22,622 results match your criteria Paraneoplastic Encephalomyelitis

Refractory Anti-NMDA Receptor Encephalitis in Early Pregnancy: A Case Report of Treatment Course and Pregnancy Outcomes.

Neurol Neuroimmunol Neuroinflamm 2022 Sep 21;9(5). Epub 2022 Jun 21.

From the Department of Neurology (S.F., C.W., L.T., K.M.B., S.V.), University of Texas Southwestern Medical Center, Dallas, TX; and Department of Neurology (R.N.), Barrow Neurological Institute, Phoenix, AZ.

Background And Objectives: Anti-N-methyl d-aspartate receptor (NMDAR) encephalitis classically affects women of childbearing age, producing a disproportionate number of pregnant women with anti-NMDAR encephalitis. The typical presentation includes progressive neuropsychiatric symptoms, seizures, and alterations in consciousness, all of which present potential risks to the fetus. First-line and second-line treatments similarly pose teratogenic potential; therefore, randomized studies with supportive data on pregnancy and fetal outcomes are lacking. Read More

View Article and Full-Text PDF
September 2022

Immune Response and Safety of SARS-CoV-2 mRNA-1273 Vaccine in Patients With Myasthenia Gravis.

Neurol Neuroimmunol Neuroinflamm 2022 Jul 21;9(4). Epub 2022 Jun 21.

From the Neuromuscular Diseases Unit (D.R.-L., R.R.-G., J.T.-S., L.Q., M.O., R.Á.-V., M.C.-Á., Á.C., A.V.-C., I.I., E.C.-V.), Department of Neurology, Hospital de la Santa Creu i Sant Pau, "Departament de Medicina," Universitat Autònoma de Barcelona; Neuromuscular Diseases Lab (D.R.-L., R.R.-G., J.T.-S., L.Q., M.O., R.Á.-V., M.C.-Á., Á.C., A.V.-C., I.I., E.G., E.C.-V.), Institut de Recerca Hospital de la Santa Creu i Sant Pau (IIB-Sant Pau), Barcelona; Centro de Investigación Biomédica en Red de Enfermedades Raras (CIBERER) (D.R.-L., F.P.-J., R.R.-G., J.T.-S., L.Q., M.O., R.Á.-V., M.C.-Á., Á.C., I.I., E.G., E.C.-V.), Valencia; Unidad de Enfermedades Infecciosas (J.L.-C., P.D.), Servicio de Medicina Interna, Hospital de la Santa Creu i Sant Pau; and Department of Immunology (E.M., E.L.-P.), Hospital de la Santa Creu i Sant Pau, Biomedical Research Institute Sant Pau (IIB Sant Pau), Universitat Autònoma de Barcelona, Spain.

Background And Objectives: Evidence regarding the safety and efficacy of messenger RNA (mRNA) vaccines in patients with myasthenia gravis (MG) after immunosuppressive therapies is scarce. Our aim is to determine whether the mRNA-1273 vaccine is safe and able to induce humoral and cellular responses in patients with MG.

Methods: We performed an observational, longitudinal, prospective study including 100 patients with MG of a referral center for MG in our country, conducted from April 2021 to November 2021 during the vaccination campaign. Read More

View Article and Full-Text PDF

Hippocampal Neuronal Cultures to Detect and Study New Pathogenic Antibodies Involved in Autoimmune Encephalitis.

J Vis Exp 2022 Jun 2(184). Epub 2022 Jun 2.

ICFO-Institut de Ciències Fotòniques, The Barcelona Institute of Science and Technology; Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Hospital Clínic, Universitat de Barcelona;

Over the last 15 years, a new category of antibody-mediated diseases of the central nervous system (CNS) has been characterized and is now defined as "autoimmune encephalitis" (AE). There are currently 17 known AE syndromes, and all are associated with antibodies against the neuronal cell surface or synaptic proteins. The clinical syndromes are complex and vary according to the type of associated antibody. Read More

View Article and Full-Text PDF

Clinical Variables, Deep Learning and Radiomics Features Help Predict the Prognosis of Adult Anti-N-methyl-D-aspartate Receptor Encephalitis Early: A Two-Center Study in Southwest China.

Front Immunol 2022 1;13:913703. Epub 2022 Jun 1.

Department of Radiology, The First Affiliated Hospital, Chongqing Medical University, Chongqing, China.

Objective: To develop a fusion model combining clinical variables, deep learning (DL), and radiomics features to predict the functional outcomes early in patients with adult anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis in Southwest China.

