Original Paper

Early Mitochondrial Changes in Chronic Progressive Ocular Myopathy
J. Aasly^a, S. Lindal^b, T. Torbergsen^a, O. Borud^c, S.-I. Mellgren^a

Departments of
^aNeurology,
^bPathology and
^cClinical Chemistry, University Hospital, Tromsø, Norway

Address of Corresponding Author

Eur Neurol 1990;30:314-318 (DOI: 10.1159/000117363)

  Key Words

• Mitochondrial myopathy
• Ophthalmoplegia, inheritability

  Abstract

Two sisters with chronic progressive external ophthalmoplegia (CPEO) and their in all 7 healthy children were investigated. Both ophthalmoplegic patients had histopathological changes typical of mitochondrial myopathy. The same type of muscular pathology was also found among the healthy children. The most common muscular changes were subsarcolemmal accumulation of pathological mitochondria, including vacuoles, abnormal cristae and sometimes also inclusion bodies. Biochemical studies showed partial complex III deficiency, with low succinate-cytochrome c reductase activity in 1 of the ophthalmoplegic patients. These findings suggest that CPEO is a slowly progressive muscle disease, starting early in life. The widespread occurrence among the children may indicate maternal inheritance.

Copyright © 1990 S. Karger AG, Basel

  Author Contacts

J. Aasly, MD, Department of Neurology, University Hospital, N-7006 Trondheim (Norway)

  Article Information

Received: April 6, 1989
Accepted: November 21, 1989
Published online: February 14, 2008
Number of Print Pages : 5