Cardiac autonomic control during non-REM and REM sleep stages in paediatric patients with Prader-Willi syndrome.

Cardiac death is the second most prevalent cause in Prader-Willi syndrome (PWS). Paediatric patients with PWS often present cardiac autonomic dysfunction during wakefulness, obesity and sleep-disordered breathing. However, the extent of cardiac autonomic modulation during sleep in PWS has not been documented. The objective of this study was to assess alterations in cardiac autonomic modulation of paediatric patients with PWS during different sleep stages. Thirty-nine participants in three groups: 14 PWS, 13 sex and age-matched lean controls (LG) and 12 obese-matched controls (OB). All participants underwent overnight polysomnography, including continuous electrocardiogram recordings. Heart rate variability (HRV) was analysed during representative periods of each sleep stage through time and frequency domains calculated across 5-min periods. Between-within ANOVAs were employed (p < .05). The results show that total HRV was lower in PWS than OB and LG during slow-wave sleep (SWS) (standard deviation of all NN intervals [SDNN] ms, p = .006). Parasympathetic modulation assessed by time-domain analysis was lower during SWS in PWS compared to both OB and LG (square root of the mean of the sum of the squares of differences between adjacent NN intervals [RMSSD] ms, p = .004; SDSD, standard deviation of differences between adjacent NN intervals [SDSD] ms, p = .02; number of adjacent NN intervals differing by >50 ms [NN50] ms, p = .03; proportion of adjacent NN intervals differing by >50 ms [pNN50] ms, p = .01). Sympathovagal balance assessed by frequency-domain analysis was lower during both N2 and SWS than during the rapid eye movement (REM) sleep stage, but not different among groups. In conclusion, this group of paediatric patients with PWS had impaired cardiac autonomic balance due to reduced parasympathetic modulation during SWS. This result could imply an underlying increased cardiovascular risk in PWS even during early age and independent of obesity.

Personality Characteristics of Children and Adolescents with Anxiety Disorder from a Maternal Perspective: A Brief Report.

The present study assessed the personality characteristics of children and adolescents with anxiety disorder from a maternal perspective. A total of 48 children and adolescents aged between 8 and 17 years participated in this study, which was organized as follows: a clinical group (24 children and adolescents with anxiety disorders and their respective mothers) and a control group (24 children and adolescents without psychiatric diagnosis and their mothers). The participants were submitted to the WASI, CBCL, MASC-2, and EPQ-J tests and their mothers to the SRQ-20 and PIC-2 tests. The results showed higher rates of internalizing symptoms in the clinical group. In addition, patients showed less interest in hobbies, less adherence to social organizations, impairment in social activities, and commitment to school performance compared to the control group. There was a positive correlation between the mothers' symptoms and each of the following PIC-2 domains: somatic concern (p < 0.01) and psychological discomfort (p < 0.01). In conclusion, youths with AD showed a withdrawn and reserved personality profile, involving distrust of impulses and avoidance of interactions with peers. Furthermore, psychoemotional problems of mothers adversely influenced the perception followed by anxiety and adjustment characteristics. More studies are needed to assess the maternal personality in youths with anxiety.

[Brazilian guidelines for the diagnosis of narcolepsy]. / Diretrizes brasileiras para o diagnóstico da narcolepsia.

This manuscript contains the conclusion of the consensus meeting on the diagnosis of narcolepsy based on the review of Medline publications between 1980-2010. Narcolepsy is a chronic disorder with age at onset between the first and second decade of life. Essential narcolepsy symptoms are cataplexy and excessive sleepiness. Cataplexy is defined as sudden, recurrent and reversible attacks of muscle weakness triggered by emotions. Accessory narcolepsy symptoms are hypnagogic hallucinations, sleep paralysis and nocturnal fragmented sleep. The clinical diagnosis according to the International Classification of Sleep Disorders is the presence of excessive sleepiness and cataplexy. A full in-lab polysomnography followed by a multiple sleep latency test is recommended for the confirmation of the diagnosis and co-morbidities. The presence of two sleep-onset REM period naps in the multiple sleep latency test is diagnostic for cataplexy-free narcolepsy. A positive HLA-DQB1*0602 with lower than 110pg/mL level of hypocretin-1 in the cerebrospinal fluid is required for the final diagnosis of cataplexy- and sleep-onset REM period -free narcolepsy.

