Clinical Features and Prognostic Factors in Anti-Myelin Oligodendrocyte Glycoprotein Antibody Positive Optic Neuritis.

In order to review the clinical features of anti-myelin oligodendrocyte glycoprotein antibody positive optic neuritis (MOGON), we investigated the clinical characteristics, visual function, optical coherence tomography findings, and magnetic resonance imaging of 31 patients (44 eyes). MOGON was more common in middle age without sex difference and was characterised by pain on eye movement and optic disc swelling. Magnetic resonance imaging lesions tended to be long with inflammation around the optic nerve sheath; longer lesions were associated with worse visual acuities at onset. Recurrence was significantly associated with retinal nerve fibre layer thinning, and thus, it is important to reduce recurrence as much as possible.

A Study of Neuromyelitis Optica Spectrum Disorders (NMOSD): Disease Pattern Based on Antibody Status.

Introduction: Neuromyelitis optica (NMO) is a central demyelinating disorder, predominantly affecting the optic nerves and spinal cord and autoimmune basis. We aimed to analyze the clinical, laboratory, and imaging features associated with NMO spectrum disorders (NMOSD) according to the aquaporin 4 antibody (AQP4-Ab) serology status. Methods: The inclusion of the patients was based on the Wingerchuk criteria (2006) for NMO, known antibody status and has minimum 1-year follow-up. We analyzed and compared 46 patients with known antibody status. Results: AQP4-Ab positivity was 56.5%. The male to female ratio in the seropositive group was 1:7.7 and 1:1.2 in the seronegative group. The mean age of onset in seropositive patients was 36.8 years (vs 28.8 years in seronegative NMOSD patients). Clinical feature, cerebrospinal fluid (CSF) and magnetic resonance imaging (MRI) features were also different, but data from two subsets did not reach statistical significance. The relapse rate was higher in AQP4 positive NMOSD (84.6% vs 55% in the seronegative group). The recovery rate for AQP4 positive patients was poor (15%). Summary: We found differences in age, gender, and prognosis between the two groups. Antibody status may be a guiding factor in deciding the treatment approach during the first attack of NMOSD.

The Oldest Japanese Case of Combined Central and Peripheral Demyelination, which Developed Nine Years After the First Instance of Optic Neuritis.

Combined central and peripheral demyelination (CCPD) causes demyelination in both the central and peripheral nervous systems. Anti-neurofascin 155 antibody plays an important pathogenic role in CCPD, but evidence concerning an association between this antibody and CCPD remains inconclusive. Although there have been no reports of precedent optic neuritis developing into CCPD, we herein report a Japanese man in whom optic neuritis recurred four times over nine years and who developed CCPD without positive anti-neurofascin 155 antibody. This case suggests the possibility of developing CCPD after optic nerve neuritis and the existence of an unknown antibody that induces CCPD.

Intravenous Immunoglobulin Treatment for Recurrent Optic Neuritis.

INTRODUCTION: Recurrent optic neuritis neuritis (rON) is an autoimmune inflammatory condition of unknown cause. Intravenous immunoglobulin (IVIg) treatment is used for many autoimmune disorders; however we do not have any information about its effect in rON, other than case reports. We aimed to evaluate our patients with rON who were treated with IVIg. METHODS: Data from all our patients with rON with or without anti aquaporin4 (AQP4) seropositivity, seen between April 2011 and October 2015, who received IVIg treatment were retrospectively evaluated. RESULTS: Nine patients (all female) with rON had received IVIg. These patients were aged between 34 and 65 years, and had started receiving monthly IVIg from 6 to 58 months after onset of disease. In three out of nine rON patients serum AQP4 antibody were positive. Under current treatments the patients had continued to have attacks, therefore monthly IVIg was given in addition to the existing immunosuppressant drug. The follow up duration was between 6 to 31 months. Three patients, each suffered one relapse under IVIg treatment. Mean number of relapses in the year prior to treatment was 1.4±0.72, whereas it was 0.3±0.5 during the year after IVIg therapy. During follow-up with IVIg administration only one patient had fever and no other adverse events were reported. CONCLUSION: Monthly IVIg is well-tolerated and safe and it seems to be effective in rON as an add on treatment. However, since our study is a retrospective case series, future randomized controlled trials with IVIg are needed.

Chronic Relapsing Inflammatory Optic Neuropathy.

