Prevalence of serum MOG antibody and AQP4 antibody in optic neuritis after SARS-CoV-2 infection.

Purpose: To evaluate the prevalence of serum myelin oligodendrocyte glycoprotein antibody (MOG-Ab) and aquaporin-4 antibody (AQP4-Ab) in optic neuritis (ON) patients after severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection by cell-based indirect immunofluorescence assay (CBA). Methods: In this prospective case series study, 35 patients clinically diagnosed as ON and laboratory-confirmed SARS-CoV-2 infection from 8 December 2022 to 8 February 2023 were included. All patients' clinical and laboratory data were collected and analyzed. Results: The mean age of the 35 patients (46 eyes) was 38.2 years (ranging from 6 to 69 years), and 17 cases were female patients. Thirty-three and two cases showed positive SARS-CoV-2 RNA test results before or shortly after ON onset, respectively. ON occurred unilaterally in 24 cases and bilaterally in 11 cases. Ophthalmic examination revealed swollen optic disc in 37 eyes, normal optic disc in 6 eyes, and temporally or wholly paled optic disc in 3 eyes. CBA revealed seropositive MOG-Ab in 10 cases and AQP4-Ab in 2 cases, respectively, of which 2 AQP4-Ab-seropositive cases and 1 MOG-Ab-seropositive case had a past medical history of ON. Most ON patients showed a rapid and dramatic response to pulse steroid therapy. The median of BCVA at the onset and at the last follow-up was 20/500 (ranging from light perception to 20/20) and 20/67 (ranging from counting fingers to 20/20), respectively. Conclusion: Serum MOG-Ab and AQP4-Ab were detected in 28.6% (10/35) and 5.7% (2/35) ON cases after SARS-CoV-2 infection. SARS-CoV-2 infection may trigger an onset or a relapse of ON, as well as the production of MOG-Ab.

Case Report: Optic neuritis as the initial presentation of Orientia tsutsugamushi infection detected by metagenomic next-generation sequencing.

Scrub typhus is an acute febrile illness caused by Orientia tsutsugamushi infection, and typically manifested as fever, eschar, lymphadenopathy, rash, and other flu-like signs. Ocular involvement was not uncommon, and mostly occurred at post-fever or recovery stage in scrub typhus cases. We hereby report a case of scrub typhus presenting as unilateral optic neuritis (ON). A 56-year-old man going wild fishing nearly every day complained of a blurred vision and an inferior visual field defect in the right eye two or three days after an insect-bite like shin induration in his left leg. He was diagnosed as ON, and treated with dexamethasone in the local hospital. Unfortunately, his right eye vision progressively deteriorated during steroid therapy. Three days after steroid therapy ceased, he suffered from a high fever and painful subcutaneous masses in the left groin. Peripheral blood test by metagenomic next-generation sequencing (mNGS) was positive for Orientia tsutsugamushi, but negative for other pathogens. The diagnosis was then revised to scrub typhus and ON. His systemic symptoms rapidly disappeared after oral doxycycline and omadacycline therapy. However, his right eye vision continuously deteriorated to hand motion. Further serum tests for aquaporin 4-IgG antibody and myelin oligodendrocyte glycoprotein-IgG antibody were both negative, but for anticardiolipin IgM and beta-2-glycoprotein-I IgM were both positive. The patient's right eye vision gradually improved after doxycycline combined with steroid pulse therapy. Our case indicates that ON in scrub typhus cases may present as a parainfectious inflammation, and that mNGS is a useful and valuable method for early diagnosis of scrub typhus.

Detecting changes in the blood flow of the optic disk in patients with nonarteritic anterior ischemic optic neuropathy via optical coherence tomography-angiography.

