Abstract Details

Title Retinal Neurodegeneration in Wilson's Disease Revealed by Spectral Domain Optical Coherence Tomography

Topic Neuro-ophthalmology/Neuro-otology

Presentation(s) P06 - (-)

Poster/Presentation
Number 008

Objective

Background BACKGROUND: In addition to cirrhosis of the liver, Wilson's disease leads to copper accumulation and widespread degeneration of the nervous system. Delayed visual evoked potentials (VEPs) suggest changes to the visual system and potential structural changes of the retina.

Design/Methods DESIGN/METHODS: We used the latest generation of spectral domain optical coherence tomography to assess the retinal morphology of 42 patients with Wilson's disease and 76 age- and sex-matched controls. We measured peripapillary retinal nerve fiber layer (RNFL) thickness and total macular thickness and manually segmented all retinal layers in foveal scans of 42 patients with Wilson's disease and 76 age- and sex-matched controls. The results were compared with VEPs and clinical parameters.

Results RESULTS: The mean thickness of the RNFL, paramacular region, retinal ganglion cell/inner plexiform layer and inner nuclear layer was reduced in Wilson's disease. VEPs were altered with delayed N75 and P100 latencies, but the N140 latency and amplitude was unchanged. An analysis of the laboratory parameters indicated that the serum concentrations of copper and caeruloplasmin positively correlated with the thickness of the outer plexiform layer and with N75 and P100 VEP latencies.

Conclusions CONCLUSIONS: Neuronal degeneration in Wilson's disease involves the retina and changes can be quantified by optical coherence tomography. While the VEPs and the thickness of the outer plexiform layer appear to reflect the current copper metabolism, the thicknesses of the RNFL, ganglion cell/inner plexiform layer, inner nuclear layer and the total paramacular thickness may be the best indicators of chronic neuronal degeneration.

Authors/Disclosures
Philipp Albrecht, PhD (Neurologische Universitatsklinik Dusseldorf) PRESENTER	Dr. Albrecht has received personal compensation for serving as an employee of Abbvie. Dr. Albrecht has received personal compensation for serving as an employee of Biogen. Dr. Albrecht has received personal compensation for serving as an employee of Bristol Myers Squibb. Dr. Albrecht has received personal compensation for serving as an employee of Ipsen. Dr. Albrecht has received personal compensation for serving as an employee of Merck. Dr. Albrecht has received personal compensation for serving as an employee of Merz. Dr. Albrecht has received personal compensation for serving as an employee of Novartis. Dr. Albrecht has received personal compensation for serving as an employee of Roche. Dr. Albrecht has received personal compensation for serving as an employee of Sanofi. Dr. Albrecht has received personal compensation for serving as an employee of Lilly.
Carlos Silvado, MD (Universidade Federal do Parana)	No disclosure on file
	No disclosure on file
Marius Ringelstein	Marius Ringelstein has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Roche. Marius Ringelstein has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Alexion.
	No disclosure on file
Orhan Aktas, MD (Neurologische Klinik)	Dr. Aktas has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Alexion. Dr. Aktas has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Amgen/Horizon. Dr. Aktas has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Roche. Dr. Aktas has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Mitsubish Tanake. Dr. Aktas has received personal compensation in the range of $500-$4,999 for serving as a Consultant for UCB. Dr. Aktas has received personal compensation in the range of $500-$4,999 for serving as a Consultant for AstraZeneca. Dr. Aktas has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Sanofi. Dr. Aktas has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Alexion. Dr. Aktas has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Roche. Dr. Aktas has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Amgen/Horizon. The institution of Dr. Aktas has received research support from German Research Foundation. The institution of Dr. Aktas has received research support from German Ministry of Science. The institution of Dr. Aktas has received research support from Neuromyelitis optica Study Group (NEMOS).
Hans-Peter Hartung, MD, FAAN (Heinrich Heine University Medical Faculty)	Dr. Hartung has received personal compensation in the range of $500-$4,999 for serving as a Consultant for BMS Celgene. Dr. Hartung has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Geneuro. Dr. Hartung has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Novartis. Dr. Hartung has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Novartis. Dr. Hartung has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Merck. Dr. Hartung has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Bayer. Dr. Hartung has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for TG Therapeutics. Dr. Hartung has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Roche. Dr. Hartung has received personal compensation in the range of $500-$4,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for Frontiers Neurology.
	No disclosure on file
Axel Methner, MD (University Hospital Duesseldorf)	No disclosure on file

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