Effect of positive airway pressure treatment on excessive fragmentary myoclonus in 100 sleep-related breathing disorder patients.

STUDY OBJECTIVES: Excessive fragmentary myoclonus (EFM) is a frequent finding in patients undergoing video-polysomnography (VPSG). We aimed to evaluate the potential effect of sleep-related breathing disorder's treatment with positive airway pressure (PAP) therapy on EFM. METHODS: One hundred consecutive patients with EFM and sleep-related breathing disorder subsequently treated with PAP at the sleep lab of the Medical University of Innsbruck, Department of Neurology, Austria, were included. Each patient underwent two nights of VPSG: the first night without and the second night with PAP therapy. Fragmentary myoclonus was automatically scored with validated software, and fragmentary myoclonus index (FMI) and minutes of non-rapid eye movement (NREM) sleep with EFM (minNREM+EFM) were calculated. RESULTS: Under PAP therapy there was a significant decrease in the minNREM+EFM - 60.5 (9.5-161.8) at baseline vs. 37.5 (6.3-168.8) minutes under PAP, p = 0.025. No significant differences were observed for FMI between the two nights. Sleep variables, sleep diagnoses, comorbidities, and medication did not influence FMI or the minNREM+EFM. CONCLUSIONS: The initiation of PAP treatment led to a significant reduction of minNREM+EFM, but not of FMI. The results suggest that PAP therapy might influence the distribution of FM potentials.

[Restless sleep disorder a new entity to considered in children]. / Trastorno de sueño inquieto: una nueva entidad clínica a considerar en niños.

Restless sleep disorder (RSD) is a condition recently described by a group of sleep experts who developed diagnostic and polysomnographic criteria after conducting a comprehensive review of the available literature where poor sleep or restless sleep is a symptom alone or that accompanies other disorders. This group defined RSD as a condition characterized by parental complaints of frequent jerking movements during sleep, position changes, and sleep disruption that cause significant impairment during the day. Diagnostic criteria include the presence of symptoms for at least 3 months, 3 times a week, and at least 5 movements per hour on polysomnography. Changes in behavior during the day, such as drowsiness, irritability, and hyperactivity that are not explained by a medical, pharmacological, or behavioral condition, should be considered. Its estimated prevalence is 7.7% of children referred for sleep problems. Children often have ferritin levels below 50 µg/l, a point in common with restless legs syndrome. Treatment consists of iron supplements, which have shown benefits in these children. To establish the diagnosis, secondary symptoms of medical origin or other sleep disorders such as restless legs syndrome or periodic limb movement disorder must be ruled out. The objective of this report is to review the current recommendations on this entity, describe the clinical, pathophysiological, and polysomnographic keys, in order to highlight the need to publicize this condition and expand studies in age groups other than those already defined and to generate treatment guidelines.

Sleep Disorders.

Sleep disorders are frequent and can have serious consequences on patients' health and quality of life. While some sleep disorders are more challenging to treat, most can be easily managed with adequate interventions. We review the main diagnostic features of 6 major sleep disorders (insomnia, circadian rhythm disorders, sleep-disordered breathing, hypersomnia/narcolepsy, parasomnias, and restless legs syndrome/periodic limb movement disorder) to aid medical practitioners in screening and treating sleep disorders as part of clinical practice.

Sleep Disorders and Their Management in Children With Ehlers-Danlos Syndrome Referred to Sleep Clinics.

STUDY OBJECTIVES: The nature of sleep disorders in children with Ehlers-Danlos syndrome (EDS) is unknown. We aimed to describe the type, the management, and the short-term outcome of sleep disorders in children with EDS referred to sleep clinics. METHODS: This is a retrospective review of medical records and polysomnography tests of children with EDS younger than 18 years who were referred to the sleep clinic. Demographic information and medical history were collected, and polysomnography tests were reviewed. Questionnaires completed during previous clinic visits, including the Pediatrics Sleep Questionnaire (PSQ), Epworth Sleepiness Scale (ESS), and Pediatric Quality of Life Inventory (PedsQL), were also evaluated. RESULTS: Sixty-five patients with EDS-hypermobility type were included. The mean age was 13.15 ± 3.9 years. There were 68% of patients who were female, and 91% of patients were Caucasian. The mean follow-up period was 1.14 ± 1.55 years. Common sleep diagnoses included insomnia (n = 14, 22%), obstructive sleep apnea (OSA) (n = 17, 26%), periodic limb movement disorder (PLMD) (n = 11, 17%), and hypersomnia (n = 10, 15%). In addition, 65% required pharmacologic treatment and 29% were referred to behavioral sleep medicine. For OSA, two patients required continuous positive airway pressure. A significant improvement was observed in the PSQ, ESS, and PedsQL scores during follow-up visits after treatment (n = 34; P = .0004, 0.03, and 0.01, respectively). CONCLUSIONS: There is a high prevalence of sleep disorders, including OSA, insomnia, PLMD, and hypersomnia in children with EDS referred to sleep clinics. Specific management can improve quality of life and questionnaire scores of this patient population. Our study emphasizes the importance of screening for sleep disorders in children with EDS.