Methods: From January 2012, a two-center study of anti-NMDAR encephalitis was initiated to collect clinical and MRI data from acute patients in Southwest China. Two experienced neurologists independently assessed the patients' prognosis at 24 moths based on the modified Rankin Scale (mRS) (good outcome defined as mRS 0-2; bad outcome defined as mRS 3-6). Read More

View Article and Full-Text PDF

Anti-N-methyl-D-aspartate receptor encephalitis: characteristics and rapid diagnostic approach in the emergency department.

BMC Neurol 2022 Jun 18;22(1):224. Epub 2022 Jun 18.

Emergency Department, State Key Laboratory of Complex Severe and Rare Disease, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China.

Background: Anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis is a common type of autoimmune encephalitis. Patients with this condition are frequently very ill but are often misdiagnosed in the Emergency Department (ED). The objective of this study was to analyze the clinical characteristics of anti-NMDAR patients in the ED and to identify any associations with a diagnosis of anti-NMDAR encephalitis. Read More

View Article and Full-Text PDF

Neuropsychiatric lupus erythematosus with neurogenic pulmonary edema and anti-α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor limbic encephalitis: a case report.

BMC Neurol 2022 Jun 17;22(1):222. Epub 2022 Jun 17.

Department of Rheumatology and Immunology, Tianjin Medical University General Hospital, No.154 Anshan Road, Tianjin, 300052, China.

Background: Systemic lupus erythematosus (SLE) is a chronic multisystem autoimmune inflammatory disease predominantly found in women of child-bearing age. Neurogenic pulmonary edema (NPE) is a recalcitrant complication that occurs after injury to the central nervous system and has an acute onset and rapid progression. Limbic encephalitis is an inflammatory encephalopathy caused by viruses, immune responses, or other factors involving the limbic system. Read More

View Article and Full-Text PDF

Long non-coding RNA growth arrest specific 5 regulates the T helper 17/regulatory T balance by targeting miR-23a in myasthenia gravis.

J Int Med Res 2022 Jun;50(6):3000605211053703

Department of Emergency, The Second Affiliated Hospital of Fujian Medical University, Quanzhou City, Fujian Province, P. R. China.

Objective: Myasthenia gravis (MG) is a chronic autoimmune neuromuscular disorder. Recent studies report that long non-coding RNAs (lncRNAs) play vital roles in the pathogenesis of various diseases. This study explored the molecular mechanism of lncRNA growth arrest specific 5 (GAS5) in regulating the T helper 17 (Th17)/regulatory T (Treg) cell balance in MG. Read More

View Article and Full-Text PDF

Decreased salivary α-amylase activity responding to citric acid stimulation in Myasthenia gravis with malnutrition.

PLoS One 2022 15;17(6):e0269621. Epub 2022 Jun 15.

Science and Technology Innovation Center, Guangzhou University of Chinese Medicine, Guangzhou, PR China.

Objectives: Malnutrition, defined according to Nutritional risk screening (NRS 2002), is commonly observed in patients of Myasthenia gravis (MG), a neuromuscular disorder manifested by varied degrees of skeletal muscle weakness. Because biochemical composition of saliva changes in correspondence to alterations in nutritional status, we tested our hypothesis that a certain saliva component(s) might serve as a biomarker(s) for nutrition status of MG, particularly for those MG patients with high risk of malnutrition.

Materials And Methods: 60 MG patients and 60 subjects belonging to the healthy control group (HCG) were enrolled in this case-control study. Read More

View Article and Full-Text PDF

Immune characteristics of children with autoimmune encephalitis and the correlation with a short-term prognosis.

Ital J Pediatr 2022 Jun 13;48(1):94. Epub 2022 Jun 13.

Tianjin Children's Hospital (Tianjin University Children's Hospital), Tianjin, 300134, China.

Background: Autoimmune encephalitis (AE) is a type of encephalopathy mediated by an antigenic immune response in the central nervous system. Most research related to autoimmune encephalitis (AE) is focused on early diagnosis, treatment and prognosis analysis; there has been little research conducted on the characteristics of immune function, and the relationship between immune function and prognoses of patients with autoimmune encephalitis needs to be studied further.

Methods: A total of 33 children with autoimmune encephalitis were identified through the clinic database and inpatient consults at Tianjin Children's Hospital from January 2013 to January 2021. Read More

View Article and Full-Text PDF

[Clinical and paraclinical profile of autoimmune myasthenia gravis in Ouagadougou, Burkina Faso].

Med Trop Sante Int 2021 12 11;1(4). Epub 2021 Nov 11.

CHU Yalgado Ouédraogo de Ouagadougou, Unité de formation et de recherches des sciences de la santé, Université Joseph Ki-Zerbo, Ouagadougou, Burkina Faso.

