scholarly journals Bilateral Retrobulbar Optic Neuritis as the First Manifestation of Neuro-Behçet Disease

2020 ◽  
Vol 2020 ◽  
pp. 1-3
Author(s):  
Mohsen Jari ◽  
Taiiebeh Mohammadi ◽  
Ensiyeh Taheri
Keyword(s):  
Optic Neuritis ◽  
Brain Magnetic Resonance Imaging ◽  
Human Leukocyte ◽  
Oligoclonal Bands ◽  
Behçet Disease ◽  
Normal Brain ◽  
Behcet Disease ◽  
Fluid Analysis ◽  
Acute Optic Neuritis ◽  
Retrobulbar Optic Neuritis

Background. Behçet disease (BD) is a polygenic and chronic autoinflammatory multisystem vasculitis. Acute optic neuritis has been rarely reported in patients with BD, especially in children. Case Presentation. We reported an 8-year-old girl with a sudden visual loss and color vision impairment. The patient had a history of recurrent oral aphthous ulcers, genital ulcers, and chronic abdominal pain. On ophthalmic examination, anterior and posterior chambers and funduscopy of both eyes were normal. The results of laboratory tests for infectious and rheumatic diseases were normal. Brain magnetic resonance imaging and the result of cerebrospinal fluid analysis for oligoclonal bands and auto-antibodies were also normal. Pathergy skin test and human leukocyte antigens (HLA) B5 and HLA-B51 were positive. The patient was recognized as a case of BD-related bilateral retrobulbar optic neuritis and was treated by corticosteroids, azathioprine, colchicine, and infliximab. Conclusion. Retrobulbar optic neuritis may be the first manifestation of neuro-BD.

scholarly journals An 8- year-old Behcet Disease girl initially presenting with bilateral retrobulbar optic neuritis

2020 ◽  
Author(s):  
Mohsen Jari ◽  
Taiebeh Mohammadi ◽  
Ensieh Taheri
Keyword(s):  
Optic Neuritis ◽  
Brain Magnetic Resonance Imaging ◽  
Human Leukocyte ◽  
Oligoclonal Bands ◽  
Behçet Disease ◽  
Normal Brain ◽  
Behcet Disease ◽  
Fluid Analysis ◽  
Acute Optic Neuritis ◽  
Retrobulbar Optic Neuritis

Abstract Background: Behçet disease(BD) is a polygenic and chronic autoinflamatory multisystem vasculitis. Acute optic neuritis has been rarely reported in patients with BD, especially in children.Case presentation :We reported an 8-year-old girl with suddenly visual loss and color vision impairment.The patient has a history of recurrent oral aphthous ulcers, genital ulcers and chronic abdominal pain.On ophthalmic examination, anterior and posterior chambers and funduscopy of both eyes were normal.The results of laboratory tests for infectious and rheumatic diseases were normal. Brain magnetic resonance imaging and the result of cerebrospinal fluid analysis for oligoclonal bands autoantibodies were normal too. Pathergy skin test and human leukocyte antigen ( HLA) B5 and HLA-B51 were positive.The patient was recognized as a case of BD-related bilateral retrobulbar optic neuritis and was treated by corticosteroid , azathioprine, colchicine and infliximab.Conclusion: Retrobulbar optic neuritis may be first manifestation of BD.

Progressive Leukodystrophy-Like Demyelinating Syndromes with MOG-Antibodies in Children: A Rare Under-Recognized Phenotype

2021 ◽  
Author(s):  
Elise Yazbeck ◽  
Hélène Maurey ◽  
Carole Leroy ◽  
Philippe Horellou ◽  
Silvia Napuri ◽  
...  
Keyword(s):  
Brain Magnetic Resonance Imaging ◽  
Brain Biopsy ◽  
Active Immunotherapy ◽  
Oligoclonal Bands ◽  
Second Child ◽  
Fluid Analysis ◽  
Appropriate Treatment ◽  
Neurological Prognosis ◽  
Mog Antibodies

AbstractAcquired demyelinating syndromes (ADS) are frequently associated with myelin oligodendrocytes glycoprotein (MOG) antibodies in children. Clinical phenotypes are heterogeneous and may delay the diagnosis, especially when they relapse and are atypical, mimicking diseases such as multiple sclerosis or neuromyelitis optica spectrum disorders . Here, we describe two children: one with a progressive cognitive and behavioral deterioration with seizures after only one relapse and the other with similar clinical impairments associated with multiple relapses. Brain magnetic resonance imaging revealed a subsequent progressive leukodystrophy-like lesion with diffuse bilateral white matter injuries in both patients. Cerebrospinal fluid analysis showed pleiocytosis, increased level of proteins with no oligoclonal bands. Metabolic and inflammatory blood markers were all negative. Brain biopsy was performed in the second child and nonspecific inflammatory lesions with no argument for histiocytosis or tumor were observed. Clinical and radiological stabilization were obtained after active immunotherapy. Retrospective analysis of anti-MOG antibodies in these two children was positive at the earlier stage of the disease and turned negative after treatment and during follow-up. Leukodystrophy-like ADS with anti-MOG-antibodies may display distinct progressive phenotype and have a severe neurological prognosis. Early diagnosis and appropriate treatment may improve outcome in these children.

