Remission of nonparaneoplastic autoimmune retinopathy after minimal steroid treatment.

PURPOSE: To describe the clinical findings in a patient demonstrating recovery from nonparaneoplastic autoimmune retinopathy after a minimal course of steroid treatment. METHODS: Clinical presentation was documented, and paraclinical tests were obtained using Humphrey automated perimetry for visual fields, Western blotting for antiretinal antibodies, and electroretinography for evaluation of rod and cone function. RESULTS: Initial presentation revealed marked visual field deficits, electroretinographic dysfunction, and the presence of α-enolase autoantibodies. After a brief course of oral corticosteroids, the patient demonstrated improvement in visual fields, disappearance of α-enolase autoantibodies, partial recovery of the cone on-response, and complete recovery of the rod response. CONCLUSION: This case is distinguished from previous reports by the rapidity and degree of recovery, the brevity of treatment required, and the unique electroretinographic recovery pattern with concomitant disappearance of α-enolase autoantibodies. These findings suggest a pathologic role for α-enolase autoantibodies in autoimmune rod bipolar cell dysfunction. Identification of other cases exhibiting such improvements and associated autoantibody activity may expand our understanding of nonparaneoplastic autoimmune retinopathy disease pathogenesis.

Creutzfeldt-Jakob disease presenting with visual manifestations.

BACKGROUND: Creutzfeldt-Jakob disease (CJD) is a rare and progressive degenerative disease of the central nervous system (CNS), characterized pathologically by spongiform changes in various CNS tissues. It most often presents with rapidly progressing dementia and cognitive decline, along with other neurologic findings that correspond to affected areas of the CNS. We present 5 sporadic cases of CJD presenting with visual manifestations, 2 of which were consistent with the Heidenhain variant, which predominantly affects the occipital lobe. METHODS: Each of the cases demonstrated electroencephalographic changes suggestive of CJD. The neuro-ophthalmic findings included both sensory and motor manifestations, including cortical blindness, hemianopsia, dysmetria, visual hallucinations, hypometric saccades, and diplopia. RESULTS: All patients died within 5 months after the onset of signs of the disease and pathologic specimens were obtained in 4 of the cases. INTERPRETATION: Although CJD is not a commonly seen condition, its possibility should be entertained in any patient presenting with typical neurologic deterioration and complaining of visual symptoms.

Giant cell arteritis presenting with aortic aneurysm, normal erythrocyte sedimentation rate, and normal C-reactive protein.

CASE REPORT: We report on an extremely rare case of giant cell arteritis (GCA) presenting without elevated erythrocyte sedimentation rate or C-reactive protein level, with aortic root involvement, and in the absence of typical clinical findings. COMMENTS: The clinical signs and symptoms as well as the laboratory investigations involved in the diagnosis of GCA are discussed.

Comparison of in vitro toxicity of single vs double exposures of indocyanine green in human retinal pigment epithelial cell cultures.

PURPOSE: To compare the in vitro effect of a single brief indocyanine green (ICG) exposure with a double exposure on retinal pigment epithelial (RPE) cells. DESIGN: In vitro laboratory experimental study. METHODS: Human ARPE-19 cells were exposed to a single dilute ICG exposure (0.5 mg/ml) or to two sequential exposures of identical volume and concentration. Viability was measured with a mitochondrial dehydrogenase assay and compared with nonexposed control cells. RESULTS: Cell viability was not statistically different between the single-exposed, double-exposed, or control cell populations. CONCLUSIONS: When used intraoperatively to stain the internal limiting membrane, dilute ICG dye sometimes does not adequately enhance visualization of the internal limiting membrane. Occasionally, it is necessary to repeat the dye exposure to achieve the desired visibility. In this study, RPE cells subjected to two consecutive short exposures of ICG showed no marked difference in cell survival when compared with cells exposed to a single application of ICG and to control cells.

Development of macular hole in the early postoperative period following pneumatic retinopexy.

Pneumatic retinopexy is a minimally invasive procedure used to repair uncomplicated superior rhegmatogenous retinal detachments. Macular holes have been reported in only 6 cases as a rare sequel to this procedure. The development of macular hole in a patient in the early perioperative period following pneumatic retinopexy is described.

Comparison of the in vitro toxicity of indocyanine green to that of trypan blue in human retinal pigment epithelium cell cultures.

PURPOSE: To compare the in vitro toxicity of indocyanine green (ICG) to that of trypan blue (TB) in human retinal pigment epithelium cell cultures. The use of ICG and TB in macular hole surgery is discussed. DESIGN: In vitro cell biology experimental study. METHODS: The ICG dye and TB were applied to ARPE-19, a commercially available human retinal pigment epithelium cell line. Cultures were established and maintained according to supplier protocols. The ICG dye, TB or Hank's balanced salt solution (controls) were then applied to the cells at varying concentrations and over various exposure periods. Fiberoptic light was also applied to cells to assess for the possibility of a potentiating phototoxic effect. Cell viability fractions were determined using a well-studied mitochondrial dehydrogenase assay. RESULTS: The TB was not toxic to the retinal pigment epithelium cell cultures at any concentration or over any period of exposure, whereas ICG dye demonstrated dose-dependent and exposure-dependent toxicity. The ICG dye was found to be toxic to the cells at all tested concentrations between 5.0 mg/ml (stock concentration, 26.1% cell survival) and 0.5 mg/ml (92.8% cell survival) over a 3-minute exposure. No toxicity to TB was seen at the stock concentration of 1.5 mg/mL. Addition of light to the cultures did not significantly alter cell viability with either dye. Long periods of exposure, 2 hours, 24 hours, and 72 hours, to minute concentrations of either dye did not produce significant cell death. CONCLUSIONS: Indocyanine green demonstrates more toxicity than TB to human retinal pigment epithelium cell cultures. This is independent of any phototoxic potentiating effect of fiberoptic light or solvent toxicity. A clinically useful concentration of 0.5-mg/ml ICG causes low cytotoxicity at 3 minutes' exposure (cell survival 92.8%) and shows no detectable toxicity at 1-minute exposure (cell survival 102%).