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Journal of Translational Medicine Dec 2021Myasthenia gravis is a neuromuscular autoimmune disorder characterized by weakness and disability in the voluntary muscles. There have been several preliminary studies... (Meta-Analysis)
Meta-Analysis
BACKGROUND
Myasthenia gravis is a neuromuscular autoimmune disorder characterized by weakness and disability in the voluntary muscles. There have been several preliminary studies on the epidemiology of myasthenia gravis in different parts of the world and the effectiveness of common drugs in its treatment, but there has been no comprehensive study of the efficacy of common drugs in the treatment of myasthenia gravis. Therefore, this study aimed to determine the epidemiology of myasthenia gravis globally and the effectiveness of common drugs in its treatment using systematic review and meta-analysis.
METHODS
Research studies were extracted from IranDoc, MagIran, IranMedex, SID, ScienceDirect, Web of Sciences (WoS), ProQuest, Medline (PubMed), Scopus and Google Scholar based on Cochran's seven-step guidelines using existing keywords extracted in MeSH browser. The I test was used to calculate the heterogeneity of studies, and Begg and Mazumdar rank correlation tests were used to assess publication bias. Data were analyzed using Comprehensive Meta-Analysis software (Version 2).
RESULTS
In the search for descriptive studies based on the research question, 7374 articles were found. After deleting articles unrelated to the research question, finally, 63 articles with a sample size of 1,206,961,907 people were included in the meta-analysis. The prevalence of MG worldwide was estimated to be 12.4 people (95% CI 10.6-14.5) per 100,000 population. For analytical studies on the effectiveness of common myasthenia gravis drugs, 4672 articles were found initially, and after removing articles unrelated to the research question, finally, 20 articles with a sample size of 643 people in the drug group and 619 people in the placebo group were included in the study. As a result of the combination of studies, the difference between the mean QMGS score index after taking Mycophenolate and Immunoglobulin or plasma exchange drugs in the group of patients showed a significant decrease of 1.4 ± 0.77 and 0.62 ± 0.28, respectively (P < 0.01).
CONCLUSION
The results of systematic review of drug evaluation in patients with myasthenia gravis showed that Mycophenolate and Immunoglobulin or plasma exchange drugs have positive effects in the treatment of MG. It also represents the positive effect of immunoglobulin or plasma exchange on reducing SFEMG index and QMGS index and the positive effect of Mycophenolate in reducing MG-ADL index, SFEMG and Anti-AChR antibodies index. In addition, based on a meta-analysis of the random-effect model, the overall prevalence of MG in the world is 12.4 people per 100,000 population, which indicates the urgent need for attention to this disease for prevention and treatment.
Topics: Humans; Immunosuppressive Agents; Myasthenia Gravis; Plasma Exchange; Prevalence
PubMed: 34930325
DOI: 10.1186/s12967-021-03185-7 -
European Journal of Neurology Feb 2022New-onset refractory status epilepticus (NORSE) is a clinical presentation, neither a specific diagnosis nor a clinical entity. It refers to a patient without active... (Review)
Review
BACKGROUND AND PURPOSE
New-onset refractory status epilepticus (NORSE) is a clinical presentation, neither a specific diagnosis nor a clinical entity. It refers to a patient without active epilepsy or other pre-existing relevant neurological disorder, with a NORSE without a clear acute or active structural, toxic or metabolic cause. This study reviews the currently available evidence about the aetiology of patients presenting with NORSE and NORSE-related conditions.
METHODS
A systematic search was carried out for clinical trials, observational studies, case series and case reports including patients who presented with NORSE, febrile-infection-related epilepsy syndrome or the infantile hemiconvulsion-hemiplegia and epilepsy syndrome.
RESULTS
Four hundred and fifty records were initially identified, of which 197 were included in the review. The selected studies were retrospective case-control (n = 11), case series (n = 83) and case reports (n = 103) and overall described 1334 patients both of paediatric and adult age. Aetiology remains unexplained in about half of the cases, representing the so-called 'cryptogenic NORSE'. Amongst adult patients without cryptogenic NORSE, the most often identified cause is autoimmune encephalitis, either non-paraneoplastic or paraneoplastic. Infections are the prevalent aetiology of paediatric non-cryptogenic NORSE. Genetic and congenital disorders can have a causative role in NORSE, and toxic, vascular and degenerative conditions have also been described.
CONCLUSIONS
Far from being a unitary condition, NORSE is a heterogeneous and clinically challenging presentation. The development and dissemination of protocols and guidelines to standardize diagnostic work-up and guide therapeutic approaches should be implemented. Global cooperation and multicentre research represent priorities to improve the understanding of NORSE.
