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Chronic inflammatory demyelinating polyneuropathy
Authors:Said Gérard
Affiliation:1. Department of Clinical Medicine, Aalborg University, Denmark;2. K.G. Jebsen Thrombosis Research and Expertice Centre (TREC), Department of Clinical Medicine, UiT The Arctic University of Norway, Tromsø, Norway;3. Division of Internal Medicine, University Hospital of North Norway, Tromsø, Norway;4. Department of Hematology, Trondheim University Hospital, Trondheim, Norway;5. Department of Clinical Epidemiology, Leiden University Medical Center, Leiden, The Netherlands;6. Department of Public Health, Section for Epidemiology, Aarhus University, Aarhus, Denmark;7. Department of Cardiology, Aalborg University Hospital, Aalborg, Denmark;8. Department of Cancer Research and Molecular Medicine, Norwegian University of Science and Technology, Trondheim, Norway;9. Diet, Genes and Environment, Danish Cancer Society Research Center, Copenhagen, Denmark;10. Department of Clinical Biochemistry, Aalborg University Hospital, Aalborg, Denmark;11. Department of Hematology, Aalborg University Hospital, Aalborg, Denmark
Abstract:Chronic inflammatory demyelinative polyneuropathy (CIDP) is an acquired neuropathy, presumably of immunological origin. Its clinical presentation and course are extremely variable. CIDP is one of the few peripheral neuropathies amenable to treatment. Typical cases associate progressive or relapsing-remitting motor and sensory deficit with increased CSF protein content and electrophysiological features of demyelination. In other instances the neuropathy is predominantly or exclusively motor or sensory, CSF normal and electrophysiological studies fail to show evidence of demyelination. In such cases conventional diagnostic criteria are not filled yet the patient may respond to immunomodulatory treatments. In this paper we review the diagnostic pitfalls and clinical variants of CIDP to illustrate the problems that may arise. The different therapeutic options are reviewed. Axon loss associated with demyelination is the most important factor of disability and resistance to treatment.
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