Simultaneous Optic Neuritis Research Articles

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Risk and characteristics of attacks occurring after vaccination in patients with neuromyelitis optica spectrum disorders: A systematic review and meta-analysis.

Vaccination in patients with neuromyelitis optica spectrum disorders (NMOSD) is challenging because there is a concern that vaccines can lead to clinical attacks. However, little is known about the risk and the characteristics of attacks occurring after vaccination. We performed a systematic review and meta-analysis using PubMed and Embase databases to estimate a summary frequency of attacks occurring after vaccination and describe the clinical features of theses attacks. We defined attacks occurring after vaccination as typical NMOSD attacks that occurred up to 30 days after vaccine administration. For the frequency of attacks occurring after vaccination, we selected observational studies that reported the number of attacks and total number of patients that received vaccines; for the clinical description of the attacks, case reports and case series were also included. We included 377 participants from 5 studies to estimate the frequency of NMOSD attacks occurring after vaccination. We found a summary frequency of of 2% (95% CI 1-4%, I2=0%). We evaluated 17 studies to identify that 13 different vaccines were associated with NMOSD attacks. A higher-than-expected proportion of males, simultaneous optic neuritis and transverse myelitis attacks, and anti-aquaporin 4 antibody negative cases were identified in vaccine-associated attacks from 24 participants from 17 studies. Nearly two-thirds of attacks occurring after vaccination were an initial event of NMOSD. The frequency of NMOSD attacks occurring after vaccination is low and non-specific to different vaccine technologies. Our work reinforces the safety of vaccine recommendations in patients with NMOSD.

An interesting case of seronegative neuro myelitis optica spectrum disorder

Neuromyelitis optica spectrum disorder (NMOSD) is an autoimmune disease that causes severe demyelination, especially in the optic nerve and spinal cord with typical clinical manifestations of acute optic neuritis and transverse myelitis. Seventy to ninety percent of the cases of NMOSD test positive for aquaporin 4 IgG Antibodies (AQP4 IgG Ab). Here we report a case of 22-year-old female who presented with history of diffuse headache since 1 week, history of double vision since1 week and excessive day time sleepiness since2 months. Patient had a past history of bilateral and simultaneous optic neuritis 9 months back. On further evaluation patient was diagnosed as seronegative NMOSD (AQP4 IgG Ab–negative).

Clinical Profile and Treatment Outcome in MOGAD: A Single-Center Case-Series Study in Guiyang, China.

BackgroundThe clinical spectrum of myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) is expanding over time. However, the long-term management and prognosis of this disorder are still controversial. Therefore, this study aimed to report the clinical profiles and treatment outcomes of MOGAD in our center.MethodsThis was a single-center case-series study. Clinical and para-clinical data, along with treatment outcomes of patients with MOGAD were analyzed.ResultsA total of 27 patients were identified, of which 19 (70%) patients were women, and the median age at disease onset was 40 years (range 20–67). A total of 47 episodes were observed, with optic neuritis (53%) being the most frequent presentation and 60% of them were unilateral. Other presentations included rhombencephalitis (RE) (17%), limbic encephalitis (9%), simultaneous optic neuritis and myelitis (9%), acute disseminated encephalomyelitis (ADEM)-like presentation (6%), myelitis (4%), and ADEM (2%). One patient presenting with RE also met the diagnostic criteria of area postrema syndrome (APS). Another patient with RE presented with imaging characteristics of chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS). A total of 29 lumbar punctures were recorded, among which an elevated protein level was found in 34% of the samples, pleocytosis was found in 14% of the samples, and positive intrathecal oligoclonal bands were found in 19% of the patients. One patient was found to have anti-N-methyl-D-aspartate receptor antibodies both in his serum and cerebrospinal fluid. Intravenous methylprednisolone (IVMP) was administrated for 85% of the attacks while both IVMP and intravenous immunoglobulin were for 6% of the attacks. Moreover, nine patients received maintenance therapy. Among them, six patients were treated with mycophenolate mofetil, three patients were treated with prednisone, rituximab, and teriflunomide, respectively. The median follow-up period was 20 months (range 6–127). At follow-up, twelve (44%) patients experienced a relapsing course, and the median time to the first relapse was 9.5 months (range 2–120). The median Expanded Disability Status Scale score at nadir was 3.5 (range 2–8) and was 0 (range 0–3) at the last follow-up.ConclusionThe clinical spectrum of MOGAD is heterogenous, wherein APS and CLIPPERS-form can occur. The long-term outcome of MOGAD seems benign. Further studies are warranted to determine the risk factors of relapse and identify the optimal steroid-sparing agents.

