This study used a quantitative MRI to assess the nerve tissue. This type of technology is very sensitive to how the cells change over time.

The researchers compared the results of this test in CMT1a patients (32) as well as healthy volunteers (13). The controls were matched to the patients by their sex and age. On average, patients had been diagnosed for 25 years and were 37.5 years old. For most, symptoms began in their childhood. They measured the following in both the thigh and the leg-

• Nerve volume
• Proton density
• Magnetization Transfer Ratio

They wanted to uncover whether these impacted-

• Functional disability in patient’s daily life (using ONLS and ONLSL)
• Muscle strength
• Disease severity
• Clinical disability

Results

The team found that both nerve volume and the proton density were higher for CMT1a patients than the controls in the study. This was true for both the thigh and leg region. However, the magnetization transfer ratio was lower for patients in both.

Overall, the researchers determined that the thigh’s nerve volume was correlated with all of the clinical measures. The same was not true for the other nerve region. Proton density in the nerve was also correlated with functional disability measures. The magnetization transfer ratio was correlated to the strength of the quadriceps. These findings and others mean that the qMRI measurements for the thigh may be the best measurements for assessing disease progression and therapeutic progress.

Looking Forward