선천비진행야맹증의 분류와 다국소망막전위도 소견

The Classification & Multifocal ERG Findings of Congenital Stationary Night Blindness

Purpose: To report on the classification of congenital stationary night blindness (CSNB) and multifocal ERG findings. Methods: We studied three patients (case 1-3) with night blindness but normal fundus and one patient (case 4) with decreased vision after trauma with respect to the patients` medical history, familial history, refraction, slit lamp examination, fundus examination, FAG, ERG, and multifocal ERG. Results: The ERG in case 1 showed a decreased rod response, and this patient was diagnosed with Riggs-type CSNB. The typical “negative ERG” was found in all three cases of ERG (except case 1), and these cases were diagnosed with Schubert-Bornschein-type CSNB. Based on Miyake`s classification, case 2 was incomplete, and cases 3 and 4 were complete. The mfERG of the cases with complete Schubert-Bornschein type had delayed implicit times of the first-order kernel and decreased amplitudes of the second-order kernel. The delayed implicit times and the decreased amplitudes of the first- and second-order kernels were found in the cases of Riggs- type and incomplete Schubert-Bornschein-type.