Mallory body‐like inclusions in a hereditary congenital neuromuscular disease

Muscle and Nerve - Tập 6 Số 3 - Trang 195-200 - 1983
Anna Fidziańska1, Hans H. Goebel1, Mary Osborn2, H. G. Lenard3, G. Osse3, U. Langenbeck4
1department of Neuropathology, University of Göttingen, Göttingen, FRG
2Max-Planck-Institute for Biophysical Chemistry, Göttingen, FRG
3Department of Pediatrics, University of Göttingen, Göttingen, FRG
4Department of Genetics, University of Göttingen, Göttingen, FRG

Tóm tắt

AbstractSubsequent to an earlier report on clinical and light microscopic data, peculiar Mallory body‐like inclusions are described in muscle fibers of three genetically linked children. These Mallory body‐like inclusions were unlike other well‐defined intramuscular inclusions, such as nemaline, cytoplasmic, fingerprint, or sarcoplasmic bodies, but morphologically quite similar to hepatic Mallory bodies, because they were composed of three components: granular material and two types of filaments. Evidence is presented that these inclusions may contain desmin, the intermediate filament type characteristic of muscle. The exclusive appearance of these Mallory body‐like inclusions in muscle biopsy specimens from theree genetically related children of a large kinship emphasizes the uniqueness of these Mallory body‐like inclusions in these muscle fibers as well as the special form of this congenital neuromuscular disorder.

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Muscle and Nerve

Tập 6 Số 3

195-200

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