Purpose: This study aimed to evaluate the changes in the blood flow of the optic disk in patients with nonarteritic anterior ischemic optic neuropathy (NAION) using optical coherence tomography-angiography (OCTA) and to investigate the relationship among the changes in the blood flow of the optic disk, visual field defect, peripapillary retinal nerve fiber layer (RNFL), and macular ganglion cell complex (mGCC). Methods: This was a prospective observational case series study. A total of 89 patients (89 eyes) with NAION were included in this study. All patients underwent best corrected visual acuity (BCVA), slit-lamp and direct ophthalmoscopic examinations, color fundus photography, visual field test, and blood flow indicators of the radial peripapillary capillaries (RPC) including whole en face image vessel density (VD), peripapillary VD by OCTA, the peripapillary RNFL, and mGCC by spectral-domain optic coherence tomography (OCT). The changes of blood flow in the optic disk at ≤3, 4-8, 9-12, 13-24, and >24 weeks of the natural course of NAION were measured, and the relationship among the changes in the blood flow of the optic disk, visual field defect, peripapillary RNFL, and mGCC was also analyzed. Results: The mean age of 89 patients with NAION was 56.42 ± 6.81 years (ranging from 39 to 79). The initial RPC whole en face image VD was significantly reduced after acute NAION (≤3 weeks) (F = 45.598, P < 0.001) and stabilized from the eighth week onward. Over the course of NAION, the superonasal RPC, superior mGCC, and superotemporal RNFL decreased mostly with time (F = 95.658, 109.787, 263.872, respectively; P < 0.001). Maximal correlations were found between superior mGCC and temporosuperior RPC in the NAION phase (R = 0.683, P < 0.01) and between superonasal RPC and superonasal RNFL (R = 0.740, P < 0.01). The mean defect was correlated with temporosuperior RPC (R = -0.281, P < 0.01) and superior mGCC (R = -0.160, P = 0.012). Conclusion: Over the course of NAION, OCTA shows a tendency toward change in the retinal capillary plexus of the optic disk. OCTA is proved to be a practical and useful tool for observing papillary perfusion in NAION.

Case Report: Severe Optic Neuritis after Multiple Episodes of Malaria in a Traveler to Africa.

Although local transmission of malaria has been eliminated, the disease is frequently imported to China by Chinese travelers returning from Africa. Optic neuritis (ON) is occasionally reported in malarial cases and usually shows good visual recovery and prognosis. Herein, we report severe visual loss with poor recovery due to bilateral ON in a malarial patient who traveled from Nigeria. While he was still in Nigeria, his visual acuity dropped to no light perception in both eyes after the third episode of malaria, which was confirmed by a positive blood smear for malarial parasites. His general condition gradually improved after a 6-day course of artesunate therapy. However, visual acuity in both eyes remained unchanged after artesunate therapy alone, with gradual improvement subsequently shown after pulse steroid therapy. Our case indicates that early antimalarial drugs combined with pulse steroid therapy may be of great importance for good visual recovery in ON cases after malarial infection.

Acute-Onset Visual Impairment in Wilson's Disease: A Case Report and Literature Review.

Objective: We reported the case of a patient with Wilson's disease (WD) with acute-onset visual impairment and summarized previously reported cases to make physicians aware of the complicated clinical expressions of WD and improve diagnosis efficiency. Methods: The patient was recruited from the Second Affiliated Hospital of Zhejiang University School of Medicine. Clinical data, including cranial images, laboratory tests, and ophthalmic findings were obtained. The PubMed database was searched for published cases of WD with visual impairment. Results: We reported a 22-year-old male who presented with hand tremor, personality change, and acute-onset binocular vision blurring. WD was considered to be closely correlated with neuropsychiatric and ocular involvements. After low-copper diet and regular copper-chelation therapy, the related symptoms improved compared to before. Six WD cases of optic neuropathy have been reported, including ours. The patients usually had neurological and/or hepatic symptoms for a period without any treatment. All the reported cases manifested as acute episodes of visual changes, and the ocular manifestations improved after copper-chelation treatment. Conclusions: Excess copper accumulation may be a rare cause of visual impairment in patients with WD. While the etiology behind patients' acute-onset visual impairment remained uncertain, the possibility of WD should be considered through neuropsychiatric and hepatic symptoms, corneal K-F rings, decreased serum ceruloplasmin, and low likelihood or exclusion of other causes. Clinicians need to recognize this rare manifestation and give appropriate treatment to avoid misdiagnosis and unnecessary overtreatment.

