Autoimmune Enzephalitiden

Zusammenfassung

Autoimmune Enzephalitiden können durch Autoimmunreaktionen gegen extrazellulär auf Nervenzellen exprimierte Proteine oder gegen intrazellulär oder/und nukleär lokalisierte Proteine verursacht werden. Bisher sind bereits etwa 20 verschiedene Autoantigene beschrieben worden, die bei einer autoimmunen Enzephalitis die Zielstrukturen für die adaptive Immunantwort (T Zellen, B Zellen, Autoantikörper) sind. Ein Teil der Enzephalitiden kann eine paraneoplastische Ursache haben. Die Diagnose ist häufig schwierig zu finden, da die Symptome viele zerebrale Funktionssysteme betreffen, Erkrankungen aus dem klassischen psychiatrischen Formenkreis imitieren können und der Diagnoseaufwand hoch ist. Die Therapie sollte abhängig von dem der Erkrankung zu Grunde liegenden immunologischen Pathomechanismus gewählt werden und bei paraneoplastischen Formen ist zudem die Behandlung des zugrundeliegenden Tumorleidens unumgänglich.

Abstract

Autoimmune encephalitis can be caused by autoimmune reactions against extracellular proteins expressed on nerve cells or against intracellular or/and nuclear localised proteins. Up to now about 20 different autoantigens have been described that can be the target structures for the adaptive immune response (T cells, B cells, autoantibodies). Some of the autoimmune encephalitis forms can have a paraneoplastic origin. The diagnosis is often difficult because the symptoms can arise from multiple cerebral functional systems and can imitate those of classic psychiatric diseases so that the diagnostic effort and cost can be high. The therapeutic approach depends on the underlying immunological pathomechanisms and in the case of paraneoplastic forms, therapy for the underlying malignancy is mandatory.

Schlüsselwörter

Autoimmune Enzephalitis - paraneoplastisch - Autoantigen - Neuron - Immuntherapie

Key words

autoimmune encephalitis - paraneoplastic - autoantigen - neuron - immune therapy

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