Pediatric neuromyelitis optica spectrum disorders in Portugal: A multicentre retrospective study

IntroductionNeuromyelitis optica spectrum disorders (NMOSD) are more prevalent in adulthood, with few cases reported in pediatric age (<18 years). In this group, anti-aquaporin 4 (AQP4) antibodies are less frequent, while antibodies against myelin oligodendrocyte glycoprotein (MOG) are more commonly detectable than in adults. Objective and methodsDescription of pediatric NMOSD cases identified in a national multicentric NMOSD Portuguese registry. ResultsTwenty (11.1%) NMOSD cases were diagnosed in pediatric age. Twelve (60%) were female, with a median age of onset of 12.5 (6.8–16.5) years. The presenting feature was transverse myelitis in 10 (50%), 4 of which with simultaneous optic neuritis and 2 with concomitant brainstem syndrome. Nine patients (45%) had pleocytosis in the CSF. Six (30.0%) exhibited anti-AQP4 antibodies, 13 (65.0%) anti-MOG antibodies, and one was seronegative for both. Four anti-AQP4 antibodies-positive patients had ≥1 relapse. Most anti-MOG-positive cases were monophasic (53.8%). In the acute phase, all patients received IV methylprednisolone, nine received IVIg and four plasma exchange. One anti-AQP4-positive patient died. Ten patients (5 anti-AQP4-positive/5 anti-MOG-positive) were on maintenance immunosuppressive therapy at the time of data collection. ConclusionNMOSD may present in pediatric age. It is essential to establish the diagnosis and promptly start therapy to improve the prognosis.

Bilateral Simultaneous Retrobulbar Neuritis After Swine Flu: A Clinical Case Report

Retrobulbar neuritis is most commonly demyelinating and associated with multiple sclerosis. Rarely, it has been associated with viral and bacterial infections, systemic autoimmune diseases, vaccinations, and certain medications. We present a case of bilateral simultaneous optic neuritis following swine flu. We did a case report on a 56-year-old male patient with retrobulbar neuritis after swine flu, who had been treated in Alexandrovska Hospital, Sofia, Bulgaria. The diagnosis was based on the clinical exam, clinical course and on specialized investigations. The follow-up period was from February 2015 until April 2021. On admission in the hospital the best-corrected visual acuity of both eyes was decreased – VOD=0.6, VOS=PPLC. 30-2 visual field testing demonstrated no light sensitivity in the left eye, and decreased – in the right. Therapy was begun with methylprednisolone, pentoxiphylline, vinpocetine, piracetam, and clopidogrel. On day 19 the visual fields were significantly improved. On discharge, BCVA was 0.6-0.7 of the right eye and 0.7-0.8 – of the left, and improved to 1.0 during the follow-up period. Bilateral simultaneous retrobulbar neuritis is an infrequent immune complication after swine flu. In our case, we achieved recovery of normal visual acuity and a significant improvement in the visual fieds of both eyes. Therapy included systemic corticosteroids, pentoxiphylline, vinpocetine, piracetam, and clopidogrel.

