Disease status in chronic inflammatory demyelinating polyneuropathy: Inter-centre comparative analysis and correlates

Background and Purpose: Chronic inflammatory demyelinating polyneuropathy (CIDP) may have variable evolution profiles, which have not been compared between cohorts. The relationship of disease status with motor strength, function and electrophysiology is uncertain. Methods: Disease status was studied with a simplified proposed scale in two patient cohorts totalling 72 subjects from Leicester, UK, and Angers, France. Clinical and electrophysiological records were analysed. Results: Independent ascertainment of disease status in each cohort revealed similar rates of remission (P = 0.23), stable/improving disease (P = 0.34) and unstable/active disease (P = 1). No correlation was ascertained with strength or function. Median nerve compound muscle action potential was the only independent electrophysiological predictor of disease status ascertained (P = 0.046). Conclusions: Disease status distribution may represent an important comparative indicator for management of CIDP cohorts and could be useful for benchmarking service and treatment provision. Degree of upper limb motor axonal loss may represent a useful electrophysiological marker of disease status in CIDP.