Myasthenia gravis and myasthenic disorders:
Saved in:
| Format: | Book |
|---|---|
| Language: | English |
| Published: |
New York, NY [u.a.]
Oxford Univ. Press
1999
|
| Subjects: | |
| Online Access: | Inhaltsverzeichnis |
| Physical Description: | XX, 310 S. Ill. |
| ISBN: | 0195129709 |
Staff View
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| 245 | 1 | 0 | |a Myasthenia gravis and myasthenic disorders |c ed. by Andrew G. Engel |
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Record in the Search Index
| _version_ | 1804127671039295488 |
|---|---|
| adam_text | CONTENTS
Part I Approach to Diseases of the
Neuromuscular Junction
1. ANATOMY AND MOLECULAR ARCHITECTURE OF
THE NEUROMUSCULAR JUNCTION 3
Andrew G. Engel
THE INNERVATION OF MUSCLE 5
THE PRESYNAPTIC REGION 6
THE NERVE TERMINAL 7
Giant Synaptic Vesicles • Coated Vesicles • Dense Core
Vesicles • Small Clear Synaptic Vesicles • Vesicular ACh
Uptake • Synaptic Vesicles Move to and Dock at the Active
Zones • Exocytotic Machinery • SNARE Complex • Other
Proteins Implicated in Exocytosis • Voltage Gated Ca2+ Channels at
the Active Zones • Voltage Gated K+Channels of the Presynaptic
Membrane • Endocytosis
THE SYNAPTIC SPACE 16
Synaptic Basal Lamina • Asymmetric AChE • Synaptic
Laminins • Agrin and Its Receptors and Effectors • The
ARIA/Neuregulin Signaling Pathway
THE POSTSYNAPTIC REGION 22
General Architecture of the Junctional Folds • AChRs on the
Junctional Folds • Functional Significance of the Junctional
Folds • Structure Function Correlations • Regulation of the
Distribution and Subunit Composition of AChR • AChR Synthesis
and Degradation • The Postsynaptic Cytoskeleton • The
Junctional Sarcoplasm
2. STRUCTURE AND KINETIC PROPERTIES
OF THE ACETYLCHOLINE RECEPTOR 40
Henry J. Kaminski and Robert L. Ruff
STRUCTURE OF THE AChR 40
SUBUNIT COMPOSITION OF THE AChR 41
LOCATION OF THE AGONIST BINDING
SITES ON THE AChR 41
xi
i Contents
DIFFERENCES BETWEEN THE ADULT
AND FETAL TYPE AChRS 44
AChR ISOFORMS IN MULTI INNERVATED
MUSCLE FIBERS 44
EXTRAOCULAR MUSCLES EXPRESS ADULT
AND FETAL TYPE AChRS 45
MECHANISMS OF ION PASSAGE 46
MICROSTRUCTURE OF AChR SUBUNITS 46
The Main Immunogenic Region of the AChR • The Ionic Pore Is
Formed by the Ml and M2 Segments • Altering the Fixed Charges
in the Segments Surrounding M2 Changes the Size of Single Channel
Currents • Possible Effect of the Amino Acid Sequence in M2 and
Other Segments on Channel Open Times and Conductance • The
Intracellular Segment Between M3 and M4 Is the Major Determinant
of the Difference in Gating Between the y and e Subunits
ARRANGEMENT OF THE SEGMENTS WITHIN
ASUBUNIT 51
CLINICAL IMPACT OF NATURALLY OCCURRING
AChR SUBUNIT MUTATIONS 51
REGULATION OF AChR EXPRESSION 52
AChR SUBUNIT EXPRESSION IN THE THYMUS 54
RELATIONSHIP OF THE AChR TO OTHER
POSTSYNAPTIC MEMBRANE PROTEINS 54
THE SAFETY FACTOR FOR NEUROMUSCULAR
TRANSMISSION 57
3. ELECTRODIAGNOSIS OF ENDPLATE DISEASE 65
C. Michel Harper, Jr.
REPETITIVE NERVE STIMULATION STUDIES 65
The Safety Margin of Neuromuscular Transmission • Repetitive
Compound Muscle Action Potentials • Pseudofacilitation vs True
