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Tables of Contents for Diagnosis and Management of Peripheral Nerve Disorders
Chapter/Section Title
Page #
Page Count
Contributors
xxi
PART 1 Approach to the Patient with Peripheral Neuropathy
Evaluation of the Patient with Peripheral Neuropathy: The Challenges
3
7
David R. Cornblath
Jerry R. Mendell
John T. Kissel
The Rational Diagnostic Process
6
3
Summary
9
1
Clues to Diagnosis of Peripheral Neuropathy: History and Examination of the Patient
10
20
Jerry R. Mendell
John T. Kissel
David R. Cornblath
Historical Information Important for Diagnosis of Peripheral Nerve Disease
11
3
Chief Complaint and History of Present Illness
Past Medical History
Family History
Social History
Review of Systems
Examining the Patient with Peripheral Neuropathy
14
13
General Physical Examination
Neurological Examination
Summary
27
3
Electrodiagnostic Evaluation of the Peripheral Neuropathy Patient
30
8
David R. Cornblath
Vinay Chaudhry
Technique
30
1
Sensory Nerve Conduction Studies
31
2
Motor Nerve Conduction Studies
33
1
Electromyography
34
1
Predicting Pathology
34
1
The Clinical Neurophysiology Report
35
1
Clinical-Electrodiagnostic Correlation
35
1
Limitations
35
2
Case Report
37
1
Summary
37
1
Evaluation of the Peripheral Neuropathy Patient Using Quantitative Sensory Testing
38
5
Jerry R. Mendell
David R. Cornblath
Sensory Fibers and Receptors
38
1
Testing and Algorithms
39
1
Applications of Quantitative Sensory Testing
40
1
Summary
41
2
Evaluation of the Peripheral Neuropathy Patient Using Autonomic Reflex Tests
43
24
Vera Novak
Jerry R. Mendell
Evaluation of Sudomotor Function
43
7
Neural Pathways Innervating Sweat Glands
Quantitative Sudomotor Axon Reflex Test
Silastic Imprints
Sympathetic Skin Response
Thermoregulatory Sweat Test
Evaluation of Cardiovagal and Adrenergic Functions
50
12
Neural Pathways Controlling Heart Rate and Blood Pressure
Deep Breathing Valsalva Maneuver
Head-Up Tilt
Management of Orthostatic Hypotension
62
3
Nonpharmacologic Approaches
Pharamcologic Management
Summary
65
2
The Role of Autoantibody Testing
67
23
John T. Kissel
Anti-Ganglioside Antibodies and Associated Neuropathies
67
5
Biochemistry and Immunology
Multifocal Motor Neuropathy
Guillain-Barre Syndrome
Anti-Ganglioside GQ1b and Fisher Syndrome
72
1
Biochemistry and Immunology
Antibody Testing
Conclusions
Anti-Myelin Associated Glycoprotein and Associated Neuropathies
73
4
Biochemistry and Immunology
Antibody Testing
Conclusions
Anti-Hu (Anti-Neuronal Nuclear Antibody, Type 1) and Associated Neurologic Syndromes
77
4
Biochemistry and Immunology
Clinical and Laboratory Features
Antibody Testing
Pathogenesis
Conclusions
Anti-Sulfatide Antibodies and Associated Neuropathy
81
2
Biochemistry and Immunology
Antibody Testing
Pathogenesis
Conclusions
Other Antibodies
83
1
Summary
83
7
The Role of Peripheral Nerve and Skin Biopsies
90
39
Jerry R. Mendell
Sevim Erdem
Dimitris P. Agamanolis
Nerve Biopsy Sites, Complications, and Pattern of Nerve Regeneration
90
3
Tissue Preparation
93
4
Specific Pathologic Abnormalities Identifiable by Nerve Biopsy
97
20
Abnormal Features of Stromal or Supportive Constituents of the Nerve
Abnormalities of Nerve Fibers
Neuropathies with Supportive Abnormalities on Nerve Biopsy
117
6
Charcot-Marie-Tooth Neuropathies
