Neurobiology of Disease

Front Cover

Title Page

Copyright Page

Table of Contents

Contributors

Editors

Preface

PART A. CENTRAL NERVOUS SYSTEM

Section I. Metabolic Diseases

Chapter 1. Lysosomal Disorders of the Nervous System

I. Introduction

II. Classifying Lysosomal Storage Diseases by Their Clinicopathological Features

III. Classifying Lysosomal Diseases by Their Defective Proteins

IV. The Greater Lysosomal System as a Central Coordinator of Neuron Metabolism and Function

V. Dysfunction of the Lysosomal System in Neurons Leads to Complex Pathogenic Cascades

VI. Understanding Lysosomal Disease Pathogenesis Provides Key Insight for Development of Therapy

References

Chapter 2. Neurobiology of Peroxisomal Disorders

I. History and Nomenclature

II. The Peroxisomal Disorders

III. Etiology

IV. Molecular Pathogenesis and Pathophysiology

V. Human Pathology and Pathogenesis

VI. Mouse Models and Pathogenesis

VII. Clinicopathological Correlations

References

Chapter 3. Creatine Deficiency Syndromes

I. History and Nomenclature

II. Etiology and Biochemical Pathogenesis

III. Pathophysiology

IV. Natural History

V. Prospects

References

Chapter 4. Leukodystrophies

I. Introduction

II. Lipid Metabolism Disorders

III. Myelin Protein Disorders

IV. Organic Acid Disorders

V. Other Leukodys

References

Section II. Neurodegenerative Disorders

Chapter 5. Parkinson’s Disease

I. Brief History

II. Epidemiology

III. General Pathology (and Basic Structural Detail)

IV. Pathogenesis

V. Pathophysiology

VI. Pathophysiology of Symptoms

VII. Pharmacology, Biochemistry, Molecular Mechanism

VIII. Natural History of Parkinson’s Disease and Its Realationship to Pharmacology

References

Chapter 6. Alzheimer’s Disease

I. History and Nomenclature

II. Epidemiology

III. Etiology and Risk Factors

IV. Pathogenesis

V. Neuropathology of AD (Relevant Structural Details)

VI. Pathophysiology

VII. Biochemistry and Molecular Mechanisms

VIII. Explanation of Symptoms in Relation to Pathophysiology

IX. Natural History

X. Management of AD

References

Chapter 7. Multiple System Atrophy

I. Brief History and Nomenclature

II. Epidemiology

III. Pathogenesis

IV. Structural Details

V. Biochemical and Neuropharmacological Findings

VI. Molecular Biology

VII. Animal Models

VIII. Clinical Picture

IX. Clinical Diagnosis and Clinical Diagnostic Criteria

X. Natural History of the Disease

XI. Conclusion

References

Chapter 8. Olivopontocerebellar Atrophy (OPCA)

I. Brief History and Nomenclature

II. Etiology and Pathogenesis

III. Diagnosis

IV. Treatment

References

Chapter 9. Neurobiology of Progressive Supranuclear Palsy

I. History and Nomenclature

II. Etiology

III. Pathogenesis

IV. Relevant Structural

V. Pharmacology, Biochemistry, Molecular Mechanisms

VI. Explanation of Symptoms in Relation to Pathophysiology

VII. Natural History

References

Section III. Genetic Diseases

Chapter 10. Protein Aggregation Disorders

I. Protein Folding and Misfolding

II. Protein Aggregation Disorders

III. “Natively Unfolded’’ Proteins and Other Structural Determinants of Protein Aggregation

IV. Cellular Quality Control Systems for Protein Folding as Targets for Therapeutic Intervention in Neurodegenerative Diseases

