scholarly journals Remedial Effect of Intravenous Cyclophosphamide in Corticosteroid-Refractory Patients in the Acute Phase of Neuromyelitis Optica Spectrum Disorder-Related Optic Neuritis

2021 ◽  
Vol 11 ◽  
Author(s):  
Ling Wang ◽  
Kaiqun Liu ◽  
Xiao Tan ◽  
Lin Zhou ◽  
Yuxin Zhang ◽  
...  
Keyword(s):  
Optic Neuritis ◽  
Acute Phase ◽  
Neuromyelitis Optica ◽  
Visual Evoked Potential ◽  
Evoked Potential ◽  
Spectrum Disorder ◽  
Neuromyelitis Optica Spectrum Disorder ◽  
Intravenous Cyclophosphamide ◽  
Rnfl Thickness

Background: To investigate the remedial efficacy and safety of intravenous cyclophosphamide (CP) in the acute phase in patients with neuromyelitis optica spectrum disorder-related optic neuritis (NMOSD-ON) who are refractory to intravenous methylprednisolone (MP) treatment.Design: This study was a single-center, retrospective, observational case-control cohort study.Methods: Thirty-six patients who had acute NMOSD-ON attacks and were refractory to MP treatment were included. Patents were divided into two groups: the remedial CP group, and the MP group. The best-corrected visual acuity (BCVA), mean deviation (MD) of the visual field (VF), visual evoked potential amplitude (VEP-A), visual evoked potential latency (VEP-T), and average thickness of the retinal nerve fiber layer (RNFL) at onset, 1 month (m), 3 m, and 6 m after the attack were analyzed. Routine blood test results, liver and kidney function, routine urinalysis results and general condition were analyzed for safety issues at each follow-up. Fisher's exact test, the Mann-Whitney U test, the Kruskal-Wallis test and the Wilcoxon rank-sum test were used for statistical analysis.Results: The remedial CP group showed significant improvement over 6 m with regard to BCVA and MD (P < 0.05),whereas MP group only showed significant improvement in MD (P < 0.05). Regarding remedial CP intervention time window, the CP ≤ 30 days group showed significant improvement over 6 m with regard to BCVA (P = 0.002), MD (P = 0.003), and VEP-A (P = 0.036), while those CP > 30 days group did not. Both two subgroups showed significantly RNFL thickness reduction, however, BCVA, MD, VEP-A, VEP-T, and RNFL thickness showed no significant differences between the two subgroups at any follow-up point (P > 0.05).Conclusion: CP within 30 days of attack onset is safe and might have a beneficial degree of therapeutic efficacy for acute-phase treatment of NMOSD-ON that is refractory to MP treatment alone.

scholarly journals Gender effect on neuromyelitis optica spectrum disorder with aquaporin4-immunoglobulin G

2016 ◽  
Vol 23 (8) ◽  
pp. 1104-1111 ◽  
Author(s):  
Sung-Min Kim ◽  
Patrick Waters ◽  
Mark Woodhall ◽  
Yoo-Jin Kim ◽  
Jin-Ah Kim ◽  
...  
Keyword(s):  
Optic Neuritis ◽  
Neuromyelitis Optica ◽  
Immunoglobulin G ◽  
Age At Onset ◽  
Disease Onset ◽  
Clinical Manifestations ◽  
Spectrum Disorder ◽  
Pattern Reversal ◽  
Neuromyelitis Optica Spectrum Disorder

Background: Neuromyelitis optica spectrum disorder with aquaporin4-immunoglobulin G (NMOSD-AQP4) is an inflammatory disease characterised by a high female predominance. However, the effect of gender in patients with NMOSD-AQP4 has not been fully evaluated. Objective: The aim of this study was to determine the effect of gender in clinical manifestations and prognosis of patients with NMOSD-AQP4. Methods: The demographics, clinical and radiological characteristics, pattern reversal visual evoked potential (VEP) test results, and prognosis of 102 patients (18 males) with NMOSD-AQP4 were assessed. Results: Male patients had a higher age at onset (48.7 vs 41 years, p = 0.037) and less optic neuritis as the onset attack (17% vs 44%, p = 0.026), higher tendency to manifest as isolated myelitis over the follow-up period (67% vs 28%, p = 0.005), fewer optic neuritis attacks per year (0.08 vs 0.27, p < 0.001), and shorter relative P100 latency on VEP testing (97.1% vs 108.3%, p = 0.001). Moreover, male gender was significantly associated with the absence of optic neuritis attacks over the follow-up period independent of their age of onset. Conclusion: In NMOSD-AQP4 patients, gender impacts on disease onset age and site of attack. This may be an important clue in identifying NMOSD-AQP4 patients with limited manifestations as well as in predicting their clinical courses.