Case Reports. A case of Adamantiades-Behçet disease with ischemic optic neuritis (posterior optic neuropathy)

2007 ◽  
Vol 5 (11) ◽  
pp. 1010-1014 ◽  
Author(s):  
Satoko Shima ◽  
Keisuke Nishimura ◽  
Kei-ichi Yamanaka ◽  
Arata Hakamada ◽  
Ken-ichi Isoda ◽  
...  
Keyword(s):  
Optic Neuritis ◽  
Optic Neuropathy ◽  
Case Reports ◽  
Behçet Disease ◽  
Behcet Disease

scholarly journals Association Between Novel Coronavirus Disease 2019 (COVID-19) and Idiopathic Intracranial Hypertension

2021 ◽  
Vol In Press (In Press) ◽  
Author(s):  
Ahmad Talebian ◽  
Babak Soltani ◽  
Dawood Aghadoost ◽  
Javid Azadbakht ◽  
Alireza Rezaee ◽  
...  
Keyword(s):  
Intracranial Hypertension ◽  
Idiopathic Intracranial Hypertension ◽  
Neurological Complication ◽  
Brain Magnetic Resonance Imaging ◽  
Pseudotumor Cerebri ◽  
Normal Brain ◽  
Fluid Analysis ◽  
History Of ◽  
Magnetic Resonance Imaging Mri ◽  
Novel Coronavirus

Introduction: Idiopathic intracranial hypertension (IIH), also known as pseudotumor cerebri, is a rare neurological manifestation of coronavirus disease 2019 (COVID-19) infection. Case Presentation: In this case study, we present a 10-year-old girl with headache, diplopia, bilateral sixth nerve palsy, and papilledema following a history of seven days of fever about two weeks ago. In lumbar puncture, the opening pressure was 56 cmH2O. Cerebrospinal fluid analysis was normal. Brain magnetic resonance imaging (MRI) was suggestive of IIH. Nasopharyngeal reverse transcription-polymerase chain reaction (RT-PCR) was positive for COVID-19. Oral acetazolamide was prescribed, and the patient improved completely after three weeks of continuous medical treatment. Conclusions: Since IIH is a rare neurological complication of COVID-19, we have to suspect COVID-19 infection in any patient with IIH.

scholarly journals IL-6 increase in CSF as additional diagnostic criteria for definite neuro-behcet disease

2019 ◽  
Author(s):  
Mariem Kchaou ◽  
Khadija Bahrini ◽  
Meriam Belghith ◽  
Aroua Cherif ◽  
Olfa Maghrebi ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Cerebrospinal Fluid ◽  
Nervous System ◽  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Oligoclonal Bands ◽  
Behçet Disease ◽  
Behcet Disease ◽  
Behcet's Disease

Abstract Background: When the central nervous system (CNS) is the primary affected site in an initial attack of Behcet’s disease (BD), the differential diagnosis is particularly challenging. Some cases remain unclassified or qualified as probable Neuro-Behçet disease (NBD). Moreover, it was demonstrated that cytokines play a crucial role in the pathogenesis of NBD. We therefore studied peripheral and cerebrospinal inflammatory profile of these patients. Methods: Twenty two parenchymal NBD patients diagnosed according to the international consensus recommendation criteria and classified into definite (d-NBD; n= 13) and probable (p-NBD ; n=9) were sampled at their first neurological symptoms and compared with healthy control subjects (n=10). Oligoclonal bands of IgG were detected by isoelectric focusing on agarose and immunoblotting of matched serum and Cerebrospinal fluid (CSF) sample pairs. Cytokines and transcription factors related to TH1, TH2, TH17 and T regulatory populations were studied by quantitative RT-PCR in the CSF. Results: Oligoclonal bands (OCB) were present in only 1/22 patients. Two d-NBD patients had OCB in the CSF showing pattern 4. In NBD CSF samples, INF gamma, IL-17 and IL-10 expressions were significantly elevated compared with controls, however no difference in those cytokine expressions was observed between d-NBD compared to p-NBD. The most stricking finding was the significant increase of CSF IL-6 in d-NBD compared to p-NBD. Conclusion:These results indicate the rare presence of OCB in parenchymal NBD patients. Additionally, CSF IL-6 could help us to identify definite NBD. Keywords: Behçet’s disease, central nervous system, cerebrospinal fluid, cytokine.

scholarly journals Risk of Multiple Sclerosis after Idiopathic Optic Neuritis in a Pakistani Population

2010 ◽  
Vol 37 (2) ◽  
pp. 258-263 ◽  
Author(s):  
Syed Faraz Kazim ◽  
Muhammad Islam ◽  
Mustafa Khan ◽  
Bilal Hameed ◽  
Saad Shafqat
Keyword(s):  
Multiple Sclerosis ◽  
Optic Neuritis ◽  
Brain Mri ◽  
Brain Magnetic Resonance Imaging ◽  
Oligoclonal Bands ◽  
Cumulative Probability ◽  
South Asian Population ◽  
Increased Risk ◽  
Risk Of Progression ◽  
Csf Oligoclonal Bands