V. Conclusions

References

Chapter 11. RNA-Based Disorders of Muscle and Brain

I. Overview

II. Myotonic Dystrophy Type 1 and Type 2

III. Fragile X–Associated Tremor/Ataxia Syndrome

IV. Prospects for RNA Pathogenic Mechanisms in Other Neuromuscular Disorders

References

Chapter 12. Ion Channel Disorders

I. Introduction

II. Epilepsy

III. Ataxia

IV. Neuromuscular Disorders

References

Chapter 13. Spinocerebellar Ataxia Type 1

I. SCA1: The Disease

II. SCA1 Pathogenesis: Regional Involvement

III. SCA1 Pathogenesis: Molecular Mechanisms

IV. SCA1: Recovery from Disease

V. Linking Pathology to Pathophysiology

VI. Concluding Comments

References

Chapter 14. Mitochondrial Genetic Diseases

I. Mutations in Mitochondrial Protein-Encoding Genes

II. Mutations in Mitochondrial Protein Synthesis Genes

III. Defective Mitochondrial Function Caused by Nuclear DNA Mutations

References

Section IV. Neuroimmunological Disorders

Chapter 15. Paraneoplastic Neurological Disorders

I. Introduction

II. History and Nomenclature

III. Etiology and Pathogenesis

IV. Pathophysiology

V. Symptoms and Natural History

VI. Summary

References

Chapter 16. Systemic Lupus Erythematosus: Descriptive Past and Mechanistic Future

