[Electron microscopical findings in the sural nerve of twins with metachromatic leukodystrophy. An attempt to classify the findings according to distinctive phases of the disease (author's transl)].
Sural nerve biopsies taken from a pair of twins with metachromatic leukodystrophy were investigated by electron microscopy. The morphological findings showed distinct alterations. In the first twin the particularity was a large number of granules containing sulfatides. In the second twin, however, the main finding was a damage of myelin sheaths evidently caused by an excessive and morphologically as well as chemically defective myelin formation. According to the grading given by Ulrich the first case corresponds to the stadium one, in which a storage of sulfatides dominates, whereas case two showing a severe damage of myelin sheaths is characteristic of the second stadium of metachromatic leukodystrophy.
D001706:Biopsy; D004200:Diseases in Twins; D006801:Humans; D007223:Infant; D007966:Leukodystrophy, Metachromatic / Q000145:classification / Q000235:genetics / Q000473:pathology*; D008297:Male; D008854:Microscopy, Electron; D009186:Myelin Sheath; D013127:Spinal Nerves / Q000473:pathology*; D013433:Sulfoglycosphingolipids / Q000032:analysis; D013497:Sural Nerve / Q000473:pathology*
D013433:Sulfoglycosphingolipids
Elektronenmikroskopische Befunde am Nervus suralis von Zwillingskindern mit metachromatischer Leukodystrophie. Versuch einer Zuordnung zu unterschiedlichen Erkrankungsstadien