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Aktuelle Neurologie 2016; 43(07): 441-451
DOI: 10.1055/s-0042-112808
DOI: 10.1055/s-0042-112808
Übersicht
Aktuelle Therapie von Immunneuropathien
Current Treatment Strategies for Immune-Mediated NeuropathiesFurther Information
Publication History
Publication Date:
08 September 2016 (online)
Zusammenfassung
Seit der Erstbeschreibung einer akuten monophasisch verlaufenden peripheren Lähmung vor genau 100 Jahren, dem Guillain-Barré Syndrom (GBS), dauerte es nochmals 42 Jahre, bis eine chronische, Kortikosteroid-responsive Polyneuropathie erstmalig beschrieben wurde. Seither hat sich das Spektrum der Immunneuropathien stetig erweitert und noch immer werden einzelne Subformen neu definiert. Die Behandlung immunvermittelter Polyneuropathien, insbesondere chronisch verlaufender Varianten, wie die chronisch inflammatorische (entzündliche) demyelinisierende Polyneuropathie (CIDP) sowie die multifokal motorische Neuropathie (MMN), stellt im klinischen Alltag eine Herausforderung dar. Aufgrund ihrer klinischen Heterogenität und geringen Prävalenzen gibt es nur wenige Therapiestudien höchster Evidenzklasse, die eine eindeutige Empfehlung ermöglichen. Aktuelle Fortschritte in der Klassifikation mittels neuer diagnostischer Kriterien sowie die rapide Zunahme moderner Immuntherapeutika in der Neurologie lassen hoffen, dass eine verbesserte Klassifikation und ein breiteres therapeutisches Repertoire für den klinischen Alltag in Zukunft zur Verfügung stehen werden. In dem folgenden Übersichtsartikel werden die aktuellen Empfehlungen sowie neusten Entwicklungen auf dem Therapiesektor zur Behandlung akuter als auch chronischer Immunneuropathien dargestellt.
Abstract
Since the first description of an acute monophasic paralysis 100 years ago, later designated as the Guillain-Barré syndrome (GBS), another 42 years passed before a chronic, steroid-responsive polyneuropathy was reported. Since then, the spectrum of immune-mediated polyneuropathies has been broadened and different subforms have been identified. In daily clinical practice, treating immune-mediated neuropathies, especially its various chronic variants, such as the chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) or the multifocal motor neuropathy (MMN), represents a challenge. The large clinical heterogeneity and the low prevalence of these different neuropathies are reasons for the very few clinical trials of high scientific standard offering therapeutic recommendations based on highest clinical evidence. As such, it is hoped that with better clinical criteria and the emergence of modern immunotherapies, the therapeutic armamentarium against these disabling disease might be broadened in the future. This review article summarizes current treatment recommendations for acute as well as chronic immune-mediated neuropathies.
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