I. Introduction

II. Epidemiology, Etiology, and Pathogenesis

III. Neuropsychiatric SLE

IV. Neuroimaging in SLE

V. Pathogenesis of NPSLE

VI. Anti-Phospholipid Antibodies and Anti-Phospholipid Syndrome

VII. Summary

References

Chapter 17. Progressive Multifocal Leukoencephalopathy

I. Brief History and Nomenclature

II. Etiology

III. Pathogenesis

IV. Lymphocyte Control on Latency

V. Pathophysiology

VI. Molecular Mechanisms

VII. Explanation of Symptoms in Relation to Pathophysiology

VIII. Natural History

References

Chapter 18. Immunopathogenesis of Multiple Sclerosis

I. Introduction

II. Immune Surveillance of the CNS

III. Animal Models of CNS Inflammation

IV. Immunology of the Multiple Sclerosis Lesion

References

Chapter 19. Immune-Mediated Neuropathies

I. Introduction

II. Acute Immune-Mediated Neuropathies

III. Chronic Immune-Mediated Neuropathies

IV. Systemic Autoimmune Disease Affecting the Peripheral Nerve

V. Immune-Mediated Exacerbation of Nonimmune Peripheral Nerve Disease

References

Chapter 20. Hashimoto Encephalopathy

I. History and Nomenclature

II. Clinical Features

III. Etiology

IV. Pathogenesis

V. Clinical Course

References

Section V. Cerebrovascular Diseases

Chapter 21. Vascular Cognitive Impairment

I. Introduction

II. Nomenclature, Epidemiology, and Natural History

III. Etiology of Vascular Cognitive Impairment

IV. Causes of Hereditary Vascular Cognitive Impairment

V. Causes of Sporadic Vascular Cognitive Impairment

VI. Cardiovascular Causes of Sporadic Vascular Cognitive Impairment Independent of Structural Brain Abnormality

VII. Pathophysiology of Vascular Cognitive Impairment

VIII. Clinical Features

IX. Summary

References

Chapter 22. Cardioembolism

I. Nomenclature

II. Etiology

III. Pathophysiology

IV. Molecular Mechanisms

References

Chapter 23. Clinical and Neurobiological Aspects of Stroke Recovery

I. Introduction

II. Brief History: The “General Course of Recovery’’

III. Emergence after Brain Injury of Progressive and Mutable Reflexive Motor Power

IV. Unmasking: Phenomena of Altering Ineffective Synaptic Potential

V. Bench to Bedside: Experimental Precedents Fuel Clinical Treatment

VI. Evidence for Functional Reorganization in Stroke Recovery: Neuroimaging Tools

VII. Motor Systems Cortical Physiology: Influences from the Bench

VIII. From Mutable Motor Maps to Neuroimaging to Magnetism

IX. Motor Learning as a Guide to Pharmacological Interventions

X. Conclusions

References

Chapter 24. Nonatherosclerotic Cerebral Vasculopathies

I. Introduction

II. Noninflammatory Arteriopathies

III. Inflammatory Arteriopathies

IV. Conclusion

References

Chapter 25. Subarachnoid Hemorrhage

I. Introduction

II. Etiology

III. Epidemiology and Genetics

IV. Aneurysm Pathogenesis

V. Pathophysiology of Aneurysm Rupture

VI. Pathophysiology of Delayed Cerebral Ischemia

VII. Cerebral Salt Wasting Syndrome

VIII. Conclusion

References

Chapter 26. Cerebral Ischemia: Molecular Mechanisms and Protective Therapies

I. Introduction

II. Definitions: Ischemic Core and Penumbra

III. Cellular and Biochemical Responses to Focal Cerebral Ischemia

IV. The Neurovascular Unit

V. Blood-Brain Barrier Damage in Cerebral Ischemia

VI. Postischemic Necrosis and Apoptosis

VII. Molecular Events Underlying Ischemic Cell Death

VIII. Gene Expression Following Cerebral Ischemia

IX. The Inflammatory Response to Cerebral Ischemia

X. Hyperglycemia and Hemorrhagic Infarct Conversion

XI. Acute Treatments for Cerebral Ischemia

XII. Global Cerebral Ischemia

References

Chapter 27. Intracerebral Hemorrhage and Intraventricular Hemorrhage–Induced Brain Injury

I. Introduction

II. Etiology

III. Pathophysiology

IV. Mechanisms of Intracerebral Hemorrhage–Induced Brain Injury

V. Mechanisms of Intraventricular Hemorrhage–Induced Brain Injury

VI. Therapeutic Interventions for Intracerebral Hemorrhage–Induced Brain Injury

VII. Therapeutic Interventions for Intraventricular Hemorrhage–Induced Brain Injury

VIII. Summary

References

Section VI. Paroxysmal Disorders

Chapter 28. Idiopathic Generalized Epilepsy

I. Clinical Overview

II. Animal Models

III. Genetic Models of Absence Seizures

IV. Pharmacological Models of Absence Seizures

V. The Nature of Thalamocortical Synchronized Activity

VI. Genetics

VII. Concluding Remarks

References

Chapter 29. Paroxysmal Dyskinesia

I. Brief History and Nomenclature

II. Paroxysmal Kinesigenic Dyskinesia (Formerly Paroxysmal Kinesigenic Choreoathetosis)

III. Paroxysmal Nonkinesigenic Dyskinesia (Formerly Familial Paroxysmal Dystonia or Paroxysmal Dystonic Choreoathetosis)

IV. Paroxysmal Exercise–Induced Dystonia

V. Paroxysmal Hypnogenic Dyskinesia

VI. Conclusion

References

Chapter 30. Myoclonus

I. History and Nomenclature

II. Pathophysiology

III. Pharmacology and Molecular Genetics

IV. Relationship between Pathophysiology and Symptoms

V. Natural History

References

Chapter 31. Channelopathies of the Nervous System

I. Introduction

II. Channelopathies: Defects of Ion Channel Function

III. Channelopathies of the Peripheral Nervous System

IV. Channelopathies of the Central Nervous System

V. Acquired Channelopathies of the Nervous System

References

Chapter 32. Migraine as a Cerebral Ionopathy with Abnormal Central Sensory Processing

I. Migraine: A Common, Disabling, Episodic Disorder

II. The Migraine Attack: Clinical Phases and Pathophysiology

III. The Migraine Trigger Threshold: Repeated Recurrence of Attacks

IV. Conclusions

References

Chapter 33. Temporal Lobe Epilepsy

I. Historical Background

II. Pathology of Temporal Lobe Epilepsy

III. Pathophysiological Mechanisms

IV. Summary and Future Directions: Developing a Comprehensive Hypothesis for the Pathology and Pathophysiology of Temporal Lobe Epilepsy

