OBJECTIVE: To study the clinical features and recurrence factors of myelin oligodendrocyte glycoprotein (MOG) antibody disease in children and the effect of recurrence prevention regimens. METHODS: A retrospective analysis was performed on the medical data of 41 children with MOG antibody disease who were hospitalized in the Department of Pediatric Neurology, Xiangya Hospital of Central South University, from December 2014 to September 2020. According to the presence or absence of recurrence, they were divided into a monophasic course group ( RESULTS: For these 41 children, acute disseminated encephalomyelitis was the most common initial manifestation and was observed in 23 children (56%). Of the 41 children, 22 (54%) experienced recurrence, with 57 recurrence events in total, among which optic neuritis was the most common event (17/57, 30%). The proportion of children in the recurrence group who were treated with corticosteroids for less than 3 months in the acute phase was higher than that in the monophasic course group (64% CONCLUSIONS More than half of the children with MOG antibody disease may experience recurrence. Most children with recurrence are treated with corticosteroids for less than 3 months in the acute phase. Rituximab and azathioprine may reduce the risk of recurrence.
Autoantibodies ; Child ; Humans ; Myelin-Oligodendrocyte Glycoprotein ; Optic Neuritis ; Recurrence ; Retrospective Studies

PURPOSE: We report an unusual case of Leber hereditary optic neuropathy presenting with optic disc hyperfluorescence. CASE SUMMARY: A 17-year-old male with sequential painless visual loss 3 weeks apart affecting first the left and then the right eye presented to our neuro-ophthalmology clinic. His best-corrected visual acuity was counting fingers in the right eye and 0.32 in the left eye. Fundus examination showed mild optic disc edema and hyperemia in both eyes, which were worse in the right eye. Fluorescein angiography revealed dye leakage from the right optic disc in the late phase. The results of magnetic resonance imaging of the brain and spinal cord were normal, and lumbar puncture study was unremarkable. Mitochondrial DNA sequencing revealed a pathognomonic 11778 mutation for Leber hereditary optic neuropathy. His vision deteriorated to 0.03 in both eyes 6 months later, but slowly started to improve 11 months after onset. At 2 years, his corrected visual acuity was 0.2 in both eyes. CONCLUSIONS: To our knowledge, this is the first report of optic disc hyperfluorescence in Leber hereditary optic neuropathy. This finding suggests that this mitochondrial optic neuropathy can masquerade as optic neuritis.
Adolescent ; Brain ; DNA, Mitochondrial ; Edema ; Fingers ; Fluorescein Angiography ; Humans ; Hyperemia ; Magnetic Resonance Imaging ; Male ; Optic Atrophy ; Optic Atrophy, Hereditary, Leber ; Optic Nerve Diseases ; Optic Neuritis ; Spinal Cord ; Spinal Puncture ; Visual Acuity

PURPOSE: To report a patient stung by a bee, who was diagnosed with sterile endopthalmitis and another patient diagnosed with optic neuritis, with decreasing visual acuity, after refined bee venom injection around the orbital tissue. CASE SUMMARY: A 82-year-old female visited our hospital for decreased visual acuity in the right eye and ocular pain due to a bee sting. The bee sting penetrated the sclera into the vitreous. In the anterior segment, severe cornea edema and anterior chamber cells were seen. Using ultrasonography, inflammation was seen around the intravitreal area. After 3 months, intravitreal inflammation regressed but the patient's visual acuity was light perception negative, and corneal opacity, neovascularization, and phthisis bulbi were detected. A 55-year-old male visited our hospital for ocular pain in the right eye and decreasing visual acuity after refined bee venom injection around the orbital tissue. The best-corrected visual acuity in the right eye was 15/100, there was moderate injection on the conjunctiva. A relative afferent pupillary defect, abnormal color vision test results, and a defect in the visual field test were observed. There was no pain during external ocular movement, and other general blood tests, and a brain MRI were normal. Based on these symptoms, methylprednisolone megatherapy was started for treatment of optic neuritis. After treatment, visual acuity of the right eye was 9/10 and all other clinical optic neuritis symptoms regressed. CONCLUSIONS: Based on these two cases, ocular toxicity from bee venom could result from both direct and indirect courses. Treatment using refined bee venom might be harmful, and caution is recommended in its use.
Aged, 80 and over ; Anterior Chamber ; Bee Venoms ; Bees ; Bites and Stings ; Brain ; Color Vision ; Conjunctiva ; Cornea ; Corneal Opacity ; Edema ; Female ; Hematologic Tests ; Humans ; Inflammation ; Magnetic Resonance Imaging ; Male ; Methylprednisolone ; Middle Aged ; Optic Neuritis ; Orbit ; Pupil Disorders ; Sclera ; Ultrasonography ; Visual Acuity ; Visual Field Tests

