Chronic Inflammatory Demyelinating Polyradiculoneuropathy
Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an acquired demyelinating neuropathy in which motor symptoms generally predominate over sensory symptoms and large fiber sensory dysfunction is more common than small fiber loss. The course may either be relapsing and remitting or chronically progressive. CIDP may occur in isolation or in the context of a number of systemic disorders, including HIV and hepatitis C infection, inflammatory bowel disease, lymphoproliferative disorders and osteosclerotic myeloma. The importance of distinguishing CIDP from among other causes of peripheral neuropathy lies in the observation that a significant proportion of patients with CIDP may respond to immune-modifying therapy. In this chapter, we consider important diagnostic questions that relate to the optimal electrophysiological criteria that should be used to define the disorder and the relative diagnostic utility of sural nerve biopsy. Many different forms of immunosuppressive therapy have been proposed for the treatment of CIDP, and although some of these therapeutic modalities have been studied in randomized controlled trials, many have not. We shall also consider the evidence supporting the use of these various immunosuppressive agents as well as the prognosis for patients who are treated in this manner.
KeywordsCompound Muscle Action Potential Motor Neuron Disease Chronic Inflammatory Demyelinating Polyneuropathy Chronic Inflammatory Demyelinating Polyradiculoneuropathy Sensory Nerve Action Potential
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