Objective To observe the characteristics of indocyanine green angiography (ICGA) in inactive polypoidal lesions of polypoidal choroidal vasculopathy (PCV). Methods The clinical data of 36 PCV patients (37 eyes) with inactive polypoidal lesions were retrospectively analyzed. The follow-up of 11 eyes were ranged from nine to 29 months, with a mean of (12.3plusmn;5.5) months. All the patients were examined for visual acuity, intraocular pressure, slit lamp microscope, fundus photography, fundus fluorescein angiography (FFA) and ICGA. According to the ICGA characteristics, PCV lesions were divided into active polypoidal lesions (pocket like hyperfluorescence at early stage and fluorescence leakage or stained with fluorescein at late stage) and inactive polypoidal lesions (pocket like hyperfluorescence and it was gradually faded). According to clinical and ICGA characteristics, inactive polypoidal lesions were divided into asymptomatic group, atrophic and/or cicatricial group and combined (with active polypoidal lesions) group. The visual acuity, fundus, lesions change and image characteristics of three groups were evaluated and analyzed. Results Among the 37 eyes, the time from indocyanine green (ICG) injection to inactive polypoidal lesions begin showing was ranged from 8.2 to 27.0 minutes, with a mean of (15.5plusmn;4.8) minutes. There were five eyes (13.5%), eight eyes (21.6%) and 24 eyes (64.9%) in asymptomatic group, atrophic and/or cicatricial group and combined group, respectively. The results of fundus examination showed that there was no hemorrhage, exudates, retinal pigment epithelium detachment (PED) and/or neural retina detachment in asymptomatic group; atrophy lesions and/or scar lesions were observed in atrophic and/or cicatricial group and there was also no hemorrhage, exudate, PED and/or neural retina detachment; there was no atrophy lesion and/or scar lesion, but there were 10 eyes with subretinal hemorrhage, 15 eyes with retinal exudate, 10 eyes with PED and four eyes with neural retina detachment in combine group. The results of ICGA showed that there were inactive polypoidal lesions in asymptomatic group; inactive polypoidal lesions located at the border of atrophy lesions and/or scar lesions in atrophic and/or cicatricial group; active polypoidal lesions and inactive polypoidal lesions coexisted in combine group. In 11 eyes which completed the follow-up, inactive polypoidal lesions regressed in three eyes (27.3%), partial regressed in two eyes (18.2%), unchanged in six eyes (54.5%). Conclusions The inactive polypoidal lesions of PCV mainly appear in the middle or late stage of ICGA and are manifested in asymptomatic, atrophic and/or cicatricial and combined eyes. The combined type which coexisted with active polypoidal lesions is the main form.
ObjectiveTo observe the clinical features and visual prognostic factors of ethambutol-induced optic neuropathy (EON).MethodsA cohort study. Twenty-four inpatients (46 eyes) identified as EON in Neuro-Ophthalmology Department of Chinese PLA General Hospital from January 2014 to December 2017 were enrolled, including 14 males (26 eyes) and 10 females (20 eyes) with a ratio of 1.4/1 male/female. The average age was 42.79±15.12 years and the average weight was 62.46±12.31 kg. The average time duration between oral administration of ethambutol and occurrence of EON was 9.94±16.49 months. The average time of ethambutol duration was 7.06±11.68 months, with an average accumulative dose of 156.7±1 779.0 g and the average daily dose of 15.07±8.95 mg/(kg·d). All patients were tested with visual acuity, fundus photos, colour vision, OCT, visual field, VEP, orbital MRI and the gene of OPA1 and mitochondrial deoxyribonucleic acid (mtDNA). All the patients accepted drug withdrawal immediately after diagnosis, and were given the treatment of systemic nerve nutrition and improvement of microcirculation for 2 weeks. The time of follow-up was more than 12 