Peripheral Neuropathy: 2-Volume Set

Contributing Authors ......Page 2
Preface ......Page 13
Segmental Demyelination and Remyelination ......Page 15
Development of Peripheral Nerve Fibers ......Page 16
References ......Page 18
Gross Anatomy of the Peripheral Nervous System ......Page 21
Gross Anatomy ......Page 28
References ......Page 42
Microscopic Anatomy of the Peripheral Nervous System ......Page 44
General Organization of Myelinated Axons ......Page 54
The Paranode-node-paranode Region ......Page 60
The Schwann Cell ......Page 69
Historical Background ......Page 71
Development ......Page 73
General Organization ......Page 74
Morphometry ......Page 75
General Form ......Page 76
Fiber Transition ......Page 77
Tissue Composition ......Page 80
Development ......Page 84
Mechanical Features ......Page 86
References ......Page 88
Channel Function in Mammalian Axons and Support Cells ......Page 101
2 ......Page 105
K ......Page 110
References ......Page 114
Early Excitability Studies ......Page 119
Nerve Excitability and Nerve Conduction Studies Compared ......Page 120
Currently Used Measures of Nerve Excitability and Their Biophysical Basis ......Page 121
Nerve Excitability Measures and Membrane Potential ......Page 125
Modality and Regional Differences Between Different Axons ......Page 127
Changes in Axonal Membrane Potential in Peripheral Neuropathies ......Page 128
References ......Page 132
The Muscle Spindle ......Page 136
Muscle Spindles of the Cat Soleus ......Page 137
Abundance of Muscle Spindles and Afferent Axons ......Page 140
Development of Muscle Spindles ......Page 141
Intrafusal Muscle Fibers ......Page 142
Sensory Innervation ......Page 143
Motor Innervation ......Page 149
References ......Page 152
Tendon Organ Structure ......Page 156
Physiologic Responses ......Page 158
Transduction ......Page 161
Recovery from Nerve Lesions ......Page 162
Central Projections and Proprioception ......Page 163
References ......Page 164
Overview ......Page 167
Afferent Receptive Properties ......Page 169
Electrical Membrane Properties of Drg Neurons ......Page 174
Chemical Phenotype of Drg Neurons ......Page 175
Transmitters, Neuromodulators, Peptides, and Their Receptors ......Page 188
Membrane Receptors and Sensory Transduction ......Page 190
K ......Page 193
Summary ......Page 195
References ......Page 196
The Pupil ......Page 207
References ......Page 217
Organization of Cardiac Vagal and Sympathetic Nervous System ......Page 220
Neurochemical Transmission in the Cns ......Page 223
Chemical Transmission at the Cardiac Neuroeffector Junction ......Page 225
Communication Between the Cardiac Sympathetic and Parasympathetic Systems ......Page 227
Assessing Cardiac Neural Drive: Physiologic Outcome ......Page 228
Impairment of Cardiac Neural Control: Clinical Outcome ......Page 229
References ......Page 230
Overview ......Page 251
Historical Perspective ......Page 252
Ens Conceptual Model ......Page 253
Histoanatomy ......Page 254
Enteric Neuronal Morphology ......Page 255
Enteric Sensory Neurons ......Page 256
Interneurons ......Page 258
Enteric Motor Neurons ......Page 259
Disinhibitory Motor Disorders ......Page 261
Neuronal Electrical Behavior ......Page 263
Synaptic Transmission ......Page 265
Conclusions ......Page 273
References ......Page 274
Embryology ......Page 280
Mechanisms of Continence and Defecation ......Page 286
Anorectal Dysfunction in Neurologic Disorders ......Page 287
Management of Fecal Incontinence ......Page 293
References ......Page 294
Anatomy ......Page 300
Innervation ......Page 302
Anatomy of Central Nervous Pathways Controlling the Lower Urinary Tract ......Page 307
Neurogenic Dysfunction of the Lower Urinary Tract ......Page 314
