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[PMID]: 29222573
[Au] Autor:Stunkel L; Kung NH; Wilson B; McClelland CM; Van Stavern GP
[Ad] Address:Department of Neurology, Washington University School of Medicine, St Louis, Missouri.
[Ti] Title:Incidence and Causes of Overdiagnosis of Optic Neuritis.
[So] Source:JAMA Ophthalmol;136(1):76-81, 2018 Jan 01.
[Is] ISSN:2168-6173
[Cp] Country of publication:United States
[La] Language:eng
[Ab] Abstract:Importance: Diagnostic error is an important source of medical error. Overdiagnosis of optic neuritis may prompt unnecessary and costly diagnostic tests, procedures, and treatments. Objective: To assess the incidence of and characterize factors contributing to overdiagnosis of acute optic neuritis. Design, Setting, and Participants: In this retrospective clinic-based cross-sectional study of new patient encounters, 122 patients referred for acute optic neuritis at a university-based Midwestern neuro-ophthalmology clinic between January 2014 and October 2016 were studied. Data were analyzed from September 2016 to July 2017. Interventions: Definite diagnosis was determined by neuro-ophthalmologists. For patients with alterative diagnoses, the Diagnosis Error Evaluation and Research taxonomy tool was applied to categorize the type of diagnostic error. Main Outcomes and Measures: The primary outcome was the primary type of diagnostic error in patients erroneously diagnosed as having optic neuritis. Secondary outcomes included final diagnosis and interventions undergone prior to referral. Results: A total of 122 patients were referred with acute optic neuritis during the study period; 88 (72.1%) were female, and the mean (SD) age was 42.6 (14.0) years. Of these, 49 patients (40.2%; 95% CI, 31.4-49.4) were confirmed to have optic neuritis, and 73 (59.8%; 95% CI, 50.6-68.6) had an alternative diagnosis. The most common alternative diagnoses were headache and eye pain, functional visual loss, and other optic neuropathies, particularly nonarteritic anterior ischemic optic neuropathy. The most common diagnostic error was eliciting or interpreting critical elements of history, which occurred in 24 of 73 patients (33%) with alternative diagnoses. Other common errors included errors weighing or considering alternative diagnoses (23 patients [32%]), errors weighing or interpreting physical examination findings (15 patients [21%]), and misinterpreting diagnostic test results (11 patients [15%]). In patients with alterative diagnoses, 12 (16%) had normal magnetic resonance imaging findings preceding the referral, 12 (16%) had received a lumbar puncture, and 8 (11%) had received unnecessary treatment with intravenous steroids. Conclusions and Relevance: These data suggest that nearly 60% (95% CI, 50.6-68.6) of patients referred for optic neuritis have an alternative diagnosis, with the most common errors being overreliance on a single item of history and failure to consider alternative diagnoses. Understanding pitfalls leading to overdiagnosis of optic neuritis may improve clinicians' diagnostic process.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1712
[Cu] Class update date: 180311
[Lr] Last revision date:180311
[St] Status:In-Data-Review
[do] DOI:10.1001/jamaophthalmol.2017.5470

  2 / 6915 MEDLINE  
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[PMID]: 29519255
[Au] Autor:Rissewijck J; Siezenga MA
[Ad] Address:Ziekenhuis Gelderse Vallei, afd. Interne Geneeskunde, Ede.
[Ti] Title:Oogzenuwontsteking door lues. [Optic nerve infection caused by syphilis].
[So] Source:Ned Tijdschr Geneeskd;162(0):D1735, 2018.
[Is] ISSN:1876-8784
[Cp] Country of publication:Netherlands
[La] Language:dut
[Ab] Abstract:BACKGROUND: Syphilis, 'the great imitator', can present with a variety of symptoms. CASE DESCRIPTION: A 54-year-old woman attended the hospital clinic for vision problems, preceded by mouth ulcers. Following extensive serological investigations, the diagnosis 'syphilitic optic neuritis' was made. CONCLUSION: It is important to be thoughtful of systemic causes, like syphilis, when patients present with local symptomatology.
[Pt] Publication type:ENGLISH ABSTRACT; JOURNAL ARTICLE
[Em] Entry month:1803
[Cu] Class update date: 180309
[Lr] Last revision date:180309
[St] Status:In-Data-Review