Optic neuritis as the ocular manifestation of dengue infection: a case report

Optic neuritis is characterized by decreased vision, impaired colour perception, relative afferent pupillary defects, and scotoma. Optic neuritis following dengue infection is rare and might be underdiagnosed. The pathophysiology of optic neuritis after dengue infection is still unclear and there are only a few reports. We report a case of bilateral simultaneous optic neuritis in a young female adult following dengue haemorrhagic fever. On presentation, she complained of blurred vision, pain around the eyes, central scotoma, and progressively worsening visual acuity to no perception of light in both eyes. The next day, laboratory examination showed leucopoenia (6.74 [4.5–11.5 103/μL]) and lymphocytosis (52.7 [18–42%]), suggesting viral infection with positive anti-dengue IgM and IgG. The patient received intravenous pulse steroid therapy according to the Optic Neuritis Treatment Trial. At 3 months follow-up, best corrected visual acuity improved to 6/6, with pale optic discs but normal perimetry. Although complications of dengue fever in the eye are rare, early recognition must be established to prevent permanent vision loss.

Epidemiological findings of neuromyelitis optica spectrum disorders in a Venezuelan study

BackgroundNeuromyelitis optica spectrum disorders (NMOSD), is a rare autoimmune inflammatory disease of the central nervous system. Since the countries of Latin America (LATAM) show contrast in geographic, social, environmental factors, and genetic heterogeneity, the information about NMOSD epidemiology in the region allows a better understanding of the disease and its clinical outcome. ObjectivesTo determine the prevalence, relative frequency (RF), and clinical characteristics of NMOSD in a multiethnic Venezuelan cohort of patients with demyelinating disorders. MethodsWe conducted a retrospective descriptive multicenter study of hospital case records of individuals with an established diagnosis of MS and NMOSD in the National Program for Multiple sclerosis (MS) from 2011 to 2018. We selected those NMOSD cases based on the 2006 Wingerchuck and the 2015 International panel for the diagnosis of Neuromyelitis optica (IPND) criteria. ResultsWe identified 249 patients with NMOSD. The prevalence was 2.11 per100,000 individuals (95% confidence interval (CI)1.85 2.37), the RF was 23%, and the MS/NMOSD ratio was 3.2:1. The average disease onset occurred by the fourth decade of life (34±14.8 years of age); with a strong female predominance (female to male ratio: 4:1). Mestizos constituted 86,7% of this cohort. Most of the patients presented initially with simultaneous optic neuritis (ON) and acute transverse myelitis (ATM) and a recurrent course was registered in 82.3% of cases. The mean of the expanded disability status scale (EDSS) was 3.5 (IQR 2-7). Abnormal brain and spine MRI were present in 47.8% and 81.1% of patients, respectively. Antibodies against aquaporin-4 (AQP4) which were measured through a cell-based assay were positive in 55.3% of the individuals tested. The most used immunosuppressant agent was Azathioprine (57.4%). ConclusionNMOSD in Venezuela affects mainly young Mestizo women and shows one of the highest relative frequency in the region. Planning and developing healthcare programs for underserved populations as well as more comprehensive LATAM studies are required to identify the distribution and variations of its epidemiological picture.

Do Myelin Oligodendrocyte Glycoprotein Antibodies Represent a Distinct Syndrome?

Do Myelin Oligodendrocyte Glycoprotein Antibodies Represent a Distinct Syndrome?

Distinction between MOG antibody-positive and AQP4 antibody-positive NMO spectrum disorders

To evaluate clinical features among patients with neuromyelitis optica spectrum disorders (NMOSD) who have myelin oligodendrocyte glycoprotein (MOG) antibodies, aquaporin-4 (AQP4) antibodies, or seronegativity for both antibodies. Sera from patients diagnosed with NMOSD in 1 of 3 centers (2 sites in Brazil and 1 site in Japan) were tested for MOG and AQP4 antibodies using cell-based assays with live transfected cells. Among the 215 patients with NMOSD, 7.4% (16/215) were positive for MOG antibodies and 64.7% (139/215) were positive for AQP4 antibodies. No patients were positive for both antibodies. Patients with MOG antibodies represented 21.1% (16/76) of the patients negative for AQP4 antibodies. Compared with patients with AQP4 antibodies or patients who were seronegative, patients with MOG antibodies were more frequently male, had a more restricted phenotype (optic nerve more than spinal cord), more frequently had bilateral simultaneous optic neuritis, more often had a single attack, had spinal cord lesions distributed in the lower portion of the spinal cord, and usually demonstrated better functional recovery after an attack. Patients with NMOSD with MOG antibodies have distinct clinical features, fewer attacks, and better recovery than patients with AQP4 antibodies or patients seronegative for both antibodies.

