Table 1 of Kloeckener-Gruissem, Mol Vis 2006; 12:350-355.

Table 1. Clinical features in Wagner syndrome

Patients of the original Wagner syndrome family were assigned to the older or younger group based on the age of approximately 30 years. This age limit should be considered of gliding nature. The division into hallmark and additional features reflects our hypothesis that hallmark features represent the primary defect while additional features may be of secondary, consequential nature. More detailed information about the clinical observations can be found in the literature [4,6].

                                                      Observation in              Observations in
 Features            Ocular symptoms                 younger patients             older patients
----------   -------------------------------   -----------------------------   ---------------------
Hallmark     Empty vitreous with fibrillary    Frequent                        Not observed anymore

             Avascular strands and veils       Frequent                        Almost always

Additional   Chorioretinal atrophy             Frequent                        Always present

             Rhegmatogenous retinal            Rare                            Not observed

             Peripheral tractional retinal     Rare                            Common

             Cataracts                         Frequent                        Does not apply

             Visual acuity                     Normal to subnormal             Severely affected

             Refractive error                  Frequent mild to moderate       Does not apply

             Visual field                      Minor defects                   Marked field loss

             Abnormal pattern of the central   Rare                            Frequent
             retinal vessels

             Optic atrophy                     Not observed                    Frequent if advanced
                                                                               chorioretinal atrophy

             Electroretinography (ERG) dark    Frequently pathologic rod       Progressively
             adaptation                        threshold frequently elevated   pathologic

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