In VivoConfocal Microscopy of Hereditary Sensory and Autonomic Neuropathy
- 1 January 2008
- journal article
- research article
- Published by Taylor & Francis Ltd in Current Eye Research
- Vol. 33 (11-12), 940-945
- https://doi.org/10.1080/02713680802450992
Abstract
Purpose: To observe the morphology of the corneal cells and corneal nerve fibers in patients with type IV or V hereditary sensory and autonomic neuropathy (HSAN) by in vivo confocal microscopy and elucidate the mechanism leading to the loss of corneal sensation in this disease. Methods: In vivo confocal microscopy was performed on the central cornea of the right eye in 3 patients with HSAN (ages 17, 20, and 32 years), and their corneal morphology was compared with that of 3 healthy subjects (ages 28, 30, and 36 years). Corneal sensation was tested with a Cochet-Bonnet esthesiometer. Results: The superficial epithelial cell density was lower in the HSAN patients compared with the healthy subjects (1525, 1225, and 1250/mm2 vs. 2225, 1750, and 2500/mm2), but the basal epithelial cell density of the patients was similar to that of the healthy subjects. Nerve bundles were clearly observed in the sub-basal nerve plexus layer of the cornea in the healthy subjects, but were undetectable at the central cornea in the patients with HSAN. The corneal sensation of the patients with HSAN was much weaker than that of the healthy subjects (2.79, 40.30, and 132.50 g/mm2 vs. 1.47, 1.47, and 1.47 g/mm2). Conclusions: Superficial keratopathy accompanied with neurotrophic keratopathy and tear film instability observed clinically agrees with the large keratinized cells in the superficial corneal epithelium by in vivo confocal microscopy in these patients. Our findings suggest that the loss of corneal nerves contributes to impairment of corneal sensation in patients with type IV or V HSAN.Keywords
This publication has 30 references indexed in Scilit:
- Measurement of Light Transmission of Human Limbal Epithelial Cells Cultured on Human Amniotic MembranesCornea, 2007
- White Light Confocal Microscopy of Preserved Human Corneas From an Eye BankCornea, 2007
- Preoperative Evaluation of Cultured Human Corneal Limbal Epithelium on Amniotic Membrane by Confocal MicroscopyCurrent Eye Research, 2007
- Laser Scanning In Vivo Confocal Microscopy of the Normal Human Corneoscleral LimbusInvestigative Ophthalmology & Visual Science, 2006
- Ocular Manifestations of Congenital Insensitivity to Pain With AnhidrosisAmerican Journal of Ophthalmology, 2006
- The anaesthetic management of patients with congenital insensitivity to pain with anhidrosisPediatric Anesthesia, 2004
- Corneal Innervation and Morphology in Primary Sjögren’s SyndromeInvestigative Ophthalmology & Visual Science, 2003
- Multisystem Involvement in Congenital Insensitivity to Pain with Anhidrosis (CIPA), a Nerve Growth Factor Receptor(Trk A)-Related DisorderNeuropediatrics, 2000
- Congenital insensitivity to pain with anhidrosis (hereditary sensory and autonomic neuropathy type IV)Pediatric Neurology, 1994
- Anesthetic Management of Congenital Insensitivity to Pain with AnhydrosisAnesthesiology, 1985