Movement Disorders in Childhood

Harvey S. Singer, Jonathan W. Mink, Donald L. Gilbert, Joseph Jankovic, Jonathan Mink

• Publisher: Academic Press
• Year: 2015
• Print ISBN: 9780124115736
• eBook ISBN: 9780124115804
• Edition: 2
• Topic: Medicine
• Subtopic: Neurology

Movement Disorders in Childhood, Second Edition, provides the most up-to-date information on the diseases and disorders that affect motor control, an important area of specialization within child neurology. Over the past several decades, advances in genetics, neuroimaging, neurophysiology, and other areas of neuroscience have provided new understanding of the underlying etiologies and mechanisms of these conditions as well as new opportunities for more accurate diagnosis and effective treatment. This new edition builds upon the success of the first edition, with comprehensive scientific and clinical updates of all chapters. In addition, there are new chapters on hereditary spastic paraplegia, quantitative motor assessments, autoimmune disorders, and movement disorders in the developmental neuropsychiatric disorders ADHD, OCD, and autism. Additional materials are provided on the latest in drug treatments, computer based strategies for genetic diagnosis, and helpful videos for phenomenology. - Provides the only current reference specifically focused on childhood movement disorders - Investigates the underlying etiologies and mechanisms of these disorders - Completely revised and updated with new materials and a more disease-oriented approach - New coverage of genetics and movement disorders, immunology and movement disorders, and an introduction to the latest quantitative analysis - New videos of instructive and unusual childhood movement disorders - 2016 BMA Medical Book Awards Highly Commended in Neurology

Section IV

Hyperkinetic and Hypokinetic Movement Disorders

Outline

Chapter 10 Chorea, Athetosis, and Ballism

Chapter 11 Dystonia

Chapter 12 Myoclonus

Chapter 13 Tremor

Chapter 14 Ataxia

Chapter 15 Parkinsonism

Chapter 16 Hereditary Spastic Paraplegia

Chapter 10

Chorea, Athetosis, and Ballism

Harvey S. Singer¹, Jonathan W. Mink², Donald L. Gilbert³ and Joseph Jankovic⁴, ¹Department of Neurology, Johns Hopkins Hospital, Baltimore, MD, USA, ²Division of Child Neurology, University of Rochester Medical Center, Rochester, NY, USA, ³Division of Neurology, Cincinnati Children’s Hospital Medical Center, Cincinnati, OH, USA, ⁴Department of Neurology, Baylor College of Medicine, Houston, TX, USA

Abstract

This chapter discusses the large variety of diagnoses in childhood where chorea may be a prominent feature. Chorea is uncommon in children. It nearly always causes some interference with life activities and is perceived as involuntary by the individual. The terms “athetosis” and “ballism” designate similar movements, and are reviewed in this chapter. The most common cause of chorea in childhood is Sydenham’s chorea. This chapter provides a systematic approach to diagnosis and treatment of chorea in childhood, based on childhood development, epidemiology, primary features, associated symptoms, and understanding of neuroanatomy and pathophysiology.

Keywords

Sydenham’s chorea; chorea diagnosis; chorea treatment; athetosis; ballism; autoimmune chorea; vascular chorea; metabolic chorea; basal ganglia; neuroleptics

Outline

Introduction and Overview 144

Definitions of Chorea, Athetosis, and Ballism 144

Clinical Characteristics—Phenomenology of Chorea, Athetosis, and Ballism in Children 145

Chorea 145

Patient History 145

Examination 145

Ballism 146

Athetosis 146

Localization and Pathophysiology 146

Neuroanatomy of Chorea, Athetosis, and Ballism 146

Neurophysiology of Chorea, Athetosis, and Ballism 147

Diseases and Disorders 147

Etiologic Categories of Diseases Producing Chorea, Athetosis, and Ballism 147

Physiologic Chorea 151

Chorea in Infancy 151

Chorea Minor 152

Primary Choreas 152

Benign Hereditary Chorea 152

Secondary (Acquired) Choreas 154

Choreas Occurring in Immunologic/Autoimmune Disease 154

Chorea in Primary Anti-Phospholipid Antibody Syndrome (APS) and Systemic Lupus Erythematosus (SLE) 160

