“…L ater th e degeneration progresses and the site of th e fibres is m arked solely by long rows of nuclei [13,42,43]. Sim ilar changes were found in th e m uscles of th e upper oeso phagus, in extrinsic m uscles of the lary n x and in th e diaphragm [31,42,43,44,45,46], V ariab ility in size of fibres, a tro p h y and som etim es fibrosis or fa tty degeneration were observed in th e h ea rt muscle [44,47]. No changes in th e ce n tral nervous system were found in pure cases of MD [30,31,41].…”