References

Section VII. Neoplastic Diseases

Chapter 34. Central Nervous System Metastases

I. Overview

II. Brief History and Nomenclature

III. Natural History of CNS Metastasis

IV. Etiology and Pathogenesis: The Brain Metastatic Process

V. Pathophysiology: An Unique Environment?

VI. Conclusion

References

Chapter 35. Meningioma

I. History

II. Incidence and Etiology

III. Meningioma Morphology and Subtypes

IV. Clinical Aspects

V. Pathogenesis and Molecular Mechanisms

VI. Special Forms of Meningioma

VII. Pathophysiology

VIII. Cell Lines and Animal Models

IX. Future Directions

References

Chapter 36. Primary Central Nervous System Lymphoma

I. Introduction

II. Epidemiology

III. Pathology and Biology

IV. Clinical Features of PCNSL

V. Treatment Options for PCNSL

VI. Summary

References

Chapter 37. Neurofibromatosis 1

I. History

II. Nomenclature

III. Etiology

IV. Pathogenesis

V. Natural History

VI. Structural Detail

VII. Biochemistry and Molecular Mechanisms

VIII. Symptoms in Relation to Pathophysiology

References

Chapter 38. Medulloblastoma and Primitive Neuroectodermal Tumors

I. Historical Concepts and Definitions

II. Neuroembryogenesis of the Cerebellum: Clues to the Origin of Medulloblastoma

III. Histopathology of Medulloblastoma

IV. Molecular Genetics of Medulloblastoma

V. Medulloblastoma and Inherited Cancer Syndromes

VI. Developmental Signaling Pathways and Medulloblastoma

VII. Molecular Profiling and Patient Outcome in Medulloblastoma

VIII. Treatment of Medulloblastoma: The Promise of New Pharmacotherapeutics

IX. Summary

References

Chapter 39. Glioma

I. Introduction

II. Nomenclature

III. Locations and Symptoms

IV. Epidemiology

V. Etiology

VI. Histopathology

VII. Genetic Alterations

VIII. Cell of Origin

IX. Signaling Pathways

X. Pharmacology

XI. Future Therapeutic Interventions

References

Section VIII. Infectious Diseases

Chapter 40. Bacterial and Fungal Infections of the Nervous System

I. History and Nomenclature

II. Etiology

III. Pathogenesis

IV. Relevant Structural Detail

V. Pathophysiology

VI. Pharmacology

VII. Signs and Symptoms

VIII. Natural History

References

Chapter 41. Parasitic Infections

I. Introduction

II. Protozoans

III. Helminths: Cestodes

IV. Helminths: Nematodes

V. Helminths: Trematodes

References

Chapter 42. Prion Diseases

I. History of Prion Diseases

II. The “Protein-Only’’ Hypothesis (What Is a Prion?)

III. Prion Diseases of Animals and Humans

IV. Animal Models of Prion Diseases

V. Peripheral Prion Pathogenesis

VI. Therapy

References

Chapter 43. Central Nervous System Viral Infections: Clinical Aspects and Pathogenic Mechanisms

I. Introduction

II. Neuropathogenesis of CNS Viral Infections

III. Diagnostic Considerations

IV. Acute CNS Viral Infection: Herpes Simplex Encephalitis

V. Chronic CNS Viral Infection: Neurocognitive Syndromes in HIV Infection

VI. Future Perspectives

References

Section IX. Motor Neuron Diseases

Chapter 44. Spinal Muscular Atrophy

I. Introduction

II. Spinal Muscular Atrophy

III. Genetic Basis of SMA

IV. SMN Gene Expression

V. Splicing of SMN Transcripts

VI. SMN Protein

VII. Animal Models of SMA

VIII. Therapeutic Strategies for SMA

IX. Remaining Questions

References

Chapter 45. Amyotrophic Lateral Sclerosis–Like Syndromes Associated with Malignancy

I. Brief History and Nomenclature

II. Etiology

III. Pathogenesis, Pathophysiology, and Relevant Structural Detail

IV. Pharmacology, Biochemistry, and Molecular Mechanisms

V. Explanation of Symptoms in Relation to Pathophysiology

VI. Natural History

References

Chapter 46. Amyotrophic Lateral Sclerosis: Idiopathic and Inherited

I. History and Nomenclature

II. Epidemiology

III. Etiology and Pathogenesis

IV. Pathophysiology

V. Symptoms in Relation to Pathophysiology and Natural History

VI. Structural Detail

VII. Molecular Mechanisms

VIII. Pharmacology and Treatment

References

Chapter 47. Hereditary Spastic Paraplegia and Primary Lateral Sclerosis

I. Definitions

II. Historical Aspects

III. Epidemiology

IV. Clinical Features of HSP

V. Neuropathology of HSP

VI. Molecular Genetics of HSP

VII. Nosology of PLS

VIII. Common Molecular Mechanisms Underlying HSP and PLS

References

Chapter 48. Poliomyelitis

I. Background

II. Epidemiology

III. Pathophysiology

IV. Treatment

V. Summary

References

Chapter 49. Spinobulbar Muscular Atrophy (Kennedy’s Disease)

