Progression of myopathology in Kearns-Sayre syndrome: a morphological follow-up study.
We report on the progression of myopathology by comparing two biopsies from a patient with a Kearns-Sayre-Syndrome. The first biopsy was taken in 1979 and showed 10% ragged-red fibers. Myopathic changes were slight including internal nuclei and fiber splitting in 10% of the fibers. Electron microscopy revealed typical mitochondrial abnormalities with regard to number and shape. In 1989 a second biopsy was performed for an extended analysis of mitochondrial DNA. This time less than 5% of all fibers were ragged-red. Severe myopathic changes could be detected which so far has rarely been reported in mitochondrial cytopathy.
Department of Neurology, University of Würzburg, Germany.
H
H
Reichmann
R
R
Gold
B
B
Meurers
M
M
Naumann
P
P
Seibel
U
U
Walter
T
T
Klopstock
D015139:Blotting, Southern; D030401:Cytochrome-c Oxidase Deficiency; D005500:Follow-Up Studies; D006801:Humans; D007150:Immunohistochemistry; D007625:Kearns-Sayre Syndrome / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D008931:Mitochondria, Muscle / Q000648:ultrastructure; D009132:Muscles / Q000473:pathology*