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Motor Neuron Disease: Biology and Management Edition. ed. [Kõva köide]

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  • Formaat: Hardback, 486 pages, kõrgus x laius: 235x155 mm, kaal: 1030 g, biography
  • Ilmumisaeg: 21-Dec-1994
  • Kirjastus: Springer-Verlag Berlin and Heidelberg GmbH & Co. K
  • ISBN-10: 3540196854
  • ISBN-13: 9783540196853
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  • Formaat: Hardback, 486 pages, kõrgus x laius: 235x155 mm, kaal: 1030 g, biography
  • Ilmumisaeg: 21-Dec-1994
  • Kirjastus: Springer-Verlag Berlin and Heidelberg GmbH & Co. K
  • ISBN-10: 3540196854
  • ISBN-13: 9783540196853
Teised raamatud teemal:
A systematic overview of the clinical, pathological and research aspects of motor neuron disease, which contains a description of the anatomy, physiology and toxicology of the motor system, a review of historical developments and a survey of current concepts and controversies.

Here is the first book to provide a comprehensive overview of the clinical, pathological, and research aspects of motor neuron disease (MND). The text contains all essential features of the anatomy, physiology, pharmacology and toxicology of the motor system, a full description of MND and its variants, as well as historical developments and a review of the current concepts and controversies. This book comes at a time of increasing interest in neurodegenerative disorders and MND in particular. It will prove a key reference book with an integrated overview of the field, and will be indispensable to practicing neurologists, researchers, and all those with an interest in MND.

Arvustused

"This book is worthwhile to every neurologist and particularly those interested in ALS. It is an interesting book to everybody involved in research on motor neuron diseases. it certainly is one of the better volumes on MND available." Acta Neurologica Belgica

