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Autoimmune Encephalitides: A Broadening Field of Treatable Conditions

Kalman, Bernadette MD, PhD, DSc

doi: 10.1097/NRL.0000000000000087
Review Article

Background: Neurology has been continuously transforming by the refinement of molecular diagnostics and the development of disease-modifying treatments. The discovery of new antibody markers has elucidated the pathogenesis, provided the means of diagnostics, and offered cure or treatment for several immune-mediated neurological and neuropsychiatric disorders. The identification of pathogenic and marker autoantibodies has also facilitated defining the associated phenotypic spectra and the overlap among the phenotypes linked to individual immune markers.

Review Summary: This survey presents the list of currently known autoimmune encephalitis entities along with the associated marker autoantibodies, highlights the phenotypic and immune pathogenic relationships, calls attention to the recently described rare syndromes, discusses the biological significance of the autoantibodies and targeted molecules, points out the potential postinfectious origin of immune pathogenesis in several of the disorders, and directs the readers to the latest diagnostic guidelines as well as to the generally used treatment approaches.

Conclusions and Future Directions: Owing to the successful and usually combined use of various methods to detect serum and cerebrospinal fluid autoantibodies on rodent brain sections, in primary neuronal cell culture, in immune precipitation, and cell-based assays, or in other antigen-specific immune assays (Western blot, enzyme-linked immunosorbent assay, and radioimmune assay), the subgroup of antibody marker-negative autoimmune encephalopathy syndromes is contracting, whereas the numbers of entities within the overall group are expanding. Recognition of the correct diagnosis is becoming increasingly rewarding not only for neurologists, but also for pediatric neurologists and psychiatrists.

*University of Pecs, Pecs

Markusovszky University Teaching Hospital, Center for Research and Education, Szombathely, Hungary

The present scientific contribution is dedicated to the 650th anniversary of the foundation of the University of Pecs, Hungary.

In 2015, B.K. received honorarium from Pfizer for a talk not related to this review and travel support from Genzyme for attending the AAN meeting in Washington, DC. B.K.’s salary is supported by the University of Pecs and the Markusovszky University Teaching Hospital.

Reprints: Bernadette Kalman, MD, PhD, DSc, Markusovszky University Teaching Hospital, Markusovszky Street 5., 9700 Szombathely, Hungary. E-mail: kalman.bernadett@markusovszky.hu.

Copyright © 2017 Wolters Kluwer Health, Inc. All rights reserved.