Hohlfeld, Reinhard

Kappos, Ludwig

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INTRODUCTIONProgress in understanding inflammatory and autoimmunediseases of the central nervous systemReinhard Hohlfeld & Ludwig KapposReceived: 28 September 2009 /Accepted: 3 November 2009 /Published online: 3 December 2009# Springer-Verlag 2009This issue of Seminars in Immunopathology is devoted tothe topic of inflammatory and autoimmune diseases of thecentral nervous system (CNS). As editors, we are gratifiedthat a panel of distinguished expert colleagues kindlyaccepted our invitation to contribute an article, eachcovering an essential aspect of the overall topic. Takentogether, the articles cover a broad spectrum of neuro-immunology and neuroinflammation.In the first article, Wei Hu and Claudia F. Lucchinettidiscuss the neuropathological spectrum of CNS inflamma-tory demyelinating diseases. The authors emphasize theemerging heterogeneity of these disorders, with multiplesclerosis (MS) being the most common but by no meansonly the disease in this category. They also provide us withan update on the exciting new developments in neuro-myelitis optica, a disorder which has recently beenredefined by the presence of autoantibodies against theaquaporin-4 water channel. The second article, authored byMonika Bradl and Hans Lassmann, focuses on progressiveMS, the “step-child of MS therapeutics.” The authors demon-strate that also in progressive MS, inflammation is the drivingforce for CNS injury, and they present plausible explanationswhy it is so difficult to treat progressive forms of MS.In the third article, Klaus Dornmair, Edgar Meinl, andReinhard Hohlfeld discuss novel approaches for identifyingtarget antigens of autoreactive human B and T cells.Whereas many antigens have shown encephalitogenicproperties in animal models, it has been difficult to identifythe target antigens of pathogenic immune reactions inhuman diseases. New techniques allow the isolation ofindividual tissue-infiltrating B and T cells, so that theirclonotypical antigen-specific receptors can be reconstructedfrom single cells. The reconstructed antigen-specific recep-tors are then expressed in living cell lines, which can beused in the search for unknown target antigens.In the next article, merits and shortcomings of animalmodels of MS are critically discussed in detail by BettinaSchreiner, Frank L. Heppner, and Burkhard Becher. Variousexperimental autoimmune encephalomyelitis (EAE) modelshave greatly contributed to our current understanding ofMS pathogenesis, albeit it has been difficult to bridge thegap between EAE and MS research. The most recentgeneration of EAE models make extensive use of geneticengineering methods, such as transgenic and knockouttechniques, allowing detailed studies of various aspects ofimmunopathogenesis. Special emphasis is placed on mod-els elucidating the role of newly emerging subsets ofautoimmune T cells, including TH17 cells and regulatory Tcells (Treg).Britta Engelhardt and Lydia Sorokin provide firsthandinsight into the intricate workings of one of the mostessential areas of neuroinflammation, the blood–brain andblood–cerebrospinal fluid barriers. The authors discuss thestructure of the blood–brain barrier under physiologicalconditions and emphasize how changes of its functionbecome crucially important in pathological conditions. Theblood–brain barrier is also a key site for therapeuticintervention, as recently evidenced by the approval of thetherapeutic monoclonal antibody natalizumab, which isdirected against alpha-4-beta-1-integrin and inhibits theinflux of autoaggressive T cells into the CNS.L. Kappos (*)Neurology and Department of Biomedicine, University Hospital,Basel, Switzerlande-mail: lkappos@uhbs.chR. HohlfeldInstitute of Clinical Neuroimmunology,Ludwig Maximilians University of Munich,Munich, Germanye-mail: reinhard.hohlfeld@med.uni-muenchen.deSemin Immunopathol (2009) 31:437–438DOI 10.1007/s00281-009-0184-1Lisa Walter and Harald Neumann then authoritativelydiscuss the role of microglia in neuronal degeneration andregeneration, emphasizing that microglia has a double-sidednature because it can both mediate neuroprotection andneurotoxicity.Neil J. Scolding provides an overview on CNS vascu-litis, describing different subtypes of vasculitis and discus-sing diagnostic and therapeutic approaches.In the last article of this issue, Jan Bauer andChristian G. Bien consider different kinds of encephalitisassociated with epilepsy: Rasmussen encephalitis, para-neoplastic encephalitis and certain forms of nonparaneo-plastic encephalitis.We hope that the readers are as excited as we are aboutthis unique collection of articles covering a wide spectrumof current basic and clinical neuroimmunology. We wouldlike to thank all authors and co-authors for their excellentcontributions and, last but not least, would like to expressour gratitude to the editorial staff of Springer for compe-tently supporting us in preparing this issue.438 Semin Immunopathol (2009) 31:437–438

Progress in understanding inflammatory and autoimmune diseases of the central nervous system

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Progress in understanding inflammatory and autoimmune diseases of the central nervous system

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