Tremors: Essential Tremor and Beyond
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 impairmen...
Source: Seminars in Pediatric Neurology - February 8, 2018 Category: Neurology Authors: Chandrabhaga Miskin, Karen S. Carvalho Source Type: research

Management of Pediatric Movement Disorders: Present and Future
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. (Source: Seminars in Pediatric Neurology)
Source: Seminars in Pediatric Neurology - February 8, 2018 Category: Neurology Authors: Jeffrey B. Russ, Akila M. Nallappan, Amy Robichaux-Viehoever Source Type: research

The Twists of Pediatric Dystonia: Phenomenology, Classification, and Genetics
The aim of this review is 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. (Source: Seminars in Pediatric Neurology)
Source: Seminars in Pediatric Neurology - February 8, 2018 Category: Neurology Authors: Inge A. Meijer, Toni Pearson Tags: Invited review Source Type: research

Tremors: Essential Tremor and Beyond
Tremor is a fairly common movement disorder presenting to an outpatient pediatric neurology practice. Tremors can be primary or secondary to underlying neurological or systemic diseases. When assessing a child with tremor, it is paramount to assess 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 impairmen...
Source: Seminars in Pediatric Neurology - February 8, 2018 Category: Neurology Authors: Chandrabhaga Miskin, Karen S. Carvalho Source Type: research

Management of Pediatric Movement Disorders: Present and Future
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 treatments which continues 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. (Source: Seminars in Pediatric Neurology)
Source: Seminars in Pediatric Neurology - February 8, 2018 Category: Neurology Authors: Jeffrey B. Russ, Akila M. Nallappan, Amy Robichaux-Viehoever Source Type: research

Inherited and Acquired Choreas
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 earl...
Source: Seminars in Pediatric Neurology - January 16, 2018 Category: Neurology Authors: Claudio M. de Gusmao, Jeff L. Waugh Source Type: research

Inherited and acquired choreas
Chorea is a symptom of a broad array of genetic, structural, and metabolic disorders. While chorea can result from systemic illness and injury to diverse brain structures, injury to or derangement of the putamen and/or globus pallidus appear to be 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....
Source: Seminars in Pediatric Neurology - January 16, 2018 Category: Neurology Authors: Claudio M. de Gusmao, Jeff L. Waugh Source Type: research

Pediatric Ataxia: Focus on Chronic Disorders
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. (Source: Seminars in Pediatric Neurology)
Source: Seminars in Pediatric Neurology - January 5, 2018 Category: Neurology Authors: David R. Lynch, Ashley McCormick, Kimberly Schadt, Elizabeth Kichula Source Type: research

Pediatric Ataxia: Focus on Chronic Disordersca
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 causes 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. Friedrich Ataxia is the most common, and genetic testing can easily confirm the suspicion. (Source: Seminars in Pediatric Neurology)
Source: Seminars in Pediatric Neurology - January 5, 2018 Category: Neurology Authors: David R Lynch, Ashley McCormick, Kimberly Schadt, Elizabeth Kichula Source Type: research

Anatomy, Physiology, and Clinical Syndromes of the Basal Ganglia: A Brief Review
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. (Source: Seminars in Pediatric Neurology)
Source: Seminars in Pediatric Neurology - December 27, 2017 Category: Neurology Authors: Arash Fazl, Jori Fleisher Source Type: research

Autoimmune Movement Disorders in Children
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 neurologis...
Source: Seminars in Pediatric Neurology - December 27, 2017 Category: Neurology Authors: Coral M. Stredny, Jeff L. Waugh Source Type: research

Paroxysmal Dyskinesias
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. (Source: Seminars in Pediatric Neurology)
Source: Seminars in Pediatric Neurology - December 27, 2017 Category: Neurology Authors: Sara McGuire, Swati Chanchani, Divya S. Khurana Source Type: research

Anatomy, Physiology, and Clinical Syndromes of the Basal Ganglia: A Brief Review
Movement disorders typically arise from dysfunction of the basal ganglia, cerebellum, or both. The basal ganglia —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 basal ganglia and cerebellum, and briefly highlight the clinical syndromes that may arise in the context of their injury or dysfunction. (Source: Seminars in Pediatric Neurology)
Source: Seminars in Pediatric Neurology - December 27, 2017 Category: Neurology Authors: Arash Fazl, Jori Fleisher Source Type: research

Autoimmune movement disorders in children
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 characteristic movement disorder that can be a powerful aid to diagnosis. Frequently, movement disorders in autoimmune conditions are the sole or are 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...
Source: Seminars in Pediatric Neurology - December 27, 2017 Category: Neurology Authors: Coral M. Stredny, Jeff L. Waugh Source Type: research