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Diagnosis and Treatment of Paraneoplastic Neurologic Syndromes.

Paraneoplastic antibody syndromes result from the anti-tumor antibody response against normal antigens ectopically expressed by tumor cells. Although this antibody response plays an important role in helping clear a nascent or established tumor, the engagement of antigens expressed in healthy tissues can lead to complex clinical syndromes with challenging diagnosis and management. The majority of known paraneoplastic antibody syndromes have been found to affect the central and peripheral nervous system. The present review provides an update on the pathophysiology of paraneoplastic neurologic s

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… Syndromes Affecting the Neuromuscular Junction (NMJ) 3.3.1. … Diseases of the neuromuscular junction, such as LEMS or myasthenia gravis, do not involve loss of the parent neuron and thus can recover its function once the causal insult (the pathogenic autoantibodies) has been removed, either directly through immunosuppression or indirectly through the treatment …

BDNF in the Aged Brain: Translational Implications for Parkinson's Disease.

The International Diabetes Federation (Europe) has updated these guidelines on hypertension management specifically in Type 2 diabetes in the light of recent results of the first prospective, randomized controlled studies to investigate clinical outcomes in people with diabetes and hypertension. The guidelines are knowledge based, i.e. based not only on evidence originating from clinical trials, but also from epidemiological and pathophysiological studies. A successful management strategy requires the following components: 1. Regular surveillance to detect developing hypertension and other car

Микробные профили пациентов с оптическим неуритом или миастенией гравис.

ЦЕЛЬ: Характеристика кишечного микробного состава пациентов с оптическим невритом (ON) или миастенией грависа (MG). МЕТОДЫ: Пробы стула были собраны у 45 пациентов с НО, 13 пациентов с MG и 20 здоровых методов контроля. Микробиальная геномная ДНК была извлечена, и V3-V4 области бактериальных генов 16S rRNA были расширены и секвенированы. Для сопоставления альфа-разнообразия, бета-разнообразия, таксономических задач и бактериального богатства групп был проведен биоинформатический анализ. Различия в изобилии микробных таксов были выявлены с использованием линейного анализа эффекта дискриминантов

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… Introduction Optic neuritis (ON) and myasthenia gravis (MG) are neuroimmune disorders that affect the optic nerve and the neuromuscular junction, respectively, and have distinct clinical manifestations. … ON and MG, despite affecting different anatomical sites (ON affects the optic nerve and MG affects the neuromuscular junction), are both immune-mediated neurologic disorders, and therefore there is a rationale for a comparative analysis to be performed. …

A Practical Approach to Managing Patients With Myasthenia Gravis-Opinions and a Review of the Literature.

When the diagnosis of myasthenia gravis (MG) has been secured, the aim of management should be prompt symptom control and the induction of remission or minimal manifestations. Symptom control, with acetylcholinesterase inhibitors such as pyridostigmine, is commonly employed. This may be sufficient in mild disease. There is no single universally accepted treatment regimen. Corticosteroids are the mainstay of immunosuppressive treatment in patients with more than mild MG to induce remission. Immunosuppressive therapies, such as azathioprine are prescribed in addition to but sometimes instead of

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… Introduction Myasthenia gravis (MG) is a rare acquired autoimmune disorder of the neuromuscular junction (NMJ), caused by antibodies that target the post-synaptic membrane ( 1 ). … This is an acetylcholinesterase inhibitor which blocks the degradation of acetylcholine at peripheral cholinergic synapses, including the neuromuscular junction (NMJ). …

Principles of Therapeutic Apheresis in Neurological Disease.

BackgroundTherapeutic plasma exchange (TPE) is a well-known apheresis technology since many years and is available worldwide. Myasthenia gravis is one of the first neurological diseases successfully treated with TPE. TPE is also frequently applied in acute inflammatory demyelinating polyradiculoneuropathy (Guillain-Barré syndrome). Both neurological disorders are immunologically mediated and might cause life-threatening symptoms in patients.SummaryThere is a large body of evidence from many randomized controlled trials (RCTs) that the application of TPE in myasthenia gravis crisis or in acute

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… Myasthenia Gravis MG, one of the first neurological diseases successfully treated with TPE, is a rare autoimmune disease characterized by a disturbance of the postsynaptic neuromuscular junction. … Key neuromuscular junction protein directed autoantibodies which disrupt the neuromuscular transmission cause grave weakness typically found in MG. 85% of patients with MG have anti-acetylcholine-receptor antibodies which deplete the ACh-receptor (ACh-R) function. …

Изменения лакта и лактилации в неврологических расстройствах.

