Neuro-Behcet's Disease Presenting as Isolated Vestibular Syndrome.
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ABSTRACT:
SUBMITTER: Park E
PROVIDER: S-EPMC7354970 | biostudies-literature | 2020 Jul
REPOSITORIES: biostudies-literature
Publications
Neuro-Behçet's Disease Presenting as Isolated Vestibular Syndrome.
Park Euyhyun E Lee Sun Uk SU Kim Hyo Jung HJ Choi Jeong Yoon JY Im Gi Jung GJ Yu Sungwook S Kim Ji Soo JS
Journal of clinical neurology (Seoul, Korea) 20200701 3
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Project description:Objective Brain parenchymal involvement in Behçet's disease (BD) (neuro-Behçet's disease, NB) can be classified into acute type (ANB) and chronic progressive type (CPNB) based on differences in the clinical course and responses to corticosteroid treatment. The present study developed evidence-based recommendations for the management of NB.Methods The task force of the research subcommittee consisted of seven board-certified rheumatologists (one was also a board-certified neurologist) and three board-certified neurologists. First, several clinical questions (CQs) were established. A systematic literature search was performed by The Japan Medical Library Association in order to develop recommendations. The final recommendations for each CQ developed from three blind Delphi rounds, for which the rate of agreement scores [range 1 (strongly disagree)-5(strongly agree)] was determined through voting by the task force.Results A flow chart of the algorithm was established for the management of ANB and CPNB. Thirteen recommendations were developed for NB (general 1, ANB 7, CPNB 5). The strength of each recommendation was established based on the evidence level as well as the rate of agreement.Conclusion The recommendations generated in this study are based on the results of uncontrolled evidence from open trials, retrospective cohort studies and expert opinions, due to the lack of randomized clinical trials. Nevertheless, these recommendations can be used for international studies, although verification by further properly designed controlled clinical trials is required.
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Project description:Background and purposeNeurological involvement in Behçet's disease [neuro-Behçet's disease (NBD)] is uncommon, but it is worth investigating since it can cause substantial disability. However, difficulties exist in understanding the clinical features of NBD due to regional variations and the lack of studies utilizing well-established diagnostic criteria. We therefore analyzed the clinical features of patients with NBD based on the recent international consensus recommendation.MethodsWe retrospectively searched electronic databases for patients with Behçet's disease (BD) between 2000 and 2017, and reviewed their medical records. Based on the recent international consensus recommendation, patients with definite or probable NBD were included.ResultsOf 9,817 patients with the diagnosis code for BD, 1,682 (17.1%) visited the neurology clinic and 110 (1.1%) were classified as NBD. Ninety-eight patients exhibited parenchymal NBD and 12 exhibited nonparenchymal NBD. Their age at the onset of NBD was 37.6±10.6 years and the male-to-female ratio was 1.24:1. Brainstem syndrome (43.9%) was the most common condition in the 98 patients with parenchymal NBD, followed by multifocal (32.7%) and spinal cord (12.2%) syndromes. 72.4% exhibited acute NBD and 27.6% exhibited a progressive disease course. Frequent manifestations included pyramidal signs (52.0%), headache (45.9%), dysarthria (42.9%), and fever (31.6%). A frequent pattern in brain MRI was an upper brainstem lesion extending to the thalamus and basal ganglia.ConclusionsApproximately 1% of the patients with suspected BD exhibited NBD. Neurologists must understand the clinical characteristics of NBD in order to perform the differential diagnosis and management of these patients.
