Suresh Kotagal

• Mayo Foundation for Medical Education and Research

Purposeof Review To provide an update on central hypersomnia disorders in children because they are distinct from those seen in adults. Recent Findings Despite its striking clinical features, there are delays in the diagnosis of narcolepsy, especially in pre-school age children. The delay in diagnosis and instituting timely and appropriate managem...

This article provides an overview of the unique features of normal sleep and breathing in infancy, as well as various conditions associated with disordered breathing in this age group. A practical, clinical approach is utilized for describing diagnostic assessment and management of the various entities.

Clinical neurophysiologic testing plays a critical role as a complement to the clinical assessment in patients who are being evaluated for a variety of neurologic symptoms. Many different techniques and methods of assessment can be used to evaluate the function of the nervous system, including electroencephalography, electromyography, evoked potent...

Introduction Pediatric Restless Legs Syndrome (RLS)/Periodic Limb Movement Disorder (PLMD) are treatable disorders affecting quality of life. The first line therapy is oral iron, which may have gastrointestinal side effects or suboptimal absorption. Consequently, parenteral iron preparations are needed, but have been insufficiently studied in child...

Introduction Moebius syndrome (MBS) is a rare disorder characterized by failure of development of the 6th and 7th cranial nerve nuclei located in the pons. Though the congenital lesion abuts the pontine tegmentum, sleep architecture has not been evaluated in MBS. Methods We report the clinical features of three children with MBS, and discuss impli...

Genetically determined or acquired sleep–wake disorders that have a significant autonomic component occur over a background of constantly maturing neurorespiratory and cardiovascular function. The pathophysiology of sudden infant death syndrome is now been better elucidated. The entity of brief resolved unexplained events (BRUE) now has helpful man...

This systematic review assessed the prevalence of restless sleep in children, documented the association of restless sleep with other conditions, and summarized the existing evidence regarding whether restless sleep should be considered a distinct sleep disorder. A comprehensive search of electronic databases was performed using the broad search te...

Objectives/Background Most middle-aged and older adult patients with isolated (idiopathic) REM sleep behavior disorder (RBD) eventually develop parkinsonism, dementia with Lewy bodies, or multiple system atrophy. We aimed to describe the current sleep medicine specialist approach toward RBD prognostic counseling, and to determine physician beliefs...

Background Restless sleep is a frequent complaint in clinical practice and has been reported in the medical literature since the 1970s. Most often, it has been described in association with specific sleep or medical conditions. However, more recently, publications have emerged that describe a disorder characterized by restless sleep as the core fea...

Objective To determine if Angelman syndrome patients with sleep complaints have an increased risk of iron deficiency, and if iron therapy improves their sleep difficulties. Background About two-thirds of Angelman syndrome patients experience sleep difficulties, which are likely multifactorial. Because iron deficiency can contribute toward restless...

This chapter covers a varied group of disorders that develop as a consequence of genetic abnormalities, prenatal or perinatal infections, ischemia, or uncertain etiology. All lead to abnormal development of the nervous system. Some disorders have been selected because of the challenge they pose in diagnosis and management, and others because they i...

Objectives/background: Prognostic counseling about the risk for developing overt neurodegenerative disorders for patients with idiopathic REM sleep-behavior disorder (iRBD) and isolated REM sleep without atonia (iRSWA) is difficult, given lack of disease-modifying interventions and uncertainty in accurate prognostication for individuals. We aimed...

While REM sleep behavior disorder (RBD) is typically observed among older adults, with characteristic clinical and polysomnographic features and risk for future neurodegenerative disease in the majority, it has also been reported among children and adolescents. The clinical presentation, associated conditions, and clinical course have not been stud...

Treatment options may be limited for infants with obstructive sleep apnea when there is no surgically correctable upper airway lesion. We therefore evaluated, retrospectively, the efficacy of low-flow oxygen as a therapeutic option for infant obstructive sleep apnea. We reviewed the medical charts of 23 infants who had undergone a therapeutic trial...

Study Objective To assess comorbidities in a community-based cohort of narcolepsy. Methods A 2000-2014 community-based narcolepsy cohort was identified in Olmsted County, Minnesota. Records were reviewed by a certified sleep specialist for accuracy of diagnosis and comorbidities were extracted and analyzed. Comorbidities in narcolepsy subjects, bo...