I. Introduction

II. Clinical Features

III. Pathological Features

IV. Kennedy’s Disease as a Polyglutamine Disease: Molecular Pathogenesis

V. Female Carriers of Kennedy’s Disease

VI. Pharmacology: The Role of Ligand in the Pathogenesis

VII. Genetically Modified Animal Models

VIII. Symptoms in Relation to Pathophysiology

IX. Potential for Therapy

X. Conclusions

References

Section X. Malformations and Developmental Disorders

Chapter 50. Neurobiology of Genetic Mental Retardation

I. Introduction

II. Dendritic and Synaptic Abnormalities: Fundamental Features of Genetic MR

III. Down Syndrome

IV. X-Linked MR Disorders

V. Other Genetic MR Disorders

VI. Conclusion

References

Chapter 51. Cerebral Palsy

I. Brief History and Nomenclature

II. Etiology

III. Pathogenesis

IV. Pathophysiology

V. Relevant Structural Detail

VI. Pharmacology, Biochemistry, and Molecular Mechanisms

VII. Symptoms in Relation to Pathophysiology

VIII. Natural History

References

Chapter 52. Autism

I. Brief History and Nomenclature

II. Etiology

III. Pathogenesis

IV. Pathophysiology

V. Brain Structure

VI. Pharmacology, Biochemistry, and Molecular Mechanisms

VII. Explanation of Symptoms in Relation to Pathophysiology

VIII. Natural History

References

Chapter 53. Neurobiology of Dyslexia

I. Definition and History

II. Natural History

III. Behavioral Models of Dyslexia

IV. Neurobiology of Dyslexia

V. Relating Neurobiology to Reading Behavior

VI. Summary

References

Chapter 54. Neonatal Brain Injuries

I. Nomenclature

II. Etiology and Pathogenesis

III. Pathophysiology: Relevant Structural Detail and Molecular Mechanisms

IV. Explanation of Symptoms in Relation to Pathophysiology and Natural History

V. Summary

References

Chapter 55. Spina Bifida

I. Brief History and Nomenclature

II. Etiology

III. Pathogenesis

IV. Pathophysiology

V. Relevant Structural Details

VI. Pharmacology, Biochemistry, and Molecular Mechanisms

VII. Natural History

References

Chapter 56. Circuits to Synapses: The Pathophysiology of Tourette Syndrome

I. Introduction

II. Brief History and Nomenclature

III. Natural History

IV. Relevant Structural Detail and Neurophysiology

V. Location of the Primary Dysfunction

VI. Neurochemical Basis for TS

VII. Autoimmunity as a Mechanism for TS

VIII. Summary

References

Chapter 57. Attention-Deficit Hyperactivity Disorder

I. History and Nomenclature

II. Etiology

III. Pathogenesis and Pathophysiology

IV. Relevant Neurological Findings

V. Pharmacology, Biochemistry, and Molecular Mechanisms

VI. Natural History

References

Chapter 58. Congenital Hydrocephalus

I. Brief History and Nomenclature

II. Etiology

III. Pathogenesis

IV. Pathophysiology

V. Relevant Structural Details

VI. Pharmacology, Biochemistry, and Molecular Mechanisms

VII. Natural History

References

Section XI. Neurologic Manifestations of Medical Diseases

Chapter 59. Neurological Manifestations of Hematological Disease

I. Vitamin B12 Deficiency

II. Paraproteinemias: Syndromes Associated with a Monoclonal Immunoglobulin

III. Hemoglobinopathies

IV. Acute Porphyrias

V. Thrombotic Thrombocytopenic Purpura

VI. Myeloproliferative Syndromes

References

Chapter 60. Neurological Manifestations of Renal Disease

I. Introduction

II. Neurological Syndromes in Chronic Kidney Disease

III. Neurological Complications of Dialysis

IV. Renal Complications of Neurological Syndromes

V. Neurological Manifestations of Electrolyte Imbalances