Restless Legs Syndrome and Sleep-Related Movement Disorders.

PURPOSE OF REVIEW: This article provides an update on six sleep-related movement disorders: restless legs syndrome (RLS), periodic limb movement disorder, sleep-related leg cramps, bruxism, rhythmic movement disorder, and propriospinal myoclonus, with an emphasis on RLS. RECENT FINDINGS: RLS is a common sensorimotor disorder that impairs quality of life. RLS is frequently comorbid to neurologic, psychiatric, vascular, and inflammatory diseases. Accumulating evidence implicates the pathophysiology of RLS as a state of dopamine dysfunction and iron deficiency that occurs on a background of genetic susceptibility conferred by 6 gene polymorphisms. Multiple treatments approved by the US Food and Drug Administration (FDA) are available. Dopamine agonists and α2δ calcium channel ligands are considered first-line treatments, but these treatments have very different side effect profiles that should be taken into consideration. SUMMARY: Sleep-related movement disorders are frequently encountered in clinical practice. For some disorders, particularly RLS and periodic limb movement disorder, our understanding of biology, epidemiology, and treatment is advanced. For others, much work is needed to determine optimal treatment strategies.

Sleep-related movement disorders and disturbances of motor control.

PURPOSE OF REVIEW: Review of the literature pertaining to clinical presentation, classification, epidemiology, pathophysiology, diagnosis, and treatment of sleep-related movement disorders and disturbances of motor control. RECENT FINDINGS: Sleep-related movement disorders and disturbances of motor control are typically characterized by positive motor symptoms and are often associated with sleep disturbances and consequent daytime symptoms (e.g. fatigue, sleepiness). They often represent the first or main manifestation of underlying disorders of the central nervous system, which require specific work-up and treatment. Diverse and often combined cause factors have been identified. Although recent data provide some evidence regarding abnormal activation and/or disinhibition of motor circuits during sleep, for the majority of these disorders the pathogenetic mechanisms remain speculative. The differential diagnosis is sometimes difficult and misdiagnoses are not infrequent. The diagnosis is based on clinical and video-polysomnographic findings. Treatment of sleep-related motor disturbances with few exceptions (e.g. restless legs/limbs syndrome) are based mainly on anecdotal reports or small series. SUMMARY: More state-of-the-art studies on the cause, pathophysiology, and treatment of sleep-related movement disorders and disturbances of motor control are needed.

Restless Legs Syndrome/Willis-Ekbom Disease and Periodic Limb Movements in Sleep in the Elderly with and without Dementia.

There is great interest in the study of sleep in healthy and cognitively impaired elderly. Sleep disorders have been related to quality of aging. Sleep-related movements are a frequent cause of disordered sleep and daytime sleepiness. Restless legs syndrome/Willis-Ekbom disease (RLS/WED) is often unrecognized in the elderly. This review explores RLS/WED in the elderly population. The elderly population may be subdivided into 3 groups: healthy, dependent, and frail. The RLS/WED could be a predictor for lower physical function; its burden on quality of life and health care-related costs, in the elderly, should be an important clinical and public health concern.

Pediatric restless legs syndrome diagnostic criteria: an update by the International Restless Legs Syndrome Study Group.

BACKGROUND: Specific diagnostic criteria for pediatric restless legs syndrome (RLS) were published in 2003 following a workshop at the National Institutes of Health. Due to substantial new research and revision of the adult RLS diagnostic criteria, a task force was chosen by the International Restless Legs Syndrome Study Group (IRLSSG) to consider updates to the pediatric diagnostic criteria. METHODS: A committee of seven pediatric RLS experts developed a set of 15 consensus questions to review, conducted a comprehensive literature search, and extensively discussed potential revisions. The committee recommendations were approved by the IRLSSG executive committee and reviewed by the IRLSSG membership. RESULTS: The pediatric RLS diagnostic criteria were simplified and integrated with the newly revised adult RLS criteria. Specific recommendations were developed for pediatric application of the criteria, including consideration of typical words used by children to describe their symptoms. Pediatric aspects of differential diagnosis, comorbidity, and clinical significance were then defined. In addition, the research criteria for probable and possible pediatric RLS were updated and criteria for a related condition, periodic limb movement disorder (PLMD), were clarified. CONCLUSIONS: Revised diagnostic criteria for pediatric RLS have been developed, which are intended to improve clinical practice and promote further research.

