Paraneoplastic Neurological Disorders

2017 ◽  
Author(s):  
John C. Probasco
Keyword(s):  
Nervous System ◽  
Autonomic Nervous System ◽  
Diagnostic Test ◽  
Neurological Disorders ◽  
Clinical Presentation ◽  
Test Results ◽  
Patients With Cancer ◽  
Immune Mediated ◽  
Paraneoplastic Neurological Disorders ◽  
Symptoms And Signs

Paraneoplastic neurological disorders (PNDs) are estimated to affect approximately 0.01% of all patients with cancer. The majority of PNDs are thought to be the byproduct of immune-mediated processes directed against tumor-related antigens, processes which are sometimes effective against a systemic cancer. The inciting cancer is often asymptomatic or occult, with patients presenting to the neurologist with a variety of neurological symptoms and signs depending on the area(s) of the central, peripheral, and autonomic nervous system involved. The diagnosis of a PND is reserved for patients with histologically proven cancer; however, clinical presentation and diagnostic test results may make the diagnosis of a PND highly probable in the absence of the diagnosis of a cancer.

scholarly journals Exploration of Anti-Yo and Anti-Hu paraneoplastic neurological disorders by PhIP-Seq reveals a highly restricted pattern of antibody epitopes

2018 ◽  
Author(s):  
Brian D O'Donovan ◽  
Caleigh Mandel-Brehm ◽  
Sara E Vazquez ◽  
Jamin Liu ◽  
Audrey V Parent ◽  
...  
Keyword(s):  
Immune Response ◽  
Neurological Disorders ◽  
Diagnosis And Prognosis ◽  
Clinical Assay ◽  
Immune Mediated ◽  
Proper Diagnosis ◽  
Paraneoplastic Neurological Disorders ◽  
Restricted Pattern ◽  
Antibody Epitopes ◽  
Potential Biomarkers

Paraneoplastic neurological disorders (PNDs) are immune-mediated diseases of the nervous system understood to manifest as part of a misdirected anti-tumor immune response. Identifying PND-associated autoantibodies and their cognate antigens can assist with proper diagnosis and treatment while also enhancing our understanding of tumor-associated immune processes, triggers for autoimmune disease, and the functional significance of onconeuronal proteins. Here, we employed an enhanced version of phage display immunoprecipitation and sequencing (PhIP-Seq) leveraging a library of over 731,000 unique phage clones tiling across the entire human proteome to detect autoantibodies and create high-resolution epitope profiles in serum and CSF samples from patients suffering from two common PNDs, the anti-Yo (n = 36 patients) and anti-Hu syndromes (n = 44 patients). All patient samples positive for anti-Yo antibody by a validated clinical assay yielded polyspecific enrichment of phage presenting peptides from the canonical anti-Yo (CDR2 and CDR2L) antigens, while 38% of anti-Hu patients (17/44) had a serum and/or CSF sample that significantly enriched peptides deriving from the ELAVL family of proteins, the anti-Hu autoantigenic target. The anti-Hu antibodies showed a remarkably convergent antigenic signature across 15/17 patients corresponding to residues surrounding and including the degenerate motif, RLDxLL, shared by ELAVL2, 3 and 4. Lastly, PhIP-Seq identified several known and novel autoantigens in these same patient samples, representing potential biomarkers that could aid in the diagnosis and prognosis of PND and cancer.