A questionnaire study on sleep disturbances associated with Prader-Willi syndrome.

Background This study aimed to investigate the presence of sleep disturbances in children with Prader-Willi syndrome (PWS) using the Sleep Disturbance Scale for Children (SDSC). Methods The SDSC, which was designed to identify the presence and severity of different sleep disorders, was applied to 50 patients with PWS and 112 controls. Results Patients with PWS achieved worse scores in the sleep-disordered breathing and disorders in initiating and maintaining sleep in the SDSC questionnaire as compared with controls. We also observed that patients with PWS were more prone to having hyperhidrosis. We did not observe significant differences in the presence of other types of sleep disorders (such as hypersomnolence) between the PWS and control groups. Conclusions The results obtained with the SDSC questionnaire showed that children with PWS have more sleep breathing disorders and disorders in initiating and maintaining sleep as compared to controls. Additionally, we demonstrated that patients with PWS associates significantly with the presence of hyperhidrosis during sleep. However, SDSC was not reliable to identify the excessive daytime somnolence in patients with PWS, as previously reported in the literature.

Is there a connection between long airplane flight, venous thromboembolism, and sleep-disordered breathing?

Commercial passenger flights have been increasing around the world. The effect of these flights on health is unclear. Venous thromboembolism has been noted after recent long-distance airplane flight, even in the absence of other risk factors. Hypoxia caused by the low ambient pressure during flights could contribute, and individuals with obstructive sleep apnea may be particularly vulnerable. The association between the effects of long airplane travel and sleep-disordered breathing deserves further study.

Sleep and Daytime Complaints During Manic and Depressive Episodes in Children and Adolescents With Bipolar Disorder.

INTRODUCTION: Depressive and manic episodes of bipolar disorder can interact with sleep complaints, followed by a worsened psychiatric condition. The aim of this study was to examine the interaction of sleep disorders with bipolar disorder in youths during depressive and manic episodes. METHODS: The target population was children and adolescents drawn from the Children and Adolescents Affective Disorder Program. Clinical assessment for current psychiatric diagnosis was done by direct clinical interview, Diagnostic Interview for Children and Adolescents (DSM-IV), and best-estimated clinical consensus. We applied sleep questionnaires from which we obtained sleep and daytime complaints during manic and depressive episodes. All statistical tests of significance were done using 2-tailed tests with α = 0.05. RESULTS: Participants in this study comprised 29 children (age = 10 ± 3 years, boys = 23) and 43 adolescents (age = 15 ± 2.4 years, boys = 30). Sleep complaints were observed in 66.4% of participants during manic episodes and 52.3% during depressive episodes. 37.9% of patients had sleep complaints in both episodes. Time in bed was longer during depressive episodes than manic episodes (p = 0.01). We found a high prevalence of nocturnal enuresis in depressive episodes in children and adolescents, which was statistically significant compared with manic episodes (p < 0.05). Unrested sleep was higher in adolescents in both episodes, and it was statistically significant during manic episodes (p < 0.05). CONCLUSION: According to our analyses, the minority of patients had sleep complaints in both episodes. Our data showed that nocturnal enuresis occurred more frequently during depressive than manic episodes. Further research is necessary to understand the implications of these data.

Reduction in Parasympathetic Tone During Sleep in Children With Habitual Snoring.

Introduction: Changes in the autonomic nervous system due to Obstructive Sleep Apnea (OSA) during the life span have been described. Some pediatric studies have shown cardiovascular effects in children who do not fit the criteria for OSA; namely children with mild sleep disordered breathing. Objective: We investigated heart rate variability (HRV) during sleep in children with chronic snoring and flow limitation events during sleep. Methods: Ten children and adolescents with chronic snoring and an apnea hypopnea index < 1, associated to high Respiratory Index, and 10 controls matched for age, gender, and Tanner stage were monitored following one night of habituation in the sleep laboratory. HRV was studied at each sleep stage. The time and frequency domains were calculated for each 5-min period. Results: All patients were chronic heavy snorers. They presented an apnea hypopnea index = 0.8, respiratory disturbance index = 10.2/h with lowest O2 saturation 96.1 ± 2.4%. The total power of HRV was decreased in all stages (p < 0.05). There was also a decrease in NN50 and pNN50 during all sleep stages compared to healthy controls (p = 0.0003 and p = 0.03, respectively). Conclusion: A reduction in parasympathetic tone was found in the patient group. This may represent an autonomic impairment during sleep in children with mild SDB. A reduction in HRV in children with habitual snoring could be associated with possible increases in cardiovascular risk in adulthood. Significance: The study indicates that children with habitual snoring have important parasympathetic tone changes during sleep.