Facilitation • Drug Effects • Methodological Considerations
NEEDLE ELECTROMYOGRAPHY 72
The Properties of Motor Unit Potentials • The MUP in Endplate
Diseases
SINGLE FIBER ELECTROMYOGRAPHY 73
Usefulness and Limitations • Stimulated SFEMG
ELECTROMYOGRAPHIC FINDINGS IN SPECIFIC
ENDPLATE DISEASES 73
Autoimmune Myasthenia Gravis • Lambert Eaton Myasthenic
Syndrome • Botulism • Congenital Myasthenic Syndromes
¦ : • .. ¦ .¦¦¦¦ I
I
Contents xiii
Part II Myasthenia Gravis
4. THE IMMUNOPATHOGENESIS OF MYASTHENIA GRAVIS 87
Reinhard Hohlfeld and Hartmut Wekerle
IMMUNOLOGICAL PRINCIPLES 88
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
Between B Cells and T Cells
THE ROLE OF THE THYMUS IN MYASTHENIA GRAVIS 96
Early Notions on the Role of the Thymus in MG • Myogenesis in
the Thymus and the Thymic Hypothesis of MG • Autoantigens and
Autoreactive Lymphocytes in the Human MG Thymus • The
Thymus in Experimental Autoimmune MG • The Thymus in a
Transgenic Model of Experimental MG • The Human MG Thymus
in the SCID Mouse Host • MG and Thymoma
B CELL RESPONSES IN MYASTHENIA GRAVIS 99
General Characteristics of Anti AChR Antibodies • Epitopes
Recognized by Anti AChR Antibodies • Seronegative Myasthenia
T CELL RESPONSES IN MYASTHENIA GRAVIS 102
Isolation of AChR Specific T Cell Lines • Epitopes Recognized by
AChR Specific T Cells
COOPERATION BETWEEN B CELLS AND T CELLS
IN MYASTHENIA GRAVIS 103
PATHOGENIC EFFECTS OF THE ANTI AChR
ANTIBODIES 104
5. EXPERIMENTAL AUTOIMMUNE MYASTHENIA GRAVIS:
INDUCTION AND TREATMENT 111
Jon M. Lindstrom
HISTORICAL PERSPECTIVE 111
Discovery of EAMG • Muscle Acetylcholine Receptors
• Neuronal Acetylcholine Receptors
PATHOLOGICAL MECHANISMS OF EAMG AT THE
NEUROMUSCULAR JUNCTION 113
Species Differences • EAMG in the Lewis Rat
SPECIFICITIES OF ANTIBODIES TO MUSCLE AChRS 120
The Main Immunogenic Region • Blocking Antibodies
SPECIFICITIES OF T HELPER CELLS IN EAMG 122
T Cell vs B Cell Epitopes • HLA Proteins Are Important in Antigen
Presentation to T Cells • Diverse T Cell Receptor Specificities React
with AChR Peptides
V Contents
EXPERIMENTAL SPECIFIC IMMUNOTHERAPIES FOR
MYASTHENIA GRAVIS 123
EAMG as a Model for the Therapy of Human MG • Anti B Cell
Therapy • Anti T Cell Therapy
FUTURE PROSPECTS 125
6. NATURAL HISTORY OF MYASTHENIA GRAVIS 131
David Grab
ONSET 132
OCULAR MANIFESTATIONS 132
Localized Ocular MG
GENERALIZED MYASTHENIA GRAVIS 135
Time Between Onset of Ocular and Generalized
Manifestations • Signs and Symptoms • Exacerbation
• Remission • Course • Serum Level of Antibody to
Acetylcholine Receptor • Pregnancy • Mortality
MYASTHENIA GRAVIS IN CHILDREN 140
Transient Neonatal Myasthenia Gravis • Congenital Myasthenic
Syndromes • Juvenile Myasthenia Gravis
FAMILIAL INCIDENCE OF AUTOIMMUNE
MYASTHENIA GRAVIS 141
DISORDERS ASSOCIATED WITH MYASTHENIA GRAVIS 141
Thymoma • Hyperthyroidism • Other Autoimmune Diseases
i
I
7. DIAGNOSIS OF MYASTHENIA GRAVIS 146
Marjorie E. Seybold
CLINICAL DIAGNOSIS 146
DIFFERENTIAL DIAGNOSIS 148
Ptosis • Diplopia • Dysarthria and Dysphagia • Respiratory
Muscle Weakness • Fatigue
