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Chronic inflammatory demyelinating polyradiculoneuropathy: classification and treatment options
  1. James R Overell,
  2. Hugh J Willison
  1. Department of Neurology, Institute of Neurological Sciences, Southern General Hospital, Glasgow, UK
  1. Correspondence to:
 Dr J Overell, Department of Neurology, Institute of Neurological Sciences, Southern General Hospital, 1345 Govan Road, Glasgow G51 4TF, UK
 j.overell{at}clinmed.gla.ac.uk

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Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an uncommon and probably underdiagnosed disorder that often results in appreciable disability. Its importance lies in its generally good response to treatment. Although it is often contextualised by neurologists as “a chronic form of Guillain-Barré syndrome” (GBS), this oversimplifies the relationship between the two disorders: there is more to the distinction than one taking longer than the other to present and develop. Because CIDP is treatable, and because treatment is complex, largely non-evidence based, and potentially toxic, it is an important disorder to understand. The clinical spectrum is broad, but clinical subclassification is worthwhile, because it informs treatment decisions—certain subtypes respond better to certain treatment options, and some subtypes don’t respond well, if at all. We will discuss the clinical presentation and suggest a simple and practical classification system as a step towards rationalising the many different immunomodulatory strategies available.

CLINICAL PRESENTATION

When should the clinician consider CIDP? There are three main scenarios:

  • The typical clinical picture is of a symmetrical, progressive, mixed sensory and motor neuropathy (but largely motor and often proximal), which results in significant disability (typically problems with walking) and areflexia.

  • Sometimes CIDP is diagnosed when “GBS” develops over a longer period than expected (clinical deterioration should proceed for less than four weeks in GBS and more than eight weeks in CIDP, the middle ground being classified as “SIDP”—subacute IDP). The neurophysiological criteria for CIDP have been defined and modified by numerous authors and are discussed in detail elsewhere,1,2 but essentially require a combination of motor conduction block, reduced motor and sensory conduction velocities with temporal dispersion, prolonged distal motor latencies, and prolonged F wave latencies.

  • A third “route in” to the diagnosis is finding demyelinating neurophysiology in a patient with a paralytic syndrome that hadn’t been clearly classified through …

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