VI. Drug Toxicities

References

Chapter 61. Diabetes and Endocrine Disorders

I. Diabetes Mellitus

II. Diabetic Neuropathies

III. Other Endocrine Disorders

References

Chapter 62. Mechanisms and Consequences of Central Nervous System Hypoxia

I. Cerebral Energy Metabolism

II. Cerebral Oxygen Supply

III. Cerebral Blood Flow

IV. Ventilatory Control

V. Oxygen-Sensing Mechanisms

VI. Neurobiology of Hypoxic-Ischemic Injury

VII. Clinical Consequences of Hypoxia

VIII. Conclusions

References

Chapter 63. Neurological Manifestations of Gastrointestinal and Hepatic Diseases

I. Introduction

II. Diseases of the Alimentary Tract

III. Hepatic Diseases

IV. Vitamin and Mineral Deficiencies

V. Conclusion

References

Chapter 64. Neurosarcoidosis

I. Neurosarcoidosis

II. Neurosarcoidosis: Sites of Involvement

III. Conclusion

References

Section XII. Sleep Disorders

Chapter 65. REM Sleep Behavior Disorder

I. Brief History and Nomenclature

II. Etiology

III. Pathogenesis

IV. Pathophysiology

V. Pharmacology, Biochemistry, and Molecular Mechanisms

VI. Explanation of Symptoms in Relation to Pathophysiology

VII. Natural History

References

Chapter 66. Neurobiology of Narcolepsy and Hypersomnia

I. Narcolepsy-Cataplexy

II. Narcolepsy without Cataplexy

III. Idiopathic Hypersomnia

References

Chapter 67. Restless Legs Syndrome and Periodic Limb Movements in Sleep

I. Natural History of Restless Legs Syndrome

II. Etiology of Restless Legs Syndrome

III. Pathophysiology and Molecular Mechanisms of Restless Legs Syndrome

IV. Explanation of Restless Legs Syndrome Symptoms in Relation to Pathophysiology

V. Periodic Limb Movements

VI. Conclusion

References

Chapter 68. Neurobiology of Insomnia

I. Introduction

II. Definition of Insomnia

III. Theoretical Perspectives on Insomnia

IV. Brief Review of the Neurobiology of Sleep and Wakefulness

V. Neurobiology of Sleep and Wakefulness: Implications for Insomnia

VI. Neurophysiologic, Neuroendocrine, and Neuroimaging Measures of Insomnia

VII. Call for an Integrative Perspective on Insomnia

References

Section XIII. Substance Abuse and Basic Toxicology

Chapter 69. Organic Chemicals

I. History and Nomenclature

II. Epidemiology

III. Risk Factors

IV. Pathogenesis: Etiological Agents and Factors Affecting Mode of Action

V. Neuropathology of Organic-Chemical-Induced Neurotoxicity

VI. Pathophysiology

VII. Biochemistry and Molecular Mechanisms

VIII. Explanation of Signs and Symptoms in Relation to Pathophysiology

IX. Natural History

X. Management of Organic-Chemical-Induced Neurotoxicity

References

Chapter 70. Metals

I. Introduction

II. Metals Causing Nervous System Disease

III. Pathophysiology

IV. Targets

V. Biochemical and Molecular Mechanisms

VI. Nature of Neurotoxic Syndromes

VII. Age-Related Variables

References

Chapter 71. Neurobiology of Drug Addiction

I. Drug Addiction versus Dependence

II. Traditional Conceptions of Drug Addiction: The Hedonic Model

III. Dopamine as the Hedonic Signal

IV. Incentive Salience Model of Drug Addiction

V. Aberrant Learning Model of Drug Addiction

VI. Drug Addiction Involves Multiple Neural Circuits, Transmitter Systems, and Processes

VII. Drug Addiction Reflects the Interaction of Neurobiological, Genetic, and Environmental Factors

VIII. Stress and Vulnerability to Drug Abuse

References

Section XIV. Imaging the Nervous System

Chapter 72. Assessment of Neurobiological Diseases with Magnetic Resonance Spectroscopy

