Induction of Central Nervous System Disease by the Adaptive Immune Response

Front Cover
Robert Weissert, Fabienne Brilot
Frontiers Media SA, Nov 23, 2017 - Electronic book

 Over the last years it has become evident that many neurological diseases of the central nervous system (CNS) are induced by a specific adaptive immune response directed against molecules expressed on CNS-resident cells. Well-recognized examples are anti-N-Methyl-D-Aspartate Receptor (NMDAR) encephalitis which is characterized by the presence of antibodies against neuron-expressed NMDAR, or neuromyelitis optica (NMO), induced by antibodies to astrocyte-expressed aquaporin-4. Many more examples exist, and antibodies, and T or/and B cells have increasingly been associated with CNS disease. Often the symptoms of these diseases have not been typically reported to have an immune aetiology. Beside classical neurological symptoms like ataxia, vision disturbance, and motor or sensory symptoms, these can include cognitive disturbances, behavioral abnormalities, or/and epileptic seizures. Although much has been learned regarding the pathophysiology of prototypic examples of these disorders, there are still major gaps in our understanding of their biology. This may be due to the fact that they are rare diseases, and their therapies are still very limited. This research topic includes contributions addressing the analysis of the adaptive immune response driving disease including target antigens, molecular epitope mapping, and factors involved in the disease pathogenesis such as complement activation cascades, genetic and genomic regulation, as well as environmental triggers. Diagnostic criteria and methods, and treatment are also discussed. The overall aim of the volume is to review progress in our pathophysiological understanding of immune-mediated CNS disorders in order to advance diagnostic and therapeutic approaches, and ultimately improve outcomes for patients. 


 

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Contents

Induction of Central Nervous System Disease by the Adaptive Immune Response
6
Untangling Apparent Inconsistencies for Clinical Practice
8
How Autoantibodies Circumvent the BloodBrain Barrier in Autoimmune Encephalitis
15
Are There Implications for Depression?
30
Expanding Role of T Cells in Human Autoimmune Diseases of the Central Nervous System
42
Adaptive Immunity Is the Key to the Understanding of Autoimmune and Paraneoplastic Inflammatory Central Nervous System Disorders
59
The Multiple Sclerosis MS Genetic Risk Factors Indicate both Acquired and Innate Immune Cell Subsets Contribute to MS Pathogenesis and Identify ...
69
Different Phenotypes at Onset in Neuromyelitis Optica Spectrum Disorder Patients with Aquaporin4 Autoimmunity
75
Increased Circulating T Follicular Helper Cells Are Inhibited by Rituximab in Neuromyelitis Optica Spectrum Disorder
84
Deciphering a Target in Inflammatory Demyelinating Diseases
93
Reactivity to Novel Autoantigens in Patients with Coexisting Central Nervous System Demyelinating Disease and Autoimmune Thyroid Disease
108
Autophagy and AutophagyRelated Proteins in CNS Autoimmunity
121
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