journal
https://read.qxmd.com/read/38494301/preface
#1
EDITORIAL
Bruno Giometto, Sean J Pittock
No abstract text is available yet for this article.
2024: Handbook of Clinical Neurology
https://read.qxmd.com/read/38494300/foreword
#2
EDITORIAL
Michael J Aminoff, François Boller, Dick Swaab
No abstract text is available yet for this article.
2024: Handbook of Clinical Neurology
https://read.qxmd.com/read/38494299/overview-of-treatment-strategies-in-paraneoplastic-neurological-syndromes
#3
JOURNAL ARTICLE
Jeroen Kerstens, Maarten J Titulaer
Treatment strategies in paraneoplastic neurological syndromes rely on the three pillars of tumor treatment, immunotherapy, and symptomatic treatment, the first one being by far the most important in the majority of patients and syndromes. Classically, antibodies against extracellular antigens are directly pathogenic, and patients with these syndromes are more responsive to immunomodulatory or immunosuppressive treatments than the ones with antibodies against intracellular targets. This chapter first discusses some general principles of tumor treatment and immunotherapy, followed by a closer look at specific treatment options for different clinical syndromes, focusing on symptomatic treatments...
2024: Handbook of Clinical Neurology
https://read.qxmd.com/read/38494298/clinical-approach-to-diagnosis-of-paraneoplastic-neurologic-syndromes
#4
JOURNAL ARTICLE
Francesc Graus
The correct diagnosis of a paraneoplastic neurologic syndrome (PNS) first requires the identification of the syndrome as one of those defined as high-risk (previously called classical) or intermediate-risk for cancer in the 2021 PNS diagnostic criteria. Testing for neuronal antibodies should be restricted to these syndromes as indiscriminate request decreases the diagnostic value of the antibodies. Identifying onconeural (high-risk for cancer) or intermediate-risk for cancer antibodies supports the paraneoplastic diagnosis and mandates the search for an underlying cancer...
2024: Handbook of Clinical Neurology
https://read.qxmd.com/read/38494297/epidemiology-of-paraneoplastic-neurologic-syndromes
#5
JOURNAL ARTICLE
Robert Kadish, Stacey L Clardy
Paraneoplastic neurologic syndromes (PNS), initially depicted as seemingly cryptic remote manifestations of malignancy, were first described clinically in the early 20th century, with pathophysiologic correlates becoming better elucidated in the latter half of the century. There remain many questions not only about the pathophysiology but also regarding the epidemiology of these conditions. The continuous discovery of novel autoantigens and related neurologic disease has broadened the association in classical PNS to include conditions such as paraneoplastic cerebellar degeneration...
2024: Handbook of Clinical Neurology
https://read.qxmd.com/read/38494296/neurologic-manifestations-of-autoimmunity-with-immune-checkpoint-inhibitors
#6
JOURNAL ARTICLE
Jennifer A McCombe, Elia Sechi, Anastasia Zekeridou
Immune checkpoint inhibitors (ICIs) are cancer immunotherapies that enhance the body's own immune system to treat cancer. ICI treatment, however, can cause immune-related adverse events (irAEs) that can affect any organ, resulting in significant morbidity and mortality. Neurologic irAEs (nirAEs) are rare and can affect the peripheral nervous system more commonly than the central nervous system. Treatment is dependent on the severity of the neurologic manifestations and often includs discontinuation of the ICI and initiation of steroid therapy as the first line; other treatments have also been used...
2024: Handbook of Clinical Neurology
https://read.qxmd.com/read/38494295/paraneoplastic-neurologic-syndrome-and-autoantibody-accompaniments-of-germ-cell-tumors
#7
JOURNAL ARTICLE
M Bakri Hammami, Mohamed Rezk, Divyanshu Dubey
Paraneoplastic neurologic syndromes (PNSs) are a group of diseases affecting the central and/or peripheral nervous system caused by immune-mediated processes directed toward antigens with shared expression in tumor and neural tissue. Germ cell tumors (GCTs) are associated with PNSs with varied clinical phenotypes. Early diagnosis of PNS is vital to potentially uncover and treat underlying tumors, improving the chances of recovery, and preventing permanent neurologic complications. In this chapter, we outline the pathophysiology and epidemiology of PNS...
2024: Handbook of Clinical Neurology
https://read.qxmd.com/read/38494294/hematologic-malignancies-and-hematopoietic-stem-cell-transplantation
#8
JOURNAL ARTICLE
Chiara Briani, Andrea Visentin
Paraneoplastic neurologic syndromes are rarely associated with hematologic malignancies. In their rarity, lymphomas are the diseases with more frequent paraneoplastic neurologic syndrome. High-risk antibodies are absent in most lymphoma-associated paraneoplastic neurologic syndromes, with the exception of antibodies to Tr/DNER in paraneoplastic cerebellar degeneration, mGluR5 in limbic encephalitis, and mGluR1 in some cerebellar ataxias. Peripheral nervous system paraneoplastic neurologic syndromes are rare and heterogeneous, with a prevalence of demyelinating polyradiculoneuropathy in non-Hodgkin lymphoma...
