Chronic progressive external ophthalmoplegia: II. A qualitative and quantitative electronmicroscopy study of skeletal muscles
This study quantifies the maior electron microscopic changes in limb muscle biopsies from 31 out of 34 patients with the syndrome of chronic progressive external ophthalmoplegia. Patients were divided into three clinical groups - A) 10 sporadic cases with muscle weakness only; B) 9 familial cases wi...
Saved in:
| Main Author: | |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
Thieme Revinter Publicações
1988-06-01
|
| Series: | Arquivos de Neuro-Psiquiatria |
| Online Access: | http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0004-282X1988000200005&lng=en&tlng=en |
| Tags: |
Add Tag
No Tags, Be the first to tag this record!
|
| _version_ | 1849702994777473024 |
|---|---|
| author | Elza Dias-Tosta |
| author_facet | Elza Dias-Tosta |
| author_sort | Elza Dias-Tosta |
| collection | DOAJ |
| description | This study quantifies the maior electron microscopic changes in limb muscle biopsies from 31 out of 34 patients with the syndrome of chronic progressive external ophthalmoplegia. Patients were divided into three clinical groups - A) 10 sporadic cases with muscle weakness only; B) 9 familial cases with muscle weakness only; C) 15 cases with muscle weakness and one or more of the following features: pigmentary retinopathy, cerebellar ataxia, pyramidal signs and peripheral neuropathy. Electron microscopic mitochondrial abnormalities were found in all groups (8 patients from group A, 3 from group B, 14 from group C). Quantitative measurements of certain muscle fibre constituents, using a point-counting technique, revealed decreased myofibril volume-fractions and increased volume-fractions of mitochondria, glycogen and lipid in some biopsies from each group. Mitochondrial volume-fractions correlated positively with lipid content, the proportion of type 1 fibres, and the percentage of fibres with increased oxidative enzyme activity. The three groups defined clinically showed no significant differences in terms of the relative proportions of these measured constituents. |
| format | Article |
| id | doaj-art-0fd5a9e73d0941238afe5d10689dff4c |
| institution | DOAJ |
| issn | 1678-4227 |
| language | English |
| publishDate | 1988-06-01 |
| publisher | Thieme Revinter Publicações |
| record_format | Article |
| series | Arquivos de Neuro-Psiquiatria |
| spelling | doaj-art-0fd5a9e73d0941238afe5d10689dff4c2025-08-20T03:17:27ZengThieme Revinter PublicaçõesArquivos de Neuro-Psiquiatria1678-42271988-06-0146214315510.1590/S0004-282X1988000200005S0004-282X1988000200005Chronic progressive external ophthalmoplegia: II. A qualitative and quantitative electronmicroscopy study of skeletal musclesElza Dias-Tosta0University of LondonThis study quantifies the maior electron microscopic changes in limb muscle biopsies from 31 out of 34 patients with the syndrome of chronic progressive external ophthalmoplegia. Patients were divided into three clinical groups - A) 10 sporadic cases with muscle weakness only; B) 9 familial cases with muscle weakness only; C) 15 cases with muscle weakness and one or more of the following features: pigmentary retinopathy, cerebellar ataxia, pyramidal signs and peripheral neuropathy. Electron microscopic mitochondrial abnormalities were found in all groups (8 patients from group A, 3 from group B, 14 from group C). Quantitative measurements of certain muscle fibre constituents, using a point-counting technique, revealed decreased myofibril volume-fractions and increased volume-fractions of mitochondria, glycogen and lipid in some biopsies from each group. Mitochondrial volume-fractions correlated positively with lipid content, the proportion of type 1 fibres, and the percentage of fibres with increased oxidative enzyme activity. The three groups defined clinically showed no significant differences in terms of the relative proportions of these measured constituents.http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0004-282X1988000200005&lng=en&tlng=en |
| spellingShingle | Elza Dias-Tosta Chronic progressive external ophthalmoplegia: II. A qualitative and quantitative electronmicroscopy study of skeletal muscles Arquivos de Neuro-Psiquiatria |
| title | Chronic progressive external ophthalmoplegia: II. A qualitative and quantitative electronmicroscopy study of skeletal muscles |
| title_full | Chronic progressive external ophthalmoplegia: II. A qualitative and quantitative electronmicroscopy study of skeletal muscles |
| title_fullStr | Chronic progressive external ophthalmoplegia: II. A qualitative and quantitative electronmicroscopy study of skeletal muscles |
| title_full_unstemmed | Chronic progressive external ophthalmoplegia: II. A qualitative and quantitative electronmicroscopy study of skeletal muscles |
| title_short | Chronic progressive external ophthalmoplegia: II. A qualitative and quantitative electronmicroscopy study of skeletal muscles |
| title_sort | chronic progressive external ophthalmoplegia ii a qualitative and quantitative electronmicroscopy study of skeletal muscles |
| url | http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0004-282X1988000200005&lng=en&tlng=en |
| work_keys_str_mv | AT elzadiastosta chronicprogressiveexternalophthalmoplegiaiiaqualitativeandquantitativeelectronmicroscopystudyofskeletalmuscles |