  3 / 6915 MEDLINE  
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[PMID]: 29517994
[Au] Autor:Dal Monte M; Cammalleri M; Locri F; Amato R; Marsili S; Rusciano D; Bagnoli P
[Ad] Address:Department of Biology, University of Pisa, via San Zeno 31, 56127 Pisa, Italy. massimo.dalmonte@unipi.it.
[Ti] Title:Fatty Acids Dietary Supplements Exert Anti-Inflammatory Action and Limit Ganglion Cell Degeneration in the Retina of the EAE Mouse Model of Multiple Sclerosis.
[So] Source:Nutrients;10(3), 2018 Mar 08.
[Is] ISSN:2072-6643
[Cp] Country of publication:Switzerland
[La] Language:eng
[Ab] Abstract:Optic neuritis is an acute inflammatory demyelinating disorder of the optic nerve (ON) and is an initial symptom of multiple sclerosis (MS). Optic neuritis is characterized by ON degeneration and retinal ganglion cell (RGC) loss that contributes to permanent visual disability and lacks a reliable treatment. Here, we used the experimental autoimmune encephalomyelitis (EAE) mouse model of MS, a well-established model also for optic neuritis. In this model, C57BL6 mice, intraperitoneally injected with a fragment of the myelin oligodendrocyte glycoprotein (MOG), were found to develop inflammation, Müller cell gliosis, and infiltration of macrophages with increased production of oncomodulin (OCM), a calcium binding protein that acts as an atypical trophic factor for neurons enabling RGC axon regeneration. Immunolabeling of retinal whole mounts with a Brn3a antibody demonstrated drastic RGC loss. Dietary supplementation with Neuro-FAG (nFAG ), a balanced mixture of fatty acids (FAs), counteracted inflammatory and gliotic processes in the retina. In contrast, infiltration of macrophages and their production of OCM remained at elevated levels thus eventually preserving OCM trophic activity. In addition, the diet supplement with nFAG exerted a neuroprotective effect preventing MOG-induced RGC death. In conclusion, these data suggest that the balanced mixture of FAs may represent a useful form of diet supplementation to limit inflammatory events and death of RGCs associated to optic neuritis. This would occur without affecting macrophage infiltration and the release of OCM thus favoring the maintenance of OCM neuroprotective role.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1803
[Cu] Class update date: 180308
[Lr] Last revision date:180308
[St] Status:In-Process

  4 / 6915 MEDLINE  
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[PMID]: 29494741
[Au] Autor:McDougald DS; Dine KE; Zezulin AU; Bennett J; Shindler KS
[Ad] Address:Center for Advanced Retinal and Ocular Therapeutics, F. M. Kirby Center for Molecular Ophthalmology, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania, United States.
[Ti] Title:SIRT1 and NRF2 Gene Transfer Mediate Distinct Neuroprotective Effects Upon Retinal Ganglion Cell Survival and Function in Experimental Optic Neuritis.
[So] Source:Invest Ophthalmol Vis Sci;59(3):1212-1220, 2018 Mar 01.
[Is] ISSN:1552-5783
[Cp] Country of publication:United States
[La] Language:eng
[Ab] Abstract:Purpose: Optic neuritis is a condition defined by autoimmune-mediated demyelination of the optic nerve and death of retinal ganglion cells. SIRT1 and NRF2 stimulate anti-inflammatory mechanisms and have previously demonstrated therapeutic value in preclinical models of neurodegenerative disease. Here we investigated the neuroprotective potential of SIRT1 or NRF2 gene transfer using adeno-associated virus (AAV) vectors in the experimental autoimmune encephalomyelitis (EAE) model of multiple sclerosis. Methods: C57Bl/6J mice were administered intravitreal doses of AAV2 vectors and immunized to induce EAE symptoms. Visual function was examined by recording the optokinetic response (OKR) just prior to EAE induction and once every 7 days postinduction for 7 weeks. Retina and optic nerves were harvested to investigate retinal ganglion cell survival (immunolabeling with Brn3a antibodies); inflammation (hematoxylin and eosin staining); and demyelination (luxol fast blue staining). Results: Animals modeling EAE demonstrate reduced visual acuity compared to sham-induced controls. Intravitreal delivery of AAV2-NRF2 did not preserve visual function. However, AAV2-SIRT1 mediated significant preservation of the OKR compared to AAV2-eGFP controls. Treatment with AAV2-NRF2 promoted RGC survival while AAV2-SIRT1 mediated an upward trend in protection compared to vehicle and AAV2-eGFP controls. Neither NRF2 nor SIRT1 gene augmentation was able to suppress optic nerve inflammation or demyelination. Conclusions: AAV-mediated overexpression of NRF2 or SIRT1 within RGCs mediates distinct neuroprotective effects upon visual function and RGC survival. This study expands our understanding of SIRT1 and NRF2-mediated neuroprotection in the context of MS pathogenesis and optic neuropathies.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1803
[Cu] Class update date: 180309
[Lr] Last revision date:180309
[St] Status:In-Data-Review
[do] DOI:10.1167/iovs.17-22972

