Central nervous system involvement in primary Sjögren's syndrome (pSS) is less common and usually presents as white matter lesions, neuromyelitis optica spectrum disorder (NMOSD), or transverse myelitis. NMOSD is an immune-mediated inflammatory demyelinating disease of the central nervous system with a high rate of relapse and significant disability. Studies have shown that patients with pSS combined with NMOSD have more severe symptoms and poorer prognosis. Here, we present a case of critical illness in pregnancy-associated NMOSD combined with Sjögren's syndrome. The patient was a 30-year-old pregnant woman with a history of Sjögren's syndrome who was diagnosed with NMOSD. She received combination therapy with steroids, intravenous immunoglobulin (IVIG), and hydroxychloroquine during pregnancy, resulting in partial resolution of numbness below the waist. However, due to irregular medication adherence outside the hospital setting, she developed weakness in her right lower limb accompanied by inability to move it, while her left lower limb still had some mobility but occasional numbness along with urinary and fecal incontinence. Ten days later, she was admitted to the emergency department where an emergency cesarean section was performed to deliver a healthy baby boy. However, her condition worsened postpartum as she developed high fever accompanied by bilateral lower limb paralysis and weakness along with loss of voluntary control over urination and defecation. The patient underwent ano-ther course of treatment consisting of steroids and IVIG; however there was limited improvement in symptoms observed after this intervention. Following administration of rituximab for the first time, the patient developed urinary tract infection which was successfully managed before continuing regular infusions. In later stages the patient could walk slightly with a limp and regained control over urination and defecation, allowing her to resume normal activities. This case suggests that combination therapy with steroids, IVIG, and hydroxychloroquine should be considered for the patients with pregnancy-associated NMOSD combined with Sjögren's syndrome. Rituximab can significantly improve symptoms such as postpartum paralysis in patients with NMOSD, however, there may be a risk of infection associated with its use.
Adult ; Female ; Humans ; Pregnancy ; Cesarean Section/adverse effects* ; Critical Illness ; Hydroxychloroquine/therapeutic use* ; Hypesthesia/complications* ; Immunoglobulins, Intravenous/therapeutic use* ; Inflammation/complications* ; Neuromyelitis Optica/diagnosis* ; Paralysis/complications* ; Pregnancy Complications/therapy* ; Rituximab/therapeutic use* ; Sjogren's Syndrome/complications* ; Steroids/therapeutic use* ; Vision Disorders

OBJECTIVES: To study the virtual reality-pattern visual evoked potential (VR-PVEP) P100 waveform characteristics of monocular visual impairment with different impaired degrees under simultaneous binocular perception and monocular stimulations. METHODS: A total of 55 young volunteers with normal vision (using decimal recording method, far vision ≥0.8 and near vision ≥0.5) were selected to simulate three groups of monocular refractive visual impairment by interpolation method. The sum of near and far vision ≤0.2 was Group A, the severe visual impairment group; the sum of near and far vision <0.8 was Group B, the moderate visual impairment group; and the sum of near and far vision ≥0.8 was Group C, the mild visual impairment group. The volunteers' binocular normal visions were set as the control group. The VR-PVEP P100 peak times measured by simultaneous binocular perception and monocular stimulation were compared at four spatial frequencies 16×16, 24×24, 32×32 and 64×64. RESULTS: In Group A, the differences between P100 peak times of simulant visual impairment eyes and simultaneous binocular perception at 24×24, 32×32 and 64×64 spatial frequencies were statistically significant (P<0.05); and the P100 peak time of normal vision eyes at 64×64 spatial frequency was significantly different from the simulant visual impairment eyes (P<0.05). In Group B, the differences between P100 peak times of simulant visual impairment eyes and simultaneous binocular perception at 16×16, 24×24 and 64×64 spatial frequencies were statistically significant (P<0.05); and the P100 peak time of normal vision eyes at 64×64 spatial frequency was significantly different from the simulant visual impairment eyes (P<0.05). In Group C, there was no significant difference between P100 peak times of simulant visual impairment eyes and simultaneous binocular perception at all spatial frequencies (P>0.05). There was no significant difference in the P100 peak times measured at all spatial frequencies between simulant visual impairment eyes and simultaneous binocular perception in the control group (P>0.05). CONCLUSIONS VR-PVEP can be used for visual acuity evaluation of patients with severe and moderate monocular visual impairment, which can reflect the visual impairment degree caused by ametropia. VR-PVEP has application value in the objective evaluation of visual function and forensic clinical identification.
Humans ; Evoked Potentials, Visual ; Vision, Ocular ; Vision, Binocular/physiology* ; Vision Disorders/diagnosis* ; Virtual Reality

