A primer of neuroimmunological disease

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1. Verfasser: Pachner, Andrew R. (VerfasserIn)
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Sprache:English
Veröffentlicht: New York Springer c2012
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adam_text Titel: A primer of neuroimmunological disease Autor: Pachner, Andrew R. Jahr: 2012 Contents 1 Immunology for the Non-immunologist...................................... 1 1 The Beginnings of Immunology............................................... 1 2 The Components of the Healthy Immune Response................. 2 2.1 White Blood Cells............................................................ 4 2.2 Molecules......................................................................... 8 References....................................................................................... 13 2 Neurology for the Non-neurologist.............................................. 15 1 Organization of the Nervous System........................................ 15 1.1 Electrical Nature: Nerve Transmission and Neurotransmitters...................................................... 15 1.2 Cells of the Nervous System............................................ 16 1.3 Structure of the Nervous System: CNS, PNS, Upper and Lower Motor Neurons.................................... 18 2 The Neurological Evaluation.................................................... 21 2.1 Neurological Examination............................................... 21 2.2 Imaging of the Nervous System....................................... 22 2.3 EEG and EMG................................................................. 23 References....................................................................................... 24 3 Neuroimmunology for the Non-neuroimmunologist.................. 25 1 The Beginnings of Neuroimmunology: Post-vaccinial Encephalomyelitis..................................................................... 25 2 Semple Rabies Vaccine Autoimmune Encephalomyelitis: Temporal Progression. Interplay Between the Nervous and Immune Systems................................................................ 29 2.1 Stage 1. DAY 0: Exposure to the Antigen....................... 29 2.2 Stage 2. Days 0-7: Lymph Node Drainage and Processing of Antigen in Peripheral Lymph Node..................................................................... 29 2.3 Stage 3. Days 7-10: Recruitment of Myelin-Specific Lymphocytes into the CNS.............................................. 30 2.4 Stage 4. Days 10-20: Maximal Inflammation with Involvement of Local CNS Immunity, Including Cervical Lymph Nodes.................................... 30 2.5 Stage 5. Days 20 and Later: Recovery............................. 31 3 The Tools of the Neuroimmunologist....................................... 31 4 Aspects of Inflammation in Neuroimmunology Unique to the Nervous System.............................................................. 32 5 The Necessity for Great Care in Classifying a Neuroimmunological Disease as Autoimmune .................. 33 6 The Importance of Antibodies.................................................. 34 References....................................................................................... 35 The Prototypic Neuroimmunological CNS Disease: Multiple Sclerosis, a Precis.......................................................... 37 1 Definition.................................................................................. 37 2 Etiopathogenesis....................................................................... 37 3 Pathology.................................................................................. 38 4 Genetics and Epidemiology...................................................... 39 5 Clinical Manifestations............................................................. 39 5.1 Initial Symptoms.............................................................. 39 5.2 The MS Attack................................................................. 40 6 Natural History and Prognosis.................................................. 42 7 MS Clinical Classifications....................................................... 43 7.1 Form of MS .................................................................. 44 7.2 Disability.......................................................................... 45 7.3 Activity............................................................................. 45 7.4 Severity............................................................................ 46 References....................................................................................... 46 Multiple Sclerosis: Diagnosis....................................................... 49 1 History and Examination.......................................................... 50 2 Laboratory Findings.................................................................. 51 2.1 Routine Studies: Blood, Urine, Chest X-Ray.................. 51 2.2 CNS Imaging................................................................... 51 2.3 Cerebrospinal Fluid Analysis........................................... 54 References....................................................................................... 55 Multiple Sclerosis Mimics............................................................ 57 1 Cerebrovascular Disease/Stroke................................................ 58 2 Neurological Infections............................................................. 60 2.1 HIV.................................................................................. 60 2.2 Neurosyphilis................................................................... 60 2.3 Neurocysticercosis........................................................... 61 2.4 Lyme Disease................................................................... 61 2.5 Tropical Spastic Paraparesis............................................ 