scholarly journals Anti -Iglon5 Syndrome: What we know so far? A non-systematic review

2021 ◽  
Author(s):  
Gustavo Figueiredo da Silva ◽  
Caroline Figueiredo da Silva ◽  
Washigton Luiz Gomes de Medeiros Junior ◽  
Marcus Vinícius Magno Gonçalves
Keyword(s):  
Systematic Review ◽  
Current Knowledge ◽  
Immune Therapy ◽  
Case Series ◽  
Neuronal Loss ◽  
Autoimmune Response ◽  
Cervical Cord ◽  
Protein Accumulation ◽  
Antibody Testing ◽  
Pubmed Database

Background: The first report of Anti-IgLON5 syndrome was in 2014. AntiIgLON5 antibodies have a prevalence of 12 in 150,000 patients per year. However, considering the unreported patients, the prevalence can be much higher. Objectives: Provide an overview of the current knowledge of Anti- IgLON5 syndrome. Design and setting: Narrative review. Methods: Non- systematic review on Pubmed database. Results: The IgLON proteins are a family of cell adhesion molecules and the presence of antibodies against IgLON5 is crucial for the AntiIgLON5 Syndrome diagnosis. This syndrome has an expanded clinical spectrum that involves prominent sleep disorder, progressive bulbar dysfunction, gait instability with abnormal eye movements reminiscent, and cognitive deterioration sometimes associated with chorea. The main neuropathological finding is the neuronal loss with hyperphosphorylated tau protein accumulation at the hypothalamus, brainstem tegmentum, hippocampus, periaqueductal gray matter, medulla oblongata, and upper cervical cord. The exact pathogenesis is still unclear and involves a neurodegenerative process and autoimmune response. Early diagnosis is important to avoid unnecessary tests and prevent complications. Important resources for diagnosis are the antibody testing of serum and cerebrospinal fluid for IgLON5-IgG. The Anti-IgLON5 syndrome mortality is high and new studies published described a good response to immune therapy, however, depends on some clinical and analytical characteristics. Conclusions: The Anti-Iglon5 syndrome is a pathology still poorly studied and described in the medical literature (only in case series, for example), being a syndrome probably underdiagnosed. Future studies are needed to thoroughly analyze the aspects of pathogenesis and treatment of this important pathological syndrome.

scholarly journals Anti Iglon5 Syndrome: What We Know So Far? A Non-Systematic Review

2020 ◽  
Author(s):  
Caroline Figueiredo da Silva ◽  
Gustavo Figueiredo da Silva ◽  
Washigton Luiz Gomes de Medeiros Junior ◽  
Marcus Vinícius Magno Gonçalves
Keyword(s):  
Current Knowledge ◽  
Immune Therapy ◽  
Neuronal Loss ◽  
Autoimmune Response ◽  
Cervical Cord ◽  
Protein Accumulation ◽  
Antibody Testing ◽  
Neurodegenerative Process ◽  
Upper Cervical ◽  
Neuropathological Finding

The objective of this review is to do an overview about the current knowledge of Anti Iglon5 Syndrome, a disease that was first described in 2014. The IgLON proteins are a family of cell adhesion molecules and the presence of antibodies against IgLON5 are crucial for diagnosis of Anti IgLON5 Syndrome. This syndrome has an expanded clinical spectrum that involves prominent sleep disorder, progressive bulbar dysfunction, gait instability with abnormal eye movements reminiscent and cognitive deterioration sometimes associated with chorea. The main neuropathological finding is the neuronal loss with hyperphosphorylated tau (p-Tau) protein accumulation at hypothalamus, brainstem tegmentum, hippocampus, periaqueductal gray matter, medulla oblongata and upper cervical cord. The exact pathogenesis is still unclear and involves a neurodegenerative process and autoimmune response. The early diagnosis is important to avoid unnecessary tests and prevent complications. Important resources for diagnosis are the antibody testing of serum and CSF for IgLON5-IgG. The mortality of anti IgLON5 syndrome is high and new studies published described a good response to immune therapy. However, the response to immune therapy depends of some clinical and analytical characteristic. In addition, future studies are needed to thoroughly study the aspects of pathogenesis and treatment of this important pathological syndrome.