Emission-ejaculation ......Page 317
Central Reflex Pathways ......Page 318
References ......Page 319
Baroreflexes ......Page 324
Direct Measurement of Sympathetic Traffic in Humans Via Microneurography ......Page 325
Baroreceptor Control of Msna ......Page 326
Msna Responses to Orthostatic Challenge ......Page 327
Msna Responses to Chemoreceptor Activation ......Page 328
Msna and Autonomic Responses to Exercise ......Page 329
Autonomic and Msna Responses to Mental Stress ......Page 332
Aging, Hypertension, and Congestive Heart Failure ......Page 333
Autonomic Responses to Body Heating ......Page 334
References ......Page 335
Outline of Schwann Cell Development ......Page 339
Differentiation Markers Associated with Embryonic Schwann Cell Development ......Page 341
Neural Crest Origins of Schwann Cells ......Page 344
Schwann Cell Precursors ......Page 346
Developmental Potential of Early Peripheral Glia ......Page 347
Importance of Neuregulin-1 in Schwann Cell Development ......Page 349
Neuregulin-1 Signaling Pathways in Schwann Cells ......Page 350
Regulation of the Precursor–schwann Cell Transition ......Page 351
Signals That Control Schwann Cell Survival ......Page 352
Cell Culture Studies on Schwann Cell Proliferation and Differentiation ......Page 354
Transcription Factors Involved in Myelination ......Page 355
Signaling Pathways and Growth Factors That Regulate Myelination ......Page 359
Neural Activity and Schwann Cell Development ......Page 360
References ......Page 363
Neurotrophic Factors in the Peripheral Nervous System ......Page 375
Neurotrophin Families ......Page 376
Neurotrophin Signaling ......Page 379
References ......Page 381
Axonal Transport: Properties, Mechanisms, and Role in Nerve Disease ......Page 385
Theoretical Implications of Transport Abnormalities ......Page 390
Evidence for Transport Abnormalities in Human Neuropathy ......Page 392
, ......Page 396
Genetically Based Animal Neuropathies ......Page 398
Conclusion ......Page 400
References ......Page 401
Myelination ......Page 408
References ......Page 422
Cytology of the Axon ......Page 429
Does Axonal Caliber Influence Myelination? ......Page 432
References ......Page 439
Growth Cones and Cytoskeletal Dynamics ......Page 443
Axon Guidance in Development ......Page 449
Axon Guidance in Disease ......Page 461
Acknowledgments ......Page 465
References ......Page 466
Aging in the Peripheral Nervous System ......Page 478
Conclusions ......Page 495
References ......Page 496
Free Radical Biology ......Page 503
Diabetic Neuropathy ......Page 505
Aspartate and Glutamate Receptors ......Page 518
References ......Page 521
Transgenic Models of Nerve Degeneration ......Page 528
Transgenic Models Related to the Organization of the Node of Ranvier ......Page 542
References ......Page 545
Introduction to Immune Reactions in the Peripheral Nervous System ......Page 550
Tolerance and Autoimmunity ......Page 558
Breakdown of Tolerance ......Page 559
References ......Page 560
Immune Responses in Peripheral Nerve ......Page 564
Molecular Mimicry and Microbial Antigens ......Page 581
Pathophysiologic Relevance of Autoantigens in Human Neuropathies ......Page 585
References ......Page 589
Experimental Autoimmune Neuritis ......Page 600
Experimental Treatments in Ean ......Page 614
References ......Page 619
Principles of Immunotherapy ......Page 625
References ......Page 634
Blood-nerve Interface and Endoneurial Homeostasis ......Page 639
Dynamics of Blood-nerve Exchange ......Page 640
Endoneurial Fluid Dynamics ......Page 643
Endoneurial Homeostasis ......Page 645
Factors Regulating Bni Permeability ......Page 648
Therapeutic Implications for Peripheral Neuropathies ......Page 649
