Electron-dense lipidic capillary deposits in Rett syndrome.
Conjunctival, muscle, and peripheral artery biopsies were examined by light and electron microscopy in a patient with clinically diagnosed Rett syndrome. In capillaries, amorphous and membranous electron-dense deposits were observed (a) endoluminally attached to endothelial cell membranes, (b) within junctional clefts of adjacent endothelial cells, and (c) at abluminal sites of endothelial cells. In addition, electron-dense deposits lay isolated or inside multivesicular bodies in endothelial cells and pericytes. These findings have not been reported before in Rett syndrome. They were not seen in age-matched normal or pathological control cases and are suggested to indicate deposition of an as yet unknown lipidic plasma component possibly in association with impairment of the blood-tissue barrier.
Dieler
R
R
Institut für Neuropathologie, Klinikum der Rheinisch-Westfälischen Technischen Hochschule, Aachen, Federal Republic of Germany.
Schröder
J M
JM
Reddemann
K
K
D001706:Biopsy; D002196:Capillaries / Q000378:metabolism / Q000473:pathology*; D002675:Child, Preschool; D005260:Female; D006801:Humans; D050356:Lipid Metabolism; D008854:Microscopy, Electron; D015518:Rett Syndrome / Q000378:metabolism / Q000473:pathology*