Abstract
Chronic inflammatory demyelinating polyneuropathy (CIDP) is the commonest treatable
neuropathy in the western world. Untreated it may result in severe disability but
if diagnosed and treated early there is effective treatment for the majority of patients.
Typical CIDP is readily recognised but the diagnosis of other subgroups can be more
challenging. The pathology of polyradiculoneuropathies such as CIDP characteristically
affects the most proximal regions of the peripheral nervous system, nerve roots and
major plexuses. It is important to test these regions with electrodiagnostic studies
since routine neurophysiology may not encounter regions of pathology. Although accepted
as an autoimmune disorder with an underlying immunopathology involving T cell and
B cell responses, there is no agreement on major target antigens; however recent studies
have highlighted a role for molecules in non compact myelin which play an essential
role in the formation and maintenance of the nodal structures and hence in the function
of ion channels central to saltatory conduction. Controlled trials have proven the
efficacy of corticosteroid, intravenous immunoglobulin and plasma exchange in the
short term and intravenous immunoglobulin also in the long term. Immunosuppressive
agents are widely used but their efficacy has not been proven in controlled trials.
Recent trials have shown the importance of attempting treatment withdrawal in patients
apparently in remission to conserve treatments that are very expensive and in short
supply, since a significant proportion of patients may enter long lasting remission
following short term therapy. For the relatively small group of patients who do not
respond to these first line therapies new agents including monoclonal antibodies may
have a role.
Keywords
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Article info
Publication history
Published online: November 12, 2012
Accepted:
October 22,
2012
Received in revised form:
October 14,
2012
Received:
September 20,
2012
Identification
Copyright
© 2012 Published by Elsevier Inc. All rights reserved.
