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Refractory chronic inflammatory demyelinating polyradiculoneuropathy


Authors: E. Ehler 1;  I. Štětkářová 2
Authors place of work: Neurologická klinika, Fakulta zdravotnických studií, Univerzita Pardubice, Nemocnice Pardubice 1;  Neurologická klinika 3. LF UK a Fakultní nemocnice Královské Vinohrady, Praha 2
Published in the journal: Cesk Slov Neurol N 2026; 89(4): 242-246
Category: Přehledný referát
doi: https://doi.org/10.48095/cccsnn2026242

Summary

Refractory chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is characterized with ineffectiveness of CIDP first-line therapy –⁠ corticosteroids, plasmapheresis and immunoglobulins. Clinical and electrophysiological diagnosis of refractory CIDP is important for therapy and prognosis. First, it is necessary to revise the CIDP diagnosis and reliably exclude other neuropathies, that mimic CIDP and can be confused with it. Based on auxiliary examinations (electrophysiology as well as imaging techniques), it is necessary to determine if this is the endstage of secondary axonal lesion no longer influencable by therapy or whether functional reserve still exists and CIDP may be improved. In refractory CIDP, it is recommended and, among immunosuppressants, to administer cyclophosphamide, cyclosporin A, and chimeric antibody against CD20 –⁠ rituximab. Among recently developed drugs, there are inhibitors of individual components of complement and neonatal Fc receptor blockers.

Keywords:

plasmapheresis – corticosteroids – immunoglobulins – Neurophysiology – immunosuppressive drugs – CIDP


Zdroje

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Štítky
Detská neurológia Neurochirurgia Neurológia

Článok vyšiel v časopise

Česká a slovenská neurologie a neurochirurgie

Číslo 4

2026 Číslo 4
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