First Clinical Experience with Anti-myelin Oligodendrocyte Glycoprotein Antibody-Positive Optic Neuritis

2020 ◽  
Vol 237 (04) ◽  
pp. 458-463 ◽  
Author(s):  
Jan Heckmann ◽  
Margarita Todorova ◽  
Stefanie Müller ◽  
Philip Julian Broser ◽  
Veit Sturm
Keyword(s):  
Visual Acuity ◽  
Optic Neuritis ◽  
Pulse Therapy ◽  
Myelin Oligodendrocyte Glycoprotein ◽  
Individual Treatment ◽  
Igg Antibodies ◽  
Intravenous Methylprednisolone ◽  
Finger Counting ◽  
The Right

Abstract Background Antibodies against myelin oligodendrocyte glycoprotein (MOG-IgG) have been consistently found in a range of demyelinating disorders. In this context, MOG-IgG-associated optic neuritis (ON) has been suggested as a new subset of optic neuropathy. However, clinical manifestations and distinctive characteristics have only rarely been described. Patients and Methods A retrospective case series of three patients with MOG-IgG-associated ON. Clinical morphological features using imaging techniques are presented. Results Three patients (8-year-old boy, 28-year-old female, 48-year-old male) were included. An 8-year-old boy suffered from a bilateral ON with severe visual loss. The best-corrected visual acuity (BCVA) was 0.05 in the right eye and finger counting in the left eye. The patient had a previous episode of acute disseminated encephalomyelitis (ADEM) with a right abducens nerve palsy. Visual acuity recovered after repeated cycles of intravenous methylprednisolone pulse therapy and 10 cycles of plasma exchange. During the last follow-up, BCVA was 0.9 in the right eye and 0.8 in the left eye. A 28-year-old female presented with a bilateral ON. Her BCVA was 0.5 in the right eye and 0.8 in the left eye. She fully recovered with pulse methylprednisolone therapy (1000 mg/d) with tapering after the second cycle and had a BCVA of 1.0 during the last follow-up visit. A 48-year-old male suffered from a relapsing bilateral ON. At first presentation, BCVA was 0.1 in the right eye and finger counting in the left eye. BCVA fully recovered after each pulse therapy with intravenous methylprednisolone (two cycles). Since the first relapse, the patient has been receiving long-term immunosuppression with rituximab. Despite rituximab and low-dose oral prednisone, the patient had another relapse with a left ON. After a third cycle with intravenous methylprednisolone, he partially recovered. BCVA at last follow-up was 1.0 in the right and 0.8 in the left eye. Conclusions MOG-IgG antibodies have been identified in different acquired demyelinating syndromes. The patients reported had an ADEM followed by bilateral ON, an isolated bilateral ON, and a relapsing bilateral ON. Individual treatment strategies led to substantial visual recovery in all patients. We recommend inclusion of MOG-IgG antibodies in the diagnostic workup at least after the first recurrence of ON since they can serve as a diagnostic and potential prognostic tool and might lead to specific therapeutic recommendations.

scholarly journals Leber’s hereditary optic neuropathy following unilateral painful optic neuritis: a case report

2020 ◽  
Vol 20 (1) ◽  
Author(s):  
Chaeyeon Lee ◽  
Kyung-Ah Park ◽  
Ga-In Lee ◽  
Sei Yeul Oh ◽  
Ju-Hong Min ◽  
...  
Keyword(s):  
Visual Acuity ◽  
Optic Neuritis ◽  
Optic Neuropathy ◽  
Visual Loss ◽  
Early Stage ◽  
Leber’S Hereditary Optic Neuropathy ◽  
Leber's Hereditary Optic Neuropathy ◽  
Intravenous Methylprednisolone ◽  
The Right ◽  
Hereditary Optic Neuropathy

Abstract Background Leber’s hereditary optic neuropathy (LHON) is a maternally inherited mitochondrial disease, characterized by acute or subacute, painless, bilateral visual loss. LHON is often misdiagnosed as optic neuritis at an early stage because of the similarity of their clinical presentation. To date, there has been no reported case of actual optic neuritis and LHON in one patient. Case presentation A 40-year-old, healthy man was referred to our clinic with acute painful visual loss in the right eye for 2 weeks. In the right eye, visual acuity decreased to 20/40, and the Ishihara colour test score was 8/14 with a relative afferent pupillary defect. Optic disc swelling was found only in the right eye, and magnetic resonance imaging revealed enhancement of the the right optic nerve, consistent with optic neuritis. After receiving 1 g of intravenous methylprednisolone daily for three days, his ocular pain resolved, and visual acuity improved to 20/20 within 2 weeks. Seven months later, the patient developed acute painless visual loss in the right eye. Visual acuity decreased to 20/200 in the right eye. There was no response to the intravenous methylprednisolone therapy at that time. Eight months later, he developed subacute painless visual loss in the left eye. Genetic testing for LHON was performed and revealed the pathologic mtDNA 11778 point mutation. Conclusions We report a case with painful unilateral optic neuritis preceding the onset of LHON. Even if a typical optic neuritis patient has completely recovered from steroid treatment once in the past, it is advisable to keep in mind the possibility of LHON if acute or subacute loss of vision subsequently or simultaneously occurs in both eyes and does not respond to steroids.