months. According to whether the visual acuity (VA) in any of eyes was over than 0.1 at the last follow-up, all the patients were divided into two groups: the bad VA group (VA less than or equal to 0.1) and the better VA group (VA over than 0.1) group. The χ2 test and Fisher's exact probabilistic method test were used to compare the counting data between groups, and the Wlincox rank sum test was used to compare the measurement data. Multiple factors of VA outcome between the patients with bad or better va were analyzed by logistic regression.ResultsThirty eyes (65.2%) had VA less than or equal to 0.1 and 5 eyes (10.9%) had VA over than 0.5 at EON onset. The VA of the rest 11 eyes (23.9%) was higher than 0.1 and lower than 0.5. At the last follow-up, 20 eyes (43.5%) had VA less than or equal to 0.1 and 9 eyes (19.6%) had VA over than 0.5, the VA of the rest 17 eyes (36.9%) was higher than 0.1 and lower than 0.5. Fundus examination revealed 7 eyes (15.3%) with optic disc edema. OCT revealed significant loss of the retinal nerve fiber layer (RNFL) in the affected eyes, mainly in the temporal RNFL of the optic disc. All patients had dyschromasia, mainly in distinguishing the color of red and green. The types of visual field defect was as following: central dark spot (52.2%), diffuse visual acuity decreased (30.4%), temporal hemianopsia (17.4%). Orbital MRI revealed that 12/24 (50.0%) patients had T2 lesions with T1 enhancement in 6/24 patients (25.0%). Genetic test showed that 4 patients (16.7%) had gene mutation. Among them, there were 2 patients with OPA1 mutation, 1 with mtDNA 14340 point mutation and 1 with the mtDNA 11778 point mutation. Thirteen patients showed better VA outcomes (over than 0.1) while 11 showed bad VA outcomes after discontinuation of ethambutol. Between the better VA group and the bad VA group, there were statistically significant differences in the daily dose of ethambutol and gene mutation (P=0.031, 0.023). The daily dose was related to visual prognosis of EON while only the daily dose of more than 18 mg/(kg·d) may lead to bad VA outcomes according to the logistic analysis (95% CI 0.007-0.736, OR=0.069, P=0.027).ConclusionsEON may have OPA1 and mtDNA mutation with more bilateral eyes involved and less optic edema, which about 43.5% of the patients showed irreversible visual impact. The daily dose of ethambutol is related to the vision recovery.
The research on brain functional mechanism and cognitive status based on brain network has the vital significance. According to a time–frequency method, partial directed coherence (PDC), for measuring directional interactions over time and frequency from scalp-recorded electroencephalogram (EEG) signals, this paper proposed dynamic PDC (dPDC) method to model the brain network for motor imagery. The parameters attributes (out-degree, in-degree, clustering coefficient and eccentricity) of effective network for 9 subjects were calculated based on dataset from BCI competitions IV in 2008, and then the interaction between different locations for the network character and significance of motor imagery was analyzed. The clustering coefficients for both groups were higher than those of the random network and the path length was close to that of random network. These experimental results show that the effective network has a small world property. The analysis of the network parameter attributes for the left and right hands verified that there was a significant difference on ROI2 (P = 0.007) and ROI3 (P = 0.002) regions for out-degree. The information flows of effective network based dPDC algorithm among different brain regions illustrated the active regions for motor imagery mainly located in fronto-central regions (ROI2 and ROI3) and parieto-occipital regions (ROI5 and ROI6). Therefore, the effective network based dPDC algorithm can be effective to reflect the change of imagery motor, and can be used as a practical index to research neural mechanisms.
