title: Selective reduction of S-cone response and on-response in the cone electroretinograms of patients with X-linked retinoschisis.
abstract: OBJECTIVE
  To examine the electroretinograms (ERGs) of the short-wavelength-sensitive (S-) and the mixed long- and middle-wavelength-sensitive (L,M-) cones, and the ON- and OFF-responses of the cone ERGs in three patients with X-linked juvenile retinoschisis (XLRS).
  
  
  METHODS
  Cone ERGs elicited by different color flashes and those elicited by long duration stimuli under Ganzfeld conditions were recorded from three patients with XLRS.
  
  
  RESULTS
  The S-cone b-waves were undetectable to short-wavelength stimuli in all three XLRS patients, while the L,M-cone ERG b-waves were within the normal range. To long-duration white stimuli, the ON-response (b-wave) was reduced and delayed in all patients compared with that of the normal subjects, while the d-wave or OFF-response appeared normal in amplitude and implicit time.
  
  
  CONCLUSIONS
  These results support the hypothesis that the normal S-cone ERG arises primarily from the ON-pathway of the cone ERGs and the hypothesis that ON-bipolar cells are predominant in the S-cone system.
authors.affiliation: Department of Ophthalmology, Toho University Sakura Hospital, 564-1 Shimoshizu, Sakura, Chiba 285-8741, Japan. shuyama@med.toho-u.ac.jp
authors.forename: Shuichi
authors.initials: S
authors.lastname: Yamamoto
authors.forename: Masanori
authors.initials: M
authors.lastname: Hayashi
authors.forename: Mieko
authors.initials: M
authors.lastname: Tsuruoka
authors.forename: Kazuha
authors.initials: K
authors.lastname: Ogata
authors.forename: Itsuro
authors.initials: I
authors.lastname: Tsukahara
authors.forename: Teiko
authors.initials: T
authors.lastname: Yamamoto
authors.forename: Shinobu
authors.initials: S
authors.lastname: Takeuchi
mesh_terms: D000328:Adult; D002648:Child; D004596:Electroretinography; D008040:Genetic Linkage; D006801:Humans; D007395:Interneurons / Q000502:physiology; D008297:Male; D008875:Middle Aged; D010775:Photic Stimulation; D017949:Retinal Cone Photoreceptor Cells / Q000503:physiopathology*; D012162:Retinal Degeneration / Q000235:genetics / Q000503:physiopathology*; D014960:X Chromosome