Patient with Adult T-cell Leukemia and Lung Infection caused by Mycobacterium abscessus: Successful Treatment with Intensive Chemotherapy Followed by Haploidentical Hematopoietic Stem Cell Transplantation: A Case Report.

A 63-year-old woman with adult T-cell leukemia (ATL) lymphomatous type developed a mild dry cough. Computed tomography revealed lung lesions with a tree-in-bud appearance during intensive chemotherapy. Antibodies against Mycobacterium avium complex were positive. Bronchoalveolar lavage culture showed growth of M. abscessus complex. Finally, M. abscessus subsp. massiliense was also identified. Sequential use of antimicrobials, including macrolides, was introduced during intensive chemotherapy, and the patient successfully underwent allogeneic hematopoietic stem cell transplantation (AHSCT). This is the first case report of a patient with ATL complicated by M. massiliense lung infection, who was successfully treated with haploidentical AHSCT using various combinations of antimicrobials.

[Development of thrombocytopenic purpura following BNT162b2 mRNA COVID-19 vaccination].

Because the coronavirus disease 2019 (COVID-19) pandemic is still rampant, vaccination is being promoted worldwide. However, the safety of various COVID-19 vaccines remains poorly understood. We herein report the case of a 37-year-old woman who experienced thrombocytopenia following BNT162b2 mRNA COVID-19 vaccination. The patient presented with purpura on the extremities 10 days after the first vaccination. She had marked thrombocytopenia and no thrombosis. Thrombocytopenia resolved spontaneously. Given the possibility of occurrence of post-vaccination thrombocytopenia, vaccinated persons should be instructed to consult a medical institution if they experience bleeding symptoms.

Detection of increase in corneal irregularity due to pterygium using Fourier series harmonic analyses with multiple diameters.

PURPOSE: To determine steep increase of corneal irregularity induced by advancement of pterygium. METHODS: A total of 456 eyes from 456 consecutive patients with primary pterygia were examined for corneal topography and advancement of pterygium with respect to the corneal diameter. Corneal irregularity induced by the pterygium advancement was evaluated by Fourier harmonic analyses of the topographic data that were modified for a series of analysis diameters from 1 mm to 6 mm. Incidences of steep increases in the asymmetry or higher-order irregularity components (inflection points) were determined by using segmented regression analysis for each analysis diameter. RESULTS: The pterygium advancement ranged from 2% to 57%, with a mean of 22.0%. Both components showed steep increases from the inflection points. The inflection points in the higher-order irregularity component altered with the analysis diameter (14.0%-30.6%), while there was no alternation in the asymmetry components (35.5%-36.8%). For the former component, the values at the inflection points were obtained in a range of 0.16 to 0.25 D. CONCLUSION: The Fourier harmonic analyses for a series of analysis diameters revealed that the higher-order irregularity component increased with the pterygium advancement. The analysis results confirmed the precedence of corneal irregularity due to pterygium advancement.

Proposal for a Novel Severity Grading System for Pterygia Based on Corneal Topographic Data.

PURPOSE: To propose a grading system of pterygium severity based on corneal higher-order irregularity (HOI) and to evaluate postoperative changes in corneal irregularity of the graded pterygia. METHODS: In 268 eyes of 226 patients undergoing excision surgery of primary nasal pterygium, Placido corneal topography images were taken before and until 6 months after surgery. The best-corrected visual acuity (BCVA) and pterygium size with respect to the corneal diameter were also measured. HOI components within 1.0-, 3.0-, and 5.0-mm diameters were obtained using Fourier harmonic analysis of corneal topography data. With threshold levels when the HOIs for 3 diameters steeply increased, 4 levels of grading from 0 (mild) to 3 (severe) were determined. Associations of the grades with the preoperative surface regularity index (SRI), irregular components (IRRs) obtained from the 3.0-mm diameter Fourier analysis, and BCVA were examined. Time-course changes in the indexes after surgery were evaluated for each grade. RESULTS: There were 62, 65, 41, and 100 eyes in grades 0 to 3, respectively. Preoperatively, the SRI in grade 3 was significantly higher than in the other grades (P < 0.001), and the IRR and BCVA varied with the grade (P < 0.001). Between 1 and 6 months postoperatively, grade 3 pterygia showed significant changes in the SRI and IRR (P = 0.013 and 0.017, respectively), whereas there was no change after 3 months postoperatively in the SRI, IRR, and BCVA for all other grades. CONCLUSIONS: The proposed grading effectively classified the severity of pterygia and evaluated postoperative restoration of corneal irregularity. Using corneal topographic data allowed for objective evaluation of pterygium severity.

