4.4 Article

OFFICE IMMUNOTHERAPY IN CHRONIC INFLAMMATORY DEMYELINATING POLYNEUROPATHY AND MULTIFOCAL MOTOR NEUROPATHY

Journal

MUSCLE & NERVE
Volume 52, Issue 4, Pages 488-497

Publisher

WILEY
DOI: 10.1002/mus.24707

Keywords

CIDP; immune therapy; MMN; neurophysiologic tests; signs

Funding

  1. Mayo Foundation
  2. Pfizer
  3. Isis
  4. Alnylam
  5. National Institutes of Health [NS 36797]
  6. U.S. Food and Drug Administration

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Intravenous immunoglobulin [IVIg], plasma exchange [PE], and corticosteroids are efficacious treatment in chronic inflammatory demyelinating polyneuropathy [CIDP]. IVIg is effective in multifocal motor neuropathy [MMN]. NIS, NIS-weakness, sum scores of raw amplitudes of motor fiber (CMAPs) amplitudes, and Dyck/Rankin score provided reliable measures to detect and scale abnormality and reflect change; they are therefore ideal for office management of responsebased immunotherapy (R-IRx) of CIDP. Using efficacious R-IRx, a large early and late therapeutic response (>= one-fourth were in remission or had recovered) was demonstrated in CIDP. In MMN only an early improvement with late non-significant worsening was observed. The difference in immunotherapy response supports a fundamental difference between CIDP (immune attack on Schwann cells and myelin) and MMN (attack on nodes of Ranvier and axons).

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