Table of Contents
1. Basic Concepts and Glossary of Common Clinical Terms Definition of the Peripheral Nervous System (PNS) Relationships Fundamental to an Understanding of Disease of Peripheral Nerve Fibers Neuron Cell Body and Axon Axon, Schwann Cell and Myelin Axon and End Organ Wallerian Degeneration and Axon Regeneration Glossary of Common Clinical Terms 2. Anatomic Classification of Peripheral Nervous System Disorders Symmetric Generalized Neuropathies (Polyneuropathies) Distal Axonal Degeneration Segmental (Non-Uniform) Myelinopathy Diffuse (Uniform) Myelinopathy Neuronopathy (Ganglionopathy) Focal (Mononeuropathy) and Multifocal (Multiple Mononeuropathy) Neuropathies Ischemia Infiltration Physical Injuries 3. Evaluation and Management of the Patient with Peripheral Neuropathy General Principles and the Algorithmic Approach Chronic Idiopathic Axonal Polyneuropathy / Small Fiber Neuropathy Treatment of Neuropathic Pain Differential Diagnoses and Work-ups for the Varied Neuropathy Phenotypes (Tables) Sensorimotor Polyneuropathies - Axonal Sensorimotor Polyneuropathies - Demeyelinating or Mixed Sensory, Small Fiber, Painful Polyneuropathies Sensory, Large Fiber, Ataxic Polyneuropathies Motor Neuropathies Autonomic Neuropathies Mononeuropathy Multiplex Myeloneuropathies Neuromyopathies Polyneuropathy and Optic Neuropathy Polyradiculopathies Plexopathies/Radiculoplexopathies Facial Neuropathy Trigeminal Sensory Neuropathy Unusual Neuropathy Patterns 4. Electrodiagnostic, Imaging, Nerve and Skin Biopsy Investigations in Peripheral Nerve Disease Electromyography and Nerve Conduction Studies Sensory Nerve Conduction Studies Motor Nerve Conduction Studies Late Responses Blink Reflex Studies Needle Electromyography Studies of Autonomic Function Quantitiative Sudomotor Axon Reflex Test Thermoregulatory Sweat Test Sympathetic Skin Response Heart Rate Response to Deep Breathing Valsalva Maneuver Quantitative Sensory Testing Developing Electrophysiologic and Imaging Techniques Motor Unit Number Estimation Electrical Impedence Myography High-Resolution Sonography of Peripheral Nerve Magnetic Resonance Neurography Muscle MRI Positron Emission Tomography Nerve Biopsy Skin Biopsy 5. Case Presentations Illustrating the Diagnostic Method Case 1: Painful small fiber neuropathy and dysautonomia Case 2: Insidious onset of distal weakness in an adult with deformed feet Case 3: Lower limb paresthesias in a middle-aged adult with diabetes. Case 4: A middle-aged woman with muscle twitching and episodic numbness Case 5: Six days of cranial neuropathies and hyporeflexia Case 6: A two-month onset of sensory neuropathy in a woman with ovarian carcinoma Case 7: A 47-year-old man with 10 years of progressive bilateral hand weakness Case 8: Chronic sensory loss and unsteady gait in a 59-year-old woman Case 9: An elderly man with acral paresthesias and gait unsteadiness Case 10: Foot drop in an 81-year-old woman Case 11: A middle-aged man with multifocal pain, sensory loss and weakness Case 12: Five-day onset of diffuse weakness 6. Acute Immune-mediated Neuropathies Demyelinating Immune-mediated Neuropathies Acute Inflammatory Demyelinating Polyradiculoneuropathy (AIDP) and Fisher Syndrome (FS) Axonal Immune-mediated Neuropathies Acute Motor Axonal Neuropathy (AMAN) and Acute Motor and Sensory Axonal Neuropathy (AMSAN) Neuronopathies Sensory (Idiopathic, Sjogren Syndrome, Paraneoplastic) and Motor (Paraneoplastic) Neuronopathies and Autoimmune Autonomic Ganglionopathy 7. Chronic Immune-mediated Neuropathies Chronic Inflammatory Demyelinating Polyradiculoneuropathy (CIDP) CIDP Variants: Multifocal Motor Neuropathy with Conduction Block (MMN) Lewis-Sumner Syndrome (LSS)/Multifocal Acquired Demyelinating Sensory and Motor Neuropathy (MADSAM) Distal Acquired Demyelinating Symmetric Neuropathy (DADS) CIDP with CNS Features Chronic Immune Sensory Polyradiculopathy (CISP) Chronic Sensory Demyelinating Polyneuropathy 8. Neuropathies Associated with Monoclonal Gammopathies and Cancer Multiple Myeloma Osteosclerotic Myeloma Primary Systemic Amyloidosis (AL Amyloidosis) Waldenstrom Macroglobulinemia Monoclonal Gammopathy of Undetermined Significance 9. Infectious and Granulomatous Neuropathies Herpes Zoster/Herpes Simplex Leprosy Sarcoidosis Lyme Disease HIV Hepatitis C/Cryoglobulinemia Diphtheria 10. Diabetic and Other Endocrine Neuropathies Diabetic Neuropathies Distal Symmetric Sensorimotor/Autonomic Polyneuropathy Proximal Multifocal Neuropathies Lumbosacral Radiculoplexus Neuropathy Thoracolumbar “Truncal” Radiculoneuropathy Focal Limb Neuropathies (Entrapment Sites) Isolated Cranial Neuropathies Acute Painful Neuropathy (“Diabetic Neuropathic Cachexia”) Diabetic Motor-predominant Neuropathies Treatment-induced Neuropathy (“Insulin Neuritis”) Hyperglycemic Neuropathy Acromegalic Neuropathy Hypothyroid Neuropathy 11. Neuropathies Associated with Vitamin Deficiencies and Malabsorption Vitamin B12 (Cobalamin) Deficiency Vitamin B1 (Thiamine) Deficiency Celiac Disease Vitamin E (?