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DOI: 10.1055/s-0043-101227
Neue Entität der Paranodopathien: eine Zielstruktur mit therapeutischen Konsequenzen
The New Entity of Paranodopathies: a Target Structure with Therapeutic ConsequencesPublikationsverlauf
Publikationsdatum:
25. April 2017 (online)
![](https://www.thieme-connect.de/media/aktneu/201703/lookinside/thumbnails/115_10-1055-s-0043-101227-1.jpg)
Zusammenfassung
Immunneuropathien mit Autoantikörpern gegen paranodale Proteine wurden erst in den letzten Jahren beschrieben. Es handelt sich dabei um eine Subgruppe der entzündlichen Neuropathien mit IgG4-Autoantikörpern gegen die Proteine Neurofascin-155, Contactin-1 und Caspr-1. Diese Gruppe macht in den meisten Studien zwar < 10 % der Patienten mit Diagnose einer chronisch-inflammatorischen demyelinisierenden Polyradikuloneuropathie (CIDP) aus, jedoch ist der Autoantikörpernachweis von diagnostischer Relevanz, da sich die Patienten im Ansprechen auf Therapien von anderen Patienten mit CIDP unterscheiden: Obwohl aufgrund der bislang begrenzten Fallzahl keine Therapiestudien vorliegen, ist in allen Fallberichten ein sehr gutes Ansprechen auf Behandlungen mit Rituximab beschrieben, meist kein Ansprechen auf die Standardtherapie der CIDP mit Immunglobulinen. Klinisch fallen die Patienten durch einen relativ akuten Erkrankungsbeginn, eine schwere motorisch-betonte Polyneuropathie, teilweise einen Aktionstremor und eine sensible Ataxie auf. Elektrophysiologisch finden sich Kennzeichen einer demyelinisierenden Neuropathie bei jedoch eher axonalem histologischen Phänotyp in der Nervenbiopsie. Erklärt werden kann letzteres durch das pathophysiologische Konzept einer „Paranodopathie/Nodopathie“, einer Schnürringerkrankung, bei der weder die Myelinscheide noch das Axon primärer Angriffspunkt der Immunantwort sind, sondern die Ranvierschen Schnürringe.
Abstract
Autoimmune neuropathies with autoantibodies against paranodal proteins have been described in the last few years. They comprise a subgroup of inflammatory neuropathies with IgG4 autoantibodies against the paranodal proteins neurofascin-155, contactin-1 and caspr-1. Although this subtype of autoimmune neuropathy represents just less than 10 % of all patients diagnosed with CIDP, it is of high interest as they show a different response to treatment. Even though there are no therapeutic studies available due to the limited number of patients identified so far, all case reports that have been published so far report an excellent response to treatment with rituximab, and in most cases no response to treatment with IVIG, the standard therapy of CIDP. The typical clinical picture of patients with autoantibodies against paranodal proteins is characterized by an acute onset of severe predominantly motor neuropathy, often accompanied by action tremor and sensory ataxia, with demyelinating features in the nerve conduction studies but an axonal histological phenotype. The latter may be explained by the pathogenetic concept of a paranodopathy/nodopathy, a disease of the paranode/node of Ranvier.
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