scholarly journals Villoglandular adenocarcinoma of the uterine cervix: a systematic review and meta-analysis

2021 ◽  
Author(s):  
Anna K. Dietl ◽  
Matthias W. Beckmann ◽  
Konrad Aumann
Keyword(s):  
Systematic Review ◽  
Uterine Cervix ◽  
Case Reports ◽  
Case Series ◽  
Disease Free Survival ◽  
Conservative Group ◽  
Pubmed Database ◽  
Significant Difference ◽  
Stage Ia ◽  
Invasive Management

Abstract Purpose Villoglandular adenocarcinoma (VGA) of the uterine cervix has been classified as a rare subtype of cervical adenocarcinoma with good prognosis. A conservative surgical approach is considered feasible. The main risk factor is the presence of other histologic types of cancer. In this largest systematic review to date, we assess oncological outcomes associated with conservative therapy compared to those associated with invasive management in the treatment of stage Ia and Ib1 VGA. Methods Case series and case reports identified by searching the PubMed database were eligible for inclusion in this review (stage Ia–Ib1). Results A total of 271 patients were included in our literature review. 54 (20%) patients were treated by “conservative management” (conization, simple hysterectomy, and trachelectomy) and 217 (80%) by “invasive management” (radical hysterectomy ± radiation, hysterectomy, and radiation). Recurrences of disease (RODs) were found in the conservative group in two (4%) cases and in the invasive group in nine (4%) cases. There was no significant difference in disease-free survival (DFS) according to conservative or invasive treatment (p = 0.75). The histology of VGA may be complex with underlying usual adenocarcinoma (UAC) combined with VGA. Conclusion The excellent prognosis of pure VGA and the young age of the patients may justify the management of this tumor using a less radical procedure. The histological diagnosis of VGA is a challenge, and pretreatment should not be based solely on a simple punch biopsy but rather a conization with wide tumor-free margins.

scholarly journals 119. Nocardia Bacteremia Is Almost Always Associated with Immune Compromise or an Intravascular Device: Single-Center Case Series and Systematic Review of the Literature

2019 ◽  
Vol 6 (Supplement_2) ◽  
pp. S90-S90
Author(s):  
Eloise Williams ◽  
Adam W Jenney ◽  
Denis W Spelman
Keyword(s):  
Systematic Review ◽  
Literature Review ◽  
Systematic Literature Review ◽  
English Language ◽  
Case Series ◽  
Blood Cultures ◽  
Single Center ◽  
Intravascular Device ◽  
Pubmed Database ◽  
All Cause Mortality

Abstract Background Nocardia bacteremia is a rare but important phenomenon, with previous studies describing a 50% mortality rate. We undertake a single-center review and the largest systematic review of Nocardia bacteremia performed over the past 20 years. Methods A single-center review of cases of Nocardia bacteremia was performed using hospital microbiology records from January 1, 2010 to December 31, 2017. A systematic literature review was also performed to identify cases of Nocardia bacteremia described in the English language literature between January 1, 1999 and December 31, 2018 using the NCBI PubMed database and snowballing from citations of relevant publications. Results Single-center case series: Four cases of Nocardia bacteremia are described. Three patients had an intravascular device in situ prior to the onset of Nocardia bacteremia and three patients were immunocompromised; one patient had both risk factors. Systematic literature review: A systematic review identified 50 publications that described 85 cases with sufficient patient data to be reviewed in detail. Including the 4 cases described in our institution, 89 cases of Nocardia bacteremia were included in the analysis. The median age was 57 years [interquartile range (IQR) 42–68] and 69% were male. Eighty-two percent of cases were immunocompromised and 38% had endovascular devices. Pulmonary infection was the most common concurrent site of clinical disease (66%), followed by central nervous system (25%), pleural (17%) disease, and endocarditis (11%). Blood cultures were the only positive microbiological specimen that isolated Nocardia in 45% of cases. Median incubation time to blood culture positivity was 4 days [IQR 3–6]. Thirty-day all-cause mortality was 24% and overall all-cause mortality was 42%. Conclusion Four new cases of Nocardia bacteremia are described. Isolation of Nocardia from blood cultures is rare but represents serious infection with high associated overall mortality. Nocardia bacteremia is most frequently identified in immunocompromised patients and those with intravascular devices. Disclosures All authors: No reported disclosures.