References ......Page 650
Unique Aspects of Special Physiology of Nerve Microvasculature ......Page 654
Regulation of Nerve Blood Flow ......Page 655
Pathogenesis of Nerve Ischemia ......Page 663
References ......Page 664
Pathology of Peripheral Neuron Cell Bodies ......Page 668
Biochemical Alterations ......Page 674
Neuron Death ......Page 680
Lysosomal Storage Disorders ......Page 691
Neurologic Mutants ......Page 695
References ......Page 702
Role of Pathologic Study of Peripheral Nerves ......Page 718
Postmortem Spinal Cord and Nerve Removal and Processing ......Page 724
Morphometry of Neuron Somas ......Page 739
Morphometry of Peripheral Nerves ......Page 741
Normal Numbers of Fibers in Control Human Nerves ......Page 748
Artifacts of Surgical Removal and of Histologic Preparation ......Page 750
Debris Removal from the Endoneurium ......Page 776
Interstitial Pathology of Nerves ......Page 778
Perineurial Pathology ......Page 795
Parenchymatous Pathologic Alterations ......Page 797
References ......Page 809
Diseases of the Neuromuscular Junction ......Page 815
Myasthenia Gravis ......Page 817
Lambert-eaton Myasthenic Syndrome ......Page 818
Paucity of Synaptic Vesicles and Reduced Quantal Release ......Page 825
Cms with Reduced Quantal Release and Central Nervous System Symptoms ......Page 826
Cms Caused by Rapsyn Deficiency ......Page 839
Myasthenia Gravis ......Page 843
References ......Page 844
Overview ......Page 852
Anatomic Features of the Skin ......Page 853
Microscopy ......Page 859
Contributions of Skin Biopsy to Diagnosis ......Page 866
Cadasil ......Page 870
Research Uses of Skin Biopsy ......Page 871
References ......Page 874
Nerve Conduction and Needle Electromyography ......Page 879
Spontaneous Activity ......Page 921
Motor Unit Action Potential ......Page 927
References ......Page 938
Nerve Tests Expressed as Percentiles, Normal Deviates, and Composite Scores ......Page 950
Nerve Tests for Which Reference Values Can Be Set ......Page 951
Statistical Methods for Computing Normal Values* ......Page 952
7 Tests, and Other Composite Scores ......Page 955
Individual Attributes Versus Composite Scores of Nerve Conduction Abnormality: Sensitivity, Reproducibility, Representativeness, ......Page 956
Functional Abnormality Even When Test Values Fall Within the Range of Normal Values ......Page 958
Composite Scores in Genetic Linkage Analysis ......Page 960
References ......Page 962
Single-fiber Electromyography and Other Electrophysiologic Techniques for the Study of the Motor Unit ......Page 963
References ......Page 977
Technique of Microneurography ......Page 980
Sensory Innervation of the Skin ......Page 981
References ......Page 988
Compound Action Potentials of Sural Nerve in Vitro in Peripheral Neuropathy ......Page 992
Fiber Groups and Sensation ......Page 993
Comments ......Page 1003
References ......Page 1005
Overview ......Page 1006
History of Scoring Neurologic Signs ......Page 1007
Definitions ......Page 1008
End Points to Be Scored ......Page 1009
Scoring Muscle Weakness (and Strength) ......Page 1011
Quantitating Sensory Loss ......Page 1012
Muscle Twitch ......Page 1013
Handicap ......Page 1023
References ......Page 1024
Health Outcomes and Quality of Life ......Page 1027
The Concept of Quality of Life ......Page 1028
How Should Quality of Life Be Measured? ......Page 1029
Requirements of Health Outcome Measures ......Page 1030
Assessment of Quality of Life in Diseases Associated with Peripheral Neuropathy ......Page 1032
References ......Page 1034
Uses of Quantitative Sensation Testing ......Page 1037
Assessing Sensation and Vision and Hearing ......Page 1038
Clinical Assessment Versus Qst ......Page 1039