2024: Handbook of Clinical Neurology
https://read.qxmd.com/read/38494293/paraneoplastic-neurologic-syndrome-associated-with-gynecologic-and-breast-malignancies
#9
JOURNAL ARTICLE
Elise Peter, Jérôme Honnorat, Virginie Desestret
Gynecologic and breast malignancies are the cancers most commonly associated with paraneoplastic neurologic syndromes, of which the foremost is Yo [Purkinje cell antibody, type 1 (PCA-1)] paraneoplastic cerebellar degeneration. Yo syndrome affects women in the sixth decade and manifests as a subacute severe cerebellar ataxia. The association of the typical clinical picture with the detection of Yo antibodies in a patient's serum or CSF defines the diagnosis. Yo syndrome is always associated with a cancer, and the search for the underlying tumor should focus on ovarian and breast cancers and be repeated overtime if negative...
2024: Handbook of Clinical Neurology
https://read.qxmd.com/read/38494292/paraneoplastic-neurologic-manifestations-of-neuroendocrine-tumors
#10
JOURNAL ARTICLE
Marco Zoccarato, Wolfgang Grisold
Neuroendocrine neoplasms (NENs) are a heterogeneous group of tumors arising from the transformation of neuroendocrine cells in several organs, most notably the gastro-entero-pancreatic system and respiratory tract. The classification was recently revised in the 5th Edition of the WHO Classification of Endocrine and Neuroendocrine Tumors. NENs can rarely spread to the central or peripheral nervous systems. Neurologic involvement is determined by the rare development of paraneoplastic syndromes, which are remote effects of cancer...
2024: Handbook of Clinical Neurology
https://read.qxmd.com/read/38494291/paraneoplastic-autoimmune-neurologic-disorders-associated-with-thymoma
#11
JOURNAL ARTICLE
Raffaele Iorio, Vanda A Lennon
Thymoma is often associated with paraneoplastic neurologic diseases. Neural autoantibody testing is an important tool aiding diagnosis of thymoma and its autoimmune neurologic complications. Autoantibodies specific for muscle striational antigens and ion channels of the ligand-gated nicotinic acetylcholine receptor superfamily are the most prevalent biomarkers. The autoimmune neurologic disorders associating most commonly with thymoma are myasthenia gravis (MG), peripheral nerve hyperexcitability (neuromyotonia and Morvan syndrome), dysautonomia, and encephalitis...
2024: Handbook of Clinical Neurology
https://read.qxmd.com/read/38494290/optimizing-the-diagnostic-performance-of-neural-antibody-testing-for-paraneoplastic-and-autoimmune-encephalitis-in-clinical-practice
#12
JOURNAL ARTICLE
Adrian Budhram, Eoin P Flanagan
The detection of neural antibodies in patients with paraneoplastic and autoimmune encephalitis has majorly advanced the diagnosis and management of neural antibody-associated diseases. Although testing for these antibodies has historically been restricted to specialized centers, assay commercialization has made this testing available to clinical chemistry laboratories worldwide. This improved test accessibility has led to reduced turnaround time and expedited diagnosis, which are beneficial to patient care...
2024: Handbook of Clinical Neurology
https://read.qxmd.com/read/38494289/antibodies-to-neural-cell-surface-and-synaptic-proteins-in-paraneoplastic-neurologic-syndromes
#13
JOURNAL ARTICLE
Adrian Budhram, Elia Sechi
Among patients with paraneoplastic neurologic syndromes (PNS), emphasis has historically been placed on neural antibodies against intracellular proteins that have a strong association with malignancy. Because of the intracellular location of their antigenic targets, these antibodies are typically considered to be non-pathogenic surrogate markers of immune cell-mediated neural injury. Unfortunately, patients with these antibodies often have suboptimal response to immunotherapy and poor prognosis. Over the last two decades, however, dramatic advancements have been made in the discovery and clinical characterization of neural antibodies against extracellular targets...
2024: Handbook of Clinical Neurology
https://read.qxmd.com/read/38494288/paraneoplastic-antibodies-targeting-intracellular-antigens
#14
JOURNAL ARTICLE
Jenny J Linnoila
Although they are relatively rare, the diagnosis of paraneoplastic neurologic syndromes (PNS) can be aided by the identification of neural autoantibodies in patients' serum and cerebrospinal fluid (CSF). They often clinically manifest as characteristic syndromes, including limbic encephalitis, opsoclonus-myoclonus syndrome, paraneoplastic cerebellar degeneration, and paraneoplastic encephalomyelitis. The antibodies are directed either toward intracellular targets, or epitopes on the cell surface. As compared to cell surface antibodies, intracellular paraneoplastic autoantibodies are more classically associated with cancer, most often lung, breast, thymoma, gynecologic, testicular, and/or neuroendocrine cancers...