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Project description:Background Cases of SARS-COV-2 triggering or exacerbating autoimmune responses has been described in the literature, and it has shown that use of steroids in non-severe COVID-19 may potentially increase mortality. Case presentation A 22 year-old African-American man presented with headache, weight, loss, and oral/scrotal ulcerations. Case report Neurological exam revealed somnolence and right hemiplegia. MRI was remarkable multiple enhancing lesions involving the brainstem and left hemisphere. He was found to have a positive SARS-CoV-2 test. Work-up was unrevealing, and he was diagnosed with Neuro-Behcet's disease (NBD) based on the International Criteria for Behcet's Disease (ICBD)ackspaceD)BackspaceBackspacep. The patient was treated with systemic steroids, which resulted in both clinical and radiological improvement of his disease without exacerbation of his SAR-CoV-2 infection. Conclusions This case presentation suggests that IV steroids may be safe in the treatment of NBD in adult patients presenting with SARS-CoV-2 infection.
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Project description:Neuro-Behçet's disease (NBD) is a rare clinical entity that frequently presents neuro-psychiatric symptoms, and mesodiencephalic and pontobulbar lesions. There is only one published report about osteonecrosis in NBD. We report a patient whose first presentation was neurological NBD with presenting bilateral femoral heads osteonecrosis. A 36-year-old male was hospitalized with gait disorder, diplopia and speech disorder. The past medical history of the patient was unremarkable. The MR image showed mesencephalic lesion with oedemaas a hyperintense area. The present case was diagnosed as NBD and treated with methylprednisolone (1g /day) only for five days. One year after, bilateral hip pain developed. MR image of both hips showed well-demarcated areas of osteonecrosis in the bilateral femoral heads. The patient was operated by an orthopedic surgeon. Because early diagnosis and immediate treatment of osteonecrosis is very important, the physician must bear in mind that osteonecrosis might result from impaired microvascular involvement in even NBD.
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Project description:Background/Objectives: Neuro-Behçet's disease (NBD) is one of the most severe complications of Behçet's disease (BD). The incidence of NBD varies widely worldwide. This study aimed to estimate its current incidence in Northern Spain. Methods: This was a retrospective population-based cohort study of 120 patients in Northern Spain diagnosed with BD according to the 2013 International Criteria for BD (ICBD) between 1 January 1999 and 31 December 2019. NBD diagnoses were made according to International Consensus Recommendation (ICR) criteria. Overall, 96 patients were included, and their demographic and clinical data were collected. The incidence of NBD was estimated by age, gender, and year of diagnosis between 1999-2019. Results: NBD was diagnosed in 23 of 96 (24%) patients (15 women/8 men) (mean age: 44 ± 13.9 years). HLA-B51 was positive in 5 of 13 (38.4%) cases tested. A total of 10 (43.5%) patients had parenchymatous NBD, 10 (43.5%) had non-parenchymatous NBD, and 3 (13%) had mixed NBD. Incidence during the study period was 0.13 (95% CI, 0.11-0.26) per 100,000 people-years. There were no significant differences in gender in the incidence rate stratified by age (p > 0.05). Furthermore, there was a linear relationship with a mild decrease in age at diagnosis over time. Conclusions: Epidemiological characteristics of NBD in Northern Spain are similar to those of neighboring countries, except female gender predominance.