Obesity in children is associated with several co-morbidities including dyslipidemia. Obstructive sleep apnea (OSA) is commonly seen in obese children. In adults, diagnosis of OSA independent of obesity is associated with cardiometabolic risk factors including dyslipidemia. There is limited data on the impact of treatment of OSA on lipids in childr...

The comprehensive management of chronic disorders such as hypersomnias of childhood requires combining life-style changes with rational pharmacotherapy that is based on treating the symptoms that are most bothersome, the age, comorbidities, and metabolic and endocrine status of the patient. The excessive sleepiness of narcolepsy and idiopathic hype...

Evidence-based clinical practice guidelines and policy statements from the AAP! Clinical practice guidelines have long provided physicians with an evidence-based decision-making tool for managing common pediatric conditions. Policies issued and endorsed by the American Academy of Pediatrics (AAP) represent the AAP position on child health care issu...

Individuals with mental health and/or neurodevelopmental conditions often display disruptive day- and night-time behaviours associated with abnormally increased muscular activity and/or presentations described as hyperkinesia, hypermotor-restlessness and hyper-/hypo-arousability (all summarized as H-behaviours). H-behaviours are used as a diagnosti...

Members of the American Academy of Sleep Medicine developed consensus recommendations for the amount of sleep needed to promote optimal health in children and adolescents using a modified RAND Appropriateness Method. After review of 864 published articles, the following sleep durations are recommended: Infants 4 months to 12 months should sleep 12...

Sleep is essential for optimal health in children and adolescents. Members of the American Academy of Sleep Medicine developed consensus recommendations for the amount of sleep needed to promote optimal health in children and adolescents using a modified RAND Appropriateness Method. The recommendations are summarized here. A manuscript detailing th...

Background: This article describes the use of complementary and alternative medicines in an outpatient pediatric neurology clinic, and assesses family attitudes toward the efficacy of complementary and alternative medicines versus prescription medications. Complementary and alternative medicine is an important element of the modern health care lan...

Establishing a diagnosis of narcolepsy can be challenging in children given the variability in clinical presentation, the limited descriptive ability of the child, and the reliability of the parents as historians. In the preschool-age child, it may be hard to distinguish physiologic napping from pathologic sleepiness. In school-age children, the co...

Editorial—Kotagal Idiopathic narcolepsy generally has onset during childhood and adolescence, though there may be a delay of several years before a firm diagnosis is established. In the United Kingdom, the time between onset of clinical symptoms and establishment of a definitive diagnosis of narcolepsy was reported to be 10.5 years. 1 Children are...

There is a close relationship between sleep and childhood neurodevelopmental/neurodegenerative disorders. Understanding the sleep issues may provide greater insight into pathophysiology and treatment of these disorders. Copyright © 2015 Elsevier Inc. All rights reserved.

Rapid eye movement (REM) sleep behavior disorder (RBD) is a type of parasomnia that arises out of REM sleep and is characterized by aggressive or violent motor dream enactment in conjunction with preservation of tonic electromyographic activity (ie, REM sleep without atonia). RBD occurs at all ages and in both sexes, although it remains relatively...

Background Although poor sleep is common in numerous gastrointestinal diseases, data are scarce on the risk of poor sleep in celiac disease. The objective of this study was to estimate the risk of repeated use of hypnotics among individuals with celiac disease as a proxy measure for poor sleep.Methods This is a nationwide case¿control study includi...

Study objective: The clinical characteristics of obstructive sleep apnea (OSA) in infants have been insufficiently characterized. Our aim was to describe identifiable comorbidities in infants with obstructive sleep apnea, which may assist in recognizing these patients earlier in their disease course and help improve management. Methods: This was...

Introduction Growing evidence suggests that idiopathic REM sleep behavior disorder (RBD) may be a forme fruste of synucleinopathy neurodegeneration in older adults. The clinical significance of REM sleep without atonia (RSWA) and overt RBD in children and adolescents remains unclear. Furthermore, the lower age bound for occurrence of RSWA, the neur...

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 u...