PURPOSE: To describe the clinical characteristics and course of optic neuritis (ON) and its association with neuromyelitis optica spectrum disorder (NMOSD) and multiple sclerosis (MS) in Korea. METHODS: In this retrospective case series, 125 eyes of 91 Korean patients with ON were included. The medical documents of adult patients with ON were retrospectively reviewed. Patients were assigned into idiopathic ON, NMOSD, and MS groups according to the presence of an association with NMOSD or MS for subgroup analysis. Clinical characteristics, disease course, and visual and systemic prognosis were analyzed. RESULTS: During the mean follow-up of 3.7 years, 73 patients were diagnosed as idiopathic ON, 14 patients were diagnosed as NMOSD, and four patients developed definite MS. At the final visit, there were 13 (13%) eyes out of 100 eyes with idiopathic ON, nine (43%) eyes out of 21 eyes with NMOSD, and one (25%) eye out of four eyes with MS had a severe visual loss of 20 / 200 or less. The mean Expanded Disability Status Scale was 3.1 ± 1.5 in NMOSD and 1.8 ± 1.5 in the MS group at the final visit. In the NMOSD group, 50% of patients showed severe visual loss in at least one eye or were unable to ambulate without assistance at the final visit (5.3 ± 4.4 years after the initial episode of ON). CONCLUSIONS: Fourteen percent of patients showed positive results for NMO-immunoglobulin G test and 50% of patients with NMOSD showed a severe visual loss in at least one eye or were unable to ambulate without assistance. The proportion of MS was relatively low in Korean ON patients.
Adult ; Follow-Up Studies ; Humans ; Korea ; Multiple Sclerosis ; Neuromyelitis Optica ; Optic Neuritis ; Prognosis ; Retrospective Studies

Objective To evaluate the optic nerve impairment using MRI histogram texture analysis in the patients with optic neuritis.Methods The study included 60 patients with optic neuritis and 20 normal controls. The coronal T2 weighted imaging (T2WI) with fat saturation and enhanced T1 weighted imaging (T1WI) were performed to evaluate the optic nerve. MRI histogram texture features of the involved optic nerve were measured on the corresponding coronal T2WI images. The normal optic nerve (NON) was measured in the posterior 1/3 parts of the optic nerve. Kruskal-Wallis one-way ANOVA was used to compare the difference of texture features and receiver operating characteristic (ROC) curve were performed to evaluate the diagnostic value of texture features for the optic nerve impairment among the affected optic nerve with enhancement (ONwEN), affected optic nerve without enhancement (ONwoEN), contralateral normal appearing optic nerve (NAON) and NON.Results The histogram texture Energy and Entropy presented significant differences for ONwEN vs. ONwoEN (both P=0.000), ONwEN vs. NON (both P=0.000) and NAON vs. NON (both P<0.05). ROC analysis demonstrated that the area under the curve (AUC) of histogram texture Energy were 0.758, 0.795 and 0.701 for ONwEN vs. ONwoEN, ONwEN vs. NON and NAON vs. NON, AUC of Entropy were 0.758, 0.795 and 0.707 for ONwEN vs. ONwoEN, ONwEN vs. NON and NAON vs. NON.Conclusions The altered MRI histogram texture Energy and Entropy could be considered as a surrogate for MRI enhancement to evaluate the involved optic nerve and normal-appearing optic nerve in optic neuritis.
Adolescent ; Adult ; Humans ; Magnetic Resonance Imaging ; Middle Aged ; Optic Nerve ; diagnostic imaging ; Optic Neuritis ; diagnostic imaging

A 6-year-old intact male Maltese dog presented with a history of blindness and ataxia. Neuro-ophthalmic examination revealed dilated pupils with absent pupillary light reflexes and menace response in both eyes. Mild peripapillary edema was noted in the fundus of the right eye. After magnetic resonance imaging, the dog was provisionally diagnosed with meningoencephalitis of unknown etiology. Follow-up funduscopy was performed to monitor the condition of the optic discs for three years. Despite of the treatment with prednisolone, the optic nerve progressed to atrophy and the dog couldn't restore vision.
Animals ; Ataxia ; Atrophy ; Blindness ; Child ; Dogs ; Edema ; Follow-Up Studies ; Humans ; Magnetic Resonance Imaging ; Male ; Meningoencephalitis ; Optic Nerve ; Optic Neuritis ; Prednisolone ; Pupil ; Reflex

Iso-oncotic human serum albumin (HSA) is the primary replacement fluid of choice during therapeutic plasma exchange (TPE). Hypersensitivity reactions to HSA are rare, but require proper evaluation and management. In this article, we report two cases of hypersensitivity reactions to 5% HSA during TPE and discuss strategies to address this problem. The first case was a 60-year-old female patient, who was scheduled for TPE for treatment of recurrent focal segmental glomerulosclerosis after ABO-incompatible kidney transplantation. She developed a pruritic rash on her entire body during the first two sessions of TPE using 5% HSA. The third session was conducted using 500 mL normal saline, 1,000 mL 10% pentastarch, and 750 mL 5% HSA, where she eventually developed a pruritic rash when HSA was infused. There were no adverse events during the fourth and fifth session when fresh frozen plasma was used in place of HSA. The second case was a 50-year-old male patient diagnosed with optic neuritis, who was admitted for five sessions of TPE. The patient developed a pruritic rash on his entire body during the first session of TPE using 5% HSA. The patient experienced no adverse events during the following four sessions using fresh frozen plasma. Certain elements contained in HSA, such as albumin aggregates, prekallikrein activator, and caprylate-modified albumin, might be the reason for these hypersensitivity reactions. Careful selection of alternative replacement fluids is important to avoid premature termination of TPE procedures and secure optimal treatment options for patients.
Caprylates ; Exanthema ; Factor XIIa ; Female ; Glomerulosclerosis, Focal Segmental ; Humans ; Hydroxyethyl Starch Derivatives ; Hypersensitivity ; Kidney Transplantation ; Male ; Middle Aged ; Optic Neuritis ; Plasma Exchange ; Plasma ; Serum Albumin