Criteria and Standards for L-qst Using Natural Stimuli ......Page 1042
Touch-pressure Sensation ......Page 1051
Vibration Sensation ......Page 1053
Thermal Sensation ......Page 1058
Qst in Medical Practice ......Page 1062
Qst in Research ......Page 1064
References ......Page 1066
Clinical Examination ......Page 1068
Instrumented Strength Testing ......Page 1069
Intramuscular Pressure Measurement ......Page 1070
Acknowledgments ......Page 1072
References ......Page 1073
Indications for Autonomic Function Tests ......Page 1076
The Autonomic Reflex Laboratory ......Page 1078
Routine Tests of Autonomic Function ......Page 1079
Evaluation of Central Autonomic Pathways ......Page 1098
References ......Page 1100
Distribution and Patterns of Involvement ......Page 1107
Clinical Course ......Page 1111
References ......Page 1125
Diagnostic Yield in Assessment of Polyneuropathy ......Page 1132
Approaches to the Diagnosis of Neuropathy ......Page 1133
Family History ......Page 1139
References ......Page 1145
Epidemiologic Methods ......Page 1150
Clinical Applications: Results of Epidemiologic Studies ......Page 1153
References ......Page 1158
Overview ......Page 1160
Clinical Features ......Page 1164
Congenital Defects ......Page 1166
Effects of Trauma ......Page 1167
Neoplasms ......Page 1168
Vascular Diseases and Aneurysms ......Page 1170
Infections and Toxins ......Page 1171
References ......Page 1172
Anatomy and Physiology ......Page 1176
Trigeminal Nerve Lesions ......Page 1178
Investigation of Trigeminal Nerve Lesions ......Page 1181
Trigeminal Neuralgia ......Page 1182
Acknowledgments ......Page 1184
References ......Page 1185
Gross Anatomy ......Page 1187
Vascular Anatomy ......Page 1188
Neuroimaging ......Page 1189
Functional Anatomy ......Page 1190
References ......Page 1214
Age-related Changes of the Eighth Nerve ......Page 1221
Diagnostic Approach ......Page 1222
References ......Page 1236
Diseases of the Ninth, Tenth, Eleventh, and Twelfth Cranial Nerves ......Page 1241
References ......Page 1256
The Peripheral Nerve Involvement of Spinal Cord, Spinal Roots, and Meningeal Disease ......Page 1262
Overview ......Page 1263
Anatomic Considerations ......Page 1264
Sensory Syndromes ......Page 1274
Combined Motor and Sensory Syndromes ......Page 1277
References ......Page 1283
Gross Anatomy ......Page 1290
The Diagnosis of Radiculopathy ......Page 1291
Polyradiculopathy ......Page 1294
References ......Page 1301
Anatomy ......Page 1305
Anatomic Regions ......Page 1307
Specific Etiologies of Brachial Plexopathy ......Page 1319
References ......Page 1337
Anatomy ......Page 1340
Investigation ......Page 1342
References ......Page 1351
Mechanisms of Acute and Chronic Compression Neuropathy ......Page 1356
Ischemia and Increased Endoneurial Fluid Pressure ......Page 1361
References ......Page 1365
Overview of the Response to Injury ......Page 1368
Classification of Nerve Injuries ......Page 1369
Wallerian Degeneration: an Overview ......Page 1370
Acutely Denervated Schwann Cells ......Page 1373
Chronic Axotomy and Chronic Denervation ......Page 1375
Inflammation and Wallerian Degeneration ......Page 1378
Matrix Metalloproteinases and Chondroitin Sulfate Proteoglycans ......Page 1383
Neuropathic Pain ......Page 1384
Axonal Regrowth ......Page 1386
References ......Page 1390
Carpal Tunnel Syndrome ......Page 1399
Median Nerve Lesions in the Arm ......Page 1416
Anterior Interosseous Nerve Syndrome ......Page 1417
References ......Page 1418
Pathophysiology ......Page 1426
Medial Antebrachial Cutaneous Neuropathy ......Page 1446
References ......Page 1447
Pathophysiology ......Page 1450
Obturator Neuropathy ......Page 1451
Saphenous Neuropathy ......Page 1454
