The authors declare that they have no conflict of interest. The peer review history for this article is available at https://www.webofscience.com/api/gateway/wos/peer-review/10.1111/nan.12966. The datasets used and analysed during the present study are available from the corresponding author on reasonable request. Table S1. Primer pairs used for Sanger sequencing. Table S2. Primary antibodies. Table S3. Distribution of neuronal loss and TDP-43 inclusions. Figure S1. Mild neurogenic muscle atrophy is shown by mild small-group atrophy and some angulated fibres in the diaphragm (A) and iliopsoas muscle (B). HE stained, formalin-fixed, paraffin-embedded tissue sections. Bar = 100 μm. Please note: The publisher is not responsible for the content or functionality of any supporting information supplied by the authors. Any queries (other than missing content) should be directed to the corresponding author for the article.