Mossakowski Medical Research Center Polish Academy of Sciences. Department of Neuromuscular
A 38-year-old patient with suspected muscular dystrophy was examined. A biopsy of biceps sinister was performed. Electronmicroscopy analysis revealed some fibers of correct diameter and preserved myofibrils structure. Also fibers with normal located under the sarcolemma nuclei but with visible alterations in the miofibrils architecture were seen.In some fibers, multiply centrally located nuclei were visible, and between the fibers abundant connective tissue was present.Numerous muscle fibers of myotube-like morphology, characterized by small diameter and centrally located nuclei, usually single or in number of two were observed. They showed abnormal sarcomeres structure and loss of A line.Intramuscular nerves characterized by the presence of a 2-3-layer myelin sheath resembling the "onion bulb" structure were observe.Microscopic image of the biopsy suggests congenital fiber-type disproportion (CFTS) associated with defect of actin or tropomyosin.
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Mossakowski Medical Research Institute PAS
Library of the Mossakowski Medical Research Institute PAS
Feb 1, 2022
Aug 27, 2020
40
https://rcin.org.pl./publication/112204
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opis 23/12 | Feb 1, 2022 |
Fidziańska-Dolot, Anna (1930–2015)
Fidziańska-Dolot, Anna (1930–2015)