Motor system disorders. Part II, Spinal cord, neurodegenerative, and cerebral disorders and treatment /

Bibliographic Details
Corporate Author: ScienceDirect (Online service)
Other Authors: Younger, David S. (Editor)
Format: eBook
Language:English
Published: Amsterdam, Netherlands ; Cambridge, MA : Elsevier, [2023]
Series:Handbook of clinical neurology ; v. 196.
Subjects:
Online Access:Connect to the full text of this electronic book
Table of Contents:
  • Intro
  • Motor System Disorders, Part II: Spinal Cord, Neurodegenerative, and Cerebral Disorders and Treatment
  • Copyright
  • Foreword
  • Preface
  • Contributors
  • Contents
  • Spinal cord diseases
  • Chapter 1: Spinal cord motor disorders
  • Introduction
  • Embryology
  • Spinal cord
  • Vascular system
  • Neuroanatomy
  • Bony spine
  • Spinal cord
  • Vasculature
  • Spinal Motor Control
  • Supraspinal connections
  • Posture and gait integration
  • Spinal Cord Lesions
  • Spinal cord syndromes in the transverse plane
  • Anterior spinal cord
  • Anterior horn
  • Centromedullary
  • Brown-Séquard
  • Posterior spinal artery syndrome
  • Transverse complete spinal cord syndrome
  • Spinal cord syndromes in the longitudinal axis
  • Ischemia of the cervical region
  • Ischemia of the thoracic region
  • Ischemia of the lumbar region
  • History and Physical Examination
  • Laboratory Evaluation
  • Noninvasive spinal cord imaging
  • Neurophysiology
  • Somatosensory evoked potentials
  • Transcranial magnetic stimulation
  • Cerebrospinal fluid analysis
  • Vascular imaging
  • Conventional MRI
  • Advanced MRI techniques
  • Digital subtraction angiography
  • Tumors
  • Advances in tumor classification
  • Gliomas
  • Astrocytomas
  • Ependymomas
  • Meningiomas
  • Metastases
  • Lymphoma
  • CNS Demyelinating Diseases
  • Multiple sclerosis
  • NMO-spectrum disorders
  • MOG-antibody disease
  • Stroke
  • Spinal cord infarction
  • Vascular malformations
  • Arteriovenous fistula
  • Arteriovenous malformations
  • Cavernous malformations
  • Motor Neuron Disease
  • Spinal muscular atrophy
  • Progressive muscular atrophy
  • Primary lateral sclerosis
  • Poliomyelitis
  • Spinal Cord Injury
  • Intramedullary insults
  • Muscle and motor neuron excitability
  • Short- and long-term potentiation and depression
  • Implications for neural plasticity
  • Collateral sprouting
  • Regeneration.
  • Future Research
  • References
  • Chapter 2: Childhood spinal muscular atrophy
  • History
  • Classification
  • Epidemiology
  • Classification
  • SMA type 1
  • SMA type 2
  • SMA type 3
  • SMA type 4
  • Laboratory Evaluation
  • Genetics
  • Other Forms of Spinal Muscular Atrophy
  • Very severe SMA
  • SMA-plus types
  • Etiopathogenesis
  • SMN protein
  • Neurofilaments
  • SMN2 copy number
  • Splicing regulators as modifiers of phenotype
  • Epigenetic modifications: Methylation
  • Electrophysiological biomarkers
  • Neuroimaging
  • Management
  • Natural History and Prognosis
  • Disease-Modifying Molecular Therapy
  • Nusinersen, antisense oligonucleotide treatment to promote full-length SMN protein
  • Onasemnogene
  • Risdiplam
  • Conclusion and Future Directions
  • References
  • Chapter 3: The hereditary spastic paraplegias
  • Introduction11Abbreviations used in the chapter are listed at the end of the chapter before References section.