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Project description:BackgroundBehçet's syndrome (BS) is an immune-mediated disease characterized by recurrent oral ulcers, genital ulcers, uveitis, and skin symptoms. HLA-B51, as well as other genetic polymorphisms, has been reported to be associated with BS; however, the pathogenesis of BS and its relationship to genetic risk factors still remain unclear. To address these points, we performed immunophenotyping and transcriptome analysis of immune cells from BS patients and healthy donors.MethodsImmuNexUT is a comprehensive database consisting of RNA sequencing data and eQTL database of immune cell subsets from patients with immune-mediated diseases and healthy donors, and flow cytometry data and transcriptome data from 23 BS patients and 28 healthy donors from the ImmuNexUT study were utilized for this study. Differential gene expression analysis and weighted gene co-expression network analysis (WGCNA) were performed to identify genes associated with BS and clinical features of BS. eQTL database was used to assess the relationship between genetic risk factors of BS with those genes.ResultsThe frequency of Th17 cells was increased in BS patients, and transcriptome analysis of Th17 cells suggested the activation of the NFκB pathway in Th17 cells of BS patients. Next, WGCNA was used to group genes into modules with similar expression patterns in each subset. Modules of antigen-presenting cells were associated with BS, and pathway analysis suggested the activation of antigen-presenting cells of BS patients. Further examination of genes in BS-associated modules indicated that the expression of YBX3, a member of a plasmacytoid dendritic cell (pDC) gene module associated with BS, is influenced by a BS risk polymorphism, rs2617170, in pDCs, suggesting that YBX3 may be a key molecule connecting genetic risk factors of BS with disease pathogenesis. Furthermore, pathway analysis of modules associated with HLA-B51 indicated that the association of IL-17-associated pathways in memory CD8+ T cells with HLA-B51; therefore, IL-17-producing CD8+ T cells, Tc17 cells, may play a critical role in BS.ConclusionsVarious cells including CD4+ T cells, CD8+ T cells, and antigen-presenting cells are important in the pathogenesis of BS. Tc17 cells and YBX3 may be potential therapeutic targets in BS.
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Project description:Vestibular schwannoma (VS) is commonly accompanied by hearing loss, tinnitus, and dizziness and tends to be chronically progressive in nature. We report a case of VS presenting as left vestibular neuritis (VN) in a previously healthy 57-year-old patient. Right-beating horizontal-torsional spontaneous nystagmus was observed, and the bedside head impulse test revealed a left catch-up saccade. The bithermal caloric test showed left canal paresis, and pure-tone audiometry revealed an average threshold of 22.5 dB bilaterally. Brain magnetic resonance imaging (MRI) demonstrated a 0.7-cm enhancing mass in the left internal auditory canal, consistent with VS. The patient was administered with high-dose systemic corticosteroids and vestibular suppressants with antiemetic, which relieved acute vertigo. Although dizziness in VS is chronically progressive in nature, VS may present as an acute vestibular syndrome that mimics VN. VS should be considered a potential cause of acute vestibular syndrome, and thorough neurological examination with MRI may be helpful for accurate diagnosis.
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| S-EPMC8762499 | biostudies-literature
Project description:BackgroundBehçet's disease is a multisystem autoimmune disorder of unknown origin, characterized by frequent oral and genital ulcerations, ocular and cutaneous lesions, arthritis, and it may also involve the gastrointestinal, and central nervous system. In this paper we discuss a case of Behcet syndrome presenting with isolated oral aphthous initially and spotlight on the clinical features and treatment.Case reportA case of a 32-year-old male with repetitive oral aphthous on the tongue and the inner cheeks for four days with a history of frequent recurrence. The patient responded to topical triamcinolone acetonide and 5-10 mg/day prednisone. Three weeks later, there was a recurrence of oral aphthous, genitalia lesions, and blurred vision with left eye redness. A uveitis diagnosis was made, and the patient was placed on 75mg (1mg/kg) prednisone with a resultant response. There was another recurrence with all the previous symptoms, so the decision with Dopson treatment was made. The patient was placed on Dapsone, resulting in complete resolution of the lesions with no reproduction within and after six months of several recall visits. The diagnosis of Bechet syndrome was made.DiscussionAphthous ulcers may be seen in a variety of disorders, while recurrent and persistent >6 weeks, individual non-healing ulcers require a review of the diagnosis. Behcet disease usually appears 2-3 years after oral and genital ulcers, but oral aphthae may be its first manifestation in 10-20% of cases. AS oral ulcers are among the common complaints of the polyclinics-patients, it is recommended to educate the health professionals to diagnose these unusual cases in the first instance, thus avoiding underdiagnosed and the time management to reduce complications and improve the quality of life in these patients.ConclusionA case of Behçet's disease in a 32-year-old male who presented initially with oral mucosa and later caused uveitis and genitalia aphthae.
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