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Predicting response to treatment in chronic inflammatory demyelinating polyradiculoneuropathy

Abstract

Objective: To discover whether Inflammatory Neuropathy Cause and Treatment Group (INCAT) electrophysiological criteria for demyelinating neuropathy predict response to immunotherapy in chronic inflammatory demyelinating polyradiculoneuropathy (CIDP).

Methods: This was a retrospective case note study of patients who had attended Guy’s Hospital Peripheral Nerve Clinic between January 2001 and March 2004, been diagnosed as having CIDP, and given treatment with corticosteroids, intravenous immunoglobulin (IVIg), or plasma exchange (PE). Patients’ nerve conduction studies (NCS) were reviewed for evidence of demyelination and whether the abnormalities fulfilled modified INCAT electrophysiological criteria. Patients whose NCS fulfilled the criteria were assigned to the neurophysiologically definite CIDP group, while those that did not were labelled as neurophysiologically probable CIDP. Responses to any of the three immunotherapy agents were compared between the two groups.

Results: Out of 50 patients, 27 (54%) were classified as neurophysiologically definite and 23 (46%) as neurophysiologically probable CIDP patients. Twenty (74%) neurophysiologically definite and 17 (73.9%) neurophysiologically probable CIDP patients responded to treatment.

Conclusions: INCAT electrophysiological criteria did not predict a higher rate of response to immunotherapy. Neurophysiologically probable CIDP patients should be given a trial of immunotherapy.

  • ASSM, asymmetrical sensorimotor
  • CIDP, chronic inflammatory demyelinating polyradiculoneuropathy
  • CSF, cerebrospinal fluid
  • CV, conduction velocity
  • DA, demyelination abnormality
  • DML, distal motor latency
  • DSSM, distal symmetrical sensorimotor
  • INCAT, Inflammatory Neuropathy Cause and Treatment Group
  • IVIg, intravenous immunoglobulin
  • MRC, Medical Research Council
  • NCS, nerve conduction studies
  • ODSS, Overall Disability Sum Score
  • PE, plasma exchange
  • ULN, upper limit of normal values
  • chronic inflammatory demyelinating polyradiculoneuropathy
  • electrophysiological criteria
  • immunotherapy

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