Synonyms: Cone dystrophy with supernormal rod ERG | Cone dystrophy with supernormal rod electroretinogram | Cone dystrophy with supernormal scotopic electroretinogram
Cone dystrophy with supernormal rod response (CDSRR) is an inherited retinopathy with an onset in the first or second decade of life characterized by poor visual acuity (due to central scotoma) photophobia severe dyschromatopsia and occasionally nystagmus. Night blindness usually develops later in the course of the disease but it can also be apparent from childhood. A hallmark of CDSRR is the decreased and delayed dark-adapted response to dim flashes in electroretinographic recordings which contrasts with the supernormal b-wave response at the highest levels of stimulation.
Data from Orphanet are used to provide information on a disease's name, synonym(s), and overview. Reference: Access aggregated data from Orphanet at Orphadata. Orphadata: Free access data from Orphanet. © INSERM 1999. Available on http://www.orphadata.org. Data version September 2024
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Cone dystrophy with supernormal rod response?
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