Objective To observe the hereditary types and clinical characteristics of 137 patients with retinitis pigmentosa (RP) in Ningxia. Methods One hundred and thirty-seven patients with RP who diagnosed by the examinations of visual acuity, optometry, direct or indirect ophthalmoscope, visual field, optical coherence tomography (OCT) and electroretinogram were enrolled. The hereditary types and clinical characteristics were analyzed according to the family history and the Results of ophthalmologic examinations. Results One hundred and thirty-seven patients included 29 autosomal dominant RP (ADRP) patients from 8 families (7.4%), 16 autosomal recessive RP (ARRP) patients from 15 families (13.9%), 10 X-linked RP (XLRP) from 3 families (2.8%), and 82 simplex RP (SRP) patients (75.9%). There were 15 consanguineous marriage families out of 26 families with RP history (57.7%). The patients were classified as typical RP (102 patients, 74.5%) and atypical RP (35 patients, 25.5%). All the ADRP and XLRP patients showed typical clinical features of RP. Ten (62.5%) of ARRP patients and 53 (64.6%) of SRP patients had typical features of RP. Six (37.5%) of ARRP patients and 29 (35.4%) of SRP patients had atypical features of RP. Among atypical RP patients, 17 (48.6%) patients were nonpigmented RP which including 3 patients were misdiagnosed as amblyopia during childhood. The logarithm of minimal angle of resolution (logMAR) best corrected visual acuity (BCVA) of ADRP patients was 1.04plusmn;0.51 at the age older than 51 years, while the BCVA of ARRP and XLRP patients were 0.92plusmn;0.61 and 1.70plusmn;0.02 respectively at 21 to 30 years of age. One hundred and twentythree (89.8%) patients suffered from varying degrees of myopia. OCT showed that the average thickness of macular fovea in ADRP patients was (185.73plusmn;1.23) mu;m at the age older than 51 years, while in ARRP and XLRP patients were (173.21plusmn;0.98) and (170.49plusmn;1.15) mu;m respectively at 21 to 30 years of age. Conclusions ADRP and XLRP are typical RP. All atypical RP are ARRP and SRP. Non-pigmented RP are mainly seen in atypical RP which often misdiagnosed as amblyopia during childhood. The photoreceptors in macula are damaged in the early stage and the decline of visual acuity occurred at 21 to 30 years of age in patients with ARRP and XLRP. The ADRP patients has late slower decline of visual acuity and retain some visual acuity at the age older than 51 years.
Objective To observe the fundus characteristics of human immunodeficiency virus with acquired immune deficiency (HIV/AIDS). Methods A total of 1041 HIV/AIDS patients were enrolled in this study. The patients included 882 males (88.70%) and 159 females (11.30%). The patientsprime; ages ranged from 12 to 73 years, with a mean age of 41 years. The median time of HIV/AIDS diagnosis was 12 months, which ranged from one month to 10 years. HIV infection was acquired through sexual contact, intravenous drug use, blood transfusion or mother-to-child transmission in 475 patients (45.63%), 508 patients (48.80%), 44 patients (4.25%) and 14 patients (1.34%), respectively. Ocular examinations (vision acuity, slit lamp microscope and fundus examination) were performed on recruited patients with HIV/AIDS. Additional exams (intraocular pressure, fundus photography and fundus fluorescein angiography) were done if abnormal ocular fundus was found. The ocular manifestations were diagnosed according to clinic reference. Results Ocular manifestations of HIV/AIDS were detected in 247 patients (23.73%). Of 247 patients, the most common ocular manifestation was HIV retinopathy, which was present in 132 patients (53.44%); cytomegalovirus retinitis (CMVR) was second place, affecting 70 participants (28.34%). Clinic findings of HIV retinopathy included retina microaneurysm, hemorrhage along the blood vessel with cotton-wool spots, while irregular dry edge, granular appearing border, were present in CMVR, and the optic nerve may be affected. Fluorescein angiogram of HIV retinopathy demonstrated that hemorrhage was shown as sheltered fluorescence, with b fluorescence without leakage in center of hemorrhage. Fluorescein angiogram of CMVR demonstrated significant hemorrhage appearing as sheltered fluorescence with leakage and/or transparent fluorescence. The optic disk and lesioned area were stained with fluorescence. Conclusions There are various HIV/AIDS related ocular manifestation. HIV retinopathy and CMVR are common ocular manifestations. The main clinical findings of HIV retinopathy are hemorrhage and/or cotton-wool spots, while irregular granular appearing edges and hemorrhage were observed in CMVR.