scholarly journals Factors influencing intravenous methylprednisolone pulse therapy in Chinese patients with isolated optic neuritis associated with AQP4 antibody-seropositive neuromyelitis optica

2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Sitong Guo ◽  
Hanqiu Jiang ◽  
Libin Jiang ◽  
Jingting Peng ◽  
Hongjuan Liu ◽  
...  
Keyword(s):  
Visual Acuity ◽  
Optic Neuritis ◽  
Neuromyelitis Optica ◽  
Age At Onset ◽  
Chinese Patients ◽  
Intravenous Methylprednisolone ◽  
Methylprednisolone Pulse ◽  
Factors Influencing ◽  
Intravenous Methylprednisolone Pulse ◽  
Aqp4 Antibody

AbstractThis study investigated the factors influencing intravenous methylprednisolone pulse (IVMP) therapy for recovering visual acuity in Chinese patients with aquaporin-4 (AQP4) antibody-seropositive neuromyelitis optica-related optic neuritis (NMO-ON). This retrospective case series included 243 affected eyes of 182 patients (36 male, 146 female) diagnosed with NMO-ON in the Neuro-Ophthalmology Clinic of Beijing Tongren Hospital from September 2012 to September 2020. All patients with AQP4-antibody seropositivity had clinical manifestations of acute ON, excluding other diagnoses and received IVMP treatment at 500 mg/day or 1000 mg/day for 3 days. Primary outcome was the extent of improvement in logMAR visual acuity after IVMP treatment. The therapeutic influences of sex, age, baseline visual acuity, therapeutic intervals, and IVMP dose on acute NMO-ON were analysed. Chi-square tests, Mann–Whitney U-tests, Kruskal–Wallis tests, Spearman’s correlation coefficients, and multiple linear regression were used for statistical analysis. Age ranged between 7 and 80 years (median age, 44; interquartile range [IQR], 29–52) years. Among the 243 eyes, the median improvement in logMAR visual acuity was 0.3 (IQR, 0–0.9). Therapeutic efficacy of IVMP was significantly higher in female than in male patients (Z = 2.117, P = 0.034). The treatment effect gradually decreased with increase in age at onset (Rs = 0.157, P = 0.015), and visual improvement was significantly lower in patients aged > 50 years than in those ≤ 50 years (Z = 2.571, P = 0.010). When patients had low visual acuity at onset, improvements were more obvious (rho =  − 0.317, P < 0.001); however, final visual acuity was still low (rho = 0.688, P < 0.001). Therapeutic effect was negatively correlated with therapeutic intervals (rho = 0.228, P = 0.001). Dosage of methylprednisolone (1000 mg/day or 500 mg/day) did not significantly influence treatment efficacy (Z = 0.951 P = 0.342). Therefore, IVMP therapy can improve visual acuity in the affected eyes of patients with AQP4 antibody-seropositive NMO-ON with similar effect at 500 mg/day and 1000 mg/day doses. Sex, age at onset, and therapeutic intervals may influence the efficacy of IVMP in patients with NMO-ON.

scholarly journals Pediatric Optic Neuritis: Description of Four Cases and Review of the Literature

Children ◽  
2021 ◽  
Vol 8 (10) ◽  
pp. 855
Author(s):  
Anna Presicci ◽  
Maria Serra ◽  
Mariaclara Achille ◽  
Elvita Caputo ◽  
Lucia Margari
Keyword(s):  
Optic Neuritis ◽  
Recent Literature ◽  
Systemic Disease ◽  
Treatment Options ◽  
Igg Antibodies ◽  
Careful Evaluation ◽  
Demyelinating Disorders ◽  
Infective Etiology