Guillain-Barre Syndrome
Chronic Inflammatory Demyelinating Polyradiculoneuropathy
Skin Biopsies
123
6
Approach to Painful Peripheral Neuropathies
129
16
David R. Cornblath
Jonathan D. Glass
Pathophysiology
130
1
Differential Diagnosis
131
5
Diabetes Mellitus
Malnutrition, Alcohol, Vitamin Deficiencies
Amyloidosis
Human Immunodeficiency Virus Infection
Toxins and Drugs
Uremia
Abnormalities of Serum Proteins
Dorsal Root
Ganglionopathy
Sjogren's Syndrome
Porphyric Neuropathy
Fabry Disease
Tangier Disease
Insulinoma
Multiple Mononeuropathy
Syndromes
Guillain-Barre Syndrome
Idiopathic Painful Sensory Neuropathy
Treatment
136
3
Summary
139
6
PART 2 Peripheral Nerve Disorders
Guillain-Barre Syndrome
145
28
John T. Kissel
David R. Cornblath
Jerry R. Mendell
History and Terminology
145
2
Guillain-Barre Syndromes with Weakness as the Predominant Feature
147
14
Epidemiology
Clinical Presentation
Course
Laboratory Studies
Treatment
Guillain-Barre Syndromes Lacking Weakness as the Predominant Feature
161
6
Fisher Syndrome
Acute Panautonomic Neuropathy
Pure Sensory
Neuropathy
Summary
167
6
Chronic Inflammatory Demyelinating Polyradiculoneuropathy
173
19
John T. Kissel
Jerry R. Mendell
Historical Background
173
1
Clinical Features
174
2
Chronic Inflammatory Demyelinating Polyradiculoneuropathy with Concurrent Conditions
176
2
Laboratory Features
178
4
Cerebrospinal Fluid
Electrodiagnostic Studies
Nerve Biopsy
Other Laboratory Studies
Diagnostic Criteria and Potential Limitations
182
1
Managing Patients Not Fulfilling Cidp Criteria
183
1
Pathogenesis
184
1
Treatment
185
1
Primary Therapy
Supplementary Therapy
Prognosis
186
1
Summary
186
6
Multifocal Motor Neuropathy
192
10
Vinay Chaudhry
Clinical Features
192
1
Electrodiagnostic Features
193
1
Other Laboratory Features
194
1
Differential Diagnosis
195
1
Response to Treatment
195
4
Pathogenesis
199
1
Summary
200
2
Vasculitic Neuropathy
202
31
John T. Kissel
Michael P. Collins
Jerry R. Mendell
Historical Overview
202
1
Epidemiology
203
1
Classification of the Vasculitides
204
6
Direct Infection of Vessels
Vasculities Resulting from Immunologic Mechanisms
Clinical Features of Vasculitic Neuropathy
210
2
Laboratory Features
212
2
Serologic Studies
Electrodiagnostic Studies
Nerve Biopsy
Differential Diagnosis
214
2
Pathogenesis
216
3
Treatment
219
5
Antigen Removal
Vaso-occlusion
Immunosuppressive Therapy
Limiting Medication Side Effects
Supportive Care
Prognosis
224
1
Summary
225
8
Peripheral Neuropathies Associated with Connective Tissue Diseases
233
23
Robert P. Lisak
Jerry R. Mendell
Systemic Lupus Erythematosus
233
5
Clinical Features
Peripheral Nervous System
Scleroderma (Systemic Sclerosis)
238
3
Clinical Features
Peripheral Nervous System
Eosinophilia-Myalgia Syndrome
241
1
Clinical Features
Peripheral Nervous System
Mixed Connective Tissue Disease
242
1
Clinical Features
Trigeminal Sensory Neuropathy
Sensorimotor Polyneuropathy
Rheumatoid Arthritis
243
2
Clinical Features
Peripheral Nervous System
Sjogren's Syndrome
245
4
Clinical Features
Peripheral Nervous System
Relapsing Polychondritis
249
1
Clinical Features
Cranial Neuropathies
Behcet's Disease
250
1
Clinical Features
Peripheral Nervous System
Summary
251
5
Sarcoid Peripheral Neuropathy
256
16
Robert E. Minahan
Systemic Involvement
256