Background and objective:Optic neuritis (ON) is associated with a 38% ten-year risk of developing multiple sclerosis (MS) in Western populations, but the corresponding risk in non-Western populations is unclear. We conducted this study to estimate the risk of progression to MS after an episode of ON in a South Asian population.Methods:Two hundred and fifty-three patients with idiopathic ON were identified by reviewing records of visual evoked potentials and chart notes from a single academic center spanning the years 1990-2007. A structured telephone interview was then conducted to identify patients who had subsequently received a diagnosis of MS. The diagnosis was corroborated from chart notes, where possible. Cumulative probability of conversion to MS was calculated using Kaplan-Meier survival analysis.Results:The five-year risk of developing MS was 14.6% and the ten-year risk was 24%. Patients (N=218) who had one or more typical demyelinating lesions on baseline brain magnetic resonance imaging (MRI) had a 68% 10-year risk; those with no lesions or non-typical lesions had a 14% risk (p<0.001). Female gender, recurrent ON, and occurrence of ON in winter months were also associated with increased risk (p≤ 0.001). Severity of ON and likelihood of detecting cerebrospinal fluid (CSF) oligoclonal bands were higher in patients who developed MS.Conclusion:Idiopathic ON in Pakistan carries a lower risk of progression to MS compared with Western data. As in Western populations, however, presence of abnormal baseline brain MRI and CSF oligoclonal bands correlate with increased MS risk.

scholarly journals Very Rare Case Report of Multiple Sclerosis in a Case of Chronic Kidney Disease

2020 ◽  
Vol 2 (5) ◽  
pp. 1-4
Author(s):  
Vajed Mogal ◽  
◽  
Mahendra Wawhal ◽  
Keyword(s):  
Multiple Sclerosis ◽  
Chronic Kidney Disease ◽  
Case Report ◽  
Kidney Disease ◽  
Optic Neuritis ◽  
World Literature ◽  
Oligoclonal Bands ◽  
Unknown Etiology ◽  
First Case ◽  
Acute Optic Neuritis

Multiple sclerosis is a neurological disease, which affects young adults. Its course is unpredictable and runs over decades. It is considered as disease of unknown etiology in which there is demyelination of the brain and spinal cord. There is one case report of membranous GN in a case of progressive multiple sclerosis in the extant world literature. We report here perhaps the first case in the world of the occurrence of multiple sclerosis in a known case of chronic kidney disease with the presentation of acute optic neuritis, having a characteristics picture on MRI of multiple sclerosis in brain, abnormal VEP, oligoclonal bands in CSF and a prior episode of acute optic neuritis 3 years back. In our opinion, this case opens the flood gates for research & work to investigate whether the oxidative stress and the smouldering proinflammatory state in CKD can precipitate multiple sclerosis in a genetically predisposed host in a given environment. Multiple Sclerosis is often associated with a neurogenic bladder, but renal function is generally considered to be normal.

Predicting multiple sclerosis at optic neuritis onset

2003 ◽  
Vol 9 (2) ◽  
pp. 135-141 ◽  
Author(s):  
Y P Jin ◽  
J de Pedro-Cuesta ◽  
Y H Huang ◽  
M Söderström
Keyword(s):  
Multiple Sclerosis ◽  
Optic Neuritis ◽  
Brain Mri ◽  
Brain Magnetic Resonance Imaging ◽  
Oligoclonal Bands ◽  
Individual Risk ◽  
Relative Hazard ◽  
Hazard Ratios ◽  
Individual Probability

Using multivariate analyses, individual risk of clinically definite multiple sclerosis (C DMS) after monosymptomatic optic neuritis (MO N) was quantified in a prospective study with clinical MO N onset during 1990 -95 in Stockholm, Sweden. During a mean follow-up time of 3.8 years, the presence of MS-like brain magnetic resonance imaging (MRI) lesions and oligoclonal immunoglobulin (Ig) G bands in cerebrospinal fluid (CSF) were strong prognostic markers of C DMS, with relative hazard ratios of 4.68 {95% confidence interval (CI) 2.21 -9.91} and 5.39 (95% C I 1.56 -18.61), respectively. Age and season of clinical onset were also significant predictors, with relative hazard ratios of 1.76 (95% C I 1.02 -3.04) and 2.21 (95% C I 1.13 -3.98), respectively. Based on the above two strong predicto rs, individual probability of C DMS development after MO N was calculated in a three-quarter sample drawn from a cohort, with completion of follow-up at three years. The highest probability, 0.66 (95% C I 0.48 -0.80), was obtained for individuals presenting with three or more brain MRI lesions and oligoclonal bands in the C SF, and the lowest, 0.09 (95% C I 0.02 -0.32), for those not presenting with these traits. Medium values, 0.29 (95% C I 0.13 -0.53) and 0.32 (95% C I 0.07 -0.73), were obtained for individuals discordant for the presence of brain MRI lesions and oligoclonal bands in the C SF. These predictions were validated in an external one-quarter sample.

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