Gluteal Neuropathies ......Page 1456
Tarsal Tunnel Syndrome ......Page 1466
Sural Neuropathy ......Page 1470
References ......Page 1471
Overview ......Page 1474
Clinical Diagnosis ......Page 1475
Pain ......Page 1476
Principles of Operating upon the Nerves ......Page 1478
Iatropathic Nerve Injury ......Page 1479
Injuries of the Brachial Plexus ......Page 1484
Repair of the Brachial Plexus ......Page 1486
Obstetric Brachial Plexus Palsy ......Page 1488
References ......Page 1491
Definition and History ......Page 1496
Epidemiology ......Page 1497
Pathology ......Page 1500
Hypotheses of Causation ......Page 1501
Treatments ......Page 1502
References ......Page 1503
Mendelian and Mitochondrial Inheritance, Gene Identification, and Clinical Testing ......Page 1508
References ......Page 1522
Peripheral Myelin Protein 22 ......Page 1524
Myelin Protein Zero ......Page 1535
Early Growth Response Gene 2 ......Page 1538
Connexin 32 ......Page 1539
Periaxin ......Page 1541
References ......Page 1542
Sod1 Transgenic Models ......Page 1547
Neurofilament Transgenic Models ......Page 1550
Double Transgenic Models ......Page 1551
Animals Models in Screening for New Therapeutic Agents ......Page 1552
References ......Page 1553
Pathology, Epidemiology, Clinical Features, and Management ......Page 1556
Setx ......Page 1560
References ......Page 1561
Classification ......Page 1563
Diagnosis ......Page 1565
References ......Page 1578
Hereditary Motor and Sensory Neuropathies: an Overview of Clinical, Genetic, Electrophysiologic, and Pathologic Features ......Page 1582
Historical Overview ......Page 1583
Clinical Features ......Page 1590
Nerve Conduction and Electromyographic Features ......Page 1596
Hmsn I ......Page 1598
Hmsn Ii ......Page 1602
Course and Outcome ......Page 1610
References ......Page 1612
The Cmt1a Duplication and Hnpp Deletion ......Page 1618
Pmp22 Point Mutations as the Cause of Demyelinating Neuropathies ......Page 1624
Inherited Brachial Plexus Neuropathies ......Page 1629
Generation of the Cmt1a Duplication/hnpp Deletion by Unequal Crossing over ......Page 1630
Molecular Diagnostics for Detection of Cmt1a Duplication/hnpp Deletion ......Page 1631
References ......Page 1632
Historical Background ......Page 1640
The Mpz Molecule ......Page 1641
Management ......Page 1658
References ......Page 1660
Egr2 Function ......Page 1666
Egr2 Mutations and Myelinopathies ......Page 1667
Conclusions ......Page 1672
References ......Page 1673
Definition and Overview ......Page 1675
Hmsn Iva/cmt4a (gdap1) ......Page 1693
References ......Page 1702
Historical Overview ......Page 1710
Clinical Features ......Page 1711
Natural History ......Page 1713
Precipitating or Associated Factors ......Page 1714
Differential Diagnosis ......Page 1715
Laboratory Tests ......Page 1717
Pathologic Alterations of Biopsied Nerves ......Page 1718
Pathogenesis ......Page 1720
Molecular Genetics ......Page 1721
Treatment ......Page 1722
References ......Page 1723
Autosomal Recessive Hereditary Motor and Sensory Neuropathies ......Page 1725
Autosomal Recessive Hmsn Type I ......Page 1727
Autosomal Recessive Hmsn Type Ii ......Page 1734
References ......Page 1741
Mutations That Cause X-linked Charcot-marie-tooth Disease ......Page 1747
Neuromuscular Manifestations ......Page 1752
Other Manifestations ......Page 1753
Clinical Electrophysiology ......Page 1754
Genotype-phenotype Correlations ......Page 1755
References ......Page 1756
Multiple Endocrine Neoplasia, Type 2b ......Page 1761
Alimentary Tract Ganglioneuromatosis ......Page 1762
References ......Page 1763
Hsans: Clinical Features, Pathologic Classification, and Molecular Genetics ......Page 1765