Introduction: Certain differences in the epidemiological, clinical, paraclinical and evolution profiles of autoimmune myasthenia gravis (AIMG) are increasingly described in patients according to geographic origins. The present study was carried out in order to help characterize the socio-demographic, clinical and paraclinical profile of AIMG in Ouagadougou, Burkina Faso.

Patients And Methods: This was a cross-sectional, descriptive, multicenter, hospital study carried out in Ouagadougou (Burkina Faso), over a period of 5 years 6 months, from March 2015 to September 2019. Read More

View Article and Full-Text PDF
December 2021

[Clinical analysis of 11 cases of lymphoma complicated with paraneoplastic neurological syndrome].

Zhonghua Xue Ye Xue Za Zhi 2022 Apr;43(4):311-315

Department of Hematology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing 100730, China.

To evaluate the clinical characteristics, treatment, and prognosis of patients with paraneoplastic neurological syndrome (PNS) associated with lymphoma. Between January 2012 and May 2021, the clinical data of 11 patients with lymphoma complicated with PNS treated at Peking Union Medical College Hospital were retrospectively reviewed. Among the 11 patients (8 male and 3 female) , the median onset age was 61 (range, 33-78) years. Read More

View Article and Full-Text PDF

Understanding and management of anti-N-methyl-D-aspartate receptor encephalitis from a child psychiatry perspective: report of five cases.

Neurocase 2022 Apr 7;28(2):239-245. Epub 2022 Jun 7.

Department of Child Neurology, Faculty of Medicine, Ege University, Izmir, Turkey.

Anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis is a rare autoimmune entity in psychiatry literature that occurs when antibodies attack NMDA-type glutamate receptors in the brain. Principle clinical features include a neurological domain such as seizure, orofacial dyskinesia, dystonia, and choreic-like movements of extremities. Also the psychiatric manifestations of this form of encephalitis may vary from psychotic-like symptoms to mood symptoms like depression or mania. Read More

View Article and Full-Text PDF

Multiple autoimmune disorders in a patient with neuromyelitis optica spectrum disorder presenting with rhabdomyolysis.

BMJ Case Rep 2022 Jun 7;15(6). Epub 2022 Jun 7.

Internal Medicine, PGIMER, Chandigarh, India

Neuromyelitis optica spectrum disorder (NMOSD) is a demyelinating disease of the central nervous system characterised by longitudinal extensive transverse myelitis and involvement of the optic nerve and is associated with many autoimmune disorders. The index case, a known case of Hashimoto's thyroiditis, presented with quadriparesis and tea-coloured urine. Investigations revealed ongoing rhabdomyolysis related to autoimmune myositis and autoimmune haemolytic anaemia leading to pigment-induced acute kidney injury. Read More

View Article and Full-Text PDF

Giant cell myositis associated with metastatic thymoma and granulomatous hypercalcaemia.

BMJ Case Rep 2022 Jun 6;15(6). Epub 2022 Jun 6.

Department of Medicine, University of California San Francisco, San Francisco, California, USA

Giant cell myositis (GCM) is a rare inflammatory myopathy associated with myasthenia gravis and thymoma. Here, we report on a woman in her late 50s with a history of myasthenia gravis, systemic lupus erythematosus and stage IV thymoma with pleural metastases, who presented with proximal weakness, neuromuscular respiratory failure and hypercalcaemia. She was diagnosed with GCM via muscle biopsy and screened for myocarditis but showed no evidence of myocardial involvement. Read More

View Article and Full-Text PDF

Case Report: Neuromyelitis Optica Spectrum Disorder With Progressive Elevation of Cerebrospinal Fluid Cell Count and Protein Level Mimicking Infectious Meningomyelitis: A Diagnostic Challenge.

Front Immunol 2022 19;13:864664. Epub 2022 May 19.

Department of Neurology, Tongde Hospital of Zhejiang Province, Hangzhou, China.

Neuromyelitis optica spectrum disorder (NMOSD) is an autoimmune-mediated idiopathic inflammatory demyelinating disease with a typical clinical presentation of optic neuritis, acute myelitis, and area postrema syndrome. Most NMOSD patients are seropositive for disease-specific and pathogenic aquaporin-4 (AQP4) antibodies, which are key markers for the NMOSD diagnosis. Herein, we report an atypical case of a 41-year-old man who complained of intractable hiccups and vomiting at disease onset, followed by fever, headache, back pain, progressive paresthesia, and weakness of extremities later on. Read More

View Article and Full-Text PDF

DCAF12 and HSPA1A May Serve as Potential Diagnostic Biomarkers for Myasthenia Gravis.