Chronic relapsing inflammatory optic neuropathy (CRION) is a form of recurrent, isolated, subacute optic neuropathy. A 33-year-old female presented at an outpatient clinic with a pain-ful reduction of vision in the left eye that had developed 10 days earlier. The patient provided a background history of 5 similar attacks over the past 5 years. CRION was diagnosed following ophthalmological and imaging examinations, which revealed optic neuritis without demye-lination. The patient was successfully treated with steroids. The early detection of CRION is important because of the associated risk of blindness if CRION is treated inappropriately.

Anti-myelin oligodendrocyte glycoprotein antibodies: Magnetic resonance imaging findings in a case series and a literature review.

Myelin oligodendrocyte glycoprotein is a protein exclusively expressed on the surface of oligodendrocytes and myelin in the central nervous system. Antibodies against myelin oligodendrocyte glycoprotein were initially detected in children with demyelinating syndromes, and more recently reported in a broad spectrum of central nervous system demyelinating diseases in adults, including neuromyelitis optica spectrum disorders and bilateral optic neuritis. Patients with myelin oligodendrocyte glycoprotein antibody-associated demyelination appear to have unique clinical and radiological features. To the best of our knowledge a series of Italian patients with optic neuritis and positivity to myelin oligodendrocyte glycoprotein antibodies has not yet been reported and the paper on myelin oligodendrocyte glycoprotein antibodies are more focused on clinical features, diagnosis and outcome than on the radiological appearance, so we want to retrospectively report magnetic resonance imaging features of a group of eight patients, who came to our Ophthalmologic Emergency Department for optic neuritis and were found seropositive for myelin oligodendrocyte glycoprotein antibodies, comparing our data with the findings described in the literature.

Diagnostic implications of MOG/AQP4 antibodies in recurrent optic neuritis.

The present study aimed to detect myelin oligodendrocyte glycoprotein (MOG) and aquaporin-4 (AQP4) antibodies in serum specimens of patients with recurrent optic neuritis (RON) through establishing 293 cells with stable expression of MOG and the complete genomic sequence as the substrate using a cell-based assay (CBA). Furthermore, the clinical features of MOG antibody-positive recurrent optic neuritis (MOG-RON) were assessed. A total of 43 RON patients admitted to Beijing Tongren Hospital from December 2014 to May 2015 were enrolled, including 11 males and 32 females. The serum was collected from all patients, and the MOG and AQP4 antibodies were detected via the CBA. According to the results, the 43 patients were divided into four groups, namely the MOG antibody-positive group (n=11), the AQP4 antibody-positive group (n=20), the MOG/AQP4 antibody-positive group (n=1) and the MOG/AQP4 antibody-negative group (n=11). Clinical data were collected and all patients were followed up for 6 months, with parameters observed including the visual acuity, visual field and ocular fundus. The differences in the demographics, clinical features, characteristics of imaging examination, vision at onset and visual function recovery at 6 months after treatment were compared among the different groups. The characteristics of MOG antibody-positive RON were summarized. Of the 43 RON patients, 2.33% was both MOG and AQP4 antibody-positive, 27.91% were MOG antibody-positive. Compared with the AQP4-RON patients, there were relatively less MOG-RON patients (63.6 vs. 95.0%) and the canal segment and intracranial segment of the optic canal were less involved (P<0.05). The visual acuity at onset of MOG-RON was not inferior to that of AQP4-RON, and the visual recovery degree of MOG-RON was better (P<0.05). MOG antibody may be detected in the serum of certain RON patients, which have unique and different characteristics from AQP4 antibody-positive RON patients, so it may be used as a prognostic biomarker for RON. Furthermore, MOG antibody is present in the serum of patients with neuromyelitis optica spectrum disorders and may be a potential biomarker for these conditions.

Time is vision in recurrent optic neuritis.

