Table 1 of Zeitz, Mol Vis 2005; 11:179-183.


Table 1. Clinical data of the described patients

The clinical data of eight Dutch patients shows typical findings for patients with XLCSNB. Right eye (RE), left eye (LE), electroretinogram (ERG), light peak/dark trough (lp/dt), and not measured (NM) are abbreviated in the table. The retina of the left eye was ablated in patient 2417.

                                                                     Photopic        Electro-
                                Visual     Scotopic ERG RE/LE       ERG RE/LE       oculography
                       Age      acuity     ------------------    ----------------     (lp/dt)
                        at    ----------    a-wave     b-wave    a-wave    b-wave   -----------      Dark
Patient   Diagnosis    exam   RE     LE      [μV]       [μV]      [μV]      [μV]     RE     LE    adaptation   Nystagmus
-------   ----------   ----   ---   ----   --------   --------   -------   ------   ----   ----   ----------   ---------
 2417     CSNB,         27    0.8   0.3     30/ 25     60/70       0/100   60/70    1.75   1.94    -           Yes
          ablation
          LE
 2436     CSNB          34    0.3   0.3     90/ 80     20/25      60/ 50   35/40     -      -      Affected    Yes

 2445     CSNB,         19    0.4   0.4     50/ 70     50/75      35/ 55   60/70     -      -      Affected    Yes
          strabismus
 2446     CSNB          11    0.2   0.2    130/ 70     50/60      75/ 50   30/20     -      -      Normal      Yes

 2454     CSNB          11    0.2   0.2     15/ 15     50/60      45/ 45   50/50    2.24   2.2     Affected    Yes (L>R)
 2477     CSNB,         36    0.3   0.3    110/100      5/20     100/ 85   10/10    1.26   1.38    Affected    Yes
          tilted
          disc
 2490     CSNB          66                  20/ 20     90/90      50/ 50   30/40     -      -      -           Unknown

 2496     CSNB,          8    0.4   0.4     60/NM      40/NM      75/ 65   55/55     -      -      -           Yes
          strabismus

Zeitz, Mol Vis 2005; 11:179-183 <http://www.molvis.org/molvis/v11/a20/>
©2005 Molecular Vision <http://www.molvis.org/molvis/>
ISSN 1090-0535