The effects of periodic limb movements in sleep (PLMS) on cardiovascular disease.

Periodic limb movements in sleep (PLMS) are uncontrollable nocturnal movements that occur during sleep and increase with age. Research has implicated PLMS as a contributing factor to the development of cardiovascular disease (CVD). The purpose of this manuscript is to 1) explain the sleep disorder of PLMS and implications on CVD; 2) identify the impact of PLMS on CVD; 3) discuss treatment options for PLMS; 4) present future research needs for PLMS/RLS; 5) provide implications to health care providers to improve the care and health outcomes of persons with PLMS.

Bilateral globus pallidus internus deep brain stimulation in Parkinson's disease with periodic limb movement disorder.

Deep brain stimulation for Parkinson's disease is a well-established therapeutic intervention for refractory disease. The main nuclear targets are the subthalamic nucleus and the globus pallidus internus. Periodic limb movements are often an associated condition in patients with Parkinson's disease. Both conditions can respond to dopaminergic agents. The case of a 57-year-old man with severe Parkinson's disease and periodic limb movement who underwent bilateral globus pallidus deep brain stimulation surgery for his Parkinson's disease is presented and discussed. Intraoperatively the patient's periodic limb movements responded to voltage stimulation higher than those required for improvement of his parkinsonian symptoms. The immediate and reversible improvement in these symptoms contralateral to the stimulation site suggests pallidal stimulation may directly ameliorate periodic limb movement symptoms.

The treatment of restless legs syndrome and periodic limb movement disorder in adults--an update for 2012: practice parameters with an evidence-based systematic review and meta-analyses: an American Academy of Sleep Medicine Clinical Practice Guideline.

A systematic literature review and meta-analyses (where appropriate) were performed to update the previous AASM practice parameters on the treatments, both dopaminergic and other, of RLS and PLMD. A considerable amount of literature has been published since these previous reviews were performed, necessitating an update of the corresponding practice parameters. Therapies with a STANDARD level of recommendation include pramipexole and ropinirole. Therapies with a GUIDELINE level of recommendation include levodopa with dopa decarboxylase inhibitor, opioids, gabapentin enacarbil, and cabergoline (which has additional caveats for use). Therapies with an OPTION level of recommendation include carbamazepine, gabapentin, pregabalin, clonidine, and for patients with low ferritin levels, iron supplementation. The committee recommends a STANDARD AGAINST the use of pergolide because of the risks of heart valve damage. Therapies for RLS secondary to ESRD, neuropathy, and superficial venous insufficiency are discussed. Lastly, therapies for PLMD are reviewed. However, it should be mentioned that because PLMD therapy typically mimics RLS therapy, the primary focus of this review is therapy for idiopathic RLS.

Change in frequency of periodic limb movements during sleep with usage of continuous positive airway pressure in obstructive sleep apnea syndrome.

Periodic limb movements during sleep (PLMS) sometimes newly appear on the night of continuous positive airway pressure (CPAP) titration in patients with obstructive sleep apnea syndrome (OSAS). To ascertain the incidence and causative factors of this phenomenon, we investigated differences in its prevalence and the factors associated with newly appeared and persistent PLMS on CPAP titration night. We retrospectively analyzed polysomnographic data of 997 consecutive OSAS outpatients who had undergone overnight CPAP titration. On the basis of changes in periodic limb movements index (PLMI) values (cut off level≥15/h) from baseline polysomnography (BPSG) to CPAP titration PSG, patients were assigned to one of four groups: persistent, CPAP-emergent, CPAP-disappeared, and non-PLMS. The rate of patients was 6.7% in the persistent group, 8.0% in the CPAP-emergent group, 4.0% in the CPAP-disappearance group, and 81.2% in the non-PLMS group. Multivariate logistic regression analysis revealed that a higher apnea-hypopnea index (AHI) on BPSG and ≥47years of age appeared to be associated with the CPAP-emergent group. The results suggest that elderly patients with higher AHI at BPSG may present with CPAP-emergent PLMS.

Polysomnography reveals unexpectedly high rates of organic sleep disorders in patients with prediagnosed primary insomnia.