Demographic, Clinical and Laboratory Characteristics of Ocular Syphilis: 6-Years Case Series Study From an Eye Center in East-China.

Purpose: To report the demographic, clinical, and laboratory characteristics of ocular syphilis based on a 6-year case series study from an eye center in East-China. Methods: A total of 131 cases (191 eyes) of ocular syphilis and the annual number of total syphilis cases from January 2016 to December 2021, were included in this study. Detailed medical records including systemic and ophthalmic medical history, a complete ophthalmic examination, color fundus photography, B-type ultrasound, fundus fluorescein angiography (FFA), spectral domain optical coherence tomography (SD-OCT), laboratory tests of the serum and cerebrospinal fluid (CSF) samples, as well as visual field test and orbital or cranial MRI in cases with suspected optic neuritis or optic atrophy, were collected and analyzed. Pearson Chi-square or Fisher's exact tests was used for statistics analysis. Results: Of the 131 cases with ocular syphilis, 86 cases were in men and 6 cases had a past medical history or systemic manifestation of syphilis. HIV was found in only 2 of 70 cases undergoing serum HIV test. The average age was 54.0 years, ranging from 26-85 years. The average percentage of ocular syphilis out from the total syphilis cases was 5.1%, the average titer of serum rapid plasma regain (RPR) at presentation was 1:32, ranging from 1:1-1:512. The most predominant manifestation of ocular syphilis was posterior uveitis, followed by optic neuritis, optic atrophy, panuveitis, retinal vasculitis, and retinitis. The median of BCVA of all 191 eyes was 20/200 (ranging from no light perception to 20/20), and 20/40 (ranging from no light perception to 20/20) at presentation and final follow-up, respectively. Ocular syphilis with active inflammation responded well to penicillin therapy, no matter the initial visual acuity, ocular disease type, or RPR titers, as long as it was diagnosed early and treated properly and promptly. However, cases with optic atrophy, acute retinal necrosis, late diagnosis, permanent disruption, or loss of outer segment of photoreceptors of macular retina on SD-OCT showed poor visual improvement after therapy. Conclusions: Early diagnosis of ocular syphilis is still challenging in clinical practice and syphilis tests should be routinely performed in patients with uveitis, retinitis, optic neuritis, and optic atrophy.

Serum and Cerebrospinal Fluid Testing in Optic Neuropathy Patients with Malignant Tumors.

