scholarly journals Atypical Multiple Sclerosis Presenting as Bilateral Optic Neuritis

2021 ◽  
Vol 20 (4) ◽  
pp. 182-188
Author(s):  
Sun Young Lee ◽  
Mi-Ra Park

Purpose: We report a case of multiple sclerosis with bilateral optic neuritis that was atypical in terms of both the clinical signs and symptoms.Case summary: A 34-year-old female visited the department of neurology with a complaint of sudden-onset, left lower extremity weakness and numbness that had developed 3 weeks prior, and bilateral blurred vision that had commenced 2 weeks prior. The patient was diagnosed with multiple sclerosis and prescribed high-dose intravenous methylprednisolone for 3 days, but the blurred vision did not improve. The patient was referred to the ophthalmology department. The initial best-corrected visual acuity was 0.04 in both eyes, and the relative afferent pupillary defect test was positive for the left eye. The Ishihara color test scores were 1/17 for both eyes. No optic disc swelling was evident on fundus examination. Brain and spine magnetic resonance imaging (T2-weighted) performed during the initial visit revealed lesions of high signal intensity in the frontal, subcortical white matter; the optic chiasm; the left retrobulbar optic segment; the medulla; and the spinal C2-5 processes. Aquaproin-4 immunogloblin G antibody (AQP4-IgG Ab) was not detected in serum. One month after prescription of the oral steroid, the vision improved to 1.0 in the right and 0.8 in the left eye. The patient was lost to follow-up after prescription of interferon-beta for 4 years, without recurrence.Conclusions: Binocular optic neuritis accompanied by severe visual loss is a rare form of multiple sclerosis. A thorough diagnosis (with a focus on exclusion) is required, as is appropriate treatment.

2016 ◽  
Vol 8 (2) ◽  
pp. 102-107 ◽  
Author(s):  
Nabeel Badri ◽  
Mohamed Teleb ◽  
Saad Syed ◽  
Miraie Wardi ◽  
Mateo Porres-Aguilar ◽  
...  

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.


2021 ◽  
Vol 19 (1) ◽  
Author(s):  
Stephanie Wong ◽  
Erkan Demirkaya ◽  
Roberta Berard

Abstract Background Neonatal systemic vasculitis syndromes have been reported in infants born to mothers with systemic lupus erythematosus, Sjögren’s syndrome, Behҫet’s disease, cutaneous polyarteritis nodosa and anti-neutrophil cytoplasmic antibody-associated vasculitides. Here we report a novel association of a case of new-onset maternal seronegative inflammatory arthritis associated with a transient systemic vasculitis in a neonate. Case presentation In the first 24 h of life, a preterm Caucasian baby boy was noted to have blue discoloration to all four extremities. Despite antibiotics, fresh frozen plasma and anticoagulation, the discoloration remained, particularly in the left index finger. This was associated with fever and a maximum C-reactive protein (CRP) of 148 mg/L. Intravenous immunoglobulin (IVIG) was given with short-term improvement. Initial echocardiogram showed enlarged coronary arteries with normalization on repeat 1 week later. Clinical signs and symptoms responded to high dose oral steroid administration. MRI angiography (MRA) of the body and heart showed tortuosity of arteries in the upper and lower extremities with gadolinium uptake, suggestive of vasculitis. Autoantibody profile negative. Genetic panel for hereditary autoinflammatory diseases was negative as was whole exome sequencing performed on the trio. The baby was weaned off steroids by 5 months of age. A small distal autoamputation of the left index finger occurred. He was born to a 28-year-old woman who developed new onset severe symmetrical polyarthritis at 8 weeks gestation. This was presumed a reactive arthritis secondary to a dental infection. Infectious work up and autoantibodies were negative. She was treated with high dose prednisone for the remainder of her pregnancy. The mother was weaned off prednisone, treated with hydroxychloroquine for 8 months post-partum and remains in remission. A repeat MRA done at 1 year old showed mild residual tortuosities of the arteries in the forearms. The remainder of the medium and large vessels were within normal limits with no gadolinium enhancement to suggest active disease. The child is now 4 years old with normal growth and development. Conclusion This is a unique case of new-onset seronegative presumed reactive arthritis in a mother with the rare development of a successfully treated medium vessel vasculitis in an infant.


2018 ◽  
Vol 1 (01) ◽  
pp. 56-63
Author(s):  
Jyoti Bastola Paudel ◽  
Ananda Kumar Sharma ◽  
Sanjeeta Sitaula ◽  
Madhu Thapa

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.


CNS Drugs ◽  
2018 ◽  
Vol 32 (7) ◽  
pp. 661-672 ◽  
Author(s):  
Ayman Tourbah ◽  
Olivier Gout ◽  
Alain Vighetto ◽  
Véronique Deburghgraeve ◽  
Jean Pelletier ◽  
...  

2008 ◽  
Vol 25 (3) ◽  
pp. 463-468 ◽  
Author(s):  
ANA LAURA DE ARAÚJO MOURA ◽  
ROSANI APARECIDA ANTUNES TEIXEIRA ◽  
NESTOR N. OIWA ◽  
MARCELO F. COSTA ◽  
CLAUDIA FEITOSA-SANTANA ◽  
...  

