Atjaunināt sīkdatņu piekrišanu

Myasthenia Gravis and Myasthenic Disorders 2nd Revised edition [Hardback]

Edited by (McKnight-3M professor of Neuroscience and Professor of Neurology, McKnight-3M professor of Neuroscience and Professor of Neurology, Director, Neuromuscular Disease laboratory, Mayo Clinic, Rochester, MN)
  • Formāts: Hardback, 304 pages, height x width x depth: 180x254x20 mm, weight: 930 g
  • Sērija : Contemporary Neurology Series
  • Izdošanas datums: 19-Apr-2012
  • Izdevniecība: Oxford University Press Inc
  • ISBN-10: 019973867X
  • ISBN-13: 9780199738670
Citas grāmatas par šo tēmu:
  • Hardback
  • Cena: 246,54 €
  • Grāmatu piegādes laiks ir 3-4 nedēļas, ja grāmata ir uz vietas izdevniecības noliktavā. Ja izdevējam nepieciešams publicēt jaunu tirāžu, grāmatas piegāde var aizkavēties.
  • Daudzums:
  • Ielikt grozā
  • Piegādes laiks - 4-6 nedēļas
  • Pievienot vēlmju sarakstam
  • Bibliotēkām
    • Oxford Medicine Online e-books
  • Formāts: Hardback, 304 pages, height x width x depth: 180x254x20 mm, weight: 930 g
  • Sērija : Contemporary Neurology Series
  • Izdošanas datums: 19-Apr-2012
  • Izdevniecība: Oxford University Press Inc
  • ISBN-10: 019973867X
  • ISBN-13: 9780199738670
Citas grāmatas par šo tēmu:
Myasthenia Gravis and Myasthenic Disorders, Second Edition is a thoroughly re-written and updated version of the highly successful first edition published in 1999. The current edition begins with an overview of the anatomy and molecular architecture of the neuromuscular junction and the electrophysiologic diagnosis of its disorders. The introductory chapters are followed by a detailed exposition of the pathogenesis, natural history, diagnosis and therapy of the autoimmune myasthenias, the Lambert-Eaton myasthenic syndrome, and the increasingly complex and fascinating diseases collectively referred to as congenital myasthenic syndromes. The acetylcholine receptor is a major target of both autoimmune and inherited myasthenias and a separate chapter reviews its structural and functional properties. The book also describes disorders that often target terminal nerve segment near the neuromuscular junction-- the syndrome of peripheral nerve hyperexcitability and the Guillain-Barr? syndrome.

Comprehensively written by leaders at the forefront of research, not to mention thoroughly referenced throughout and gorgeously illustrated, this new edition of the classic 1999 text will cement its place as the text on Myasthenia Gravis and related disorders for years to come.

Recenzijas

Dr. Engel is a consummate investigator and master of the multiple sciences involved in understanding the causes and pathogenesis of myasthenia gravis and related syndromes. As editor of this encyclopedic book, he has gathered a stellar group of co-authors to cover every conceivable aspect of these diseases. The discussions will help students, clinicians and scientists who seek the details they need to understand the diseases and to deliver the best possible treatments. These diseases are among the best understood human autoimmune conditions. The antigens and antibodies are known, the immune system has been dissected, and treatment has improved to the point that almost no one now dies of what was once a lethal disease. * Lewis P. Rowland MD, Columbia University Medical Center, New York, NY * The second edition of Engel's Myasthenia Gravis and Myasthenic Disorders will be warmly welcomed by neurologists and the neuromuscular fraternity. This magnum opus is a masterwork, beautifully orchestrated by Andrew Engel, and covering the whole range of major advances in recent years. In addition to his own contribution in several chapters, he has assembled an impressive array of experts in the field and covered topics ranging from anatomy and molecular architecture of the neuromuscular junction in his own opening chapter, through diagnostic techniques and special chapters on Congenital Myasthenic Syndromes and Lambert-Eaton syndrome, to advances in therapy. It should continue to maintain its position as a pre-eminent guide and reference for this important field. * Victor Dubowitz, Neuromuscular Disorders *

Contributors xv
1 The Anatomy And Molecular Architecture Of The Neuromuscular Junction
1(36)
Andrew G. Engel
Basic Concepts
1(2)
The Innervation Of Muscle