In optic neuritis (ON) inflammation precedes onset of demyelination and axonal loss. The anti-inflammatory properties of corticosteroids may be most effective in the early inflammatory phase, but rapid patient recruitment remains a logistic challenge. The aim of the study was to review the effect of time to initiation of treatment on visual outcome in recurrent ON. A retrospective case note review of patients known to our centre with recurrent ON. The primary clinical outcome was change of best corrected high contrast visual acuity (BCVA). The secondary outcome was the change of optical coherence tomography (OCT) thickness of the peripapillary retinal nerve fibre layer (pRNFL) and macular ganglion cell layer (mGCL) from baseline and after a minimum of 3months following the episode of recurrent ON. Of 269 patients with a previous episode of ON, 54 experienced recurrent ON. In total 40 OCT documented episodes of relapsing ON were captured in 19 patients. Treatment within <2days led to better recovery of the BCVA (+0.02) and mGCL (-2.4µm) if compared to delayed treatment (BCVA -0.2, p=0.036, mGCL -25.6µm, p=0.019) or no corticosteroids treatment (BCVA -0.2, p=0.045, GCL -5.0µm, p=0.836). These data suggest a beneficial effect of hyperacute corticosteroid treatment. A pragmatic approach for a prospective treatment trial should consider patients with recurrent ON for logistic reasons.

Recurrent isolated optic neuritis: A study on 22 patients.

Background: Isolated relapsing optic neuropathy is a recurrent painful optic nerve inflammation without any sign of other demyelinating diseases such as multiple sclerosis (MS) or neuromyelitis optica (NMO) spectrum disorders, and the attacks are purely responsive to steroid therapy. Methods: Recurrent isolated optic neuritis (RION) was diagnosed in patients who presented with at least two disseminating episodes of optic neuritis, and negative clinical, para-clinical, and radiological features of the demyelinating, infiltrative and vasculitis disorders involving optic nerve. The patients were assigned into two groups, chronic recurrent isolated optic neuritis (CRION) entailing patients with steroid dependent attack of optic neuritis and RION patients without steroid dependent attack of optic neuritis. They were monitored over a median of 4.0 ± 2.5 years. Results: There were 16 women and six men with CRION and RION; with the median age of 31.7 ± 9.8 (29.3 ± 9.7 for women and 37.7 ± 7.7 for men). The women to men ratio was 2.6:1. The mean optic neuritis attack was 2.95 ± 1.32 in total. Eight patients were RION while 14 patients fulfilled CRION criteria and took long term immuno-suppressive drugs. In their follow-up, 4 out of 14 CRION cases (28.5%) showed clinical and concordant para-clinical features of NMO spectrum disorder. The analysis of demographic data showed that the average number of ON attacks in CRION patients (3.79 ± 2.32) was significantly more than the average in patients with RION (2.25 ± 0.46, P = 0.02). Conclusion: CRION is a disease which requires aggressive glucocorticoid and long-term immunosuppressive therapy to restore visual acuity.

Seronegative Neuromyelitis Optica: A Case Report of a Hispanic Male.

Neuromyelitis optica (NMO) is a rare disease, common in white females and rarely reported in Hispanic males. It is usually associated with recurrent demyelinating spectrum that is autoimmune in nature. The diagnosis is usually confirmed by antibody biomarkers; however, they can be negative and lead to more dilemma in diagnosis. Furthermore, the course of disease and prognosis are different in seronegative as compared to seropositive NMO. Treatment is similar in both subgroups with new approaches under investigation for seronegative NMO patients. We present an interesting case of a 37-year-old Hispanic male who presented with sudden onset of lower extremity weakness, numbness, blurry vision, and urinary retention. Magnetic resonance imaging (MRI) of the thoracic spine showed multiphasic demyelinating process involving the thoracic spinal cord. His brain MRI also revealed changes suggesting optic neuritis. The patient met the criteria for diagnosis of NMO by having optic neuritis and myelitis by imaging studies despite having negative aquaporin-4 antibodies (AQP4-Ab). His condition improved after plasma exchange. NMO can be difficult to distinguish from acute multiple sclerosis in the early stages of the disease. Having AQP4-Ab testing is important for diagnosis with imaging studies; however, negative antibody results cannot exclude the diagnosis, but rather group it in seronegative subtype. Ongoing studies and research suggest that seronegative NMO might have a different pathophysiology, manifestation, and prognosis.

Chronic relapsing inflammatory optic neuropathy.

Chronic relapsing inflammatory optic neuropathy (CRION) is a recently described recurrent optic neuropathy which is steroid responsive. Several features distinguish this entity from optic neuritis associated with demyelinating disorders and connective tissue diseases. The severe degree of visual loss, persistence of pain after onset of visual loss, and recurrent episodes are unique to this disorder. We describe here a patient who presented with recurrent episodes of painful visual loss, followed by resolution of deficits, over a period of ten years. He was diagnosed as isolated optic neuritis conforming to features of CRION.