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: 21.9). 04/2010; 9(4):402-12. DOI: 10.1016/S1474-4422(10)70041-7
Source: PubMed


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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    • "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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    • "Treatment choice will depend on several variables such as initial disease severity, age, general health status, and potential contraindications [61]. The recent economic crisis is opening remarkable questions about the sustainability of expensive drugs such as IVIg in Western countries and some national audits [62] or studies [63–65] have been already performed or are still ongoing (e.g., the prospective observational study “TEPORE” in Northern Italy) to clarify this issue. "
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    ABSTRACT: Chronic inflammatory demyelinating polyneuropathy (CIDP) is an autoimmune disease of the peripheral nervous system, in which both cellular and humoral immune responses are involved. The disease is clinically heterogeneous with some patients displaying pure motor form and others also showing a variable degree of sensory dysfunction; disease evolution may also differ from patient to patient, since monophasic, progressive, and relapsing forms are reported. Underlying such clinical variability there is probably a broad spectrum of molecular dysfunctions that are and will be the target of therapeutic strategies. In this review we first explore the biological bases of current treatments and subsequently we focus on the practical management that must also take into account pharmacoeconomic issues.
    Full-text · Article · Jan 2014
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    • "On the other hand, the typical chronic inflammatory demyelinating polyneuropathy (CIDP) arises between the ages of 30 and 60 years and is characterised by a progressive, symmetric, proximal and distal muscle weakness, paresthesis, sensory dysfunction and impaired balance, which may evolve slowly over at least 2 months (Koller et al., 2005; Vallat et al., 2010). Although CIDP symptoms do not usually reach their most severe until at least 2 months from disease onset (Dalakas, 2011), about 16% of the patients may have a subacute onset with a monophasic course (Vallat et al., 2010; Said, 2006; Hughes et al., 2006, Odaka et al., 2003). "

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