The significance of ASDA arousals in children.

Sleep disorders are common in children. The sleep disturbances associated with these disease processes may impact neurodevelopment and result in daytime behavioral and cognitive changes. Currently, there are no precise methods to accurately assess sleep disruption in the pediatric age group. There is evidence that American Sleep Disorders Association (ASDA) arousals are insufficient markers of sleep disruption in children. Other techniques that have been used to assess sleep disruption include unconventional means of evaluating the electroencephalogram (EEG) during sleep and evaluating subcortical or autonomic activation. The aim of this review is to discuss the application of conventional and unconventional markers of sleep disruption in children.

Chronic fatigue, unrefreshing sleep and nocturnal polysomnography.

BACKGROUND AND PURPOSE: To investigate the complaint of unrefreshing sleep with study of sleep electroencephalogram (EEG) in patients with chronic fatigue. PATIENTS AND METHODS: Fourteen successively seen patients (mean age: 41.1 9.8) who complained of chronic fatigue but denied sleepiness and agreed to participate were compared to 14 controls (33.6+/-10.2 years) who were monitored during sleep recorded in parallel. After performing conventional sleep scoring we applied Fast Fourier Transformation (FFT) for the delta 1, delta 2, theta, alpha, sigma 1, sigma 2, beta EEG frequency bands. The presence of non-rapid eye movement (NREM) sleep instability was studied with calculation of cyclic alternating pattern (CAP) rate. Two-way analysis of variance (ANOVA) was performed to analyze FFT results and Mann-Whitney U-test to compare CAP rate in both groups of subjects. RESULTS: Slow wave sleep (SWS) percentage and sleep efficiency were lower, but there was a significant increase in delta 1 (slow delta) relative power in the chronic fatigue group when compared to normals (P<0.01). All the other frequency bands were proportionally and significantly decreased compared to controls. CAP rate was also significantly greater in subjects with chronic fatigue than in normals (P=0.04). An increase in respiratory effort and nasal flow limitation were noted with chronic fatigue. CONCLUSIONS: The complaints of chronic fatigue and unrefreshing sleep were associated with an abnormal CAP rate, with increase in slow delta power spectrum, affirming the presence of an abnormal sleep progression and NREM sleep instability. These specific patterns were related to subtle, undiagnosed sleep-disordered breathing.

Visual and automatic cyclic alternating pattern (CAP) scoring: inter-rater reliability study.

The classification of short duration events in the EEG during sleep, as the A stage of the cyclic alternating pattern (CAP) is a tedious and error prone task. The number of events under normal conditions is large (several hundreds), and it is necessary to mark the limits of the events with precision, otherwise the time sensitive classification of the CAP phases (A and B) and specially the scoring of different types of A phases will be compromised. The objective of this study is to verify the feasibility of visual CAP scoring with only one channel of EEG, the evaluation of the inter-scorer agreement in a variety of recordings, and the comparison of the visual scorings with a known automatic scoring system. Sixteen hours of one channel (C4-A1 or C3-A2) of NREM sleep were extracted from eight whole night recordings in European Data Format and presented to the different scorers. The average inter-scorer agreement for all scorers is above 70%, the pair wise inter-scorer agreement found was between 69% up to 77.5%. These values are similar to what has been reported in different type studies. The automatic scoring system has similar performance of the visual scorings. The study also has shown that it is possible to classify the CAP using only one channel of EEG.

Sleep Complaints as Risk Factor for Suicidal Behavior in Severely Depressed Children and Adolescents.