Biomed Res Int 2022 24;2022:8587273. Epub 2022 May 24.

Department of Neurology, First Affiliated Hospital, Guangxi Medical University, Nanning, China 530021.

Background: Myasthenia gravis (MG) is an autoimmune disease that severely affects the life quality of patients. This study explores the differences in immune cell types between MG and healthy control and the role of immune-related genes in the diagnosis of MG.

Methods: The GSE85452 dataset was downloaded from the Gene Expression Omnibus (GEO) database and analyzed using the limma package to determine differentially expressed genes (DEGs) between patients with MG and the control group. Read More

View Article and Full-Text PDF

Ovarian Teratoma-Related Paraneoplastic Neurological Syndromes.

Front Oncol 2022 16;12:892539. Epub 2022 May 16.

Department of Neurology, West China Hospital, Sichuan University, Chengdu, China.

Paraneoplastic neurological syndromes (PNSs) are a group of neurological disorders triggered by an underlying remote tumor. Ovarian teratoma (OT) is the most common histologic type of germ cell tumor in females. The most common PNSs associated with OT is anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis. Read More

View Article and Full-Text PDF

Autoimmune Encephalitis in Children.

Duriel Hardy

Pediatr Neurol 2022 Jul 13;132:56-66. Epub 2022 May 13.

Department of Neurology, Dell Medical School, University of Texas at Austin, Austin, Texas. Electronic address:

Autoimmune encephalitis is a group of central nervous system (CNS) inflammatory disorders that most commonly affect young adults and children. These disorders are closely associated with antibodies against neuronal cell-surface proteins, receptors, and ion channels; however, some forms of the disorder have no known antibody at this time. In children, neurological manifestations such as seizure, movement disorders, and focal neurological deficits are more prominent at initial presentation than psychiatric or behavioral symptoms. Read More

View Article and Full-Text PDF

Pharmacotherapy of Generalized Myasthenia Gravis with Special Emphasis on Newer Biologicals.

Drugs 2022 Jun 31;82(8):865-887. Epub 2022 May 31.

Ellen and Martin Prosserman Centre for Neuromuscular Diseases, University Health Network, 5EC-309, Toronto General Hospital, University of Toronto, 200 Elizabeth St, Toronto, M5G 2C4, Canada.

Myasthenia gravis (MG) is a chronic, fluctuating, antibody-mediated autoimmune disorder directed against the post-synaptic neuromuscular junctions of skeletal muscles, resulting in a wide spectrum of manifestations ranging from mild to potentially fatal. Given its unique natural course, designing an ideal trial design for MG has been wrought with difficulties and evidence in favour of several of the conventional agents is weak as per current standards. Despite this, acetylcholinesterases and corticosteroids have remained the cornerstones of treatment for several decades with intravenous immunoglobulins (IVIG) and therapeutic plasma exchange (PLEX) offering rapid treatment response, especially in crises. Read More

View Article and Full-Text PDF

Relapsing MOG-IgG-associated diseases coexisting with anti-NMDAR encephalitis: a case report and literature review.

J Integr Neurosci 2022 Apr;21(3):82

Department of Neurology, The Second Hospital of Dalian Medical University, 116027 Dalian, Liaoning, China.

Background: In recent years, an overlapping syndrome, MNOS, of MOG encephalomyelitis and NMDARE has been clinically identified. In these diseases, both MOG-Ab and NMDAR-Ab are positive. Previous studies were almost case reports and incomprehensive which focused on this kind of overlapping syndrome in adults. Read More

View Article and Full-Text PDF

Intrapericardial Thymoma Presented as Pericardial Tamponade with Post-Operative Myasthenia Gravis.

Medicina (Kaunas) 2022 Apr 27;58(5). Epub 2022 Apr 27.

Division of Thoracic Surgery, Department of Surgery, National Defense Medical Center, Tri-Service General Hospital, Taipei 11490, Taiwan.

Background: Thymoma is an epithelial mass arising from the thymus. Most thymomas are located in the anterior mediastinum. Ectopic intrapericardial thymoma is very unusual; to date, only eight cases of pericardial thymoma have been reported. Read More

View Article and Full-Text PDF

Increased serum IL-2, IL-4, IL-5 and IL-12p70 levels in AChR subtype generalized myasthenia gravis.

BMC Immunol 2022 May 27;23(1):26. Epub 2022 May 27.

Huashan Rare Disease Center and Department of Neurology, Huashan Hospital Fudan University, No. 12 Middle Wulumuqi Road, Shanghai, 200040, China.

