Mimura Tatsuya, Amano Shiro, Fukuoka Shima, Honda Norihiko, Arita Reiko, Ochiai Makiko, Yanagisawa Mieko, Usui Tomohiko, Ono Kyoko, Araki Fumiyuki, Yamagami Satoru, Araie Makoto, Awaya Yutaka
Department of Ophthalmology, University of Tokyo Graduate School of Medicine, Bunkyo-ku, Tokyo, Japan.
Curr Eye Res. 2008 Nov;33(11):940-5. doi: 10.1080/02713680802450992.
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.
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.
The superficial epithelial cell density was lower in the HSAN patients compared with the healthy subjects (1525, 1225, and 1250/mm(2) vs. 2225, 1750, and 2500/mm(2)), 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/mm(2) vs. 1.47, 1.47, and 1.47 g/mm(2)).
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.
通过活体共聚焦显微镜观察Ⅳ型或Ⅴ型遗传性感觉和自主神经病变（HSAN）患者的角膜细胞和角膜神经纤维形态，阐明该疾病导致角膜感觉丧失的机制。
对3例HSAN患者（年龄分别为17、20和32岁）右眼中央角膜进行活体共聚焦显微镜检查，并将其角膜形态与3例健康受试者（年龄分别为28、30和36岁）进行比较。用Cochet-Bonnet感觉计检测角膜感觉。
与健康受试者相比，HSAN患者的浅表上皮细胞密度较低（分别为1525、1225和1250/mm²，而健康受试者为2225、1750和2500/mm²），但患者的基底上皮细胞密度与健康受试者相似。在健康受试者角膜的基底神经丛下层可清晰观察到神经束，但在HSAN患者的中央角膜未检测到。HSAN患者的角膜感觉比健康受试者弱得多（分别为2.79、40.30和132.50 g/mm²，而健康受试者为1.47、1.47和1.47 g/mm²）。
临床观察到的伴有神经营养性角膜病变和泪膜不稳定的浅表角膜病变与这些患者活体共聚焦显微镜下浅表角膜上皮中的大角质化细胞相符。我们的研究结果表明，角膜神经的丧失导致Ⅳ型或Ⅴ型HSAN患者角膜感觉受损。
