Age of
  Person   Age   onset           Visual acuity                       Other clinical information
  ------   ---   ------   ----------------------------   --------------------------------------------------

Mutation Arg677X (Figure 1)

  III:1    58    34       Age 48: 6/24                   Diffuse RP with macular edema, rapid progression
                          Age 53: Hand motions           with the onset of macular degeneration, cataracts,
                                                         waxy pallor of discs, attenuated vessels,
                                                         extensive distribution of bone spicule in retinal
                                                         periphery, vitreal floaters.

   IV:1    31    Early    Age 22: 6/6                    Diffuse RP
                 20s      Age 27: 6/7.5

Mutation Ser911X (Figure 2)

  III:2    69    About    Age 68: 6/60 OU, which         Early onset of nyctalopia, diffuse RP, cystoid
                 40       improved with Diamox to 6/21   macular edema, mild nuclear sclerosis, pale and
                          OS after 6 months              waxy discs, bone spicule scattered peripherally in
                                                         the retina, floaters and asteroid hyalosis of the
                                                         vitreous.

   IV:4    43    19                                      Nyctalopia began at age 19, followed by loss of
                                                         visual fields in early 20s.

   IV:5    40                                            No apparent manifestation

   IV:7    34                                            No apparent manifestation

Mutation 2590-2599del ATAACTTTAA (Figure 3)

  III:4          30s                                     Nyctalopia began at age 48. Deceased


   IV:2    71    17                                      Nyctalopia began at age 17, by the age of 40
                                                         peripheral vision was very poor, hyperopia at age
                                                         60.

    V:1    51    Early    Age 50: 6/15 OD, 6/9 OS        Diffuse RP with slow progression, cataracts, waxy
                 teens                                   pallor of the discs, attenuated vessels,
                                                         granularity of the macula, bleakish fovea, bone
                                                         spicule and white dots on retina, no cellularity
                                                         or vitreous floaters.

   VI:2    28                                            No apparent manifestation. Retinoblastoma in one
                                                         eye at 6 months of age.

   VI:4    26     24                                     Nyctalopia began in 20s, diffuse RP with slow
                                                         progression, waxy pallor of discs, attenuated
                                                         vessels, normal macula and fovea, occasional and
                                                         diffuse pigment in the periphery of the retina,
                                                         bone spicule, normal vitreous.