Seminars in Pediatric Neurology

Neuromyelitis Optica Spectrum Disorder (NMOSD) is a demyelinating disease with a high relapse rate and risk of disability accrual. The condition is an astrocytopathy, with antibodies to the aquaporin-4 (AQP4) water channel being detected in AQP4-IgG seropositive disease. Presentation is uncommon in the pediatric age range, accounting for about 3%-5% of cases. NMOSD is more prevalent in populations of Black or East Asian ancestry. Core clinical syndromes include optic neuritis, acute myelitis, area postrema syndrome, acute brainstem syndrome, acute diencephalic syndrome, and symptomatic cerebral syndrome...

A structured health care transition is essential for adolescents with chronic disease to ensure continuity of care without treatment lapse. Though rare, multiple sclerosis is diagnosed in children and adolescents and these patients will eventually require transition to adult care in late adolescence and early adulthood. Some barriers to transition include limited independence of the adolescent, fear of an unknown adult care model, and difficulty ending close relationships with longstanding pediatric providers...

Pediatric multiple sclerosis (MS) accounts for 3%-10% of all patients diagnosed with MS. Complex interplay between environmental factors impacts the risk for MS and may also affect disease course. Many of these environmental factors are shared with adult-onset MS. However, children with MS are in closer temporal proximity to the biological onset of MS and have less confounding environmental exposures than their adult counterparts. Environmental factors that contribute to MS risk include: geographical latitude, viral exposures, obesity, vitamin deficiencies, smoking, air pollution, perinatal factors, gut microbiome, and diet...

Acquired demyelinating syndromes of the central nervous system are immune-mediated demyelinating disorders that can affect the brain, optic nerves, and spinal cord. These disorders have become increasingly recognized in children due to advances in imaging techniques, improvements in diagnostic testing, extensive research into understanding the pathophysiology underlying these disorders, and collaborative multi-institutional efforts to raise awareness of these disorders in children. Moreover, developments in the field of neuroimmunology have allowed us to identify autoantibodies that have presumed causal roles in acquired demyelinating syndromes...

Long-stay patients are an impactful, vulnerable, growing group of inpatients in today's (and tomorrow's) tertiary hospitals. They can outlast dozens of clinicians that necessarily rotate on and off clinical service. Yet, care from such rotating clinicians can result in fragmented care due to a lack of continuity that insufficiently meets the needs of these patients and their families. Using long-stay PICU patients as an example, this focused review discusses the impact of prolonged admissions and how our fragmented care can compound this impact...

The recent emergence of promising therapies to treat neuromuscular diseases such as spinal muscular atrophy raises important questions regarding the ethical permissibility of allowing a parent to refuse these Food and Drug Administration-approved drugs. The 3 most recent drugs targeting spinal muscular atrophy have all been approved since 2019, lack long-term data regarding potential side-effects and long-term benefits, and are costly. Indeed, onasemnogene abeparvovec-xioi (Zolgensma) has been called the most expensive drug in the world...

Since the concept of death by neurologic criteria (DNC) or "brain death" was articulated by the Harvard Ad Hoc Committee in 1968, efforts to establish and uphold DNC as equivalent to biologic death have been supported through federal and state legislation, professional guidelines, and hospital policies. Despite these endeavors, DNC remains controversial among bioethics scholars and clinicians and is not universally accepted by patient families and the public. In this focused review, we outline the current points of contention surrounding the diagnosis of DNC in pediatric patients...

The development of status myoclonus (SM) in a postcardiac arrest patient has historically been thought of as indicative of not only a poor neurologic outcome but of neurologic devastation. In many instances, this may lead clinicians to initiate conversations about withdrawal of life sustaining therapies (WLST) regardless of the time from return of spontaneous circulation (ROSC). Recent studies showing a percentage of patients may make a good recovery has called into question whether a self-fulfilling prophecy has developed where the concern for a poor neurologic outcome leads clinicians to prematurely discuss WLST...

No abstract text is available yet for this article.

The management of pediatric neurosurgical disease often requires families to choose between long-term disability and premature death. This decision-making is codified by informed consent. In practice, decision-making is heavily weighted toward intervening to prevent death, often with less consideration of the realities of long-term disability. We analyze long-term disability in pediatric neurosurgical disease from the perspectives of patients, families, and society. We then present a pragmatic framework and conversational approach for addressing informed consent discussions when the outcome is expected to be death or disability...