Proposed modified diagnostic criteria for recurrent painful ophthalmoplegic neuropathy: Five case reports and literature review

Cephalalgia ◽  
2020 ◽  
Vol 40 (14) ◽  
pp. 1657-1670
Author(s):  
Yinglu Liu ◽  
Miao Wang ◽  
Xiangbing Bian ◽  
Enchao Qiu ◽  
Xun Han ◽  
...  
Keyword(s):  
Diagnostic Criteria ◽  
Cranial Nerve ◽  
Current Knowledge ◽  
Case Reports ◽  
Case Series ◽  
Oculomotor Nerve ◽  
Multiplanar Reformation ◽  
Unilateral Headache ◽  
Pubmed Database ◽  
Recurrent Painful Ophthalmoplegic Neuropathy

Background Recurrent painful ophthalmoplegic neuropathy (RPON) is an uncommon disorder characterized by recurrent unilateral headache attacks associated with ipsilateral ophthalmoplegia. We intend to study the clinical picture in our case series along with the published literature to discuss the pathogenesis and propose modified diagnostic criteria for recurrent painful ophthalmoplegic neuropathy. Methods We reported five cases diagnosed as ophthalmoplegic migraine/RPON in our medical centers and reviewed the published literature related to RPON from the Pubmed database between 2000 and 2020. In one of these cases, a multiplanar reformation was performed to look at the aberrant cranial nerve. Results The mean onset age for RPON was 22.1 years, and the oculomotor nerve was the most commonly involved cranial nerve (53.9%) in 165 reviewed patients. In most patients, ophthalmoplegia started within 1 week of the headache attack (95.7%, 67/70). Additionally, 27.6% (40/145) of patients presented enhancement of the involved nerve(s) from MRI tests. Finally, 78 patients received corticosteroids, out of which 96.2% benefited from them. Conclusion This is the first time multiplanar reformation has been performed to reveal the distortion of the oculomotor nerve. Modified diagnostic criteria are proposed. We hope to expand the current knowledge and increase the detection of recurrent painful ophthalmoplegic neuropathy in the future.

scholarly journals Role of active patient involvement in undergraduate medical education: a systematic review

BMJ Open ◽  
2020 ◽  
Vol 10 (7) ◽  
pp. e037217
Author(s):  
Stijntje Willemijn Dijk ◽  
Edwin Johan Duijzer ◽  
Matthias Wienold
Keyword(s):  
Systematic Review ◽  
Medical Education ◽  
Undergraduate Medical Education ◽  
Patient Involvement ◽  
Current Knowledge ◽  
Daily Practice ◽  
Pubmed Database ◽  
Wide Range ◽  
Active Patient