TESTS FOR MYASTHENIA GRAVIS 150
Pharmacological Tests • Electromyography • Acetylcholine
Receptor Antibody Tests • Other Diagnostic Tests • Comparison
of Available Tests
CRITERIA PROPOSED FOR THE DIAGNOSIS
OF MYASTHENIA GRAVIS 158
Definite MG with Typical Presentation • Definite MG with Atypical ;
Presentation • Probable MG with Typical Presentation • Probable ]
MG with Atypical Presentation • Possible MG with Typical J
Presentation • Possible MG with Atypical Presentation
• Suspected MG with Atypical Presentation
i
. li
Contents XV
FURTHER WORK UP 160
Laboratory Tests • Imaging Studies • Pulmonary Function
Studies
8. TREATMENT OF MYASTHENIA GRAVIS 167
Marjorie E. Seybold
SYMPTOMATIC TREATMENT 168
Cholinesterase Inhibitors (Anti AChE Medications) • Other
Medications • Other Supportive Measures • Adverse Effects of
Medications on MG
IMMUNOLOGICAL INTERVENTIONS 173
Thymectomy • Corticosteroids • Azathioprine •
Cyclosporine • Cyclophosphamide • Plasmapheresis
• Intravenous Immunoglobulin # Radiation • Antilymphocyte
Globulin and Antithymocyte Globulin
ALTERNATIVES FOR SPECIFIC CLINICAL SITUATIONS 187
Ocular MG • Mild Generalized MG • Severe Generalized MG
• Myasthenic Crisis • Thymoma • Pregnancy • Neonatal MG
• Childhood and Juvenile MG • Seronegative MG
• Penicillamine Induced MG
Part III Myasthenic Syndromes and Related Disorders
9. THE LAMBERT EATON MYASTHENIC SYNDROME 205
John Newsom Davis and Bethan Lang
CLINICAL FEATURES 206
General Characteristics • Cancer Associations • Associated
Paraneoplastic Syndromes • Epidemiology
PATHOLOGY 208
Electrophysiological Abnormalities • Pharmacological
Abnormalities • Morphological Abnormalities
PATHOGENESIS 212
The Peptide Hypothesis • Clinical Immunological
Associations • Clinical Evidence for a Humoral Factor • Passive
Transfer of Immunoglobulins: Physiological and Pharmacological
Effects • Passive Transfer of Immunoglobulins: Morphological
Effects • Functional Effects of Immunoglobulins on Cultured
Cells
VOLTAGE GATED CALCIUM CHANNELS
AS PUTATIVE ANTIGENS 215
VGCC Subtypes • Animal Model • Anti VGCC Antibodies:
Western Blotting ¦ Anti VGCC Antibodies: Radioimmuno
assays * Anti VGCC Antibodies: Autonomic Studies
VI Contents
SYNAPTOTAGMIN AS A PUTATIVE ANTIGEN 219
SMALL CELL LUNG CANCER AND
THE ETIOLOGY OF LEMS 220
DIAGNOSIS 221
MANAGEMENT 221
Pharmacological Treatment • Specific Cancer
Therapy • Immunological Treatment
10. NEUROMYOTONIA AND ANTIGANGLIOSIDE
ASSOCIATED NEUROPATHIES 229 I
Ian Hart, Angela Vincent, and Hugh Willison
NEUROMYOTONIA 230
Clinical Features • Electrophysiology • Morphologic
Features • Differential Diagnosis • Pathogenesis • Treatment
ANTIGANGLIOSIDE ANTIBODIES AND DISORDERS
OF MOTOR NERVE FUNCTION 239
Clinical Features • Nature and Distribution of
Gangliosides • Pathophysiological Effects of Antibodies to ]
Gangliosides
11. CONGENITAL MYASTHENIC SYNDROMES 251 f
Andrew G. Engel, Kinji Ohno, and Steven At. Sine
MECHANISMS COMPROMISING THE SAFETY MARGIN
OF NEUROMUSCULAR TRANSMISSION 252
THE CLASSIFICATION OF CONGENITAL
MYASTHENIC SYNDROMES 252
THE INVESTIGATION OF CONGENITAL f