I. Introduction

II. History, Nomenclature, and Basic Technological Concepts

III. Biochemistry and the Interpretation of Magnetic Resonance Spectra

IV. Pathophysiology of Neurological Diseases of the Brain and Magnetic Resonance Spectroscopy

V. Summary

References

Chapter 73. Magnetic Resonance Imaging

I. History of Magnetic Resonance Imaging

II. Magnetic Resonance Imaging Hardware

III. Basic Principles of Magnetic Resonance Imaging

IV. Image Analysis

V. Basic Clinical Magnetic Resonance Imaging

VI. Perfusion and Diffusion Magnetic Resonance Imaging

VII. Other Sources of Image Contrast in Proton Magnetic Resonance Imaging

VIII. Exogenous Contrast Agents

IX. Imaging Nuclei Other Than Protons

X. Conclusion

References

Chapter 74. Neurovascular Computed Tomography Angiography

I. Introduction

II. Fundamentals

III. Specific Neurovascular Clinical Scenarios

IV. Conclusions and Future Directions

References

Chapter 75. PET Imaging in Parkinson’s Disease and Other Neurodegenerative Disorders

I. Introduction

II. Diagnosis of Parkinson’s Disease

III. Treatment

IV. Cognitive Impairment: New Imaging Approaches

V. PET Imaging and Clinical Diagnosis

VI. Conclusion

References

Chapter 76. Single-Photon Emission Computed Tomography

I. Brief History and Method

II. SPECT in Diseases of the Brain

References

Chapter 77. Functional Magnetic Resonance Imaging

I. Overview and Methods

II. Functional Magnetic Resonance Imaging of Cognition and Cognitive Deficits

III. Clinical Application in Neuronavigation

IV. Monitoring Recovery of Function

V. Phenotyping and Genotyping

VI. Conclusion

References

PART B. PERIPHERAL NERVOUS SYSTEM

Section XV. Peripheral Neuropathies

Chapter 78. Impaired Glucose Regulation and Neuropathy

I. Introduction

II. Spectrum of Impaired Glucose Regulation

III. Pathophysiology of Neuropathy Related to Impaired Glucose Regulation

IV. Symptoms in Relation to Pathophysiology

V. Natural History

VI. Conclusion

References

Chapter 79. Acquired Inflammatory Demyelinating and Axonal Neuropathies

I. Acute Inflammatory Neuropathies

II. Chronic Inflammatory Neuropathies

III. Chronic Inflammatory Demyelinating Polyneuropathy and Closely Related Neuropathies

IV. Neuropathies Associated with Monoclonal Gammopathies

V. Treatment

VI. Summary

References

Chapter 80. Toxic and Drug-Induced Neuropathies

I. Antimicrobial Agents

II. Nucleoside Reverse Transcriptase Inhibitors

III. Cardiovascular Drugs

IV. Statins

V. Disulfiram

VI. Colchicine

VII. Chloroquine

VIII. Gold Salts

IX. Leflunomide

X. Tacrolimus (FK506)

XI. Interferons

XII. Antiepileptic Drugs

XIII. Antineoplastic Drugs

XIV. New Generation Antineoplastic Drugs

References

Chapter 81. Inherited Peripheral Neuropathies

I. Introduction

II. Classification

III. Different Types of Hereditary Neuropathies: Clinical, Genetic, and Pathological Features and Pathomechanisms

IV. Hereditary Motor Neuropathies

V. Hereditary Sensory and Autonomic Neuropathies

VI. Hereditary Brachial Plexus Neuropathy

VII. Rare Forms of Hereditary Peripheral Neuropathies

References

Chapter 82. Neurological Manifestations of Vasculitis

I. Introduction

II. History and Nomenclature

III. Pathogenesis

IV. Primary Vasculitides

V. Secondary Vasculitides

VI. Central and Peripheral Nervous System Manifestations

References

Chapter 83. Neuropathies Associated with Infections

I. Introduction

II. Peripheral Neuropathy Syndromes Related to Bacterial, Mycobacterial, and Spirochetal Infections

III. Peripheral Neuropathy Syndromes Related to Viral Infections

IV. Conclusion

References