2
Peripheral Neuropathies
258
7
Onset and Evolution in Relation to Underlying Sarcoidosis
Distal Symmetric Sensorimotor Polyneuropathy
Mononeuropathy and Multiple
Mononeuropathies
Guillian-Barre Syndrome
Radiculopathy
Preferential Small-Fiber Sensory and Autonomic Neuropathy
Subclinical Neuropathies
Neurosarcoidosis Confined to the Peripheral Nervous System
Pathogenesis of Sarcoid Neuropathy
265
1
Patient Evaluation
266
2
Treatment
268
1
Summary
269
3
Neuropathies Associated with Monoclonal Gammopathies
272
25
John T. Kissel
Jerry R. Mendell
Definition of Terms and Incidence
272
2
Methods of Detection
274
1
Patient Evaluation
275
1
Specific Disorders Associated with Monoclonal Antibodies
275
16
Multiple Myeloma
Osteosclerotic Myeloma
Primary Amyloidosis
Waldenstrom's Macroglobulinemia
Other Malignant Diseases
Cryoglobulinemia
Monoclonal Gammopathy of Undetermined Significance (MGUS)
Summary
291
6
Toxic Neuropathies: Drugs, Metals, and Alcohol
297
47
Sevim Erdem
John T. Kissel
Jerry R. Mendell
Neurotoxic Drugs
297
28
Amphiphilic Cationic Drugs
Chemotherapeutic Agents
Nucleoside Analogs
Antibiotics
Anticonvulsants
Vitamins
Anesthetic Agents
Immune Suppressant Drugs
Other Drugs Causing Toxicity
Metal Toxicity
325
6
Arsenic
Lead
Alcohol-Related Neuropathy
331
3
Clinical Features
Laboratory Features
Pathogenesis
Treatment
Summary
334
10
Porphyric Neuropathy
344
23
James W. Albers
Heme Biosynthetic Pathway
344
1
The Hepatic Porphyrias
345
17
Precipitating Factors
Clinical Features
Laboratory Features
Genetics
Pathogenesis
Treatment
Case Reports
Toxic Porphyria
362
1
Summary
363
4
Critical Illness Polyneuropathy and Related Conditions
367
6
Jerry R. Mendell
Critical Illness Polyneuropathy and Related Neuropathic Conditions
367
3
Clinical Features
Laboratory Features
Pathogenesis
Treatment
Hopkins Syndrome
370
1
Summary
371
2
Diabetic Neuropathies
373
27
Jerry R. Mendell
Classification of Diabetes
373
1
Diagnosis of Diabetes Mellitus
374
2
Focal and Multifocal Diabetic Neuropathies
376
8
Cranial Neuropathies
Mononeuropathies
Diabetic Truncal Radiculoneuropathy
Diabetic Lumbosacral Radiculoplexus Neuropathy (Bruns-Garland Syndrome)
Differential Diagnosis of Diabetic Lumbosacral Radiculoplexus Neuropathy
Diabetic Polyneuropathy
384
6
Clinical Features
Laboratory Studies
Pathogenesis of Diabetic Neuropathy
Treatment of Diabetic Polyneuropathy
390
2
Hyperglycemic Control
Aldose Reductase Inhibitors
Myo-inositol Administration
α-Lipoic Acid
Neurotrophic Factors
Pancreatic Transplantation
Uncommon Neuropathies Associated with Diabetes
392
1
Diabetic Neuropathic Cachexia (Acute Painful Neuropathy of Diabetes)
Treatment-Induced Diabetic Neuropathy (Insulin Neuritis)
Summary
393
7
Peripheral Neuropathy Associated with HIV Infection
400
29
Richard J. Barohn
John T. Kissel
Historical Overview and Epidemiology of Aids
401
1
Classification of HIV Infection
401
2
Prevalence of Peripheral Neuropathy in HIV-Infected Patients
403
3
Symptomatic HIV Infection
Asymtomatic HIV Infection
Summary of Prevalence Studies
Prevalence of Cerebrospinal Fluid Abnormalities in HIV-Positive Individuals
406
1
HIV-Related Peripheral Neuropathies
407
14
Inflammatory Demyelinating Polyradiculoneuropathy
Multiple Mononeuropathy
Distal Symmetrical Polyneuropathy
