Endocrine functions were examined in 21 patients with mitochondrial myopathies presenting with chronic progressive external ophthalmoplegia and other additional neurological and multisystemic symptoms. Ten patients had the features of the Kearns-Sayre syndrome. Deletions of the mitochondrial DNA wer
Mitochondrial abnormalities in selenium-deficient myopathy
β Scribed by Yasushi Osaki; Ichizo Nishino; Nobuyuki Murakami; Kozo Matsubayashi; Keisuke Tsuda; Yu-Ichi Yokoyama; Masanori Morita; Saburo Onishi; Yu-Ichi Goto; Ikuya Nonaka
- Book ID
- 101250608
- Publisher
- John Wiley and Sons
- Year
- 1998
- Tongue
- English
- Weight
- 284 KB
- Volume
- 21
- Category
- Article
- ISSN
- 0148-639X
No coin nor oath required. For personal study only.
β¦ Synopsis
We report a man who developed selenium-deficient myopathy during long-term parenteral nutrition. Muscle biopsy showed marked mitochondrial depletion in the deep sarcoplasm and enlarged mitochondria at the periphery mainly in type 2 fibers. Muscle weakness improved gradually after the second course of selenium supplementation. The peculiar mitochondrial abnormalities in muscle fibers appear to play a key role in the pathogenesis of selenium-deficient myopathy.
π SIMILAR VOLUMES
We studied two siblings with a mitochondrial myopathy, familial thiamine deficiency, and an A3243G mutation of the mitochondrial DNA (mtDNA). The elder brother (patient 1, now 36 years old) developed myopathy and beriberi heart at 20 years of age. Thiamine therapy resolved the cardiac symptoms and h