Restless legs syndrome is highly prevalent in patients with post-polio syndrome.

OBJECTIVE: Few studies have quantified the prevalence of restless legs syndrome (RLS) in patients with post-polio syndrome (PPS). Our objective was to assess the prevalence and severity of RLS in patients with PPS and to examine the demographic characteristics of this population. METHOD: This was a cross-sectional study conducted from April 2010 to May 2012 at the outpatient Neuromuscular Disorders clinic of Universidade Federal de São Paulo, São Paulo, Brazil. We evaluated 119 patients with PPS, consecutively recruited, and investigated for RLS based on the diagnostic criteria established by the International Restless Legs Syndrome Study Group (IRLSSG). Patients were evaluated with the Brazilian version of the IRLSSG severity scale. RESULTS: The prevalence of RLS was 36% (n = 43; 32 women and 11 men). The ages at onset of RLS (median = 41 years) and PPS (median = 41 years) were concurrent, and the correlation between onset of symptoms of RLS and onset of symptoms of PPS was positive and very strong (Spearman r = 0.93, p = 0.01). The median RLS severity was 23 (range, 20-28). Low educational achievement and depression were predictive of RLS development. CONCLUSION: In the largest population of patients with PPS studied to date, our results indicate a high prevalence of RLS, marked disease severity, and concomitant onset of both conditions in many patients with PPS. Further studies are needed to elucidate a possible pathophysiologic mechanism linking these two conditions. We suggest that all post-polio patients with sensory and motor complaints in the legs be investigated for RLS.

Pharmacotherapy for residual excessive sleepiness and cognition in CPAP-treated patients with obstructive sleep apnea syndrome: A systematic review and meta-analysis.

Pharmacotherapy has been used as an adjunct to CPAP for treatment of residual excessive sleepiness in patients with a diagnosis of obstructive sleep apnea syndrome (OSAS). However, no studies with a high level of evidence have been conducted to support this practice and confirm its effectiveness. We conducted a meta-analysis to summarize and quantify the effects of pharmacological treatment in adults with OSAS who experience residual excessive sleepiness despite adequate CPAP use. We reviewed clinical trials that compared medications to placebo and evaluated the outcomes residual excessive sleepiness, cognition, and quality of life, as well as treatment effectiveness and safety. The MEDLINE, EMBASE, LILACS, Cochrane Central Register of Controlled Trials - CENTRAL, and PsycINFO electronic databases were searched using highly sensitive search strategies. Trials were only included if measures were taken to ensure effective CPAP treatment. Eight randomized clinical trials were included. Pharmacotherapy with modafinil and armodafinil led to improvement of excessive daytime sleepiness, attention/alertness, and clinical condition as measured with the CGI-C. No improvements in quality of life or other cognitive domains (including memory, executive function, and language) could be confirmed. Pharmacotherapy did not cause any severe adverse effects, but was associated with significant dropout rates as compared with placebo. In conclusion, although our results demonstrate the effectiveness of pharmacological treatment as an adjunct to CPAP, further investigation is necessary to improve confidence in its effects. Many findings on the impact of pharmacotherapy on cognition and quality of life were evaluated through analysis of single studies, with heterogeneity in tests and absence of standardization, which reduced certainty as to whether actual improvement occurred in these outcomes.

Sleep disorders in Machado-Joseph disease.

PURPOSE OF REVIEW: This article provides a description on clinical features and pathophysiology of the main sleep disorders observed in Machado-Joseph disease (MJD). RECENT FINDINGS: Pathological studies have clearly demonstrated that degenerative process in MJD is widespread in the nervous system, and not restricted to the cerebellum. Nonmotor manifestations are frequent and may include pain, cramps, dysautonomia, cognitive deficits, psychiatric manifestations, olfactory deficits, fatigue, nutritional issues, and sleep disorders. SUMMARY: Sleep disorders are frequent in MJD, and include restless legs syndrome, rapid eye movement sleep behavior disorder, excessive daytime sleepiness, insomnia, sleep apnea, periodic limb movements during sleep, parasomnia, and others. Pathophysiological mechanisms related to sleep disorders in Machado-Joseph are complex and poorly understood. Considering that sleep complaints are a treatable condition, recognizing sleep disorders in MJD is relevant.