ObjectiveTo observe the clinical characteristics of retinoblastoma (RB) in Southwest China.MethodsA retrospective clinical study. From January 2010 to December 2017, 66 RB patients diagnosed in Ophthalmology Department of West China Hospital of Sichuan University were included in the study. All the patients underwent ocular B-ultrasound, orbital CT or MRI examination. Ten patients underwent RetCam examination at the same time. Twenty-nine patients were diagnosed by histopathological examination, and 37 patients were diagnosed by clinical symptoms and imaging examination. According to whether the tumor invaded the orbit and optic nerve, it could be divided into extraocular stage and intraocular stage. Intraocular tumors were divided into A-E stages according to the international intraocular RB classification. Treatments were performed according to different stages. The general information, age at diagnosis, course of diseases (the time between onset symptoms and diagnosis), causes of visiting a doctor, classification, treatment methods and eyeball preservation rate were retrospectively analyzed.ResultsPatients all came from Southwest China (56 patients from Sichuan Province, 2 patients from Yunnan Province, 2 patients from Guizhou Province, and 6 patients from Tibet). The permanent residence were identified in 43 patients, including 27 patients (62.8%) from rural areas. There were 38 males (57.6%); 50 unilateral tumors (75.8%) and 16 bilateral tumors (24.2%); 51 first-visiting patients (77.3%) and 15 re-visiting patients (22.7%). The average diagnostic age of first-visiting patients was 20.9±14.4 months, with 23.2±14.7 and 11.2±7.6 months for unilateral and bilateral tumors, respectively. There were 41 patients had definite course and causes, of whom the average course was 90.6±115.2 days. The most common cause was leucocoria in 32 patients (62.7%), followed by redness and swelling in 4 patients (9.8%), and other causes in 5 patients (12.2%). Among the 15 re-visiting patients, the average diagnostic age was 63.6±46.8 months, the average course was 32.8±45.5 months. Recurrence was occurred in 5 patients (33.3%), leucocoria in 4 patients (26.7%), postoperative complication in 3 patients (20.0%), protrusion in 2 patients (13.3%) and redness in 1 (6.7%) patient, respectively. Fifty out of 82 eyes were admitted to hospital, including 37 eyes of first-visiting patients and 13 eyes of re-visiting patients. Among 37 first-visiting eyes, there were 5 eyes (13.5%) in stage A-C, 26 eyes (70.3%) in stage D-E, 6 eyes (16.2%) in extraocular stage. Five eyes in stage A-C were treated with laser photocoagulation and (or) cryotherapy combined with systemic chemotherapy. Four eyes in stage D were treated with intraocular arterial chemotherapy. Nineteen eyes (51.3%) were performed with enucleation, 2 eyes (5.4%) with evisceration and 7 eyes (18.9%) abandoned treatment. Among 13 re-visiting eyes, 6 eyes (46.2%, with 5 eyes of recurrence) had been enucleated before, 4 eyes (30.8%) were in extraocular stage and 3 eyes (23.1%) in stage D-E. Five eyes (38.5%) were treated with evisceration, 4 eyes (30.8%) with enucleation, 1 eye with oculoplastic surgery and 3 eyes (23.1%) abandoned treatment. The rate of eye preservation was 18.0%, 29.0% for intraocular stage and 0% for extraocular stage, respectively.ConclusionRB patients in Southwest China have a longer course between onset symptoms and diagnosis, more advanced classification and lower rate of eye preservation.
