Failure of “Dystrophic” Neurones to Support Functional Regeneration of Normal or Dystrophic Muscle in Culture

Abstract

THE traditional concept that muscular dystrophy is a “primary degenerative myopathy”¹ has recently been challenged, and the suggestion put forward that the disease may in fact have a neural basis. This has been based on several independent investigations, along different lines. Dubowitz² observed “dystrophy-like” changes in the histochemical preparations of experimentally reinnervated muscle, and postulated that muscular dystrophy might be caused by an aberration of the normal controlling influence of the nervous system on muscle. In a series of electrophysiological studies in Duchenne and other forms of muscular dystrophy, McComas et al.^3–5 demonstrated a marked reduction in the number of motor units. Similarly, in the dystrophic mouse it was found that many muscle fibres were functionally denervated⁶ and that there were fewer motor units, which were still of normal size⁷.