PURPOSE: To evaluate the value of serum and cerebrospinal fluid (CSF) testing in optic neuropathy (ON) patients with malignant tumors. METHODS: Fourteen patients clinically diagnosed as ON with malignant tumors but without intracranial or orbital mass in MRI were included in this study. Detailed medical records including medical history, complete ophthalmic examination, colour fundus photography, visual field test, orbital MRI examination, serum and CSF testing data were collected and analyzed. The diagnosis of paraneoplastic optic neuropathy (PON) based on the 2004 recommended criteria of the paraneoplastic syndrome- Euronetwork consortium for paraneoplastic neurological disorders, and current adaption for neuropathies. All patients underwent serum tests for pathogens and autoantibodies including antinuclear antibodies, anticardiolipin antibodies, antineutrophil cytoplasmic antibodies, AQP4-Ab and MOG-Ab, as well as CSF tests for malignant cells under microscope. Serum paraneoplastic antibodies were detected in PON patients. Monkey cerebellar tissue-based assay was used to detect unknown serum anti-neuron antibodies in PON patients with negative paraneoplastic antibody testing results. RESULTS: Fourteen ON patients were classified as four groups based on their clinical and MRI characteristics, as well as serum and CSF testing results: [1] definite PON, 6 cases (11 eyes); [2] possible PON, 3 case (5 eyes); [3] meningeal carcinomatosis-associated optic neuropathy (MCON), 4 cases (6 eyes); [4] infiltrative optic neuropathy (ION), 2 cases (2 eyes). Malignant cells were found under microscope in CSF samples from MCON and ION patients, contrast to no malignant cells in CSF samples from PON cases. All 14 ON patients with malignant tumors showed negative results in serum tests for pathogens and autoantibodies. Serum paraneoplastic antibodies were tested in PON patients, anti- CV2, anti-Yo, and anti- amphiphysin were detected positive in 2, 1, and 1 case, respectively, in definite PON group, whereas no serum paraneoplastic antibody detected in possible PON group. Two unknown serum antineuronal antibodies (an anti- Purkinje cell antibody and an anti-granular cell antibody) were detected using monkey cerebellar tissue-based assay in 2 of 5 PON patients with negative paraneoplastic antibody test results. CONCLUSIONS: Serum and CSF tests are of great importance in differentiating different subtypes of ON with malignant tumors. Current diagnosis of PON still depends on combination of clinical and MRI manifestations, as well as serum and CSF tests. Tissue-based assay may help to detect new biomarkers for ON etiology and diagnosis.

Optical Coherence Tomography Angiography-Based Quantitative Assessment of Morphologic Changes in Active Myopic Choroidal Neovascularization During Anti-vascular Endothelial Growth Factor Therapy.

Purpose: To establish quantitative profile of the morphologic changes among patients with active myopic choroidal neovascularization (mCNV) before and after anti-vascular endothelial growth factor (VEGF) therapy using optical coherence tomography angiography (OCTA) to assess the therapeutic response. Methods: Patients with active mCNV who received anti-VEGF injections between February 2017 to October 2020 and fit the study criteria were retrospectively reviewed. Quantitative analysis of their OCTA images were carried out to evaluate the morphologic features and vascular changes of mCNV lesions in response to anti-VEGF therapy. For further quantitative profiling, mCNV area, fractal dimension, vessel area, vessel density, vessel diameter, vessel length, vessel junction, junction density, and vessel tortuosity were obtained by means of advanced skeletonization postprocessing analyses. Results: Thirty-one eyes of 29 consecutive patients with OCTA-positive mCNV lesions (mean spherical equivalent: -12.55 ± 3.24 diopters) were included. The 31 cases were divided into two phenotypes at baseline: organized interlacing pattern (83.87%) and disorganized vascular loops pattern (16.13%). The values of mCNV area, fractal dimension, vessel area, vessel length, vessel junction, and junction density decreased remarkably 1 month after the initial anti-VEGF injection (p < 0.001). Although, vessel density, vessel diameter, and vessel tortuosity increased meanwhile, only vessel diameter displayed statistical significance (p = 0.027). Of note, relative ratio analysis showed that vessel junction was the most sensitive biomarker in response to anti-VEGF therapy, reflecting a mean decrease of 50.36%. Sensitivity lowered successively in biomarkers of vessel length, vessel area, junction density, mCNV area, and fractal dimension. In addition, percent change of mCNV area (r = 0.552, p = 0.002), fractal dimension (r = 0.446, p = 0.017), vessel area (r = 0.518, p = 0.005), and vessel length (r = 0.440, p = 0.019) were moderately associated with that of central retinal thickness. Conclusions: The study showed morphological as well as quantitative changes on OCTA responding to anti-VEGF treatment in mCNV patients, among which vessel junctions might be the most predictive biomarker. OCTA-based analysis, providing intuitive images and a large spectrum of quantitative data at the same time, could promote new insights into the therapeutic response assessment in mCNV patients.

Mitochondrial 13513G>A Mutation With Low Mutant Load Presenting as Isolated Leber's Hereditary Optic Neuropathy Assessed by Next Generation Sequencing.

