Differential diagnosis of congenital tritanopia and dominantly inherited juvenile optic atrophy
Y. Miyake, K. Yagasaki and H. Ichikawa
To determine whether congenital tritanopia and dominantly inherited
juvenile optic atrophy (DIJOA) are the same clinical entity, we used
electroretinograms of the blue-sensitive cone system (blue cone ERGs),
comparing those of two patients with congenital tritanopia from two
pedigrees with those of four patients with DIJOA from two pedigrees. The
examinations also included visual acuity and visual field tests, fundus
examination, the dark-adaptation test, and several color vision tests. The
blue cone ERG confirmed a difference between the two groups; it was
unrecordable in the patients with congenital tritanopia but within the
normal range in those with DIJOA. We believe that congenital tritanopia and
DIJOA are distinct disease entities and that the blue cone ERG is a key
factor in the differential diagnosis.