János Hargitai | PubMed Health

PubMed2004

Auto-immune-like cone dystrophy.

PURPOSE: To describe rapid loss of cone vision in an adult due to putative auto-immune rejection. METHODS: Clinical and electrophysiological examination, including full-field and multi-focal electroretinograms (ERGs), were used to assess retinal function. Serum was analyzed for antibodies to retinal antigens. RESULTS: The patient lost cone vision in the course of several months while rod vision remained unaffected. Initially short wavelength (S) cone function appeared more resistant to the degeneration. Cancer associated retinal antibodies were present in the sera of the patient but no cancer has been found. CONCLUSION: Rapid loss of cone function can occur in an adult without a concomitant neoplasm although serum antibodies to retinal antigens suggest an autoimmune cause.

Aged↗

PubMed2002

Macular pigment and visual acuity in Stargardt macular dystrophy.

PURPOSE: To test the hypothesis that macular pigment reflects foveal cone function and possibly the presence of foveal cones in recessive Stargardt macular dystrophy. METHODS: Sixteen patients (32 eyes) diagnosed to have Stargardt macular dystrophy by clinical criteria were studied with a scanning laser ophthalmoscope (SLO) comparing argon laser blue (488 nm), green (514), helium-neon laser red (633 nm) and infrared diode laser (780 nm) images for the presence or absence of macular pigment in the fovea. Fifteen of the patients were screened for mutations in the ABCR gene. Eyes were graded into three categories: those without foveal macular pigment, those with partial pigment and those with normal amounts of macular pigment. These categories were compared with visual acuity determined by the Snellen chart. RESULTS: All patients with a visual acuity of 20/200 or worse had no macular pigment in the fovea. All patients with visual acuity of 20/40 or better had a normal amount of macular pigment in the fovea. Patients with partial macular pigment had intermediary acuity values except for two eyes, one with 20/20 and another with 20/200 acuity. Infrared light revealed more retinal abnormalities than blue light at early stages of the disease. CONCLUSION: Foveal macular pigment is related to foveal cone acuity in Stargardt macular dystrophy and may be a marker for the presence of foveal cones. Infrared light is a sensitive monitor of early Stargardt macular dystrophy.

Adolescent↗