  5 / 6915 MEDLINE  
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[PMID]: 29443442
[Au] Autor:Wong YYM; Hacohen Y; Armangue T; Wassmer E; Verhelst H; Hemingway C; van Pelt ED; Catsman-Berrevoets CE; Hintzen RQ; Deiva K; Lim MJ; Rostásy K; Neuteboom RF
[Ad] Address:Department of Neurology, Erasmus MC, Rotterdam, The Netherlands.
[Ti] Title:Paediatric acute disseminated encephalomyelitis followed by optic neuritis: disease course, treatment response and outcome.
[So] Source:Eur J Neurol;, 2018 Feb 14.
[Is] ISSN:1468-1331
[Cp] Country of publication:England
[La] Language:eng
[Ab] Abstract:BACKGROUND AND PURPOSE: Acute disseminated encephalomyelitis followed by optic neuritis (ADEM-ON) is a rare demyelinating syndrome that is different from multiple sclerosis and neuromyelitis optica spectrum disorder. The aim of this study was to describe the disease course, treatment response and outcome of children with ADEM-ON. METHODS: Children of <18 years of age were identified from six countries of the EU Paediatric Demyelinating Disease Consortium. Patients fulfilled the diagnostic criteria for ADEM followed by at least one ON. Anti-myelin oligodendrocyte glycoprotein (MOG) antibodies were tested in all patients. RESULTS: In this study of 17 patients (nine boys) with ADEM-ON, anti-myelin oligodendrocyte glycoprotein (MOG) antibodies were identified in 16 patients. Age at onset was 6.1 years (interquartile range, 5.1-9.2 years). Twelve patients received oral prednisolone and 10 received maintenance immunosuppression (e.g. azathioprine, intravenous immunoglobulins, Rituximab). During a follow-up of 5.3 years (interquartile range, 1.8-10.2 years), 54 relapses occurred with a median of 3 relapses per patient (range, 1-9 per patient). Patients relapsed on all treatments but no relapses occurred on a prednisolone dose >10 mg/day. Visual and cognitive residual deficits were common in this group. CONCLUSIONS: Acute disseminated encephalomyelitis followed by optic neuritis is an anti-MOG antibody-associated relapsing disorder that can have a heterogeneous disease course. Patients were refractory for maintenance immunosuppression and appeared to be corticosteroid-dependent. Further international collaborations are now required to unify guidelines in this difficult-to-manage group of patients.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1802
[Cu] Class update date: 180309
[Lr] Last revision date:180309
[St] Status:Publisher
[do] DOI:10.1111/ene.13602

  6 / 6915 MEDLINE  
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[PMID]: 29517664
[Au] Autor:Chi LM; Gao Y; Nan GX
[Ad] Address:China-Japan Union Hospital of Jilin University, Changchun City, Jilin Province, China.
[Ti] Title:A case report of neuromyelitis optica spectrum disorder with peripheral neuropathy as the first episode.
[So] Source:Medicine (Baltimore);97(10):e0059, 2018 Mar.
[Is] ISSN:1536-5964
[Cp] Country of publication:United States
[La] Language:eng
[Ab] Abstract:RATIONALE: Neuromyelitis optica spectrum disorders (NMOSDs) represent recurrent autoimmune diseases, generally beginning with optic nerve neuritis or acute transverse myelitis. PATIENT CONCERNS: A 57-year-old male with long-term alcohol intake was hospitalized because of limb numbness. EMG examination showed the peripheral sensory nerve was in demyelination and an axonal injury was found. His symptoms could not be improved by vitamin B injection but were later significantly attenuated by dexamethasone treatment. Four months later, symptoms of optic neuritis in the left eye appeared, and 6 months later he exhibited peripheral neuropathy with acute myelitis. DIAGNOSES: He was diagnosed NMOSD. OUTCOMES: Immunotherapy improved his peripheral neuropathy and myelitis symptoms. LESSONS: NMOSD patients could represent peripheral neuropathy as the first episode.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1803
[Cu] Class update date: 180308
[Lr] Last revision date:180308
[St] Status:In-Process
[do] DOI:10.1097/MD.0000000000010059