scholarly journals Aquaporin-4-IgG-positive neuromyelitis optica spectrum disorder with recurrent short partial transverse myelitis and favorable prognosis: Two new cases

2017 ◽  
Vol 23 (14) ◽  
pp. 1950-1954 ◽  
Author(s):  
Jinhua Zhang ◽  
Fang Liu ◽  
Yiqi Wang ◽  
Ying Yang ◽  
Yuehong Huang ◽  
...  
Keyword(s):  
Neuromyelitis Optica ◽  
Transverse Myelitis ◽  
Acute Attack ◽  
Spectrum Disorder ◽  
Aquaporin 4 ◽  
Neuromyelitis Optica Spectrum Disorder ◽  
Attack Phase ◽  
Long Term Prognosis

Understanding the characteristics of neuromyelitis optica spectrum disorder (NMOSD) with recurrent short partial transverse myelitis (SPTM), which is very rare, contributes to the differential diagnosis of multiple sclerosis (MS). We present two Chinese aquaporin-4 immunoglobulin G (AQP4-IgG)-seropositive NMOSD cases who had at least twice SPTM during 4 and 6 years of follow-up, respectively. Their SPTMs have been mild and responded well to corticosteroids just like in the case of MS. The findings highlight the need of searching for serum AQP4-IgG (cell-based assay strongly recommended) in patients with recurrent SPTM and suggest that those patients may have a mild acute attack phase and favorable long-term prognosis.

Optic Neuritis–Independent Retinal Atrophy in Neuromyelitis Optica Spectrum Disorder

2021 ◽  
Vol Publish Ahead of Print ◽  
Author(s):  
Angeliki G. Filippatou ◽  
Eleni S. Vasileiou ◽  
Yufan He ◽  
Kathryn C. Fitzgerald ◽  
Grigorios Kalaitzidis ◽  
...  
Keyword(s):  
Optic Neuritis ◽  
Neuromyelitis Optica ◽  
Spectrum Disorder ◽  
Neuromyelitis Optica Spectrum Disorder ◽  
Retinal Atrophy

scholarly journals Clinical Features and Visual outcome in Acute and Recurrent Case of Optic Neuritis.

2018 ◽  
Vol 1 (01) ◽  
pp. 56-63
Author(s):  
Jyoti Bastola Paudel ◽  
Ananda Kumar Sharma ◽  
Sanjeeta Sitaula ◽  
Madhu Thapa
Keyword(s):  
Multiple Sclerosis ◽  
Natural History ◽  
Optic Neuritis ◽  
Clinical Features ◽  
Visual Evoked Potential ◽  
Evoked Potential ◽  
Oral Steroid ◽  
Visual Recovery ◽  
Visual Evoked

Introduction: Optic neuritis is an inflammation of the optic nerve that usually affects young females. In Western countries, natural history and treatment of optic neuritis(ON) has been studied extensively. However aetiology, natural history, clinical features of ON and their relation to multiple sclerosis in Asian population needs to be defined yet. Methods: 30 patients who were diagnosed as optic neuritis were included between June 2013 to December 2014 at BP Koirala Lions Centre for Ophthalmic Studies (BPKLCOS). A detailed history was obtained followed by examination of anterior and posterior segment. Assessment of visual acuity, color vision, contrast sensitivity, visual evoked potential (VEP),visual field and MRI of orbit and brain was done in all cases. All patients were treated with intravenous Methylprednisolone 500mg twice daily for 3 days followed by oral steroid for 11 days which was tapered in the next 4 days.The patients were reassessed at 2 weeks, 1 month and 3months. Results: Commonest presenting symptom was diminution of vision(65%). MRI showed multiple paraventricular oval plaques definite of multiple sclerosis in one patient and one was diagnosed as probable MS who had a single periventricular plaque. Visual evoked potential (VEP) showed increase in the mean P100 latency at 60’ and reduction in amplitude in eyes affected with optic neuritis compared to normal eyes. At 3 months follow up, 70% had good visual recovery (>6/18). The cause of non-improvement in vision was disc pallor. Optic disc pallor was detected in 37.5% of the eyes during follow up. Conclusions: Good visual recovery was observed in most eyes with acute optic neuritis. Multiple sclerosis was seen in 1 patient who had recurrent optic neuritis.