An alternative treatment approach is needed for children who cannot tolerate oral iron preparations or when there is a need for rapid replenishment of iron stores. We report on the safety, adverse effects, and efficacy of intravenous iron sucrose in a retrospective sample of children with restless legs syndrome (RLS) or periodic limb movement disor...

a b s t r a c t Objective: To evaluate nocturnal polysomnogram findings in children with suspected symptomatic Chiari type I malformation, correlate them with clinical and magnetic resonance imaging data and to determine if this information has value in clinical decision making process. Methods: A retrospective review identified 24 children with ty...

Cerebral venous sinus thrombosis (CVST) is an uncommon but recognized complication of treatment for leukemia. Our goal was to determine the long-term neurocognitive outcomes in childhood cancer survivors who had CVST during therapy. Nine patients were identified from an institutional database. All had experienced CVST in the setting of L-asparagina...

Sleep-wake problems are common during childhood and adolescence. They are of diverse cause, and can contribute significantly to alterations in behavior, cognition, and learning. Obstructive sleep apnea, central hypoventilation syndrome, narcolepsy, periodic hypersomnia, delayed sleep phase syndrome, restless legs syndrome, parasomnias, and sleep di...

This evidence-based review provides a systematic and comprehensive review of the literature regarding the utility of polysomnography for the evaluation of non-respiratory sleep disorders in children including hypersomnias, parasomnias, sleep-related movement disorders, and sleep in other special populations. A task force of pediatric sleep medicine...

Opinion statement: Dyssomnias are sleep disorders associated with complaints of insomnia or hypersomnia. The daytime sleepiness of narcolepsy is treated by a combination of planned daytime naps, regular exercise medications such as modafinil, or salts of methylphenidate, or amphetamine. Cataplexy that accompanies narcolepsy is treated with anticho...

Daytime sleepiness is a common symptom during childhood and adolescence. It may be related to a variety of disorders, such as inadequate sleep hygiene, narcolepsy, sleep-disordered breathing, and circadian rhythm disorders. Consequent daytime sleepiness can lead to impaired mood and cognition and slowing of motor reaction time, with increased prope...

To describe our experience regarding the clinical and polysomnographic features of REM sleep behavior disorder (RBD) in childhood. This was a retrospective chart review of children and adolescents with RBD and REM sleep without atonia. Demographics, and clinical and polysomnographic information were tabulated. Our findings were compared with those...

To evaluate the efficacy and side effect profile of sodium oxybate in the treatment for narcolepsy-cataplexy in the pediatric age group. A retrospective study was conducted on 15 children and adolescents with narcolepsy-cataplexy who had been treated with sodium oxybate. The mean age at diagnosis of narcolepsy was 11 years (range 3-17 years). Subje...

The aim of this study was to describe the polysomnographic features of childhood‐onset narcolepsy. A retrospective review was performed on children with narcolepsy. The polysomnogram findings were compared with reference values obtained from normative data in the general population. Narcolepsy subjects had a mean initial sleep latency of 10.9 min o...

Children and adolescents with restless legs syndrome (RLS) are commonly diagnosed with comorbid attention deficit hyperactivity disorder and behavioral disturbances. Uncertainty exists over the significance of other co-occurring psychiatric disorders and their pharmacologic management in children with RLS. The purpose of this study was to determine...

Chapter 26 provides an overview of common, emergent conditions of sleep problems in children that may be encountered by sleep clinician.

This resource provides information for the management of disorders occurring during sleep and brings greater awareness to the treatment of sleep disorders, as well as treatments of neurological, medical, and psychiatric disorders.

Co-morbid sleep disorders are quite common in Child Neurology. Formal training in the field of sleep medicine and routine attention to sleep-wake function in clinical practice enhances the ability of the child neurologist to deliver comprehensive care.

Opinion statement: An array of surgical, medical and orthodontic treatments is available for treating childhood obstructive sleep apnea. Adenotonsillectomy remains the first choice in treatment, with a need for subsequent clinical and polysomnographic reassessment in selected cases to determine residual sleep-disordered breathing. Residual obstruc...

Establishing a diagnosis of narcolepsy can be challenging in children, given the non-specific initial symptoms, limited descriptive ability of the child, and variability in reliability of the parents as historians. In the pre-school-age child, it may be hard to distinguish physiologic napping from pathologic sleepiness. In school age children, the...