BACKGROUND: Methyl alcohol poisoning in mobile phone–manufacturing factories during 2015–2016 was caused by methyl alcohol use for cleaning in computerized numerical control (CNC) processes. To determine whether there were health complications in other workers involved in similar processes, the Occupational Safety and Health Research Institute conducted a survey. METHODS: We established a national active surveillance system by collaborating with the Ministry of Employment and Labor and National Health Insurance Service. Employment and national health insurance data were used. Overall, 12,048 employees of major domestic mobile phone companies and CNC process dispatch workers were surveyed from 2016 to 2017. We investigated methyl alcohol poisoning by using the national health insurance data. Questionnaires were used to investigate diseases due to methyl alcohol poisoning. RESULTS: Overall, 24.9% of dispatched workers were employed in at least five companies, and 23.9% of dispatched workers had missing employment insurance history data. The prevalence of blindness including visual impairment, optic neuritis, visual disturbances, and alcohol toxicity in the study participants was higher than that reported in the national health insurance database (0.02%, 0.07%, 0.23%, and 0.03% versus 0.01%, 0.07%, 0.13%, and 0.01%, respectively, in 2015). Moreover, 430 suspicious workers were identified; 415 of these provided an address and phone number, of whom 48 responded (response rate, 11.6%). Among the 48 workers, 10 had diseases at the time of the survey, of whom 3 workers were believed to have diseases related to methyl alcohol exposure. CONCLUSION: This study revealed that active surveillance data can be used to assess health problems related to methyl alcohol poisoning in CNC processes and dispatch workers.
Academies and Institutes ; Blindness ; Cell Phones ; Employment ; Insurance ; Mass Screening ; Methanol ; National Health Programs ; Occupational Diseases ; Occupational Health ; Optic Neuritis ; Poisoning ; Prevalence ; Vision Disorders

PURPOSE: To report a case of optic neuritis accompanied by optic perineuritis after retinalamin peribulbar injection. CASE SUMMARY: A 46-year-old Kazakhstan woman presented with sudden vision loss, headache, and pain in both eyes which began 2 hours after the peribulbar injection of retinalamin approximately 10 days prior to her initial visit. At the initial visit, the best-corrected visual acuity was light perception in both eyes. A fundus examination showed bilateral optic disc swelling and fluorescein angiography showed late-phase leakage around the optic nerve and optic nerve sheath enhancement. On brain magnetic resonance imaging, the tram track sign and donut sign were seen in both eyes. Steroid pulse therapy was started after the diagnosis of optic neuritis accompanied by optic perineuritis. After 6 months, the patient's visual acuity improved up to 0.8 in the right eye and 1.0 in the left eye. The light reflex was recovered, the relative afferent pupillary defect decreased, and there was no evidence of optic disc swelling. CONCLUSIONS: When an unverified drug injection is performed (e.g., retinalamin), it could result in complications such as optic neuritis and optic perineuritis from an allergic reaction or peribulbar injection trauma. In such cases, high-dose steroid pulse therapy may be considered.
Brain ; Diagnosis ; Female ; Fluorescein Angiography ; Headache ; Humans ; Hypersensitivity ; Kazakhstan ; Magnetic Resonance Imaging ; Middle Aged ; Optic Nerve ; Optic Neuritis ; Pupil Disorders ; Reflex ; Visual Acuity

High-quality clinical evidence, derived from well-designed and implemented clinical trials, serves to advance clinical care and to allow physicians to provide the most effective treatments to their patients. The field of ophthalmology, including the subspecialty of neuro-ophthalmology, abounds with such high-quality clinical trials that provide Level 1 clinical evidence. This review article summarizes the research design, key findings, and clinical relevance of select monumental clinical studies in neuro-ophthalmology with the primary goal of providing the readers with the rationale for current standard of care of various neuro-ophthalmic diseases. This includes the Optic Neuritis Treatment Trial, Ischemic Optic Neuropathy Decompression Trial, Idiopathic Intracranial Hypertension Treatment Trial, Rescue of Hereditary Optic Disease Outpatient Study, and Controlled High-Risk Avonex® Multiple Sclerosis Study
Optic Neuritis ; Optic Neuropathy, Ischemic ; Intracranial Hypertension