ObjectivesTo identify the scope of active patient involvement in medical education, addressing the current knowledge gaps relating to rationale and motivation for involvement, recruitment and preparation, roles, learning outcomes and key procedural contributors.MethodsThe authors performed a systematic search of the PubMed database of publications between 2003 and 2018. Original studies in which patients take on active roles in the development, delivery or evaluation of undergraduate medical education and written in English were eligible for inclusion. Included studies’ references were searched for additional articles. Quality of papers was assessed using the Mixed Methods Appraisal Tool.Results49 articles were included in the review. Drivers for patient involvement included policy requirements and patients’ own motivations to contribute to society and learning. Patients were engaged in a variety of educational settings in and outside of the hospital. The vast majority of studies describe patients taking on the role of a patient teacher and formative assessor. More recent studies suggest that patients are increasingly involved in course and curriculum development, student selection and summative assessment. The new body of empirical evidence shows the wide range of learning objectives was pursued through patient participation, including competencies as professional, communicator, collaborator, leader and health advocate, but not scholar. Measures to support sustainable patient involvement included longitudinal institutional incorporation, patient recruitment and/or training, resource support and clear commitment by faculty. The importance and advantages of patient involvement were highlighted by students, faculty and patients themselves; however, organisations must continue to consider, monitor and take steps to mitigate any potential harms to patients and students.DiscussionThis systematic review provides new knowledge and practical insights to physicians and faculty on how to incorporate active patient involvement in their institutions and daily practice, and provides suggested action points to patient organisations wishing to engage in medical education.

scholarly journals Vasculitis induced by biological agents used in rheumatology practice: A systematic review

2021 ◽  
Author(s):  
Camila da Silva Cendon Duran ◽  
Adriane Souza da Paz ◽  
Mittermayer Barreto Santiago
Keyword(s):  
Systematic Review ◽  
Leukocytoclastic Vasculitis ◽  
Case Reports ◽  
Case Series ◽  
Final Analysis ◽  
Infusion Reaction ◽  
Biological Agents ◽  
Necrosis Factor Alpha ◽  
Pubmed Database ◽  
Meta Analyses

Objectives: Biological medications have been used with an increasing frequency to treat rheumatological diseases. Autoimmune events can be induced by these drugs, such as psoriasiform lesions, alopecia, lupus and, vasculitis, which more often affects the skin (small-sized vessels) and eventually other organs. In this review, we describe the clinical profile of patients with vasculitis induced by the main biological agents used in rheumatology. Patients and methods: We performed a systematic review following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) recommendations. The PubMed database was used for searching eligible articles. We included case reports, case series, and letter to the editor of patients on anti-tumor necrosis factor-alpha (anti-TNF-a) molecules, as well as tocilizumab, ustekinumab, secukinumab, rituximab, and abatacept, who had vasculitis induced by these agents. Results: Eighty-one articles were included for final analysis (n=89). Twenty-seven patients were using infliximab, 20 adalimumab, 18 etanercept, seven secukinumab, four certolizumab, four rituximab, three golimumab, three ustekinumab, two abatacept, and one tocilizumab. Unspecific leukocytoclastic vasculitis (LCV) was the most common type of vasculitis (n=37), followed by anti-neutrophil cytoplasmic antibody (ANCA)- associated vasculitis (n=16). The medication was replaced with another biological molecule in 23 cases, with only four relapses. In six cases, the biological was maintained, but vasculitis worsened/persisted in one case, being necessary drug removal. Conclusion: Infections, infusion reaction, cancer, and autoimmune events are well-known side effects of biological therapy. This review demonstrates that vasculitis is another adverse effect of this type of therapy, particularly the anti-TNF-a molecules, and LCV the most reported type of vasculitis.

scholarly journals Impact of COVID-19 on the placental pathology of pregnant women and their infants: A systematic review protocol

2020 ◽  
Author(s):  
Irina Oltean ◽  
Dina El Demellawy
Keyword(s):  
Systematic Review ◽  
Pregnant Women ◽  
Clinical Outcomes ◽  
Viral Infections ◽  
Case Reports ◽  
Case Series ◽  
Cell Mediated Immunity ◽  
Case Control Studies ◽  
Antibody Testing ◽  
Adverse Pregnancy