Longitudinally Extensive Transverse Myelitis With and Without Aquaporin 4 Antibodies

Aquaporin 4 antibody (AQP4-Ab)-negative patients with longitudinally extensive transverse myelitis (LETM) behave differently from those with AQP4-Ab. Aquaporin 4 antibody-negative neuromyelitis optica (NMO) is rare when good assays are used. To assess if AQP4-Ab-negative patients with LETM share similar disease characteristics with AQP4-Ab-positive patients or whether they have distinct features and alternative diagnoses. DESIGN We collated clinical and paraclinical data on patients with LETM identified through the Oxford NMO clinical database. Aquaporin 4 antibodies were tested using 2 sensitive assays. We describe the features of patients with LETM, compare findings between patients with and without AQP4-Ab, and describe alternative diagnoses in AQP4-Ab-negative patients. Single specialist UK center for NMO. Seventy-six adult patients with LETM. Comparison of clinical and paraclinical data. Fifty-eight percent of patients were AQP4-Ab positive. Alternative diagnoses could usually be identified in AQP4-Ab-negative patients, including those fulfilling NMO diagnostic criteria. Only 6.5% of patients had "true" seronegative NMO and 6.5% had idiopathic LETM. There were some important differences between AQP4-Ab-positive and -negative cases, including older onset age, higher proportion of females, lower incidence of simultaneous optic neuritis, lower frequency of conus involvement, and higher prevalence of coexisting autoimmune disorders in AQP4-Ab-positive cases. Attack severity and degree of recovery were similar in the 2 groups. Patients with LETM without AQP4-Ab include a number of different diagnostic categories and it is not surprising therefore that they show important differences compared with AQP4-Ab-positive patients, even when considering only those fulfilling current NMO diagnostic criteria. Thus, we suggest that diagnoses such as myelin-oligodendrocyte glycoprotein antibody disease, multiple sclerosis, acute disseminated encephalomyelitis, and postinfectious disorders should be exclusions in the NMO diagnostic criteria and AQP4-Ab-positive and antibody-negative NMO/NMO spectrum disorder cohorts should be analyzed separately.

Glycine receptor and myelin oligodendrocyte glycoprotein antibodies in Turkish patients with neuromyelitis optica

BackgroundSeronegative NMO is highly prevalent in non-Western countries implying the presence of as yet unknown antibodies (Ab). We investigated potential novel Ab in aquaporin-4 Ab (AQP-4-Ab) positive and negative NMO patients. MethodsSera of 20 NMO patients were examined for AQP-4, myelin oligodendrocyte glycoprotein (MOG) and glycine receptor (GlyR) Ab by cell-based assays. ResultsAQP-4-Ab was identified in 10 NMO patients, MOG-Ab was detected only in one AQP-4-Ab positive patient and GlyR-Ab was detected in two AQP-4-Ab negative patients. GlyR-Ab positive patients displayed simultaneous optic neuritis and transverse myelitis attacks and relatively low disability, whereas MOG and AQP-4-Ab double positive patient had a significantly increased disability. ConclusionThis study showed for the first time the presence of GlyR-Ab in Turkish NMO patients. In contrast with previous reports, MOG Ab does not appear to be a distinctive marker for Turkish AQP-4-Ab negative NMO patients.

