GNE and myopathy: GNE (bifunctional UDP-N-acetylglucosamine 2-epimerase/N-acetylmannosamine kinase) myopathy has been described for first time in 1981 by Ikuya Nonaka and colleagues as a distal myopathy with rimmed vacuoles and lamellar (myeloid) body depositions thus receiving the name of “Nonaka Distal Myopathy” or “Distal Myopathy with Rimmed Vacuoles” (DMRV) [1].