OBJECTIVES: To investigate the association between sleep complaints and suicidal behaviors among severely depressed children and adolescents. METHODS: The sample was 214 youths (56.1% males, mean age 12.5 years) with diagnosis of DSM-IV major depressive disorder consecutively recruited from a university-based outpatient clinic specialized in mood disorders. The structured interview for children and adolescents was applied to participants. The Children's Depression Rating Scale-revised version-scored the severity of depression, and the Children's Global Assessment Scale assessed the global functioning. Subgroups of patients were compared for psychopathological association by means of logistic regression, in accordance with presence and absence of sleep complaints and suicidality. RESULTS: The frequency of sleep complaints and suicidal behaviors was, respectively, 66.4% and 52.3%, and both symptoms were observed in 37.9% of patients. Initial insomnia was the most frequent manifestation (58%), followed by night awakening (36%), daytime sleepiness (31%), and early awakening (29.9%). Significant association between sleep disturbance and suicidal behavior was found (odds ratio range of 2.3-10.8). CONCLUSION: Sleep disturbances are potential warning manifestations of suicidal behaviors in depressed youth. Possibly, the severity of the active affective episode likely underlies in both sleep complaints and suicidal behaviors among depressed underage patients.

Cyclic alternating pattern in peripubertal children.

STUDY OBJECTIVES: The aim of this study is to complement the data on the expression and characteristics of cyclic alternating pattern (CAP) events in children, specifically in the peripubertal age group of 8 to 12 years and to analyze the association of CAP events with arousals. The study of CAP and arousal is a useful tool for assessing sleep instability and fragmentation in children. DESIGN: Descriptive study. PARTICIPANTS: Ten sex-matched healthy children, aged 8 to 12 years, underwent standard polysomnography after 1 adaptation night in the sleep laboratory. Sleep stages, CAP, and arousals were analyzed according to standard international rules. RESULTS: The mean CAP rate was 62.1% +/- 10.8% and the mean CAP cycle duration, 24.6 +/- 2.1 minutes. CAP A1 phase was the most numerous (85.5% +/- 3.9%), whereas the A2 phase was 9.1% +/- 4.7%, and the A3 phase as 5% +/- 2.3%, (P < .01). Differences between boys and girls were detected by analysis of variance, namely increases of phase A2 and A3 subtypes in girls (P < .001). Stronger phase A1 subtype expression in slow-wave sleep was verified in both sexes. Positive correlation between electroencephalogram arousals and the sum of phase A2 and A3 subtypes was also present. The overall CAP rate is higher in this age group than the rate previously reported in children aged 6 to 10 years (62.1% +/- 10.8% vs 33.4% +/- 5.3%). CONCLUSIONS: Our study provides normative data on CAP in children aged 8 to 12 years and indicates that age and Tanner stages must both be considered when investigating peripubertal children.

Non-REM-sleep instability in recurrent sleepwalking in pre-pubertal children.

BACKGROUND AND PURPOSE: We questioned whether or not the sleep of pre-pubertal children with recurrent sleepwalking was different from that recorded in normal children. PATIENTS AND METHODS: Twelve pre-pubertal chronic sleepwalkers were compared to age- and gender-matched normal children. All children had a clinical evaluation covering pediatric, sleep, neuropsychiatric and otolaryngological fields. Two standardized sleep questionnaires were administered, and a minimum of two successive polysomnograms were performed with monitoring of sleep electroencephalographic (EEG) and cardiorespiratory variables. The research investigations were performed on nights without sleepwalking to search for the presence of other sleep disorders, including upper airway resistance syndrome (UARS). Sleep was scored using standard atlases, but it was also evaluated for the cyclic alternating pattern (CAP) rate. RESULTS: All sleepwalkers presented with either obstructive sleep apnea (n=2) or UARS (n=10). Compared to normal children, sleepwalkers had shorter total sleep time but no significant change in wake after sleep onset when considering all arousals > 3 s. CAP analysis showed a significantly higher CAP rate than in controls. CONCLUSIONS: Chronic sleepwalkers have instability of non-rapid eye movement (NREM) sleep detectable only by the calculation of CAP rate. Instability of NREM sleep was seen even on nights without sleepwalking and is probably related to the presence of the associated sleep disorders. We hypothesize that chronic NREM-sleep instability is a risk factor for occurrence of sleepwalking when further sleep disruption is triggered by external events.

Brazilian consensus on guidelines for diagnosis and treatment for restless legs syndrome.

