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Contrasting histochemical features of various mitochondrial syndromes
Author(s) -
Collins S.,
Byrne E.,
Dennett X.
Publication year - 1995
Publication title -
acta neurologica scandinavica
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.967
H-Index - 95
eISSN - 1600-0404
pISSN - 0001-6314
DOI - 10.1111/j.1600-0404.1995.tb07007.x
Subject(s) - mitochondrial myopathy , myoclonus , lactic acidosis , cytochrome c oxidase , chronic progressive external ophthalmoplegia , myopathy , encephalopathy , pathology , medicine , biology , mitochondrion , biochemistry , anesthesia , mitochondrial dna , gene
A comparative histochemical analysis of the prevalence and cytochrome oxidase staining characteristics of ragged‐red fibres in limb skeletal muscles was performed in 19 patients spanning four distinct mitochondrial syndromes: chronic progressive external ophthalmoplegia; myoclonus epilepsy with ragged‐red fibres; mitochondrial myopathy, encephalopathy, lactic acidosis and stroke‐like episodes; and pure limb myopathy. The percentage occurrence of non‐ragged red but cytochrome oxidase negative fibres was additionally noted. Ragged‐red fibres and cytochrome oxidase‐negative fibres were generally more prevalent in the chronic progressive external ophthalmoplegia syndrome than in myoclonus epilepsy ragged‐red fibres syndrome or mitochondrial myopathy encephalopathy lactic acidosis and stroke‐like episodes syndrome. Isolated cytochrome oxidase‐negative fibres were a common finding in each phenotypic syndrome except pure limb myopathy and could involve any of the major fibre types non‐specifically. Ragged‐red fibres were devoid of cytochrome oxidase activity in chronic progressive external ophthalmoplegia, but commonly displayed activity in the other three syndromes providing a clue to syndromal differentiation on a histochemical basis.