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Severe neonatal myotonic dystrophy (quadriceps femoris muscle biopsy) (2)

Longitudinal section shows the centronuclear fibers have large, vesicular nuclei in a row, but they are not closely spaced, and clear spaces are seen between nuclei. These spaces contain mitochondria, as in fetal myotubes. X250. (Reproduced with permission from: Sarnat HB, O’Connor T, Byrne PA. Clinical effects of myotonic dystrophy on pregnancy and the neonate. Arch Neurol 1976;33:459-65. Copyrighted 1976, American Medical Assoc.)

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Associated Disorders

  • Steinert disease, neonatal form
  • congenital myopathies
  • myotonic disorders