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Files for neuromuscular diseases (2014) - nr 24/14
Creator:Fidziańska-Dolot, Anna (1930–2015)
Contributor:Mossakowski Medical Research Center Polish Academy of Sciences. Department of Neuromuscular
Place of publishing: Date issued/created: Description:Muscle segment examination in light & electon microscopy
Subject and Keywords:Myasthenic syndrome ; Glycogen ; Nucleopathy
Abstract:A 5-month-old patient with suspected myasthenic syndrome was examined. A biopsy of quadricepswas performed. Electronmicroscopy analysis revealed numerous ultrastructural abnormalities ofmuscle fibers with features of immaturity. Local disintegration of myofibrils and swollenmitochondria were observed. Mitochondria were characterized by partial or total lack ofmitochondrial cristae. Massive glycogen depositions were seen (Fig. 1,2). In some part of myocytesloss of myofibrils were visible. Empty areas were filled with homogeneous myofibril-like material(Fig. 3).Multi-shaped nuclei with the characteristic accumulation of heterochromatin and cleavageofthe outer nuclear membrane were observed. Mitochondria were swollen, with blurred cristae andmitochondrial membranes (Fig 4,5,6). Nuclei of irregular shape with clear features of nucleopathy(disrupted nuclear membrane, mitochondria within the nuclei) were seen (Fig 8,9).
Resource type: Detailed Resource Type: Format: Language: Language of abstract: Rights:Creative Commons Attribution BY 4.0 license
Terms of use:Copyright-protected material. [CC BY 4.0] May be used within the scope specified in Creative Commons Attribution BY 4.0 license, full text available at: ; -
Digitizing institution:Mossakowski Medical Research Institute PAS
Original in:Library of the Mossakowski Medical Research Institute PAS
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