary snoring (PS), and identified which areas in the OSA-18 questionnaire are more affected., Methods: This is a historical cohort cross-sectional study carried out on a consecutive sample of children with history of snoring and adenotonsillar hyperplasia. The subject's quality of life was assessed based on the answers their caregivers gave in the OSA-18 questionnaire and on diagnostic polysomnography tests., Results: A number of 59 children participated in this study with mean age of 6.7 ± 2.26 years. The mean score of the OSA-18 was 77.9 ± 13.22 and the area most affected were "caregiver concerns" (21.8 ± 4.25), "sleep disturbance" (18.8 ± 5.19), "physical suffering" (17.3 ± 5.0). The impact was low in 6 children (10.2%), moderate in 33 (55.9%) and high in 20 (33.9%). PS was found in 44 children (74.6%), OSAS in 15 (25.6%). OSAS had higher score on "physical suffering" area than PS (p = 0.04). The AI (r = 0.22; p = 0.08) and AHI (r = 0.14; p = 0.26) were not correlated with OSA-18., Conclusion: Sleep disordered breathing in childhood cause impairment in quality of life and areas most affected the OSA-18 were: "caregiver concerns", "sleep disturbance" and "physical suffering". OSAS has the domain "physical suffering" more affected than primary snorers.

Unlabelled: Postpolio syndrome (PPS) is characterized by progressive muscle weakness due to former infection with poliomyelitis and can be associated with other symptoms such as cold intolerance (CI). Dăoyĭn Qìgōng (DQ) is a technique in Traditional Chinese Medicine that impacts the circulation of energy and blood., Objective: It was to verify the effects of DQ in PPS patients complaining of cold intolerance., Methods: Ten PPS patients were assessed using the visual analogue scale (VAS) adapted for CI before and after intervention with DQ; patients practiced it in a sitting position for 40 minutes, 3 times per week over 3 consecutive months. Patients were reassessed three months after ceasing DQ., Results: There was a statistically significant difference in local and systemic VAS-Cold both at the end of DQ training and three months past the end of this., Conclusion: The DQ technique ameliorated CI complaints in patients with PPS.

Purpose: The study aims to assess the risk factors for the presence and severity of obstructive sleep apnea (OSA) among severely obese patients evaluated for bariatric surgery., Patients and Methods: Polysomnography recordings were performed in consecutive patients undergoing Roux-en-Y gastric bypass from January 2004 to January 2007. Sleep apnea was noted as present or absent and graded from mild to severe according to the apnea/hypopnea index. Patient gender, age, weight, height, body mass index, neck circumference, and waist circumference were recorded., Results: A total of 132 patients were included in the study group, and 85 patients had a confirmed diagnosis of OSA (64.4%). The prevalence of OSA was 55.7% in female and 77.4% in male. The prevalence of moderate or severe sleep apnea was higher in males (71.6%) than in females (31.6%). In OSA patients, body mass index (p = 0.020), neck circumference (p < 0.001), and age (p = 0.003) were higher as compared with obese patients without OSA, whereas no differences were found in waist circumference between groups. After multiple regression analysis, body mass index, age, and male gender were independent predictors of sleep apnea. In the female group, age greater than 49 years was the only significant predictor of moderate or severe OSA (odds ratio 5.42 (95% confidence interval 1.61-18.1); p = 0.006)., Conclusion: Males and females with age greater than 49 years are at greatest risk for OSA. Preoperative sleep studies should be mandatory in this group of severely obese patients.

Objective: In comparison with basal physiological levels, acute, high levels of cortisol affect learning and memory. Despite reports of cortisol-induced episodic memory effects, no study has used a comprehensive battery of tests to evaluate glucocorticoid effects on the multicomponent model of working memory. Here, we report the results of a double-blind, placebo-controlled, between-subjects study., Methods: Twenty healthy young men were randomly assigned to either acute cortisol (30 mg hydrocortisone) or placebo administration. Participants were subjected to an extensive cognitive test battery that evaluated all systems of the multicomponent model of working memory, including various executive domains (shifting, updating, inhibition, planning and access to long-term memory)., Results: Compared with placebo, hydrocortisone administration increased cortisol blood levels and impaired working memory in storage of multimodal information in the episodic buffer and maintenance/reverberation of information in the phonological loop. Hydrocortisone also decreased performance in planning and inhibition tasks, the latter having been explained by changes in storage of information in working memory., Conclusions: Thus, hydrocortisone acutely impairs various components of working memory, including executive functioning. This effect must be considered when administering similar drugs, which are widely used for the treatment of many clinical disorders., (Copyright © 2011 John Wiley & Sons, Ltd.)

Narcolepsy has been studied as a possible autoimmune disease for many years, and recent findings lend more credence to this belief. Although recent and important advances have been done, no study has analyzed the role of the CD40L in patients with narcolepsy. The purpose of this study was to assess CD40L levels, CD3, TCD4, TCD8, CD19, and CD56 lymphocytes, as well as levels of tumor necrosis factor-α and interleukin-6 in narcoleptic patients. We quantified the levels of CD40L, different types of lymphocytes, and levels of tumor necrosis factor-α and interleukin-6 in narcoleptic patients and control subjects. Narcoleptic patients had lower levels of CD40L. Total lymphocytes; CD3, and TCD4 were lower than in the control group. Our findings highlight the important role of CD40L in narcolepsy., (Copyright © 2011 American Society for Histocompatibility and Immunogenetics. Published by Elsevier Inc. All rights reserved.)

