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Endogenous Endophthalmitis and Subretinal Abscess Following Rituximab Therapy in Myasthenia Gravis: A Report of Two Cases.

PURPOSE: Endogenous endophthalmitis is a serious vision-threatening condition commonly associated with immunosuppression and is invariably linked to a systemic focus. Rituximab infusion is one of the treatment options for refractory Myasthenia Gravis but can become a double-edged sword, increasing susceptibility to serious infections. This study reports the occurrence and management of Endogenous endophthalmitis in two patients after rituximab infusion given for refractory Myasthenia Gravis. Endogenous endophthalmitis post Rituximab has not been reported. METHOD: A retrospective review of pat…

Inpatient Utilization of Therapeutic Plasma Exchange in the United States: 2016-2021.

RATIONALE & OBJECTIVE: Therapeutic plasma exchange (TPE) is used in a variety of autoimmune, inflammatory, hematological, and metabolic diso … f the total hospitalizations) were treated by TPE. The 5 most common conditions treated by TPE were myasthenia gravis with (acute) exacerbation (9.53%), Guillain-Barre syndrome (6.19%), thrombotic microangiopathy (6.18%), kidney transplant rejection (5.42%), and sepsis, unspecified organism (3.62%). AKI was the 13th most common condition, accounting f …

Sepsis complicated by haemophagocytic lymphohistiocytosis triggered by methicillin-resistant Staphylococcus aureus and human herpesvirus 8 in an immunocompromised elderly patient: A case report.

Sepsis is a life-threatening organ dysfunction caused by a dysregulated host response to infection. A rare but severe complication is haemophagocytic lymphohistiocytosis (HLH), an aggressive syndrome of excessive immune activation. Managing both conditions is particularly challenging in patients with multiple comorbidities. A 78-year-old male with a complex history, including myasthenia gravis and chronic kidney disease, was admitted with sepsis. Investigations confirmed infection with methicillin-resistant Staphylococcus aureus and human herpesvirus 8. He was subsequently diagnosed with HLH b…

Adverse drug events (ADEs) risk signal mining related to eculizumab based on the FARES database.

INTRODUCTION: Eculizumab is a C5 complement inhibitor approved by the FDA for the targeted treatment of four rare diseases, paroxysmal nocturnal hemoglobinuria (PNH), atypical hemolytic uremic syndrome (aHUS), generalized myasthenia gravis (gMG), and aquaporin-4 immunoglobulin G-positive optic neuromyelitis optica spectrum disorders (AQP4-IgG+NMOSD). The current study was conducted to assess real-world adverse events (AEs) associated with eculizumab through data mining of the FDA Adverse Event Reporting System (FAERS). METHODS: Disproportionality analyses, including Reporting Ratio Ratio (ROR…

Long-Term Efficacy and Safety of Ravulizumab in Adults With Anti-Acetylcholine Receptor Antibody-Positive Generalized Myasthenia Gravis: Final Results From the Phase 3 CHAMPION MG Open-Label Extension.

BACKGROUND: Ravulizumab, an anti-complement C5 monoclonal antibody, was efficacious with acceptable safety in the randomized controlled period (RCP) and interim open-label extension (OLE) periods of the CHAMPION MG phase 3 trial in adults with anti-acetylcholine receptor antibody-positive (AChR-Ab+) generalized myasthenia gravis (gMG). Here, we report final results from the OLE. METHODS: Patients who completed the 26-week RCP could enter the OLE and receive ravulizumab for up to 4 years. Efficacy and safety were assessed throughout the OLE. RESULTS: Among all ravulizumab-treated patients (n …

Emergent role of complement inhibitors in myasthenic crisis: Understanding why, when and how.

We report the case of a 32-year-old woman with an 11-year history of thymomatous AChR antibody-positive generalized myasthenia gravis (gMG, MGFA IIIb) who experienced a myasthenic crisis (MC) during the postpartum period, requiring mechanical ventilation (MV). Initial treatment with therapeutic plasma exchange (TPE) and high-dose prednisone was ineffective, and pyridostigmine worsened airway secretions. Due to contraindications for IVIg, she was treated with the complement inhibitor eculizumab, following prophylactic meningococcal vaccination and antibiotic therapy. Six days after starting ecu…

First analysis of the Myasthenia Gravis SPOTLIGHT Registry: outcomes with eculizumab and ravulizumab.

BACKGROUND: Complement component 5 inhibitors are indicated for the treatment of anti-acetylcholine receptor antibody-positive generalized myasthenia gravis (gMG). Clinical trials have demonstrated improved functional ability, muscle strength, and quality of life (QOL) in patients treated with eculizumab or ravulizumab. Evidence for their effectiveness and safety in clinical practice is reported here. METHODS: Data from the global MG SPOTLIGHT Registry were collected from patients with gMG currently or previously treated with eculizumab or ravulizumab in routine clinical practice. Effectivene…

Ravulizumab in the Management of Refractory Myasthenic Crisis: Clinical and Ventilatory Evidence of Early Recovery-A Case Report.

