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Seminars in Pediatric Neurology

Coral M Stredny, Jeff L Waugh
Over the last decade, there have been significant advances in the identification, characterization, and treatment of autoimmune neurologic disorders in children. Many of these diseases include a typical movement disorder that can be a powerful aid to diagnosis. Frequently, movement disorders in autoimmune conditions are the sole or among a few presenting symptoms, allowing for earlier diagnosis of an underlying malignancy or systemic autoimmune disease. Given that early detection and treatment with immunotherapy may confer improved outcomes, recognizing these patterns of abnormal movements is essential for child neurologists...
April 2018: Seminars in Pediatric Neurology
Celanie K Christensen, Laurence Walsh
Many inherited metabolic diseases or inborn errors of metabolism (IEM) cause movement disorders in children. This review focuses on chorea, dystonia, myoclonus, tremor, and parkinsonism. Broad neurometabolic categories commonly responsible for pediatric movement disorders include mitochondrial cytopathies, organic acidemias, mineral metabolism and transport disorders, neurotransmitter diseases, purine metabolism abnormalities, lipid storage conditions, and creatine metabolism dysfunction. Each movement disorder can be caused by many IEM and several of them can cause multiple movement abnormalities...
April 2018: Seminars in Pediatric Neurology
Sara McGuire, Swati Chanchani, Divya S Khurana
Paroxysmal dyskinesias (PD) are hyperkinetic movement disorders where patients usually retain consciousness. Paroxysmal dyskinesias can be kinesigenic (PKD), nonkinesigenic (PNKD), and exercise induced (PED). These are usually differentiated from each other based on their phenotypic and genotypic characteristics. Genetic causes of PD are continuing to be discovered. Genes found to be involved in the pathogenesis of PD include MR-1, PRRT2, SLC2A1, and KCNMA1. The differential diagnosis is broad as PDs can mimic psychogenic events, seizure, or other movement disorders...
April 2018: Seminars in Pediatric Neurology
Inge A Meijer, Toni S Pearson
This article aims to provide a practical review of pediatric dystonia from a clinician's perspective. The focus is on the underlying genetic causes, recent findings, and treatable conditions. Dystonia can occur in an isolated fashion or accompanied by other neurological or systemic features. The clinical presentation is often a complex overlap of neurological findings with a large differential diagnosis. We recommend an approach guided by thorough clinical evaluation, brain magnetic resonance imaging (MRI), biochemical analysis, and genetic testing to hone in on the diagnosis...
April 2018: Seminars in Pediatric Neurology
David R Lynch, Ashley McCormick, Kimberly Schadt, Elizabeth Kichula
Evaluation of a pediatric patient presenting with ataxia can be expensive and time consuming. Acute causes tend to have a clear developmental paradigm, but chronic presentations are more likely to be secondary to a genetic disorder, either one that primarily causes ataxia or that presents ataxia as one of a multitude of symptoms. Evaluation should focus on a quick diagnosis for those that have treatment options and for those that require other systemic monitoring. Friedreich ataxia is the most common, and genetic testing can easily confirm the suspicion...
April 2018: Seminars in Pediatric Neurology
Claudio M de Gusmao, Jeff L Waugh
Chorea is a symptom of a broad array of genetic, structural, and metabolic disorders. While chorea can result from systemic illness and damage to diverse brain structures, injury to the basal ganglia, especially the putamen or globus pallidus, appears to be a uniting features of these diverse neuropathologies. The timing of onset, rate of progression, and the associated neurological or systemic symptoms can often narrow the differential diagnosis to a few disorders. Recognizing the correct etiology for childhood chorea is critical, as numerous disorders in this category are potentially curable, or are remediable, with early treatment...
April 2018: Seminars in Pediatric Neurology
Chandrabhaga Miskin, Karen S Carvalho
Tremor is a fairly common movement disorder presenting to an outpatient pediatric neurology practice. Tremors can be primary or secondary to underlying neurologic or systemic diseases. When assessing a child with tremor, it is paramount to evaluate the phenomenology of the tremor, determine the presence or absence of other neurologic signs and symptoms, and the possible modifying influence of medications. Proper classification is essential for specific diagnosis and prompt adequate management. Treatment considerations should take into account objective assessment of tremor severity and the degree of disability or impairment experienced by the child...
April 2018: Seminars in Pediatric Neurology
Joanna S Blackburn
Tics are the most common movement disorder in childhood and are a frequent reason for referral to child neurology clinics. The purpose of this review is to examine the phenomenology of tics, discuss what is known regarding their genetic and pathophysiological causes and to evaluate current treatment options. The evidence for the evaluation and treatment of the controversial diagnosis of pediatric autoimmune neuropsychiatric disorders associated with group A streptococci (PANDAS) will also be reviewed. With improved understanding of tic disorders, their etiology and response to current treatment options, we may be able to more effectively diagnose them and identify novel treatment strategies...
April 2018: Seminars in Pediatric Neurology
Arash Fazl, Jori Fleisher
Movement disorders typically arise from dysfunction of the basal ganglia (BG), cerebellum, or both. The BG-a group of deep, subcortical structures-form complex circuits that shape motor control and motor learning, as well as limbic and associative functions. In this article, we summarize the anatomy and physiology of the BG and cerebellum, and briefly highlight the clinical syndromes that may arise in the context of their injury or dysfunction.