Objective: Mitochondrial 13513G>A mutation presenting as isolated Leber's hereditary optic neuropathy (LHON) without any extraocular pathology has not been reported in literature. We herein evaluate the clinical characteristics and heteroplasmy of m.13513G>A mutation manifesting as isolated LHON. Methods: Seven members of a Chinese family were enrolled in this study. All subjects underwent detailed systemic and ophthalmic examinations. Mitochondrial DNA in their blood was assessed by targeted PCR amplifications, next generation sequencing (NGS), and pyrosequencing. One hundred of blood samples from ethnic-matched healthy volunteers were tested by NGS and pyrosequencing as normal controls. Results: Isolated LHON without any other ocular or extraocular pathology was identified in a 16 year old patient in this family. Heteroplasmic m.13513G>A mutation was detected by NGS of the full mtDNA genome in the patient with mutant load of 33.56%, and of 26% 3 months and 3 years after the onset of LHON, respectively. No m.13513G>A mutation was detected in all his relatives by NGS. Pyrosequencing revealed the mutant load of m.13513G>A mutation of the LHON patient, his mother, father and sister were 22.4, 1.9, 0, and 0%, respectively. None of 100 healthy control subjects was detected to harbor m.13513G>A mutation either by NGS or by pyrosequencing of the full mt DNA genome. Conclusions: We first report m.13513G>A mutation with low mutant load presenting as isolated LHON. NGS of the full mitochondrial DNA genome is highly recommended for LHON suspects when targeted PCR amplification for main primary point mutations of LHON was negative.

Role of the Eye in Transmitting Human Coronavirus: What We Know and What We Do Not Know.

The outbreak of the current 2019 novel coronavirus (2019-nCoV, now named SARS-CoV-2) infection has become a worldwide health threat. Currently, more information is needed so as to further understand the transmission and clinical characteristics of 2019-nCoV infection and the infection control procedures required. Recently, the role of the eye in transmitting 2019-nCoV has been intensively discussed. Previous investigations of other highly infectious human CoVs, that is, severe acute respiratory syndrome coronavirus (SARS-CoV) and the Middle East respiratory syndrome coronavirus (MERS-CoV), may provide useful information. In this review, we describe the genomics and morphology of human CoVs, the epidemiology, systemic and ophthalmic manifestations, and mechanisms of human CoV infection, and recommendations for infection control procedures. The role of the eye in the transmission of 2019-nCoV is discussed in detail. Although the conjunctiva is directly exposed to extraocular pathogens, and the mucosa of the ocular surface and upper respiratory tract are connected by the nasolacrimal duct and share the same entry receptors for some respiratory viruses, the eye is rarely involved in human CoV infection, conjunctivitis is quite rare in patients with 2019-nCoV infection, and the CoV RNA positive rate by RT-PCR test in tears and conjunctival secretions from patients with 2019-nCoV and SARS-CoV infection is also extremely low. This suggests that the eye is neither a preferred organ of human CoV infection nor a preferred gateway of entry for human CoVs for infecting the respiratory tract. However, pathogens that the ocular surface is exposed to might be transported to nasal and nasopharyngeal mucosa by constant tear rinsing through the lacrimal duct system and then cause respiratory tract infection. Considering that close doctor-patient contact is quite common in ophthalmic practice and is apt to transmit human CoVs by droplets and fomites, strict hand hygiene and proper personal protection are highly recommended for health care workers to avoid hospital-related viral transmission during ophthalmic practice.

Macular hole retinal detachment after intravitreal Conbercept injection for the treatment of choroidal neovascularization secondary to degenerative myopia: a case report.