Pregnant women are susceptible to viral infections due to physiological changes such as cell-mediated immunity. No severe adverse pregnancy or neonatal outcomes have been consistently reported in COVID-19 positive pregnancy cases. There are controversies around the role of COVID-19 in pregnancy. A systematic review was conducted to examine clinical maternal and neonatal clinical outcomes. Studies were included if they reported COVID-19 infection among pregnant women and/or COVID-19 positive neonates as validated by positive antibody testing or viral testing using Polymerase Chain Reaction. Case series, case reports, case-control studies, and comparative studies were included. 837 records were identified, resulting in 525 records for level I screening. 41 were included after full-text review. Results suggest elevated rates of ICU admission, gestational diabetes, preeclampsia, C-sections, pre-term birth, and CRP in comparison to pregnant women without SARS-CoV-2. Careful monitoring of pregnancies with SARS-CoV-2 to minimize adverse clinical outcomes is recommended.

Motor Recovery of the Suprascapular Nerve after Arthroscopic Decompression in the Scapular Notch – a Systematic Review

2020 ◽  
Author(s):  
Marius von Knoch ◽  
Stephan Frosch ◽  
Mike H. Baums ◽  
Wolfgang Lehmann
Keyword(s):  
Systematic Review ◽  
Case Series ◽  
Clinical Results ◽  
Suprascapular Nerve ◽  
Motor Recovery ◽  
Cochrane Library ◽  
Suprascapular Notch ◽  
Compression Syndrome ◽  
Pubmed Database ◽  
Scapular Notch

Abstract Background The suprascapular nerve can be compromised as a result of a compression syndrome in different locations. A (proximal) compression within the scapular notch can lead to dorsal shoulder pain and simultaneous weakness of the infraspinatus and supraspinatus muscles. By transection of the lig. transversum this compression syndrome can be treated. By means of a systematic review, the present work analyzes the motor recovery potential after arthroscopic decompression. Material and Methods A systematic review of the U. S. National Library of Medicine/National Institutes of Health (PubMed) database and the Cochrane Library was performed using the PRISMA checklist. The search words used were “suprascapular” and “arthroscopic”; “suprascapular” and “arthroscopy”. Based on the evaluated literature, articles in English with at least a partial arthroscopic case series from 4 cases on and a compression syndrome of the suprascapular nerve treated with arthroscopic decompression in the scapular notch were identified. Motor recovery was described by means of EMG, clinical strength and MRI. Results Primarily 408 hits were generated. Six articles met the inclusion criteria and were further analyzed. The number of arthroscopic cases was between 4 and a maximum of 27. The level of evidence was between III and IV. The majority of the reported clinical results were good. Motor recovery as measured by EMG was observed, recovery of full strength was not achieved in the majority of reported cases (60%), neither was regression of structural (fatty) degeneration of the muscle bellies. Conclusion Arthroscopic decompression of the suprascapular nerve in the scapular notch provides good clinical results and considerable pain relief. However, in the majority of cases it does not lead to a complete recovery of the strength of the supra- and infraspinatus muscles. Patients should be informed about this. An early decompression after diagnosis in the event of proximal compression within the suprascapular notch combined with beginning EMG or MRI changes appears reasonable. These beginning changes should be further defined. Future studies should develop prognostic criteria for motor recovery. Awareness regarding the diagnosis needs to be improved due to the probably time-dependent irreversibility of resulting muscular weakness.

Arthroscopic Reconstruction of Intratendinous Lesions of the Supraspinatus Tendon – a Systematic Review

2018 ◽  
Vol 156 (05) ◽  
pp. 547-553
Author(s):  
Marius von Knoch ◽  
Stephan Frosch ◽  
Wolfgang Lehmann
Keyword(s):  
Systematic Review ◽  
Conservative Treatment ◽  
Mr Imaging ◽  
Case Series ◽  
Supraspinatus Tendon ◽  
Cochrane Library ◽  
Arthroscopic Reconstruction ◽  
Future Studies ◽  
Supraspinatus Tear ◽  
Pubmed Database

