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Changes in motor innervation and histochemical pattern of muscle fibers in some congenital myopathies

From the Department of Neurology, Medical Clinic, Brugmann Hospital, Brussels (Drs. Coërs and Telerman-Toppet); Department of Neurology, Medical Clinic, St. Pierre Hospital, and Department of Electron Microscopy, Brugmann Hospital, Brussels (Dr. Gérard); Service of Pediatry, St. Pierre Hospital, and Laboratory of Cytology and Experimental Cancerology; Brussels (Dr. Szliwowski); and Muscle Research Centre of the Department of Neurology, University of Amsterdam, Wilhelmina Gasthuis, Amsterdam (Drs. Bethlem and van Wijngaarden).

Changes in motor innervation were compared with histologic and histochemical pattern of muscle fibers in three biopsies of central core disease, four biopsies of nemaline myopathy, one biopsy of myotubular myopathy, and three biopsies of mitochondrial myopathy. Evidence of collateral reinnervation was obtained only in one biopsy from central core disease. In other biopsies, no structural or ultrastructural abnormality of axis cylinders, myelin, or myoneural junction suggesting denervation were observed. The only relevant change found in centronuclear myopathy and to a lesser extent in nemaline myopathy was an unusual smallness and simplification of motor endings, suggesting delayed or impaired maturation. Muscle fibers strongly reactive for both adenosinetriphosphatase and nicotinamide-adenine dinucleotide diaphorase, observed in central core disease and mitochondrial myopathy, were not associated with increased terminal innervation ratio.

Requests for reprints should be addressed to Dr. Coërs, Neurology Department, Service de Médecine, Hôpital Brugmann, 4 Place Van Gehuchten, 1020 Bruxelles, Belgium.

This work was supported by grants from the F.R.S.M. of Belgium, the Free University of Brussels and the Prinses Beatrix Fonds of The Netherlands.

Received for publication December 9, 1975.

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