ABSTRACT
To report a case of Boucher-Neuhauser syndrome, which is an autosomal recessive disorder characterized by the triad of spinocerebellar ataxia, chorioretinal dystrophy, and hypogonadotropic hypogonadism. An 18-year-old man was seen for visual problems, which had been diagnosed as retinitis pigmentosa at the age of 12 years. His puberty was delayed. At 16 years of age, the patient experienced progressive deterioration of his balance and gait disturbance. Then he was referred to our clinic because Boucher-Neuhauser syndrome was suspected. He had no specific family history; his visual acuity was 0.04 in both eyes. We observed broad retinal pigment epithelium atrophy and degeneration in both fundi. Both fluorescein and indocyanine green angiography showed choriocapillaris atrophy in the posterior pole area and midperiphery. Macular optical coherence tomography showed thinning of the neurosensory retina. An electroretinographic examination showed no photopic or scotopic responses. The Boucher-Neuhauser syndrome should be included in the differential diagnosis of patients with retinitis pigment epithelium atrophy and degeneration.
Subject(s)
Adolescent , Humans , Male , Atrophy , Cerebellum/pathology , Coloring Agents , Electroretinography , Fluorescein Angiography , Hypogonadism/diagnosis , Indocyanine Green , Magnetic Resonance Imaging , Photoreceptor Cells, Vertebrate/physiology , Retinal Degeneration/diagnosis , Retinal Pigment Epithelium/pathology , Retinitis Pigmentosa/diagnosis , Spinocerebellar Degenerations/diagnosis , Syndrome , Tomography, Optical CoherenceABSTRACT
PURPOSE: To evaluate the prognostic factors that predict visual outcome in eyes with successfully repaired idiopathic macular hole (MH) using optical coherence tomography (OCT). METHODS: We studied 48 eyes with stage 3 or 4 idiopathic MH that had undergone vitrectomy. All cases had anatomic closures and were followed-up for 6 months or more. We analyzed the preoperative macular hole in terms of minimum diameter, stage, hole form factor (HFF), and macular hole index (MHI), as well as the postoperative OCT images of the repaired macular hole and their relevance to visual recovery. RESULTS: We found significant differences in the preoperative minimum diameter of MH, HFF, the postoperative OCT images of the repaired macular hole, and postoperative visual acuvity. In contrast, we found no statistically significant differences in the preoperative base MH diameter, stage, HFF, MHI, and their relevance to visual recovery. CONCLUSIONS: Minimum diameter of MH, HFF, and the postoperative OCT images of the repaired MH are significant prognostic factors after vitrectomy for the treatment of idiopathic MH.
Subject(s)
Retinal Perforations , Tomography, Optical Coherence , VitrectomyABSTRACT
PURPOSE: We evaluated whether lipoic acid as antioxidant could inhibit expression of VEGF and STAT3 in experimental diabetic rat retina. METHODS: Diabetes was induced chemically by injection of streptozotocin in 12 rats of 18 Sprague-Dawley rats. After induction of diabetes, lipoic acid was injected into the peritonium in 6 rats. So all rats were divided into 3 groups, normal group (n=6), diabetes mellitus (DM) group (n=6), lipoic acid treated group (n=6). The ocular tissue of the rats were collected on 8 weeks after diabetes induction. Difference of VEGF and STAT3 expression was investigated by immunohistochemistry, RT-PCR, western blot. The change of VEGF and STAT3 in lipoic acid treated group were evaluated in these experimental model. RESULTS: The VEGF and STAT3 expression was elevated in diabetic rat retina. The active form STAT3, phosphorylated STAT3 was also elevated. The VEGF and STAT3 expression in lipoic acid treated group was lower than DM group. CONCLUSIONS: The lipoic acid could inhibit the VEGF and STAT3 expression in diabetic rat retina.
Subject(s)
Animals , Rats , Blotting, Western , Diabetes Mellitus , Diabetic Retinopathy , Immunohistochemistry , Models, Theoretical , Rats, Sprague-Dawley , Retina , Streptozocin , Thioctic Acid , Vascular Endothelial Growth Factor AABSTRACT
PURPOSE: To evaluate the therapeutic effect of cyclosporin A, corticosteroid, autologous serum in patients with severe dry eye syndrome. METHODS: Patients (72 eyes) with severe dry eye syndrome were classified into four groups were treated for 3 months. Each group was treated with 0.05% cyclosporin A, 1% rimexolone, 20% autologous serum, and artificial tears. Before and after treatment (2, 4, 8, 12 weeks), Ocular Surface Disease Index (OSDI), tear film break-up time (BUT), Schirmer test, corneal sensitivity, and lissamine green staining score were measured. RESULTS: After treatment, OSDI, tear film BUT, Schirmer test, corneal sensitivity, and the lissamine green staining score were improved in groups using cyclosporin A, autologous serum and corticosteroid(p0.05). CONCLUSIONS: Antiinflammatory theraphy is more effective than the conventional symptomatic theraphy in patients with severe dry eye syndrome.
Subject(s)
Humans , Cyclosporine , Dry Eye Syndromes , Ophthalmic Solutions , TearsABSTRACT
PURPOSE: To evaluate the advantages and complications of triamcinolone acetonide (TA)-assisted pars plana vitrectomy (PPV) for proliferative diabetic retinopathy (PDR). METHODS: We retrospectively examined 110 eyes with vitreous hemorrhage or tractional retinal detachment resulting from a PDR after surgery. The TA-assited PPV [TA(+)] consisted of 58 eyes, and the conventional PPV[TA(-)]group consisted of 52 eyes. The improvement in vision, residual vitreous cortex (VC) pattern, and postoperative complications were studied. RESULTS: The residual VC pattern was divided into 3 groups: the focal type in 34 eyes (58.6%): the diffuse type in 22 eyes (37.9%): and no residual VC, seen in 2 eyes (3.4%). The TA (+) group had a lower incidence of rebleeding (p=0.0149) and of a preretinal membrane (p=0.0138) than the TA (-) group. No apparent persistant ocular hypertension occurred in any eyes. CONCLUSIONS: Triamcinolone-assisted PPV appears to be potentially useful to remove residual VC and to protect from postoperative complications.