Objective To observe the fundus characteristics of acquired immune deficiency syndrome (AIDS) with human immunodeficiency virus (HIV) retinopathy. Methods Eighty eyes of 52 AIDS patients with HIV retinopathy were enrolled in this study. The patients included 42 males (67 eyes) and 10 females (13 eyes). The patients ages ranged from 16 to 78 years, with a mean age of (43plusmn;12) years. All patients' visual acuity, intraocular pressure, slit-lamp microscopy and mydriatic indirect ophthalmoscopy, fundus color photography and CD4+ T cell count was documented. Experienced ocular fundus doctors carried out fundus examinations. Retinopathy characteristics were recorded. Seventeen patients (24 eyes) were followed for a period between two days to two years, with a median of 125 days. We failed to follow up the remaining 35 patients (56 eyes) due to death or moving away. Results Among 52 patients (80 eyes), 28 patients (56 eyes, 70.0%) had bilateral HIV retinopathy and 24 patients (24 eyes, 30.0%) had unilateral HIV retinopathy. Cotton-wool spots (CWS), mostly located close to temporal peripapillary vessels, were found in 46 patients (72 eyes, 90.0%). Six patients (eight eyes, 10.0%) were found to have flaming or spotting hemorrhage located in posterior pole. Among 72 eyes with CWS, 57 eyes were found to have CWS only and 15 eyes were found to also have retinal hemorrhage, mostly located near CWS. Among 24 eyes of 17 followed-up patients, three eyes of three patients were found with no significant changes during the less than two week follow-up. In 18 eyes of 11 patients, CWS or hemorrhage disappeared after one to three months without treatment and in five eyes new CWS or hemorrhage were found in other parts of the posterior pole. Three eyes of three patients initially considered as lint plaque-like lesions were eventually detected with CMVR as lesions during one to five months follow-up. Conclusion CWS are the most common ocular lesions in HIV retinopathy.
Autosomal recessive Best disease (ARB) is a rare clinical fundus disease caused by BEST1 mutation. The critical features of ARB are the presence of multifocal subretinal yellowish lesions, which corresponding to the hyperfluorescent spots on FAF, scattered over the posterior pole of the retina, absent of typical vitelliform lesions in the macula. Imaging of OCT is often manifested as subretinal or intraretinal fluid, and cystoid macular edema, and hypereflective focus at RPE level. EOG shows an absent or severely reduced light rise (decreased value of Arden), which often accompanied by reduction and delay of the rod and cone ERG. Some patients with ARB show hyperopia, short axial length and shallow anterior chambers, with a corresponding high incidence of angle-closure glaucoma. Though there isn't any effective therapeutic methods of ARB at present, prevention and treatment for its complications such as angle-closure glaucoma and choroidal neovascularization should be considered. Present study about ARB mainly focus on some retrospective cases, and ARB is often misdiagnosed with Best vitelliform macular dystrophy, central serous chorioretinopathy and other fundus diseases in clinic. A detailed understanding of the clinical features and genetic characteristics of ARB might be helpful in clinical diagnosis and treatment. Research with larger sample size are expected to further investigate the different stages of ARB and its developing process, the potential pathological mechanism, the relationship between genotype and phenotype, so as to improve the understanding of the disease.
Objective
To observe the clinical characteristics of demyelinating optic neuritis (DON) in Chinese children under the age of 16.
Methods
A retrospective review of the medical charts of 42 pediatric patients with DON was conducted in this study. Twenty-two patients (52.4%) were male, and 20 patients (47.6%) were female. The patients aged from 3 to 15 years, with the mean age of (9.5±2.3) years. There were 35 bilateral patients and 7 unilateral patients. Twenty-seven patients (64.3%) had prodromal symptoms before onset. All patients underwent visual function and imaging tests, such as best corrected visual acuity (BCVA), fundus photography, visual evoked potential (VEP), visual field, MRI. The patients were tested for serum levels of antibodies for aquaporin 4 (AQP4) and myelin oligodendrocyte glycoprotein (MOG) with a cell-based assay. All patients were received corticosteroid therapy. The mean follow-up was (1.17±0.42) years. The children who had coordination ability and with BCVA≥0.3 were received examination of Humphery automatic perimeter. Data were collected on the age, gender, clinical features, neuroimaging, serological specific antibodies, treatment and vision prognosis.