Bilateral papillomacular retinoschisis and macular detachment accompanied by focal lamina cribrosa defect in glaucomatous eyes.

PURPOSE: We report here a patient with bilateral papillomacular retinoschisis with an enlarged glaucomatous optic nerve head cup and a focal lamina cribrosa defect, the findings of our clinical investigations of this case, and the chosen treatment and outcome. DESIGN: This is an observational case report. METHODS: Clinical examinations were performed using simultaneous confocal scanning laser ophthalmoscopy and optical coherence tomography (OCT). The patient was treated by pars plana vitrectomy (PPV). RESULTS: The left eye had a macular detachment with a presumed acquired pit of the optic nerve, while the right eye did not have an obvious optic nerve pit. Enhanced depth imaging OCT showed focal lamina cribrosa defects in both eyes. PPV was performed on the left eye, which resulted in a re-attachment of the macula and improvement of the visual acuity. CONCLUSIONS: Our findings suggest that the pathological changes were most likely due to focal lamina cribrosa defects in both glaucomatous eyes. This type of maculopathy can be successfully treated with PPV.

Prevalence and spatial distribution of cystoid spaces in retinitis pigmentosa: investigation with spectral domain optical coherence tomography.

PURPOSE: To investigate the prevalence and spatial distribution of cystoid spaces (CS) in retinitis pigmentosa patients with spectral domain optical coherence tomography. METHODS: A total of 529 eyes of 275 patients with retinitis pigmentosa were examined with spectral domain optical coherence tomography. The presence or absence of CS was judged for each eye. Retinal layer and outer retinal status where the CS existed were also investigated. Statistical analysis was performed using 1 eye per 1 patient. RESULTS: Cystoid spaces were present in 119 of 529 eyes (22.5%) of 74 of 275 patients (26.9%). There were no significant differences between the cases with and without CS except for central foveal thickness (P < 0.001). Cystoid spaces were noted in the inner nuclear layer in almost all eyes (98.6%), and outer nuclear layer/outer plexiform layer was also involved in many eyes (27.8%). Cystoid spaces were sometimes seen in ganglion cell layer (6.9%). Cystoid spaces were predominantly (78.9%) distributed in the relatively preserved retina where external limiting membrane was retained. The presence of epiretinal membrane or posterior vitreous adhesion was associated with the presence of CS (P < 0.001) but showed no relationship with the spatial location of CS (P = 1.000). CONCLUSION: The prevalence of CS in patients with retinitis pigmentosa was 26.9% and contrary to previous reports, most CS were present in inner nuclear layer. In addition, most CS were observed in relatively retained retina, which is compatible to the prevailing notion. Epiretinal membrane or posterior vitreous adhesion was also associated with the development of CS. The distribution of CS in inner and preserved retina may provide insight for the pathogenesis of CS in retinitis pigmentosa.

Clinical and immunological characterization of paraneoplastic retinopathy.

