With the clinical finding of scapular winging or distal weakness and myopathological finding of rimmed vacuoles as well as TDP-43 immunopositive inclusions, inclusion body myopathy is often confused with GNE myopathy (glucosamine (UDP-N-acetyl)-2-epimerase/N -acetylmannosamine kinase) or facioscapulohumeral dystrophy [2]. This evidence concerns the gene GNE and GNE myopathy.