61 2.6 Tuberculosis..................................................................... 61 2.7 Brain Abscess................................................................... 62 3 NMO......................................................................................... 62 4 ADEM....................................................................................... 63 5 Leukodystrophies...................................................................... 63 Contents 6 Other Conditions....................................................................... 63 6.1 Neurosarcoidosis.............................................................. 63 6.2 Subacute Combined Degeneration................................... 65 6.3 Other Neuroinflammatory Diseases................................. 65 References....................................................................................... 66 7 Multiple Sclerosis Therapy.......................................................... 69 1 Disease-Modifying Drugs....................................................... 69 1.1 How Medications Become Approved for Use in MS .... 69 1.2 The Primary Endpoint.................................................... 70 1.3 When Should One Treat with DMDs?........................... 71 1.4 General Approach to Therapy........................................ 71 1.5 Current Agents............................................................... 72 2 Symptomatic Therapies.......................................................... 78 References..................................................................................... 80 8 Experimental Models of MS........................................................ 83 1 Animal Models of MS............................................................ 83 1.1 Autoimmune.................................................................. 83 1.2 Viral................................................................................ 87 1.3 Demyelination Induced by Toxins................................. 89 2 In Vitro Models of MS............................................................ 89 3 Animal Models of Neuromyelitis Optica (NMO) and the Use of Passive Transfer Models................................. 89 References..................................................................................... 90 9 Guillain-Barré Syndrome (GBS) and Other Immune-Mediated Neuropathies................................................. 93 1 Definition................................................................................ 93 2 Etiopathogenesis..................................................................... 93 3 Pathology................................................................................ 95 4 Epidemiology and Etiopathogenesis....................................... 95 5 Clinical Manifestations........................................................... 96 6 Natural History and Prognosis................................................ 96 7 Classification........................................................................... 96 8 Diagnosis................................................................................. 97 9 Therapy................................................................................... 97 10 GBS s Cousin: Chronic Inflammatory Demyelinating Polyneuropathy....................................................................... 98 11 Neuropathy Associated with Monoclonal Gammopathy........ 98 12 Experimental Neuritis............................................................. 100 References....................................................................................... 101 10 Neuromuscular Neuroimmunological Diseases: Myasthenia Gravis........................................................................ 103 1 Definition................................................................................ 103 2 Etiopathogenesis..................................................................... 103 3 Pathology................................................................................ 104 4 Genetics and Epidemiology.................................................... 105 Contents 5 Clinical Manifestations........................................................... 105 6 Natural History and Prognosis................................................ 106 7 Classification........................................................................... 106 8 Diagnosis................................................................................. 106 9 Therapy................................................................................... 108 10 Lambert-Eaton Myasthenic Syndrome................................... 108 11 Neonatal MG and Arthrogryposis Multiplex Congenita........ 109 12 Experimental Models of MG.................................................. 109 References....................................................................................... 110 11 The Prototypic Neuroimmunological Muscle Disease: Dermatomyositis........................................................................... 111 1 Definition................................................................................ 111 2 Etiopathogenesis..................................................................... 111 3 Pathology................................................................................ 111 4 Genetics and Epidemiology.................................................... 112 5 Clinical Manifestations........................................................... 112 6 Natural History and Prognosis................................................ 112 7 Classification........................................................................... 112 8 Diagnosis................................................................................. 112 9 Therapy................................................................................... 