Neuroimmunology

2019 ◽  
Keyword(s):  
Neurological Disorders ◽  
Clinical Presentation ◽  
Diagnostic Testing ◽  
Mechanisms Of Action ◽  
Diverse Group ◽  
Neuromyelitis Optica Spectrum Disorder ◽  
Inflammatory Myopathies ◽  
Immune Mediated ◽  
Adults And Children

This book provides clinical and supporting scientific background on a diverse group of neurological disorders in an expanding field of neurology, that of neuroimmunology. It includes chapters on multiple sclerosis and related disorders in adults and children, neuromyelitis optica spectrum disorder, Guillain-Barré syndrome, chronic inflammatory deymyelinating polyradiculoneuropathy and variants, immune-mediated disorders of the neuromuscular junction, inflammatory myopathies, paraneoplastic disorders and autoimmune encephalitities, and neurologic manifestations of systemic immune-mediated diseases. In addition there is an introductory chapter dealing with basic of immunology and another on mechanisms of action of therapies used in neuroimmunologic disorders. The clinical chapters cover epidemiology, pathology, pathogenesis, and pathophysiology of the different diseases along with clinical presentation, diagnostic testing, differential diagnosis, and treatment.

scholarly journals Ischemic and nonischemic manifestations of antiphospholipid syndrome

2015 ◽  
Vol 96 (1) ◽  
pp. 61-69 ◽  
Author(s):  
S I Tukhfatullina ◽  
D D Gaynetdinova
Keyword(s):  
Nervous System ◽  
Pregnant Women ◽  
Antiphospholipid Syndrome ◽  
Neurological Disorders ◽  
Antiphospholipid Antibodies ◽  
Cerebrovascular Disorders ◽  
Central Nervous System Damage ◽  
Immune Mediated ◽  
Mother And Child ◽  
Cerebral Pathology

Hypercoagulability syndrome is an actual multidisciplinary problem of the last decade. Blood clotting disorders leading to hypercoagulability syndrome may manifest as different diseases in neurology, obstetrics, rheumatology, surgery, including diseases of pregnancy. Antiphospholipid syndrome is the most common form of hypercoagulability syndrome, which often develops at a young age, in children and even infants, with female-male ratio of 5. Causes and pathogenesis of antiphospholipid syndrome are not fully clear. This problem is especially important for pregnant women. Almost a third of refractory pregnancy losses are associated with antiphospholipid antibodies formation. Main manifestations of this condition include thrombotic events during pregnancy and the postpartum period, including cerebral pathology in both mother and child. Central nervous system damage in this condition may have both ischemic (cerebrovascular accident) and nonischemic (primary immune-mediated damage of the nervous system) genesis. The variety of neurological disorders associated with antiphospholipid syndrome is very wide, from cerebrovascular disorders, migraine and migrainous headaches to chorea and seizures. Headaches are the most common reason for pregnant women to seek neurologic help. Pregnancy associated with high titers of antiphospholipid antibodies often end as early gestation stage stillbirth. Children born to mothers with antiphospholipid syndrome in 20% of cases develop neurological symptoms of ischemic and non-ischemic origin. Thus, antiphospholipid syndrome requires special attention for early and timely diagnosis, especially in women planning pregnancy and in pregnant women for the preventing serious complications, both in mother and fetus.

Exploring autonomic nervous system dysfunction in patients with cancer cachexia: A pilot study

2012 ◽  
Vol 166 (1-2) ◽  
pp. 93-95 ◽  
Author(s):  
Alpna Chauhan ◽  
Ashika Sequeria ◽  
Cathann Manderson ◽  
Matthew Maddocks ◽  
David Wasley ◽  
...  
Keyword(s):  
Nervous System ◽  
Autonomic Nervous System ◽  
Pilot Study ◽  
Cancer Cachexia ◽  
Autonomic Nervous System Dysfunction ◽  
Autonomic Nervous ◽  
Patients With Cancer

scholarly journals Autonomic Involvement in Subacute and Chronic Immune-Mediated Neuropathies

2013 ◽  
Vol 2013 ◽  
pp. 1-7 ◽  
Author(s):  
Anna Mazzeo ◽  
Claudia Stancanelli ◽  
Rita Di Leo ◽  
Giuseppe Vita
Keyword(s):  
Nervous System ◽  
Autonomic Nervous System ◽  
Autonomic Function ◽  
Signs And Symptoms ◽  
Autonomic Functions ◽  
Autonomic Nervous ◽  
Immune Mediated ◽  
Chronic Course

