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Motor System Disorders, Part I
Normal Physiology and Function and Neuromuscular Disorders
- 1st Edition, Volume 195 - August 8, 2023
- Editor: David S. Younger
- Language: English
- Hardback ISBN:9 7 8 - 0 - 3 2 3 - 9 8 8 1 8 - 6
- eBook ISBN:9 7 8 - 0 - 3 2 3 - 9 5 8 5 4 - 7
Motor System Disorders, Volume 195, Part One, Normal Physiology and Function and Neuromuscular Disorders summarizes recent advances and best practice for understanding normal… Read more
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Request a sales quoteMotor System Disorders, Volume 195, Part One, Normal Physiology and Function and Neuromuscular Disorders summarizes recent advances and best practice for understanding normal physiology and function of the larger motor system as well as the diagnosis, course, and treatment of neuromuscular disorders. The work includes neuroanatomy, circuits, and synapses, and discusses how different parts of the brain contribute to motor control, including both vestibular and cerebellar motor control, as well as the influences of the parietal and frontal lobes. Diagnostic methods include genetic evaluation, electrodiagnosis, pathology, and brain imaging. Neuromuscular disorders of both children and adults are included.
- Identifies normal motor control physiology and function, including neuroanatomy, circuits, and synapses
- Discusses vestibular and cerebral motor control and contributions of parietal and frontal lobes
- Provides diagnostic methods, including genetic evaluation, electrodiagnosis, brain imaging, and pathology analyses
- Summarizes the diagnosis, course, and best practice treatment of neuromuscular disorders
- Explores neuromuscular disorders in adults and children
Practicing neurologists, research neurologists; General MDs interested in motor disfunction
- Cover image
- Title page
- Table of Contents
- Series Page
- Copyright
- Handbook of Clinical Neurology 3rd Series
- Foreword
- Preface
- Contributors
- Section 1: Normal physiology and function
- Chapter 1: Skeletal muscle structure, physiology, and function
- Abstract
- Introduction
- Skeletal Muscle Structure
- Contractile Mechanisms
- Muscle Fiber Types
- Calcium Activation of Contraction
- Skeletal Muscle Function
- Neuromuscular Junction Structure and Development
- Neurotransmission
- Motor Unit Properties
- Motor Unit Recruitment
- References
- Chapter 2: Upper and lower motor neuron neurophysiology and motor control
- Abstract
- Introduction
- Functional Motor Systems in Brain and Spinal Cord
- Evolution of the Motor System
- Motor Cortex
- Spinal Cord
- The Cerebellum
- The Lower Motor Neuron System
- Conclusion
- References
- Chapter 3: Vestibular motor control
- Abstract
- Overview
- Vestibular Motor Control: Neuronal Coding in Alert Animals
- Nonlinear & Spike Timing Codes: Implications for Motor Control and the Restoration of Function
- The Vestibulo-Ocular Reflex
- Vestibulospinal Reflex Pathways
- The Vestibular Cerebellum: Internal Models of Externally-Applied and Active Self-Motion
- Compensation and Extra-Vestibular Sensory Substitution in Central Pathways: Implications for Motor Control
- Voluntary Behavior: Steering, Reaching and Navigation
- References
- Chapter 4: Autonomic failure: Clinicopathologic, physiologic, and genetic aspects
- Abstract
- Overview
- Autonomic Control of Bladder, Bowel and Sexual Function
- Neuropathologic Characterization
- Neurodegenerative Autonomic Failure
- Hereditary Sensory and Autonomic Neuropathies
- Autoimmune Autonomic Failure
- Future Directions
- References
- Chapter 5: Gait control by the frontal lobe
- Abstract
- Introduction
- Frontal Lobe Control of Gait
- Core Posture Gait Mechanisms in the Brainstem and Spinal Cord
- Functional Neuroanatomy of the Cortical Control of Behavior Expression
- Possible Cortical Mechanisms of Human Posture–Gait Control
- Posture–Gait Control by the Cerebellum, BG, and Emotional Systems
- References
- Chapter 6: Parietal control of hand movement
- Abstract
- Introduction
- Parallel Visuomotor Processing
- Sensorimotor Apraxia
- Limb Apraxia
- Executive Apraxia
- Conclusion
- References
- Chapter 7: Immunology and microbiome: Implications for motor systems
- Abstract
- Introduction
- Immunology and Motor Systems
- The Microbiota-Gut-Brain Axis and Motor Systems
- Conclusions
- Future Directions
- References
- Chapter 8: COVID-19 (novel SARS-CoV-2) neurological illness
- Abstract
- Background
- Epidemiology
- Susceptibility to Infection
- Acute COVID-19 Infection
- COVID-19 Diagnostic Testing
- Neurological Presentation