-tocopherol) Deficiency Copper Deficiency Other: Cuban Epidemic Optic and Peripheral Neuropathy; Deficiencies: Riboflavin (B2), Pyridoxine (B6), Folate, Zinc; Bariatric Surgery 12. Vascular/Ischemic Neuropathies Vasculitic Neuropathies Neuropathies Associated with Peripheral Arterial Occlusive Disease Neuropathies Associated with Compartment Syndromes 13. Neuropathies Associated with Organ Failure Pulmonary Failure Hepatic Failure Renal Failure Uremic Polyneuropathy Mononeuropathies Ischemic Monomelic Neuropathy Organ Transplantation Critical Illness Polyneuropathy Differential Diagnosis 14. The Hereditary Neuropathies Hereditary Motor and Sensory Neuropathies Charcot-Marie Tooth Disease, Type 1 Hereditary Neuropathy with Liability to Pressure Palsy Charcot-Marie Tooth Disease, Type 2 Dejerine-Sottas Disease/Congenital Hypomyelinating Neuropathy Charcot-Marie Tooth Disease, Type 4 Charcot-Marie Tooth, Type X Charcot-Marie-Tooth Disease-Dominant Intermediate Hereditary Sensory and Autonomic Neuropathies Distal hereditary Motor Neuropathy/Neuronopathy Hereditary Ataxia with Neuropathy Autosomal Dominant Autosomal Recessive X-linked Hereditary Spastic Paraplegia with Neuropathy Hereditary Brachial Plexus Neuropathy/Hereditary Neuralgic Amyotrophy Hereditary Peripheral Nerve Channelopathies Sodium Channelopathies Potassium Channelopathies 15. Hereditary Metabolic/Multisystem Disorders with Neuropathy Familial Amyloid Polyneuropathies Disorders of Lipid Metabolism Lysosomal Disorders Fabry Disease Leukodystrophies Metachromatic Leukodystrophy Krabbe Disease Peroxisomal Disorders Refsum Disease Adrenomyeloneuropathy Lipoprotein Deficiencies Tangier Disease Abetalipoproteinemia Familial Hypobetalipoproteinemia Cerebrotendinous Xanthomatosis Disorders of Heme Metabolism - Porphyria Disorders of Defective DNA Repair Mitochondrial Disorders Neuroacanthocytosis Syndromes Chorea-Acanthocytosis McLeod Syndrome Neurofibromatous Neuropathy Glycogen Storage Diseases Adult Polyglucosan Body Disease 16. The Toxic Neuropathies: Principles of General and Peripheral Neurotoxicology; Pharmaceutical Agents Principles of General Neurotoxicology Principles of Peripheral Neurotoxicology Peripheral Neurotoxicity Associated with Pharmaceutical Agents Amiodarone Bortezomib Colchicine Dapsone Disulfiram Ethambutol Ethanol Isoniazid Metronidazole Misonidazole Nitrous Oxide Nucleoside Analogues Phenytoin Platinum Pyridoxine Suramin Tacrolimus Taxanes Thalidomide Vinca Alkaloids 17. The Toxic Neuropathies: Industrial, Occupational and Environmental Agents Peripheral Neurotoxicity Associated with Industrial, Occupational and Environmental Agents Arsenic Ethylene Oxide Hexacarbons (n-hexane) Lead Methyl Bromide Organophosphates Thallium 18. Focal neuropathies: Nerve injuries, Entrapments and Other Mononeuropathies Nerve Injuries Anatomy and Pathophysiology of Nerve Injuries Clinical Classification or Nerve Injuries Electrodiagnostic Features of Nerve Injuries Nerve Regeneration and Repair Focal Neuropathies: The Upper Extremity Median Nerve Ulnar Nerve Radial Nerve Other Upper Extremity Mononeuropathies Focal Neuropathies: The Lower Extremity Sciatic nerve Peroneal Nerve Tibial Nerve Femoral Nerve Lateral Femoral Cutaneous Nerve Other Lower Extremity Mononeuropathies Focal Neuropathies: Cranial Neuropathies Idiopathic Facial Nerve Paralysis (Bell's palsy) 19. Plexopathies Brachial Plexopathy Anatomy Etiology Trauma Thoracic Outlet Syndrome Neoplastic Brachial Plexopathy Radiation-induced Brachial Plexopathy Immune Brachial Plexus Neuropathy (Neuralgic Amyotrophy) Lumbosacral Plexopathy Anatomy Etiology Trauma and Ischemia Retroperitoneal Hemorrhage Neoplastic Lumbosacral Plexopathy Radiation-induced Lumbosacral Plexopathy Non-diabetic Lumbosacral Radiculoplexus Neuropathy 20. Disorders of Peripheral Nerve Hyperexcitability Generalized Neuromyotonia Cramps Fasciculations Tetany Localized Facial Myokymia Localized or Focal Myokymia Hemifacial Spasm Hemimasticatory Spasm Hypothenar Dimpling Index
