Stargardt Hastalığının Elektrofizyolojik Özellikleri ve Bir Olguda Progresyonun Optik Koherens Tomografi, Elektroretinografi ve Elektrookülografi ile Araştırılması.

Purpose: Investigation the properties and progression in Stargardt's disease. Material and Methods: The electrophysiological features of fundus flavimaculatus (FFM) in 18 cases and fundus flavimaculatus with Stargardt's disease (FFM+STGD) in 5 cases were revisited and the progression in a young patient with STGD was investigated using optical coherence tomography (OCT), electroretinography (ERG) and electrooculography (EOG). Results: EOG Arden index (EOG AI) and rod ERG amplitudes did not differ significantly between FFM and FFM+STGD (t=1.02 and p>0.05). There was a difference in cone ERG amplitudes at the level of 1% probability and was significantly decreased in FFM+STGD group (t=5.03 and p<0.01). There was a significant difference between the two groups in visually evoked cortical response latencies (t=5.56 and p<0.01) and in amplitudes (t=4.84 and p<0.01). In Stargardt's case, all ERG parameters worsened to pathological levels from subnormal values and EOG AI to subnormal-abnormal ratios from normal ratios within a three year period. Subtle changes in optical coherens tomography (OCT) were noted during this period. Conclusion: STGD may be seen in variable appearances. In FFM which is accepted as the variant of STGD, the lesions are located peripherally and the disease manifests itself in advanced ages. The electrophysiological parameters are less affected in FFM. The gross changes in ERG and EOG findings within time whereas subtle changes occurring in OCT implies that ERG and EOG can be preferred in the detection of progression. [ABSTRACT FROM AUTHOR]