PURPOSE: To report the clinical and immunological characterization of paraneoplastic retinopathy (PR) and to investigate the association between spectral-domain optical coherence tomography (SDOCT) findings and the targets of autoantibodies in PR. METHODS: We retrospectively enrolled eight patients (age range, 57-85 years; four men and four women) suspected of having PR. All patients underwent comprehensive ophthalmic examinations, including best-corrected visual acuity (BCVA) measurement, slitlamp examinations, kinetic visual field testing with Goldmann perimetry, electroretinography (ERG), fundus photography, fluorescein angiography, fundus autofluorescence (FAF), SDOCT, and serum sample tests (Western blot analysis and immunohistochemistry [IHC]). RESULTS: Three patients had a history of malignant tumors, and four patients were newly diagnosed as having neoplastic tumors (small cell lung carcinoma [SCLC], thymoma, pancreatic neuroendocrine neoplasm, and colon cancer). Another de novo malignancy (SCLC) was detected in a patient with a history of malignancy (bladder cancer and liposarcoma). The BCVA in these patients ranged from hand motion to 1.5. Goldmann perimetry revealed island, ring-shaped, concentric, or central scotoma. All patients showed nonrecordable or reduced amplitude results on ERG. Fluorescein leakage was detected in five patients. Hyperautofluorescence and/or hypoautofluorescence on FAF was detected in six patients. The serum sample tests identified anti-retinal antibodies in all patients. Patients whose serum contained anti-photoreceptor or anti-retinal pigment epithelium antibody on IHC showed damage of the outer retina on SDOCT. CONCLUSIONS: In this case series, PR was associated with a variety of neoplasms and autoantibodies. Spectral-domain OCT can be used to characterize morphologic changes, and the changes were associated with the targets of autoantibodies.

[Genotype screening of retinal dystrophies in the Japanese population using a microarray].

PURPOSE: To investigate the pathogenic variants of retinal dystrophies in the Japanese population using microarray analysis. SUBJECTS AND METHODS: DNA extracted from the blood samples of 84 families (87 patients) with retinal dystrophies (retinitis pigmentosa, Leber congenital amaurosis, cone-rod dystrophy and Bietti's crystalline retinopathy) was screened by Asper Biotech services. All the variants detected by microarray analysis were verified by direct sequencing. RESULTS: Mutations were detected in 2 of 36 families with autosomal dominant retinitis pigmentosa, 2 of 4 with Leber congenital amaurosis, 11 of 24 with cone-rod dystrophy, 3 of 7 with macular dystrophy and 6 of 7 with Bietti's crystalline retinopathy. CONCLUSION: Genotype screening using microarray analysis can be effectively used to determine the variants of retinal dystrophies, except retinitis pigmentosa, in the Japanese population.

Concentric division of 10° visual field tests in retinitis pigmentosa.

PURPOSE: The purpose of this study was to estimate the optimal size of visual field test for detecting longitudinal changes in retinitis pigmentosa (RP) by dividing the visual field. METHODS: We reviewed the results of 10° static visual field tests in 19 eyes of 19 RP patients. Sixty-eight numeric value points were divided into two area types: concentric areas (A1, A1-2, A1-3, A1-4, A1-5, A1-6) and circular areas (A1, A2, A3, A4, A5, A6). Serial values of mean sensitivity in each area of each patient were analyzed by linear regression. RESULTS: Analysis of the concentric areas showed that 10 of 19 eyes had the best R (2) value in the most central area, A1. Analysis of circular areas showed that 7 of 19 eyes had the steepest slope of decline in A1. The inner-segment/outer-segment (IS/OS) line was significantly shorter in eyes with low variability and evident disease progression in the inner areas than the ones in the outer areas. CONCLUSIONS: The optimal size of monitoring RP progression was different in each case and may depend on the remaining morphology of the outer retina.

The time course changes of choroidal neovascularization in angioid streaks.

PURPOSE: To assess the clinical course of choroidal neovascularization (CNV) in patients with angioid streaks using optical coherence tomography and fluorescein angiography/indocyanine green angiography. METHODS: We examined a consecutive series of 88 eyes of 44 patients with angioid streaks using color fundus photography, optical coherence tomography, and fluorescein angiography/indocyanine green angiography. RESULTS: At the initial visit, 33 eyes exhibited no CNV, 2 exhibited polypoidal choroidal vasculopathy, 8 exhibited Type 1 CNV, 32 exhibited active Type 2 CNV, and 13 exhibited a fibrotic scar. In addition to the 2 eyes that exhibited macular polypoidal choroidal vasculopathy at the initial visit, 3 exhibited peripapillary polypoidal lesions, and 2 exhibited polypoidal lesions at the edge of the preexisting Type 2 CNV/fibrosis. During the follow-up, Type 2 CNV developed in 4 eyes on the basis of Type 1 CNV. Visual acuity was worse in eyes with Type 2 CNV and fibrosis than in those with Type 1 CNV, while polypoidal choroidal vasculopathy did not affect the visual acuity. CONCLUSION: Eyes with angioid streaks can develop any form of CNV including polypoidal choroidal vasculopathy. Considering the worse visual acuity in eyes with Type 2 CNV and fibrosis, patients should be carefully observed so as to treat them promptly when Type 2 CNV occurred beneath the fovea.

