Question
| A. | Stargardt disease |
| B. |
Best disease |
| C. |
Fundus flavimaculatus |
| D. |
Sticklers Syndrome |
|
Correct Answer � B Explanation |
 |
Answer B) Best disease
· The picture shows Best disease with classical egg yolk appearance at macula.
· Inheritance is autosomal dominant with variable penetrance and expressivity.
The five stages of Best’s disease are:
• Stage 1—pre vitelliform (normal fundus but abnormal EOG)
• Stage 2—vitelliform (egg-yolk stage) as shown in this case
• Stage 3—pseudohypopyon (part of lesion becomes absorbed)
• Stage 4—vitelliruptive (scrambled-egg)
• Stage 5—end stage is characterized by one of the following: Hypertrophic macular scar, Atrophic macular scar, Vascularized fibrous macular scar.
· Visual acuity is usually no worse than 6/60.
· EOG is severely subnormal during all stages and also abnormal in carriers with normal fundi