Topics: Adult; Child; Drug Resistant Epilepsy; Encephalitis; Epileptic Syndromes; Humans; Retrospective Studies; Status Epilepticus
PubMed: 34661330
DOI: 10.1111/ene.15149 -
Journal of Cancer 2024There remains a scarcity of published data on the clinical significance of paraneoplastic cutaneous manifestations in hepatocellular carcinoma (HCC). A systematic... (Review)
Review
There remains a scarcity of published data on the clinical significance of paraneoplastic cutaneous manifestations in hepatocellular carcinoma (HCC). A systematic search of MEDLINE was performed in December 2022. Inclusion criteria comprised studies reporting on patients with HCC, who had paraneoplastic cutaneous manifestations. Outcomes of interests comprise survival and response to cancer-directed and/or skin directed therapy. A total of 48 studies comprising 60 HCC patients were included in the analysis. The most frequent reported skin abnormalities were dermatomyositis, pityriasis rotunda, and porphyria. Most patients presented with dermatomyositis had underlying viral hepatitis, while all reported porphyria and acanthosis cases were associated with metabolic causes of HCC, such as steatosis. Paraneoplastic skin changes were more common in patients with metastatic disease. Pityriasis Rotunda was associated with the lowest risk of death, (OR: 0.05, 95% CI: 0.003 to 0.89; p = 0.04), while dermatomyositis had a statistically significant higher risk of death (OR: 3.37, 95% CI: 1.01-12.1; p = 0.03). Most patients showed an improvement in their cutaneous abnormalities, following cancer-directed therapy. Paraneoplastic cutaneous manifestations are reported more frequently in patients with a higher burden of disease, especially presence of metastases. Certain cutaneous manifestations have prognostic implication.
PubMed: 38230223
DOI: 10.7150/jca.88931 -
Academic Radiology Oct 2017This study aimed to assess the clinical efficacy of positron emission tomography (PET) or combined PET-computed tomography (CT) with F-fluorodeoxyglucose (FDG) for... (Review)
Review
RATIONALE AND OBJECTIVES
This study aimed to assess the clinical efficacy of positron emission tomography (PET) or combined PET-computed tomography (CT) with F-fluorodeoxyglucose (FDG) for whole-body cancer screening in patients with suspected paraneoplastic neurological syndromes (PNS). The following main research questions were addressed: What is the percentage of positive findings to be expected in whole-body FDG-PET-CT in adult patients with PNS? How many false positives can be expected as assessed by clinical and histopathological workup? Are there patients who present with a tumor despite initially negative findings?
MATERIALS AND METHODS
This is a systematic review of the literature and retrospective analysis of FDG-PET-CT and clinical follow-up data from 45 consecutive patients (age: 56.6 ± standard deviation 15.8 years, 14 female, 31 male). Suspicious lesions were identified and correlated with immediate workup and clinical follow-up.
RESULTS
Fourteen studies were included in the review. Eleven malignancies (24.4% of patients) were identified by FDG-PET-CT in this sample. This is a higher percentage of positive findings compared to most previous reports. There was one initially negative finding.
CONCLUSIONS
Whole-body FDG-PET-CT is suitable to identify additional malignancies in patients with suspected classical PNS referred to a tertiary medical center. The utility by means of true-positive findings is higher in classical PNS than suggested by studies in less select patient populations.
Topics: False Positive Reactions; Female; Fluorodeoxyglucose F18; Humans; Male; Middle Aged; Neoplasms; Paraneoplastic Syndromes, Nervous System; Positron Emission Tomography Computed Tomography; Positron-Emission Tomography; Radiopharmaceuticals; Retrospective Studies; Whole Body Imaging
PubMed: 28551401
DOI: 10.1016/j.acra.2017.03.022 -
Frontiers in Neurology 2020Algorithms for the detection of a malignancy in patients with unclear neurologic symptoms of suspicious paraneoplastic origins are not universally applied. Frequently,...
Algorithms for the detection of a malignancy in patients with unclear neurologic symptoms of suspicious paraneoplastic origins are not universally applied. Frequently, circulating tumor markers (TMs) are considered a valuable tool for cancer diagnosis in patients with paraneoplastic neurologic syndromes (PNS). Our aim was to extract the recommendations on the use of TMs and onconeural antibodies (Abs) for the diagnosis of malignancies in PNS from clinical practice guidelines and put them forward as evidence in a common framework to facilitate diffusion, dissemination, and implementation. Systematic literature searches were performed for guidelines on both oncology and PNS published since 2007. Guidelines containing information and recommendations for clinical practice pertaining to the screening and diagnosis of PNS were selected. Information on circulating TMs and onconeural Abs was extracted and synthesized in consecutive steps of increasing simplification. We retrieved 799 eligible guidelines on oncology for the potential presence of information on PNS but only six covered treated diagnosis or the screening of cancer in PNS, which were then selected. Seventy-nine potentially relevant guidelines on PNS were identified as eligible and 15 were selected. Synoptic tables were prepared showing that classical TMs are not recommended for the screening or the diagnosis of a malignancy in patients with a suspected PNS. Neither should onconeural Abs be considered to screen for the presence of a malignancy, although they could be helpful to define the probability of the paraneoplastic origin of a neurologic disorder. The present work of synthesis may be a useful tool in the diffusion, dissemination, and implementation of guideline recommendations, potentially facilitating the decrease of the inappropriate use of circulating biomarkers for cancer screening in the presence of PNS.