Two-year outcome of photodynamic therapy combined with intravitreal injection of bevacizumab and triamcinolone acetonide for polypoidal choroidal vasculopathy.

PURPOSE: To compare the 2-year results after photodynamic therapy (PDT) alone and PDT combined with intravitreal injections of bevacizumab and triamcinolone acetonide (triple therapy) for polypoidal choroidal vasculopathy (PCV). METHODS: We retrospectively reviewed the medical records of 40 consecutive patients (40 eyes) with subfoveal PCV. Of these 40 eyes, 16 were treated with PDT alone and 24 were treated with triple therapy. RESULTS: The change in visual acuity in the triple therapy group was significantly better than that in the PDT group (P < 0.001). At 24 months, improvement in visual acuity was seen in only two eyes (12.5 %) of the PDT group, while it was seen in ten eyes (41.7 %) of the triple therapy group. Retreatment was given to 12 eyes (75.0 %) in the PDT group and to nine eyes (37.5 %) in the triple therapy group, although the retreatment-free period was significantly longer in the triple therapy group than in the PDT group (P < 0.001). Post-treatment vitreous hemorrhage was seen in only two eyes (12.5 %), all of which were in the PDT group. CONCLUSION: Compared with PDT alone, triple therapy appears to reduce the postoperative hemorrhagic complications and recurrences of PCV and to improve the 2-year visual outcomes of PCV.

Contrast visual acuity in patients with retinitis pigmentosa assessed by a contrast sensitivity tester.

PURPOSE: To assess contrast visual acuity (CVA) in patients with retinitis pigmentosa (RP) and compare the result with standard visual acuity (VA), retinal thickness, status of inner segment/outer segment junction, and central visual field. MATERIALS AND METHODS: Thirty-nine eyes of 39 patients with RP and 39 eyes of 39 healthy individuals were studied. To see the difference in CVA between RP patients and normal controls, only subjects with standard VA of 1.0 (20/20) or better were included. This was a cross-sectional study. CVA in various light conditions was measured with CAT-2000 and was compared between patients and controls. CVA of patients was further analyzed for association with other parameters including foveal retinal thickness, outer nuclear layer thickness, the status of inner segment/outer segment junction measured with optical coherence tomography (OCT), and visual field mean deviation (MD) measured with Humphrey field analyzer 10-2 program. RESULTS: CVA impairment was evident in RP patients compared to controls (P < 0.01, in all measurement conditions). Multivariate analysis showed association of logarithm of the minimum angle of resolution (logMAR) with CVAs in several conditions. None of the OCT measurements was associated with CVA. When patients were divided into three groups based on MD, the most advanced group (MD worse than or equal to -20 dB) showed impairment of mesopic CVA (P < 0.05, under mesopic condition of 100% without glare, with glare, and 25% without glare). CONCLUSION: CVA impairment was confirmed in RP patients, especially in advanced cases. CVA measured with CAT-2000 may be a useful tool for assessing foveal function in RP patients.

Knockout of ccr2 alleviates photoreceptor cell death in a model of retinitis pigmentosa.