INTRODUCTION: Generalized myasthenia gravis (gMG) is an autoimmune disorder impairing neuromuscular transmission, most commonly through anti-AChR antibodies that activate the complement cascade. This can lead to severe complications such as myasthenic crisis (MC), which often requires intensive care. While plasma exchange (PLEX) and intravenous immunoglobulins (IVIG) are standard first-line therapies, approximately 30% of patients may show suboptimal response. Ravulizumab, a long-acting C5 complement inhibitor, has been approved for anti-AChR-positive gMG, but data on its use in MC remain limi…

True Thymic Hyperplasia: An Extremely Rare Incidental Finding.

Thymic hyperplasia is a rare and benign cause of mass in the anterior mediastinum in adults. This pathology is characterized by an increase … sent histologically with lymphoid follicles and is not associated with autoimmune diseases, such as myasthenia gravis and Graves' disease. Here we present the case of an adult patient with thymic hyperplasia incidentally diagnosed in imaging exams. The established treatment was complete surgical resection, which is indicated as the first-line treatmen …

Botulism, Lambert-Eaton Myasthenic Syndrome, and Congenital Myasthenic Syndromes.

OBJECTIVE: This article covers the clinical presentations, investigations, differential diagnosis, and principles of management of botulism, Lambert-Eaton myasthenic syndrome (LEMS), and congenital myasthenic syndromes. LATEST DEVELOPMENTS: Well-recognized guidelines exist for the management of botulism and LEMS, but resource limitations may affect implementation globally. Several genes recently reported to cause congenital myasthenic syndromes are involved in ubiquitously expressed proteins and present with a complex non-neuromuscular junction phenotype, wherein abnormal neuromuscular juncti…

A Hospitalist's Challenge: Systemic Botulism Following a Cosmetic Injection Requiring Antitoxin Therapy and Percutaneous Endoscopic Gastrostomy (PEG).

Botulinum toxin type A (BoNT-A) is widely used in cosmetic practice and generally considered safe, with adverse effects usually limited to t … ections to the face and masseter muscles. Imaging and endoscopic evaluations were unremarkable, and myasthenia gravis (MG) was excluded. Despite initial partial improvement on pyridostigmine, her bulbar dysfunction worsened, necessitating administration of botulinum antitoxin and eventual percutaneous endoscopic gastrostomy (PEG) placement for nutriti …

Iatrogenic Botulism Following Cosmetic Botulinum Toxin Injection Presenting With Respiratory Distress: A Case Report.

Botulinum toxin (BoNT) is extensively used across a wide range of therapeutic and cosmetic applications due to its efficacy in inhibiting ne … iagnostic challenges, as the early clinical features mimicked other neuromuscular disorders such as myasthenia gravis or brainstem stroke. Despite this, prompt recognition based on clinical suspicion led to the timely administration of heptavalent botulinum antitoxin, which was followed by gradual neurological improvement. This case underscores the im …

Аутоиммунные заболевания и беременность.

Цель этого исследования состоит в том, чтобы описать воздействие некоторых наиболее распространенных аутоиммунных заболеваний, таких, как множественный склероз (MS) и расстройства спектрального спектра нейромиелита (NMOSD), миастения гравис (MG), хроническое воспалительное заболевание кишечника (IBD), диабет типа 1 (T1D), аутоиммунное заболевание щитовидной железы, ревматоидный артрит (RA), системный волшебный эритематоз (SLE), псориаз, васкулит и антифосфолипидный синдром (APS), на беременность женщин. В рамках этого обзора изучается риск для потомства женщин, т.е.

Совпадение в аннотации оригинала
This study aims to describe the impact of some of the most common autoimmune diseases, such as multiple sclerosis (MS) and neuromyelitis optica spectrum disorders (NMOSD), myasthenia gravis (MG), chronic inflammatory bowel disease (IBD), type 1 diabetes (T1D), autoimmune thyroid disease, rheumatoid arthritis (RA), systemic lupus erythematosus (SLE), psoriasi…

Влияние регулирования разбрызгивания на нервно-мышечные расстройства и текущую нейромускульную генетическую терапию.

Эукариотические гены содержат некодирующие сегменты, известные как интроны, которые прерывают последовательности кодирования. Таким образом, эукариотическая транскрипция производит прекурсор-прекурсор РНК (до-мРНК), который основывается на точной скрещивании для удаления весьма разнообразных интронов из генома и создания зрелого mRNA, необходимого для поддержания нормальной клеточной активности. Широкая неоднородность нейронов требует сложного регулирования сплетения, особенно альтернативного сплечения, для обеспечения точности генного выражения в нейрогенессе, трансдукции сигналов и синаптиче

Совпадение в аннотации оригинала
Eukaryotic genes contain non-coding segments known as introns, which interrupt coding sequences. Consequently, eukaryotic transcription prod … LS), Duchenne muscular dystrophy (DMD), myotonic dystrophy (DM), Charcot-Marie-Tooth disease (CMT), myasthenia gravis (MG), and multiple sclerosis (MS). Studying the correlation between splicing defects and neuromuscular disorders is crucial for gaining a more comprehensive understanding of the pathogenesis of these diseases and for developing effecti …

[Treatment of neuroimmunological diseases with chimeric antigen receptor (CAR) T cells : Experience and new developments].

