Journal
MUSCLE & NERVE
Volume 52, Issue 4, Pages 488-497Publisher
WILEY
DOI: 10.1002/mus.24707
Keywords
CIDP; immune therapy; MMN; neurophysiologic tests; signs
Categories
Funding
- Mayo Foundation
- Pfizer
- Isis
- Alnylam
- National Institutes of Health [NS 36797]
- 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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