Neuroinflammation involving CC chemokines such as monocyte chemoattractant protein-1 (MCP-1) has been demonstrated in the pathological process of retinitis pigmentosa (RP), an inherited degenerative retinal disease. However, the mechanism of MCP-1 and its receptor CCR2 involvement in the disease remains unclear. To investigate the role of MCP1/CCR2 in RP pathogenesis, ccr2 mutant RP mice (ccr2(-/-) rd10) were created and analyzed. The expression of MCP-1, RANTES, stromal cell-derived factor (SDF-1), and tumor necrosis factor-α (TNF-α) in the retinas of wild-type, rd10, and ccr2(-/-) rd10 mice was analyzed using quantitative RT-PCR. Photoreceptor apoptosis (TUNEL staining) and the number of microglia (positive for the F4/80 antibody) in the retina were examined. Retinal function was assessed using electroretinograms, and the structure of the whole retina was analyzed from images obtained using optical coherence tomography (OCT) and by histological examination. The expression levels of MCP-1, RANTES, and SDF-1 increased with time in the rd10 mice but not in the wild-type mice. Rearing the mice in the dark prevented degeneration and resulted in thicker photoreceptor layers at each time point. In those mice, the peaks of chemokine expression shifted to a later time with degeneration, suggesting that the expression of these chemokines was induced during the progression of degeneration. Although the difference was not so obvious, the retina in the ccr2(-/-) rd10 mice was consistently and significantly thicker than that in the rd10 (ccr2(+/+) rd10) mice at all time points. Rhodopsin gene expression was also higher in the ccr2(-/-) rd10 mice than in rd10 (ccr2(+/+) rd10) mice, suggesting photoreceptor survival in the former. Retinal function was also better preserved in the ccr2(-/-) rd10 mice than in the rd10 mice. The number of microglia in the retinas of the ccr2(-/-) rd10 mice was significantly lower than that in the retinas of the rd10 mice. Interestingly, the MCP-1 induction that was observed in the retinas of the rd10 mice was diminished in the retinas of the ccr2(-/-) rd10 mice. Our results suggest that the MCP-1/CCR2 system plays a role in retinal degeneration in rd mouse retinas. Retinal MCP-1 expression in the rd mouse retina may be partially controlled by ccr2-positive circulating cells.

Association of ARMS2 genotype with bilateral involvement of exudative age-related macular degeneration.

PURPOSE: To study the association of ARMS2 A69S genotype with the development of exudative age-related macular degeneration (AMD) in the unaffected fellow eye and to estimate the duration until the development of AMD in the second eye. DESIGN: Retrospective cohort study. METHODS: We retrospectively reviewed 326 patients who had exudative AMD in at least 1 eye, genotyping of ARMS2 A69S, and a minimum follow-up of 2 years. Survival analysis and Cox proportional hazard regression analysis were used to examine the association between candidate factors and the duration until the development of AMD in the second eye. RESULTS: One hundred nineteen patients (36.5%) had bilateral exudative AMD at the initial visit. A risk allele of ARMS2 A69S was more frequently seen in patients with bilateral AMD (P = .0270) than in those with unilateral AMD. Of the 207 unilateral AMD patients, 23 (11.1%) had AMD in the fellow eye after a mean duration of 56.3 ± 40.4 months. Fellow-eye involvement was associated with ARMS2 A69S genotype (hazard ratio [HR], 2.673; P = .0013), age (HR, 1.102; P = .0005), and smoking history (HR, 0.680; P = .3663). As HRs indicate, correlation of genotype (2.673) was as high as that of 10-year aging (1.102(10) = 2.641). Survival analysis revealed that patients with risk homozygous (TT) genotype had second-eye involvement significantly earlier than those with other genotypes (P = .0028). When the observation duration reached 120 months, second-eye involvement had developed in 50%, 6.6%, and 11.2% of the TT, GT, and GG cohorts, respectively. CONCLUSION: ARMS2 A69S genotype is associated with second-eye involvement of exudative AMD and with the period between first- and second-eye involvements.

Retinal structural alterations and macular sensitivity in idiopathic macular telangiectasia type 1.