Indifference to Pain ......Page 1767
Historical Overview of Hsan Classification ......Page 1769
Autosomal Dominant Hsan (type I) ......Page 1770
Mechanisms and Treatment of Acral Mutilation and Arthropathy ......Page 1794
References ......Page 1795
Lysosomal and Peroxisomal Disorders ......Page 1801
Sphingolipidoses ......Page 1803
Adrenoleukodystrophy and Adrenomyeloneuropathy ......Page 1822
Neonatal Adrenoleukodystrophy ......Page 1824
Infantile Refsum Disease ......Page 1828
References ......Page 1829
Biosynthesis of Heme ......Page 1839
Effects of Drugs on Porphyrin Metabolism ......Page 1840
Mechanism of Neuronal Damage ......Page 1841
Clinical Features of Porphyria ......Page 1842
Laboratory Studies ......Page 1845
Treatment ......Page 1846
References ......Page 1847
Clinical Manifestations ......Page 1849
Pathology and Organ System Involvement ......Page 1850
Histochemical Studies and Ultrastructural Changes ......Page 1852
Treatment ......Page 1856
Conclusions ......Page 1857
References ......Page 1859
Td Lipoprotein Metabolism ......Page 1861
Hdl and Cholesterol Transport in Plasma ......Page 1862
Diagnosis ......Page 1864
Pathology ......Page 1865
Pathogenesis ......Page 1867
Speculation on Neuropathophysiology ......Page 1870
Treatment ......Page 1871
References ......Page 1872
History ......Page 1876
Ttr Amyloidosis ......Page 1877
Apolipoprotein A-i Amyloidosis ......Page 1886
Acknowledgments ......Page 1887
References ......Page 1888
Peripheral Nerve Diseases Associated with Mitochondrial Respiratory Chain Dysfunction ......Page 1891
Basic Features of the Mitochondrial Respiratory Chain ......Page 1892
Making the Diagnosis of Mitochondrial Disease: Histopathology, Biochemistry, and Genetics ......Page 1894
How Common Is Peripheral Nerve Dysfunction in Mitochondrial Respiratory Chain Disease? ......Page 1896
Neuropathology of Peripheral Nerve in Patients with Mitochondrial Disease ......Page 1897
Mitochondrial Diseases with Neuropathy as a Major Clinical Feature ......Page 1898
References ......Page 1901
Diabetic Neuropathies ......Page 1904
Epidemiology, Clinical Course, and Risk Factors ......Page 1905
Clinical Features ......Page 1906
Pathologic Changes ......Page 1918
Relationship of Pathologic Changes to Clinical Syndromes ......Page 1920
Pathogenesis and Clinical Trials ......Page 1921
Treatment of Neuropathic Complications ......Page 1923
Treatment of Painful Diabetic Neuropathy ......Page 1924
Animal Models of Causation ......Page 1925
References ......Page 1933
Overview ......Page 1945
Historical Review ......Page 1946
Clinical Testing in Dlrpn and Lrpn ......Page 1953
Immunotherapy ......Page 1963
Symptomatic Treatment ......Page 1964
References ......Page 1965
Neuropathies and Hepatic Disorders ......Page 1968
Neuropathies and Respiratory Disease ......Page 1972
Neuropathies and Renal Disease ......Page 1973
Neuropathies and Critical Illness ......Page 1977
References ......Page 1982
Hypothyroid Neuropathy ......Page 1989
Hyperthyroid Neuropathy ......Page 1993
References ......Page 1996
Polyneuropathy Caused by Nutritional and Vitamin Deficiency ......Page 2000
Vitamin B12 Deficiency ......Page 2002
Vitamin E Deficiency ......Page 2004
Vitamin B6 Toxicity ......Page 2005
Gastric Surgery ......Page 2006
References ......Page 2007
Historical Aspects ......Page 2012
Classification ......Page 2017
Etiology ......Page 2020
Summary ......Page 2025
References ......Page 2026
Leprosy ......Page 2030
General Evaluation and Classification of Patients ......Page 2031
The Syndrome of Superficial Neuropathy ......Page 2036
Neuritis in Leprosy Reactions ......Page 2044
Differential Diagnosis ......Page 2045
Electrophysiologic Testing ......Page 2047