3(1)
The Presynaptic Region
3(1)
The Nerve Terminal
4(11)
Giant Synaptic Vesicles
Coated Vesicles
Dense-core Vesicles
Small Clear Synaptic Vesicles
Vesicular ACh Uptake
Synaptic Vesicle Pools
Synaptic Vesicles Move to and Dock at the Active Zones
Exocytotic Machinery
SNARE Complex
Priming of Docked Synaptic Vesicles
Synaptotagmin-1
Steps in Exocytosis
Other Proteins Modulating Exocytosis
Presynaptic Cytoskeletal Components
The Active Zone and the Voltage-Gated Ca2' Channels
Voltage-gated K' Channels of the Presynaptic Membrane
Endocytotic Events and the Formation of New Synaptic Vesicles
The Synaptic Space
15(1)
Synaptic Basal Lamina
Asymmetric AChE
Collagen XIII
Synaptic Laminins
The Postsynaptic Region
16(9)
The Junctional Folds and Their Cytoskeletal Components
AChRs on the Junctional Folds
The Saturating Disk Model of Neuromuscular Transmission
Regulation of the Distribution and Subunit Composition of AChR
The Postsynaptic Cytoskeleton
The Junctional Sarcoplasm
AChR Synthesis and Degradation
Signaling Mechanisms At The Neuromuscular Junction
25(12)
Agrin, MuSK, and LRP4
Dok-7 and Its Downstream Activators Crk, Crk-L, and Tid 1
The Neuregulin/ARIA Signaling Pathway
2 Electrodiagnosis Of Myasthenic Disorders
37(23)
C. Michel Harper
Introduction
37(1)
Standard Motor Nerve Conduction Studies
38(1)
Repetitive Compound Muscle Action Potentials
Repetitive Nerve Stimulation Studies
39(7)
The Safety Margin of Neuromuscular Transmission
Pseudofacilitation vs. True Facilitation
Drug Effects
Choice of Muscles and Stimulation Sites
Troubleshooting Technical Problems
Special Considerations in Infants and Young Children
What Constitutes an Adequate Repetitive Stimulation Examination?
Needle Electromyography
46(1)
The Properties of Motor Unit Potentials
MUP Changes Observed in Myasthenic Disorders
Single-Fiber Electromyography
Electrodiagnostic Findings In Specific Myasthenic Disorders
47(13)
Autoimmune Myasthenia Gravis
Lambert-Eaton Syndrome
Botulism
Congenital Myasthenic Syndromes
3 The Immunopathogenesis Of Myasthenia Gravis
60(30)
Reinhard Hohlfeld
Hartmut Wekerle
Alexander Marx
Introduction
60(1)
Immunological Principles
61(9)
Basic Properties of B Cells and T Cells
The Intrathymic Differentiation of T Cells
Tolerance and Autoimmunity
Antigen Recognition by B Cells and Antibodies
Antigen Recognition by T Cells
Cooperation of B Cells and Helper T Cells
Myasthenia With Thymic Hyperplasia
70(4)
Early Notions on the Role of the Thymus in MG
Intrathymic Myoid Cells and AChR
Key Tenets of "Intrathymic Pathogenesis"
Intrathymic Antigen Presentation
Thymic Autoantibody Production
Intrathymic Germinal Centers and B Cells
Human MG Thymus Grafted into SCID Mice
Two-Step Model of Intrathymic Autosensitization
"Promiscuous" Expression of Autoantigens by Medullary Thymic Epithelial Cells
Role of Regulatory T Cells (Treg)
Possible Role of Viruses
Myasthenia Gravis With Thymoma
74(4)
Epidemiology
Classification of Thymomas
Thymoma Features Associated with MG
Autoantibodies in Thymoma-Associated MG
Pathogenesis of Thymoma-Associated MG
Models of Tolerance Breakdown in TAMG
B Cell Responses In Myasthenia Gravis
78(3)
General Features of Anti-AChr Antibodies
Epitopes Recognized by Anti-AChR Antibodies
Anti-Striational Antibodies
"MuSK-positive" and "Seronegative" Myasthenia
T Cell Responses In Myasthenia Gravis
81(1)
AChR-Specific T Cells
Antigenic Epitopes Recognized by AChR-Specific T Cells
Cooperation Between B Cells And T Cells In Mg
82(1)
Pathogenic Effects Of The Anti-Achr Antibodies
83(1)
Summary
84(6)
4 Natural History Of Myasthenia Gravis
90(18)
Vladimir Luchanok
Henry J. Kaminski
The History And "Natural" History Of Myasthenia Gravis
90(2)
Epidemiology
92(1)
Onset
92(1)
Ocular Myasthenia
93(1)
Generalized Myasthenia Gravis
94(5)
Time Between Onset and Generalized Manifestations
Signs and Symptoms
Exacerbation
Remission
Course
Mortality
Specific Clinical Situations
99(1)
MuSK-Myasthenia Gravis
Pregnancy Neonatal Myasthenia Gravis
Age-Related Issues For Myasthenia Gravis
100(1)
Familial Incidence Of Myasthenia Gravis
101(1)
Disorders Associated With Myasthenia Gravis
101(1)
Thymoma
Thyroid Disorders
Other Autoimmune Diseases
Summary
102(6)
5 The Diagnosis Of Myasthenia Gravis And Other Disorders Of Neuromuscular Transmission
108(22)
James F. Howard, Jr.
