Diffusion tensor imaging yields valid quantitative biomarkers in CIDP and might aid in diagnosis with high diagnostic accuracy. Fractional anisotropy and RD may serve as parameters of myelin sheath integrity, but AD is unable to reflect axonal damage in CIDP.

• Peripheral nerve diffusion tensor imaging (DTI) parameters depend on demographic variables. • Fractional anisotropy (FA) declines with increasing age and weight. • Gender does not systematically affect peripheral nerve DTI. • The formula presented here allows adjustment of FA for demographic variables.

Patients with Fabry disease have severely enlarged dorsal root ganglia with dysfunctional perfusion. This may be due to glycolipid accumulation in the dorsal root ganglia mediating direct neurotoxic effects and decreased neuronal blood supply. These alterations were less pronounced in peripheral nerve segments. Thus, the dorsal root ganglion might play a key pathophysiologic role in the development of neuropathy and pain in Fabry disease.