We assessed chromatic discrimination in multiple sclerosis (MS) patients both with (ON) and without (no ON) a history of optic neuritis using the Cambridge color test (CCT). Our goal was to determine the magnitude and chromatic axes of any color vision losses in both patient groups, and to evaluate age-related changes in chromatic discrimination in both patient groups compared to normals. Using the CCT, we measured chromatic discrimination along the protan, deutan and tritan axes in 35 patients with MS (17 ON eyes) and 74 age matched controls. Color thresholds for both patient groups were significantly higher than controls' along the protan and tritan axes (p < 0.001). In addition, the ON and no-ON groups differed significantly along all three-color axes (p < 0.001). MS patients presented a progressive color discrimination impairment with age (along the deutan and tritan axes) that was almost two times faster than controls, even in the absence of ON. These findings suggest that demyelinating diseases reduce sensitivity to color vision in both red-green and blue-yellow axes, implying impairment in both parvocellular and koniocellular visual pathways. The CCT is a useful tool to help characterize vision losses in MS, and the relationship between these losses and degree of optic nerve involvement.


2021 ◽  
Vol 62 (8) ◽  
pp. 1160-1166
Author(s):  
Su Hwan Park ◽  
Min Seung Kang ◽  
Sang Yoon Kim ◽  
Ji-Eun Lee ◽  
Su Jin Kim

Purpose: We report a case of optic neuritis related to infliximab treatment in a patient with Crohn’s disease, along with a review of the relevant literature.Case summary: A 22-year-male patient complained of blurred vision in the left eye for 2-3 weeks. His best-corrected visual acuity was 8/20 in the left eye. Relative afferent pupillary defect was detected in the left eye and the pupils were of equal size. There was no pain on ocular movement. The results of slit-lamp and fundus examinations were normal. A visual field test revealed a central to inferior visual field defect in the left eye. Orbit magnetic resonance imaging revealed perineural enhancement of the left optic nerve. He had a 5-year history of Crohn’s disease and had been treated with intravenous infliximab (600 mg every 2 weeks). A diagnosis of retrobulbar optic neuritis associated with infliximab was made. He was infused with high-dose methylprednisolone, which was changed to per oral administration. His visual acuity and visual field defect improved after 3 months.Conclusions: Tumor necrosis factor-α inhibitors, such as infliximab, may cause optic neuritis. Therefore, history-taking is important for differential diagnosis and appropriate treatment.


2020 ◽  
Author(s):  
Jian Kang ◽  
Shu-Qing Liu ◽  
Yi-Fan Song ◽  
Meng-Ru Wang ◽  
Yao-Juan Chu ◽  
...  

Abstract Background: Inflammatory demyelination and axonal injury of the optic nerve are hallmarks of optic neuritis (ON), which often occurs in multiple sclerosis and is a major cause of visual disturbance in young adults. Although a high dose of corticosteroids can promote visual recovery, it cannot prevent permanent neuronal damage. Novel and effective therapies are thus required. Given the recently defined capacity of matrine (MAT), a quinolizidine alkaloid derived from the herb Radix Sophorae flavescens, in immunomodulation and neuroprotection, we tested in this study the effect of matrine on rats with experimental autoimmune encephalomyelitis, an animal model of multiple sclerosis. Results: MAT administration, started at disease onset, significantly suppressed optic nerve infiltration and demyelination, with reduced numbers of Iba1 + macrophages/microglia and CD4 + T cells, compared to those from vehicle-treated rats. Increased expression of neurofilaments, an axon marker, reduced numbers of apoptosis in retinal ganglion cells (RGCs), and reduced numbers of Iba1 + macrophages/microglia and CD4 + T cells were also observed in the retina after MAT treatment. Conclusions: Taken as a whole, our results demonstrate that MAT attenuated inflammation, demyelination and axonal loss in the optic nerve, and protected RGCs from inflammation-induced cell death. MAT may therefore have potential as a novel treatment for this disease that may result in blindness.


2019 ◽  
Author(s):  
Jian Kang ◽  
Shu-Qing Liu ◽  
Yi-Fan Song ◽  
Meng-Ru Wang ◽  
Yao-Juan Chu ◽  
...  

Abstract Background: Inflammatory demyelination and axonal injury of the optic nerve are hallmarks of optic neuritis (ON), which often occurs in multiple sclerosis and is a major cause of blindness in young adults. Although a high dose of corticosteroids can promote visual recovery, it cannot prevent permanent neuronal damage. Novel and effective therapies are thus required. Given the recently defined capacity of matrine (MAT), a quinolizidine alkaloid derived from the herb Radix Sophorae flavescens, in immunomodulation and neuroprotection, we tested in this study the effect of matrine on ON in rats with experimental autoimmune encephalomyelitis, an animal model of multiple sclerosis. Results: MAT administration, started at disease onset, significantly suppressed optic nerve infiltration and demyelination, with reduced numbers of Iba1+ macrophages/microglia and CD4+ T cells, compared to those from vehicle-treated rats. Increased expression of neurofilaments, an axon marker, and decreased apoptosis in retinal ganglion cells (RGCs) were also observed after MAT treatment. Conclusions: Taken as a whole, our results demonstrate that MAT attenuated inflammation, demyelination and axonal loss in the optic nerve, and protected RGCs from inflammation-induced cell death. MAT may therefore have potential as a novel treatment for this disease that causes blindness.


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