MYASTHENIC SYNDROMES 252
Clinical Observations • Morphologic Studies • 125I a Bgt
Binding Sites per Endplate • In Vitro Electrophysiological
Studies • Molecular Genetic Studies
PRESYNAPTIC CONGENITAL MYASTHENIC SYNDROMES 259
Paucity of Synaptic Vesicles and Reduced Quantal Release • Defect
in Acetylcholine Resynthesis or Packaging • Congenital Myasthenic
Syndrome Resembling Lambert Eaton Syndrome
CONGENITAL MYASTHENIC SYNDROME
DUE TO A SYNAPTIC DEFECT 264
Endplate Acetylcholinesterase Deficiency
POSTSYNAPTIC CONGENITAL MYASTHENIC SYNDROMES 273 ;
Increased Response to ACh: Slow Channel Syndromes * Decreased ]j
Response to ACh: The Low Affinity Fast Channel I
Contents XVii
Syndromes • Decreased Response to ACh: Fast Channel Syndrome
Due to a Gating Abnormality • Decreased Response to ACh: Mode
Switching Kinetics • AChR Deficiency Caused by Recessive
Mutations in AChR Subunits
E INCOMPLETELY CHARACTERIZED SYNDROMES 290
I Familial Limb Girdle Myasthenia • Benign CMS in Iraqi and
f Iranian Jews with Facial Malformations
INDEX 299
t
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| spelling | Myasthenia gravis and myasthenic disorders ed. by Andrew G. Engel New York, NY [u.a.] Oxford Univ. Press 1999 XX, 310 S. Ill. txt rdacontent n rdamedia nc rdacarrier Myasthenia Gravis Myasthenia gravis Neuromuscular Diseases Myasthenie idszbz Myasthenie (DE-588)4170913-5 gnd rswk-swf Myasthenia gravis (DE-588)4075144-2 gnd rswk-swf Myasthenia gravis (DE-588)4075144-2 s 1\p DE-604 Myasthenie (DE-588)4170913-5 s 2\p DE-604 Engel, Andrew G. Sonstige (DE-588)136095585 oth HBZ Datenaustausch application/pdf http://bvbr.bib-bvb.de:8991/F?func=service&doc_library=BVB01&local_base=BVB01&doc_number=008839173&sequence=000002&line_number=0001&func_code=DB_RECORDS&service_type=MEDIA Inhaltsverzeichnis 1\p cgwrk 20201028 DE-101 https://d-nb.info/provenance/plan#cgwrk 2\p cgwrk 20201028 DE-101 https://d-nb.info/provenance/plan#cgwrk |
| spellingShingle | Myasthenia gravis and myasthenic disorders Myasthenia Gravis Myasthenia gravis Neuromuscular Diseases Myasthenie idszbz Myasthenie (DE-588)4170913-5 gnd Myasthenia gravis (DE-588)4075144-2 gnd |
| subject_GND | (DE-588)4170913-5 (DE-588)4075144-2 |
| title | Myasthenia gravis and myasthenic disorders |
| title_auth | Myasthenia gravis and myasthenic disorders |
| title_exact_search | Myasthenia gravis and myasthenic disorders |
| title_full | Myasthenia gravis and myasthenic disorders ed. by Andrew G. Engel |
| title_fullStr | Myasthenia gravis and myasthenic disorders ed. by Andrew G. Engel |
| title_full_unstemmed | Myasthenia gravis and myasthenic disorders ed. by Andrew G. Engel |
| title_short | Myasthenia gravis and myasthenic disorders |
| title_sort | myasthenia gravis and myasthenic disorders |
| topic | Myasthenia Gravis Myasthenia gravis Neuromuscular Diseases Myasthenie idszbz Myasthenie (DE-588)4170913-5 gnd Myasthenia gravis (DE-588)4075144-2 gnd |
| topic_facet | Myasthenia Gravis Myasthenia gravis Neuromuscular Diseases Myasthenie |
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