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Fortschr Neurol Psychiatr 2016; 84(05): 271-280
DOI: 10.1055/s-0042-104194
Übersicht
© Georg Thieme Verlag KG Stuttgart · New York

Klinische Phänomenologie der Autoimmunenzephalitiden

Clinical Phenomenology of Autoimmune Encephalitis
J. F. Holle
1   Klinik und Poliklinik für Psychiatrie und Psychotherapie, Universitätsklinikum Köln
2   Klinik und Poliklinik für Neurologie, Universitätsklinikum Köln
,
F. Jessen
1   Klinik und Poliklinik für Psychiatrie und Psychotherapie, Universitätsklinikum Köln
3   DZNE-Standort Bonn, Deutsches Zentrum für Neurodegenerative Erkrankungen (DZNE), Bonn
,
J. Kuhn
1   Klinik und Poliklinik für Psychiatrie und Psychotherapie, Universitätsklinikum Köln
4   Klinik für Psychiatrie, Psychotherapie und Psychosomatik, Johanniter Krankenhaus Oberhausen
› Author Affiliations
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Publication History

Publication Date:
14 June 2016 (online)

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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-GABAA und GABABR-, 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-GABAA and GABABR, 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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