Значительное внимание стало уделяться изучению изменений в лактилации различных целевых органов как в области здравоохранения, так и в области болезней. Многие жизненно важные процессы и начало болезней связаны не только с избытком белков, но и в первую очередь регулируются различными посттрансляционными изменениями белков. Лактат, который раньше считался лишь побочным продуктом анаэробного метаболизма, превратился в важнейшую энергетическую субстрату и сигнализирующую молекулу, связанную как с физиологическими, так и патологическими процессами в нервной системе. Кроме того, в недавних исследо

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… Myasthenia gravis MG is an autoimmune disease characterized by dysfunction at the neuromuscular junction, which causes the body’s immune system to attack acetylcholine receptors. … In the context of MG, this review explores the impact of lactylation on protein function at the neuromuscular junction, offering new insights into the understanding of the neural signal transmission issues associated with the disease. …

Therapeutic Plasma Exchange: For Cancer Patients.

Therapeutic plasma exchange is used as a trial method for the treatment of cancer patients. Therapeutic plasma exchange uses in vitro technology to remove pathogenic factors in the plasma, returning the replacement and remaining components to the patient to facilitate cure. In the effort to explore new methods of cancer treatment, the introduction of therapeutic plasma exchange brings new hope for cancer treatment; however, the current evidence supporting therapeutic plasma exchange is controversial, and most of the evidence comes from observational studies, lacking large prospective randomize

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… junction and belongs to the paraneoplastic syndromes. … Myasthenia gravis and the neuromuscular junction . Curr Opin Neurol . 2013 ; 26 ( 5 ): 459 – 465 . doi: 10.1097/WCO.0b013e328364c079 23945282 66. Gilhus NE , Verschuuren JJ . Myasthenia gravis: subgroup classification and therapeutic strategies . …

Novel Immunotherapies for Myasthenia Gravis.

Myasthenia gravis (MG), a prototype autoimmune neurological disease, had its therapy centred on corticosteroids, non-steroidal broad-spectrum immunotherapy and cholinesterase inhibitors for several decades. Treatment-refractory MG and long-term toxicities of the medications have been major concerns with the conventional therapies. Advances in the immunology and pathogenesis of MG have ushered in an era of newer therapies which are more specific and efficacious. Complement inhibitors and neonatal Fc receptor blockers target disease-specific pathogenic mechanisms linked to myasthenia and have pr

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… Introduction and Background Introduction Myasthenia gravis (MG) is a chronic autoimmune disease of the neuromuscular junction (NMJ) clinically characterized by fatigable weakness of the ocular, limb and bulbar (speech, swallowing and respiratory) muscles. … The neuromuscular junction in health and disease: molecular mechanisms governing synaptic formation and homeostasis. Front Mol Neurosci . 2020;13:610964. doi: 10.3389/fnmol.2020.610964 13. Borges LS, Richman DP. Muscle-specific kinase myasthenia gravis. …

Current and future applications of ultrasound imaging in peripheral nerve disorders.

Neuromuscular ultrasound (NMUS) is a rapidly evolving technique used in neuromuscular medicine to provide complimentary information to standard electrodiagnostic studies. NMUS provides a dynamic, real time assessment of anatomy which can alter both diagnostic and management pathways in peripheral nerve disorders. This review describes the current and future techniques used in NMUS and details the applications and developments in the diagnosis and monitoring of compressive, hereditary, immune-mediated and axonal peripheral nerve disorders, and motor neuron diseases. Technological advances have

PROCEEDINGS OF THE XVII CONGRESS OF THE ITALIAN ASSOCIATION OF MYOLOGY Siracusa, Italy May 31 - June 3, 2017.

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… Schoser (Munich, Germany) 10.10 - 10.30 Coffee break 10.30 - 11.30 WORKSHOP-3: "Neuromuscular junction disorders" Chairpersons: G. Antonini, M. Grandis 10.30 MUSK (Muscle Specific Kinase)-positive myasthenia. A. …

Thrombosis in Neuromuscular Medicine: Current Evidence, Unmet Needs, and Future Directions.