Autonomic function can be impaired in many disorders in which sympathetic, parasympathetic, and enteric arms of the autonomic nervous system are affected. Signs and symptoms of autonomic involvement are related to impairment of cardiovascular, gastrointestinal, urogenital, thermoregulatory, sudomotor, and pupillomotor autonomic functions. Availability of noninvasive, sensitive, and reproducible tests can help to recognize these disorders and to better understand specific mechanisms of some, potentially treatable, immune-mediated autonomic neuropathies. This paper describes autonomic involvement in immune-mediated neuropathies with a subacute or chronic course.

Peripheral nerve hyperexcitability syndromes

2015 ◽  
Vol 26 (2) ◽  
Author(s):  
Cem Ismail Küçükali ◽  
Murat Kürtüncü ◽  
Halil İbrahim Akçay ◽  
Erdem Tüzün ◽  
Ali Emre Öge
Keyword(s):  
Nervous System ◽  
Peripheral Nerve ◽  
Immune Modulation ◽  
Symptomatic Treatment ◽  
Immune Mediated ◽  
Morvan’S Syndrome ◽  
Peripheral Nerve Hyperexcitability ◽  
The Central Nervous System ◽  
Electrophysiological Findings ◽  
Symptoms And Signs

AbstractPeripheral nerve hyperexcitability (PNH) syndromes can be subclassified as primary and secondary. The main primary PNH syndromes are neuromyotonia, cramp-fasciculation syndrome (CFS), and Morvan’s syndrome, which cause widespread symptoms and signs without the association of an evident peripheral nerve disease. Their major symptoms are muscle twitching and stiffness, which differ only in severity between neuromyotonia and CFS. Cramps, pseudomyotonia, hyperhidrosis, and some other autonomic abnormalities, as well as mild positive sensory phenomena, can be seen in several patients. Symptoms reflecting the involvement of the central nervous system occur in Morvan’s syndrome. Secondary PNH syndromes are generally seen in patients with focal or diffuse diseases affecting the peripheral nervous system. The PNH-related symptoms and signs are generally found incidentally during clinical or electrodiagnostic examinations. The electrophysiological findings that are very useful in the diagnosis of PNH are myokymic and neuromyotonic discharges in needle electromyography along with some additional indicators of increased nerve fiber excitability. Based on clinicopathological and etiological associations, PNH syndromes can also be classified as immune mediated, genetic, and those caused by other miscellaneous factors. There has been an increasing awareness on the role of voltage-gated potassium channel complex autoimmunity in primary PNH pathogenesis. Then again, a long list of toxic compounds and genetic factors has also been implicated in development of PNH. The management of primary PNH syndromes comprises symptomatic treatment with anticonvulsant drugs, immune modulation if necessary, and treatment of possible associated dysimmune and/or malignant conditions.

scholarly journals When do the symptoms of autonomic nervous system malfunction appear in patients with Parkinson’s disease?

2014 ◽  
Vol 71 (4) ◽  
pp. 346-351 ◽  
Author(s):  
Luka De ◽  
Marina Svetel ◽  
Tatjana Pekmezovic ◽  
Branislav Milovanovic ◽  
Vladimir Kostic
Keyword(s):  
Nervous System ◽  
Autonomic Nervous System ◽  
Autonomic Neuropathy ◽  
Rating Scale ◽  
Clinical Presentation ◽  
De Novo ◽  
The Body ◽  
Factors Affecting ◽  
Autonomic Nervous ◽  
Sympathetic Dysfunction

