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<p>The term autoimmune encephalitis (AE) refers to a&#xa0;group of immune-mediated central nervous system disorders characterized by distinct clinical syndromes, different pathomechanisms and varying target antigens. In addition to established diagnostic criteria, recognition of characteristic clinical symptoms should strengthen the clinical suspicion. Antibody testing is central to the diagnosis; however, its correct interpretation requires an understanding of the underlying assay principles. For example, antibodies against surface antigens, such as NMDAR, LGI1 or CASPR2 can only be reliably detected using cell-based assays (CBA) and low-titer serum findings can lead to misdiagnosis. Importantly, accurate diagnosis based on both the clinical presentation and antibody testing is crucial for guiding treatment. In cases involving antibodies against surface antigens, immunotherapy is often effective. In cases with onconeural (paraneoplastic) antibodies, treatment of the underlying tumor takes priority. Acute treatment options include glucocorticoids, intravenous immunoglobulins and apheresis procedures, while rituximab is most commonly used for relapse prevention. Emerging therapeutic approaches include anti-CD19 B‑cell-depleting strategies, FcRn blockade and proteasome inhibitors and are currently being evaluated in randomized clinical trials.</p>

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Autoimmunenzephalitis

  • 期刊论文

  • 发表日期：

    2026年6月18日

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  • M. Brokbals,
  • M. Krämer,
  • F. Leypoldt

摘要

The term autoimmune encephalitis (AE) refers to a group of immune-mediated central nervous system disorders characterized by distinct clinical syndromes, different pathomechanisms and varying target antigens. In addition to established diagnostic criteria, recognition of characteristic clinical symptoms should strengthen the clinical suspicion. Antibody testing is central to the diagnosis; however, its correct interpretation requires an understanding of the underlying assay principles. For example, antibodies against surface antigens, such as NMDAR, LGI1 or CASPR2 can only be reliably detected using cell-based assays (CBA) and low-titer serum findings can lead to misdiagnosis. Importantly, accurate diagnosis based on both the clinical presentation and antibody testing is crucial for guiding treatment. In cases involving antibodies against surface antigens, immunotherapy is often effective. In cases with onconeural (paraneoplastic) antibodies, treatment of the underlying tumor takes priority. Acute treatment options include glucocorticoids, intravenous immunoglobulins and apheresis procedures, while rituximab is most commonly used for relapse prevention. Emerging therapeutic approaches include anti-CD19 B‑cell-depleting strategies, FcRn blockade and proteasome inhibitors and are currently being evaluated in randomized clinical trials.