Case Series: Feasibility of Longitudinal Assessment of the Sciatic Nerve in CMT1A Using High-Resolution 7T MRI.

INTRODUCTION/AIMS: There is limited data on the sensitivity and responsiveness of high-resolution imaging techniques in the longitudinal assessment of hereditary neuropathies. In this study, our aims were to investigate the ability of ultra-high field magnetic resonance imaging to detect longitudinal changes in the peripheral nerves of Charcot-Marie-Tooth (CMT) 1A patients, and to evaluate the potential benefits of doing so at the nerve fascicle level.

METHODS

We performed magnetic resonance imaging (MRI) to simultaneously obtain high-resolution anatomical and quantitative data at ultra-high 7 Tesla field strength in peripheral nerves of four patients with CMT1A disease at baseline and follow up. We compared the resulting measurements of T2 in sciatic, tibial, and fibular nerves within individual fascicles of the three nerve regions.

RESULTS

Analyzing individual fascicle distributions, we demonstrated a significantly elevated T2 in the fibular nerve over the course of the study, with a mean increase of 3.55 ms (p = 0.01). Changes in the sciatic nerve were marginally significant (mean increase 1.42 ms, p = 0.05), and tibial nerve changes were not significant (mean increase 1.31 ms, p = 0.18). Combining fascicles across subjects showed significant changes in all three nerves over time.

DISCUSSION: Our results indicate that longitudinal MRI assessment of individual nerve fascicles may serve as a quantitative biomarker of disease progression in patients with hereditary neuropathies. Further, our study demonstrates that the data provided by fascicle-level analysis may provide better analytical abilities than whole-nerve imaging.