Objective: There are no data adressing the prevalence of restless legs syndrome in subjects who have knee prosthesis. Therefore, we conducted a cross-sectional survey of subjects who underwent knee prosthesis surgery., Method: A total of 107 subjects (30 male, 77 female) were interviewed over the telephone regarding restless legs syndrome symptoms. If the patients exhibited symptoms of the syndrome, we conducted face-to-face interviews. Lastly, a therapeutic test with pramipexole was proposed for each subject., Results: In our cohort, 7 males (23%) and 30 females (39%) had restless legs syndrome. Of these, 6 males and 23 females were submitted to face-to-face-interview. Of the males, 5 (83%) had restless legs after the knee surgery-exclusively in the operated leg- and reported no family restless legs history. One man had a prior case of bilateral restless legs syndrome, a positive family history and claimed exacerbation of symptoms in the operated leg. Among the females, 16 (69%) had restless legs prior to surgery. A total of 10 female patients reported bilateral symptoms, with fewer symptoms in the operated leg, while 6 displayed a worse outcome in the operated leg. The 7 females (31%) without restless legs prior to surgery and without a family history experienced symptoms only in the operated leg. All subjects responded favorably to the pramipexole therapeutic test., Conclusion: Our results suggest that secondary unilateral restless legs syndrome may ensue from knee prosthesis surgery and that the symptoms are generated in the peripheral nervous system.

Unlabelled: This study was thought to characterized clinical and laboratory findings of a narcoleptic patients in an out patients unit at São Paulo, Brazil., Method: 28 patients underwent polysomnographic recordings (PSG) and Multiple Sleep Latency Test (MSLT) were analyzed according to standard criteria. The analysis of HLADQB1*0602 allele was performed by PCR. The Hypocretin-1 in cerebral spinal fluid (CSF) was measured using radioimmunoassay. Patients were divided in two groups according Hypocretin-1 level: Normal (N) - Hypocretin-1 higher than 110 pg/ml and Lower (L) Hypocretin-1 lower than 110 pg/ml., Results: Only 4 patients of the N group had cataplexy when compared with 14 members of the L group (p = 0.0002)., Discussion: This results were comparable with other authors, confirming the utility of using specific biomarkers (HLA-DQB1*0602 allele and Hypocretin-1 CSF level) in narcolepsy with cataplexy. However, the HLADQB1*0602 allele and Hypocretin-1 level are insufficient to diagnose of narcolepsy without cataplexy.

Objective: The aim of this study is to complement existing data on the expression and characteristics of cyclic alternating pattern (CAP) in children, specifically in the 12 to 24 month age bracket., Method: Descriptive study., Settings: a university pediatric sleep laboratory., Participants: Twelve normal and healthy subjects (6 girls and 6 boys, mean age 18.9 ± 4.72 months; range 12-24 months underwent a standard polysomnography night recording in our pediatric sleep laboratory. Sleep stages and CAP were analyzed according to standard international rules., Results: CAP rate found in children of 12 to 24 months of age was (25.78 ± 10.18%) and it is characterized by a linear increase of 2% per month, from 12% at 12 months reaching 35% at 24 months. With coefficient of determination R² of 0.91. The duration of A phases was 6.93 ± 1.06 seconds, and B phases was found to last 21.44 ± 2.31 seconds. The number of CAP cycles was 173.25 ± 7 3.85 with an average index per hour of 33.55 ± .61. The number of CAP sequences reached 25.25 ± 9.55 per recording. CAP rate for the different type of A phases are (21.83 ± 9.68%), for A1, (2.43 ± 2.30%) for A3 and (1.67 ± 1.11%) for A2., Conclusion: Our study provides normative data on CAP in a group of young children (12 to 24 months). The most salient result of this study is the strong correlation of CAP rate of 2% per month in this age group.

This manuscript contains the conclusion of the consensus meeting of the Brazilian Sleep Association with Brazilian sleep specialists on the treatment of narcolepsy based on the review of medical literature from 1980 to 2010. The manuscript objectives were to reinforce the use of agents evaluated in randomized placebo-controlled trials and to issue consensus opinions on the use of other available medications as well as to inform about safety and adverse effects of these medications. Management of narcolepsy relies on several classes of drugs, namely, stimulants for excessive sleepiness, antidepressants for cataplexy and hypnotics for disturbed nocturnal sleep. Behavioral measures are likewise valuable and universally recommended. All therapeutic trials were analyzed according to their class of evidence. Recommendations concerning the treatment of each single symptom of narcolepsy as well as general recommendations were made. Modafinil is the first-line pharmacological treatment of excessive sleepiness. Second-line choices for the treatment of excessive sleepiness are slow-release metylphenidate followed by mazindol. The first-line treatments of cataplexy are the antidepressants, reboxetine, clomipramine, venlafaxine, desvenlafaxine or high doses of selective serotonin reuptake inibitors antidepressants. As for disturbed nocturnal sleep the best option is still hypnotics. Antidepressants and hypnotics are used to treat hypnagogic hallucinations and sleep paralysis.