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Multifocal oscillatory potentials in CSNB1 and CSNB2 type congenital stationary night blindness

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We evaluated the function of the inner retina in patients with congenital stationary night blindness of the complete (CSNB1) and the incomplete type (CSNB2) by recording multifocal oscillatory potentials (mf-OPs). The VERIS system was used to record mf-OPs from 61 areas of the central retina from 5 CSNB1 patients (4 with NYX gene mutation), 6 CSNB2 patients (2 with CACNA1F mutation) and 11 control subjects. For each subject group, the first- and second-order kernel responses for one eye were analysed and the amplitudes and implicit times of their major components compared to 5 concentric rings centred on the fovea. In CSNB1 patients, the mf-OP peak amplitudes of the first-order kernel responses showed a significant reduction of the first peak without significant reduction of the second, whereas in CSNB2 both peak amplitudes were barely discernable from noise for all eccentricities. In the second-order kernel, the third peak was reduced in CSNB1 patients, and again not discernable from noise in CSNB2 patients. The difference in amplitude between the control and CSNB1 groups was significant for the late components of the first- and the second-order kernel. Implicit times were not significantly altered. The difference in mf-OP amplitude between CSNB1 and CSNB2 patients reflects the different molecular mechanisms underlying the two types of disease, which differentially affect the postreceptoral pathways of cone signal processing. The well-preserved peak 2 amplitudes of first-order mf-OPs and peak 3 amplitudes of second-order mf-OPs in CSNB1 patients point to a major impact of OFF-pathway components on these responses which are not present in CSNB2 patients. In conclusion, our results show that CSNB1 and CSNB2 are two different types of disease, not only on a genetic but also on a pathophysiological level.
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Document Type: Research Article

Affiliations: Department of Pathophysiology of Vision and Neuroophthalmology, University Eye Hospital, Tübingen, Germany

Publication date: January 1, 2005

More about this publication?
  • The International Journal of Molecular Medicine is a monthly, peer-reviewed journal devoted to the publication of high quality studies related to the molecular mechanisms of human disease. The journal welcomes research on all aspects of molecular and clinical research, ranging from biochemistry to immunology, pathology, genetics, human genomics, microbiology, molecular pathogenesis, molecular cardiology, molecular surgery and molecular psychology.

    The International Journal of Molecular Medicine aims to provide an insight for researchers within the community in regard to developing molecular tools and identifying molecular targets for the diagnosis and treatment of a diverse number of human diseases.
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