PURPOSE: To evaluate the relationship between the abnormalities of retinal structures showed on spectral-domain optical coherence tomography and the changes in the macular sensitivity measured by microperimetry in eyes with idiopathic macular telangiectasia type 1. METHODS: Eleven eyes of 11 patients with macular telangiectasia type 1 were reviewed. Morphologic changes in the retina and retinal sensitivity of eyes with macular telangiectasia type 1 were studied using spectral-domain optical coherence tomography and microperimetry. RESULTS: Spectral-domain optical coherence tomographic images revealed disruptions in the photoreceptor inner segment-outer segment (IS/OS) junction in all the eyes and intraretinal cystoid spaces in 10 eyes. In the points that had intraretinal cystoid spaces, the mean retinal sensitivity was 6.8 ± 5.5 dB where the IS/OS was disrupted and 13.4 ± 4.0 dB where the IS/OS was intact (P < 0.001). In the points that had intact IS/OS and no cystoid spaces, the mean retinal sensitivity was 15.3 ± 4.3 dB, which was better than that of points that had intact IS/OS with cystoid spaces (P < 0.001). CONCLUSION: Retinal sensitivity is influenced not only by intraretinal cystoid spaces but also by IS/OS disruptions, and the IS/OS alterations reduce the visual function more severely.

Significance of C2/CFB variants in age-related macular degeneration and polypoidal choroidal vasculopathy in a Japanese population.

PURPOSE: To determine whether genetic variants in the complement component 2 and factor B gene (C2/CFB) locus are associated with the risk for typical age-related macular degeneration (AMD) or polypoidal choroidal vasculopathy (PCV) in a Japanese population. METHODS: Four single nucleotide polymorphisms (SNPs) were genotyped across the C2/CFB locus of patients with typical AMD (n = 455) or PCV (n = 581) and of 865 controls. Differences in the observed genotypic distribution between the case and control groups were tested by logistic regression analysis for age and sex adjustments. Significant associations were confirmed using a second control group of 336 cataract patients. A further model adjusting for age-related maculopathy susceptibility 2 (ARMS2) A69S, complement factor H (CFH) I62V, age, sex and smoking status was performed, to confirm their independent association from other covariates. RESULTS: C2 rs547154 and CFB rs541862 were significantly associated with typical AMD and PCV in this Japanese sample (P < 0.05). These two SNPs were also significantly associated with typical AMD and PCV in evaluation of the second control cohort (P < 0.05). Furthermore, an independent association of C2/CFB variants was found for both typical AMD and PCV with age, sex, smoking, and genetic background of ARMS2 A69S and CFH I62V (vs. typical AMD: P = 0.0073, odds ratio [OR] = 0.47; vs. PCV: P = 0.0083, OR = 0.53). CONCLUSIONS: C2/CFB variants play a protective role in the risk of developing neovascular AMD and PCV in the Japanese.

Treatment of polypoidal choroidal vasculopathy with photodynamic therapy combined with intravitreal injections of ranibizumab.

PURPOSE: To evaluate the 1-year efficacy and safety of photodynamic therapy (PDT) combined with intravitreal injections of ranibizumab for polypoidal choroidal vasculopathy (PCV). DESIGN: Retrospective chart review. METHODS: We retrospectively reviewed the medical records of 63 consecutive patients (66 eyes) with subfoveal PCV who were treated with PDT combined with intravitreal injections of ranibizumab. Of the 66 eyes, 29 had no history of treatment for PCV, 10 had been treated previously with only intravitreal injections of anti-vascular endothelial growth factor agents, and 27 had been treated previously with PDT. All eyes had a minimal follow-up of 12 months. RESULTS: The combined therapy reduced substantially the exudative change immediately after initiation of treatment. In treatment-naïve eyes, mean VA before treatment (0.47 ± 0.37 logarithm of the minimal angle of resolution [logMAR]) improved to 0.32 ± 0.30 (P < .01) at 3 months and to 0.29 ± 0.29 (P < .01) at 12 months. Polypoidal lesions were reduced in all eyes and disappeared completely in 79.1% of cases. In eyes treated previously with only anti-vascular endothelial growth factor therapy, some visual improvement was achieved, but in eyes treated previously with PDT, mean visual acuity (0.61 ± 0.45) deteriorated to 0.68 ± 0.52 at 12 months. Of all 66 eyes, 5 showed extensive postoperative subretinal hemorrhage, in 2 of which a vitreous hemorrhage developed, necessitating pars plana vitrectomy. CONCLUSIONS: PDT combined with ranibizumab led to significant visual recovery in treatment-naïve eyes with PCV, but not in eyes with PCV that had demonstrated recurrence after previous PDT. PDT in combination with ranibizumab still has a risk of the postoperative hemorrhagic complications.