OBJECTIVE: It is a matter of debate whether patients with primary insomnia require a polysomnographic examination in order to exclude specific sleep disorders such as sleep apnea syndrome (SAS) or periodic limb movements (PLM). Using a prospective design, we investigated the prevalence of organic sleep disorders by means of polysomnography (PSG) in a series of patients who were previously diagnosed with primary insomnia. This diagnosis was based on a clinical exam and an ambulatory monitoring device or previous PSG. METHODS: Seventy-seven women and 16 men (mean age 55.12 ± 13.21 years) who were admitted for cognitive behavioral therapy for insomnia were evaluated by PSG including cardiorespiratory parameters and tibialis EMG. Among them, 50 patients had undergone a clinical exam by a sleep specialist; in 18 patients, actigraphy or portable monitoring had been performed to exclude SAS or PLM; 25 patients had undergone PSG in another sleep lab previously. RESULTS: In 32 patients (34% of the sample), a PSG revealed a specific sleep disorder (SAS 16; PLMD 11; both 5), resulting in therapeutic consequences for 21 patients (SAS 10; PLMD 9; both 2). SAS and PLM patients were older and SAS patients had a higher body mass index than insomnia patients without additional findings. CONCLUSION: Indications for a PSG should be handled less restrictively in the diagnostic workup of older insomnia patients since they have a higher risk of comorbid sleep disorders even in the absence of the clinical signs of SAS or PLM.

Update in pediatric sleep medicine.

PURPOSE OF REVIEW: To summarize the most important recent scientific developments in the field of clinical pediatric sleep medicine, specifically as relates to sleep disorders and sleep in special pediatric populations. RECENT FINDINGS: The major themes covered in this review include sleep-disordered breathing; narcolepsy; restless legs syndrome/periodic limb movement disorder; insomnia; sleep in children with medical conditions; and sleep in children with neurodevelopmental and psychiatric disorders. SUMMARY: An expanded understanding of the pathophysiology, epidemiology, clinical evaluation methods, sequelae, and empirically supported treatment options of common pediatric sleep disorders, especially in high-risk populations, is key to a rational approach to diagnosis and management of these children in clinical settings.

Sleep disorders over the full range of chronic kidney disease.

Sleep disorders are common and underrecognized in patients at all stages of chronic kidney disease. They include sleep apnea, insomnia, excessive sleepiness, restless legs syndrome and periodic limb movement disorder. They can be related to underlying uremia or comorbidities. Sleep disorders can affect the quality of life, and some are associated with increased morbidity and mortality. Clinical assessment, polysomnography and other standardized assessments are required for diagnosis. Therapeutic approaches include improvement in uremia management, treatment of comorbidities or specific interventions directed at individual sleep disorders. Diagnosis and treatment of sleep disorders in this population may improve quality of life and patient survival.

Escalas de compromiso funcional y de movimientos involuntarios en extremidades superiores en niños con trastornos de movimiento de tipo extrapiramidal / Scales evaluating functional compromise and involuntary upper extremity movements in children with extra pyramidal disorders

Introducción. Los niños con parálisis cerebral de tipo extrapiramidal requieren de una evaluación funcional global que mida cambios antes, durante y después de tratamientos. Objetivo. Validar dos escalas de evaluación: compromiso funcional global y tipo de movimiento involuntario predominante en extremidades superiores. Pacientes y método. 63 pacientes seleccionados aleatoriamente de Teleton-Santiago-Chile-2006, diagnóstico parálisis cerebral, alteración de movimiento de tipo extrapiramidal, de 4–18 años, que cumplieron requisitos de inclusión; se aplicó escala de compromiso funcional (con dimensiones: postura sedente, actividades de la vida diaria y funcionalidad manual) y escala de movimientos Involuntarios (atetosis, coreatetosis y distonía). Resultados. La escala de compromiso funcional muestra alta consistencia interna (α Cronbach 0,94), valor kappa de variabilidad entre observadores de 0,78; validez de constructo constituido por 2 componentes principales que explicaron 75,7% de la funcionalidad. La escala clasificó a los niños en 46% como grave o severo, 25,4% como moderado, 20,6% como leve y sólo al 7,9% como clínicamente normal. La escala de movimientos involuntarios registró confiabilidad con valor kappa de 0,85 y consistencia interna de 0,97; clasificó como distónicos al 38,1% de los niños, coreatetósicos el 33,3% y 28,9% como atetósicos. Conclusión. Las escalas demostraron ser confiables y válidas por lo tanto, pueden ser utilizadas como ayuda en la planificación y evolución terapéutica(AU)

