PURPOSE: To estimate retinal function in @entity6568 using the electroretinogram. METHODS: In this observational case series, the scotopic and photopic electroretinograms in three Japanese female @entity1 (case 1, 55 years old; case 2, 56 years old; case 3, 47 years old) who showed @entity989 but no @entity2687 were recorded. The rod and cone a-waves were analyzed by using the method described by Hood and Birch (1995, 1997). The parameters Rm(p3) (maximum a-wave amplitude) and S (sensitivity) were calculated. RESULTS: In case 1, the rod Rm(p3) was @entity735 . The rod S in the right eye was within the normal range, but that in the left eye was significantly reduced. Although the cone Rm(p3) was decreased, the cone S was within the normal range. In case 2, the rod and cone Rm(p3) was reduced, but the rod and cone S was within the normal range in both eyes. In case 3, the rod and cone Rm(p3) and S were within the normal range. CONCLUSIONS: Electroretinograms illustrated different disease stages, however, no eye with normal Rm(p3) and decreased S was found in rods and cones. In the early stages of this disease, decreased numbers of photoreceptors and/or outer segment shortening may be present while phototransduction remains normal. As the damage to the retina progresses, phototransduction becomes severely affected. Because reduced cone S was not observed in our cases, cones may be less involved than rods in this disease.

Rod and cone a-waves in three cases of XXXX .

@entity1 :: ('9606', 'Species') :: ['patients'] @entity6568 :: ('MESH:C535440', 'Disease') :: ['Bietti crystalline chorioretinal dystrophy'] @entity735 :: ('MESH:D005124', 'Disease') :: ['decreased in both eyes'] @entity2687 :: ('MESH:D003316', 'Disease') :: ['corneal deposits'] @entity989 :: ('MESH:D012164', 'Disease') :: ['bilateral crystalline retinal deposits']
@entity6568 :: (MESH:C535440,Disease) :: ['Bietti crystalline chorioretinal dystrophy']