1 Motor Neuron Disease: The Clinical Syndrome.- Classification of Motor
Neuron Diseases.- Spinal Muscular Atrophies.- Motor Neuron Disease
(Amyotrophic Lateral Sclerosis).- Clinical Features.- Other Clinical
Syndromes of MND.- Outcome of MND.- Possible Associations of MND with Other
Diseases.- Poliomyelitis.- Hexosaminidase Deficiency (GM2 Gangliosidosis).-
MND and Plasma Cell Dyscrasia.- Hyperparathyroidism and Thyrotoxicosis.-
AIDS.- Creutzfeldt-Jakob Disease.- Other Disorders.- Diagnosis and
Investigation.- Differential Diagnosis.- 2 Epidemiology of Motor Neuron
Disease.- Some Important Clinical Patterns of Motor Neuron
Disease/Amyotrophic Lateral Sclerosis (MND/ALS).- Frequency of MND/ALS and
its Determinants.- Prevalence Rate.- Incidence Rate.- Mortality Rate.-
High-Risk Foci.- Risk Factors.- Case-Control Studies.- Ecological Studies.-
Definite Risk Factors for MND/ALS.- Familial Aggregation of MND/ALS and
Separation of Genetic Entities.- Aetiological Hypotheses.- 3 Familial Motor
Neuron Disease.- The Case for Studying Familial Motor Neuron Disease: Rapid
Development of Techniques of Molecular Genetics and their Successful
Application.- The Problems: Few Families, Clinical and Pathological
Heterogeneity and Incomplete Penetrance.- Profile of Familial Motor Neuron
Disease.- Phenotypic Variation in FMND.- Neuropathology.- Linkage Analysis in
Familial Motor Neuron Disease.- What is Involved in Locating a Defective
Gene?.- Linkage to Chromosome 21 Markers.- Identification of Mutations in
FMND.- Mutations of Human Cu,Zn SOD in FMND.- The Significance of Mutations
of Cu,Zn SOD.- The Next Steps: Identification of other MND genes.-
Improvements in DNA Markers.- Choice of Families - Informative Families.-
Statistical Analysis.- Candidate Genes.- Other Motor Neuron Disorders: the
Spinal Muscular Atrophies.- Androgen Receptors and Typical Motor Neuron
Disease (ALS).- Summary and Future Prospects.- 4 Pathology of Motor System
Disorder.- Physiological Anatomy of the Motor System.- Axonal Transport in
the Motor System.- Macroscopic Pathology of ALS.- Microscopic Pathology of
ALS.- Motoneuron Loss.- "Senescent Changes" in ALS.- Cytoplasmic Inclusions
in ALS.- Proximal and Distal Axonopathy in ALS.- Tract Degeneration.-
Conclusions.- 5 The Pathology of Motor Neuron Disease.- General Autopsy and
Neuropathological Findings.- Motor Cortex.- Subcortical White Matter and
Cortical Pyramidal Tracts.- Central Grey Matter and Basal Ganglia.- Brainstem
and Cranial Nerve Nuclei.- Lower Motor Neurons and Corticospinal Tracts.-
Spinocerebellar Pathways.- Sensory Systems.- Autonomic Nervous System.-
Peripheral Nerve.- Muscle.- Intellect, the Frontal Lobe and Parkinsonism.-
Relation to Other Neuronal Degenerations.- 6 Alternative Approaches to the
Pathology of Motor Neuron Disease.- Animal Models in the Study of MND.-
Naturally Occurring Animal Models of MND.- Neurotoxic Substances.-
Neurotropic Viruses.- Axonal Sprouting and the Motor End Plate.- Axonal
Transport.- Slow Axonal Transport.- Fast Axonal Transport.- Axonal Transport
in MND.- Selective Neuronal Vulnerability in MND: Onuf's Nucleus and Other
Nuclei.- Conclusion.- 7 The Molecular Pathology of Motor Neuron Disease.- The
Cytoskeleton and Cytoskeletal Pathology in Neurodegenerative Disorders.-
Neurofilaments and Neurofilamentous Pathology in MND.- Ubiquitin, the Stress
Response and Neuronal Damage in MND.- The Ubiquitin Conjugation Pathway.-
Selection of Substrates for Conjugation and Degradation.- Ubiquitin-Protein
Ligase.- Protein Degradation and Regeneration of Free Ubiquitin.- Ubiquitin
and MND.- Morphological Features of Ubiquitin-Immunoreactive Inclusions in
MND.- Clinicopathological Correlations.- Pathogenic Significance of Ub-IR
Inclusions in MND.- Conclusions.- 8 Neurochemistry of Motor Neuron Disease.-
Excitatory Amino Acids in Plasma and CSF in MND.- Glutamate, Glutamate
Dehydrogenase (GDH) and Excitotoxicity.- N-Acetyl-Aspartyl-Glutamate (NAAG)
and MND.- Plasma Cysteine and Metabolism of Sulphur-Containing Compounds.-
Glycine and GABA.- Spinal Cholinergic Systems.- Monoamines.- Neuropeptides.-
Thyrotropin-Releasing Hormone (TRH).- Substance P.- Calcitonin Gene-Related
Peptide (CGRP).- Neurotrophic Factors and Motoneuronal Death.- Summary and
Conclusions.- 9 Calcium and Aluminium in the Chamorro Diet: Unlikely Causes
of Alzheimer-Type Neurofibrillary Degeneration on Guam.- Geology, Villages
and Water Sources of Guam and Rota.- Implications of These Findings.-
Conclusions.- 10 Toxicology of Motor Systems.- Mechanistic Basis of
Neuronotoxicity.- General Considerations.- Neuroexcitatory Amino Acid
Receptors in the Central Nervous System.- The Excitotoxic Hypothesis of
Neuronal Death.- Glutamate as an Endogenous and Exogenous Factor in
Neurological Disease.- General Considerations.- Neurotoxicity of Exogenous