113 10 Related Neuroimmunological Muscle Diseases..................... 113 11 Experimental Models of Inflammatory Muscle Disease......... 114 References....................................................................................... 114 12 A Introduction to Neurological Infections: Neuro-infectious Disease as Part of Neuroimmunology......................................... 115 1 General Rules of Neuro-infectious Diseases.......................... 116 1.1 Infection of the Nervous System Follows Systemic Infection......................................................................... 116 1.2 Neuro-infectious Diseases Should Be in the Differential Diagnosis in Many Patients with Difficult Neurological Syndromes Because of Their Reversibility with Antibiotics...................................................................... 116 1.3 Diagnosis by Identification of the Infectious Agent Is Ideal, but Sometimes Impossible..................... 116 1.4 Prompt Recognition of Curable Infectious Diseases Is Critical........................................................................ 117 1.5 Therapy of Neuro-infectious Diseases Is Frequently More Difficult than Therapy of Infections Elsewhere in the Body..................................................................... 119 1.6 Always Keep HIV/AIDS in Mind.................................. 119 2 Lyme Meningitis: The Prototypic Meningitis......................... 119 2.1 Definition....................................................................... 119 2.2 Etiopathogenesis............................................................ 119 2.3 Clinical Manifestations.................................................. 121 2.4 Natural History/Prognosis.............................................. 122 Contents 2.5 Diagnosis........................................................................ 122 2.6 Therapy.......................................................................... 123 2.7 Classification.................................................................. 123 2.8 Selected Important Infectious Meningitides.................. 123 3 Herpes Simplex Encephalitis: The Prototypic CNS Parenchymal Infection............................................................ 125 3.1 Definition....................................................................... 125 3.2 Etiopathogenesis/Pathology/Genetics/ Epidemiology................................................................. 125 3.3 Clinical Manifestations.................................................. 126 3.4 Natural History and Prognosis....................................... 127 3.5 Diagnosis........................................................................ 127 3.6 Therapy.......................................................................... 128 3.7 Other Important CNS Parenchymal Infections................ 128 3.8 Rabies, Poliomyelitis, and the Wonder of Vaccination.................................................................. 132 4 Infectious Neuritis: Neuropathies of Leprosy and Varicella Zoster Virus............................................................... 133 4.1 Leprosy............................................................................ 134 4.2 VZV Infection.................................................................. 136 4.3 Immunosuppressed Patients............................................. 136 References....................................................................................... 139 13 Neurological Manifestations of Systemic Chronic Inflammatory Disease................................................................... 141 1 Neurosarcoidosis....................................................................... 141 2 Systemic Lupus Erythematosus................................................ 141 3 Antiphospholipid Antibody Syndrome..................................... 142 4 Sjogren s Syndrome.................................................................. 143 5 Vasculitis................................................................................... 144 References....................................................................................... 146 14 The Neuroimmunology of Cancer: Paraneoplastic Syndromes and Primary CNS Lymphoma .......................... 147 1 Paraneoplastic Neurological Disorders..................................... 147 1.1 Definition......................................................................... 147 1.2 Etiopathogenesis.............................................................. 147 1.3 Pathology......................................................................... 148 1.4 Genetics and Epidemiology............................................. 148 1.5 Clinical Manifestations.................................................... 148 1.6 Natural History and Prognosis of PND............................ 151 1.7 Classification.................................................................... 151 1.8 Diagnosis.......................................................................... 152 1.9 Therapy...................................................................••........ 152 2 CNS Lymphoma........................................................................ 152 References....................................................................................... 153 Contents 15 Neuroimmunology of Degenerative Diseases and Stroke.......... 155 1 Introduction............................................................................... 155 2 Neuroimmunology of Alzheimer s Disease.............................. 155 3 Neuroimmunology of Parkinson s Disease............................... 156 4 Neuroimmunology of Stroke.................................................... 157 References....................................................................................... 158 16 Important Rare Neuroimmunological Diseases......................... 