Figure 6.

Results of the examinations for patients 2IIc and 2Ib in family 2. A: In patient 2IIc, optical coherence tomography (OCT) showed attenuated retina in the innermost five segments of the macular map even before visual acuity (VA) was reduced. B: Moreover, intraretinal fluid was found in the center of the macula. C: The amplitudes of the multifocal electroretinography (mERG) were reduced especially in the center compared to the normal condition (Figure 5I), but implicit times were normal. D: Only subtle unspecific changes with discrete irregular macular pigmentation were found in the fundus. E: OCT of the father in family 2, patient 2Ib, also showed attenuated retina in the five innermost segments of the macular map although the macular contour was normal (F). G: The mERG showed reduced paracentral amplitudes. Implicit times were normal. H: This patient had previously, at another clinic, been diagnosed with age-related macular degeneration due to yellow flecks around the macula.