Office immunotherapy in chronic inflammatory demyelinating polyneuropathy and multifocal motor neuropathy

Muscle Nerve. 2015 Oct;52(4):488-97. doi: 10.1002/mus.24707. Epub 2015 Aug 13.

Abstract

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 response-based 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).

Keywords: CIDP; MMN; immune therapy; neurophysiologic tests; signs.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Action Potentials
  • Adrenal Cortex Hormones / therapeutic use
  • Electromyography
  • Female
  • Humans
  • Immunoglobulins, Intravenous
  • Immunotherapy / methods*
  • Male
  • Neural Conduction / physiology
  • Neuromuscular Diseases / immunology
  • Neuromuscular Diseases / physiopathology
  • Neuromuscular Diseases / therapy*
  • Plasma Exchange / methods
  • Polyradiculoneuropathy, Chronic Inflammatory Demyelinating / immunology*
  • Polyradiculoneuropathy, Chronic Inflammatory Demyelinating / physiopathology
  • Polyradiculoneuropathy, Chronic Inflammatory Demyelinating / therapy*
  • Retrospective Studies
  • Severity of Illness Index
  • Treatment Outcome*

Substances

  • Adrenal Cortex Hormones
  • Immunoglobulins, Intravenous