Updated estimate of AQP4-IgG serostatus and disability outcome in neuromyelitis optica

To 1) determine, using contemporary recombinant antigen-based assays, the aquaporin-4 (AQP4)-immunoglobulin G (IgG) detection rate in sequential sera of patients assigned a clinical diagnosis of neuromyelitis optica (NMO) but initially scored negative by tissue-based indirect immunofluorescence (IIF) assay; and 2) evaluate the impact of serostatus on phenotype and outcome. From Mayo Clinic records (2005-2011), we identified 163 patients with NMO; 110 (67%) were seropositive by IIF and 53 (33%) were scored seronegative. Available stored sera from 49 "seronegative" patients were tested by ELISA, AQP4-transfected cell-based assay, and in-house fluorescence-activated cell sorting assay. Clinical characteristics were compared based on final serostatus. Thirty of the 49 IIF-negative patients (61%) were reclassified as seropositive, yielding an overall AQP4-IgG seropositivity rate of 88% (i.e., 12% seronegative). The fluorescence-activated cell sorting assay improved the detection rate to 87%, cell-based assay to 84%, and ELISA to 79%. The sex ratio (female to male) was 1:1 for seronegatives and 9:1 for seropositives (p < 0.0001). Simultaneous optic neuritis and transverse myelitis as onset attack type (i.e., within 30 days of each other) occurred in 32% of seronegatives and in 3.6% of seropositives (p < 0.0001). Relapse rate, disability outcome, and other clinical characteristics did not differ significantly. Serological tests using recombinant AQP4 antigen are significantly more sensitive than tissue-based IIF for detecting AQP4-IgG. Testing should precede immunotherapy; if negative, later-drawn specimens should be tested. AQP4-IgG-seronegative NMO is less frequent than previously reported and is clinically similar to AQP4-IgG-seropositive NMO.

Aquaporin-4 antibody–negative neuromyelitis optica

To optimize aquaporin-4 (AQP4) antibody (Ab) detection and to assess the influence of the increased sensitivity of the assay on the demographic and disease-related characteristics of a group of AQP4-Ab-negative patients. Serum samples were obtained from patients included in the French NOMADMUS database with a definite diagnosis of neuromyelitis optica (NMO) (n = 87) and were compared with controls (n = 54). They were tested by indirect immunofluorescence and cell-based assays (CBAs) in various conditions and with several plasmids. We identified the CBA on live cells transfected with the untagged AQP4-M23 isoform as the best method, with a sensitivity of 74.4% and a specificity of 100%. We demonstrated a direct relationship between improvement of the sensitivity of the detection method and the distinctiveness and characteristics of the AQP4-Ab-negative NMO group. Whereas with the classic indirect immunofluorescence or current AQP4-M1 CBA we found only slight differences between the 2 populations, using the AQP4-M23 CBA, we demonstrated that patients with AQP4-Ab-negative NMO expressed specific demographic and disease-related features. They were characterized by an equal male/female ratio (p < 0.001), a Caucasian ethnicity (p = 0.029), and an overrepresentation of simultaneous optic neuritis and transverse myelitis at first episode (p = 0.015). In terms of disability, they experienced a better visual acuity at last follow-up compared with seropositive NMO (p = 0.007). This raises the question of a distinct physiopathology for patients with AQP4-Ab-negative NMO and of their place in the spectrum of the disease.

Neuromyelitis optica antibody in Leber hereditary optic neuropathy: case report

Neuromyelitis optica antibody (or aquaporin-4 antibody) is a well established serum marker associated to high-risk neuromyelitis optica syndrome that presents as an inflammatory demyelinating disease characterized by the occurrence of bilateral and simultaneous optic neuritis without complete visual recovery or it occurs as an isolated episode of transverse myelitis accompanied by longitudinally extensive spinal cord lesions. On the other hand, Leber hereditary optic neuropathy is a primarily hereditary disorder that affects all tissues of the body and its clinical presentation is tissue-specific for the optic nerve and, eventually, it might reach the spinal cord. Overlapping clinical features of neuromyelitis optica and Leber hereditary optic neuropathy may suggest common target organ diseases. The case report described herein emphasizes the coexistence of serum markers of both diseases, and suggests that further investigation of this challenging clinical presentation is warranted to confirm or rule out this association.

