Seminars in Pediatric Neurology

Clinical pharmacists are a part of the integrated health care team and provide valuable input on medication management for patients with acute and chronic disease states. Using epilepsy as a model, pharmacist involvement in patient care has been associated with significant reductions in monthly seizure frequency. Given differences in etiology, pediatric patients with epilepsy are likely to have higher number of treatments, with additional pharmacodynamic and pharmacokinetic differences, adding to the importance of utilizing a pediatric clinical pharmacist practitioner with neuropharmacology expertise...

Acute neuromuscular disorders occasionally occur in the Pediatric Neurologic Intensive Care Unit. Many of these are primary disorders of the motor unit that may present acutely or exacerbate during an intercurrent illness. Additionally, acute neuromuscular disorders may develop during an acute systemic illness requiring intensive care management that predispose the child to another set of acute motor unit disorders. This chapter discusses acute neuromuscular crises in the infant, toddler, and adolescent, as well as neuromuscular disorders resulting from critical illness...

Management of pediatric spinal cord injury (SCI) is an essential skill for all pediatric neurocritical care physicians. In this review, we focus on the evaluation and management of pediatric SCI, highlight a novel framework for the monitoring of such patients in the intensive care unit (ICU), and introduce advancements in critical care techniques in monitoring and management. The initial evaluation and characterization of SCI is crucial for improving outcomes as well as prognostication. While physical examination and imaging are the main stays of the work-up, we propose the use of somatosensory evoked potentials (SSEPs) and transcranial magnetic stimulation (TMS) for challenging clinical scenarios...

Children admitted to neurocritical care units often experience new neurodevelopmental disabilities due to both their acquired neurologic injuries and deconditioning from prolonged hospitalizations. Rehabilitation for critically ill children is multifactorial and begins in the intensive care unit itself. The goals of rehabilitation include prevention of complications associated with immobilization and evolving tone, comprehensive evaluation and treatment of functional deficits, and implementation of adaptive strategies with the goal of maximizing recovery...

Managing children with critical neurological conditions requires a comprehensive understanding of several principles of critical care. Providing a holistic approach that addresses not only the acute interactions between the brain and different organ systems, but also critical illness-associated complications and recovery is essential for improving outcomes in these patients. The brain reacts to an insult with autonomic responses designed to optimize cardiac output and perfusion, which can paradoxically be detrimental...

No abstract text is available yet for this article.

Inflammatory disorders of the central nervous system (CNS) include a wide spectrum of autoimmune, autoinflammatory, and paraneoplastic diseases. While many affected patients require acute hospital admission, a subset may present with severe neurological symptoms requiring intensive care unit (ICU) escalation due to disordered consciousness, respiratory failure, status epilepticus, intracranial hypertension, and/or severe autonomic dysregulation.

Neuromonitoring is used to assess the central nervous system in the intensive care unit. The purpose of neuromonitoring is to detect neurologic deterioration and intervene to prevent irreversible nervous system dysfunction. Neuromonitoring starts with the standard neurologic examination, which may lag behind the pathophysiologic changes. Additional modalities including continuous electroencephalography (CEEG), multiple physiologic parameters, and structural neuroimaging may detect changes earlier. Multimodal neuromonitoring now refers to an integrated combination and display of non-invasive and invasive modalities, permitting tailored treatment for the individual patient...

The post-intensive care syndrome (PICS) concept whereby the ICU experience of the patient as well as their family can have long-term deleterious health outcomes in both the patient and the family provides a rationale and impetus for modifying the ICU experience for the parents of patients receiving pediatric neurocritical care. This article uses the PICS framework to provide insight to that parental experience. Included are the words of parents who tell what they felt and what they most needed from their children's doctors while their children were receiving neurocritical care...

Medical and surgical advancements have improved survival in children with acquired and congenital heart disease (CHD), but the burden of neurological morbidity is high. Brain disorders associated with CHD include white matter injury, stroke, seizure, and neurodevelopmental delays. While genetics and disease-specific factors play a substantial role in early brain injury, therapeutic management of the heart disease intensifies the risk. There is a growing interest in understanding how to reduce brain injury and improve neurodevelopmental outcomes in cardiac diseases...

No abstract text is available yet for this article.

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...

While sleepiness is common among children, and particularly adolescents, profound sleepiness in the setting of apparently adequate sleep should prompt consideration of a central disorder of hypersomnolence. These disorders, which include narcolepsy, idiopathic hypersomnia, Kleine-Levin syndrome, and others, are likely underrecognized in the pediatric population. Narcolepsy in particular should be of interest to child neurologists as the unique signs and symptoms of this disease often prompt evaluation in pediatric neurology clinics...

Sleep disordered breathing (SDB) is prevalent among children with neuromuscular disorders (NMD). The combination of respiratory muscle weakness, altered drive, and chest wall distortion due to scoliosis make sleep a stressful state in this population. Symptomatology can range from absent to snoring, nocturnal awakenings, morning headaches, and excessive daytime sleepiness. Sequelae of untreated SDB includes cardiovascular effects, metabolic derangements, and neurocognitive concerns which can be compounded by those innate to the NMD...

Circadian Rhythm Sleep-Wake Disorders (CRSWDs) are important sleep disorders whose unifying feature is a mismatch between the preferred or required times for sleep and wakefulness and the endogenous circadian drives for these. Their etiology, presentation, and treatment can be different in pediatric patients as compared to adults. Evaluation of these disorders must be performed while viewed through the lens of a patient's comorbid conditions. Newer methods of assessment promise to provide greater diagnostic clarity and critical insights into how circadian physiology affects overall health and disease states...

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 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...

Sleep problems are widespread in children and adolescents suffering from chronic pain disorders. Sleep loss intensifies the experience of pain and is detrimental to the budding self-efficacy of a young individual with limitless horizons. Addressing sleep disorders may prevent the chronification of pain and prevent adverse health outcomes, such as functional impairment, psychiatric comorbidities and overall poor quality of life. This review will explore the cyclical nature between sleep, pain and mood, as well as the functional impact of this relationship on children and adolescents...

Daytime sleepiness is common amongst children and adolescents. Inadequate sleep duration, inappropriate school start times, and the delay in sleep phase of adolescence may all contribute. Nocturnal sleep disruption due to sleep disorders such as obstructive sleep apnea or restless legs syndrome/periodic limb movement disorder may also lead to daytime sleepiness. Profound sleepiness however, when occurring in the setting of adequate sleep duration, is rare amongst children and adolescents and may prompt consideration of a central disorder of hypersomnolence (CDH)...

The pediatric intensive care unit (PICU) is bright, loud, and disruptive to children. Strategies to improve the sleep of adults in the ICU have improved delirium and mortality rates. Children need more sleep than adults for active growth, healing, and development when well; this is likely true when they are critically ill. This review was performed to describe what we know in this area to date with the intent to identify future directions for research in this field. Since the 1990s, 16 articles on 14 observational trials have been published investigating the sleep on a total of 312 critically ill children and the melatonin levels of an additional 144...

Gregory L. Holmes Seminars in Pediatric Neurology Volume 23, Issue 2, May 2016, Pages 120-126 Epilepsy is a complex disorder, which involves much more than seizures, encompassing a range of associated comorbid health conditions that can have significant health and quality-of-life implications. Of these comorbidities, cognitive impairment is one of the most common and distressing aspects of epilepsy. Clinical studies have demonstrated that refractory seizures, resistant to antiepileptic drugs, occurring early in life have significant adverse effects on cognitive function...