Motor System Disorders. Part II, Spinal Cord, Neurodegenerative, and Cerebral Disorders and Treatment / David S. Younger.

Format:

Book

Author/Creator:

Younger, David S., author.

Series:

Issn Series.

Issn Series

Language:

English

Subjects (All):

Movement disorders.

Physical Description:

1 online resource (672 pages).

Edition:

First edition.

Place of Publication:

Amsterdam : Elsevier, [2023]

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

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

SMA type 1

SMA type 2

SMA type 3

SMA type 4

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

Chapter 3: The hereditary spastic paraplegias

Introduction11Abbreviations used in the chapter are listed at the end of the chapter before References section.

Definitions

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

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

Chapter 4: Primary lateral sclerosis

Clinical Description

Neuropathology

Electrophysiology

MRI

Positron emission tomography

Differential Diagnosis

Neurodegeneration and Relation to Motor Neuron Disease

Genetics of Primary Lateral Sclerosis

Treatment

Chapter 5: Transverse myelitis in children and adults

Infectious Myelitis

Bacterial myelitis

Viral myelitis

Fungal myelitis

Parasitic

Noninfectious Myelitis

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

Chapter 6: Multiple sclerosis: Motor dysfunction

Epidemiology and Etiopathogenic Factors

Clinical motor dysfunction

MS Variants: AQP4, NMOSD, and MOGAD

Childhood MS

Clinical features

Pediatric NMO and MOG serology

MRI findings

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

Chapter 7: Tropical spastic paraparesis

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

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

Competing interests

Ethical committee request

Chapter 9: Synucleinopathies

Historical Perspective

Pure Autonomic Failure

Pathophysiology

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

Care team

Symptom management

Prognosis and phenoconversion

Multiple System Atrophy

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

Multidisciplinary approach

Motor symptoms

Nonmotor symptoms

Disease-modifying approaches (Fig. 9.4)

Lewy Body Disorders-Parkinson Disease and Dementia with Lewy Bodies

Imaging techniques

Autonomic testing

Others

Chapter 10: Amyotrophic lateral sclerosis

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

Disrupted RNA metabolism

SCA36

Defective axonal cytoskeletal and transport

DCTN1

Profilin-1

Other putative disease mechanisms

Defective autophagy

ER stress and impaired degradation (SOD1, OPTN).

Notes:

Prion-like spread.

Description based on print version record.

ISBN:

0323958559

9780323958554

0323988172

9780323988179

OCLC:

1427669421

Access Restriction:

Restricted for use by site license.