Multifocal visual evoked potential evaluation for diagnosis of acute optic neuritis and for prediction of visual outcome and ganglion cell layer thinning following optic neuritis

2020 ◽  
pp. 135245852097573
Author(s):  
Gorm Pihl-Jensen ◽  
Benedikte Wanscher ◽  
Jette Lautrup Frederiksen
Keyword(s):  
Optic Neuritis ◽  
Ganglion Cell ◽  
Visual Function ◽  
Visual Evoked Potential ◽  
Evoked Potential ◽  
Conduction Block ◽  
Visual Outcome ◽  
Multifocal Visual Evoked Potential ◽  
Visual Evoked

Background:: While damage to the optic nerve following optic neuritis (ON) is readily quantifiable, the evaluation of prognosis for visual function and neuroaxonal loss in the acute ON is challenging. Objective:: The objective of this study is to investigate the value of multifocal visual evoked potential (mfVEP) in acute ON, diagnostically for acute ON and prognostically for visual outcome and subsequent ganglion cell/inner plexiform layer thickness (GCLIPLt). Methods:: A prospective cohort study of mfVEP and full-field visual evoked potential (ffVEP) in acute, unilateral ON (onset < 31 days) was conducted. Comparisons with healthy controls ( n = 30) and association analysis with follow-up optical coherence tomography (OCT) measurements (of the GCLIPLt) and visual function (Sloan low-contrast visual acuity (LCVA)) were conducted. Results:: Seventy-nine ON patients were included (mean: 17 days from onset). Excluding measurements with conduction block, ffVEP ( n = 54) and mfVEP ( n = 44) showed sensitivities of 89% and 84% to a specificity of 97%. 65/79 patients were re-examined (mean: 200 days follow-up). mfVEP amplitude and latency inter-eye asymmetry in acute ON correlated with GCLIPLt ( r = 0.587 and Spearman’s ρ = 0.597, for both, p < 0.001). mfVEP amplitude correlated with LCVA inter-eye asymmetry at follow-up ( r = 0.421, p < 0.001), mfVEP latency did not. Conclusion: mfVEP may support the prognostic evaluation of acute ON patients and prove valuable in future neuroprotective and remyelinating trials. In acute ON, the increase in diagnostic value of mfVEP to ffVEP may be limited due to widespread conduction block.

scholarly journals Neuromyelitis Optica Spectrum Disorder and Anti-MOG Syndromes

Biomedicines ◽  
2019 ◽  
Vol 7 (2) ◽  
pp. 42 ◽  
Author(s):  
Marco A. Lana-Peixoto ◽  
Natália Talim
Keyword(s):  
Optic Neuritis ◽  
Neuromyelitis Optica ◽  
Area Postrema ◽  
Water Channel ◽  
Clinical Manifestations ◽  
Transverse Myelitis ◽  
Myelin Oligodendrocyte Glycoprotein ◽  
Immunosuppressive Drugs ◽  
Spectrum Disorder ◽  
Neuromyelitis Optica Spectrum Disorder

Neuromyelitis optica spectrum disorder (NMOSD) and anti-myelin oligodendrocyte glycoprotein (anti-MOG) syndromes are immune-mediated inflammatory conditions of the central nervous system that frequently involve the optic nerves and the spinal cord. Because of their similar clinical manifestations and habitual relapsing course they are frequently confounded with multiple sclerosis (MS). Early and accurate diagnosis of these distinct conditions is relevant as they have different treatments. Some agents used for MS treatment may be deleterious to NMOSD. NMOSD is frequently associated with antibodies which target aquaporin-4 (AQP4), the most abundant water channel in the CNS, located in the astrocytic processes at the blood-brain barrier (BBB). On the other hand, anti-MOG syndromes result from damage to myelin oligodendrocyte glycoprotein (MOG), expressed on surfaces of oligodendrocytes and myelin sheaths. Acute transverse myelitis with longitudinally extensive lesion on spinal MRI is the most frequent inaugural manifestation of NMOSD, usually followed by optic neuritis. Other core clinical characteristics include area postrema syndrome, brainstem, diencephalic and cerebral symptoms that may be associated with typical MRI abnormalities. Acute disseminated encephalomyelitis and bilateral or recurrent optic neuritis are the most frequent anti-MOG syndromes in children and adults, respectively. Attacks are usually treated with steroids, and relapses prevention with immunosuppressive drugs. Promising emerging therapies for NMOSD include monoclonal antibodies and tolerization.

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