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Originally published In Press as doi:10.1167/iovs.08-2544 on September 29, 2008
 (Investigative Ophthalmology and Visual Science. 2009;50:950-958.)
© 2009 by The Association for Research in Vision and Ophthalmology, Inc.
doi:10.1167/iovs.08-2544
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Rod and Rod-Driven Function in Achromatopsia and Blue Cone Monochromatism

Anne Moskowitz, Ronald M. Hansen, James D. Akula, Susan E. Eklund, and Anne B. Fulton

From the Department of Ophthalmology, Children’s Hospital and Harvard Medical School, Boston, Massachusetts.

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 (Srod, Rrod) and rod-driven postreceptor (log {sigma}, Vmax) 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 SOPA1/2, and an amplitude parameter, SOPAmax, 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 (Rrod) and postreceptor (Vmax and SOPAmax) 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.






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