Venous thromboembolism (VTE), comprising deep vein thrombosis and pulmonary embolism, is an important but under-recognised complication in neuromuscular diseases. In adults, emerging epidemiological data suggests increased VTE occurrence in conditions such as Amyotrophic Lateral Sclerosis, myotonic dystrophy, myasthenia gravis, inflammatory neuropathies, inflammatory myopathies, and POEMS syndrome. This heightened risk reflects not only disease-related immobility but also disorder-specific biological mechanisms, including inflammation, endothelial dysfunction and cardiomyopathy-related stasis.

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… Myasthenia Gravis (MG) In myasthenia gravis, pathogenic antibodies attack the neuromuscular junction, causing an interruption in neuromuscular transmission resulting in clinical manifestations of fluctuating, fatigable weakness. …

Прогрессивная слюнная речь как нетипичное представление GDAP2-относительно Spinocerebellar Ataxia: доклад по делу.

47-летний мужчина с сахарным диабетом и дислипидемией за четыре года развил прогрессивную речь. МРТ не выявила атрофии мозга, и обычные лабораторные и нейрофизиологические исследования были непримечательными. У его брата были похожие, но продвинутые симптомы, включая дисфагию и серьезные походки, что привело к зависимости от инвалидных колясок. Полноэкзомная последовательность выявила вероятную патогенную гомозиговую мутацию в гене GDAP2, подтверждающую диагноз атаксии 27 (SCAR27). В этом докладе подчеркивается важность рассмотрения наследственных атаксий при дифференциальном диагностике прогр

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… Myasthenia gravis (neuromuscular junction disorder) can also be associated with dysarthria, which has diurnal variation. Additional considerations are metabolic causes, which include vitamin E deficiency, Wilson's disease, or thyroid dysfunction [ 4 ]. … There were no reported variations in predominantly diurnal symptoms, which is why neuromuscular junction disorders were less probable. The gait was maintained till the early stage, but slight instability was also observed later, especially when walking on uneven surfaces. …

Assessing Efgartigimod (VYVGART™) Social Media Posts in Generalized Myasthenia Gravis to Understand Patient, Caregiver, and Health Care Professional Sentiments.

INTRODUCTION: Myasthenia gravis (MG) is a chronic autoimmune neuromuscular disorder characterized by fluctuating muscle weakness (ocular, bulbar, limb, and respiratory). Efgartigimod (VYVGART), a first-in-class neonatal Fc receptor antagonist, was approved for treatment of generalized MG (gMG). This study aimed to explore real-world sentiments, wherein in sentiments refer to real-world perceptions expressed by patients, caregivers, and healthcare professionals (HCPs) on VYVGART and VYVGART Hytrulo, through structured social media analysis. METHODS: This social media listening study was design

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… MG is the most common disorder of the neuromuscular junction [ 2 ] with a prevalence of 10 to 20 cases per 100,000 population [ 2 ]. It predominantly affects younger women (20 to 40 years old) and older men [ 4 ]. …

Plasmapheresis in the ICU.

Therapeutic plasma exchange (TPE) is a treatment paradigm used to remove harmful molecules from the body. In short, it is a technique that employs a process that functions partially outside the body and involves the replacement of the patient's plasma. It has been used in the ICU for a number of different disease states, for some as a first-line treatment modality and for others as a type of salvage therapy. This paper provides a brief review of the principles, current applications, and potential future directions of TPE in critical care settings.

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… Myasthenia Gravis (MG) Myasthenia gravis is a chronic autoimmune disorder affecting neuromuscular junctions, leading to muscle weakness and fatigue. Plasmapheresis is a valuable strategy in the ICU. … Lambert–Eaton Myasthenic Syndrome (LEMS) LEMS is a rare autoimmune disorder characterized by muscle weakness and coordination difficulties resulting from an immune attack on calcium channels at neuromuscular junctions. …

Efficacy and Safety of Botulinum Toxin A and Pulsed Radiofrequency on Postherpetic Neuralgia: A Randomized Clinical Trial.

This study evaluated the effectiveness and safety of botulinum toxin type A (BoNT-A) and pulsed radiofrequency (RF) in the clinical treatment of postherpetic neuralgia (PHN). A total of 100 patients with PHN were randomly divided into two groups (n = 50 per group): RF group and BoNT-A group. Based on conventional drug treatment, patients were treated with either a single nerve root pulsed radiofrequency therapy or a single local subcutaneous injection of BoNT-A in the lesion area. All the patients were followed up for 24 weeks on pain scores, sleep quality, anxiety, and depression scores, etc.

