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Neuropathische Schmerzsyndrome bei Ionenkanalerkrankungen

Neuropathic pain syndromes and channelopathies

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Zusammenfassung

Hintergrund

Die Ursachen neuropathischer Schmerzen sind vielfältig und bleiben in vielen Fällen unklar. In den vergangenen Jahren konnten mehrere Schmerzsyndrome auf Veränderungen in Natriumkanalgenen zurückgeführt werden, sodass diese Gruppe seltener Erkrankungen in der Differenzialdiagnostik neuropathischer Schmerzen zunehmend zu berücksichtigen ist.

Material und Methoden

Auswertung von themenbezogener Literatur, Diskussion eigener Erfahrungen sowie Berücksichtigung bestehender Leitlinien.

Ergebnisse

Veränderungen in der elektrischen Erregbarkeit der schmerzleitenden Nervenfasern durch pathogene Varianten der Natriumkanäle führen zu Krankheitsbildern wie der Small-fiber-Neuropathie und verschiedenen Schmerzsyndromen. Die Gruppe dieser genetischen Erkrankungen wird besprochen und in die Differenzialdiagnostik des neuropathischen Schmerzes eingeordnet. Therapiekonzepte werden dargestellt und die bislang vorwiegend experimentellen Ansätze zur gezielten Modulation der Natriumkanäle diskutiert.

Schlussfolgerungen

Die Behandlung von Patienten mit chronischen neuropathischen Schmerzen erfordert interdisziplinäre Zusammenarbeit und gestaltet sich aufgrund eines unbefriedigenden Therapieansprechens oft schwierig. Zunehmende Erkenntnisse zu seltenen, genetisch bedingten Ionenkanalerkrankungen können zukünftig dazu beitragen, dass die pharmakologische Beeinflussung dieser zentralen Vermittler des Schmerzempfindens neue Impulse in der Schmerztherapie setzt.

Abstract

Background

The causes for neuropathic pain are manifold and remain unexplained in the majority of cases. In recent years a growing number of pain syndromes have been attributed to mutations in genes encoding voltage-gated sodium channels. Hence, this group of rare diseases should be considered in the differential diagnostics of neuropathic pain.

Material and methods

Evaluation of topic-related literature and discussion of own experiences as well as consideration of current guidelines.

Results

Alterations in the electrical excitability of nociceptive neurons by pathogenic mutations in sodium channels lead to disease patterns, such as small fiber neuropathy and various pain syndromes. This article summarizes the knowledge on these genetic diseases and discusses the differential diagnosis of neuropathic pain. Current treatment concepts are presented and the predominantly experimental approaches to targeted modulation of sodium channels are discussed.

Conclusion

The treatment of patients with chronic neuropathic pain requires interdisciplinary cooperation and is often difficult due to an unsatisfactory treatment response. Increasing knowledge on rare genetically determined channelopathies can contribute to the development of novel pharmaceuticals since ion channels are central players in the processing of pain.

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Author information

Authors and Affiliations

  1. Klinik für Neurologie, Uniklinik RWTH Aachen, Aachen, Deutschland

    Maike F. Dohrn

  2. Institut für Physiologie, Uniklinik RWTH Aachen, Aachen, Deutschland

    Angelika Lampert

  3. Neurologische Klinik, Universitätsklinikum Würzburg, Würzburg, Deutschland

    Nurcan Üçeyler

  4. Institut für Humangenetik, Uniklinik RWTH Aachen, Pauwelsstr. 30, 52074, Aachen, Deutschland

    Ingo Kurth

Authors
  1. Maike F. Dohrn
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  2. Angelika Lampert
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  3. Nurcan Üçeyler
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  4. Ingo Kurth
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Corresponding author

Correspondence to Ingo Kurth.

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Interessenkonflikt

M.F. Dohrn, A. Lampert, N. Üçeyler und I. Kurth geben an, dass kein Interessenkonflikt besteht.

Dieser Beitrag beinhaltet keine von den Autoren durchgeführten Studien an Menschen oder Tieren. Für Bildmaterial oder anderweitige Angaben innerhalb des Manuskripts, über die Patienten zu identifizieren sind, liegt von ihnen und/oder ihren gesetzlichen Vertretern eine schriftliche Einwilligung vor.

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J.R. Schäfer, Marburg

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Dohrn, M.F., Lampert, A., Üçeyler, N. et al. Neuropathische Schmerzsyndrome bei Ionenkanalerkrankungen. Internist 60, 90–97 (2019). https://doi.org/10.1007/s00108-018-0535-x

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  • DOI: https://doi.org/10.1007/s00108-018-0535-x

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