Introduction And Historical Background
108(1)
Clinical Presentation
109(2)
Myasthenia Gravis
MG Caused by Antibodies to Muscle-Specific Protein Kinase (MuSK-MG)
Physical Findings
111(2)
Myasthenia Gravis
MuSK-MG
Differential Diagnosis
113(3)
Differential Diagnosis of Ptosis
Differential Diagnosis of Diplopia
Differential Diagnosis of Dysarthria and Dysphagia
Differential Diagnosis of Isolated Respiratory Muscle Weakness
Differential Diagnosis of Fatigue
Differential Diagnosis of Mimicking Disorders
Diagnostic Studies For Myasthenia Gravis
116(14)
Edrophonium Test
Antibody Tests
Electrodiagnostic Testing
Ocular Cooling (Ice-Pack Test)
Comparison of Diagnostic Techniques
Other Diagnostic Tests
6 Therapy Of Myasthenia Gravis
130(26)
Daniel B. Drachman
Introduction
130(1)
Pathogensis Of Mg
130(1)
Immune Pathogenesis of MG
Origin Of Mg
131(1)
Treatment Of Mg
132(13)
Preliminaries
Treatment Strategies
Treating Mg In Special Situations
145(3)
Ocular Myasthenia
Management of Associated Conditions Co-morbidities
Drug Interactions Pregnancy and Neonatal MG
Myasthenic Crisis
Future Prospects For Treatment Of Mg
148(2)
The Paradox of Difficulty in Obtaining Approval of MG Treatments
Conclusions
150(6)
7 The Lambert--Eaton Myasthenic Syndrome
156(17)
Vern C. Juel
Donald B. Sanders
History
156(1)
Clinical Features
157(1)
Symptoms
Physical findings
Cancer-Associated Lems (Ca-Lems)
157(1)
Non-Cancer Associated Or Autoimmune Lems (Nca-Lems)
158(1)
Epidemiology
159(1)
Pathophysiology And Immunopathology
159(2)
Electrodiagnostic Findings
161(4)
Repetitive Nerve Stimulation Testing Needle Electromyography (EMG)
Single-fiber Electromyography
Electromyographic Quantitation of severity
Serological Testing
165(1)
Voltage-gated Calcium Channel Antibodies
SOXI Antibodies
Other Tests
166(1)
Differential Diagnosis
166(1)
Mg/Lems Overlap
167(1)
Treatment
167(1)
Cancer
Symptomatic Treatment
Immunotherapy
Agents That May Worsen Lems
168(1)
Prognosis
169(1)
Summary
169(4)
8 Congenital Myasthenic Syndromes
173(58)
Andrew G. Engel
Xin-Ming Shen
Kinji Ohno
Steven M. Sine
A Brief History Of The Congenital Myasthenic Syndromes
173(1)
Mechanisms Compromising The Safety Margin Of Neuromuscular Transmission
174(1)
The Classification Of Congenital Myasthenic Syndromes
174(1)
The Investigation Of Congenital Myasthenic Syndromes
174(7)
Clinical Observations
Morphology
125I-α-bgt Binding Sites per Endplate
In Vitro Electrophysiology Studies
Molecular Genetic Studies
Presynaptic Cms
181(5)
CMS Caused by Defects in Choline Acetyltransferase (ChAT)
Paucity of Synaptic Vesicles and Reduced Quantal Release
Congenital Myasthenic Syndrome Resembling the Lambert-Eaton Syndrome
Synaptic Basal-Lamina-Associated Cms
186(8)
Endplate Acetylcholinesterase Deficiency
Defects in β2-Laminin
Postsynaptic Cms Caused By Mutations In Achr
194(12)
Slow-Channel Syndromes
Fast-Channel Syndromes
AChR Deficiency Caused by Recessive Mutations in AChR Subunits