Background: Myasthenia gravis (MG) is an autoimmune disorder affecting neuromuscular junctions. Cytokines play important roles in facilitating the immune response and augmenting the pathogenic antibody production. The current study aims to sensitively characterize the serum levels of cytokines with very low concentration in generalized MG (gMG). Read More

View Article and Full-Text PDF

A Review of Disease Mechanisms and Current and Emerging Treatment Options for Generalized Myasthenia Gravis.

Prim Care Companion CNS Disord 2022 May 24;24(3). Epub 2022 May 24.

eneralized myasthenia gravis (gMG) is a disease resulting from impaired neuromuscular transmission due to presence of antibodies that block acetylcholine receptors. Autoantibodies to acetylcholine receptors directly impair the activity of ion channels that conduct nerve impulses, and cross-link acetylcholine receptors resulting in complement-mediated destruction, further worsening functional impairment and patient quality of life. Although current treatments for gMG include thymectomy, immunosuppressive therapies, intravenous immunoglobulins, and plasmapheresis, among other strategies, none of these treatments reduces all immunoglobulin G subfractions. Read More

View Article and Full-Text PDF

LRP4-IgG service line testing in seronegative myasthenia gravis and controls.

J Neuroimmunol 2022 Jul 18;368:577895. Epub 2022 May 18.

Department of Laboratory Medicine and Pathology, Mayo Clinic Foundation, Rochester, MN, USA. Electronic address:

Background: LRP4 is a post-synaptic membrane protein that promotes acetylcholine (AChR) clustering on the crest of post-synaptic neuromuscular folds. Autoantibodies against LRP4 are suggested to account for myasthenia gravis (MG) patients negative for antibodies to AChR.

Objectives: To report a clinical experience with service-line LRP4-IgG cell-based testing in electrodiagnostically confirmed MG patients and controls. Read More

View Article and Full-Text PDF

Paraneoplastic limbic encephalitis following treatment with single-agent pembrolizumab for advanced gastroesophageal adenocarcinoma.

BMJ Case Rep 2022 May 24;15(5). Epub 2022 May 24.

Department of Hematology/Oncology, Fox Chase Cancer Center, Philadelphia, Pennsylvania, USA

The use of immune checkpoint inhibitors is increasing in clinical practice. While they have provided significant benefit to many patients, a new category of adverse effects, immune-related adverse effects, has emerged with their use. These effects can range from mild to severe and affect nearly every organ system. Read More

View Article and Full-Text PDF

Academic and Employment Status in Patients With Generalized Myasthenia Gravis Treated With Eculizumab: A Case Series.

J Clin Neuromuscul Dis 2022 Jun;23(4):210-218

Department of Neurology, University of Missouri Health Care, Columbia, MO.

Objectives: To evaluate the impact of treatment with eculizumab, a terminal complement inhibitor, on academic and employment status in patients with refractory generalized myasthenia gravis (MG).

Methods: Case review of 7 US patients.

Results: Six patients were aged ≤65 years; one was a full-time student and the remainder were in employment before MG diagnosis. Read More

View Article and Full-Text PDF

What is in the Neuromuscular Junction Literature?

J Clin Neuromuscul Dis 2022 Jun;23(4):189-200

Departments of Neurology and.

Abstract: This update covers a number of treatment topics starting with Fc receptor inhibitors and the Federal Drug Administration approval of efgartigimod. Some uncertainties regarding the use of corticosteroids are addressed, namely the risk of exacerbation with initiation of treatment and how to taper. The presence and potential importance of antibody overshoot following plasmapheresis is noted and the evolving increase in usefulness of acetylcholine receptor antibodies in diagnosing ocular myasthenia. Read More

View Article and Full-Text PDF

Myxomatous tumours of the right atrium in a patient with recurring myasthenia gravis exacerbations.

BMJ Case Rep 2022 05 20;15(5). Epub 2022 May 20.

Internal Medicine, Albany Medical Center Hospital, Albany, New York, USA.

View Article and Full-Text PDF

A Case of a Splitting Headache: Paraneoplastic Rhombencephalitis.

Cureus 2022 Apr 20;14(4):e24302. Epub 2022 Apr 20.

Hematology and Medical Oncology, Mission Cancer + Blood, Des Moines, USA.

Paraneoplastic neurologic syndromes are a set of rare neurological conditions with a wide variety of presentations, ranging from headache to gait imbalance. These conditions are often underreported and underdiagnosed. Paraneoplastic rhombencephalitis is a subtype that involves inflammation of the hindbrain. Read More

View Article and Full-Text PDF