J Korean Ophthalmol Soc.  2021 Mar;62(3):337-344. 10.3341/jkos.2021.62.3.337.

The Characteristics and the Clinical Courses of Best Disease in Korean Patients

• Lee JH
• Lew YJ
• Kim JH
• Kim JW
• Kim CG
• Lee DW

• KMID: 2514248
• DOI: http://doi.org/10.3341/jkos.2021.62.3.337

Abstract

Purpose
To evaluate the clinical characteristics and courses of Best disease in Korean patients.
Methods
We retrospectively reviewed the medical records of patients with Best disease followed-up for more than 12 months after diagnosis. The disease stage and best-corrected visual acuity (BCVA) at diagnosis, and stage progression during follow-up, were evaluated. The BCVA at diagnosis was compared to that at the final follow-up. The BCVA changes were compared between those evidencing stage progression and not.
Results
A total of 16 patients (32 eyes) were enrolled. The mean follow-up period was 52.1 ± 31.8 months. At diagnosis, three eyes had stage 1, nine stage 2, seven stage 3, 11 stage 4, and two stage 5 disease. During follow-up, stage progression was observed in nine eyes (28.1%). The mean logarithm of the minimal angle of resolution (logMAR) BCVA significantly worsened from 0.19 ± 0.19 at baseline to 0.30 ± 0.27 at the final follow-up (p = 0.004). In six eyes (18.8%), a deterioration of logMAR 0.2 or greater was noted. The extent of deterioration was greater in the nine eyes (0.25 ± 0.26) exhibiting stage progression than in the 24 eyes (0.05 ± 0.08) lacking such progression (p = 0.001).
Conclusions
The visual acuity of Best disease patients was relatively good at diagnosis. However, stage progression and visual deterioration were noted in some patients. Stage progression was associated with a poor visual prognosis.

Keyword

Best disease, Characteristics, Outcome, Stage progression, Vitelliform macular dystrophy