Intake of stimulant foods is associated with development of parasomnias in children / Ingestão de alimentos estimulantes está associada ao desenvolvimento de parassonias em crianças

ABSTRACT Objective To verify if nighttime feeding habits can influence parasomnia in children. Method Seven private and four public Elementary Schools took part in the study. A total of 595 Sleep Disturbance Scale for Children were distributed to the parents of children aged from 7 to 8 years. Data of dietary recall, starting time to school, physical activity, and nutritional status were studied. Results Of the 226 questionnaires completed, 92 (41%) reported parasomnia. Girls had 2.3 times more the chance to parasomnia than boys. Children who consumed stimulant foods had 2.6 times more chance to have parasomnia than those of children who consumed non-stimulant foods. There were no difference between parasomnia and no-parasomnia groups in food type (p = 0.78) or timing of last meal before bedtime (p = 0.50). Conclusion Our findings suggest that intake of stimulant foods is associated with development of parasomnia in children.

RESUMO Objetivo Verificar se hábitos de alimentação noturna influenciam parassonias em crianças. Método Sete escolas privadas e quatro públicas, de Ensino Fundamental, fizeram parte do estudo. Um total de 595 Escalas de Distúrbio do Sono para Crianças foram distribuídas para os pais de crianças entre 7 e 8 anos. Dados de recordatório alimentar, período escolar, atividade física e estado nutricional foram estudados. Resultados Dos 226 questionários preenchidos, 92 (41%) relataram presença de parassonias. Meninas tiveram 2,3 vezes mais chance de parassonias e crianças que consumiram alimentos estimulantes tiveram 2,6 vezes mais chance de parassonias em relação àquelas que consumiram alimentos não estimulantes. Não houve diferença entre os grupos em relação ao tipo de alimento (p = 0,78) ou horário da última refeição antes de ir para a cama (p = 0,50). Conclusão Nossos achados sugerem que a ingestão de alimentos estimulantes está associada com o desenvolvimento de parassonias em crianças.

Intake of stimulant foods is associated with development of parasomnias in children.

OBJECTIVE: To verify if nighttime feeding habits can influence parasomnia in children. METHOD: Seven private and four public Elementary Schools took part in the study. A total of 595 Sleep Disturbance Scale for Children were distributed to the parents of children aged from 7 to 8 years. Data of dietary recall, starting time to school, physical activity, and nutritional status were studied. RESULTS: Of the 226 questionnaires completed, 92 (41%) reported parasomnia. Girls had 2.3 times more the chance to parasomnia than boys. Children who consumed stimulant foods had 2.6 times more chance to have parasomnia than those of children who consumed non-stimulant foods. There were no difference between parasomnia and no-parasomnia groups in food type (p = 0.78) or timing of last meal before bedtime (p = 0.50). CONCLUSION: Our findings suggest that intake of stimulant foods is associated with development of parasomnia in children.

Orthodontic and orthopaedic treatment for anterior open bite in children.

BACKGROUND: Anterior open bite occurs when there is a lack of vertical overlap of the upper and lower incisors. The aetiology is multifactorial including: oral habits, unfavourable growth patterns, enlarged lymphatic tissue with mouth breathing. Several treatments have been proposed to correct this malocclusion, but interventions are not supported by strong scientific evidence. OBJECTIVES: The aim of this systematic review was to evaluate orthodontic and orthopaedic treatments to correct anterior open bite in children. SEARCH METHODS: The following databases were searched: the Cochrane Oral Health Group's Trials Register (to 14 February 2014); the Cochrane Central Register of Controlled Trials (CENTRAL)(The Cochrane Library 2014, Issue 1); MEDLINE via OVID (1946 to 14 February 2014); EMBASE via OVID (1980 to 14 February 2014); LILACS via BIREME Virtual Health Library (1982 to 14 February 2014); BBO via BIREME Virtual Health Library (1980 to 14 February 2014); and SciELO (1997 to 14 February 2014). We searched for ongoing trials via ClinicalTrials.gov (to 14 February 2014). Chinese journals were handsearched and the bibliographies of papers were retrieved. SELECTION CRITERIA: All randomised or quasi-randomised controlled trials of orthodontic or orthopaedic treatments or both to correct anterior open bite in children. DATA COLLECTION AND ANALYSIS: Two review authors independently assessed the eligibility of all reports identified. Risk ratios (RRs) and corresponding 95% confidence intervals (CIs) were calculated for dichotomous data. The continuous data were expressed as described by the author. MAIN RESULTS: Three randomised controlled trials were included comparing: effects of Frankel's function regulator-4 (FR-4) with lip-seal training versus no treatment; repelling-magnet splints versus bite-blocks; and palatal crib associated with high-pull chincup versus no treatment.The study comparing repelling-magnet splints versus bite-blocks could not be analysed because the authors interrupted the treatment earlier than planned due to side effects in four of ten patients.FR-4 associated with lip-seal training (RR = 0.02 (95% CI 0.00 to 0.38)) and removable palatal crib associated with high-pull chincup (RR = 0.23 (95% CI 0.11 to 0.48)) were able to correct anterior open bite.No study described: randomisation process, sample size calculation, there was not blinding in the cephalometric analysis and the two studies evaluated two interventions at the same time. These results should be therefore viewed with caution. AUTHORS' CONCLUSIONS: There is weak evidence that the interventions FR-4 with lip-seal training and palatal crib associated with high-pull chincup are able to correct anterior open bite. Given that the trials included have potential bias, these results must be viewed with caution. Recommendations for clinical practice cannot be made based only on the results of these trials. More randomised controlled trials are needed to elucidate the interventions for treating anterior open bite.