Lumbosacral Polyradiculopathy or Cauda Equina Syndrome
Diffuse Infiltrative Lymphocytosis Syndrome
Less Common Neuropathies
421
1
Sensory Ataxia Due to Ganglioneuritis
Facial Nerve Palsies
Herpes Zoster Radiculitis
Mononeuropathies
Autonomic Neuropathy
Summary
422
7
Hereditary Motor and Sensory Neuropathies and Giant Axonal Neuropathy
429
31
Jerry R. Mendell
Zarife Sahenk
Charcot-Marie-Tooth and Related Neuropathies
429
20
Dilemma of Classification of CMT Neuropathies
Charcot-Marie-Tooth Neuropathy Type 1
Charcot-Marie-Tooth Neuropathy Type 2
Dejerine Sottas Disease
Charcot-Marie-Tooth Neuropathy Type 4
X-Linked Dominant Charcot-Marie-Tooth Neuropathy
Hereditary Neuropathy with Liability to Pressure Palsies
Giant Axonal Neuropathy
449
3
Clinical Features
Laboratory Features
Genetics
Pathogenesis
Treatment for Hereditary Motor and Sensory Neuropathies and Giant Axonal Neuropathy
452
1
Summary
452
8
Hereditary Sensory and Autonomic Neuropathies
460
17
Jerry R. Mendell
Background and History
461
1
Properties of the Trk Family of Neurotrophin Receptors
461
2
Hereditary Sensory and Autonomic Neuropathy Type I
463
2
Clinical Features
Laboratory Features
Genetics
Treatment
Hereditary Sensory and Autonomic Neuropathy Type II
465
2
Clinical Features
Laboratory Features
Genetics
Treatment
Hereditary Sensory and Autonomic Neuropathy Type III
467
2
Clinical Features
Laboratory Features
Genetics
Treatment
Hereditary Sensory and Autonomic Neuropathy Type IV
469
2
Clinical Features
Laboratory Features
Genetics
Treatment
Hereditary Sensory and Autonomic Neuropathy Type V
471
1
Clinical Features
Laboratory Features
Genetics
Treatment
Hereditary Sensory and Autonomic Neuropathy Type VI and Other Variants
472
1
Congenital Indifference to Pain
473
1
Summary
473
4
Familial Amyloid Polyneuropathies
477
15
Jerry R. Mendell
Classification of Familial Amyloid Polyneuropathies
478
1
Transthyretin Amyloidosis
478
5
Clinical Features
Laboratory Features
Genetics
Treatment
Apolipoprotein A-1 Amyloidosis
483
2
Clinical Features
Laboratory Features
Genetics
Treatment
Gelsolin Amyloidosis
485
2
Clinical Features
Laboratory Features
Genetics
Treatment
Diagnosis of Amyloid Polyneuropathy
487
1
Summary
488
4
Peroxisomal Defects: Refsum Disease and Adrenomyeloneuropathy
492
16
Jerry R. Mendell
Refsum Disease
497
1
Background
Clinical Features
Laboratory Features
Genetics
Pathogenesis
Treatment
X-Linked Adrenomyeloneuropathy
497
6
Classification
Clinical Features
Laboratory Features
Genetics
Pathogenesis
Treatment
Summary
503
5
α-Galactosidase a Deficiency: Fabry Disease
508
10
Jerry R. Mendell
Clinical Features
508
3
Neuropathy
Central Nervous System Disease
Skin
Heart
Kidney
Eye
Phenotypic Variants and Heterozygotes
Laboratory Features
511
1
Electrodiagnostic Studies
Pathology
α-Galactosidase A Activity
Biochemical Defect and Pathogenesis
Genetics
512
1
Treatment
513
2
Summary
515
3
Lipoprotein Disorders: Tangier Disease and Abetalipoproteinemia/Familial Hypobetalipoproteinemia
518
14
Jerry R. Mendell
Lipids and Lipoproteins
518
1
Tangier Disease
519
4
Clinical Features
Laboratory Features
Genetics
Pathogenesis
Treatment
Abetalipoproteinemia and Familial Hypobetalipoproteinemia
523
3
Clinical Features
Laboratory Features
Genetics
Pathogenesis
Treatment
Summary
526
6