Results
23.8% of the children were bilateral optic neuritis in onset stages. 64.2% were recurrent optic neuritis and 83.3% exhibited bilateral diseases eventually. BCVA had decreased to ≤0.1 in 87.0%% eyes and disc swelling was observed in 77.9% eyes during the onset stages. All eyes had visual field defects and abnormal VEP exam results, with delayed latency of P100 and P2, and varying degrees of amplitude reduction. Serum AQP4 antibody and MOG antibody were tested by cell-based assay, 2/42 children (4.7%) were positive for AQP4 antibody and 5/24 children (20.8%) were positive for MOG antibody. All of anti-AQP4+ and anti- MOG+ cases relapsed. All children underwent orbital magnetic resonance imaging (MRI), 40 cases (95.2%) showed demyelination features of optic nerve, and 5 cases (11.9%) showed long segments lesion (more than 1/2 length of the optic nerve). There were 2 anti-AQP4+ cases and 3 anti- MOG+ cases from the 5 cases with long segments lesion. MRI also showed brain demyelinating lesions in 4 children (3 of them were anti- MOG+) or spinal cord demyelinating lesions in 3 children (2 of them were anti- MOG+). After treatment with glucocorticoid, visual acuity improved in all eyes, of which 84.4% with BCVA≥0.5. Forty-eight eyes of 26 children accept dynamic visual field during the course of treatment, showed the vision abnormalities associated with optic nerve damage.
Conclusions
Children under the age of 16 with DON can experience severe visual impairment, higher recurrence tendencies, and higher rate of disc involvement, but good response to glucocorticoid therapy. AQP4 or MOG antibodies positive might be concurrent with brain and (or) spinal cord demyelinating lesions and indicated a poorer prognosis.
Objective To observe the clinical features of systemic lupus erythematosus (SLE) with retinopathy.Methods Ninety-seven SLE patients were enrolled in this comparative clinical study. The patients were divided into retinopathy group (positive group, 32 eyes of 23 patients) and non-retinopathy group (negative group, 148 eyes of 74 patients). The age, course of disease, clinical features, laboratory results in these two groups were comparatively analyzed.Results The positive rate of retinopathy in all SLE patients was 23.7%. Seventeen patients (22 eyes, 73.9%) of positive group had retinal cotton-wool spot, retinal hemorrhage, tortuous retinal vein, retinal arterial spasm, microaneurysm and hard exudates. The other six patients (10 eyes, 26.1%) in this group showed retinal main vessel occlusion. The incidence rate of rash, cutaneous vasculitis, elevated erythrocyte sedimentation rate (ESR), decreased complement C3 and positive anti double stranded-DNA (anti-ds-DNA) antibody in the positive group were higher than those in the negative group (chi;2=9.206, 6.987, 7.824, 8.581, 6.599;P＜0.05). There was no significant difference between these two groups in age, course of disease, mucosal ulcers, arthritis, fever, headache, neutropenia, thrombocytopenia, proteinuria, elevated blood urea nitrogen, increased creatinine, positive antinuclear (ANA) and anti-Sm antibodies (t=0.321, 0.063;chi;2=0.135, 0.046, 0.176, 0.002, 0.036, 0.113, 0.053,0.032,0.012,0.000,0.004;P＞0.05). Conclusions Tortuous retinal veins, retinal cotton-wool spots and retinal main vessels occlusion are the three major fundus features of SLE patient with retinopathy. Rash, cutaneous vasculitis, increased ESR, decreased complement C3 and positive anti-ds-DNA antibody are the five major systemic clinical features of SLE patient with retinopathy.