159 1 Infectious/Postinfectious........................................................... 159 1.1 Subacute Sclerosing Panencephalitis............................... 159 1.2 Whipple s......................................................................... 160 1.3 Post-Varicella Zoster Virus Cerebellar Syndrome........... 161 1.4 Sydenham s Chorea......................................................... 161 2 Autoimmune............................................................................. 162 2.1 Acquired Neuromyotonia................................................. 162 2.2 Stiff Person Syndrome..................................................... 162 2.3 Swine-Worker s Neuropathy............................................ 163 3 Idiopathic.................................................................................. 163 3.1 Hashimoto s Encephalopathy........................................... 163 3.2 Behcet s Syndrome.......................................................... 164 3.3 Rasmussen s Encephalitis................................................ 164 3.4 Acute Multifocal Placoid Pigment Epitheliopathy.......... 164 References....................................................................................... 164 17 Neuroimmunological Molecules and Translational Medicine......................................................................................... 167 1 Moving from Basic Science Discoveries to FDA-Approved Therapies................................................................................... 167 2 Biological Molecules as Therapies........................................... 167 2.1 Therapeutic Monoclonal Antibodies................................ 167 2.2 Molecules as Targets of Therapy or of Disease: CD20, Aquaporin-4 Receptor, TNF-a, Sphingosine- 1-Phosphate Receptors, CD52, MOG, Channels............. 169 2.3 Neuroprotection: The Holy Grail of Translational Neurology........................................................................ 172 References....................................................................................... 173 18 Neuroimmunological Diagnostic Tests........................................ 175 1 General Considerations for Testing.......................................... 175 1.1 Validation of Laboratory Tests by Regulatory Authorities........................................................................ 176 2 Blood Tests................................................................................ 176 2.1 Blood Tests for Inflammation.......................................... 176 2.2 Blood Tests for Specific Immunity to Pathogens or Autoantibodies................................................................. 177 2.3 Blood Tests for Detecting Specific Pathogens................. 182 Contents 3 Cerebrospinal Fluid (CSF)........................................................ 183 3.1 Antibodies in the CSF...................................................... 185 3.2 Detection of Pathogens in the CSF.................................. 186 4 Tests of Immune Function........................................................ 187 4.1 Humoral Immunity........................................................... 187 4.2 Cellular Immunity............................................................ 188 References....................................................................................... 188 19 Therapy in Neuroimmunological Disease................................... 189 1 Introduction............................................................................... 189 2 Chemicals.................................................................................. 190 2.1 Corticosteroids................................................................. 190 2.2 Immunosuppressives........................................................ 192 3 Biologies: Large Molecules Produced via Recombinant Technology or from Human Specimens................................... 196 3.1 Intravenous Immunoglobulin........................................... 196 3.2 Cytokines......................................................................... 196 3.3 Monoclonal Antibodies (See Sect. 2.1 in Chap. 17)........ 197 4 Procedures................................................................................. 197 4.1 Plasmapheresis................................................................. 197 4.2 Thymectomy.................................................................... 198 References....................................................................................... 198 Index....................................................................................................... 199
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spellingShingle Pachner, Andrew R.
A primer of neuroimmunological disease
Nervous system Diseases Immunological aspects
Autoimmune Diseases of the Nervous System diagnosis
Autoimmune Diseases of the Nervous System therapy
title A primer of neuroimmunological disease
title_auth A primer of neuroimmunological disease
title_exact_search A primer of neuroimmunological disease
title_full A primer of neuroimmunological disease Andrew R. Pachner
title_fullStr A primer of neuroimmunological disease Andrew R. Pachner
title_full_unstemmed A primer of neuroimmunological disease Andrew R. Pachner
title_short A primer of neuroimmunological disease
title_sort a primer of neuroimmunological disease
topic Nervous system Diseases Immunological aspects
Autoimmune Diseases of the Nervous System diagnosis
Autoimmune Diseases of the Nervous System therapy
topic_facet Nervous system Diseases Immunological aspects
Autoimmune Diseases of the Nervous System diagnosis
Autoimmune Diseases of the Nervous System therapy
url http://bvbr.bib-bvb.de:8991/F?func=service&doc_library=BVB01&local_base=BVB01&doc_number=027389509&sequence=000002&line_number=0001&func_code=DB_RECORDS&service_type=MEDIA
work_keys_str_mv AT pachnerandrewr aprimerofneuroimmunologicaldisease