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Neurologie up2date 2023; 06(04): 369-382
DOI: 10.1055/a-1902-2911
Myopathien und Neuropathien

Chronische inflammatorische demyelinisierende Polyradikuloneuropathie (CIDP)

Barbara Kaulen
,
Laura Hagemann
,
Helmar C. Lehmann
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Die chronische inflammatorische demyelinisierende Polyradikuloneuropathie (CIDP) ist die häufigste chronische Autoimmunerkrankung des peripheren Nervensystems. Sie ist häufig schwierig zu diagnostizieren. Die CIDP ist mittlerweile mittels verschiedener Immuntherapeutika gut behandelbar. In diesem Beitrag wird die Diagnose und Therapie der CIDP anhand eines typischen Fallbeispiels erläutert.
Kernaussagen

  • Die chronische inflammatorische demyelinisierende Polyneuropathie (CIDP) ist die häufigste chronische Immunneuropathie.

  • Wichtige Differenzialdiagnosen der CIDP umfassen andere entzündliche Polyneuropathien wie das Guillain-Barré-Syndrom, Anti-MAG-IgM-Neuropathie, das CANOMAD-Syndrom, oder andere metabolische Neuropathien wie zum Beispiel die diabetische Polyneuropathie oder die ATTRv

  • Die Elektroneurografie ist die wichtigste Zusatzdiagnostik für die Diagnose einer CIDP, andere wertvolle Untersuchungsmethoden sind

    • Nervenultraschall,

    • Liquoruntersuchung,

    • Nervenbiopsie.

  • Autoantikörper spielen in der Diagnostik der CIDP nach wie vor eine untergeordnete Rolle. Bei etwa jedem 10. Patienten mit der Verdachtsdiagnose einer CIDP findet man Antikörper gegen Proteine, die im Bereich des Ranvier’schen Schnürrings exprimiert werden.

  • Therapie der 1. Wahl sind Steroide, Immunglobuline und Plasmapherese. Darüber hinaus können auch andere Immunsuppressiva und monoklonale Antikörper (Off-Label Use) eingesetzt werden.

Schlüsselwörter

Autoimmunerkrankung - peripheres Nervensystem - Immuntherapie - polyneuropathische Symptome


Publication History

Article published online:
08 December 2023

© 2023. Thieme. All rights reserved.

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