  • Definitions
  • Classification
  • Epidemiology
  • Simplex Cases
  • Clinical Presentation
  • Illustrative cases
  • Uncomplicated spastic paraplegia
  • Insidiously progressive spastic gait
  • Cerebral palsy phenotype
  • Infantile-onset, nonprogressive spastic gait
  • Primary lateral sclerosis phenotype
  • Spastic ataxia phenotype
  • Spastic paraplegia with distal muscle wasting
  • Developmental delay, intellectual impairment, dysarthria, spastic paraparesis or quadriparesis
  • Complex neurodegenerative disorders that include spastic paraparesis
  • Clinical Aspects and Disease Course
  • Neurologic findings in subjects with ``uncomplicated´´ HSP
  • Weakness
  • Spasticity
  • Mild impairment of distal vibration perception
  • Hyperreflexia
  • Reduced speed of muscle activation
  • Gait
  • Stance
  • Forward-shifted foot strike and toe walking
  • Hip flexion is often reduced (or slightly delayed).
  • Knee adduction and incomplete extension
  • Upper extremities
  • Diagnosis
  • Differential diagnosis
  • Diagnostic evaluation
  • Routine laboratory testing
  • Neuroimaging
  • Neurophysiology
  • Genetic Testing
  • Genetic Counseling
  • Neuropathology (Schwarz, 1952
  • Schwarz and Liu, 1956
  • Behan and Maia, 1974
  • Sack et al., 1978
  • Buge et al., 1979
  • Harding
  • Genetic Basis
  • Treatment and Prognosis
  • Spasticity treatment
  • Medication
  • Orthotics
  • Physical therapy
  • Prognosis
  • Conclusions
  • Future Directions
  • Acknowledgments
  • References
  • Chapter 4: Primary lateral sclerosis
  • Introduction
  • Clinical Description
  • Neuropathology
  • Electrophysiology
  • Neuroimaging
  • MRI
  • Positron emission tomography
  • Differential Diagnosis
  • Neurodegeneration and Relation to Motor Neuron Disease
  • Genetics of Primary Lateral Sclerosis
  • Treatment
  • Conclusions
  • References
  • Chapter 5: Transverse myelitis in children and adults
  • Introduction
  • Infectious Myelitis
  • Bacterial myelitis
  • Viral myelitis
  • Fungal myelitis
  • Parasitic
  • Noninfectious Myelitis
  • Multiple sclerosis
  • Neuromyelitis optica spectrum disorders
  • Acute disseminated encephalomyelitis
  • Myelin oligodendrocyte glycoprotein-antibody-associated disease
  • Idiopathic Myelitis
  • Transverse Myelitis and Vaccines
  • Systemic Disorders
  • Sarcoidosis
  • Systemic lupus erythematosus
  • Paraneoplastic myelitis
  • Radiation therapy-related myelitis
  • Toxic-metabolic vascular mimickers
  • Conclusions
  • References
  • Chapter 6: Multiple sclerosis: Motor dysfunction
  • Introduction
  • Epidemiology and Etiopathogenic Factors
  • Clinical motor dysfunction
  • Diagnosis
  • MS Variants: AQP4, NMOSD, and MOGAD
  • Childhood MS
  • Clinical features
  • Pediatric NMO and MOG serology
  • MRI findings
  • Prognosis
  • Pathology
  • Gross and microscopic pathology.