Vitamin Deficiencies: Cobalamin (Vitamin B12), Vitamin E (Tocopherol), and Thiamine (Vitamin B1)
532
19
Jerry R. Mendell
John T. Kissel
Cobalamin Deficiency
532
6
Cobalamin Biochemistry and Metabolism
Clinical Features
Laboratory Features
Treatment
Vitamin Deficiency
538
3
Vitamin E Biochemistry
Clinical Features
Laboratory Features
Pathophysiology
Genetics
Treatment
Thiamine Deficiency
541
4
Thiamine Biochemistry
Clinical Features
Laboratory Features
Pathophysiology
Treatment
Summary
545
6
Leprous Neuropathy
551
14
Gerard Said
Characteristics of Mycobacterium Leprae
551
1
Classification of Leprosy
552
1
Clinical Features
553
5
Lepromatous Leprosy
Tuberculoid Leprosy
Borderline Leprosy
Laboratory Features
558
1
Skin Biopsy
Blood Tests
Lepromin Skin Test
Electrodiagnostic Studies
Pathology and Immunopathogenesis
558
3
Lepromatous Leprosy
Tuberculoid Leprosy
Treatment
561
2
Summary
563
2
Neuropathies Associated with Organ System Failure, Organ Transplantation, Metabolic Disorders, and Cancer
565
27
Anthony A. Amato
John T. Kissel
Jerry R. Mendell
Neuropathies Associated with Renal Failure
566
3
Focal Neuropathies
Uremic Polyneuropathy
Uremic Autonomic Neuropathy
Neuropathies Associated with Hepatic Disease
569
3
Neuropathy Associated with Chronic Hepatic Failure
Xanthomatous
Neuropathy of Primary Biliary Cirrhosis
Neuropathy Associated with Pulmonary Failure
572
1
Clinical Features
Laboratory Features
Treatment
Neuropathies Associated with Organ Transplantation
573
2
Renal Transplantation
Heart, Lung, and Heart-Lung Transplantation
Bone Marrow Transplantation
Liver Transplantation
Pancreas Transplantation
Neuropathy Associated with Systemic Metabolic Abnormalities
575
2
Neuropathies Associated with Thyroid Disorders
Neuropathy Associated with Hyperlipidemia
Neuropathy Associated with Hyperuricemia (Gout)
Neuropathies Associated with Cancer
577
7
Direct Effects of Cancer on the Peripheral Nervous System
Remote Effects of Cancer on the Peripheral Nervous system
Summary
584
8
Entrapment Neuropathies
592
47
Miriam Freimer
Thomas M. Brushart
David R. Cornblath
John T. Kissel
Predisposition to Entrapment Neuropathies
592
1
Pathophysiology of Nerve Compression
593
2
The Double-Crush Syndrome
Median Nerve
595
11
Anatomy
Carpal Tunnel Syndrome
Anterior Interosseous Nerve Syndrome
Pronator Syndrome
Ulnar Nerve
606
8
Anatomy
Ulnar Nerve Entrapment at the Elbow
Ulnar Nerve Entrapment at the Wrist
Radial Nerve
614
7
Anatomy
Proximal Radial Nerve Lesions
Distal Radial Nerve Lesions
Posterior Interosseous Nerve Syndrome
Radial Tunnel Syndrome
Superficial Radial Nerve
Personeal Nerve
621
5
Anatomy
Peroneal Nerve Entrapment (Common, Deep, Superficial)
Deep Peroneal Nerve Entrapment in the Foot
Tibial Nerve
626
3
Anatomy
Tarsal Tunnel Syndrome
Proximal Tibial Neuropathy
Other Lower Limb Nerve Entrapments
629
3
Femoral Neuropathy
Obturator Nerve Entrapment
Lateral Femoral Cutaneous Nerve Entrapment (Meralgia Paresthetica)
Summary
632
7
Plexopathies
639
38
Asa J. Wilbourn
Pathology and Pathophysiology of Plexus Lesions
639
2
The Cervical Plexus
641
2
Anatomy
Cervical Plexopathies
The Brachial Plexus
643
21
Anatomy
Brachial Plexopathies
Types of Brachial Plexus Lesions
The Lumbosacral Plexus
664
8
Anatomy
Lumbosacral Plexopathies
Clinical Features
Types of Lumbosacral Plexus Lesions
Summary
672
5
Index
677