Format

Send to

Choose Destination
Invest Ophthalmol Vis Sci. 2009 Feb;50(2):950-8. doi: 10.1167/iovs.08-2544. Epub 2008 Sep 29.

Rod and rod-driven function in achromatopsia and blue cone monochromatism.

Author information

1
Department of Ophthalmology, Children's Hospital and Harvard Medical School, Boston, Massachusetts 02115, USA. anne.moskowitz@childrens.harvard.edu

Abstract

PURPOSE:

To evaluate rod photoreceptor and postreceptor retinal function in pediatric patients with achromatopsia (ACHR) and blue cone monochromatism (BCM) using contemporary electroretinographic (ERG) procedures.

METHODS:

Fifteen patients (age range, 1-20 years) with ACHR and six patients (age range, 4-22 years) with BCM were studied. ERG responses to full-field stimuli were obtained in scotopic and photopic conditions. Rod photoreceptor (S(rod), R(rod)) and rod-driven postreceptor (log sigma, V(max)) response parameters were calculated from the a-wave and b-wave. ERG records were digitally filtered to demonstrate the oscillatory potentials (OPs); a sensitivity parameter, log SOPA(1/2), and an amplitude parameter, SOPA(max), were used to characterize the OP response. Response parameters were compared with those of 12 healthy control subjects.

RESULTS:

As expected, photopic responses were nondetectable in patients with ACHR and BCM. In addition, mean scotopic photoreceptor (R(rod)) and postreceptor (V(max) and SOPA(max)) amplitude parameters were significantly reduced compared with those in healthy controls. The flash intensity required to evoke a half-maximum b-wave amplitude (log sigma) was significantly increased.

CONCLUSIONS:

Results of this study provide evidence that deficits in rod and rod-mediated function occur in the primary cone dysfunction syndromes ACHR and BCM.

PMID:
18824728
PMCID:
PMC2631615
DOI:
10.1167/iovs.08-2544
[Indexed for MEDLINE]
Free PMC Article

Supplemental Content

Full text links

Icon for Silverchair Information Systems Icon for PubMed Central
Loading ...
Support Center