Low-dose-rate, low-dose irradiation delays neurodegeneration in a model of retinitis pigmentosa.

The existence of radiation hormesis is controversial. Several stimulatory effects of low-dose (LD) radiation have been reported to date; however, the effects on neural tissue or neurodegeneration remain unknown. Here, we show that LD radiation has a neuroprotective effect in mouse models of retinitis pigmentosa, a hereditary, progressive neurodegenerative disease that leads to blindness. Various LD radiation doses were administered to the eyes in a retinal degeneration mouse model, and their pathological and physiological effects were analyzed. LD gamma radiation in a low-dose-rate (LDR) condition rescues photoreceptor cell apoptosis both morphologically and functionally. The greatest effect was observed in a condition using 650 mGy irradiation and a 26 mGy/minute dose rate. Multiple rounds of irradiation strengthened this neuroprotective effect. A characteristic up-regulation (563%) of antioxidative gene peroxiredoxin-2 (Prdx2) in the LDR-LD-irradiated retina was observed compared to the sham-treated control retina. Silencing the Prdx2 using small-interfering RNA administration reduced the LDR-LD rescue effect on the photoreceptors. Our results demonstrate for the first time that LDR-LD irradiation has a biological effect in neural cells of living animals. The results support that radiation exhibits hormesis, and this effect may be applied as a novel therapeutic concept for retinitis pigmentosa and for other progressive neurodegenerative diseases regardless of the mechanism of degeneration involved.

Outer retinal circular structures in patients with Bietti crystalline retinopathy.

BACKGROUND: Bietti crystalline retinopathy (BCR) is a distinct retinal degenerative disease characterised by retinal degeneration with many yellow-white crystals located mainly at the posterior pole area. Using spectral domain-optical coherence tomography (SD-OCT), the structural change in retina was investigated. METHODS: Patients diagnosed with BCR (n=12), retinitis pigmentosa (RP, n=292) and cone dystrophy (n=16) were included in this study. The authors mainly examined fundus photographs and SD-OCT, infrared and fundus autofluorescence images of these patients. RESULTS: Crystalline deposits were detected in portions of the retinal pigment epithelium that lacked patchy degenerated lesions. SD-OCT revealed that most of the observed crystalline deposits were located adjacent to the inner side of retinal pigment epithelium layer. The change most frequently observed was circular hyper-refractive structures in the outer nuclear layer. Although the structures were considered to be previously reported "tubular formation" or "tubular degeneration", we determined that many of these circular structures were slices of spherical structures and were typically noted in areas suspected of ongoing active degeneration. CONCLUSION: BCR has characteristic structures in the outer nuclear layer. Although the incidence of the structure varies, it may be characteristic of retinal degeneration and can be found in many retinal degenerative diseases.

Post-cataract surgery visual disturbance in a retinitis pigmentosa patient with asteroid hyalosis.

A patient with retinitis pigmentosa showed visual disturbances following successful cataract surgery. He had a dense asteroid hyalosis in the eye before cataract surgery. After the surgery he noticed that his vision became worse. The visual disturbance was explained as being caused by the progression of retinal degeneration. Although the electroretinogram was non-recordable, the degeneration of macular area appeared relatively small. We considered that dense asteroid hyalosis was responsible for his visual disturbances, and pars plana vitrectomy (PPV) was performed to remove the asteroid hyalosis. After the PPV, rapid improvement of his visual acuity was observed. Cataract surgery may affect the status of asteroid hyalosis and cause rapid visual loss. PPV should be considered for retinitis pigmentosa patients with dense asteroid hyalosis, especially when a large decrease in visual acuity is noted shortly after cataract surgery.