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… The exclusion criteria were as follows: (1) there was a history or family history of neuromuscular junction disease; (2) drugs acting on the neuromuscular junction or aminoglycoside antibiotics had been used in the past 2 weeks; (3) there was a personal or family history of coagulation dysfunction; ( …

Acute pancreatitis with hypercalcemia caused by primary hyperparathyroidism associated with paraneoplastic syndrome: A case report and review of literature.

BACKGROUND: Although acute pancreatitis associated with hyperparathyroidism has occasionally been reported, acute pancreatitis with metabolic encephalopathy caused by hyperparathyroidism combined with paraneoplastic syndrome is an extremely rare entity and poorly described in the literature. CASE SUMMARY: We present a case of a 56-year-old female with upper abdominal discomfort and intermittent nausea and vomiting for 1 wk, without apparent abdominal pain or bloating, no jaundice and decreased blood pressure at the outset. The patient was ultimately diagnosed with moderately severe acute panc

Bortezomib Increased Vascular Permeability by Decreasing Cell-Cell Junction Molecules in Human Pulmonary Microvascular Endothelial Cells.

Bortezomib (BTZ), a chemotherapeutic drug used to treat multiple myeloma, induces life-threatening side effects, including severe pulmonary toxicity. However, the mechanisms underlying these effects remain unclear. The objectives of this study were to (1) investigate whether BTZ influences vascular permeability and (2) clarify the effect of BTZ on the expression of molecules associated with cell-cell junctions using human pulmonary microvascular endothelial cells in vitro. Clinically relevant concentrations of BTZ induced limited cytotoxicity and increased the permeability of human pulmonary m

Adult-onset hypophosphatemic osteomalacia as a cause of widespread musculoskeletal pain: A retrospective case series of single center experience.

BACKGROUND: Osteomalacia (OM) is frequently confused with various musculoskeletal or other rheumatic diseases, especially in patients with adult-onset widespread musculoskeletal pain because of its low prevalence and non-specific manifestations. AIM: To facilitate the early diagnosis and etiology-specific treatment of adult-onset hypophosphatemic OM. METHODS: A retrospective review of medical records was performed to screen adult patients who visited a physiatry locomotive medicine clinic (spine and musculoskeletal pain clinic) primarily presenting with widespread musculoskeletal pain at a s

Brazilian Academy of Neurology recommendations for diagnosis, management, and treatment of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP).

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an acquired autoimmune disorder that leads to progressive motor and sensory impairment, resulting in significant morbidity. While the incidence rates vary, CIDP remains a challenging condition requiring a standardized and optimized approach to diagnosis and management. In Brazil, a middle-income country with substantial regional disparities in healthcare access, the availability of specialized neuromuscular centers is uneven, creating obstacles to timely and effective treatment. To address these challenges, the Brazilian Acade

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… antibodies, antineutrophil cytoplasmic antibodies, Erythrocyte sedimentation rate, C-reactive protein, nerve biopsy for vasculitis Anti-MAG antibodies when IgM monoclonal gammopathy is present Motor deficit | Repetitive stimulation in low and/or high frequency and anti-VGCC or anti-ACHR or anti-Musk if neuromuscular … junction abnormalities Anti-GM1 IgM antibodies if multifocal motor neuropathy is suspected Creatine kinase, lactate and aldolase levels, myositis specific autoantibodies, muscle MRI, and muscle biopsy if inflammatory myopathy is suspected WES for genetic neuropathies, myopathies, and neuromuscular junction …

Neurologic adverse events of cancer immunotherapy.

Cancer immunotherapy encompasses a wide range of treatment modalities that harness the anti-tumor effects of the immune system and have revolutionized oncological treatment in recent years, with approval for its use in more and more cancers. However, it is not without side effects. Several neurological adverse events have been recognized associated with immune checkpoint inhibitors (ICI) and chimeric antigen receptor (CAR) T-cell therapy, the two main classes of cancer immunotherapy. With the increase in the prevalence of oncological diseases and this type of therapy, it is improbable that neu

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… Similar to non-ICI-related myasthenic syndromes, Nerve conduction study (NCS), including repetitive nerve stimulation, and single-fiber electromyography could be used to document neuromuscular junction compromise. …