Retinal Neurodegeneration in Wilson’s Disease Revealed by Spectral Domain Optical Coherence Tomography 英文参考文献.docVIP

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Retinal Neurodegeneration in Wilson’s Disease Revealed by Spectral Domain Optical Coherence Tomography 英文参考文献.doc

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Retinal Neurodegeneration in Wilson’s Disease Revealed by Spectral Domain Optical Coherence Tomography 英文参考文献

RetinalNeurodegenerationinWilson’sDiseaseRevealed bySpectralDomainOpticalCoherenceTomography PhilippAlbrecht1*,Ann-KristinMu¨ller1,MariusRingelstein1,DavidFinis2,GerdGeerling2,EvaCohn1, OrhanAktas1,Hans-PeterHartung1,HaraldHefter1.,AxelMethner1. 1Department of Neurology, Medical Faculty, Heinrich-Heine University, Du¨sseldorf, Germany, 2Department of Ophthalmology, Medical Faculty, Heinrich-Heine University,Du¨sseldorf,Germany Abstract Background/Objective:Inadditiontocirrhosisoftheliver,Wilson’sdiseaseleadstocopperaccumulationandwidespread degeneration of the nervous system. Delayed visual evoked potentials (VEPs) suggest changes to the visual system and potentialstructuralchangesoftheretina. Methods: We used the latest generation of spectral domain optical coherence tomography to assess the retinal morphologyof42patientswithWilson’sdiseaseand76age-andsex-matchedcontrols.Wemeasuredperipapillaryretinal nervefiberlayer(RNFL)thicknessandtotalmacularthicknessandmanuallysegmentedallretinallayersinfovealscansof42 patients with Wilson’s disease and 76 age- and sex-matched controls. The results were compared with VEPs andclinical parameters. Results:ThemeanthicknessoftheRNFL,paramacularregion,retinalganglioncell/innerplexiformlayerandinnernuclear layerwasreducedinWilson’sdisease.VEPswerealteredwithdelayedN75andP100latencies,buttheN140latencyand amplitudewasunchanged.Ananalysisofthelaboratoryparametersindicatedthattheserumconcentrationsofcopperand caeruloplasminpositivelycorrelatedwiththethicknessoftheouterplexiformlayerandwithN75andP100VEPlatencies. Conclusion: Neuronal degeneration in Wilson’s disease involves the retina and changes can be quantified by optical coherencetomography.WhiletheVEPsandthethicknessoftheouterplexiformlayerappeartoreflectthecurrentcopper metabolism,thethicknessesoftheRNFL,ganglioncell/innerplexiformlayer,innernuclearlayerandthetotalparamacular thicknessmaybethebestindicatorsofchronicneuronaldegeneration. Citation: Albrecht P,Mu¨llerA-K,Ringelste