Advancements in genetic testing in the healthcare setting, most recently genomic sequencing, has enhanced our ability to diagnose genetic conditions. These advances include increased accessibility and affordability of genomic technologies. With expanded use comes the potential for significant ethical challenges for clinicians, particularly considering the implications of testing a child for one condition and incidentally finding a different condition or health risk. In this focused review, we address various ethical considerations from informed consent to the rights of a child undergoing genetic testing...

Pediatric transplant centers are faced with the difficult task of maximizing the benefit of organs donated for transplantation while also ensuring that all patients undergoing transplant evaluation are fairly considered for this life-saving therapy. Children with neurodevelopmental disabilities are a complex patient population that on occasion may face the need for a solid organ transplant. Several concerns exist regarding transplantation in this population, yet standard transplant inclusion and exclusion criteria do not exist...

The decisions around whether or not to provide tracheostomy and chronic mechanical ventilation to children with acute neurologic injury are difficult for medical providers and surrogate decision makers. Consideration of the 4 primary principles of medical ethics-autonomy, beneficence, non-maleficence, and justice-can provide a framework from which constructive discussions can form. Determination of the goals of care is a good first step in navigating these complex decisions. A shared decision model should be used, including education of decision-makers by medical providers and appropriate recommendations based on the stated goals of care...

Pediatric organ transplantation remains a life-saving therapy, with donated organs being absolutely scarce resources. Efforts to both increase pediatric organ donation authorization by families of children declared dead by neurologic criteria and mitigate perception of conflicts of interest have resulted in frequent exclusion of physicians from this process. This article provides of focused review of pediatric organ donation in the setting of brain death, explores the breadth of consequences of physician exclusion in donation authorization requests, and provides an ethical framework defending physician involvement in the organ donation process for this patient population...

Pediatric stroke is associated with a range of maladaptive cognitive and behavioral outcomes that often require targeted intervention. Despite increasing research on neuropsychological outcomes over the past decade, evidence for effective therapies and interventions for the most commonly reported cognitive and behavioral challenges is still limited. The most widely prescribed interventions address more overt deficits in sensorimotor and speech/language functions, yet interventions for higher-order cognitive, linguistic and behavioral deficits are notably less defined...

Global awareness of stroke as a significant cause of neurologic sequelae and death in children has increased over the years as more data in this field becomes available. However, most published literature on pediatric stroke have limited geographic representation. Data on childhood stroke from developing countries remains limited. Thus, this paper reviewed geographic/ethnic differences in pediatric stroke risk factors highlighting those reported in low- and middle-income countries, and proposes a childhood arterial ischemic stroke diagnostic algorithm for resource limited settings...

Stroke in childhood has multiple etiologies, which are mostly distinct from those in adults. Genetic discoveries over the last decade pointed to monogenic disorders as a rare but significant cause of ischemic stroke in children and young adults, including small vessel and arterial ischemic stroke. These discoveries contributed to the understanding that stroke in children may be a sign of an underlying genetic disease. The identification of these diseases requires a detailed medical and family history collection, a careful clinical evaluation for the detection of systemic symptoms and signs, and neuroimaging assessment...

Childhood stroke is not as common as adult stroke, but it is underrecognized the world over. Diagnosis is often delayed due to lack of awareness not only by the lay public but also by emergency and front-line health care workers. Despite the relative rarity of childhood stroke, the impact on morbidity, mortality and the economic burden for families and society is high, especially in poorly resourced settings. The risk factors for stroke in children differ from the adult population where lifestyle factors play a more important role...

Pediatric stroke is a condition that often results in life-long motor, cognitive, or sensory deficits for children. The purpose of this focused review is to compile the most recent literature on pediatric stroke neuromotor interventions and summarize evidence for use by rehabilitation providers and researchers. Terms including stroke, pediatric, and neuromotor were searched with appropriate MeSH terms. Information was collected regarding interventions conducted and outcome measures used for each article. Interventions and outcome measures were organized based on ICF components (Body Structure and Function, Activity, Participation, and Environmental Factors)...

Rehabilitation for pediatric stroke survivors can maximize a child's potential through each developmental stage of life. Timely diagnosis and referral to a rehabilitation specialist may harness opportunities to maximize brain plasticity, to help children adapt and learn, and to participate in and enjoy daily life to their capability. The aim of this focused review is to explore current rehabilitation models and evidence-based interventions for pediatric stroke survivors in Low- and Middle- Income Countries (LMICs) and to provide recommendations for future research and focused areas of improvement...