{"title":"Diagnosis and Treatment of Disorders of Sleep in Children: Current Concepts","authors":"Thomas J. Dye","doi":"10.1016/j.spen.2023.101102","DOIUrl":"10.1016/j.spen.2023.101102","url":null,"abstract":"","PeriodicalId":49284,"journal":{"name":"Seminars in Pediatric Neurology","volume":"48 ","pages":"Article 101102"},"PeriodicalIF":2.7,"publicationDate":"2023-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"136010077","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
John E Pascoe , Alexander Zygmunt , Zarmina Ehsan , Neepa Gurbani
{"title":"Sleep in pediatric neuromuscular disorders","authors":"John E Pascoe , Alexander Zygmunt , Zarmina Ehsan , Neepa Gurbani","doi":"10.1016/j.spen.2023.101092","DOIUrl":"10.1016/j.spen.2023.101092","url":null,"abstract":"<div><p>Sleep disordered breathing<span><span> (SDB) is prevalent among children with neuromuscular disorders<span> (NMD). The combination of respiratory muscle weakness, altered drive, and chest wall distortion due to </span></span>scoliosis<span> make sleep a stressful state in this population. Symptomatology<span><span><span><span><span> can range from absent to snoring, nocturnal awakenings, morning headaches, and excessive daytime sleepiness. </span>Sequelae of untreated SDB includes </span>cardiovascular effects, metabolic derangements, and neurocognitive concerns which can be compounded by those innate to the NMD. The clinician should have a low threshold for obtaining </span>polysomnography and recognize the nuances of individual disorders due to disproportionately impacted muscle groups such as </span>hypoventilation<span> in ambulating patients from diaphragm weakness. Non-invasive or invasive ventilation<span> are the mainstay of treatment<span>. In this review we explore the diagnosis and treatment of SDB in children with various NMD.</span></span></span></span></span></span></p></div>","PeriodicalId":49284,"journal":{"name":"Seminars in Pediatric Neurology","volume":"48 ","pages":"Article 101092"},"PeriodicalIF":2.7,"publicationDate":"2023-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"135705983","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Sleep and Epilepsy","authors":"Wei K. Liu , Sanjeev Kothare , Sejal Jain","doi":"10.1016/j.spen.2023.101087","DOIUrl":"10.1016/j.spen.2023.101087","url":null,"abstract":"<div><p>The relationship between sleep and epilepsy is both intimate and bidirectional. The molecular mechanisms which control circadian rhythm and the sleep/wake cycle are dysregulated in epileptogenic tissue and are themselves effected by molecular pathways for epilepsy. Sleep affects the frequency of interictal epileptiform discharges<span><span> and recent research has raised new questions regarding the impact of discharges on sleep function and cognition. Epileptiform discharges themselves affect sleep architecture and increase the risk of sleep disorders. Several sleep-related epilepsy syndromes have undergone changes in their classification which highlights their intimate relationship to sleep and novel screening tools have been developed to help clinicians better differentiate </span>epileptic seizures from sleep-related paroxysmal events. Improving sleep and addressing sleep disorders has been associated with improved seizure control and increased well-being in people with epilepsy. These interactions are discussed in detail in this review.</span></p></div>","PeriodicalId":49284,"journal":{"name":"Seminars in Pediatric Neurology","volume":"48 ","pages":"Article 101087"},"PeriodicalIF":2.7,"publicationDate":"2023-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"135889112","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Sleep and Circadian Disturbances in Children With Neurodevelopmental Disorders","authors":"Sharon Tamir , Thomas J. Dye , Rochelle M. Witt","doi":"10.1016/j.spen.2023.101090","DOIUrl":"10.1016/j.spen.2023.101090","url":null,"abstract":"<div><p>Sleep problems are highly prevalent in those with neurodevelopmental disorders (NDDs). We propose this is secondary to multiple factors that directly and indirectly negatively impact sleep and circadian processes in those with NDDs, which in turn, further perturbs development, resulting in a “developmental and sleep/circadian-related encephalopathy.” In this review, we discuss select NDDs with known or suspected sleep and circadian phenotypes. We also highlight important considerations when evaluating and treating sleep and circadian disorders in these populations.