Chronic inflammatory demyelinating polyradiculoneuropathy: diagnostic and therapeutic challenges for a treatable condition

Service de Neurologie, Centre de Référence Neuropathies périphériques rares, CHU Limoges, France.
The Lancet Neurology (Impact Factor: 21.82). 04/2010; 9(4):402-12. DOI: 10.1016/S1474-4422(10)70041-7
Source: PubMed

ABSTRACT Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a chronic neuropathy of supposed immune origin. Understanding of its pathophysiology has recently improved, although its causes remain unclear. The classic presentation of CIDP includes sensory and motor symptoms in the distal and proximal segments of the four limbs with areflexia, evolving over more than 8 weeks. Raised protein concentrations in CSF and heterogeneous slowing of nerve conduction are typical of the condition. In addition to this usual phenotype, distribution of symptoms, disease course, and disability can be heterogeneous, leading to underdiagnosis of the disorder. Diagnosis is sometimes challenging and can require use of imaging and nerve biopsy. Steroids and intravenous immunoglobulin are effective, and plasma exchange can be helpful as rescue therapy. The usefulness of immunosuppressants needs to be established. The identification of specific diagnostic markers and new therapeutic strategies with conventional or targeted immunotherapy are needed to improve the outlook for patients with CIDP.

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Available from: Claudia Sommer, Aug 19, 2015
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    • "Herein we describe a new model of chronic EAN that can be easily and reliably induced by active immunization of 7–8 week male Lewis rats with S-palmitoylated P0(180–199) peptide in the presence of CFA, with selective involvement of the PNS. In contrast to the classical acute monophasic EAN induced by P0(180–199), 100% of rats immunized with S-palm P0(180–199) develop an ongoing neuropathy, either chronic or relapsing; in this, the model resembles the human disease CIDP (Vallat et al., 2010). Furthermore immunohistochemical and electrophysiological features indicate that this new model is an inflammatory , predominantly demyelinating neuropathy, with axonal degeneration and cumulative axonal diameter reduction over time. "
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    ABSTRACT: Animal model Thiopalmitoylated P0 peptide Chronic EAN CIDP Electrophysiology Sciatic nerve immunohistochemistry Immunology Our objective was to develop a chronic model of EAN which could be used as a tool to test treatment strategies for CIDP. Lewis rats injected with S-palmitoylated P0(180–199) peptide developed a chronic, sometimes relapsing– remitting type of disease. Our model fulfills electrophysiological criteria of demyelination with axonal degener-ation, confirmed by immunohistopathology. The late phase of the chronic disease was characterized by accumu-lation of IL-17 + cells and macrophages in sciatic nerves and by high serum IL-17 levels. In conclusion, we have developed a reliable and reproducible animal model resembling CIDP that can now be used for translational drug studies.
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