PubMed: 33536995
DOI: 10.3389/fneur.2020.607553 -
Lung Cancer (Amsterdam, Netherlands) Apr 2017The development of new immune treatment in oncology and particularly for lung cancer may induce new complications, particularly activation or reactivation of auto-immune... (Review)
Review
The development of new immune treatment in oncology and particularly for lung cancer may induce new complications, particularly activation or reactivation of auto-immune diseases. In this context, a systematic review on the auto-immune paraneoplastic syndromes associated with lung cancer appears useful. This article is the first of a series of five and deals with the methodology applied for the review and with renal and rheumatic syndromes.
Topics: Adult; Aged; Aged, 80 and over; Female; Humans; Immunotherapy; Kidney Diseases; Lung Neoplasms; Male; Middle Aged; Paraneoplastic Syndromes; Rheumatic Diseases
PubMed: 28285683
DOI: 10.1016/j.lungcan.2017.01.015 -
The British Journal of Oral &... Jul 2010The aim of this study was to review all the paraneoplastic syndromes of primary tumours of the oral cavity. Metastatic tumours of the mouth and primary tumours of the... (Review)
Review
The aim of this study was to review all the paraneoplastic syndromes of primary tumours of the oral cavity. Metastatic tumours of the mouth and primary tumours of the oropharynx (including tonsils), and major salivary glands were excluded. The primary search was conducted on PubMed, Scopus and EMBASE, and included every paraneoplastic syndrome from a primary oral tumour described in English, French, or German papers during the last 20 years. The secondary search was conducted by handpicking articles from reviews on paraneoplastic syndromes of the head and neck. The aim of the tertiary search was to identify conditions that had been reported only rarely. We then cross-referenced "mouth neoplasm" with every paraneoplastic condition cited in relevant review articles. We classified the paraneoplastic syndromes that arose from tumours of the head and neck into six categories: endocrine, dermatological, vascular and haematological, rheumatoid, ocular, and neurological. The following conditions are described in this review: syndrome of inappropriate antidiuretic hormone production, hypercalcaemia, hypercalcaemia-leucocytosis syndrome, ectopic production of beta-human chorionic gonadotrophin, Bazex syndrome, Sweet syndrome, tripe palm syndrome, pemphigus, pityriasis rotunda, neutrophilic leukemoid reaction, cerebral venous sinus thrombophlebitis, digital ischaemia, dermatomyositis, necrotising myopathy, autoimmune retinal degeneration, and subacute cerebellar degeneration. Paraneoplastic syndromes of the oral cavity are a heterogeneous group. Most syndromes occur from squamous cell carcinoma and their aetiology is poorly understood. They are important to recognise as they can be the presenting complaint of a malignant tumour, change the prognosis, and considerably reduce the quality of life.
Topics: Carcinoma, Squamous Cell; Humans; Mouth Neoplasms; Paraneoplastic Syndromes
PubMed: 19833419
DOI: 10.1016/j.bjoms.2009.08.025 -
Journal of the Neurological Sciences Nov 2023Paraneoplastic neurologic syndromes (PNS) and autoimmune encephalitis (AIE) are immune-mediated disorders. PNS is linked to cancer, while AIE may not Their clinical... (Review)
Review
INTRODUCTION
Paraneoplastic neurologic syndromes (PNS) and autoimmune encephalitis (AIE) are immune-mediated disorders. PNS is linked to cancer, while AIE may not Their clinical manifestations and imaging patterns need further elucidation.
OBJECTIVE/AIMS
To investigate the clinical profiles, antibody associations, neuroimaging patterns, treatments, and outcomes of PNS and AIE.
METHODS
A systematic review of 379 articles published between 2014 and 2023 was conducted. Of the 55 studies screened, 333 patients were diagnosed with either PNS or AIE and tested positive for novel antibodies. Data on demographics, symptoms, imaging, antibodies, cancer associations, treatment, and outcomes were extracted.