PURPOSE: To report a case of frosted branch angiitis (FBA) associated with Epstein-Barr virus (EBV) infection in a child. CASE SUMMARY: A 7-year-old boy presented with bilateral blurred vision. On ophthalmic examination, his best-corrected visual acuity was 20/25 in the right eye and 20/32 in the left eye. The pupils were equal, round, and reactive to light without a relative afferent pupillary defect. He had normal color vision in both eyes. Slit-lamp examination revealed no abnormalities in the anterior parts of the eyes. Fundoscopic examination revealed prominent white sheathing retinal vasculitis predominantly on the veins in all quadrants, as well as macular edema and irregular foveal reflex in both eyes. Fluorescein angiography showed normal blood flow, but late diffuse staining and leakage of the affected vessels. Spectral domain optical coherence tomography (SD-OCT) showed thickening of the vessel walls, swelling due to hyperreflective material, and hyperreflective retinal depositions. Serological tests and the serum polymerase chain reaction for EBV were positive. A diagnosis of FBA associated with EBV was made. He was treated with systemic acyclovir and steroids. The response was rapid, with improvement in visual acuity to 20/20 in both eyes by day 3. After 7 weeks, all clinical signs resolved and SD-OCT examination showed normal vessel wall thickness and the absence of hyperreflective depositions. CONCLUSIONS: EBV may present with FBA even in the absence of a systemic sign of primary EBV infection. Thus, EBV should be considered as the etiology of FBA.
Acyclovir ; Child ; Color Vision ; Diagnosis ; Epstein-Barr Virus Infections ; Fluorescein Angiography ; Herpesvirus 4, Human ; Humans ; Macular Edema ; Male ; Polymerase Chain Reaction ; Pupil ; Pupil Disorders ; Reflex ; Retinal Vasculitis ; Retinaldehyde ; Serologic Tests ; Steroids ; Tomography, Optical Coherence ; Vasculitis ; Veins ; Visual Acuity

Gastric cancer with double metastasis to the orbit and duodenum is extremely rare. We report the case of a patient with gastric adenocarcinoma who presented with synchronous orbital and duodenal metastases at the time of initial diagnosis. A 60-year-old man presented with a 1-month history of visual disorder and pain in his right eye. He underwent ophthalmological examinations. The biopsy results suggested intraocular metastatic carcinoma. We conducted a systemic evaluation to identify primary malignancy. Finally, a diagnosis of advanced gastric adenocarcinoma with multi-organ metastasis was made. He planned to be treated with systemic chemotherapy.
Adenocarcinoma ; Biopsy ; Choroid ; Diagnosis ; Drug Therapy ; Duodenum ; Humans ; Middle Aged ; Neoplasm Metastasis ; Orbit ; Stomach Neoplasms ; Vision Disorders

A 2-year-old intact female Bengal cat was presented with a 6-month history of visual impairment. The cat manifested bilateral negative menace responses and dazzle reflexes and sluggish pupillary light reflexes. Bilateral fundus changes included generalized tapetal hyperreflectivity, advanced retinal vascular attenuation, and increased pallor of the optic disc. A diagnosis of bilateral retinal degeneration was made. The clinical findings suggest that the investigated Bengal cat was most likely to have an inherited retinal degeneration. Further studies of the Bengal cat breed are needed to determine the prevalence of inherited retinal degeneration in this breed in Korea.
Animals ; Cats ; Child, Preschool ; Diagnosis ; Female ; Humans ; Korea ; Pallor ; Prevalence ; Reflex ; Retinal Degeneration ; Retinaldehyde ; Vision Disorders

