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Long-term follow-up in Bietti crystalline dystrophy
A M Mansour1, S H Uwaydat, C-C Chan
1Department of Ophthalmology, American University of Beirut, Beirut, Lebanon. dr.ahmad@cyberia.net.lb
European Journal of Ophthalmology
|August 3, 2007
Summary
This long-term study of Bietti crystalline dystrophy shows progressive retinal deposits replaced by atrophy, leading to worsening vision. Lipid crystals were found in various tissues.
Area of Science:
- Ophthalmology
- Medical Genetics
- Retinal Diseases
Background:
- Bietti crystalline dystrophy is a rare inherited disorder affecting the retina.
- Understanding its long-term progression is crucial for patient management.
Observation:
- Long-term follow-up of two brothers with Bietti crystalline dystrophy over 5 and 25 years.
- Comprehensive evaluation included clinical examination, imaging (fluorescein angiography, OCT), electrophysiology (ERG, EOG), and electron microscopy.
Findings:
- Progressive de novo retinal crystal formation, eventually replaced by choroidal atrophy.
- Gradual decline in electrooculography (EOG), electroretinography (ERG), and visual fields.
- Optical coherence tomography (OCT) revealed inner retinal crystal accumulation.
- Transmission electron microscopy identified cytoplasmic lipid crystalline inclusions in lymphocytes and conjunctival fibroblasts.
Implications:
- Bietti crystalline retinopathy is a progressive condition.
- Characterized by retinal crystals, RPE atrophy, and visual field constriction.
- Highlights the systemic nature of lipid crystal deposition.