Encephalitis associated with antibodies against the NMDA receptor (anti-NMDA-R) is characterized by prominent psychiatric symptoms, dyskinesias, seizures, autonomic instability, and central hypoventilation.1 We report a 20-month-old boy with oral-buccal dyskinesia, choreoathetosis, seizures, and encephalopathy, consistent with anti-NMDA-R encephali...

Patients being evaluated in child psychiatry clinics for behavior and mood disturbances frequently exhibit daytime sleepiness. Conversely, patients being evaluated for hypersomnia by sleep specialists may have depressed mood or hyperactive and aggressive behavior. The etiology of daytime sleepiness in children and adolescents is diverse and include...

Common childhood parasomnias, including those occurring at sleep onset and during rapid eye movement sleep or non-rapid eye movement sleep and their ontogeny are discussed. The events may be distressing to both the patient and family members. Stereotypic movements characteristic of some parasomnias most likely arise from disinhibition of subcortica...

To enhance the ability of the practitioner to diagnose and manage children with parasomnias in the office setting. Over 80% of preschool-age children experience parasomnia events. Instability in the regulation of sleep continuity might underlie sleep walking, sleep terrors, and confusional arousals. Catathernia or nocturnal groaning is a parasomnia...

We describe a 15-year-old young man with mental retardation, epilepsy, drop attacks, and dystonic gait since early childhood. The intrauterine and perinatal course was unremarkable. The drop attacks were characterized by a sudden loss of lower-extremity tone without impairment of consciousness and were triggered by the process of dressing. He would...

Associations between obstructive sleep apnea and motor speech disorders in adults have been suggested, though little has been written about possible effects of sleep apnea on speech acquisition in children with motor speech disorders. This report details the medical and speech history of a nonverbal child with seizures and severe apraxia of speech....

The potential causes of acquired macroglossia are extensive. The authors report two cases of subacute marked tongue swelling resulting in airway compromise in patients with refractory status epilepticus requiring prolonged pentobarbital coma. The hospitalization histories of the reported patients were retrospectively reviewed. The tongue swelling c...

Disturbances of sleep-wake function are common in premature infants and in those who have cerebral palsy, Down syndrome, Prader Willi syndrome, Chiari malformations, neuromuscular disorders, epilepsy, blindness, sickle cell anemia, and other disabilities. Recognizing and treating the underlying sleep disorder may enhance the quality of life in thes...

The term “cherry red spot” was used in 1887 by Bernard Sachs in his paper on “arrested development with special reference to its cortical pathology,” in describing the fundus of a child with “amaurotic familial idiocy.” The patient was confirmed on neuropathologic examination to have lipid storage in the brain [1]. Bernard Sachs acknowledged that h...

To evaluate the efficacy and side-effect profile of off-label sodium oxybate (gamma hydroxy butyrate) therapy in severe childhood narcolepsy-cataplexy. Retrospective; chart review. A multidisciplinary tertiary sleep center. A group of eight children with severe narcolepsy-cataplexy diagnosed on the basis of clinical history, nocturnal polysomnograp...

A recent telephone survey of 1125 adolescents aged 15-18 from France, Great Britain, Germany, and Italy found that about 20% were sleepy in the daytime, 25% had insomnia symptoms, and 4% met established criteria for an insomnia disorder. 1 2 Sleep-wake disorders are common and have an important impact on the quality of life of children, but if they...

General Concepts Sleep–Wake Function in Specific Neurological Disorders The Relationship between Sleep and Epilepsy References

Neonatal hyperammonemia secondary to X-linked ornithine transcarbamylase (OTC) deficiency carries a high risk of morbidity and mortality. Results of medical therapy are less than satisfactory. Experience with liver transplantation in very young affected infants is limited. We report a male newborn with severe OTC deficiency who underwent successful...

The clinical characteristics of childhood-onset restless legs syndrome are described. Thirty-two of 538 subjects (5.9%) examined in our sleep disorders center received diagnoses of restless legs syndrome. They were classified based on published criteria into probable (n = 9/32 or 28%) and definite (n = 23/32 or 78%) categories. Apart from an earlie...

