Medical Retina:  Case four

 Special thanks to Mr. Paul Parker, Chief Photographer of the Eye Hospital, Radcliffe Infirmary, Woodstock Road, Oxford for taking these pictures

Figure 1

Figure 2

 

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This 48 year old woman has a ten year history of deteriorating vision in both eyes. Her visual acuity was 6/9 in the right eye and 6/18 in the left. Her mother and one of her brothers also suffer from poor vision from the age of 40. The above pictures were taken in one of her clinic visits.

a. What is the most likely diagnosis?

b. How is this condition transmitted?

c. What would you expect to find in the electrophysiology tests?

d. What is the visual prognosis for patients with this condition?