Abstract

P 190

Electroretinography and Electrooculography in patients with pseudoxanthoma elasticum

Katharina Lammersdorf1, Robert P. Finger1, Peter Charbel Issa1, Frank G. Holz2, Hendrik P. Scholl1
1Universitäts-Augenklinik Bonn, 2GRADE Reading Center, Universitäts-Augenklinik Bonn

Objective
Patients with pseudoxanthoma elasticum (PXE) exhibit characteristic findings at the ocular fundus such as angioid streaks, peau d’orange or salmon spots. Besides these focal pathologies, panretinal alterations have been suggested by recent studies using fundus autofluorescence and electroretinography (ERG). To detect panretinal functional loss we performed a comprehensive electrophysiological study in a large PXE patient cohort. 
Methods
17 PXE patients were examined by ERG in accordance with ISCEV (International Society for Clinical Electrophysiology of Vision) – standards. Furthermore, electrooculography (EOG) was performed in 12 of those patients.
Results
ERG showed reduced scotopic and photopic responses in 11 eyes of 6 patients. In further 16 eyes there were reduced amplitudes in individual recordings. Only in 7 eyes of 5 patients, photopic and scotopic responses were normal. Arden ratio was <1.5 (abnormally low) in 2 patients, 1.5-2.0 (borderline) in 8 patients and >2.0 (normal) in only 1 patient.
Conclusions
The ERG and EOG abnormalities in patients with PXE suggest a diffuse dysfunction of the retina and pigment epithelium, respectively. Histological studies localized the primary pathology to Bruch’s membrane which could lead to a generalized alteration of the pigment epithelium and subsequently of the neurosensory retina.

Copyright: 2009 Porstmann Kongresse GmbH. All rights reserved. Impressum, Disclaimer.