Pediatric optic neuritis (PON) may be a clinically isolated and self-limiting event or may present in the context of underlying neurologic, infective, or systemic disease. PON has a high impact on the quality of life as it may or may not evolve into other acquired demyelinating syndromes (ADSs), such as multiple sclerosis (MS), neuromyelitis optica (NMO), or other syndromes related to the myelin oligodendrocyte glycoprotein IgG antibodies (MOG-IgG). These different PON phenotypes present variable clinical and radiological features, plasma and liquor biomarkers, and prognosis. We describe four pediatric cases presenting clinically with ON, with different etiopathogenetic pictures: one case had a probable infective etiology, while the others were associated with different demyelinating disorders (MS, NMO, syndrome related to MOG-IgG). We discuss the possible evolution of presenting ON in other ADSs, based on recent literature. A careful evaluation of the clinical and investigation findings and the natural course of PON is necessary to define its pathogenic pathway and evolution. Further prolonged follow-up studies are needed to highlight the predictors of PON evolution, its potential sequelae, and the best treatment options.

A Woman With Subacute Painful Vision Loss

2021 ◽  
pp. 3-6
Author(s):  
Jiraporn Jitprapaikulsan ◽  
M. Tariq Bhatti ◽  
Eric R. Eggenberger ◽  
Marie D. Acierno ◽  
John J. Chen
Keyword(s):  
Optic Neuritis ◽  
Eye Movement ◽  
Immunoglobulin G ◽  
Vision Loss ◽  
Visual Fields ◽  
Corticosteroid Treatment ◽  
Antineutrophil Cytoplasmic Antibody ◽  
Myelin Oligodendrocyte Glycoprotein ◽  
Intravenous Methylprednisolone ◽  
Eye Pain

A 51-year-old White woman sought care for vision loss 1 week after a nonspecific upper respiratory tract infection. She reported pain in both eyes exacerbated by eye movement, which lasted for several days, followed by bilateral vision loss to the level of counting fingers–only vision. Optic neuritis was diagnosed, and she was treated with 1 g intravenous methylprednisolone for 3 days. Her vision improved substantially, and the pain resolved during the corticosteroid treatment. However, 1 week later, she woke up with right eye pain and vision loss. She was again treated with 5 days of intravenous methylprednisolone, with visual improvement nearly back to baseline. Two weeks later, she had recurrence of painful vision loss in both eyes. A diagnosis of chronic relapsing inflammatory optic neuropathy was made. Tests for serum angiotensin-converting enzyme, antineutrophil cytoplasmic antibody, antinuclear antibody, Lyme disease, syphilis, tuberculosis, and aquaporin-4-immunoglobulin G antibodies were negative. Serum was definitively positive for myelin oligodendrocyte glycoprotein-immunoglobulin G antibodies at a titer of 1:1,000. Myelin oligodendrocyte glycoprotein-immunoglobulin G–associated recurrent optic neuritis was diagnosed. After her diagnosis of recurrent corticosteroid-dependent optic neuritis associated with myelin oligodendrocyte glycoprotein-immunoglobulin G positivity, the patient was treated with 5 days of intravenous methylprednisolone. The eye pain resolved, and her vision returned to normal. At follow-up evaluation, the patient’s visual acuity, color vision, and visual fields were normal in both eyes, but there was mild bilateral optic disc pallor. She has not had recurrent demyelinating episodes while on chronic immunotherapy. Optic neuritis is an inflammatory demyelination of the optic nerve manifesting as acute to subacute vision loss, classically associated with pain with eye movement. The long-term prevention and prognosis depend on the cause of the optic neuritis.

scholarly journals Bilateral central serous chorio-retinopathy in pregnancy presenting with severe visual loss

2014 ◽  
Vol 6 (2) ◽  
pp. 220-223 ◽  
Author(s):  
Chandana Chakraborti ◽  
Swapan Kumar Samanta ◽  
Khandakar Faiduddin ◽  
Krittika Pal Choudhury ◽  
Sheuli Kumar ◽  
...  
Keyword(s):  
Visual Acuity ◽  
Retinal Pigment ◽  
Third Trimester ◽  
Retinal Pigment Epithelial Detachment ◽  
Serous Macular Detachment ◽  
Macular Detachment ◽  
Severe Visual Loss ◽  
Finger Counting ◽  
Funduscopic Examination ◽  
The Right