Pediatric optic neuritis and risk of multiple sclerosis: Meta-analysis of observational studies

To determine the relationships among age, unilateral versus bilateral simultaneous presentation, magnetic resonance imaging (MRI) lesions, and the risk of multiple sclerosis (MS) in children with optic neuritis. A systematic literature review and meta-analysis were performed by the use of a MEDLINE search to identify published studies containing individual patient data for children with optic neuritis. Age, laterality (unilateral vs bilateral simultaneous optic neuritis), presence of brain MRI abnormalities, and development of MS were recorded. Logistic regression was used to determine the relationships among these parameters. Fourteen studies met inclusion criteria. Data for 223 patients (age range 2-17.8 years) were analyzed. Unilateral optic neuritis occurred more frequently in older children but was not associated with an increased risk of MS, after adjusting for age (odds ratio [OR] = 1.9, P=0.11; 95% confidence interval [CI], 0.9-4.3). For every 1-year increase in age, the odds of developing MS increased by 32% (OR = 1.3, P = 0.005; 95% CI, 1.1-1.6, adjusted for the presence of MRI lesions). The risk of MS was greater in children with abnormal brain MRI scans at presentation compared with normal MRIs (OR = 28.0, P < 0.001, 95% CI, 6.3-125.1, adjusted for age). The relationship between unilateral and bilateral optic neuritis and the development of MS is dependent on age. Older children and those with brain MRI abnormalities at presentation, are at greater risk for MS. Long-term follow-up of children with optic neuritis is needed to establish the true risk for the development of MS.

Clinical characteristics of multiple sclerosis and associated optic neuritis in Korean children

To study the clinical characteristics of multiple sclerosis and associated optic neuritis in Korean children. A retrospective analysis was performed on 10 patients with an onset of multiple sclerosis before age 16. Information on sex, age of onset, clinical course, laboratory findings, and clinical characteristics of optic neuritis was obtained. The mean age at presentation was 7.31 +/- 2.99 years, and the mean duration of observation was 36.2 +/- 26.1 months. No female predilection (50%) was observed. The disease presented as relapsing-remitting type multiple sclerosis in all patients and transited to secondary progressive type in two cases (20%). No oligoclonal bands were found in any patient. Optic neuritis occurred in eight patients (80%); five (62.5%) of these had optic neuritis at the first multiple sclerosis attack, with all five manifesting bilateral simultaneous optic neuritis. Visual acuity recovered to > or =20/40 in 8 of 15 eyes (53.3%), but in 2 eyes (13.3%) visual acuity remained at < or =20/200. In the patients with optic neuritis, the patients who showed optic neuritis at initial presentation had a worse visual prognosis (p = 0.030, Mann-Whitney U-test). In Korean children with multiple sclerosis, age of onset was younger than reported in other countries, and there was no female predominance. The prognosis for good visual acuity was worse in patients who initially presented with optic neuritis.

Acute Bilateral Simultaneous Optic Neuritis

Background : Acute bilateral simultaneous optic neuritis (ABSON) without a prior neurological history is a rare clinical problem, and little is known about its prognosis for visual recovery and for the development of multiple sclerosis (MS). Methods : We conducted a case review of six patients who presented with ABSON from 1986-98, and did a follow-up on their present medical status. The results were compared to similar case reviews, and to the unilateral Optic Neuritis Treatment Trial. Results : All six patients went on to recover normal vision. One of the six patients (16.7%) reviewed went on to develop probable MS. Interpretation : This low association with MS is in agreement with other case reports of ABSON. ABSON appears to have a significantly better prognosis for not developing neurological disease than unilateral optic neuritis. In particular, patients with ABSON without associated systemic symotoms tend to recover quickly to normal health.