Purpose:The follow-up and monitoring of response to immunomodulatory therapy in patients with chronic inflammatory demyelinating polyneuropathy are still challenging. Various outcome measures have been proposed in recent years, and some are now frequently used in daily clinical practice; however, reliable biomarkers for the disease activity and treatment response are lacking.Methods:Cross-sectional nerve area of the bilateral vagus, fifth and the sixth cervical spinal, median, ulnar, tibial, peroneal, and sural nerves were measured at 2 time points with an interval of 6 months using nerve ultrasound. The results were used to calculate the ultrasound pattern sumscore (UPSS). The correlation between UPSS change (Delta UPSS) and changes in functional and nerve conduction studies measures over the study period were assessed.Results:Sixteen patients completed this prospective, observational study. General linear model showed that Delta UPSS is significantly associated with Delta Medical Research Council sumscore (beta = -0.72, P = 0.003), Delta handgrip strength (beta = -0.57, P = 0.014), Delta Rasch-built overall disability scale (beta = -0.57, P = 0.010), and Delta overall neuropathy limitations scale (beta = 0.75, P < 0.001), after adjustment of confounding variables. Nevertheless, Delta UPSS was not correlated with other clinical measures, including Delta pinch power, Delta 9-hole peg test, Delta 10-m walking test, and Delta nerve conduction study sumscore (P values > 0.05).Conclusions:Nerve ultrasound might be an efficient method for monitoring the functional status of patients with chronic inflammatory demyelinating polyneuropathy over time because the alterations in its scores could significantly reflect clinical changes.