Chronische inflammatorische demyelinisierende Polyneuropathie

 

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Zusammenfassung

Die chronische inflammatorische demyelinisierende Polyneuropathie (CIDP) ist eine entzündliche, progredient oder schubförmig verlaufende Erkrankung des peripheren Nervensystems mit individuell unterschiedlich verteiltem, typischerweise symmetrischem Muster distaler und proximaler Paresen und fakultativ sensiblen Defiziten. Daneben gibt es atypische Varianten, die mit raschem Beginn, ausschließlich sensiblen Defiziten, fokalem oder sehr asymmetrischem Ausfallmuster einhergehen können. Die Neuropathie ist autoimmun vermittelt, an der Pathogenese sind Autoantikörper, Makrophagen, B- und T-Zellen beteiligt. Die Diagnose wird anhand klinischer Symptome, charakteristischer Veränderungen in der Elektrophysiologie und anhand des Liquorbefundes gestellt. In Zweifelsfällen kann der Nachweis entzündlicher Infiltrate in einer Biopsie des N. suralis die Diagnose stützen. Zukünftig wird vermutlich auch der MRT und der Nervensonografie Bedeutung bei der Diagnosestellung zukommen. Therapeutisch sind Steroide, Immunglobuline und Plasmapherese gleichermaßen wirksam. Darüber hinaus werden Immunsuppressiva und zunehmend auch monoklonale Antikörper eingesetzt.

Abstract

Chronic inflammatory demyelinating polyneuropathy (CIDP) is a chronic progressive or relapsing autoimmune neuropathy with heterogeneous clinical presentation. Symptoms typically include symmetrical, proximal and/or distal paresis and sensory loss. Atypical CIDP variants are increasingly recognized, including subtypes with rapid onset as well as variants with pure sensory, focal or marked asymmetrical deficits. Diagnosis is established by compatible symptoms, characteristic electrophysiological features and cerebrospinal fluid analysis. In unequivocal cases, inflammatory infiltrates in sural nerve biopsy support the diagnosis. Recent studies suggest that diagnostic imaging techniques such as MRI and nerve ultrasound may become useful tools for establishing the diagnosis. First-line therapies include immunoglobulines, steroids, and plasmapheresis. Immunosuppressant agents and monoclonal antibodies are used in therapy-refractory cases or as cortison-saving agents.

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