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Visual pathway abnormalities Wilson's disease: an electrophysiological study using electroretinography and visual
P Satishchandra1, K Ravishankar Naik
1Department of Neurology, National Institute of Mental Health and Neuro Sciences (NIMHANS), 560 029, Bangalore, India. psatish@nimhans.kar.nio.in
Journal of the Neurological Sciences
|June 24, 2000
Summary
This study reveals that Wilson's disease causes visual pathway abnormalities, measurable by pattern reversal visual evoked potential (PRVEP) and electroretinography (ERG). Treatment improved these visual electrophysiological measures, indicating reversible retinal dysfunction.
Area of Science:
- Ophthalmology
- Neuroscience
- Genetics
Background:
- Wilson's disease (WD) is a genetic disorder of copper metabolism.
- Abnormalities in pattern reversal visual evoked potential (PRVEP) are reported in WD patients.
- The specific pathogenesis and retinal contribution to PRVEP abnormalities in WD remain unclear.
Purpose of the Study:
- To investigate the pathogenesis of PRVEP abnormalities in Wilson's disease.
- To localize PRVEP abnormalities using electroretinography (ERG).
- To evaluate the effect of specific therapy on visual electrophysiological parameters in WD patients.
Main Methods:
- Ten newly diagnosed WD patients underwent PRVEP and flash ERG.
- Visual electrophysiological tests were repeated after clinical improvement with therapy in six patients.
- Data were compared with control groups and pre- vs. post-treatment.
Main Results:
- WD patients exhibited prolonged PRVEP latencies and abnormal ERG findings (prolonged A and oscillatory potentials, reduced A and B wave amplitudes).
- Post-treatment studies showed significant reductions in PRVEP and ERG A-wave latencies.
- Significant increases in PRVEP P100 and ERG A and B wave amplitudes were observed post-treatment.
Conclusions:
- Retinal dysfunction partially contributes to PRVEP abnormalities in Wilson's disease.
- Visual electrophysiological abnormalities in WD are partially reversible with specific treatment.
- This study is the first to report ERG findings in WD and assess treatment effects on PRVEP and ERG.