Abstract Details

Title Determinants of Achieving Minimal Manifestations and Drug-free remission in Multifocal Motor Neuropathy

Topic Neuromuscular and Clinical Neurophysiology (EMG)

Presentation(s) P4 - Poster Session 4 (5:00 PM-6:00 PM)

Poster/Presentation
Number 11-022

Objective Although most patients with multifocal motor neuropathy (MMN) respond to intravenous immunoglobulin, many experience residual disability and are dependent on chronic immunotherapy. The frequency of patients achieving minimal manifestations (MM) and predictors of MM status are unknown. This study aims to determine (1) frequency of patients achieving MM, (2) frequency of drug-free remission, and (3) predictors of MM or drug-free remission.

Background NA

Design/Methods We retrospectively analyzed 20 patients with MMN diagnosed per EFNS/PNS guidelines. Data on demographics, disease course, diagnostics, and treatment were collected. Outcomes (grip strength, MMN-RODS score, MRC 80 sum score, patient-reported disability) were recorded at peak disease and last follow-up. MM was defined as an MRC sum score ≥78/80 with no individual muscle <4, MMN-RODS score ≥46/50, and no patient-perceived activity limitations. Data was analyzed after stratifying patients into those with and without MM status.

Results

Mean age at onset was 41.9 years, and 70% were male. Three patients (15%) achieved drug-free remission for at least 6 months. Five (25%) met our definition of MM. Patients with MM tended to have earlier onset (35.0 vs. 44.2 years), higher MMN-RODS scores (less disability) at peak severity (46 vs.35.4), and higher MRC scores in weakest muscle group at peak (3.75 vs. 1.31). Average disease duration (onset to last follow-up) in MM group was 198 months vs 214 months without MM. GM1 antibodies were positive in 40.0% of MM patients, vs 46.7% in those without MM. Drug-free remission was associated with absence of lower-extremity involvement at peak severity (p=0.013).

Conclusions

Minimal manifestations are rare in MMN, but may be more common in patients with less disease-associated manifestations at peak severity, which can have important treatment implications. This ongoing study aims to include an additional 10 patients for the MM predictive analysis.

Authors/Disclosures
Abhigyan Datta, MD (University of Minnesota) PRESENTER	Dr. Datta has nothing to disclose.
Stephen J. Ward, DO	Dr. Ward has nothing to disclose.
Georgios Manousakis, MD, FAAN (University of Minnesota)	Dr. Manousakis has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Stealth Biotherapeutics. Dr. Manousakis has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Argenx. Dr. Manousakis has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Alexion. The institution of Dr. Manousakis has received research support from Marzolf foundation.
Jeffrey A. Allen, MD (University of Minnesota)	Dr. Allen has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Argenx. Dr. Allen has received personal compensation in the range of $500-$4,999 for serving as a Consultant for csl behring. Dr. Allen has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Takeda. Dr. Allen has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Grifols. Dr. Allen has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Alnylam. Dr. Allen has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Sanofi. Dr. Allen has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Astra Zeneca. Dr. Allen has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Johnson and Johnson. Dr. Allen has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Alexion. Dr. Allen has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Annexon. Dr. Allen has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Immunovant. Dr. Allen has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Amgen. Dr. Allen has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Dianthus. Dr. Allen has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for CSL Behring. Dr. Allen has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Annexon. Dr. Allen has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Takeda. Dr. Allen has received personal compensation in the range of $10,000-$49,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Argenx. Dr. Allen has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Dianthus. Dr. Allen has received personal compensation in the range of $10,000-$49,999 for serving on a Speakers Bureau for CSL behring. Dr. Allen has received personal compensation in the range of $5,000-$9,999 for serving on a Speakers Bureau for Takeda. Dr. Allen has received personal compensation in the range of $5,000-$9,999 for serving on a Speakers Bureau for Alnylam. Dr. Allen has received personal compensation in the range of $5,000-$9,999 for serving on a Speakers Bureau for Alexion. Dr. Allen has received personal compensation in the range of $5,000-$9,999 for serving on a Speakers Bureau for Johnson and Johnson.

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