BACKGROUND: We report a case of macular hole (MH) formation and retinal detachment after intravitreal conbercept injection for the treatment of choroidal neovascularization (CNV) secondary to degenerative myopia. CASE PRESENTATION: A 60-year-old woman presented with blurred vision in her left eye was diagnosed as CNV secondary to degenerative myopia. Intravitreal injection of conbercept, an anti -vascular endothelial growth factor (VEGF) agent, was uneventfully performed in the left eye. Unfortunately, a full thickness MH and retinal detachment was found three weeks postoperatively by ophthalmoscopy and spectral-domain optical coherence tomography. Vitrectomy, internal limiting membrane peeling and silicone oil tamponade were then performed, and macular retina was reattached soon after surgery. However, MH still kept open during three months' follow-up. CONCLUSION: MH is a quite rare complication of intravitreal anti- VEGF agent injection, tangential contraction secondary to CNV shrinkage and regression caused by anti-VEGF agent is proposed to be the major pathogenesis of MH formation.

Myopic Macular Retinoschisis in Teenagers: Clinical Characteristics and Spectral Domain Optical Coherence Tomography Findings.

To investigate the morphological characteristics of myopic macular retinoschisis (MRS) in teenagers with high myopia, six male (9 eyes) and 3 female (4 eyes) teenagers with typical MRS identified from chart review were evaluated. All cases underwent complete ophthalmic examinations including best corrected visual acuity (BCVA), indirect ophthalmoscopy, colour fundus photography, B-type ultrasonography, axial length measurement, and spectral-domain optical coherence tomography (SD-OCT). The average age was 17.8 ± 1.5 years, average refractive error was -17.04 ± 3.04D, average BCVA was 0.43 ± 0.61, and average axial length was 30.42 ± 1.71 mm. Myopic macular degenerative changes (MDC) by colour fundus photographs revealed Ohno-Matsui Category 1 in 4 eyes, and Category 2 in 9 eyes. Posterior staphyloma was found in 9 eyes. SD-OCT showed outer MRS in all 13 eyes, internal limiting membrane detachment in 7 eyes, vascular microfolds in 2 eyes, and inner MRS in 1 eye. No premacular structures such as macular epiretinal membrane or partially detached posterior hyaloids were found. Our results showed that MRS rarely occurred in highly myopic teenagers, and was not accompanied by premacular structures, severe MDC, or even obvious posterior staphyloma. This finding indicates that posterior scleral expansion is probably the main cause of MRS.

Self-assembly and morphological transitions of random amphiphilic poly(ß-D-glucose-co-1-octyl) phosphazenes.

The amphiphilic random copolymer poly(ß-d-glucose-co-1-octyl)phosphazene (PGOP) can undergo continuous morphological transitions in DMF-water mixed solvents. In this study, the ratio of glucose moieties to octyl moieties was controlled via a two-step thiol-ene reaction. As a result, polyphosphazenes with glycosyl functionalization degrees of 58.1% (PGOP-1), 74.1% (PGOP-2) and 87.0% (PGOP-3) were obtained. These amphiphilic polyphosphazenes self-assemble in both water and water-DMF mixtures. Several self-assembled morphologies including spheres, rods and vesicles were formed though careful control of the water content (WC) in the DMF solvent as well as of the hydrophilicity or hydrophobicity of the copolymers. We also found that an increase in the hydrophobic proportion led to faster morphological transitions at a constant WC. The thermodynamics of micellization were also studied by Isothermal Titration Calorimetry (ITC), and the strong hydrophobic interactions in PGOP-1 were demonstrated by their highly exothermic nature. These self-assemblies have potential applications in biosensing, lectin adsorption and drug loading with controlled release.

The effect of anti-TGF-ß2 antibody functionalized intraocular lens on lens epithelial cell migration and epithelial-mesenchymal transition.