While there have been anecdotal observations of binge eating in childhood-onset narcolepsy, the possible relationship between increased weight gain and childhood-onset narcolepsy has not been evaluated. A retrospective, case-control design was used to compare the body mass index (BMI) of 31 narcolepsy children at the time of diagnosis with that of...

The nature of sleep-related breathing problems varies with age. Ventilatory function may be compromised at the level of the brainstem, the autonomic nerves, or the upper airway musculature. These disorders are state-dependent, being impacted by both rapid eye movement and non-rapid eye movement sleep, and also by the degree of maturation of the ner...

Childhood sleep-wake disorders are common and associated with significant impairment of quality of life. The recent discovery of hypocretin deficiency as the pathophysiologic basis for narcolepsy-cataplexy is likely to spur the development of hypocretin analogs for definitive treatment [82,83]. The link between disrupted sleep and daytime learning...

To determine clinicians' patterns of use of the International Classification of Sleep Disorders (ICSD) and their ratings of its utility and organization. A telephone survey was conducted in one clinician practicing at each of 206 sleep centers accredited by the American Academy of Sleep Medicine (AASM). Descriptive analyses were performed, and rati...

This chapter covers a varied group of disorders of inherited, postinfectious, ischemic, or undetermined etiology All lead to abnormal development of the nervous system. Some disorders have been selected because of the challenge they pose in diagnosis and management, and others because they illustrate important concepts in neuroembryology or pathoph...

Various paraneoplastic autoantibodies have been linked to discrete neurologic syndromes and tumors in adults, but little is known about their incidence in children. We report a cross-sectional study of known paraneoplastic antibodies in 59 children with opsoclonus-myoclonus-ataxia, 86% of whom were moderately or severely symptomatic, and 68% of who...

Polyneuropathies are relatively uncommon in early infancy and the majority of affected children are found to have hypomyelinating neuropathies. Axonal sensorimotor neuropathies have been described in childhood but the majority of affected children present at or after 6 months of age, have nonprogressive courses, and achieve the ability to walk, alb...

Autonomic cardiorespiratory control changes with sleep-wake states and is influenced by sleep-related breathing disorders. Power spectrum (PS) analysis of instantaneous fluctuations in heart rate (HR) is used to investigate the role of the autonomic nervous system (ANS) in cardiorespiratory control. The two spectral regions of interest are the low...

The clinical course of a 6-year-old boy with adrenoleukodystrophy (ALD) who underwent allogeneic stem-cell transplantation during an early clinical stage is described. Twenty-three months after transplant, he remains neurologically stable, but with moderate neurological sequelae; the serum very long chain fatty acid profile has improved, but not no...

We report successful bone marrow transplantation in a child with a severe form of α-mannosidosis, type I. There was complete resolution of the recurrent sinopulmonary disease and organomegaly, improvement in the bony disease, and stabilization of neurocognitive function. (J Pediatr 1998;133: 282-5)

This article on school age children reviews relevant issues in sleep physiology, the classification of sleep disorders, their clinical and laboratory assessment, some common sleep disorders, the sleep-epilepsy relationship, as well as the impact of daytime sleepiness on higher cortical functions.

Among 145 patients treated with recombinant human growth hormone (GH), four devel oped sleep apnea (two obstructive, two mixed) associated with tonsillar and adenoidal hypertrophy in three. These four patients had no local risk factors predisposing to upper airway obstruction (i.e., frequent pharyngitis or sinusitis). Clinical and/or polysomnograph...

Included in the large group of children referred for attention deficit evaluations, several less common entities can be found. Careful history will enable the identification of these atypical cases of attention deficit and allow more preceise diagnosis and treatment.

Sarcomas (chondrosarcoma, Ewing sarcoma, rhabdomyosarcoma, and undifferentiated sarcoma) generally present with nonneurologic symptoms at the onset. Five children with sarcomas who presented with spinal cord compression or radiculopathy as their initial problems are described. These patients appear to be older than those with the more commonly enco...

Excessive daytime sleepiness is a disabling symptom of diverse etiologies. This article reviews the various, currently available diagnostic techniques that can be applied in the evaluation of childhood hypersomnolence, along with their merits and limitations. Normal polysomnographic data of children differ from those of adults. Serial studies may b...