BACKGROUND: Chimeric antigen receptor (CAR) T cell therapies were originally developed for the treatment of hematological malignancies; howe … euroimmunological diseases ranges from classical autoantibody-mediated autoimmune diseases, such as myasthenia gravis to immunologically complex conditions like multiple sclerosis. The CAR T cell products currently in use target B cells, leading to complete B cell depletion, including autoreactive B cell clones. The therapeutic response, measured by d …

The number of apheresis procedures to treat immune-mediated neurological diseases is on the rise.

Apheresis is used for the treatment of many different diseases, especially when conventional therapy lacks efficacy. There are however some … 001) while the overall apheresis procedures remained stable (p = 0.46). Most procedures were due to myasthenia gravis (MG; n = 11,049 (31 % of patients), Guillain-Barré Syndrome (GBS; n = 3247 (30 %), multiple sclerosis (MS; n = 2665 (18 %)), chronic inflammatory demyelinating polyradiculoneuropathy (CIDP; n = 2367 (3 %)), and neuromyelitis optica (NM …

Изучение связи между аутоиммунными неврологическими заболеваниями и психическими расстройствами: данные исследования менделийской случайности.

ВВЕДЕНИЕ: Традиционные эпидемиологические исследования свидетельствуют о том, что аутоиммунные неврологические заболевания могут быть связаны с психическими расстройствами. Мы использовали исследования менделийской случайности (MR) для изучения причинно-следственной связи между аутоиммунными неврологическими заболеваниями и MD с генетической точки зрения. МЕТОДЫ: В нашем исследовании аутоиммунные неврологические заболевания включают в себя множественный склероз (MS), оптику нейромиелита (NMO) и гравис миастении (MG); в число ДИ входят тревожное состояние, серьезное депрессивное расстройство (M

Совпадение в аннотации оригинала
INTRODUCTION: Traditional epidemiologic studies suggest that autoimmune neurologic diseases may be associated with mental disorders (MDs). W … dy, autoimmune neurologic diseases include multiple sclerosis (MS), neuromyelitis optica (NMO), and myasthenia gravis (MG); MDs include anxiety, major depressive disorder (MDD), attention deficit/hyperactivity disorder (ADHD), schizophrenia, and bipolar affective disorder (BIP). A two-sample MR approach was used to explore causal relationships. The in …

Immune checkpoint inhibitor-associated autoimmune encephalitis and other neurological immune-mediated adverse events: a pharmacovigilance study using the FAERS and JADER.

BACKGROUND: Immune checkpoint inhibitor (ICI)-associated neurological immune-related adverse events (NAEs) are rare but serious side effects … etected in all other preferred terms (PTs). These NAEs accounted for 23.7% of total mortality, with myasthenia gravis (MG) exhibiting the highest mortality rate at 30.63%. Specific PTs, such as aseptic meningitis, AIE, chronic inflammatory demyelinating polyradiculoneuropathy, Guillain-Barré syndrome, MG, myelitis, and immune-related myopathy, were as …

CAR T-клетки сталкиваются с аутоиммунными неврологическими заболеваниями: новый рассвет для терапии.

Аутоиммунный иммунитет и аутоиммунные заболевания возникают тогда, когда иммунная система ошибочно нацеливается на самоантигены, ведущие к п … ми заболеваниями, особенно те, которые влияют на нервную систему, такие, как Множественный склероз, Myastenia gravis, оптица нейромиелита, синдром стифф-лиц, аутоиммунный энцефалит, болезнь MOG-антибиоза и хроническое воспалительное демиелинирование полинеропропаты. Кроме того, на основе анализа доклинических и клинических данных мы будем оценивать эф …

Potential Causal Relationships Between Circulating Micronutrient Levels and Multiple Neuroimmune Diseases: A Genetic Association Analysis.

BACKGROUND: Growing evidence suggests a close association between circulating micronutrient levels and neuroimmune diseases. Nevertheless, t … myelitis (ADEM), acute poliomyelitis (AP), sequelae of poliomyelitis (SP), optic neuritis (ON), and myasthenia gravis (MG). Data on these seven neuroimmune diseases came from the FinnGen database and included 5523 cases and 2,860,006 controls. The inverse variance weighting (IVW) method was used as the main MR analysis method, and sensitivity analysis …