Minimal Symptom Expression and Minimal Manifestations in Chronic Inflammatory Demyelinating Polyneuropathy (CIDP)
Stephen Ward1, Abhigyan Datta1, Jeffrey Allen2
1University of Minnesota, 2UMN
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.
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