Section XVI. Myopathies and Neuromuscular Junction Disorders

Chapter 84. Muscular Dystrophies

I. Introduction

II. Duchenne’s and Becker’s Muscular Dystrophies

III. Limb-Girdle Muscular Dystrophies

IV. Congenital Muscular Dystrophies

V. Myotonic Dystrophy

VI. Facioscapulohumeral Muscular Dystrophy

VII. Conclusion

References

Chapter 85. Myasthenia Gravis and Myasthenic Syndromes

I. Neuromuscular Junction

II. Autoimmune Neuromuscular Junction Disorders

III. Congenital Myasthenic Syndromes

IV. What Determines the Phenotypic Variability in Neuromuscular Junction Disorders?

References

Chapter 86. Metabolic Myopathies

I. Brief History and Nomenclature

II. Etiology

III. Pathogenesis

IV. Pathophysiology

V. Pharmacology, Biochemistry, and Molecular Mechanisms

VI. Explanation of Symptoms in Relation to Pathophysiology

VII. Natural History

References

Chapter 87. Immunobiology of Autoimmune Inflammatory Myopathies

I. Introduction

II. Epidemiology and Immunogenetics

III. Clinicohistological Features

IV. Immunopathogenesis

V. Treatment: Present and Future

References

Section XVII. Autonomic Disorders

Chapter 88. Central Autonomic Network

I. Introduction

II. Functional Anatomy

III. Neurotransmitters and Neuromodulators

References

Chapter 89. Autonomic Neuropathies

I. Introduction

II. History and Nomenclature

III. Etiology

IV. Pathogenesis and Pathophysiology

V. Structural Basis

VI. Pharmacology, Biochemistry, Molecular Mechanisms

VII. Pathophysiological Basis of Symptoms

VIII. Natural History

References

Chapter 90. Thermoregulation and Its Disorders

I. Chapter Overview

II. Human Thermoregulation

III. Pyrogens and Fever

IV. Effect of Aging on Human Thermoregulation

V. Some Disorders of Thermoregulation

VI. Thermoregulatory Failure Due to Degenerative Disorders

References

Chapter 91. Control of Blood Pressure—Normal and Abnormal

I. History and Nomenclature

II. Key Elements of Short-Term Blood Pressure Regulation in Humans

III. The Physiological Problem of Upright Posture in Humans

IV. Failure of Blood Pressure Regulation during Orthostatic Stress

V. Ideas about Physiological “Causes’’ of Common Fainting

VI. Who Faints, How Often, and When?

VII. Summary

References

Section XVIII. Pain

Chapter 92. Neoplasm-Induced Pain

I. Introduction

II. Etiology

III. Pathogenesis

IV. Pain Characteristics

V. Assessment

VI. Pain Syndromes

VII. Pharmacological Treatment

VIII. Interventional Procedures

References

Chapter 93. Pain Associated with the Autonomic Nervous System

I. Introduction

II. Pain Generated by the Sympathetic Nervous System: Evidence from Experimental Investigations of Human Patients

III. Excitation of Afferent Neurons Dependent on Activity in Sympathetic Neurons

IV. Sensitization of Nociceptors Mediated by Sympathetic Terminals Independent of Excitation and Release of Noradrenaline

V. Sensitization of Nociceptors, Nerve Growth Factor, and Cytokines Mediated by Sympathetic Terminals

VI. Mechanical Hyperalgesic Behavior in the Rat Controlled by the Sympathoadrenal (SA) System

VII. Synopsis

References

Chapter 94. Postherpetic Neuralgia

I. Brief History and Nomenclature

II. Etiology and Molecular Mechanisms

III. Pathology and Natural History

IV. Pathophysiology, Biochemistry, and Pharmacology

V. Explanation of Symptoms in Relation to Pathophysiology

References

Chapter 95. Central Post-Stroke Pain

I. Introduction

II. Incidence and Prevalence

III. Pain Onset

IV. Pain Characteristics

V. Somatosensory Deficit

VI. Autonomic Changes

VII. Natural Course of the Disease

VIII. Treatment

IX. Possible Mechanisms of CPSP

References

Index