- Cytokine Storm
- Clinicopathological Correlation
- Covid Variants
- Immunotherapy
- Convalescent Plasma
- Vaccination
- Chronic Covid Illness
- Conclusion
- References
- Section 2: Clinical and laboratory diagnosis
- Chapter 9: Neurogenetic motor disorders
- Abstract
- Introduction
- Mitochondrial Genetics
- Clinical Clues of a Neurogenetic Motor Disorder
- Genetic Neurological Disorders
- Genetic Sequencing Strategies
- Factors Influencing Phenotypical Gene Expression
- Genetic Biomarkers
- Defects in RNA Processing
- Repeat Expansions Diseases
- Genomic Imprinting and Epigenetics
- Assignment of a Gene Defect
- References
- Chapter 10: Neuromuscular electrodiagnosis
- Abstract
- Introduction
- Organization of the Peripheral Neuromuscular System
- Pertinent Anatomy and Physiology
- The Neuromuscular Junction
- Muscle
- Nerve Conduction Studies
- Special Studies
- The Needle EMG Examination
- References
- Chapter 11: Quantitative electrodiagnosis of the motor unit
- Abstract
- Introduction
- Anatomic Motor Unit
- Electrophysiologic Motor Unit
- Motor Unit Viewing and Assessment
- Electrophysiologic Motor Unit Metrics
- Single-Fiber EMG
- Normative Data
- Motor Unit Number Estimates
- MU Metric Changes with Pathology
- Statistical Motor Unit and Muscle Characterization
- Analysis of Turns and Amplitude
- Practical Considerations
- Concluding Comments
- References
- Chapter 12: Neuromuscular pathology
- Abstract
- Introduction
- Muscle Biopsies
- Future Direction
- Conclusion
- References
- Chapter 13: Electrophysiological assessment of peripheral and central autonomic disorders
- Abstract
- Introduction
- Historical Background
- Anatomy and Physiology
- Tests of Autonomic Function
- Sudomotor Reflexes
- Future Directions
- References
- Chapter 14: On the path to evidence-based therapy in neuromuscular disorders
- Abstract
- Introduction
- Motor Neuron Disease
- Peripheral Neuropathy
- Myasthenia Gravis
- Muscular Dystrophy
- Conclusions
- References
- Chapter 15: Advances in the neuroimaging of motor disorders
- Abstract
- Introduction
- Neuroimaging Techniques
- Neuroimaging of CNS Motor Disorders
- Neuroimaging of the Spinal Cord
- Neuroimaging of PNS Disorders
- Conclusion
- References
- Chapter 16: Sleep-related motor disorders
- Abstract
- Introduction
- Sleep Motor Control
- Nrem Sleep Parasomnias
- REM Sleep Parasomnias
- Restless leg syndrome
- Periodic Limb Movement Disorder
- Isolated Motor Phenomena in Sleep
- Conclusion
- References
- Section 3: Neuromuscular disorders
- Chapter 17: Neonatal and infantile hypotonia
- Abstract
- Introduction
- Pathophysiology of motor tone
- Clinical Inspection
- Clues to The Differential Cause of Hypotonia
- References
- Chapter 18: Autoimmune inflammatory myopathies
- Abstract
- Introduction
- Historical Background
- Shared Clinical Features
- Specific Clinical Features
- Diagnosis and Diagnostic Workup
- Muscle Biopsy
- Immunopathology
- Nonimmune Factors in IBM
- Treatment for all AIM except IBM
- Step-by-Step Therapeutic Approach
- Treatment Trials in Inclusion Body Myositis
- Conclusions
- Future Directions
- References
- Chapter 19: Childhood muscular dystrophies
- Abstract
- Introduction
- Classification
- General Clinical Approach
- General Laboratory Approach
- Dystrophin-Glycoprotein Complex
- Dystrophin-Associated Muscular Dystrophy
- Limb Girdle Muscular Dystrophies
- Congenital Muscular Dystrophy
- Emery–Dreifuss Muscular Dystrophy
- Distal Myopathies
- Congenital Myotonic Dystrophy
- Conclusions
- Future Directions
- References
- Chapter 20: Distal myopathy
- Abstract
- Introduction
- Alpha-Actinin-2-Related Distal Myopathy (OMIM #618655, Myopathy Distal 6, Adult Onset)
- Alpha-B Crystallin-Related Distal Myopathy (OMIM #608810, Myofibrillar Myopathy, Type 2)
- Adenylosuccinate Synthase 1-Related Distal Myopathy (OMIM #617030, Myopathy Distal, 5)
- Anoctamin 5-Related Distal Myopathy (OMIM #613319, Miyoshi Muscular Dystrophy 3)
- Caveolin 3-Related Distal Myopathy (OMIM #614321, Myopathy, Distal, Tateyama Type)
- Desmin-Related Distal Myopathy (OMIM #601419, Myofibrillar Myopathy, Type 1)
- DNAJB6-Related Distal Myopathy (Distal Calf-Predominant Myopathy)
- Dysferlin-Related Distal Myopathy (OMIM #254130, Miyoshi Muscular Dystrophy)
- Filamin C-Related Distal Myopathy (OMIM #614065, Myopathy Distal 4)
- GNE-Related Distal Myopathy (OMIM #605820, Nonaka Rimmed Vacuolar Distal Myopathy)
- HSPB8-Related Distal Neuromyopathy
- LIM Domain-Binding Protein 3-Related Distal Myopathy (OMIM #609452, Myopathy Myofibrillar 4)
- Matrin-3-Related Distal Myopathy (Vocal Cord and Pharyngeal Distal Myopathy)