</p></div>","PeriodicalId":49284,"journal":{"name":"Seminars in Pediatric Neurology","volume":"48 ","pages":"Article 101090"},"PeriodicalIF":2.7,"publicationDate":"2023-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S1071909123000591/pdfft?md5=1b38926f948cc54b953f6612dfcb2b66&pid=1-s2.0-S1071909123000591-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"136117644","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"If the Patient Does Not Take the Medication, It Will Not Work","authors":"Iván Sánchez Fernández , Adriana Ulate-Campos","doi":"10.1016/j.spen.2023.101079","DOIUrl":"10.1016/j.spen.2023.101079","url":null,"abstract":"<div><h3>Cognitive and Behavioral Comorbidities: An Unwanted Effect of Antiepileptic Drugs in Children</h3><p>Adriana Ulate-Campos, Iván Sánchez Fernández Seminars in Pediatric Neurology Volume 24, Issue 4, November 2017, Pages 320–330</p><p>Epilepsy is one of the most common neurological disorders and, despite optimally chosen and dosed antiepileptic drugs (AEDs), approximately 20%-30% of patients will continue to have seizures. Behavior and cognition are negatively impacted by seizures, but AEDs are also a major contributor to behavioral and cognitive deficits. However, the cognitive and behavioral effect of AEDs in children is insufficiently emphasized in the literature. This review summarizes the cognitive and behavioral effects of AEDs in the pediatric population with the objective of helping pediatricians and pediatric neurologists to select the AEDs with the best profile for their individual patient's needs.</p></div>","PeriodicalId":49284,"journal":{"name":"Seminars in Pediatric Neurology","volume":"47 ","pages":"Article 101079"},"PeriodicalIF":2.7,"publicationDate":"2023-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"48076459","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Contemporary Understanding of the Central Autonomic Nervous System in Fetal-Neonatal Transition","authors":"Sarah B. Mulkey","doi":"10.1016/j.spen.2023.101081","DOIUrl":"10.1016/j.spen.2023.101081","url":null,"abstract":"<div><h3>The Critical Role of the Central Autonomic Nervous System in Fetal-Neonatal Transition</h3><p>Sarah B. Mulkey, Adre dú Plessis Seminars in Pediatric Neurology Volume 28, December 2018, Pages 29–37</p><p>The objective of this article is to understand the complex role of the central autonomic nervous system in normal and complicated fetal-neonatal transition and how autonomic nervous system dysfunction can lead to brain injury. The central autonomic nervous system supports coordinated fetal transitional cardiovascular, respiratory, and endocrine responses to provide safe transition of the fetus at delivery. Fetal and maternal medical and environmental exposures can disrupt normal maturation of the autonomic nervous system in utero, cause dysfunction, and complicate fetal-neonatal transition. Brain injury may both be caused by autonomic nervous system failure and contribute directly to autonomic nervous system dysfunction in the fetus and newborn. The central autonomic nervous system has multiple roles in supporting transition of the fetus. Future studies should aim to improve real-time monitoring of fetal autonomic nervous system function and in supporting typical autonomic nervous system development even under complicated conditions.</p></div>","PeriodicalId":49284,"journal":{"name":"Seminars in Pediatric Neurology","volume":"47 ","pages":"Article 101081"},"PeriodicalIF":2.7,"publicationDate":"2023-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"71428344","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Epilepsy Impacts Families and Communities: Persistent Gaps and Inequities","authors":"Adam P. Ostendorf","doi":"10.1016/j.spen.2023.101084","DOIUrl":"10.1016/j.spen.2023.101084","url":null,"abstract":"<div><h3>Effect of Epilepsy on Families, Communities, and Society</h3><p>Adam P. Ostendorf , Satyanarayana Gedela Seminars in Pediatric Neurology Volume 24, Issue 4, November 2017, Pages 340–347</p><p>The effect of epilepsy extends beyond those with the diagnosis and impacts families, communities and society. Caregiver and sibling quality of life is often negatively affected by frequent seizures, comorbid behavioral and sleep disorders and stigma surrounding the diagnosis. Furthermore, the negative effects can be magnified by individual coping styles and resources available to families of those with epilepsy. Beyond the family and immediate caregivers, epilepsy affects local communities by drawing additional resources from education systems. The direct costs of caring for an individual with epilepsy and the indirect costs associated with decreased productivity place financial strain on individuals and health care systems throughout the world. This review details factors affecting family and caregiver quality of life and provides several approaches through which health care providers may address these concerns. Furthermore, we examine the financial effect of epilepsy on society and review emerging strategies to lessen health care use for individuals with epilepsy.</p></div>","PeriodicalId":49284,"journal":{"name":"Seminars in Pediatric Neurology","volume":"47 ","pages":"Article 101084"},"PeriodicalIF":2.7,"publicationDate":"2023-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"71428345","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Christopher A. Lear , Austin Ugwumadu , Laura Bennet , Alistair J. Gunn