Neuropathology ......Page 2048
Treatment ......Page 2052
References ......Page 2055
History ......Page 2058
Clinical Manifestations ......Page 2059
Pathologic Aspects ......Page 2061
Experimental Neuroborreliosis ......Page 2062
References ......Page 2063
Herpes Simplex Virus ......Page 2066
Varicella Zoster Virus ......Page 2067
Epstein-barr Virus ......Page 2069
Cytomegalovirus ......Page 2070
References ......Page 2072
Peripheral Neuropathies in Human Immunodeficiency Virus Infection ......Page 2077
Epidemiology ......Page 2078
Inflammatory Demyelinating Polyneuropathy ......Page 2084
Multiple Mononeuropathy ......Page 2085
Neuropathy in Diffuse Infiltrative Lymphomatosis Syndrome ......Page 2086
References ......Page 2088
Clinical Features of Diphtheria and Diphtheritic Polyneuropathy ......Page 2094
Pathology of Diphtheritic Polyneuropathy ......Page 2095
Molecular Pathogenesis and Pathophysiology of Diphtheritic Polyneuropathy ......Page 2096
References ......Page 2097
Parasitic Infections of the Peripheral Nervous System ......Page 2099
References ......Page 2120
Polyneuropathies and Antibodies to Nerve Components ......Page 2123
Antibodies with Binding to Gangliosides ......Page 2128
Antibodies with Binding to Peripheral Nerve Glycoproteins and Glycolipids ......Page 2133
References ......Page 2136
History ......Page 2143
Differential Diagnosis ......Page 2147
Pathology ......Page 2148
Pathogenesis ......Page 2151
Management ......Page 2158
References ......Page 2159
Chronic Inflammatory Demyelinating Polyradiculoneuropathy ......Page 2166
Clinical Manifestations ......Page 2168
Treatment ......Page 2179
Pathogenesis ......Page 2188
References ......Page 2191
Overview ......Page 2199
Recognition of Monoclonal Proteins ......Page 2200
Monoclonal Gammopathy of Undetermined Significance ......Page 2203
Monoclonal Gammopathy and Motor Neuron Diseases ......Page 2213
Multiple Myeloma ......Page 2214
References ......Page 2215
Background ......Page 2221
Clinical Features ......Page 2222
Differential Diagnosis ......Page 2223
Electrophysiologic Characteristics ......Page 2224
Pathologic Changes ......Page 2230
Etiology and Pathogenesis ......Page 2231
Treatment ......Page 2234
References ......Page 2238
Historical Background ......Page 2243
Clinical Features ......Page 2244
Diagnosis and Evaluation ......Page 2245
Differential Diagnosis ......Page 2247
Pathogenesis and Pathology ......Page 2248
References ......Page 2250
Overview ......Page 2253
Historical Review ......Page 2254
Electrophysiologic Findings ......Page 2256
Pathologic Findings ......Page 2257
Differential Diagnosis ......Page 2258
References ......Page 2261
Demographics ......Page 2265
Clinical Course ......Page 2266
Utility of Serial Evaluation ......Page 2267
Treatment ......Page 2270
Differential Diagnosis ......Page 2271
References ......Page 2275
Overview of the Vasculitides ......Page 2278
Introduction to Neuropathies with Systemic Vasculitis ......Page 2280
Classification ......Page 2281
Pathogenesis ......Page 2282
Etiology ......Page 2291
Systemic Necrotizing Vasculitis ......Page 2295
Vasculitis Associated with Connective Tissue Diseases ......Page 2300
Hypersensitivity Vasculitis ......Page 2304
Temporal Arteritis ......Page 2305
Genetics ......Page 2306
Clinical Features ......Page 2307
Diagnosis ......Page 2309
Treatment ......Page 2313
Outcome ......Page 2326
References ......Page 2328
Disease Association ......Page 2348
Pathologic Alterations of Microvessels ......Page 2349
Pathologic Alteration of Nerve ......Page 2350
References ......Page 2356
History ......Page 2358
Genetics ......Page 2359
Symptoms and Signs of Sarcoid Neuropathy ......Page 2360
