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 clinical features and treatment outcomes of presumed tubercular retinal vasculitis. Methods This is a retrospective non-comparative interventional clinical research. A total of nine patients (11 eyes) with major presentation of retinal vasculitis were included in this study. Patients first consulted the eye clinic and were diagnosed presumed tubercular retinal vasculitis. The patients, seven males and two females, aged from 19 to 66 years, with an average of 43.89 years. The time interval from symptoms to diagnosis ranged from two weeks to six months with an average of 76.27 days. Visual acuity, slit lamp ophthalmoscopy, fundus fluorescein angiography (FFA), optical coherence tomography (OCT), hematological and tuberculosis related investigations were examined and analyzed. All patients had standard anti-tuberculosis treatment. Treatment outcomes were followed for six to 37 months with an average of 14.11 months. Results Baseline visual acuity ranged from hand movement to 0.8 with an average of 0.28. Among 11 eyes, six presented mild to moderate vitritis, five presented as retinal vein occlusion with no obvious vitirits. Fundus examination showed six cases with retinal hemorrhage, four cases with macular edema, two with macular epiretinal membrane, and two with vitreous hemorrhage. FFA revealed 11 cases with leakage of vessels, 11 with nonperfusion area, four with macular edema, three with retinal neovascularization, and two with choroidal lesions. OCT of nine eyes suggested six eyes with retinal edema, three with macular edema, three with macular epiretinal membrane. TST of seven patients were all b positive. T-SPOT.TB of four patients were all positive. Three of eight patients who had chest X-ray or chest CT were suggested tuberculosis infection. Four to six weeks after the start of anti-tuberculosis treatment, vitritis, exudates, retinal and macular edema subsided. During follow up, inflammation was stable with no recurrence observed. The visual acuity of last follow-up ranged from 0.15 to 0.8 with an average of 0.51. Conclusions The main presentations of presumed tubercular retinal vasculitis are vitritis, retinal vein occlusion, and retinal hemorrhage. Standard anti-tuberculosis treatment can improve inflammation and retinal hemorrhage.
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.
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.
ObjectiveTo analyze the clinical character of uveitis in second hospital of Jilin university. MethodsRetrospectively analyze the clinical data of uveitis patients referred to from Second Hospital of Jilin University from September 2009 to September 2014. According to anatomical location, the manifestation of these uveitis patients were divided into anterior uveitis, panuveitis, intermediate uveitis and posterior uveitis. To discuss the possible causes of these patients according to the general information and relevant clinical laboratory examinations results. ResultsThere were 1215 cases in this study, which included 587 male, accounting for 48.31%; and 628 female, accounting for 51.69%. The ratio of male-to-female was 0.93:1. The range of the age of these patients was from 4 to 91 years old. The mean age of these patients at the onset of these disease was (41.43±14.20) years. Of the 1215 cases, 40 male and 43 female were younger than 20 years. The ratio of male-to-female was 0.93:1; 412 male and 396 female were between 21 and 50 years old. The ratio of male-to-female was 1.04:1; 135 male 189 female were older than 50 years. The ratio of male-to-female was 0.71:1. There were 572 cases of anterior uveitis, accounting for 47.08%; 527 cases of panuveitis, accounting for 43.37%; 52 cases of intermediate uveitis, accounting for 4.28%; 64 cases of posterior uveitis, accounting for 5.27%. 703 cases had etiological diagnosis according to the clinical character and the auxiliary results, accounting for 57.68%. Vogt-koyanagi Haradal (VKH) syndrome, ankylosing spondylitis associated with uveitis and Behçet's disease were the common entity, accounting for 30.44%, 19.77% and 14.22% respectively. ConclusionsThe mean age of these patients in this study was older, compared to other reports. Female patients were more than male, especially in these patients older than 50 years. VKH syndrome, ankylosing spondylitis associated with uveitis and Behçet's disease were the common entities.
Objective To analyze the onset, clinical manifestation, causation, complications of pediatric uveitis. Methods One hundred and two patients with uveitis under 16 year-old were retrospectively studied. They visited the clinic in Peking University First Hospital from November 1979 to December 2008. Their age ranged from 2.5 to 16 years old, with a mean of 11.9 years. Routine exam was carried out, including visual acuity, slit lamp, fundus, and laboratory workup. The diagnosis and classification were made by the anatomic location according to the standard of The International Uveitis Working Group. The data of disease history, age of onset, manifestation, recurrence, causation, systemic diseases, complications, and lab examination were analyzed.Results A total of 102 patients (170 eyes) with pediatric uveitis were included in this study, 68 patients (66.6%) were bilateral cases. Anterior uveitis represented in 38 patients (37.3%), intermediate uveitis in 19 (18.6%), posterior uveitis in 10 (9.8%), and panuveitis in 35 (34.3%). The disease duration was from five days to 2.4 years, with a mean of 3.6 months. The follow-up time was two weeks to more than ten years. The first three causes of pediatric uveitis were juvenile idiopathic arthritis, Vogt-Koyanagi-Harada disease, and Behccedil;etprime;s disease. 36 patients were found with complications, and among them 19 had complicated cataract, seven had secondary glaucoma, five had corneal band dystrophy, 12 had iris synechia (both anterior and posterior), one had retinal detachment, two had eye atrophy, and one patient with juvenile idiopathic arthritis had bilateral femoral head necrosis because of the use of steroid and hip joint was replaced. There were ten children suffering more than two complications. Conclusions Pediatric uveitis is a possible blindness disease with variety of etiology and manifestations,and tends to cause complications. Early and special attention must be taken to avoid serious consequences.
