Questioning the causality of HTT CAG-repeat expansions in FTD/ALS

First is their claim that the carrier rate found in their cohort is 4.4 times higher than expected based on the 0.03% (10/31,372) rate determined by Gardiner and collaborators and data from the 100,000 genomes project. [...]the scarce clinical descriptions of the cases do not seem, from a clinician’s point of view, satisfactory enough to untangle the tricky differential diagnosis of HD versus FTD. [...]previous published works have shown that there is no correlation between the CAG repeat expansion and cortical neuronal loss, and that cortical neuron loss is often more severe than what would be expected from the CAG repeat length and duration of the disease (Nana et al., 2014).