PURPOSE: To describe a patient who presented with central serous chorioretinopathy after 2 months of tadalafil administration without any other underlying disease or medication. CASE SUMMARY: A 49-year-old male patient was transferred from a local clinic with metamorphopsia and decreased visual acuity in the right eye. His visual acuity was 6/20 in the right eye and 18/20 in the left eye. The fundus examination showed a large serous detachment between the superior and inferior blood vessel arcades in the right retina. In his medical history, he used tadalafil three times a week for 2 months. His medication was then stopped, and a follow-up examination was scheduled. After 2 months, a fundus examination showed resolution of the subretinal fluid, and his corrected visual acuity recovered to 20/20. CONCLUSIONS: Tadalafil (Cialis®) is a phosphodiesterase (PDE)-5 inhibitor and predominantly prescribed for the treatment of erectile dysfunction. PDE–5 inhibitors may be potent vasodilators in the retina and choroid, and may induce choroidal vessel engorgement leading to leakage across the retinal pigment epithelium and accumulation of subretinal fluid in selected patients. When making a diagnosis as central serous chorioretinopathy, the physician should confirm the causative drugs that are easy to miss, by performing a thorough review of the patient's medical history and promptly terminating the causative drugs.
Blood Vessels ; Central Serous Chorioretinopathy ; Choroid ; Diagnosis ; Erectile Dysfunction ; Follow-Up Studies ; Humans ; Male ; Middle Aged ; Retina ; Retinal Pigment Epithelium ; Subretinal Fluid ; Tadalafil ; Vasodilator Agents ; Vision Disorders ; Visual Acuity

PURPOSE: To report a case of acute interstitial keratitis as the first clinical sign in a patient with latent syphilis. CASE SUMMARY: A 23-year-old female presented with visual impairment and discomfort in her right eye that developed 3 days earlier. The visual acuity in the right eye was 20/200 and corrected to 20/100, and slit lamp examination showed round sub-epithelial opacification in the central cornea with stromal edema and neovascularization on the cornea of the right eye. Whole body tests including serological tests were performed. Under the suspicion of acute interstitial keratitis, topical antibiotics and steroids were applied 4 times a day initially. Serological tests were reactive for venereal disease research laboratory test (VDRL). Under the suspicion of acute interstitial keratitis due to syphilis, fluorescent treponemal antibody absorption test IgM/IgG (FTA-ABS IgM/IgG) was performed; a positive result for FTA-ABS IgG led to diagnosis of acute interstitial keratitis with latent syphilis. During treatment, systemic doxycycline 200 mg for 4 weeks with topical antibiotics and steroids were administered, the opacity and edema of the cornea regressed after 2 weeks of treatment, and visual acuity in the patient's right eye improved to 20/20. CONCLUSIONS: We report an unusual case of acute interstitial keratitis as the first clinical manifestation of latent syphilis in an immunocompetent patient.
Anti-Bacterial Agents ; Cornea ; Diagnosis ; Doxycycline ; Edema ; Female ; Fluorescent Treponemal Antibody-Absorption Test ; Humans ; Immunoglobulin G ; Keratitis* ; Patient Rights ; Serologic Tests ; Sexually Transmitted Diseases ; Slit Lamp ; Steroids ; Syphilis* ; Syphilis, Latent ; Treponema pallidum ; Vision Disorders ; Visual Acuity ; Young Adult

PURPOSE: To report a case of tamoxifen-induced retinopathy diagnosed using spectral domain optical coherence tomography (SD-OCT). CASE SUMMARY: A 44-year-old female presented with metamorphopsia in the left eye and binocular vision loss which started 5 months prior. She had no record of external trauma, diabetes or high blood pressure; however, she had been taking 21.9 g tamoxifen (20 mg/day) since October 2012 after a surgery of her left breast due to cancer. On the initial visit, fundus photography showed crystalline dot-like deposits in both parafoveae. Additionally, fluorescence angiography revealed a small leakage around the macular area. Optical coherence tomography (OCT) was obtained to differentiate from other diseases because fundus photography showed crystalline retinopathy. The OCT revealed a normal right eye but the left macula had a microcystic lesion. Based on the diagnosis of tamoxifen-induced retinopathy, the patient stopped taking tamoxifen. Three months after discontinuation of tamoxifen, fundus photography showed slightly decreased crystalline deposits in the parafoveal area and visual acuity of the right eye was slightly improved. However, SD-OCT showed a slightly aggravated disruption of the outer retina in both eyes. CONCLUSIONS: Although retinopathy caused by treatment with tamoxifen occurs infrequently, to prevent complications and irreversible damage, patients who take tamoxifen for medical purposes need to undergo a regular ophthalmologic examination.
Adult ; Breast ; Crystallins ; Diagnosis ; Female ; Fluorescein Angiography ; Humans ; Hypertension ; Photography ; Retina ; Tamoxifen ; Tomography, Optical Coherence* ; Vision Disorders ; Vision, Binocular ; Visual Acuity