Neurophysiological studies and non-motor symptoms prior to ataxia in a patient with machado-joseph disease: trying to understand the natural history of brain degeneration.

Spinocerebellar ataxia type 3 or Machado-Joseph disease is the most common spinocerebellar ataxia. In this neurological disease, anatomical, physiological, clinical, and functional neuroimaging demonstrate a degenerative process besides the cerebellum. We performed neurophysiological and neuroimaging studies-polysomnography, transcranial sonography, vestibular-evoked myogenic potential, single-photon emission computed tomography (SPECT) with (99m)Tc-TRODAT-1, and a formal neuropsychological evaluation in a patient with sleep complaints and positive testing for Machado-Joseph disease, without cerebellar atrophy, ataxia, or cognitive complaints. Polysomnography disclosed paradoxical high amplitude of submental muscle, characterizing REM sleep without atonia phenomenon. Transcranial sonography showed hyperechogenicity of the substantia nigra. There was an absence of vestibular-evoked myogenic potentials on both sides in the patient under study, in opposite to 20 healthy subjects. Brain imaging SPECT with (99m)Tc-TRODAT-1 demonstrated a significant lower DAT density than the average observed in six healthy controls. Electroneuromyography was normal. Neuropsychological evaluation demonstrated visuospatial and memory deficits. Impairment of midbrain cholinergic and pontine noradrenergic systems, dysfunction of the pre-synaptic nigrostriatal system, changes in echogenicity of the substantia nigra, and damage to vestibulo-cervical pathways are supposed to occur previous to cerebellar involvement in Machado-Joseph disease.

Excessive fragmentary myoclonus in Machado-Joseph disease.

OBJECTIVE: Machado-Joseph disease (MJD) is a neurodegenerative disease which usually presents several clinical findings including cerebellar ataxia and other extracerebellar features, such as Parkinsonism, dystonia, peripheral neuropathy, and lower motor neuron disease. Some data have demonstrated a high frequency of sleep disorders in these patients, including excessive daytime sleepiness (EDS), insomnia, obstructive sleep apnea (OSA), rapid eye movement (REM) sleep behavior disorder (RBD), and restless legs syndrome (RLS). Herein, we aimed to describe the high frequency of excessive fragmentary myoclonus (EFM) in MJD. MATERIALS AND METHODS: We recruited 44 patients with MJD and 44 healthy controls. All participants underwent an all-night polysomnography (PSG). EFM was evaluated and defined in accordance to the criteria of the American Academy of Sleep Medicine. RESULTS: Half of the MJD patients (n = 22) had EFM diagnosed through PSG, though no healthy control participant presented this finding (P < .0001). In the MJD group, older participants and men had a higher frequency of EFM. There was no correlation between EFM and the following data: body mass index (BMI), apnea-hypopnea index (AHI), EDS, loss of atonia during REM sleep, periodic limb movements during sleep (PLMS), RLS, RBD, ataxia severity, the number of cytosine-adenine-guanine trinucleotide (CAG) repeats, disease duration, sleep efficiency, sleep fragmentation, and sleep stage percentages between patients with or without EFM. CONCLUSION: EFM is highly prevalent in patients with MJD. Our study demonstrates that EFM must be included in the clinical spectrum of sleep disorders in MJD patients.

