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Published on: May 22, 2020
Refractory Form of Chronic Idiopathic Demyelinating Polyneuropathy with Rituximab Effect: A Case Report
Edvard Ehler1, Tomáš Jurka2, Ivana Štětkářová3
1Department of Neurology, Faculty of Health Studies, Pardubice University and Pardubice Regional Hospital, Pardubice, Czech Republic. edvard.ehler@nempk.cz.
Summary
Chronic idiopathic demyelinating polyneuropathy (CIDP) involves autoimmune nerve demyelination. This case highlights refractory CIDP, a severe form unresponsive to standard treatments like corticosteroids, immunoglobulins, and plasma exchange.
Area of Science:
- Neurology
- Immunology
- Pathophysiology of Neurological Disorders
Background:
- Chronic idiopathic demyelinating polyneuropathy (CIDP) is an autoimmune disorder characterized by peripheral nerve demyelination.
- Pathogenesis involves antibodies against myelin proteins and cell-mediated immunity with T-cell and macrophage infiltration.
- Standard treatments aim to suppress inflammation and remove pathogenic molecules, including corticosteroids, immunoglobulins, and plasma exchange.
Purpose of the Study:
- To present a case of refractory CIDP.
- To discuss the management challenges and current knowledge regarding refractory CIDP.
- To highlight the significance of severe neurological findings in refractory CIDP.
Main Methods:
- Case report presentation.
- Review of current literature on CIDP and refractory CIDP.
- Discussion of treatment modalities and diagnostic criteria.
Main Results:
- The presented patient exhibited severe neurological findings.
- The patient showed a lack of clinical response to standard CIDP treatments (corticosteroids, immunoglobulins, plasmapheresis).
- The condition was diagnosed as refractory CIDP, affecting up to 10% of CIDP patients.
Conclusions:
- Refractory CIDP presents a significant clinical challenge.
- Understanding refractory CIDP is crucial for managing patients unresponsive to established therapies.
- Further research into alternative treatment strategies for refractory CIDP is warranted.