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 clinical manifestation and ophthalmoscopic image characteristics of uveal metastatic carcinoma. Methods Thirty-six uveal metastatic carcinoma patients (43 eyes) were enrolled in this study. The patients included 21 males and 15 females. The patientsprime; ages ranged from 28 to 71 years, with a mean age of (47.3plusmn;10.2) years. Seven patients had bilateral carcinoma and 29 patients had unilateral carcinoma. There were 30 patients with lung cancer, three patients with breast cancer, one patient with gastric cancer and two patients without primary tumors. There were 20 patients with known primary cancer, 16 patients visited the Department of Ophthalmology first. All the patients were examined documenting visual acuity, intraocular pressure, slit-lamp microscopy and mydriatic fundus examination. Meanwhile, 22 patients (26 eyes) were examined using B-type ultrasound and/or color Doppler flow imaging (CDFI). Twelve patients (12 eyes) were examined using fundus fluorescein angiography (FFA) and/or indocyanine green angiography (ICGA). Seventeen patients (22 eyes) were examined using MRI and/or CT. The clinical manifestation and ophthalmoscopic image characteristics of uveal metastatic carcinoma patients were observed. Results Among 43 eyes, four lesions were in the iris, three lesions in the ciliary body and 32 lesions were in the choroid. Fundus examination showed an isolated mass in 26 eyes and more than two masses in nine eyes. Metastatic tumors of the iris and ciliary body often showed irregular cauliflower-like mass with gray-white or meat-red color and abundant vessels. The choroidal metastasis usually demonstrated flat rounded or irregular intraocular masses with gray-yellow or gray-white color in the posterior pole. B-type ultrasound showed ill-defined, flat, and irregular-shaped masses with uneven internal reflectivity. CDFI showed rich blood flow within the tumor. FFA and (or) ICGA showed pinpoint and mottled leaks against hyperfluorescence background. MRI revealed low or middle signal using T1WI and low signal intensity using T2WI. Conclusions The uveal metastatic carcinoma usually occurs in one eye with an isolated mass. Most of them show a flat gray-yellow mass in posterior choroids and have the primary cancer sites of the lung. FFA and/or ICGA show pinpoint and mottled leaks against hyperfluorescence background. B-type ultrasound and (or) CDFI show ill-defined, flat, and irregular-shaped mass with rich blood flow within the tumor. MRI reveals low signal intensity on T2WI.
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.
ObjectiveTo assess the fundus characteristics and their associations with refractive error, best corrected visual acuity (BCVA) of highly myopic eyes in Chinese teenagers. MethodsThis is a cross-sectional and retrospective study. 544 teenagers (1050 eyes) with refraction more than -6.00 D were recruited from Tongren Eye Care Center. All participants underwent examinations including cycloplegic auto-refractometry and retinoscopy, BCVA, slit lamp and 45℃olor funds photography centered in macular. BCVA was recorded with logarithm of the minimum angle of resolution (logMAR) acuity. 988/1050 (94.1%) fundus photographs with clearly visible optic disc and fovea were selected for analysis. Degree of tessellation in optic disc and macular was defined by the exposure of choroidal vessel. Area of beta parapapillary atrophy (PPA), maximal and minimal diameter of optic disc, degree of fundus tessellation were measured by Image J software. Optic disc ovality was calculated by maximal diameter/minimal diameter. Associations between degree of tessellation, beta PPA area, optic disc ovality and refractive error, BCVA were analyzed. Presence of high myopic retinopathy, including chorioretinal atrophy, lacquer crack and Fuchs spot were also observed. ResultsMean spherical equivalent was (-10.66±2.63) D. Mean logMAR BCVA was 0.11±0.22. Tessellation was in 66.9% eyes. Mean degree in macular and peripapillary region was 0.83±0.96 and 1.04±1.00 (r=0.875, P=0.000). Beta PPA was in 97.3% eyes and mean area was (0.45±0.57) mm2. Mean ovality factor was 1.25±0.18 and Tilted optic disc was in 28.5% eyes. Refractive error, logMAR BCVA, beta PPA area, tilted optic disc and ovality factor were related with the degree of optic disc and macular tessellation (P < 0.05). Highly myopic retinopathy was found in 28 eyes, with older age, larger area of PPA, higher presence of tilted optic disc and degree of tessellation, worse BCVA. ConclusionsBeta PPA was the main fundus characteristics in teenagers. Visual acuity can be seriously impaired by highly myopic retinopathy, such as chorioretinal atrophy.