Abstract Details

Title Minimal Symptom Expression and Minimal Manifestations in Chronic Inflammatory Demyelinating Polyneuropathy (CIDP)

Topic Neuromuscular and Clinical Neurophysiology (EMG)

Presentation(s) S16 - Updates on Nerve and Muscle Disorders (1:00 PM-1:12 PM)

Poster/Presentation
Number 001

Background NA

Objective Although most patients with chronic inflammatory demyelinating polyneuropathy (CIDP) respond to immunotherapy, residual neurologic disability is common. The objective of this study is (1) to determine the frequency of minimal symptom expression (MSE) in CIDP and (2) to use that information to develop definitions for minimal manifestations (MM).

Design/Methods

We retrospectively analyzed patients with CIDP per EAN/PNS guidelines. Demographics, disease course, diagnostics, and outcomes were collected. MSE was defined as normal patient-reported daily activities with or without mild CIDP-associated symptoms. Outcomes and disease characteristics were analyzed in those with and without MSE status.

Results Sixty-one patients were identified, 69% were classified as CIDP and 31% as possible CIDP according to EAN/PNS guidelines. 75% were classified as “typical” CIDP. Mean age of onset was 50.0 years and 44.3% were female. MSE was present in 29.5%. Mean outcomes in patients with MSE vs those without MSE at last follow up were: MRC sum 60.0 vs 57.1, I-RODS (in centile) was 84.8 vs 57.9, and dominant grip strength (percent of age and gender adjusted normal value) was 99.8% vs 84.9%. A dominant grip strength of 70% of mean age and gender-adjusted grip strength had 100% sensitivity for MSE. Immunotherapy was discontinued in 66% with MSE and 35% without. Those with MSE were more likely to be younger (41.2 vs 53.5, p=0.005), and less likely to have concomitant diabetes (p=0.005), and ataxia (p=0.036).

Conclusions

Although residual CIDP deficits are common, it is possible to reach MSE. We propose defining MM as MRC sum score of 60/60 (mild toe and finger weakness permitted), grip strength > 70% of age and gender-adjusted normal value, normal sensory examination (mild reduction in the toes or fingertips permitted), and normal gait without assistive devices. Combining the subjective MSE with objective MM definition may improve prognostic and treatment expectation management.

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.
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 $500-$4,999 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 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 Octapharma. 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 $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Argenx. 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 Alnyla,m. Dr. Allen has received personal compensation in the range of $5,000-$9,999 for serving on a Speakers Bureau for Alexion.

��ɫ��

��ɫ��