Abstract Background Intratendinous lesions of the rotator cuff of the shoulder are frequent and may be a distinct clinical entity. Nevertheless, there are only a few publications which deal specifically with this subject. This study analyses the existing literature for the arthroscopic reconstruction of the intratendinous lesion of the supraspinatus tendon, by means of a systematic review, and identifies relevant research questions for future studies. Material and Methods In January 2017, a systematic review of the U. S. National Library of Medicine/National Institutes of Health (PubMed) Database and the Cochrane Library was conducted using the PRISMA checklist. The search words were “supraspinatus” and “interstitial”; “supraspinatus”, “tear” and “intratendinous”; “supraspinatus” and “concealed”. In the course of the review, articles written in English with at least a partial arthroscopic case series dealing with the reconstruction of the supraspinatus tendon were identified and further analysed. Results Primarily 70 hits could be generated. Five articles met the inclusion criteria and were analysed in detail. The number of arthroscopic cases ranged between 6 and 33. Level of evidence was IV in all studies. The diagnosis of an intratendinous lesion was made by MR imaging when T2/fat-saturated sequences showed an intratendinous high intensity signal without disruption of the bursal or articular layer. Three different concepts were followed in surgical treatment: opening of the intratendinous lesion from the bursal or articular side or by complete resection of the lesion. The reconstruction was performed with suture anchors in all cases. In the majority of cases, an acromioplasty was also performed. The reported clinical results were mostly good. Healing of the tendon was shown by MR imaging in 81.5 to 100% of cases. Conclusion After failure of conservative treatment, symptomatic intratendinous lesions of the supraspinatus tendon can be localised intraoperatively and reconstructed after failure of conservative treatment. The expected results are good in the medium term. The evidence level of the studies analysed was low. Future studies should examine the role of alternative conservative and surgical therapies.

Uneventful delivery of two pregnancies in a woman with severe factor XII deficiency: case report and systematic review

2018 ◽  
Vol 7 (1) ◽  
Author(s):  
Stamatios Petousis ◽  
George Karavas ◽  
Chrysoula Margioula-Siarkou ◽  
Themistoklis Dagklis ◽  
Paraskevi Karapavlidou ◽  
...  
Keyword(s):  
Systematic Review ◽  
Case Reports ◽  
Case Series ◽  
Signs And Symptoms ◽  
Factor Xii ◽  
Case Presentation ◽  
Severe Deficiency ◽  
Pubmed Database ◽  
Increased Risk

Abstract Background Deficiency of factor XII (FXII) is widely considered to have a detrimental effect on pregnancy. Several reports underline the increased risk for antenatal complications with few published case reports of uncomplicated deliveries. The main objective of our article is to perform a systematic review to highlight pregnancies with severe deficiency of FXII that have been delivered uneventfully, along with presenting our relative case of a woman with severe deficiency of FXII. Materials and methods A systematic review was performed in the Pubmed database. Inclusion criteria were considered to be case reports and case series presenting delivery of uncomplicated pregnancies in women with severe FXII deficiency. Medical records of our patient were also reviewed in terms of signs and symptoms, laboratory and imaging examinations and neonatal outcomes. Results There were 62 abstracts derived while 44 were assessed for eligibility. There were finally three case reports of women with FXII deficiency delivering live newborns and one case series of 12 women with a final outcome of 19 deliveries. Regarding our case presentation, the woman with FXII levels <12%, after a neonatal death because of extreme prematurity (24 weeks + 4 days), was set in regular follow-up and treatment with bemiparin natriate, 3.5 mg/kg and acetylsalicylic acid, 100 mg/day. She finally managed to have her second pregnancy delivered at 38 weeks + 3 days, her third pregnancy ended up as a miscarriage and her fourth pregnancy was also delivered at 37 weeks + 4 days. Conclusion Despite the increased risk for antenatal complications, appropriate follow-up of pregnancies with severe FXII pregnancy may finally lead to an uneventful delivery.

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