Table 1

Clinical features of affected patients

PatientAgeVisionElectroretinography (% amp)Goldmann visual field
Right eyeLeft eyePhotopicScotopic
 II-16720/20020/3003050Central scotoma
 II-26420/20020/1501540Central scotoma
 II-35720/15020/15050100Central scotoma
 II-66220/40020/20000Central scotoma and peripheral constriction
 III-14020/7020/10030100Central scotoma
 III-24220/15020/150Central scotoma and peripheral constriction
 IV-11520/4020/4080100Small central scotoma
  IV-21820/2020/20100100Small central scotoma
  IV-51220/3020/25Mid-peripheral scotoma
Embedded Image
  • Pedigree demonstrates autosomal-dominant inheritance pattern.