GRANULAR-LATTICE (AVELLINO) CORNEAL-DYSTROPHY IN JAPANESE PATIENTS

Purpose. To determine amyloid deposition in the corneas of granular co rneal dystrophy in Japanese patients. Methods. Eight Japanese patients (10 eyes) with a clinical diagnosis of granular corneal dystrophy wer e investigated clinically and histologically. Each specimen obtained a t surgery was stained with hematoxylin-eosin, Masson trichrome or Mall ory, and Congo red stain. Amyloid deposit was identified by birefringe nce and dichroism under cross-polarized light after staining with Cong o red. Results. Seven (70%) of the 10 corneal buttons (six of eight pa tients) had amyloid deposits, as shown by Congo red staining with bire fringence and dichroism. Of the six amyloid-positive patients, two pat ients (who were siblings) showed discrete gray-white corneal deposits with additional Linear deposits. This finding is typical of Avellino c orneal dystrophy. The corneas of the remaining four patients showed th e discrete deposits typical of granular dystrophy. Some of them showed a few whitish fusiform and stellate opacities in the mid stroma, sugg estive of Avellino corneal dystrophy. Conclusion, The high frequency o f amyloid deposits in Japanese patients with granular corneal dystroph y may be caused by an allelic heterogeneity of the gene.