Sarcoidosis: a Multisystem Disease ......Page 2362
Electrophysiology ......Page 2363
Diagnosis ......Page 2364
References ......Page 2366
Microscopic Appearance and Ultrastructure ......Page 2369
Primary Amyloidosis ......Page 2370
Familial Amyloidosis ......Page 2382
References ......Page 2386
History ......Page 2394
Pathogenesis ......Page 2395
Clinical Features ......Page 2396
Diagnosis ......Page 2402
Course and Prognosis (survival) ......Page 2404
References ......Page 2405
Historical Background ......Page 2411
Incidence ......Page 2412
Immunopathogenesis ......Page 2421
References ......Page 2422
Lymphoma ......Page 2428
Chronic Lymphocytic Leukemia ......Page 2434
Acute Leukemias ......Page 2435
Polycythemia Vera ......Page 2436
Peripheral Neuropathies Related to Bone Marrow Transplantation ......Page 2437
References ......Page 2438
Human Toxic Neuropathy Caused by Industrial Agents ......Page 2443
Ethylene Oxide ......Page 2449
Methyl Bromide ......Page 2452
Organophosphates ......Page 2453
Pyriminil (vacor) ......Page 2457
References ......Page 2458
Lead ......Page 2464
Arsenic ......Page 2472
Mercury ......Page 2475
Thallium ......Page 2477
Gold ......Page 2480
Platinum ......Page 2481
References ......Page 2482
Neuropathy Caused by Drugs ......Page 2489
Amiodarone ......Page 2490
Chloroquine ......Page 2491
Colchicine ......Page 2492
Dapsone ......Page 2493
Disulfiram ......Page 2494
Isoniazid ......Page 2495
Metronidazole ......Page 2496
Nitrofurantoin ......Page 2497
Nitrous Oxide ......Page 2498
Perhexilene ......Page 2499
Phenytoin ......Page 2500
Podophyllin ......Page 2501
Pyridoxine ......Page 2502
Suramin ......Page 2503
Taxanes ......Page 2504
Tryptophan ......Page 2506
Vinca Alkaloids ......Page 2508
References ......Page 2509
Tumors and Tumor-like Conditions of Peripheral Nerve ......Page 2520
Tumor-like Conditions Affecting Peripheral Nerve ......Page 2535
References ......Page 2538
Management of Patients with Acute Neuromuscular Disease in the Intensive Care Unit ......Page 2542
General Aspects of Icu Care ......Page 2543
Management of Respiratory Failure ......Page 2546
Management of Systemic Complications ......Page 2550
References ......Page 2552
General Overview and Principles of Rehabilitation ......Page 2556
Specific Complications and Their Rehabilitation Management ......Page 2560
References ......Page 2569
Pharmacotherapy in Perspective ......Page 2572
Clinical Mechanisms Underlying Neuropathic Pain ......Page 2573
The Evidence Base and Generalizability of Clinical Trial Results ......Page 2575
Antidepressants ......Page 2576
Anticonvulsants ......Page 2577
Topical Medications ......Page 2579
Opioids ......Page 2580
The Research Landscape and Changing Clinical Practice ......Page 2581
References ......Page 2582
Definition ......Page 2588
Clinical Manifestations ......Page 2589
Course and Prognosis ......Page 2590
Treatment ......Page 2591
Bladder Dysfunction ......Page 2598
Gastrointestinal Symptoms ......Page 2599
References ......Page 2600
Overview of Cardiovascular Autonomic Failure ......Page 2605
Treatment ......Page 2607
References ......Page 2612
Regulation of Gastrointestinal Sensorimotor Function by Extrinsic Nerves ......Page 2616
Gastrointestinal Manifestations of Autonomic Neuropathy ......Page 2618
Extrinsic Neurologic Disorders Causing Gut Dysmotility ......Page 2628
References ......Page 2634
Peripheral Neuropathies in Infants and Children: Polyneuropathies, Mononeuropathies, Plexopathies, and Radiculopathies ......Page 2640
Infantile Polyneuropathies ......Page 2645
Pediatric Mononeuropathies ......Page 2657
Pediatric Radiculopathies ......Page 2669
References ......Page 2671
Index ......Page 2687