  7 / 6915 MEDLINE  
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[PMID]: 29514678
[Au] Autor:Dietrich M; Helling N; Hilla A; Heskamp A; Issberner A; Hildebrandt T; Kohne Z; Küry P; Berndt C; Aktas O; Fischer D; Hartung HP; Albrecht P
[Ad] Address:Department of Neurology, Medical Faculty, Heinrich-Heine University Düsseldorf, Moorenstr. 5, 40225, Düsseldorf, Germany.
[Ti] Title:Early alpha-lipoic acid therapy protects from degeneration of the inner retinal layers and vision loss in an experimental autoimmune encephalomyelitis-optic neuritis model.
[So] Source:J Neuroinflammation;15(1):71, 2018 Mar 07.
[Is] ISSN:1742-2094
[Cp] Country of publication:England
[La] Language:eng
[Ab] Abstract:BACKGROUND: In multiple sclerosis (MS), neurodegeneration is the main reason for chronic disability. Alpha-lipoic acid (LA) is a naturally occurring antioxidant which has recently been demonstrated to reduce the rate of brain atrophy in progressive MS. However, it remains uncertain if it is also beneficial in the early, more inflammatory-driven phases. As clinical studies are costly and time consuming, optic neuritis (ON) is often used for investigating neuroprotective or regenerative therapeutics. We aimed to investigate the prospect for success of a clinical ON trial using an experimental autoimmune encephalomyelitis-optic neuritis (EAE-ON) model with visual system readouts adaptable to a clinical ON trial. METHODS: Using an in vitro cell culture model for endogenous oxidative stress, we compared the neuroprotective capacity of racemic LA with the R/S-enantiomers and its reduced form. In vivo, we analyzed retinal neurodegeneration using optical coherence tomography (OCT) and the visual function by optokinetic response (OKR) in MOG -induced EAE-ON in C57BL/6J mice. Ganglion cell counts, inflammation, and demyelination were assessed by immunohistological staining of retinae and optic nerves. RESULTS: All forms of LA provided equal neuroprotective capacities in vitro. In EAE-ON, prophylactic LA therapy attenuated the clinical EAE score and prevented the thinning of the inner retinal layer while therapeutic treatment was not protective on visual outcomes. CONCLUSIONS: A prophylactic LA treatment is necessary to protect from visual loss and retinal thinning in EAE-ON, suggesting that a clinical ON trial starting therapy after the onset of symptoms may not be successful.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1803
[Cu] Class update date: 180308
[Lr] Last revision date:180308
[St] Status:In-Data-Review
[do] DOI:10.1186/s12974-018-1111-y

  8 / 6915 MEDLINE  
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[PMID]: 29445944
[Au] Autor:Kezuka T; Ishikawa H
[Ad] Address:KEZUKA Eye Clinic, 1-5-7 Mukojima, Sumida-ku, Tokyo, 131-0033, Japan. tkezuka1000@gmail.com.
[Ti] Title:Diagnosis and treatment of anti-myelin oligodendrocyte glycoprotein antibody positive optic neuritis.
[So] Source:Jpn J Ophthalmol;62(2):101-108, 2018 Mar.
[Is] ISSN:1613-2246
[Cp] Country of publication:Japan
[La] Language:eng
[Ab] Abstract:Anti-myelin-oligodendrocyte glycoprotein (MOG) antibody positive optic neuritis has been established as a new subset of optic neuropathy. Anti-MOG antibodies are usually measured by cell-based assay. Patients with anti-MOG antibody positive optic neuritis respond well to steroid therapy, and, while visual acuity outcomes are favorable, significant visual field defects remain. Furthermore, patients who are anti-MOG antibody positive have higher rates of recurrence compared to antibody negative patients. Based on these findings, anti-MOG antibody positive patients with optic neuritis have the characteristics of good visual outcomes, residual visual field defects, and high risk of recurrence. Tests for anti-MOG antibody are useful for the diagnosis and treatment of optic neuritis.
[Pt] Publication type:JOURNAL ARTICLE; REVIEW
[Em] Entry month:1802
[Cu] Class update date: 180308
[Lr] Last revision date:180308
[St] Status:In-Process
[do] DOI:10.1007/s10384-018-0561-1