Introduction. Children with extrapyramidal cerebral palsy require a comprehensive functional assessment that records their functions before, and measures the changes during and after the treatments. Objective. Validate two assessment scales: global functionality and type of predominant unintentional movement in upper extremities. Patients and method. 63 patients were randomly selected from Teleton-Santiago-Chile-2006, diagnosed with cerebral palsy, extrapyramidal type alteration movement, 4 to 18 years, which met the inclusion requirements: they were evaluated with the functional compromise scale (with dimensions: sitting posture, daily living activities, and manual functionality) and with the involuntary movements scale (athetosis, choreathetosis and dystonia). Results. The scale of functional assessment shows high internal consistency (α Cronbach 0.94), a kappa value of variability among observers of 0.78, and construct validity, which consists of two main components that explained 75.7% of the functionality. The scale ranked children as: 46% as serious or severe, 25.4% as moderate, 20.6% as mild, and only 7.9% as normal. The scale of involuntary movements showed high reliability with kappa value of 0.85 and internal consistency of 0.97, and ranked 38.1% of the children as dystonic, 33.3% as choreathetosics, and 28.9% as athetosics. Conclusion. The scales proved to be reliable and valid therefore can be used as an aid in planning and therapeutic evaluation(AU)

Restless legs syndrome and periodic limb movements disorder in the pediatric population.

PURPOSE OF REVIEW: Restless legs syndrome (RLS) and periodic limb movements of sleep (PLMS) are common neurological disorders in childhood which are usually underdiagnosed. As many pediatricians and pediatric pulmonologists with interest in sleep medicine will be encountering children with RLS and PLMS in their practice, we feel a comprehensive review of these disorders from a pediatric perspective would be very important in understanding basic pathophysiology, clinical features in early diagnosis, and effective management. RECENT FINDINGS: There has recently been increased awareness about these disorders in children, and the American Academy of Sleep Medicine has recently published pediatric specific diagnostic criteria. There have also been exciting discoveries into the basic pathophysiology of these conditions which have helped to better understand these disorders. Genetics plays a very important role in their development, and current management strategies have been very successful in treatment of these conditions. SUMMARY: RLS occurs in 1 out of 120 school-age children. In many children, diagnosis is not even suspected as these children present with atypical symptoms and associated comorbid conditions. In this review, we will discuss RLS and PLMS in the pediatric population and briefly review their pathophysiology, clinical presentation, risk factors, neurobehavioral consequences, and newer diagnostic criteria along with recent advances in their management.

Effects of the use of MIG3 bioceramics fabrics use - long infrared emitter - in pain, intolerance to cold and periodic limb movements in post-polio syndrome / Efeitos do uso de tecidos com biocerâmica MIG3 - emissora de infravermelho longo - na dor, intolerância ao frio e movimentos periódicos dos membros na síndrome pós-poliomielite

The main post-polio syndrome (PPS) symptoms are new-onset weakness, new-onset atrophy, fatigue, cold intolerance, and pain associated with sleep disturbances. The polysomnographic study is the gold pattern to analyze sleep disorders. OBJECTIVE: To assess pain, intolerance to cold and periodic limb movements (PLM) index before and after the use of MIG3 bioceramic fabrics over 4 weeks. METHOD: 12 patients with PPS from UNIFESP/EPM. All patients were submitted to polysomnography and infra-red examinations with answered scales of pain and intolerance to cold before and after the use of MIG3 bioceramics fabrics. RESULTS: There were significant decreases in pain and PLM index. CONCLUSION: MIG3 bioceramic fabrics can help in the treatment of pain and PLM in PPS patients.

Dentre as manifestações clínicas da SPP destacam-se nova fraqueza, fadiga, dor, intolerância ao frio, nova atrofia e transtornos do sono. A polissonografia de noite inteira permanece sendo padrão ouro para análise do sono e diagnóstico de transtornos do sono. OBJETIVO: Avaliar o comportamento da dor, intolerância ao frio (IF) e índice de movimentos periódicos de membros (PLMs) após uso de colchonete e roupas com biocerâmica MIG3 por 4 semanas. MÉTODO: 12 pacientes com SPP, da UNIFESP/EPM. Todos realizaram exames de polissonografia e infravermelho e responderam a questionários de dor e IF antes e após o uso dos materiais. RESULTADOS: Houve diminuição significativa da dor e dos PLMs. CONCLUSÃO: Os tecidos com biocerâmica MIG3 podem ser um coadjuvante ao tratamento da dor e dos PLMs nos pacientes com SPP.