PURPOSE: To investigate and compare the progression of medically treated primary open angle glaucoma according to the baseline intraocular pressure (IOP). METHODS: This study included a total of 345 eyes from 345 patients (mean follow-up period, 4.5 years). Eyes were classified into either conventional normal tension glaucoma (cNTG, < or =21 mmHg) or conventional high-tension glaucoma (cHTG, >21 mmHg) groups according to the conventional cut-off value of the IOP. Additionally, the median IOP (15 mmHg) was used to create two other groups (median NTG [mNTG] < or =15 mmHg and median HTG [mHTG] >15 mmHg). Using these values, 306, 39, 153, and 192 eyes were assigned to the cNTG, cHTG, mNTG, and mHTG groups, respectively. Glaucoma progression was determined either by optic disc/retinal nerve fiber layer photographs or serial visual field data. RESULTS: Mean reduction of IOP after medical treatment and of central corneal thickness was lower in the cNTG group, while the prevalence of disc hemorrhage and baseline visual field mean deviation did not differ between the cNTG and cHTG groups. A mean reduction in the IOP was observed after medical treatment, and central corneal thickness was lower in the mNTG group; disc hemorrhage was more frequent in the mNTG than in the mHTG group. Among the 345 analyzed eyes, 100 (29%) showed progression during the follow-up period. In the cHTG group, a higher baseline IOP (hazard ratio, 1.147; p = 0.024) was associated with glaucoma progression. Disc hemorrhage (hazard ratio, 15.533; p < 0.001) was also strongly associated with progression in the mNTG group. CONCLUSIONS: Baseline IOP was a significant risk factor for glaucoma progression in cHTG patients (10% of our total participants), while disc hemorrhage showed the strongest association with progression in the mNTG group, indicating that a cut-off value other than the conventional 21 mmHg is required to define true low-tension glaucoma in populations where NTG predominates among all glaucoma patients.
Aged ; Disease Progression ; Female ; Glaucoma, Open-Angle/*diagnosis ; Gonioscopy ; Humans ; Intraocular Pressure ; Low Tension Glaucoma/*diagnosis ; Male ; Middle Aged ; Nerve Fibers/pathology ; Optic Disk/pathology ; Optic Nerve Diseases/*diagnosis ; Photography/standards ; Retinal Ganglion Cells/pathology ; Retrospective Studies ; Tomography, Optical Coherence ; Tonometry, Ocular ; Vision Disorders/diagnosis ; Visual Field Tests/standards ; Visual Fields

PURPOSE: Our objective was to estimate the maximum color contrast sensitivity (MCCS) thresholds in individuals with chiasma opticum damage. METHODS: The pilot study tested 41 people with pituitary adenoma (PA) and 100 age- and gender-matched controls. Patients were divided into two groups according to PA size, PA ≤1 cm or PA >1 cm. A new MCCS test program was used for color discrimination. RESULTS: The mean total error score (TES) of MCCS was 1.8 in the PA ≤1 cm group (standard deviation [SD], 0.38), 3.5 in the PA >1 cm group (SD, 0.96), and 1.4 in the control group (SD, 0.31; p < 0.001). There was a positive correlation between tumor size and MCCS result (r = 0.648, p < 0.01). In the group that had PA-producing hormones, the TES was 2.5 (SD, 1.09), compared to 4.2 value in the non-functioning PA group of patients that did not have clinically significant hormone excess (SD, 3.16; p < 0.01). In patients with normal visual acuity (VA) or visual field MCCS, the TES was 3.3 (SD, 1.8), while that in patients with VA <0.00 was 4.6 (SD, 2.9). CONCLUSIONS: Results of the MCCS test TES were 1.9 times better in patients with PA ≤1 cm compared to patients with PA >1 cm (p < 0.01). In PA patients with normal VA, the TES was 2.35 times worse than that of healthy persons (p < 0.01).
Adenoma/*complications/diagnosis ; Adolescent ; Adult ; Aged ; Color Perception/physiology ; Color Perception Tests/*methods ; Contrast Sensitivity/*physiology ; *Early Diagnosis ; Female ; Humans ; Male ; Middle Aged ; *Optic Chiasm ; Pilot Projects ; Pituitary Neoplasms/*complications/diagnosis ; Time Factors ; Vision Disorders/*diagnosis/etiology/physiopathology ; Visual Fields ; Young Adult