April 2018: Seminars in Pediatric Neurology
Mackenzie Katherine
This review summarizes motor stereotypies in terms of description, prevalence, pathophysiology, diagnosis and management. They are fixed and persistent movements. Stereotypies begin before 3 years of age and continue into adulthood. Primary motor stereotypies occur in children of normal intelligence, whereas secondary stereotypies ensue in the setting of an additional diagnosis such as autism spectrum disorder or other neurologic disorders. They are highly associated with comorbidities such as anxiety, obsessive-compulsive symptoms, inattention, and tics...
April 2018: Seminars in Pediatric Neurology
Jeffrey B Russ, Akila M Nallappan, Amy Robichaux-Viehoever
Management of movement disorders in children is an evolving field. This article outlines the major categories of treatment options for pediatric movement disorders and general guidelines for their use. We review the evidence for existing therapies, which continue to lack large-scale controlled trials to guide treatment decisions. The field continues to rely on extrapolations from adult studies and lower quality evidence such as case reports and case series to guide treatment guidelines and consensus statements...
April 2018: Seminars in Pediatric Neurology
Kelda Harris Walsh, Katherine Soe, Shivali Sarawgi
Pediatric movement disorders may present with psychiatric symptoms at many points during the course of the disease. For the relatively common pediatric movement disorder, Tourette syndrome, psychiatric comorbidities are well-described and treatment is well-studied. Managing these comorbidities may be more effective than improving the movements themselves. For more uncommon movement disorders, such as juvenile-onset Huntington disease, treatment of psychiatric comorbidities is not well-characterized, and best-practice recommendations are not available...
April 2018: Seminars in Pediatric Neurology
Deepti Nagesh, Marcie Goeden, Keith A Coffman
The acute development of a movement disorder is often a dramatic and frightening experience for patients and families, often requiring urgent or emergent evaluation by a neurologist. In the assessment of these patients, one relies on the history, physical and neurologic examination to determine the etiology of the condition. We aim to demonstrate that a thorough medication history is an incredibly critical part of this evaluation as iatrogenic movement disorders can arise from exposure not only to psychoactive medications, but from drugs prescribed for a variety of nonneurologic disorders...
April 2018: Seminars in Pediatric Neurology
Jennifer A O'Malley, Donald L Gilbert
Assessing movement can be especially challenging in children. Refined yet flexible observational examination skills and utilization of established phenomenological approaches are essential in distinguishing normal from abnormal movements in the developing child and reaching an appropriate diagnosis. Mastering such skill requires an appreciation of the unique features of the developing motor system and an understanding of key concepts underlying normal motor development in children. Establishing a trusting therapeutic relationship with the patient and family, minimizing anxiety, and utilizing observation and distraction during physical examination are essential to successful diagnosis and management...
April 2018: Seminars in Pediatric Neurology
Karen S Carvalho, Daphne M Hasbani
No abstract text is available yet for this article.
April 2018: Seminars in Pediatric Neurology
Adam P Ostendorf, Satyanarayana Gedela
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...
November 2017: Seminars in Pediatric Neurology
Ahsan N V Moosa, Elaine Wyllie
Cognitive dysfunction in children with epilepsy is primarily contributed by etiology, seizures, frequency of interictal epileptiform discharges, and adverse effects of antiepileptic drugs. The direct effect of epilepsy surgery on cognitive outcome depends on two key factors: the function that is present in the epileptogenic zone to be removed, and the dysfunction outside the epileptogenic zone caused by epilepsy. Studies on cognitive outcome in children after various types of epilepsy surgery estimate "no significant change" in about 70% of children, improvement in cognition in 10%-15%, and decline in 10%-15%...
November 2017: Seminars in Pediatric Neurology
Adriana Ulate-Campos, Ivan Sánchez Fernández
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...
November 2017: Seminars in Pediatric Neurology
Christopher B Oakley, Eric H Kossoff
Headache and seizures are two of the most common complaints seen in the field of pediatric neurology with headache being number one. Both these conditions may coexist. Where the difficulty begins is when the symptoms are not clear cut in making a diagnosis, and conditions are possible as either an atypical seizure or migraine variant. What further complicates matters is the fact that there are many underlying neurologic conditions that carry with them a higher likelihood of developing both headaches and seizures, making each a distinct possibility when obtaining a history from a parent about unusual spells...
November 2017: Seminars in Pediatric Neurology
Nancy A McNamara, Erin Marie Fedak Romanowski, David P Olson, Renee A Shellhaas
Antiseizure medications and dietary therapies have associated effects on the endocrine system. We provided an overview of the relationship between epilepsy treatment and bone health in children with epilepsy. Additionally, we discussed the effects of epilepsy treatment on other endocrine systems including thyroid function, growth, reproduction, and weight. The effect of epilepsy on bone health is multifactorial; there are direct and indirect effects of medication and dietary treatments as well as a decrease in physical activity, decreased sunlight exposure, decreased vitamin D levels, and additional comorbidities...
November 2017: Seminars in Pediatric Neurology
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