Introduction: Bilateral central serous chorio-retinopathy is an unusual presentation.  Objective: To report a case of pregnancy-induced bilateral central serous chorio-retinopathy. Case: A thirty-year-old woman complaining of reduced vision in both the eyes was referred for ophthalmoscopic evaluation in her third trimester of pregnancy. The best-corrected visual acuity was 6/60 in the right eye and finger counting at four meters in the left. The funduscopic examination revealed serous macular detachment with white sub-retinal exudates in both the eyes. Optical coherence tomography sections through the macula demonstrated serous elevation of the retina and retinal pigment epithelial detachment in both the eyes. The serous macular detachment resolved and the patient recovered to the visual acuity of 6/6 in both the eyes after twelve weeks of delivery. The OCT showed resolution of the sub-retinal fluid and disappearance of the sub-macular exudate in the right eye, but the resolution was partial in the left eye.Conclusion: Pregnancy is a risk factor for CSCR and it can present with severe bilateral visual diminution.DOI: http://dx.doi.org/10.3126/nepjoph.v6i2.11711Nepal J Ophthalmol 2014; 6(12): 220-223 

scholarly journals Different Characteristics of Aquaporin-4 and Myelin Oligodendrocyte Glycoprotein Antibody-Seropositive Male Optic Neuritis in China

2019 ◽  
Vol 2019 ◽  
pp. 1-7 ◽  
Author(s):  
Honglu Song ◽  
Huanfen Zhou ◽  
Mo Yang ◽  
Junqing Wang ◽  
Hongjuan Liu ◽  
...  
Keyword(s):  
Visual Acuity ◽  
Optic Neuritis ◽  
Ganglion Cell ◽  
Nerve Fiber ◽  
Optic Disc ◽  
Myelin Oligodendrocyte Glycoprotein ◽  
Aquaporin 4 ◽  
Optic Disc Swelling ◽  
Male Patients ◽  
Retinal Nerve Fiber

Purpose. To describe different clinical characteristics and prognosis of optic neuritis (ON) in male patients with seropositive aquaporin-4 antibody (AQP4-Ab) or myelin oligodendrocyte glycoprotein antibody (MOG-Ab) in China. Method. Males with ON were recruited from the Neuro-ophthalmology Department of the Chinese People’s Liberation Army, General Hospital from January 2016 to February 2018. They were assigned to two groups based on antibodies status: MOG-Ab-seropositive ON (MOG-ON) and aquaporin-4 Ab-seropositive ON (AQP4-ON). Results. Seventy-six male patients were assessed, including 44 MOG-ON (57.9%) and 32 AQP4-ON (42.1%). The MOG-ON patients were significantly younger at onset compared to the AQP4-ON group (p<0.001). Frequencies of optic disc swelling, presence of abnormal autoimmune antibodies, and elevated levels of CSF IgG were significantly higher in the AQP4-ON group than the MOG-ON group (p=0.040, p=0.016, and p=0.10, respectively). At the final visit, 85.3% of MOG-ON eyes had increased visual acuity (≥0.5) compared to 35.1% of AQP4-ON eyes (p<0.001). The ratio of this steroid-dependent condition is higher in MOG-ON patients than the AQP4-ON group (p<0.001). The ratio of conversion to NMO is higher in the AQP4-ON group than the MOG-ON group, with more AQP4-ON patients developing NMO by the follow-up (p=0.012). MOG-ON patients had thicker average peripapillary retinal nerve fiber layers and macular ganglion cell-inner plexiform than AQP4-ON patients (p=0.008 and p=0.012, respectively). Orbital MRI revealed more AQP4-ON patients had chiasmal involvement than MOG-ON patients (p<0.001). Conclusion. Male MOG-ON patients had different clinical features including earlier age of onset, higher optic disc swelling ratio, better visual acuity recovery, thicker peripapillary retinal nerve fiber and macular ganglion cell-inner plexiform layers, and less chiasmal involvement than male AQP4-ON patients. Serum antibody may be a potential biomarker for determining visual prognosis in male ON.

scholarly journals The Use of Intravitreal Ranibizumab for Choroidal Neovascularization Associated with Vogt-Koyanagi-Harada Syndrome

2011 ◽  
Vol 2011 ◽  
pp. 1-3 ◽  
Author(s):  
A. M. Kolomeyer ◽  
M. S. Roy ◽  
D. S. Chu
Keyword(s):  
Vision Loss ◽  
Corticosteroid Treatment ◽  
Exudative Retinal Detachment ◽  
Intravitreal Ranibizumab ◽  
Loss To Follow Up ◽  
Finger Counting ◽  
Combinational Treatment ◽  
Vkh Syndrome ◽  
The Right