Glutamate.- Glutamate Metabolism and Neurological Disease.- Glutamate
Metabolism in Motor Neuron Disease.- Other Endogenous Factors:
Sulphur-Containing Amino Acids.- Amino Acids Containing Oxidised Sulphur
Atoms.- Cysteine and Cystine.- Cystine and Glutamate Transport.- Cysteine,
Sulphate and Motor Neuron Disease.- Exogenous Factors.- General
Considerations.- Lathyrus sativus Seed.- Cycas circinalis Seed.- Nitschia
pungens.- Non-NMDA Receptors on Glia.- Summary.- 11 Theories of Causation.-
Toxins.- Ageing.- Trophic Factors.- Viruses.- Metabolic Abnormalities.-
Autoimmunity.- 12 Neurotrophic Factors and Neurodegeneration.- The
Neurotrophins.- Nerve Growth Factor.- Brain-Derived Neurotrophic Factor.-
Neurotrophins -3, -4, and -5.- Ciliary Neurotrophic Factor and Related
Cytokines.- Platelet-Derived Growth Factor.- The Fibroblast Growth Factors.-
Insulin-like Growth Factors.- Epidermal Growth factor.- Growth Factor
Receptors and Signal Transduction Pathways.- Neurotrophic Factors and
Neurodegeneration.- NGF and Alzheimer's Disease.- Motor Neuron Disease and
Neurotrophic Factors.- CNTF and Motor Neuron Disease.- BDNF, Motor Neurons
and Parkinson's Disease.- 13 Somatic Motoneurons and Descending Motor
Pathways. Limbic and Non-limbic Components.- Somatic Motoneurons in Spinal
Cord and Brainstem.- Somatic Motoneurons in the Spinal Cord.- Somatic
Motoneurons in the Brainstem.- Local Projections to Motoneurons.- Recurrent
Motoneuronal Axon Collateral Projections to Motoneurons.- Muscle Spindle
Afferent Projections to Motoneurons in Spinal Cord and Brainstem.-
Propriospinal Pathways.- Bulbospinal Interneurons Projecting to Motoneurons.-
Pathways Controlling Respiration and Abdominal Pressure.- Pathways Involved
in Micturition Control.- Descending Pathways of Somatic Motor Control
Systems.- The Medial Descending System.- The Lateral Descending System.-
Descending Pathways Involved in Limbic Motor Control Systems.- Pathways
Projecting Diffusely to the Spinal Gray Matter.- Projections from the
Mesencephalon to Caudal Brainstem and Spinal Cord.- Projections from the
Hypothalamus to Caudal Brainstem and Spinal Cord.- Projections from Amygdala
and Bed Nucleus of the Stria Terminalis to Caudal Brainstem and Spinal Cord.-
Projections from the Prefrontal Cortex to Caudal Brainstem and Spinal Cord.-
Conclusions.- The First Motor System.- The Second Motor System.- The Third
Motor System.- 14 Neurophysiological Changes in Motor Neuron Disease.-
Electromyography (EMG).- Fasciculations in MND.- Fibrillation Potentials and
Positive Sharp Waves.- Other Spontaneous Discharges in MND.- Motor Unit
Potentials in MND.- Fatigue, Decrement and Neuromuscular Jitter.- Motor
Conduction in MND.- Late Responses in Motor Conduction Studies.- Sensory
Conduction in MND.- Central Motor Conduction in MND.- Stages of the Disease:
EMG Criteria.- Electrophysiological Assessment in Treatment Trials.-
Differential Diagnosis of MND by its Electrophysiological Features.- 15
Clinical Pharmacology of Motor Neurons.- Trophic Factors and Motor Neurons.-
Neurotransmitters, Neuropeptides and Receptors in the Ventral Horn.-
Excitotoxins and Motor Neurons.- Assessment Tools for Drug Effects on Human
Motor Neurons.- Clinical Measurements.- Electrophysiology.- TRH and TRH
Analogues.- Trophic and Protective Effects of TRH on Motor Neurons.-
Neurophysiological Data.- Spasticity.- Drugs Used in Spasticity.-
Conclusion.- 16 The Management of Motor Neuron Disease.- Background.-
Epidemiology.- Who Looks After MND Patients?.- The Organisation of Services.-
The Hospital.- Keyworker.- Phase I: The Stage of Diagnosis.- The Diagnosis.-
"Treatment".- Prognosis.- Telling the Diagnosis.- Phase II: The Stage of
Deterioration Without Major Disability.- Phase III: The Phase of Major
Disability With Multiple Problems.- Bulbar Problems in Motor Neuron Disease.-
Salivary Dribbling (Drooling).- Dysphagia.- Choking.- Dysarthria and
Communication Problems.- Respiratory Failure.- Weight and Nutrition.-
Constipation.- Management of Constipation.- Pain.- Control of Pain.-
Decreasing Mobility.- Wheelchairs.- Leg Swelling.- Weakness of Muscles.-
Sleeping Problems.- Emotional Problems.- Equipment.- Phase IV: The Terminal
Stage.- Use of Narcotics.- Audit.- Appendix I. MND Table of
Responsibilities.- 17 Clinical Trial Methodology.- Measurement Techniques.-
Natural History.- Sample Size Determination.- The Problem of Drop-outs.-
Controls.- Statistical Analysis.- Regulatory Processes.- 18 New Therapeutic
Approaches: Rationale and Results.- Rationale and Results.- The Use of
Thyrotropin-Releasing Hormone (TRH).- Approaches to Counteract Excitatory
Amino Acids.- Treatment of Immunological Abnormalities.- Approaches to Trace
Metal Intoxication.- Antiviral Agents.- Enhancing Axonal Sprouting and
Regeneration.- Neurotrophic Factors.- Other Approaches.- Conclusion.- 19
Living with Motor Neuron Disease.