Cell-surface CNS autoantibodies: Clinical relevance and emerging paradigms. The recent discovery of several potentially pathogenic autoantibodies has helped identify patients with clinically-distinctive central nervous system diseases that appear to benefit from immunotherapy. The associated autoantibodies are directed against the extracellular domains of cell-surface expressed neuronal or glial proteins such as LGI1, the NMDA-receptor and aquaporin-4. The original descriptions of the associated clinical syndromes were phenotypically well-circumscribed. However, as availability of antibody testing has increased, the range of associated patient phenotypes and demographics has expanded. This, in turn, has led to the recognition of more immunotherapy-responsive syndromes in patients presenting with cognitive and behavioural problems, seizures, movement disorders, psychiatric features, and demyelinating disease. While antibody detection remains diagnostically important, clinical recognition of these distinctive syndromes should ensure early and appropriate immunotherapy administration. We review the emerging paradigm of cell-surface directed antibody-mediated neurological diseases, describe how the associated disease spectrums have broadened since the original descriptions, discuss some of the issues regarding antibody detection and syndrome definitions, and emphasize considerations surrounding immunotherapy administration.
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