- Myosin Heavy Chain Beta-Related Distal Myopathy (OMIM #160500, Myopathy Distal 1, Laing Distal Myopathy)
- Myotilin-Related Distal Myopathy (OMIM #609200, Myofibrillar Myopathy, Type 3)
- Nebulin-Related Distal Myopathy (Distal Nebulin Myopathy)
- Perilipin-4–Related Distal Myopathy
- Ryanodyne Receptor 1-Related Distal Myopathy (Calf Predominant Distal Myopathy)
- Sequestosome 1 and Tia-Related Digenic Distal Myopathy (OMIM #617158, Myopathy, Distal, with Rimmed Vacuoles)
- Small Muscle Protein, X-Linked-Related Distal Myopathy (OMIM #301075, Myopathy, Distal, 7, Adult-Onset, X-Linked)
- TIA1-Related Distal Myopathy (OMIM #604454, Welander distal myopathy)
- Titin-Related Distal Myopathies
- Valosin-Containing Protein-Related Distal Myopathy (Adult-Onset Distal Myopathy due to VCP Mutation)
- Other Genetic Distal Myopathies
- Diagnosis of Distal Myopathy in the Next-Generation Sequencing Era
- Management and Treatment
- Future Directions
- Conclusions
- References
- Chapter 21: Muscle channelopathies
- Abstract
- Introduction
- Myotonia
- Pediatric Syndromes and Variants
- Periodic Paralysis
- Future Directions
- Conclusions
- Acknowledgments
- References
- Chapter 22: Congenital myopathies
- Abstract
- Introduction
- Inspection
- Congenital Myopathy Subtypes
- Natural History
- Approach to Diagnosis
- Management of Patients
- Mutation-Targeted Pharmacotherapy
- Future Considerations
- References
- Chapter 23: Mitochondrial encephalomyopathy
- Abstract
- Introduction
- Overview of Mitochondrial Genetics
- Neurological Manifestations of Mitochondrial Disease
- Diagnostic Investigation
- Management
- Conclusion
- References
- Chapter 24: Autoimmune polyneuropathies
- Abstract
- Introduction
- Section 1 Acquired Demyelinating Neuropathies
- Section 2: Radiculoplexus Neuropathies
- Section 3: Motor Neuropathies in the Setting of Rheumatological Disease
- Conclusion
- References
- Chapter 25: Hereditary neuropathy
- Abstract
- Introduction
- CMT1A
- CMT1B
- CMTX1
- CMT2A
- (SORD)-Associated CMT
- Current Management
- Conclusions
- References
- Chapter 26: Acute/chronic inflammatory polyradiculoneuropathy
- Abstract
- Introduction
- Guillain–Barre Syndrome
- CIDP
- Conclusion
- Future Directions
- References
- Chapter 27: Myasthenia gravis and congenital myasthenic syndromes
- Abstract
- Introduction
- Myasthenia Gravis
- Congenital Myasthenic Syndromes
- References
- Chapter 28: Adult and childhood vasculitis
- Abstract
- Nomenclature
- Historical Themes
- History and Examination
- Laboratory Evaluation
- Treatment
- Conclusions
- References
- Chapter 29: Critical illness–associated weakness and related motor disorders
- Abstract
- Introduction1
- Background
- Nosology
- Neuromuscular Assessment
- Respiratory Assessment
- Critical Illness Motor Disorders Prompting ICU Admission
- Critical Illness Weakness That Develops in the ICU
- Conclusion
- References
- Index
- No. of pages: 826
- Language: English
- Edition: 1
- Volume: 195
- Published: August 8, 2023
- Imprint: Elsevier
- Hardback ISBN: 9780323988186
- eBook ISBN: 9780323958547
DY
David S. Younger
David S. Younger MD DrPH, MPH MS, Affiliate Professor of Neuroscience at City University of New York Medical School and Attending Physician at White Plains Hospital, NY, has vast experience in both clinical and academic neurology and public health. He mentors students at his alma mater Columbia University College of Physicians and Surgeons. He has been Board certified in Neurology, Internal Medicine, Electrodiagnostic Medicine (AANEM) and Neurophysiology (ABCN). As Associate Editor of the Seminars in Neurology he prepared the 1st edition of his widely-acclaimed textbook, Motor Disorders, now in its 4th edition. He is best recognized as a highly trained electrophysiologist and neuromuscular specialist. For the past decade his work has focused on autoimmunity of the nervous system and the use of immunotherapy and most recently, in COVID-19 neurological illnesses. He is the author of Human Lyme Neuroborreliosis, Science of Medical Cannabis, The Vasculitides, and The Autoimmune Brain; and he has been Guest Editor of the multiple volumes of the Neurological Clinics. He has also authored more than 300 peer-reviewed articles, book chapters, correspondence and scientific abstracts on a wide variety of topics.
Affiliations and expertise
Affiliated Professor of Neuroscience, City University of New York Medical School, New York, NY, United States; Attending Physician in Neurology, White Plains Hospital, White Plains, NY, United StatesRead Motor System Disorders, Part I on ScienceDirect