Two cases of mitochondrial myopathy with predominant respiratory dysfunction.
10.3349/ymj.1991.32.2.184
- Author:
Gyung Whan KIM
1
;
Seung Min KIM
;
Il Nam SUNWOO
;
Je G CHI
Author Information
1. Department of Neurology, Yonsei University College of Medicine, Korea.
- Publication Type:Case Report
- Keywords:
Mitochondrial myopathy;
respiratory dysfunction
- MeSH:
Adolescent;
Adult;
Electromyography;
Female;
Human;
*Mitochondria, Muscle/ultrastructure;
Muscular Diseases/*complications;
Respiration, Artificial;
Respiratory Insufficiency/*etiology/therapy
- From:Yonsei Medical Journal
1991;32(2):184-189
- CountryRepublic of Korea
- Language:English
-
Abstract:
Although it is well known that the respiratory failure is a major cause of death in most patients with chronic neuromuscular disease, predominant respiratory dysfunction without severe involvement of limb muscles is an unusual complication of mitochondrial myopathy in adult age. We experienced two cases of mitochondrial myopathy with severe involvement of respiratory function and only mild involvement of limb muscles. One is a 16 year old female and another is a 22 year old male. The diagnosis is based on morphologic characteristics of "ragged red fibers" under the light microscope and abnormal mitochondrias on the electron microscope in the muscle biopsy.