Restless legs syndrome: a qualitative analysis of psychosocial suffering and interdisciplinary attention.

The aim of this study is to investigate psychosocial factors related to the diagnosis and treatment of patients with restless legs syndrome. Fifteen patients were interviewed at the Neuro-Sono Outpatient Clinic, Universidade Federal de São Paulo. The results were submitted to a qualitative analysis. We identified four content categories: illness description, illness history, illness experience, and relationships. Lack of control over the body and lack of recognition by professionals produce stigma and lead patients to suffering. The research underscores the relevance of psychosocial factors to the diagnosis and treatment of patients with restless legs syndrome and the importance of having interdisciplinary teams when attending patients with restless legs syndrome.

Restless legs syndrome in post-polio syndrome: a series of 10 patients with demographic, clinical and laboratorial findings.

BACKGROUND: Few studies have described the occurrence of restless legs syndrome in post-polio syndrome. METHODS: We studied 10 consecutive patients with post-polio syndrome and symptoms of restless legs syndrome. We look at demographic, clinical and laboratorial data. RESULTS: A remarkable finding was the concomitant onset of symptoms of both diseases, suggesting a possible underlying mechanism. Severity of restless legs symptoms was moderate to very severe. CONCLUSION: Epidemiological studies with larger samples are needed to better establish the relationship and the incidence of restless legs syndrome in post-polio syndrome.

Sleep disturbance scale for children: translation, cultural adaptation, and validation.

INTRODUCTION: The Sleep Disturbance Scale for Children (SDSC) is a 26-item instrument for evaluating sleep among children aged 3-18 years. It differentiates among conditions such as disorders of initiating and maintaining sleep, sleep breathing disorders, disorders of arousal, sleep-wake transition disorders, excessive somnolence, and sleep hyperhydrosis. The aim of this study was to translate, culturally adapt, and validate it for Brazilian Portuguese. METHOD: The study was carried out in two phases: (1) forward translation, back translation, pretesting, and calculation of sample size; (2) validation: reliability (Chronbach's alpha), convergent analysis (Pearson correlation), and discriminatory validity (comparing the scores of the test with the results of polysomnography). One hundred children, aged 3-18 years, accompanied by their parents and/or guardians participated in the phases. PSG studies have been done to calculate the sample size and validation. RESULTS: The scale instructions and items were adapted regarding semantic, experiential, conceptual, and cultural equivalence validation. The scale structure related to visual communication was also adapted to Brazilian population preference and habits, and this resulted in a chart with clear instructions and easy recognition of the statements and possible responses. Reliability analysis showed values greater than 0.55. There has been reasonable convergent validity. Discriminatory validity using the PSG study for positive sleep-disordered breathing (SDB) was 8.9, attesting discriminatory validity only for SDB. The three questions of the scale can screen SDB. CONCLUSION: The SDSC was translated, adapted and validated for Brazilian Portuguese, and it presented internal consistency and convergent and discriminatory validity. It can be used in population-based studies in order to screen for sleep-disordered breathing in children.

Translation and validation into the Brazilian Portuguese of the restless legs syndrome rating scale of the International Restless Legs Syndrome Study Group.

BACKGROUND: Restless legs syndrome (RLS) is a chronic sensory-motor disorder characterized by unpleasant limb sensations and an irresistible urge to move. The International Restless Legs Syndrome Study Group developed the Restless Legs Syndrome Rating Scale (IRLS) to assess the severity of RLS symptoms. The objective of this study was to translate and validate the IRLS into Brazilian Portuguese. METHOD: The IRLS was translated into Brazilian Portuguese, analyzed, back translated to English, and compared to the original version. It was applied to 10 patients for cultural verification. The language was adjusted and the final version was administered to 30 patients (13 male, mean age 58.88+/-14.82). RESULTS: There was correlation among the IRLS evaluation of three experts. Many linguistic adaptations were required to achieve cultural adequacy and the Cronbach's alpha coefficient showed reliability of 80%. CONCLUSION: IRLS was translated, adapted, and validated to Brazilian Portuguese language, showing good reliability and validity.

