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Electron-dense lipidic capillary deposits in Rett syndrome
R Dieler1, J M Schröder, K Reddemann
1Institut für Neuropathologie, Klinikum der Rheinisch-Westfälischen Technischen Hochschule, Aachen, Federal Republic of Germany.
Acta Neuropathologica
|January 1, 1990
Summary
New research reveals previously unreported electron-dense deposits in capillaries of a Rett syndrome patient. These findings suggest a potential lipidic plasma component and blood-tissue barrier impairment in Rett syndrome.
Area of Science:
- Neurology
- Pathology
- Microscopy
Background:
- Rett syndrome is a rare neurodevelopmental disorder.
- Understanding the underlying pathology is crucial for developing effective treatments.
Observation:
- Biopsies from conjunctiva, muscle, and peripheral arteries of a Rett syndrome patient were analyzed using light and electron microscopy.
- Specific electron-dense deposits were identified within capillary endothelial cells and pericytes.
Findings:
- Amorphous and membranous electron-dense deposits were observed in capillary lumens, endothelial cell junctions, and abluminal sites.
- These deposits were found within endothelial cells and pericytes, sometimes within multivesicular bodies.
- The observed deposits were unique to the Rett syndrome patient and not present in control groups.
Implications:
- The findings suggest the presence of an unknown lipidic plasma component in Rett syndrome.
- These deposits may indicate an impairment of the blood-tissue barrier.
- Further research is needed to elucidate the nature of these deposits and their role in Rett syndrome pathogenesis.