The Consensus on restless legs syndrome is an effort of neurologists from several Brazilian states, which tirelessly reviewed the literature of recent years in search of evidence, both in regard to diagnosis and treatment, according to the Oxford Centre for Evidence-based Medicine.

Brazilian consensus on guidelines for diagnosis and treatment for restless legs syndrome / Consenso Brasileiro para as diretrizes de diagnóstico e tratamento da síndrome das pernas inquietas

The Consensus on restless legs syndrome is an effort of neurologists from several Brazilian states, which tirelessly reviewed the literature of recent years in search of evidence, both in regard to diagnosis and treatment, according to the Oxford Centre for Evidence-based Medicine.

O Consenso em síndrome das pernas inquietas contou com a participação de neurologistas de vários estados brasileiros, os quais incansavelmente revisaram a literatura dos últimos anos em busca de evidências, tanto no que se refere ao diagnóstico como ao tratamento, de acordo com a Classificação do Centro de Oxford para Medicina Baseada em Evidências.

A case-control sleep study in children with polyarticular juvenile rheumatoid arthritis.

OBJECTIVE: To investigate the relationship between clinical manifestations and sleep abnormalities in patients with juvenile rheumatoid arthritis (JRA). METHODS: Twenty-one patients with active polyarticular JRA and 20 healthy controls were enrolled consecutively. Pain and functional impairment were assessed with standardized, validated Brazilian questionnaires. Sleep evaluation was based on parent reporting of their child's sleep habits and polysomnography; subjects underwent an adaptation night in the sleep laboratory. Sleep architecture was analyzed and spectral analysis of non-rapid eye movement (REM) sleep was carried out by electroencephalography. RESULTS: Patients with JRA exhibited higher indexes of periodic leg movements (PLM; p = 0.02), isolated leg movements (LM), and arousals, as well as increases in alpha activity in non-REM sleep (all p < 0.01), in spite of similar frequency of sleep complaints in comparison to controls. Among JRA patients, greater alpha activity in non-REM sleep was observed in the participants with greater joint involvement assessed by the Escola Paulista de Medicina-Pediatric Range of Motion Scale (p = 0.03) or joint count (p = 0.02). Correlation was observed between morning stiffness and PLM and/or LM (rS = 0.75, Sr = 0.74, p < 0.001 for both), and between self-rating scores of pain and alpha activity in non-REM sleep (rS = 0.74, p < 0.001). CONCLUSION: Pain symptoms and disability are related to sleep fragmentation in patients with active polyarticular JRA.

The effects of peroxovanadate and peroxovanadyl on glucose metabolism in vivo and identification of signal transduction proteins involved in the mechanism of action in isolated soleus muscle.

The insulin-like effects of peroxovanate (POV) and peroxovanadyl (PSV) on rates of lactate formation and glycogen synthesis were measured in isolated incubated soleus muscle preparations. In another experiment rats were made insulin deficient by streptozotocin injection and treated with POV and PSV (0.25 mM) administered in the drinking water and in the course of 7 days glycemia were determined. Also, signal transduction proteins ERK 1 and ERK 2 involved in the insulin signaling were measured in soleus muscle of diabetic rats treated with POV and PSV. Peroxides of vanadate and vanadyl significantly stimulated glucose utilization in soleus muscle preparations in vitro. The stimulation of glycogen synthesis and lactate formation by POV and PSV was similar to insulin stimuli. Rats treated with POV or PSV presented reduction of glycemia, food and fluid intake with amelioration of the diabetic state during the short period of treatment (7 days). POV and PSV modulated ERK1/2 phosphorilation and the insulin administration in these rats caused an addictive effect on phosphorilation state of these proteins.