Anti-NMDA Receptor Encephalitis with Chorea and Psychiatric Changes 161

Vascular/Hypoxic Ischemia 162

Dyskinetic/Choreoathetoid Cerebral Palsy 163

Kernicterus/Chronic Bilirubin Encephalopathy 163

Hyperglycemia/Diabetes–Associated Subacute Hemichorea or Hemiballism 163

Multisystem and Genetic Diseases Where Chorea is Prominent 164

Huntington Disease (HD) with onset in Late Teens 164

Infantile Bilateral Striatal Necrosis 165

Summary of Diagnostic and Therapeutic Approach 165

Therapeutics 165

References 169

Introduction and Overview

Chorea, athetosis, and ballism are nonpatterned, hyperkinetic movement disorders which overlap in many patients and cannot be differentiated as mutually exclusive phenomena. However, they are characterized by some salient features. Some authors place these hyperkinetic disorders on a continuum based on amplitude, velocity, and distribution: ballism → chorea → athetosis.

In practice, many children with hyperkinetic movement disorders have a combination of chorea, athetosis, or ballism. To complicate matters, dystonia, tics, or myoclonus may also be present. Moreover, the relative predominance of one type of phenomenology may be state dependent. For example, athetosis may evolve into ballism when children are stimulated or excited.

Anatomically, chorea classically results from disturbances in the striatum but can also have thalamic or cortical origin. Ballism often localizes to subthalamic nucleus. Athetosis often accompanies basal ganglia diseases that also produce chorea or dystonia. It is seen in children with globus pallidus interna (GPi) injury due to bilirubin toxicity in infancy (kernicterus). Thus, despite phenomenologic distinctions, clinicians may use the presence of any of these dyskinetic movements as key factors in directing diagnostic and therapeutic decision making toward the basal ganglia.

Definitions of Chorea, Athetosis, and Ballism

Chorea refers to an involuntary, random-appearing sequence of one or more discrete involuntary movements or movement fragments which appear random due to variable timing, duration, rate, direction, or location. All body parts may be involved, with certain distributions being more characteristic of distinct diseases or disorders. Choreic movements usually worsen during attempted voluntary action. Individuals with chorea may generate so-called parakinesias, semi-volitional movements that attempt to mask the involuntary choreic movements or incorporate them into seemingly purposeful movements, such as touching the face.

Ballism refers to involuntary, high-amplitude, flinging movements typically generated proximally. These movements may be brief or continual and may occur in conjunction with chorea. Often, one side of the body is affected, i.e., hemiballism. In many cases, hemiballism becomes milder and evolves into chorea, athetosis, or dystonia.¹ Severe continuous ballism can cause rhabdomyolysis.

Athetosis is defined as slow, writhing, continuous, involuntary movements. This may be historically referred to as choreoathetosis. In contrast to dystonia, in which there is a sustained, twisting, patterned movement, athetosis is typically a continual, nonsustained form of movement. It may have a rotary component with finger flexion and extension. Athetosis sometimes occurs as part of a mixed spastic, hyperkinetic movement disorder in children with static encephalopathy (cerebral palsy). Some experts view athetosis on a spectrum with dystonia and chorea.

Clinical Characteristics—Phenomenology of Chorea, Athetosis, and Ballism in Children

Chorea

Patient History

In childhood, chorea is most often acquired acutely or subacutely, and thus parents can describe the onset and the way in which the child’s speech and purposeful movements have changed. Acquired chorea usually interferes with purposeful movement, causing functional impairment. In subtle cases, particularly in young children with underdeveloped motor coordination or speech articulation, a parent’s report that coordination or speech has changed must be relied on.

In contrast, when chorea occurs as a late or minor feature of chronic neurologic disease, parents ...

Table of contents

• Cover image
• Title page
• Table of Contents
• Copyright
• Preface
• Acknowledgment
• Section I: Overview
• Section II: Developmental Movement Disorders
• Section III: Paroxysmal Movement Disorders
• Section IV: Hyperkinetic and Hypokinetic Movement Disorders
• Section V: Selected Secondary Movement Disorders
• Appendices