  9 / 6915 MEDLINE  
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[PMID]: 29478728
[Au] Autor:McGee JC; Minagar A
[Ad] Address:Department of Neurology, LSU Health Sciences Center, Shreveport 71130, LA, USA.
[Ti] Title:Neuromyelitis optica spectrum disorder in the very young: An in depth review of the present data.
[So] Source:J Neurol Sci;, 2018 Feb 17.
[Is] ISSN:1878-5883
[Cp] Country of publication:Netherlands
[La] Language:eng
[Pt] Publication type:EDITORIAL
[Em] Entry month:1802
[Cu] Class update date: 180307
[Lr] Last revision date:180307
[St] Status:Publisher

  10 / 6915 MEDLINE  
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[PMID]: 29507942
[Au] Autor:Morrow SA; Fraser JA; Day C; Bowman D; Rosehart H; Kremenchutzky M; Nicolle M
[Ad] Address:Department of Clinical Neurological Sciences, Western University, London, Ontario, Canada.
[Ti] Title:Effect of Treating Acute Optic Neuritis With Bioequivalent Oral vs Intravenous Corticosteroids: A Randomized Clinical Trial.
[So] Source:JAMA Neurol;, 2018 Mar 05.
[Is] ISSN:2168-6157
[Cp] Country of publication:United States
[La] Language:eng
[Ab] Abstract:Importance: Intravenous (IV) administration of corticosteroids is the standard of care in the treatment of acute optic neuritis. However, it is uncertain whether a bioequivalent dose of corticosteroid administered orally, which may be more cost-efficient and convenient for patients, is as effective as IV administration in the treatment of acute optic neuritis. Objective: To determine whether recovery of vision following treatment of acute optic neuritis with a high-dose IV corticosteroid is superior to that with a bioequivalent dose of an oral corticosteroid. Design, Setting, and Participants: This single-blind (participants unblinded) randomized clinical trial with 6-month follow-up was conducted at a single tertiary care center in London, Ontario, Canada. Participants were enrolled from March 2012 to May 2015, with the last participant's final visit occurring November 2015. Patients 18 to 64 years of age presenting within 14 days of acute optic neuritis onset, without any recovery at time of randomization and without history of optic neuritis in the same eye, were screened. Inclusion criteria included best-corrected visual acuity (BCVA) of 20/40 or worse and corticosteroids deemed required by treating physician. In total, 89 participants were screened; 64 were eligible, but 9 declined to participate. Thus, 55 participants were enrolled and randomized. Primary analysis was unadjusted and according to the intention-to-treat principle. Interventions: Participants were randomized 1:1 to the IV methylprednisolone sodium succinate (1000-mg) or oral prednisone (1250-mg) group. Main Outcomes and Measures: Primary outcome was recovery of the latency of the P100 component of the visual evoked potential at 6 months. Secondary outcomes were the P100 latency at 1 month and BCVA as assessed with Early Treatment Diabetic Retinopathy Study letter scores on the alphabet chart and scores on low-contrast letters at 1 and 6 months. Results: Of 55 randomized participants, the final analyzed cohort comprised 23 participants in the IV and 22 in the oral treatment groups. The mean (SD) age of the cohort was 34.6 (9.5) years, and there were 28 women (62.2%). At 6 months' recovery, P100 latency in the IV group improved by 62.9 milliseconds (from a mean [SD] of 181.9 [53.6] to 119.0 [16.5] milliseconds), and the oral group improved by 66.7 milliseconds (from a mean [SD] of 200.5 [67.2] to 133.8 [31.5] milliseconds), with no significant difference between groups (P = .07). Similarly, no significant group difference was found in the mean P100 latency recovery at 1 month. For BCVA, recovery between the groups did not reach statistical significance at 1 month or 6 months. In addition, improvements in low-contrast (1.25% and 2.5%) BCVA were not significantly different between treatment groups at 1 or 6 months' recovery. Conclusions and Relevance: This study finds that bioequivalent doses of oral corticosteroids may be used as an alternative to IV corticosteroids to treat acute optic neuritis. Trial Registration: clinicaltrials.gov Identifier: NCT01524250.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1803
[Cu] Class update date: 180306
[Lr] Last revision date:180306
[Cl] Clinical Trial:ClinicalTrial
[St] Status:Publisher
[do] DOI:10.1001/jamaneurol.2018.0024


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