CONTRASTING HISTOCHEMICAL FEATURES OF VARIOUS MITOCHONDRIAL SYNDROMES

被引：18

作者：

COLLINS, S ^{[1
]}

BYRNE, E ^{[1
]}

DENNETT, X ^{[1
]}

机构：

[1] UNIV MELBOURNE,STATE NEUROPATHOL SERV,PARKVILLE,VIC 3052,AUSTRALIA

来源：

ACTA NEUROLOGICA SCANDINAVICA | 1995年 / 91卷 / 04期

关键词：

MITOCHONDRIAL ENCEPHALOMYOPATHIES; CYTOCHROME OXIDASE; RAGGED-RED FIBERS; HISTOCHEMISTRY;

D O I：

10.1111/j.1600-0404.1995.tb07007.x

中图分类号：

R74 [神经病学与精神病学];

学科分类号：

摘要：

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.

引用

页码：287 / 293

页数：7

共 35 条

[11] DEFICIENCY OF SUBUNITS OF COMPLEX-I AND MITOCHONDRIAL ENCEPHALOMYOPATHY [J].