Escobar Syndrome
Cms Caused By Defects In Rapsyn
206(4)
Defects In Mechanisms Governing Endplate Development And Maintenance
210(4)
CMS Caused by Defects in Agrin
CMS Caused by Defects in MuSK
CMS Caused by Defects in Dok-7
CMS Caused by Defects in the Hexosamine Biosynthetic Pathway
Miscellaneous Rare Congenital Myasthenic Syndromes
214(17)
Sodium-Channel Myasthenia
CMS Caused by Plectin Deficiency
CMS Associated with Centronuclear Myopathy (CNM)
9 Endplate Acetylcholine Receptor, A Target For Myasthenic Disorders: Structural And Mechanistic Essentials
231(21)
Steven M. Sine
Andrew G. Engel
Introduction
231(1)
Achr Structure
231(7)
AChR Structure at the Atomic Scale
Pore Domain
Ligand-binding Domain
Interface Between Ligand-binding and Pore Domains
Mechanism Of Achr Activation
238(5)
Intermediate State Between Closed and Open States
Links Between Agonist Binding and Channel Opening
Achr Disease Mechanisms
243(5)
Slow-channel CMS
Fast-channel CMS
Perspective
248(4)
10 Peripheral Nerve Hyperexcitability Syndromes
252(13)
Srikanth Muppidi
Steven Vernino
Background
252(1)
Clinical And Electrodiagnostic Features Of Hyperexcitability Syndromes
253(2)
Pathophysiology
255(2)
Hyperexcitability of the Motor Nerve Terminal
Hyperexcitability of Neuromuscular Junction
Inherited Myokymia (Potassium-Channel Mutations)
257(1)
Autoimmune Pnii
257(3)
Neuromyotonia (Isaacs Syndrome)
Morvan Syndrome
Cramp-fasciculation Syndrome
Antibodies against Voltage-gated Potassium Channel Complex Proteins
Other Antibodies
Pnii Secondary To Toxins
260(1)
Hyperexcitability Due To Peripheral Nerve Disorders
261(1)
Other Peripheral Hyperexcitability Syndromes
261(1)
Treatment Options For Hyperexcitability Syndromes
262(1)
Summary
262(3)
11 Effects Of Anti-Ganglioside Antibodies At The Neuromuscular Junction
265(14)
Jaap J. Plomp
Hugh J. Willison
Neuronal Gangliosides
265(2)
Structure and Synthesis
Localization
Physiological Functions
Peripehral Nerve Disorders Associated With Anti-Ganglioside Antibodies
267(2)
Experimental Evidence For Anti-Ganglioside Antibody-Mediated Attack Of Nmjs
269(2)
Clinical Evidence For Nmj Synaptopathy In Anti-Ganglioside Antibody-Mediated Human Neuropathy
271(1)
Comparing Clinical And Experimental Electrophysiological Findings
272(1)
Therapeutic Considerations
273(6)
Index 279
Dr. Andrew Engel is the McKnight-3M Professor of Neuroscience and Professor of Neurology, Mayo Clinic College of Medicine as well as the Director of the Neuromuscular Disease laboratory at the Mayo Clinic, Rochester, MN campus. He earned his medical degree at McGill University, was trained in Neurology at the NIH and the Mayo Clinic and in Neuropathology at Columbia University and has spent his entire professional carrier at the Mayo Clinic. Dr Engel has had a longstanding interest in neuromuscular diseases and especially those affecting neuromuscular transmission.