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Centronuclear ("Myotubular") Myopathy
Theodore L. Munsat, MD;
Lawrence R. Thompson, MD;
Ralph F. Coleman, MD
Arch Neurol. 1969;20(2):120-131.
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| Since this article does not have an abstract, we have provided the first 150 words of the full text PDF and any section headings. |
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IN 1966 Spiro and co-workers1 reported a 12-year-old boy with slowly progressive weakness from infancy who had unusual muscle fibers with centrally placed nuclei. Histochemical alterations in the central portions of the muscle fibers were noted. Because of similarities between these muscle fibers and the myotubular stage of embryogenesis, the authors designated the disorder "myotubular" myopathy and suggested an arrested state of development persisting into adolescence. Two sisters and an additional female with similar histochemical, clinical, and structural findings have been more recently reported.2,3 The present paper describes the fifth reported case. However, certain differing structural and histochemical findings suggest a less specific pathogenesis.
Methods
Muscle biopsy was obtained with an isometric clamp. Part of the specimen was fixed in 10% formol alcohol for paraffin embedding. Hematoxylin and eosin (H & E), periodic acid-Schiff (PAS), Masson's trichrome, phosphotungstic acid hematoxylin (PTAH), and oil red O stains were done
. . . [Full Text PDF of this Article]
Author Affiliations
Los Angeles
From the Division of Neurology, Department of Medicine (Dr. Munsat), and the Department of Pathology (Drs. Thompson and Coleman), UCLA School of Medicine, Los Angeles.
Footnotes
Submitted for publication April 15, 1968; accepted Oct 3.
Read in part before the Second International Congress of Neurogenetics and Neuro-Ophthalmology, Montreal, 1967.
Reprint requests to Division of Neurology, UCLA School of Medicine, Los Angeles 90024 (Dr. Munsat).
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