Klinische Phänomenologie der Autoimmunenzephalitiden

Zusammenfassung

Antikörperassoziierte Störungen des zentralen Nervensystems bilden eine heterogene Gruppe von Erkrankungen, die grob in zwei Kategorien unterteilt werden können: klassische paraneoplastische Syndrome mit sogenannten onkoneuralen Antikörpern (paraneoplastic neurological disorders, PND) und autoimmune Störungen mit Antikörpern gegen Antigene der neuronalen Oberflächen und Synapsen (autoimmune Enzephalitis, AE). Die Entdeckung der Autoimmunenzephalitiden hat zu einem Paradigmenwechsel in der Diagnose und Therapie sowie einer Reklassifizierung einiger neuropsychiatrischer Syndrome geführt, die zuvor als idiopathisch eingeordnet oder lediglich mit deskriptiven Begriffen belegt wurden.

In diesem Übersichtsartikel soll besonders auf klinisch-syndromale Aspekte der Autoimmunenzephalitiden eingegangen werden, da es diesbezüglich innerhalb der letzten zehn Jahre einen rasanten Wissenszuwachs gegeben hat, man zudem zunehmend Overlap-Syndrome und Assoziationen zu anderen Krankheitsentitäten feststellt. Neben allgemeinen Aspekten werden Charakteristika der anti-NMDAR-, anti-LGI1-, anti-GABA_A und GABA_BR-, anti-AMPAR-, anti-CASPR2-, anti-mGluR-, anti-GlycinR-, anti-GAD-, anti-DPPX- und anti-D2R-Enzephalitis sowie der anti-IgLON5-Enzephalopathie dargestellt.

Abstract

Antibody-associated disorders of the central nervous system constitute a heterogeneous group of disorders that can be roughly divided into two categories: Classic paraneoplastic syndromes associated with so-called well-characterized antibodies (paraneoplastic neurological disorders, PND) and autoimmune disorders with antibodies to membrane-bound or synaptic antigens (autoimmune encephalitis, AE). The discovery of autoimmune encephalitis has led to a paradigm shift in diagnosis and therapy as well as a reclassification of some neuropsychiatric syndromes that were previously classified as idiopathic or simply covered with descriptive terms.

In this review article, especially clinical aspects of autoimmune encephalitis will be discussed, as there has been a rapid increase in knowledge in this regard within the past decade; increasingly overlap syndromes and associations with other disease entities have been detected. In addition to general aspects, characteristics of anti-NMDAR-, anti-LGI1-, anti-GABA_A and GABA_BR, anti-AMPAR-, anti-CASPR2-, anti-mGluR, anti-GlycinR-, anti-GAD, anti- DPPX- and anti-D2 R encephalitis and the anti-IgLON5 encephalopathy will be presented.

Schlüsselwörter

Autoimmunenzephalitis - paraneoplastisch - neuronale Oberflächen-Antikörper - klinische Charakteristik - Herpes-simplex-Enzephalitis

Key words

autoimmune encephalitis - paraneoplastic - neuronal surface antibodies - clinical characteristics - herpes simplex encephalitis

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