2024: Handbook of Clinical Neurology
https://read.qxmd.com/read/38494287/pathogenesis-and-immunopathology-of-paraneoplastic-disorders
#15
JOURNAL ARTICLE
Valérie Quinot, Romana Höftberger
Paraneoplastic neurologic syndromes (PNS) represent a rare group of immune-mediated complications associated with an underlying tumor. Ectopic protein expression in neoplastic cells or an aberrant immune regulation in the course of hematooncologic diseases or thymomas trigger an autoimmune response that may affect any part of the central and/or peripheral nervous system. Recent advances in drug therapies as well as novel animal models and neuropathologic studies have led to further insights on the immune pathomechanisms of PNS...
2024: Handbook of Clinical Neurology
https://read.qxmd.com/read/38494286/paraneoplastic-myopathies
#16
JOURNAL ARTICLE
Andrew L Mammen
This chapter reviews the association between cancer and the idiopathic inflammatory myopathies (IIM), which includes dermatomyositis (DM), antisynthetase syndrome (ASyS), immune-mediated necrotizing myopathy (IMNM), and inclusion body myositis (IBM). Accumulating evidence shows that the risk of a coexisting malignancy is high in patients with DM, especially among those with anti-Tif1γ autoantibodies. Patients with IMNM and no defined autoantibodies also have an increased risk of malignancy. Recent evidence demonstrates that many IBM patients have increased numbers of circulating CD57+ CD8+ T cells, consistent with a diagnosis of large granular lymphocytic leukemia...
2024: Handbook of Clinical Neurology
https://read.qxmd.com/read/38494285/lambert-eaton-myasthenic-syndrome
#17
JOURNAL ARTICLE
Alexander F Lipka, Jan J G M Verschuuren
Lambert-Eaton myasthenic syndrome (LEMS) is a rare autoimmune disease characterized by proximal muscle weakness, loss of tendon reflexes, and autonomic dysfunction. Muscle weakness usually starts in the upper legs and can progress to oculobulbar and in severe cases respiratory muscles. P/Q-type voltage-gated calcium channels (VGCCs) localized in the presynaptic motor nerve terminal and in the autonomic nervous system are targeted by antibodies in LEMS patients. These antibodies can be detected in about 90% of patients, and the presence of decrement and increment upon repetitive nerve stimulation is also a highly sensitive diagnostic test...
2024: Handbook of Clinical Neurology
https://read.qxmd.com/read/38494284/introduction-and-overview-of-immunopathological-mechanisms-and-future-directions
#18
JOURNAL ARTICLE
Sean J Pittock, Bruno Giometto
Paraneoplastic neurological disorders represent a significant part of the field of autoimmune neurology. Most neural autoantibodies discovered to date are associated with underlying malignancy and in that context are considered paraneoplastic antibody biomarkers. These autoantibodies can be divided into two major categories: those that target intracellular proteins (not pathogenic) and those that target plasma membrane proteins (pathogenic). Disorders accompanied by the former are mediated primarily by neural peptide-specific cytotoxic T-cells, are commonly associated with cancer, and are poorly responsive to immunotherapy...
2024: Handbook of Clinical Neurology
https://read.qxmd.com/read/38494283/myasthenia-gravis-pathophysiology-diagnosis-and-treatment
#19
JOURNAL ARTICLE
Martijn R Tannemaat, Maartje G Huijbers, Jan J G M Verschuuren
Myasthenia gravis (MG) is an autoimmune disease characterized by dysfunction of the neuromuscular junction resulting in skeletal muscle weakness. It is equally prevalent in males and females, but debuts at a younger age in females and at an older age in males. Ptosis, diplopia, facial bulbar weakness, and limb weakness are the most common symptoms. MG can be classified based on the presence of serum autoantibodies. Acetylcholine receptor (AChR) antibodies are found in 80%-85% of patients, muscle-specific kinase (MuSK) antibodies in 5%-8%, and <1% may have low-density lipoprotein receptor-related protein 4 (Lrp4) antibodies...
2024: Handbook of Clinical Neurology
https://read.qxmd.com/read/38494282/paraneoplastic-autonomic-neuropathies-and-gi-dysmotility
#20
JOURNAL ARTICLE
Elisabeth P Golden, Steven Vernino
A number of the well-recognized autoimmune and paraneoplastic neurologic syndromes commonly involve the autonomic nervous system. In some cases, the autonomic nerves or ganglia are primary targets of neurologic autoimmunity, as in immune-mediated autonomic ganglionopathies. In other disorders such as encephalitis, autonomic centers in the brain may be affected. The presence of autonomic dysfunction (especially gastrointestinal dysmotility) is sometimes overlooked even though this may contribute significantly to the symptom burden in these paraneoplastic disorders...
2024: Handbook of Clinical Neurology
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