RESULTS
The study included 333 patients (mean age 54 years, 67% males) with PNS and AIE positive for various novel antibodies. 84% had central nervous system issues like cognitive impairment (53%), rhombencephalitis (17%), and cerebellar disorders (24%). Neuroimaging revealed distinct patterns with high-risk antibodies associated with brainstem lesions in 98%, cerebellar in 91%, hippocampal in 98%, basal ganglia in 75%, and spinal cord in 91%, while low/intermediate-risk antibodies were associated with medial temporal lobe lesions in 71% and other cortical/subcortical lesions in 55%. High-risk antibodies were associated with younger males, deep brain lesions, and increased mortality of 61%, while low/intermediate-risk antibodies were associated with females, cortical/subcortical lesions, and better outcomes with 39% mortality. Associated cancers included seminomas (23%), lung (19%), ovarian (2%), and breast (2%). Treatments included IVIG, chemotherapy, and plasmapheresis. Overall mortality was 25% in this cohort.
CONCLUSION
PNS and AIE have distinct clinical and radiological patterns based on antibody profiles. High-risk antibodies are associated with increased mortality while low/intermediate-risk antibodies are associated with improved outcomes. Appropriate imaging and antibody testing are critical for accurate diagnosis.
Topics: Male; Female; Humans; Middle Aged; Nervous System Diseases; Paraneoplastic Syndromes, Nervous System; Autoantibodies; Neoplasms; Neuroimaging
PubMed: 37856996
DOI: 10.1016/j.jns.2023.120830 -
Lung Cancer (Amsterdam, Netherlands) Sep 2017The development of new immune treatment in oncology and particularly for lung cancer may induce new complications, particularly activation or reactivation of auto-immune... (Meta-Analysis)
Meta-Analysis Review
Autoimmune paraneoplastic syndromes associated to lung cancer: A systematic review of the literature Part 4: Neurological paraneoplastic syndromes, involving the peripheral nervous system and the neuromuscular junction and muscles.
The development of new immune treatment in oncology and particularly for lung cancer may induce new complications, particularly activation or reactivation of auto-immune diseases. In this context, a systematic review on the auto-immune paraneoplastic syndromes that can complicate lung cancer appears useful. This article is the fourth of a series of five and deals mainly with neurological paraneoplastic syndromes involving the peripheral nervous system and the neuromuscular junction and muscles.
Topics: Autoantibodies; Autoimmune Diseases; Autoimmunity; Humans; Lung Neoplasms; Muscles; Neuromuscular Junction; Paraneoplastic Syndromes; Paraneoplastic Syndromes, Nervous System; Peripheral Nervous System
PubMed: 28838388
DOI: 10.1016/j.lungcan.2017.07.025 -
The Oncologist Dec 2021The development of immune checkpoint inhibitors (ICIs) represents a paradigm shift in the treatment of cancers. Despite showing remarkable efficacy, these agents can be...
BACKGROUND
The development of immune checkpoint inhibitors (ICIs) represents a paradigm shift in the treatment of cancers. Despite showing remarkable efficacy, these agents can be associated with life-threatening immune-related adverse events. In recent years, several cases of myocarditis with myositis and/or myasthenia gravis overlap syndrome (IM3OS) have been reported. However, given the rarity, the clinical features and outcomes of these cases remain poorly understood. We, therefore, attempted to systematically review and summarize all cases of IM3OS reported in the literature.
MATERIALS AND METHODS
Studies reporting IM3OS were identified in Embase and MEDLINE. Only case reports and case series published in journals or presented at conferences were included. We conducted a systematic review according to the PRISMA Harms guidelines.
RESULTS
A total of 60 cases were eligible. The patients' median age was 71 years, and the majority (67%) were males; melanoma was the most common indication for ICIs (38%). The most-reported symptoms were fatigue (80%) and muscle weakness (78%). The median number of doses to the development of IM3OS was one. The average creatine kinase level was 9,645 IU/L. Cardiac arrhythmias occurred in 67% of patients, and 18% had depressed ejection fraction. Initial treatment consisted of immunosuppression with high-dose steroids and supportive therapies. Sixty percent of the patients died in hospital because of acute complications.
CONCLUSION
IM3OS can be associated with significant mortality and morbidity. Prospective studies are needed to understand the optimal approach to diagnose and manage these patients and to develop biomarkers to predict the occurrence and severity of this rare but serious condition.
IMPLICATIONS FOR PRACTICE
Clinicians should suspect coexisting myositis and/or myasthenia gravis in all patients with immune checkpoint inhibitor-induced myocarditis, given their propensity to occur together. Early recognition and prompt treatment with the help of a multidisciplinary team might help improve the outcomes of this life-threatening condition.
Topics: Aged; Humans; Immune Checkpoint Inhibitors; Myasthenia Gravis; Myocarditis; Myositis
PubMed: 34378270
DOI: 10.1002/onco.13931