Purpose. To describe the use of intravitreal ranibizumab for choroidal neovascular membrane (CNVM) secondary to Vogt-Koyanagi-Harada (VKH) syndrome.Methods. Interventional case report.Results. A 50-year-old woman presented with conjunctival injection and bilateral eye pain. Vision was 20/400 and 20/80 in the right and left eyes, respectively. Bilateral iritis, vitritis, and choroidal thickening were evident. Exudative retinal detachment was present in the left eye. Corticosteroid treatment improved vision to 20/40 bilaterally. Methotrexate (MTX) was initiated and vision remained stable for 3 months. After a 5-month loss to follow-up, vision in the left eye decreased to finger counting (CF) and a parafoveal CNVM was identified. After 3 intravitreal ranibizumab injections, vision improved to 20/40. Twelve months later, despite inflammation control, vision decrease to CF due to recurrent CNVM. A fourth ranibizumab injection was given. Twenty months later, best-corrected vision was 20/400, and an inactive CNVM was present in the left eye.Conclusion. After initial CNVM regression and visual acuity improvement due to ranibizumab, the CNVM recurred and became refractory to treatment. Despite control of inflammation and neovascularization, VKH chronicity lead to permanent vision loss in our patient. A combinational treatment approach may be required in such patients.

High-risk syndrome for neuromyelitis optica: a descriptive and comparative study

2011 ◽  
Vol 17 (6) ◽  
pp. 720-724 ◽  
Author(s):  
N Collongues ◽  
R Marignier ◽  
H Zéphir ◽  
F Blanc ◽  
S Vukusic ◽  
...  
Keyword(s):  
High Risk ◽  
Optic Neuritis ◽  
Neuromyelitis Optica ◽  
Clinical Laboratory ◽  
Age At Onset ◽  
Igg Antibodies ◽  
Resonance Imaging ◽  
National Cohort ◽  
Magnetic Resonance Imaging Mri

Background: Neuromyelitis optica (NMO) frequently begins with a monofocal episode of optic neuritis or myelitis. A concept named high-risk syndrome (HRS) for NMO has been proposed for patients with monofocal episodes and NMO-IgG antibodies. Objective: To describe HRS patients and compare them with NMO patients. Methods: We identified 30 patients with HRS: 18 with extensive myelitis (HRM) and 12 with optic neuritis (HRON), in a database pooling patients from 25 centres in France. Clinical, laboratory/magnetic resonance imaging (MRI) data and outcome were analysed and compared with a national cohort of 125 NMO patients extracted from the same database. Results: Mean follow-up was 4.8 years. Mean age at onset was 42.8 years (range: 12.4–70) with a female:male ratio of 0.9. Asymptomatic lesions were report on visual evoked potentials in 4/8 tested HRM patients and on spinal cord MRI in 2/7 HRON patients. Three patients died, two owing to a cervical lesion. HRS and NMO patients had similar clinical/paraclinical data, except for a predominance of men in the HRS group and a later mean age at onset in the HRM subgroup. Conclusion: The description of HRS patients is compatible with a monofocal form of NMO. Asymptomatic lesions could be included in a new set of NMO diagnostic criteria.

scholarly journals Optic Neuritis Caused by Rathke’s Cleft Cyst in Young Adult

2014 ◽  
Vol 2014 ◽  
pp. 1-3
Author(s):  
Namie Kobayashi ◽  
Toshiyuki Oshitari ◽  
Kentaro Kobayashi ◽  
Takatsugu Onoda ◽  
Hidetoshi Ikeda ◽  
...  
Keyword(s):  
Visual Acuity ◽  
Young Adult ◽  
Optic Neuritis ◽  
Rathke’S Cleft Cyst ◽  
Rathke's Cleft Cyst ◽  
Rathke's Cleft ◽  
One Year ◽  
The Right ◽  
Transsphenoidal Resection

We report a case of right optic neuritis caused by Rathke's cleft cyst (RCC) in a young adult. A 15-year-old boy presented with reduced visual acuity in the right eye. He was diagnosed with optic neuritis in the right eye 4 years earlier at other clinics before he was referred to our department. During our one-year examinations, the cause of the reduced vision in his right eye could not be determined conclusively. At the age of 17 years, a RCC was detected by a neurosurgeon who specialized in hypophyseal diseases. He underwent microscopic transsphenoidal resection of the cyst, and his vision recovered to 1.2 and he has had no recurrence for at least 9 months. We suggest that repeated rupturing of the RCC was the cause of the optic neuritis, and a RCC can be successfully treated by surgery even after 3 years of optic neuritis.

Sign in / Sign up

Export Citation Format

Share Document