Chapter

Chronic Immune-Mediated Neuropathies

Steven Herskovitz

in Peripheral Neuropathies in Clinical Practice

Published on behalf of Oxford University Press

Published in print November 2008 | ISBN: 9780195183269
Published online April 2013 | e-ISBN: 9780199322947 | DOI: http://dx.doi.org/10.1093/med/9780195183269.003.0007

Series: CONTEMP NEURO

Chronic Immune-Mediated Neuropathies

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>Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an acquired multifocal neuropathy that commonly has symmetric, proximal, and distal limb weakness, distal sensory loss and progresses over more than 2 months.1 The clinical and immunopathologic features suggest an autoimmune disease, analogous to acute inflammatory demyelinating polyradiculoneuropathy (AIDP), and CIDP characteristically responds to immunosuppressive therapies. The diagnosis is supported by nerve conduction or sensory nerve biopsy findings suggestive of multifocal demyelination. Since the 1980s, several phenotypic variations of chronic, presumably inflammatory, demyelinating polyneuropathies have been characterized. These variants include multifocal motor neuropathy with conduction block, Lewis-Sumner syndrome (multifocal acquired demyelinating sensory and motor neuropathy, MADSAM) and distal acquired demyelinating symmetric neuropathy (DADS).2,3 Although these syndromes may be part of a spectrum of a common disease (CIDP), the distinction has clinical utility because different variants respond to different immunosuppressive regimens (Table 7–1).

Chapter.  9612 words.  Illustrated.

Subjects: Neurology

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