  • Immunopathology
  • Pharmacotherapy
  • Disease-modifying therapy
  • Assessing motor disability
  • Rehabilitation
  • Adaptive equipment
  • Symptomatic therapy
  • Exercise
  • Increasing neural plasticity
  • Endocannabinoid signaling
  • Cytokine signaling
  • Receptor signaling
  • Future Directions
  • References
  • Chapter 7: Tropical spastic paraparesis
  • Introduction
  • Tropical Myelopathy
  • Acute tropical myeloneuropathy
  • Transverse myelitis
  • Chronic Tropical Myelopathy
  • Tropical spastic paraparesis
  • Tropical Neuropathy
  • Strachan syndrome
  • Beriberi
  • Toxic Neuropathy
  • Neurologic Disorders Associated With Dietary Cyanide Intoxication
  • References
  • Progressive neurodegenerative diseases
  • Chapter 8: Cerebellum: From the identification of the cerebellar motor syndrome to the internal models
  • Introduction: History of Cerebellar Research Since the 18th Century1
  • Anatomy: Microscopy and Macroscopy
  • Cellular Physiology
  • Simple spikes and complex spikes
  • Rebound depolarization and disinhibition
  • Plasticity mechanisms
  • Cerebro-Cerebellar Structural Connectivity
  • Cerebello-Cortical Circuits: Neurophysiological Aspects
  • The Three Clinical Syndromes to the Light of Internal Models
  • Conclusions
  • Competing interests
  • Ethical committee request
  • References
  • Chapter 9: Synucleinopathies
  • Introduction
  • Historical Perspective
  • Pure Autonomic Failure
  • Pathophysiology
  • Pathology
  • Genetics
  • Epidemiology
  • Clinical features (Fig. 9.1)
  • Neurogenic orthostatic hypotension and supine hypertension
  • Gastrointestinal dysfunction
  • Genitourinary dysfunction
  • Thermoregulatory dysfunction
  • Anosmia
  • Dream enactment behavior
  • Other clinical manifestations
  • Ancillary testing
  • Autonomic function tests
  • Laboratory testing and evaluation of end-organ damage
  • Catecholamine studies
  • Neuroendocrine studies.
  • Cardiac sympathetic neuroimaging
  • Urodynamic studies and gastrointestinal studies
  • Skin biopsy
  • Analysis of cerebrospinal fluid
  • Treatment
  • Care team
  • Symptom management
  • Prognosis and phenoconversion
  • Multiple System Atrophy
  • Pathophysiology
  • Pathology
  • Genetics
  • Epidemiology
  • Clinical features
  • Diagnostic criteria
  • Motor features
  • Nonmotor features
  • Survival
  • Paraclinical testing
  • Autonomic cardiovascular domain
  • Plasma catecholamines
  • Thermoregulatory domain
  • Urogenital, gastrointestinal, and respiratory domains
  • Brain imaging
  • Cardiac sympathetic neuroimaging
  • Skin biopsy
  • Analysis of cerebrospinal fluid
  • Treatment
  • Multidisciplinary approach
  • Symptom management
  • Motor symptoms
  • Nonmotor symptoms
  • Disease-modifying approaches (Fig. 9.4)
  • Lewy Body Disorders-Parkinson Disease and Dementia with Lewy Bodies
  • Pathophysiology
  • Pathology
  • Genetics
  • Epidemiology
  • Clinical features
  • Diagnostic criteria
  • Nonmotor features
  • Paraclinical testing
  • Imaging techniques
  • Autonomic testing
  • Plasma catecholamines
  • Skin biopsy
  • Analysis of cerebrospinal fluid
  • Others
  • Treatment
  • Conclusions
  • References
  • Chapter 10: Amyotrophic lateral sclerosis
  • Introduction
  • Epidemiology
  • Neuropathology
  • Clinical Presentation
  • Diagnosis
  • Genetic Basis of ALS
  • Cu/Zn-superoxide dismutase
  • C9orf72
  • TARDBP
  • Fused in sarcoma
  • Optineurin
  • Valsolin-containing protein
  • Dynactin
  • Senataxin
  • GLE1, RNA export mediator
  • UBQLN2, PFN1, and VAPB
  • Disease Pathogenesis
  • Instability of mutant proteins
  • SOD1
  • TARDBP
  • Disrupted RNA metabolism
  • C9orf72
  • SCA36
  • Defective axonal cytoskeletal and transport
  • DCTN1
  • Profilin-1
  • Other putative disease mechanisms
  • Defective autophagy
  • ER stress and impaired degradation (SOD1, OPTN).