Yonsei Med J.  1991 Jun;32(2):184-189. 10.3349/ymj.1991.32.2.184.

Two cases of mitochondrial myopathy with predominant respiratory dysfunction

Affiliations
  • 1Department of Neurology, Yonsei University College of Medicine, Korea.
  • 2Department of Pathology, Seoul National University, College of Medicine, Seoul, Korea.

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.

Keyword

Mitochondrial myopathy; respiratory dysfunction

MeSH Terms

Adolescent
Adult
Electromyography
Female
Human
*Mitochondria, Muscle/ultrastructure
Muscular Diseases/*complications
Respiration, Artificial
Respiratory Insufficiency/*etiology/therapy
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