Translation and validation into the Brazilian Portuguese of the restless legs syndrome rating scale of the International Restless Legs Syndrome Study Group / Tradução e validação para a língua portuguesa da escala de graduação da síndrome das pernas inquietas do Grupo Internacional do Estudo da Síndrome das Pernas Inquietas

BACKGROUND: Restless legs syndrome (RLS) is a chronic sensory-motor disorder characterized by unpleasant limb sensations and an irresistible urge to move. The International Restless Legs Syndrome Study Group developed the Restless Legs Syndrome Rating Scale (IRLS) to assess the severity of RLS symptoms. The objective of this study was to translate and validate the IRLS into Brazilian Portuguese. METHOD: The IRLS was translated into Brazilian Portuguese, analyzed, back translated to English, and compared to the original version. It was applied to 10 patients for cultural verification. The language was adjusted and the final version was administered to 30 patients (13 male, mean age 58.88±14.82). RESULTS: There was correlation among the IRLS evaluation of three experts. Many linguistic adaptations were required to achieve cultural adequacy and the Cronbach's alpha coefficient showed reliability of 80 percent. CONCLUSION: IRLS was translated, adapted, and validated to Brazilian Portuguese language, showing good reliability and validity.

INTRODUÇÃO: A síndrome das pernas inquietas (SPI) é uma doença crônica, sensório-motora, caracterizada por sensações desagradáveis nos membros e uma urgência em movimentá-los. O Grupo Internacional de Estudos da Síndrome das Pernas Inquietas desenvolveu a Escala de Graduação da Síndrome das Pernas Inquietas (EGSPI) para avaliar a gravidade dos sintomas da SPI. OBJETIVO: Traduzir, adaptar culturalmente e validar a EGSPI para o português do Brasil. MÉTODO: A escala foi vertida para o português, analisada, vertida novamente para o inglês e comparada com a versão original. Foi aplicada em 10 pacientes para adequação cultural. A linguagem foi ajustada e a versão final foi aplicada em 30 pacientes (13 homens, idade média de 58,88±14,82). RESULTADOS: Houve correlação da aplicação da escala entre três avaliadores. Foram necessárias adaptações lingüísticas para adequação cultural e o alfa de Chronbach mostrou confiabilidade de 80 por cento. CONCLUSÃO: A EGSPI foi traduzida, adequada e validada para o português do Brasil, com boa validade e confiabilidade.

Sleep disorders are associated with impulsivity in school children aged 8 to 10 years.

CONTEXT: Sleep has an important function in the physical and emotional development of children. Some studies suggest an association between impulsivity and sleep disorders. However, little is known about this association in schoolchildren aged 8 to 10 years. METHOD: We studied 1180 children, 547 with sleep disorders (SD) and 633 without SD (control group), classified with SD questionnaires. Within the SD group, 53 children with sleep-related respiratory disorders (SRRD) and 521 children with non-respiratory sleep disorders (NRSD) were analyzed. We assessed emotional indicators of impulsivity with Bender test. RESULTS: More SD children presented impulsivity than control group (p<0.05). More NRSD and 10 years old children presented impulsivity than control group of the same age (p=0.001). Impulsivity and SRRD were associated with 8 years old children (p<0.05). CONCLUSION: Children with SD, 8 years old children with SRRD, and 10 years old children with NRSD presented higher proportion of impulsivity than control children.

Sleep disorders and starting time to school impair balance in 5-year-old children.

OBJECTIVE: To verify if sleep disorders and different starting time to school have impaired motor skills in 5-year-old children. METHOD: Cross-sectional design consisting of 132 children with sleep disorders and 136 normal controls of the public school in the city of São Paulo. The group with sleep disorders was identified based on a questionnaire, and motor tests for global motor coordination, fine motor coordination, perceptual-motor coordination, and static and dynamic balance were applied in all children. RESULTS: In the static balance test, more specifically in the sharpened Romberg (Tandem) test, 34% of boys from the study group, who studied in the morning, failed the test (p < 0.05). In the single leg stance test, 62% of boys from the study group who studied in the morning failed (p < 0.05). CONCLUSION: This study suggests that sleep disorders may interact with the school period and alter motor performance, especially in boys studying in the morning.