{"title":"An Update of Our Understanding of Fetal Heart Rate Patterns in Health and Disease","authors":"Christopher A. Lear , Austin Ugwumadu , Laura Bennet , Alistair J. Gunn","doi":"10.1016/j.spen.2023.101072","DOIUrl":"10.1016/j.spen.2023.101072","url":null,"abstract":"<div><h3>Understanding Fetal Heart Rate Patterns That May Predict Antenatal and Intrapartum Neural Injury</h3><p>Christopher A. Lear, Jenny A. Westgate, Austin Ugwumadu, Jan G. Nijhuis, Peter R. Stone, Antoniya Georgieva, Tomoaki Ikeda, Guido Wassink , Laura Bennet , Alistair J. Gunn Seminars in Pediatric Neurology Volume 28, December 2018, Pages 3–16</p><p>Electronic fetal heart rate (FHR) monitoring is widely used to assess fetal well-being throughout pregnancy and labor. Both antenatal and intrapartum FHR monitoring are associated with a high negative predictive value and a very poor positive predictive value. This in part reflects the physiological resilience of the healthy fetus and the remarkable effectiveness of fetal adaptations to even severe challenges. In this way, the majority of “abnormal” FHR patterns in fact reflect a fetus' appropriate adaptive responses to adverse in utero conditions. Understanding the physiology of these adaptations, how they are reflected in the FHR trace and in what conditions they can fail is therefore critical to appreciating both the potential uses and limitations of electronic FHR monitoring.</p></div>","PeriodicalId":49284,"journal":{"name":"Seminars in Pediatric Neurology","volume":"47 ","pages":"Article 101072"},"PeriodicalIF":2.7,"publicationDate":"2023-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"45613992","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Diagnosis and Management of Tuberculous Meningitis in Children- an Update","authors":"Ronald van Toorn, Regan Solomons","doi":"10.1016/j.spen.2023.101071","DOIUrl":"10.1016/j.spen.2023.101071","url":null,"abstract":"<div><h3>Update on the Diagnosis and Management of Tuberculous Meningitis in Children</h3><p>Ronald van Toorn, Regan Solomons Seminars in Pediatric Neurology Volume 21, Issue 1, March 2014, Pages 12–18</p><p>Tuberculous meningitis (TBM), the most devastating manifestation of tuberculosis, is often missed or overlooked because of nonspecific symptoms and difficulties in diagnosis. It continues to be an important cause of neurologic handicap in resource-poor countries. Owing to the suboptimal performance of diagnostic tests of TBM, diagnosis relies on thorough history, clinical examination, and relevant investigations. The development of affordable, accurate diagnostic tests for TBM in resource-poor settings remains a priority. Short intensified treatment is safe and effective in both human immunodeficiency virus (HIV)-infected and HIV-uninfected children. Treatment of tuberculous hydrocephalus depends on the level of the cerebrospinal fluid obstruction. Corticosteroids reduce risk of neurodisability and death in HIV-uninfected children. Thalidomide should be considered in children compromised by tuberculosis abscesses and tuberculous-related optochiasmic arachnoiditis. In resource-poor countries, home-based TBM treatment after initial in-hospital stabilization is feasible in carefully selected patients. Early diagnosis and treatment of TBM is the single most important factor determining outcome.</p></div>","PeriodicalId":49284,"journal":{"name":"Seminars in Pediatric Neurology","volume":"47 ","pages":"Article 101071"},"PeriodicalIF":2.7,"publicationDate":"2023-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"47237485","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Comment: Amenable Treatable Severe Pediatric Epilepsies","authors":"Phillip L. Pearl","doi":"10.1016/j.spen.2023.101073","DOIUrl":"10.1016/j.spen.2023.101073","url":null,"abstract":"<div><h3>Amenable Treatable Severe Pediatric Epilepsies</h3><p>Phillip L. Pearl Seminars in Pediatric Neurology Volume 23, Issue 2, May 2016, Pages 158–166</p><p>Vitamin-dependent epilepsies and multiple metabolic epilepsies are amenable to treatment that markedly improves the disease course. Knowledge of these amenably treatable severe pediatric epilepsies allows for early identification, testing, and treatment. These disorders present with various phenotypes, including early onset epileptic encephalopathy (refractory neonatal seizures, early myoclonic encephalopathy, and early infantile epileptic encephalop athy), infantile spasms, or mixed generalized seizure types in infancy, childhood, or even adolescence and adulthood. The disorders are presented as vitamin responsive epilepsies such as pyridoxine, pyridoxal-5-phosphate, folinic acid, and biotin; transportopathies like GLUT-1, cerebral folate deficiency, and biotin thiamine responsive disorder; amino and organic acidopathies including serine synthesis defects, creatine synthesis disorders, molybdenum cofactor deficiency, and cobalamin deficiencies; mitochondrial disorders; urea cycle disorders; neurotransmitter defects; and disorders of glucose homeostasis. In each case, targeted intervention directed toward the underlying metabolic pathophysiology affords for the opportunity to significantly effect the outcome and prognosis of an otherwise severe pediatric epilepsy.</p></div>","PeriodicalId":49284,"journal":{"name":"Seminars in Pediatric Neurology","volume":"47 ","pages":"Article 101073"},"PeriodicalIF":2.7,"publicationDate":"2023-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"48644992","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}