Quantitative MR-neurographic parameters can determine and specify nerve injury in amyloid related polyneuropathy

Background Hereditary transthyretin-familial-amyloid-polyneuropathy (TTR-FAP) usually manifests with a rapidly progressive, distally-symmetric polyneuropathy [Plante-Bordeneuve, V. and G. Said, Lancet Neurol, 2011; Hund et al, Neurology 2001]. Recently, we were able to show that nerve-injury in TTR-FAP is detectable in-vivo by applying high-resolution MR-Neurography [Kollmer et al, Brain 2015]. The aim of the current study is to further quantify nerve-lesions at thigh-level where nerve-injury has been shown to be strongest, and to determine the ability of two quantitative parameters to clearly differentiate between symptomatic TTR-FAP, asymptomatic gene-carriers and healthy volunteers.