Migration and epithelial-mesenchymal transition (EMT) of lens epithelial cells (LECs) are main causes of central posterior capsule opacification after cataract extraction combined with intraocular lens (IOL) implantation. In this study, commercially available hydrophobic acrylic IOLs were first pretreated with atmospheric pressure glow discharge plasma to produce plenty of negatively charged chemical groups onto IOL surface, then polyethylenimine was deposited onto IOL surfaces as a precursor monolayer, and then anti-TGF-ß2 (anti-T) antibody and poly-l-lysine were sequentially deposited onto IOL surface for four cycles followed by another upmost monolayer of anti-T antibody via layer-by-layer self-assembly technique. After the fabrication of anti-T antibody multilayers on IOL surface, the surface characteristics of the anti-T antibody functionalized IOL, as well as its effect on LECs adhesion, proliferation, migration and EMT were then tested in this study. Our results revealed that anti-T antibody multilayers could be successfully immobilized onto IOL surfaces by plasma pretreatment and layer-by-layer self-assembly technique, and could keep stable for at least 3 months on IOL surface. The anti-T antibody immobilized in the multilayers on IOL surfaces showed good immunological activity by its specific antigen-antibody interaction with exogenous TGF-ß2. Anti-T antibody functionalized IOL surface was as smooth and flat as the untreated IOL surface. No difference in optical or physical properties was found between the anti-T antibody functionalized IOLs and the untreated IOLs. Compared with the untreated IOLs, the anti-T antibody functionalized IOL greatly inhibited LECs from migration and EMT, yet showed only transient inhibition to LECs adhesion and no inhibition to LECs proliferation. With these data, we demonstrate a simple, inexpensive, and feasible method to fabricate surface functionalized IOL for in situ capture and neutralization of TGF-ß2 in the capsular bag, which might be a possible solution to preventing posterior capsule opacification after cataract surgery.

[Measurement of retinal nerve fiber layer thickness in Leber hereditary optic neuropathy by optical coherence tomography].

OBJECTIVE: To evaluate retinal nerve fiber layer (RNFL) thickness in patients and unaffected carriers of Leber hereditary optic neuropathy (LHON) by optical coherence tomography (OCT). METHODS: This case-control study enrolled 42 LHON maternal family members with mitochondrial DNA G11778A mutation and 100 normal volunteers. RNFL thickness was measured by Stratus OCT in each participant. Mean RNFL thickness of each quadrant, as well as 360° average were calculated and compared in normal controls, LHON carries and LHON patients. RESULTS: Among LHON maternal family members, 15 cases were unaffected carriers who were subgrouped as normal-fundus-appearing carriers (10 cases) and preclinical carriers (5 cases). Twenty seven LHON patients included 9, 5, and 13 cases in the early, advancing and advanced stages, respectively. Normal fundus-appearing carriers showed normal RNFL thickness of each quadrant and 360° average. Preclinical carriers and early-staged patients showed no significant difference in RNFL thickness of each quadrant and 360° average (P = 0.138 to 0.645), yet both showed thicker RNFL in temporal, superior and inferior quadrant, as well as 360° average, if compared with normal controls (P = 0.000 to 0.018). Compared with normal controls, preclinical carriers and early-staged patients, advancing LHON patients showed thinner RNFL in temporal and inferior quadrant, as well as 360° average (P = 0.000 to 0.005). Advanced LHON patients showed thinner RNFL in each quadrant and 360° average, compared with normal controls, LHON carriers, and advancing cases (P = 0.000 to 0.037). CONCLUSIONS: RNFL thickness in LHON patients and unaffected carriers was characterized by OCT in this study, which would improve the understanding of the natural course of LHON.

Anterior internal lenticonus accompanied by congenital nuclear cataract.

Internal lenticonus is a very rare morphologic abnormality of crystalline lens which has been reported in only several cases in the literature. We herein reported the clinical characteristics and surgical findings of the anterior internal lenticonus accompanied by congenital nuclear cataract. Cataract extraction accompanied with intraocular lens implantation was uneventfully performed, and a good visual outcome was achieved in this case. Viral infection during embryonal and fetal period might account for the formation of the anterior internal lenticonus and congenital nuclear cataract in our case.