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Review CIDP Optimizing the Use of Outcome Measures in Chronic Inflammatory Demyelinating Polyneuropathy Jeffrey A Allen, 1,2 Deborah F Gelinas, 3 Richard A Lewis, 4 Richard J Nowak 5 and Gil I Wolfe 6 1. Department of Neurology, University of Minnesota, Minneapolis, US; 2. Department of Neurology, Northwestern University, Chicago, Illinois, US; 3. Grifols Shared Services North America, North Carolina, US; 4. Department of Neurology, Cedars-Sinai Medical Center, Los Angeles, California, US; 5. Division of Neuromuscular Medicine, Department of Neurology, Yale School of Medicine, New Haven, Connecticut, US; 6. Department of Neurology, University at Buffalo Jacobs School of Medicine and Biomedical Sciences/SUNY, Buffalo, NY, US T he challenges encountered during the assessment of patients with chronic inflammatory demyelinating polyneuropathy (CIDP) are many. Ideally, CIDP outcome measures capture impairments in disability, strength, and sensory dysfunction, and quality of life (QoL). A number of outcome measures have been validated for this purpose. Disability outcomes include the adjusted inflammatory neuropathy cause and treatment (INCAT) disability score, INCAT overall disability sum score (ODSS), and overall neuropathy limitations scale (ONLS). A more sensitive disability score, the inflammatory Rasch-built overall disability scale (I-RODS), has also been validated for use in clinical trials and may better capture clinically meaningful changes in those with CIDP. Strength and sensory impairment can be assessed in a number of ways, including the INCAT sensory subscore (ISS), Medical Research Council sum score, and Martin vigorimeter or Jamar dynamometer grip strength. However, the feasibility of applying and interpreting these measures during routine daily practice has been questioned. Furthermore, these outcome measures may not reflect other factors that can impair QoL in those affected by CIDP, such as pain and fatigue. A valid, reliable, and responsive composite measure that addresses all aspects of impairment faced by patients with CIDP remains an unmet need in clinical practice. Keywords Chronic inflammatory demyelinating polyneuropathy, disability, impairment, outcome measures, grip strength Disclosure: Jeffrey A Allen is a consultant for, and has received clinical trial support, from: Axelacare, CSL Behring, and Grifols. Deborah F Gelinas is an employee of Grifols, and is on the Avanir Speaker Bureau for Nuedexta. Richard A Lewis is a consultant for Axelacare, CSL Behring, Biotest Pharma, Kedrion, and Pharnext. Richard J Nowak is a speaker and advisor/consultant for Grifols. Gil I Wolfe participated in Shire and Grifols advisory boards and received research support from CSL Behring. Compliance with Ethics: This study involves a review of the literature and did not involve any studies with human or animal subjects performed by any of the authors. Authorship: All named authors meet the International Committee of Medical Journal Editors (ICMJE) criteria for authorship of this manuscript, take responsibility for the integrity of the work as a whole, and have given final approval to the version to be published. Open Access: This article is published under the Creative Commons Attribution Noncommercial License, which permits any noncommercial use, distribution, adaptation, and reproduction provided the original author(s) and source are given appropriate credit. Received: December 19, 2016 Accepted: February 7, 2017 Citation: US Neurology, 2017;13(1):26–34 Corresponding Author: Jeffrey A Allen, Department of Neurology, 12–150 Phillips Wangensteen Building, 516 Delaware Street SE, Minneapolis, MN 55455. E: jeffrey.allen@nm.org Support: The publication of this article was supported by Grifols. The views and opinions expressed in the article are those of the authors and not necessarily those of Grifols. US/ GX/1016/0386 Chronic inflammatory demyelinating polyneuropathy (CIDP) is an acquired immune-mediated disease that evolves in a progressive or relapsing pattern over months to years. Although “typical” CIDP is characterized by symmetric proximal and distal motor and sensory deficits, it is now recognized that multifocal (asymmetric), distally predominant, pure sensory, and pure motor variants also fall within the CIDP spectrum. First-line treatment options for CIDP include corticosteroids, intravenous immunoglobulin (IVIG), and plasmapheresis (plasma exchange). 1 For patients refractory to first-line options or those chronically dependent on high-dose first-line therapy, no evidence-based treatment recommendations exist. Cytotoxic immunosuppressant drugs are sometimes utilized. 2 Close follow-up care is essential for treatment administration and optimization. Patients treated with IVIG or plasma exchange need regular treatment visits to maintain therapeutic efficacy, typically every few weeks. Many patients with CIDP remain on such treatment for years. While, in some, chronic immunotherapy is justified on the basis of well-defined clinical changes indicative of active disease (e.g., treatment-related fluctuations or relapse); in many patients, treatment is driven by subjective feelings of benefit without objective evidence of improvement in motor and sensory deficits or disability. 3 There is an opportunity to supplement periodic outpatient clinical visits with currently available objective measures as a means to improve confidence in treatment-induced disease modification, optimize therapy, and justify treatment dependence for those on chronic therapy. Evaluating responses to treatment in CIDP may be difficult. The absence of a clear definition of treatment response, in part due to the heterogeneous nature of CIDP and its variants, is one challenge. The many scales that have 26 TOUCH ME D ICA L ME D IA