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Temporal aspects of the electroretinogram in cone-rod dystrophy

✍ Scribed by Masayuki Horiguchi; Yozo Miyake; Katsuya Yagasaki; Kazuo Ichikawa

Book ID
104648024
Publisher
Springer-Verlag
Year
1985
Tongue
English
Weight
360 KB
Volume
60
Category
Article
ISSN
0012-4486

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✦ Synopsis

Twelve patients with cone-rod dystrophy were studied in terms of the temporal aspects of electroretinography (ERG).

The peak time of scotopic b-wave was within normal limits in all patients, while the amplitude was reduced in nine patients. The normal peak time of the scotopic-b wave may help explain rod involvement with normal or only slightly elevated final rod thresholds of subjective dark adaptation.

The peak time of photopic b-wave and 30-Hz flicker response was normal in four patients and significantly delayed or nonrecordable in eight patients. Such variations of peak time in photopic and 30-Hz flicker ERG may reflect the stage of the disease or may be caused by the different hereditary mode.

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Large deletions of the KCNV2 gene are co
Large deletions of the KCNV2 gene are common in patients with cone dystrophy with supernormal rod response
✍ Bernd Wissinger; Simone Schaich; Britta Baumann; Michael Bonin; Herbert JΓ€gle; C πŸ“‚ Article πŸ“… 2011 πŸ› John Wiley and Sons 🌐 English βš– 437 KB πŸ‘ 1 views

Cone dystrophy with supernormal rod response (CDSRR) is considered to be a very rare autosomal recessive retinal disorder. CDSRR is associated with mutations in KCNV2, a gene that encodes a modulatory subunit (Kv8.2) of a voltage-gated potassium channel. In this study, we found that KCNV2 mutations