Background/Aim. Dysautonomia appears in almost all patients with Parkinson?s disease (PD) in a certain stage of their condition. The aim of our study was to detect the development and type of autonomic disorders, find out the factors affecting their manifestation by analyzing the potential association with demographic variables related to clinical presentation, as well as the symptoms of the disease in a PD patient cohort. Methods. The patients with PD treated at the Clinic of Neurology in Belgrade during a 2-year period, divided into 3 groups were studied: 25 de novo patients, 25 patients already treated and had no long-term levodopa therapy-related complications and 22 patients treated with levodopa who manifested levodopa-induced motor complications. Simultaneously, 35 healthy control subjects, matched by age and sex, were also analyzed. Results. Autonomic nervous system malfunction was defined by Ewing diagnostic criteria. The tests, indicators of sympathetic and parasympathetic nervous systems, were significantly different in the PD patients as compared with the controls, suggesting the failure of both systems. However, it was shown, in the selected groups of patients, that the malfunction of both systems was present in two treated groups of PD patients, while de novo group manifested only sympathetic dysfunction. For this reason, the complete autonomic neuropathy was diagnosed only in the treated PD patients, while de novo patients were defined as those with the isolated sympathetic dysfunction. The patients with the complete autonomic neuropathy differed from the subjects without such neuropathy in higher cumulative and motor unified Parkinson?s disease rating score (UPDRS) (p < 0.01), activities of daily living scores (p < 0.05), Schwab-England scale (p < 0.001) and Hoehn-Yahr scale. There was no difference between the patients in other clinical-demographic characteristics (sex, age at the time of diagnosis, actual age, duration of disease, involved side of the body, pain and freezing), but mini mental status (MMS) score and Hamilton depression and anxiety rating scale were significantly lower (p < 0.05). Conclusion. Our results confirm a high prevalence of autonomic nervous system disturbances among PD patients from the near onset of disease, with a predominant sympathetic nervous system involvement. The patients who developed complete autonomic neuropathy (both sympathetic and parasympathetic) were individuals with considerable level of functional failure, more severe clinical presentation and the existing anxiety and depression.

Molecular Genetic Testing in Pediatric and Adult Neurology in Iraq: New Experience and Challenges from a Developing Country

2020 ◽  
Author(s):  
Nebal Waill Saadi ◽  
Batool Ali Ghalib Yassin ◽  
Nawal Makhseed ◽  
Ameer Shaker Hadi
Keyword(s):  
Genetic Testing ◽  
Developing Country ◽  
Neurological Disorders ◽  
Medical Records ◽  
Clinical Presentation ◽  
Genetic Test ◽  
Molecular Genetic ◽  
Demographic Characteristics ◽  
Test Results ◽  
Molecular Genetic Testing

AbstractInherited neurological disorders are reasonably common in pediatric and adult neurology practices. Genetic testing for such disorders does carry promise, but is fraught with challenges and difficulties. This study was conducted to assess the utility of genetic testing in a cohort of 200 patients who had neurological disorders, suspected to be of inherited origin, and for whom molecular genetic testing was requested during the period 2014 to 2019. Two hundred and eight tests were ordered. The characteristics of that practice were reviewed. Further, we summarized the challenges and highlighted the concerns that were raised. The medical records of 200 patients were retrieved and reviewed to assess the demographic characteristics of the cohort, their clinical presentation, genetic test requested for each, and the interpretation of the test results.

Paraneoplastic and Idiopathic Autoimmune Neurological Disorders Affecting the Central Nervous System and Autoimmune Dysautonomia

2019 ◽  
pp. 245-265
Author(s):  
Nicholas L. Zalewski ◽  
Sean J. Pittock
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Neurological Disorders ◽  
Neurological Diseases ◽  
Diagnostic Evaluation ◽  
Rapid Rate ◽  
Disease Category ◽  
Cns Disorders ◽  
Immune Mediated ◽  
The Central Nervous System

This chapter is an examination of immune-mediated central nervous system (CNS) disorders, which have increasingly been recognized as a critical disease category in the field of neurology. The chapter looks at clinical presentation, diagnostic evaluation, and treatment. The chapter also looks to the future. The field of immune-mediated neurological diseases is rapidly growing. New autoantibodies are being discovered at a rapid rate, helping unveil the mystery behind the challenging neurological presentations in many patients.

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