Diretrizes brasileiras para o diagnóstico de narcolepsia / Brazilian guidelines for the diagnosis of narcolepsy

Este artigo relata as conclusões da reunião de consenso com médicos especialistas sobre diagnóstico de narcolepsia baseada na revisão dos artigos sobre narcolepsia listados no Medline entre 1980 e 2010. A narcolepsia é uma doença crônica de início entre a primeira e segunda décadas de vida do indivíduo. Os sintomas essenciais são cataplexia e sonolência excessiva. A cataplexia é definida como episódios súbitos, recorrentes e reversíveis de fraqueza da musculatura esquelética desencadeados por situações de conteúdo emocional. Os sintomas acessórios são alucinações hipnagógicas, paralisia do sono e sono fragmentado. Critérios de diagnóstico clínico de acordo com a Classificação Internacional dos Transtornos do Sono são de sonolência excessiva e cataplexia. Recomenda-se a realização de polissonografia seguida do teste de latência múltipla do sono em um laboratório de sono para confirmação e diagnóstico de comorbidades. Quando não houver cataplexia, deve haver duas ou mais sonecas com sono REM no teste de latência múltipla do sono. Tipagem HLA-DQB1*0602 positiva com níveis de hipocretina-1 abaixo de 110pg/mL devem estar presentes para o diagnóstico de narcolepsia sem cataplexia e sem sonecas com sono REM.

This manuscript contains the conclusion of the consensus meeting on the diagnosis of narcolepsy based on the review of Medline publications between 1980-2010. Narcolepsy is a chronic disorder with age at onset between the first and second decade of life. Essential narcolepsy symptoms are cataplexy and excessive sleepiness. Cataplexy is defined as sudden, recurrent and reversible attacks of muscle weakness triggered by emotions. Accessory narcolepsy symptoms are hypnagogic hallucinations, sleep paralysis and nocturnal fragmented sleep. The clinical diagnosis according to the International Classification of Sleep Disorders is the presence of excessive sleepiness and cataplexy. A full in-lab polysomnography followed by a multiple sleep latency test is recommended for the confirmation of the diagnosis and co-morbidities. The presence of two sleep-onset REM period naps in the multiple sleep latency test is diagnostic for cataplexy-free narcolepsy. A positive HLA-DQB1*0602 with lower than 110pg/mL level of hypocretin-1 in the cerebrospinal fluid is required for the final diagnosis of cataplexy- and sleep-onset REM period -free narcolepsy.

Visual and automatic cyclic alternating pattern (CAP) scoring / Estagiamento visual e automático do padrão alternante cíclico (CAP)

The classification of short duration events in the EEG during sleep, as the A stage of the cyclic alternating pattern (CAP) is a tedious and error prone task. The number of events under normal conditions is large (several hundreds), and it is necessary to mark the limits of the events with precision, otherwise the time sensitive classification of the CAP phases (A and B) and specially the scoring of different types of A phases will be compromised. The objective of this study is to verify the feasibility of visual CAP scoring with only one channel of EEG, the evaluation of the inter-scorer agreement in a variety of recordings, and the comparison of the visual scorings with a known automatic scoring system. Sixteen hours of one channel (C4-A1 or C3-A2) of NREM sleep were extracted from eight whole night recordings in European Data Format and presented to the different scorers. The average inter-scorer agreement for all scorers is above 70 percent, the pair wise inter-scorer agreement found was between 69 percent up to 77.5 percent. These values are similar to what has been reported in different type studies. The automatic scoring system has similar performance of the visual scorings. The study also has shown that it is possible to classify the CAP using only one channel of EEG.

A classificação dos eventos de curta duração no EEG durante o sono, como a fase A do padrão alternante cíclico (CAP) é tarefa tediosa e propensa ao erro. O número de eventos é geralmente grande (centenas ao milhar), e é necessário marcar os limites dos eventos com precisão, caso contrário tanto a classificação das duas fases do CAP como a distinção dos diferentes tipos de fases A podem ser comprometidas. O objetivo deste estudo é a avaliação da concordância inter-estagiadores no CAP tanto visual quanto automático usando um único canal de EEG, a avaliação do acordo inter-estagiadores em uma variedade das gravações, e a comparação das classificações visuais com um sistema classificador automático. Dezesseis horas de um canal (C4-A1 ou C3-A2) do sono de NREM foram extraídas de oito gravações inteiras da noite no Formato de dados Europeus e apresentadas a diferentes classificadores, o acordo médio inter-estagiadores para todas as classificações está acima de 70 por cento, o acordo inter-estagiadores do par encontrado entre 69 por cento até 77,5 por cento. Estes valores são similares ao que foi relatado nos diferentes tipos de estudos. O sistema marcador automático tem o desempenho similar aos dos classificadores visuais. O estudo também mostrou que é possível classificar o CAP usando somente um canal de EEG.